Severe Combined Immune Deficiency: Early Hospitalisation and Isolation

Severe Combined Immune Deficiency

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

This book is dedicated to all children with severe combined immunodeficiency and their families. It has been a privilege caring for them, knowing them, and in some small way sharing their experiences. They taught me far more about living and life, than I taught them about the immune deficiencies. Secondly, it is dedicated to Michael Westall, an old friend who always believed in me and encouraged me to push myself.

Severe Combined Immune Deficiency Early Hospitalisation and Isolation

Peter S. Vickers Cert.Ed., Dip C.D., SRN, RSCN, BA, PhD, FHEA Visiting Fellow in Child Health and Immunology School of Nursing and Midwifery The University of Hertfordshire

A John Wiley & Sons, Ltd., Publication

This edition first published 2009
C 2009 John Wiley & Sons, Ltd Wiley-Blackwell is an imprint of John Wiley & Sons, formed by the merger of Wiley’s global Scientific, Technical and Medical business with Blackwell Publishing. Registered office John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, United Kingdom Editorial office John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, United Kingdom For details of our global editorial offices, for customer services and for information about how to apply for permission to reuse the copyright material in this book please see our website at www.wiley.com/wiley-blackwell. The right of the author to be identified as the author of this work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books. Designations used by companies to distinguish their products are often claimed as trademarks. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book. This publication is designed to provide accurate and authoritative information in regard to the subject matter covered. It is sold on the understanding that the publisher is not engaged in rendering professional services. If professional advice or other expert assistance is required, the services of a competent professional should be sought. Library of Congress Cataloging-in-Publication Data Vickers, Peter S. Severe combined immune deficiency : early hospitalisation and isolation / Peter S. Vickers. p. ; cm. Includes bibliographical references and index. ISBN 978-0-470-31986-4 (pbk. : alk. paper) 1. Severe combined immunodeficiency – Patients – Hospital care – Psychological aspects. 2. Immunological deficiency syndromes in children – Patients – Hospital care – Psychological aspects. I. Title. [DNLM: 1. Severe Combined Immunodeficiency – psychology. 2. Child, Hospitalized – psychology. 3. Child. 4. Family – psychology. 5. Patient Isolation – psychology. WD 308 V637s 2009] RJ387.D42V53 2009 618.92 979–dc22 2008019027 A catalogue record for this book is available from the British Library. Set in 10/12 pt Palatino by Aptara
Inc., New Delhi, India Printed in Singapore by Markono Print Media Pte Ltd R

1 2009

Contents

Preface Acknowledgements

vii xi

Chapter 1

Introduction to the Family

1

Chapter 2

Background to the Study on Children with SCID and Their Families

7

Chapter 3

Severe Combined Immunodeficiency – A Brief Overview

29

Chapter 4

Effects of Early Hospitalisation on Children

48

Chapter 5

The Effects of Isolation and Bone Marrow Transplantation on the Child and Family

60

Chapter 6

Children’s Perceptions of Illness and Death

78

Chapter 7

Stressors and Coping Mechanisms

101

Chapter 8

Psychosocial Effects on the Children

129

Chapter 9

Anger, Aggression and Antisocial Behaviour in Children

160

Chapter 10

Neurotic and Depressive Behaviour in Children

177

Chapter 11

Psychosocial Effects on the Family

193

Chapter 12

Attachment and Alienation

223

v

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Contents

Chapter 13

Post-Traumatic Stress Disorder and Children

243

Chapter 14

Post-Traumatic Stress Disorder in Families

256

Chapter 15

Conclusion

279

Appendix Glossary References Index

285 297 303 334

Preface

Experience is viewed as a source of human significance and this involves others accepting it as perceived by the individual concerned (Jerrett 1994 p. 1054). The genesis of this book arose from research carried out towards my doctorate into the post-transplant experiences of a group of children with severe combined immunodeficiency syndrome (SCID), and so the central focus of this book deals with the long-term quality of life experienced by those children who have survived bone marrow transplantation (BMT) for SCID. However, experiences of working with children and their families with various other disorders have shown that the problems experienced by the children with SCID and their families are not unique. Any child who undergoes hospitalisation at an early age, particularly if there is some degree of isolation involved (although just being in hospital infers that there is some isolation because the child is separated from all that is familiar) is at risk of facing the same stressful events and experiences as is the child with SCID, and consequently developing similar psychosocial sequelae as these children. Such similar experiences can be found in children who have required intensive care as a neonate or a young child, as well as those children who have been hospitalised as a result of trauma or such diseases as cancer. In addition, these potential problems can arise with children who have chronic diseases, such as asthma, cystic fibrosis and diabetes, and who require many hospitalisations due to acute exacerbations of their chronic disease. The parents of these children will also undergo similar experiences to the parents of the children with SCID, and so consequently will have to cope with the many psychosocial problems that can occur when one has children with such acute or chronic diseases. This book will explore the stressful factors that are linked to early, long-term hospitalisation and isolation and their potential effects upon the children and their families. The first chapter will briefly explore the importance of the family in the context of a child with chronic or acute ill health requiring hospitalisation, vii

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and in particular will look at the concept of family-centred care of children in hospital. Because the focus of the book is centred upon children with SCID, the second chapter will give an overview of the background to the research study on the children and their families as to the effects of being treated for, and surviving, SCID, as well as looking at how the study was undertaken and a brief r´esum´e of the results. The stress and problems engendered with SCID and BMT in the early days of the children’s treatment are included in order to lead to a fuller understanding of the effects such treatment has on the families and their children’s subsequent physical and psychosocial development. This section introduces previously unpublished research on the children in the study in order to be able to compare the stresses engendered at the time of the bone marrow transplant with the present situation. Methods of investigation include parental interviews and questionnaires, teacher questionnaires, children’s drawings and medical/nursing accounts as evidenced by the children’s medical notes and personal discussion. A discussion on the merits of qualitative research is included, in addition to reasons for the use of qualitative data as well as quantitative data. Chapter 3 consists of a brief introduction to the physiology of the immune system and to the disorder known as severe combined immunodeficiency (SCID). In this overview the causes, pathophysiology, signs and symptoms, diagnosis, care and treatment of these disorders are explored. A glossary of some of the terms mentioned in this chapter and Chapter 3 will be found at the end of the book. Chapters 4 and 5 discuss the psychosocial effects of early hospitalisation, isolation and BMT on children and their families. As there is little published material concerning the psychosocial effects of bone marrow transplants for SCID, these themes are further explored by looking at children who are hospitalised and isolated (and possibly have bone marrow or solid organ transplants) for a range of other disorders, including cancer. Several psychosocial problems exist within each of these situations, and these two chapters are concerned with an exploration of these problems within the context of children with SCID and other disorders. Chapter 6 looks at children’s perceptions of health and of death. Whilst the children with SCID underwent their most acute ill health and treatment at an age when they are neither consciously nor cognitively aware of what they are going through, many of them in later childhood are still suffering physically and psychosocially from some of the effects of their disorder and treatment. Children with other disorders, where the onset of the illness and the treatment occur at a later age certainly have perceptions of health and of their own mortality, and as health care professionals we are beholden to listen to them and to allow them to express their feelings and concerns. Chapter 7 is concerned with the stresses experienced by children with chronic and acute ill health, and their families, and how they can find the resources to cope with the stressors. Suitable theoretical frameworks to underpin the study are investigated and an adaptation of those models put forward by Wallander et al. (1989) and Pot-Mees (1989) is suggested

Preface

ix

as a useful model for stress and coping in the acutely ill child who progresses to subsequent long-term illness. Within this model, the stressors and responses (or risk and resistance factors), such as disease parameters, psychosocial and intrapersonal stresses and coping mechanisms, as well as socio-ecological factors, are identified in relation to children with SCID, and other similar disorders, and their families. The next chapter (Chapter 8) is concerned with the general psychosocial effects that hospitalisation (and, in some cases, isolation) has on young children and on their families. Again, these are linked to the research study of children with SCID and their families, and this chapter explores the many facets of psychosocial development following their treatment. As with almost all the chapters in the book, links are made with children who have other disorders requiring early hospitalisation. In particular, comparisons are made between children with SCID who were treated in the UK and those who were treated in Germany, and the reasons for differences in psychosocial functioning discussed. This is important because there were found to be many differences between the UK and German children, with the UK children experiencing many more psychosocial problems. The following two chapters explore two different types of psychosocial behaviour experienced by children in the SCID study, and also children with other similar disorders. Chapter 9 looks at the incidence of anger and aggression in children, and, as with the previous and next chapters, explores these phenomena in the words and pictures of the children and their parents, whilst Chapter 10 discusses the depressive and neurotic behaviours that were identified within this group of children. Because this book is concerned not only with the children but also their families, then Chapter 11 examines the psychosocial effects on families whose children had successfully been treated for SCID. This is important because some of the parents in the SCID study were also found to be experiencing many psychosocial problems, and similar psychosocial problems has been found in the parents of children who had been treated for other serious disorders that required long-term hospitalisation, or multiple hospitalisations at an early age. Children with SCID are in a state of physical and semi-social isolation for much of their infancy and early childhood. Because of this situation they inevitably miss out on much of their social development. Thus, the seeds of the feelings of isolation and alienation within this group of children with SCID are sown from the very beginning of their diagnosis and treatment, and Chapter 12 discusses this phenomenon. Added to this are all the various illnesses and disabilities encountered post-transplant, as well as the overprotection emanating from many of the parents. All this can lead children who had SCID into an ever downwards spiral of alienation from their peers, from society and even, at times, from their own families, until a situation is reached in which feelings of acute depression, rejection and isolation are manifested, as seen in some of the adolescent children in the SCID study.

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Preface

Finally, Chapters 13 and 14 examine the possibility that for children and for parents, the situations experienced in hospital have resonances with people suffering from a post-traumatic stress disorder (PSTD). In addition, the themes which came out of the actual interviews that made up part of the SCID study had very striking parallels with PSTD. For the majority of the parents, their child’s diagnosis, illness, bone marrow transplant and supportive treatment were assessed as elements of a traumatic event. Without appropriate support and counselling, however, they had not had an opportunity to work through their psychological problems. It was obvious that there was re-experiencing of the event, along with intrusive thoughts and a variety of emotional and cognitive responses. Thus, in the SCID study, some of the parents appeared to have a form of post-traumatic stress syndrome, even many years after the transplant, as also did some of the children. Again there are parallels with children who have other similar disorders (similar in terms of the experiences that are linked with their illnesses) and their families. The final chapter (Chapter 15) concludes by summarising the major themes that have arisen during the course of this book, and looks at the quality of life that many of these children, and their families, may attain.

Acknowledgements

Writing can be a very solitary occupation, but at the same time, a book such as this needs the assistance, support and input of many people, including family, colleagues and friends, all of whom play some part – however small or large – in its conception and production. Consequently, for this book, there are so many people whose contribution I must acknowledge, most importantly all the children and their parents who so kindly and generously agreed to be involved in my original research and to discuss their experiences during their time in hospital, and afterwards. I am indebted to you all for allowing me to enter your lives and to reopen, sometimes painful, memories. For all your courage, openness, consideration and sincerity, as well as the coffee and cakes, I am truly grateful. Your courage and compassion is awe-inspiring, for without your co-operation there would have been no study, and this book would never have been conceived, let alone written. There were many other people who helped me with the original research, whose contributions were vital and much appreciated. These include particularly Carol Kennelly and Wendy Larmouth who undertook some of the interviews of children and families for me, and my supervisors at the University of Northumbria, Dr Brian Bell, Dr Rob Heyman, Professor Don Watson and Dr Sarah Nettleton, whose help, support and advice was always very much appreciated. Throughout my study, I also had much help, support and advice from specialist immunology health care professionals in the UK and Germany, and I would like to thank them all for this. These were Professor Dr Wilhelm Friedrich and the staff of Station VII, Universit¨atskinderklinik, Ulm, Dr Andrew Cant and the staff of the Bone Marrow Transplant Unit, Newcastle General Hospital, Newcastle-upon-Tyne, and Professor Stephan Strobel and Professor Roland Levinsky, of the Hospital for Sick Children, Great Ormond Street, London. In addition, I would also like to thank Dr Penny Titman, also of the Hospital for Sick Children, Great Ormond Street, London, who very kindly agreed to discuss the findings xi

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Acknowledgements

from her research into children with the same condition and which has just been concluded. From the University of Hertfordshire, my thanks also go to Liz GormleyFleming for her help on the chapter concerned with Quality of Life, and to Ian Peate for all his support and enthusiasm, and for his encouragement of me to write this book based on my research. Last, but certainly by no means least, special thanks go to Lisa Whiting, also of the University of Hertfordshire, who very kindly read everything I wrote, and offered invaluable suggestions and support over the past 12 months. To you all, I would just like to say ‘thank you’. Without your help and support the original research and the present book would not have happened.

1

Introduction to the Family

Introduction In 1951, an eight-year-old boy was rushed to hospital in the middle of the night following severe head trauma. That boy’s parents had been told that there was no hope of his surviving because of the extent of the brain damage. In actual fact, the exact words used to his parents were that he would ‘only leave the hospital in a coffin’. In spite of that, because he was admitted into the hospital in the early hours of a Sunday morning, it was to be another week before his parents were able to visit him. At that time, parents could only visit their children in hospital for one hour on a Saturday afternoon, and obviously no exceptions were to be made, even in this particular case where the boy could have died at any moment. Nethercott (1993) says that the situation of parents not being able to visit their children more than once a week, a situation that was normal at that time, occurred because it was believed that parental visits would actually inhibit the effective care given by the health care professionals. In this way, it was thought that they would be detrimental to the child – in particular a child would become distressed when the parents ‘abandoned’ him or her and returned home (Alsop-Shields 1998, Johnson 1990a). That boy did not die; otherwise he would not have been able to write this book, for I was that boy. For the 12 months following the accident I was unable to go to school. Indeed it was a further 12 months following my return to school before I was allowed to play with any other children because my parents and I had been told that another bang in the same area of the skull and there would be no second chance. So this book is a journey not only for health professionals and others to make in the understanding of the effects of early hospitalisation in isolation on children and families, but also for me to try and understand the effects on my life of one of the defining experiences of my life. Thankfully, such rigid regimes are no longer in operation in the care of children in hospital. The work of such pioneers as the Robertsons in the late 1940s and early 1950s, later to be supported by Platt (1959), has brought Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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about a complete change in the psychosocial care of children in hospital. Today, the importance of the family in the care of their children is universally accepted by health care professionals in the UK, and as Darbyshire (1994) reports, the process of change has resulted in a humanisation of paediatrics.

What Is a Family? Much of this book is concerned with the family and the relationships within a family, so the question to be asked is ‘what is a family?’ For many years the ideal family is said to have been a mother, a father and a number of children of that mother and father. Indeed, Murdoch in 1949 (cited by Whiting 2007 p. 8) defined a family as ‘a social group characterised by common residence, economic co-operation and reproduction. It includes adults of both sexes, at least two of whom maintain a socially approved sexual relationship and one or more children, own or adopted, of the sexually cohabiting adults’. Many of us now accept that families can differ greatly from that definition, and Whiting (2007 p. 8) points out that ‘it could be argued that this view (of Murdoch) reflected society at the time of writing but does not reflect the diversity of lifestyles that are now prevalent in the 21st century’. At the end of the twentieth century, the United Nations (1998 Article 16[3]) defined the family as ‘the natural and fundamental group unit of society and (which) is entitled to protection by society and the state’. Earlier, Miller (1978 p. 281) defined the family as ‘a set of interacting units with relationships among them’, whilst Richards (1995) suggested that families were likely to consist of interconnected people amongst whom are to be found emotional bonds and psychologically meaningful social interactions (Whiting 2007). It is obvious from these definitions that a family, whatever else it might be, is a group of people who are emotionally connected with strong relationship bonds. However, the same can be said of people who are very good friends. So, what is it that makes a family different from a group of friends? In the past, it was perhaps easier, because it would have been expected that the two adults in a family unit would have gone through a ceremony binding them together, i.e. a marriage ceremony of some sort. This ceremony would have the full support of the participants’ religion and the secular state. But now, that is no longer a requisite for people to consider themselves as a family. Nor can we think in terms of sexual relations between the adults as being a defining feature, because these may well take place between adults who are friends. Perhaps we need to think of the family as being the centre of a whole web of interconnecting relationships. In this scenario, the nuclear family (parents and their children) is a tight knit group which is self-contained and dependent upon one another, whilst not completely excluding others. Surrounding this nuclear family is the extended family, consisting of relationships that may be very close and emotionally binding, but not to the same extent as the relationships within the nuclear family. The extended

Introduction to the Family

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family consists of people such as grandparents, aunts, uncles, cousins, nephews and nieces. For some families, the extended family is almost as important as the nuclear family, whilst for other families, the relationships between members of the nuclear family and members of the extended family are much looser, and less emotionally binding. Outside of the extended family, there then exists a network of friends. However, relationships with some friends may be more intense than relationships with some members of the extended family, so it all gets very complicated.

The Structure of the Family As mentioned above, the make-up of the family has changed considerably over the last 50 years or so, and as Whiting (2007) notes, many of the children who are now requiring health care no longer live within a ‘traditional’ family unit (i.e. mother, father and the children of those two adults). Carling (2002) has described twenty-first century families in the UK as being complex structures, many of which have developed a variety of new structures. Whiting (2007) identified some of the factors that have influenced the structure and family dynamics in the UK at this time:

r The increasing elderly population is one factor, as this may lead to

r

r r

r

r r

children forming close relationships with previous generations in their family – such as grandparents and great-grandparents. However, because in the UK, nuclear families are often very mobile, as a society as is whole, and therefore children may live a long way away from their elderly relatives. Whiting also mentions the widening childbearing age that has occurred in recent years. The UK has the highest rate of teenage pregnancies in Western Europe (UNICEF 2001), whilst at the same time women are having children much later in life, many in their 40s. Marriage is no longer seen as a necessary requirement for the adults in a family or for the bringing up of children within that family. Parents are now more prepared to separate from each other and to take new partners, each of whom can bring children from their previous partnerships to the family. Thus, children can have a plethora of stepsiblings and half siblings, as well as multiple fathers and/or mothers – not forgetting multiple members of the extended family. Single sex relationships within a family are more accepted (these days sometimes involving firm commitments between the adults, such as civil partnerships), and it is possible for these single sex partners to bring up children as part of their family who are the result of surrogacy, in vitro fertilisation, sperm donation, adoption, or fostering. Because the UK is now very much a multicultural and multiracial society, there are a variety of differing family organisations and structures. For sometime now, it has been accepted that both parents will work outside of the home, and this can lead to children within the family

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forming close relationships with others, such as childminders, nursery nurses, friends (both child and adult friends) and even pets. All, or any, of these may be perceived by the child as important members of the family. In practical terms, as a paediatric nurse, I always considered the family of a child in my unit to be what the family itself considered that to be. That then allowed me to work with the family (as a unit or as individuals) to help their child in hospital. Whatever the particular organisation of any family, Bernardes (1997) points out that most people still consider their family to be the most important element of their lives, whilst Whiting (2007) quoting from the European Charter of Fundamental Rights (2000 p. 10) points out that Article 7 states that everyone has the right to have their family life respected and that Article 9 guarantees everyone’s right to found a family.

The Importance of the Family Family functioning, as described by Kantor and Lehr (1975), consists of the actions and the interactions of the various individuals that make up the family both within the family and outside of the family. Therefore, all members of the family are important because they determine the actions and interactions of the family as a unit, as well as themselves as individuals within that family unit (and also, of course, outside of the family unit). According to Rutherford (1998), the majority of children are born into a family, and Whiting (2007 p. 8) notes that the family ‘normally provides a stability and security that is the lynchpin to their development’. Similarly, as well as being born into a family unit, most children are reared within a family unit, and as Whiting (2007) again explains, the family unit influences the development, the maturation and the understanding of their environment (both local and global). It plays a major role (along with the genes of the child) in that child’s functioning throughout life. It is not, however, the only input that the child receives that can affect a child’s functioning throughout life, because the world outside of the family can also play a major role – as evidenced by the effects of hospitalisation in isolation but this will be discussed later in this book. Hare et al. (1989) note that the family (and individual members of the family) receives stimulus from the environment, reacts to that stimulus and then responds to the environment. However, because for many children – but not all, the family is seen as a safe environment, then children will often be able to rely upon people other than family members to help them navigate through the many dangers that they may encounter outside of the family. This is supported by Darbyshire (1994) who states that for the child, the family is often related to feelings of comfort, safety, familiarity, and sanctuary, but these feelings may be lost in an unfamiliar environment (e.g. during hospitalisation).

Introduction to the Family

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It is this safety aspect of the family that is experienced by most children that allows children to approach the world outside of their family with confidence, knowing that they will be allowed to make mistakes without ever losing the close emotional bonds with other family members. This allows them to grow and develop into, hopefully, well-rounded and mature adults and members of their society, and in turn to develop their own nuclear family, and so start the whole process all over again.

Family-Centred Care for Children in Hospital According to Smith et al. (2002 p. 22), family-centred care can be defined as ‘the professional support of the child and family through a process of involvement, participation and partnership underpinned by empowerment and negotiation’. Shields et al. (2007 p. 2) state that family-centred care is a widely used model of care in paediatric nursing and medicine, and that it is instinctively felt to be the best way to provide care to children whilst they are in hospital. They state the concept of family-centred care thus, ‘when a child comes into hospital, the whole family is affected. In giving care, nurses, doctors and those caring for the child must consider the impact of the child’s admission and all the family members’. Family-centred care has developed in the UK as a way of caring for children in hospital, and has been promoted by the Department of Health (2003). Shields et al. (2007) point out that family-centred care means that when a child is admitted into hospital, then the care that is planned by the health care professionals is not just about the individual child, but is also concerned with the whole family. The family, according to Irlam and Bruce (2002), Neff et al. (2003) and Webster and Johnson (1999), is acknowledged as an expert in their child, and in the care of their child. In addition, they have stated that the families are able to provide individualised perspectives and information on their child that can be important to clinical decisionmaking. Although the term ‘family-centred care’ is probably the one that is used mainly within health care settings to describe this type of care, Shields et al. (2007) note that, in actual fact, there are a number of related terms that can be used to describe the whole concept of family-centred care. These include:

r r r r r

partnership in care (Coyne 1996) parental involvement (Hurst 1993) nurse–parent partnership (Hill 1996) parental participation (Kristensson-Hallstrom 1999) care-by-parent (Costello & Chapman 1998, Evans 1994) (Shields et al. 2007).

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Severe Combined Immune Deficiency

In 1987, Shelton et al. developed a framework around family-centred care for children within the health services. Along with Johnson (1990b), Shelton et al. (1987) determined nine elements of family-centred care to include (Trivette et al. 1993):

r recognising the family has a constant in the child’s life r facilitating parent–professional collaboration at all levels of health care r honouring the racial, ethnic, cultural and socioeconomic diversity of families

r recognising family strengths and individuality and respecting different methods of coping

r sharing complete and unbiased information with families on a continuous basis

r encouraging and facilitating family-to-family support and networking r responding to child and family developmental needs as part of health care practices

r adopting policies and practices that provide families with emotional and financial support

r designing health care that is flexible, culturally competent and responsive to family needs (Shields et al. 2007).

Conclusion Family-centred care is all about partnership, not just a partnership between health professionals and parents, but rather a partnership between health professionals, parents, family and the child. The key as to whether or not family-centred care works in the paediatric setting is ‘negotiation’. The roles of everyone involved in the care of a child should be negotiated, so that everyone knows what is expected of them, and that they are not asked to take on something that they are neither able nor willing to do. However, family-centred care is a way in which the child can cope with the transition to hospital care, whilst still retaining some of the security experienced within the family. Relationships and emotional bonds with other members of the family are not fractured so much, and so remain intact. These are of critical importance to the child – and to the family as well.

2

Background to the Study on Children with SCID and Their Families

Introduction This research study arose out of the work that I was involved with during the 1980s and 1990s at the Hospital for Sick Children, Great Ormond Street, London, and Newcastle General Hospital, Newcastle upon Tyne, where I worked with children who were born with severe combined immunodeficiency syndrome (SCID) and other severe immune deficiencies. All the children had had bone marrow transplants for SCID from 18 months to 13 years prior to being involved in the research study. During the course of this work I became aware that many of the children, having returned home following successful treatment for their immunodeficiency, did not appear to be developing physically, cognitively, socially and emotionally as well as had been hoped. Several of the parents of these children echoed my concerns and wished to know whether other surviving children had similar problems or whether their child was unique. Allied to the above was the undeniable fact that more and more children were being treated successfully for this disorder, and medical staff were now concerned not just with the saving of lives, which must always be of paramount importance, but also with the consequent quality of life following successful treatment. In the course of this research project I became aware that, although the children may be considered immunologically ‘cured’ following bone marrow transplantation, in actual fact many of them subsequently appeared to be suffering from chronic ill health, or, in some instances, they and their families were behaving as if they did. Thus, it became apparent that some

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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of the children, who had survived bone marrow transplants for SCID, and their families, needed to be thought of, and treated, as having chronic ill health. There has been much discussion as to what constitutes a chronic disorder. Rutter et al. (1970) considered children to have a chronic disorder if their condition was associated with continuous or recurrent handicap which would last for at least a year. Pless and Pinkerton (1975) deemed a chronic disorder as being a physical condition, usually non-fatal, which lasted for more than three months in any one year, or that required hospitalisation for longer than a month in any one year. Meanwhile, Mattson (1972) also included impaired mental functioning as well as physical problems in his definition of a chronic disorder. In 1990 (p. 3), Eiser stated that ‘Chronic diseases are conditions that affect children for extended periods of time, often for life. These diseases can be “managed” to the extent that a degree of pain control or reduction in attacks (of asthma), bleeding episodes (in haemophilia) or seizures (in epilepsy) can generally be achieved. However, they cannot be cured’. Hobbs and Perrin (1985) considered chronic disease as either a disease that lasts for a substantial time, or else a disease that is debilitating for a long period of time and may last for many years with some cases remaining stable, some improving and some getting worse. So, according to Bradford (1997 p. 6) ‘There is general consensus as to what constitutes a chronic illness, namely that the condition is protracted and can result in a number of diverse and adverse outcomes, ranging from normal life expectancy to death’. Accepting that, for many children and their families, SCID leads to an actual or perceived chronic disorder, the focus of the research became the quality of life experienced by these children and, by extension, the quality of life experienced by their families. Extensive literature searches have revealed a paucity of research and information on the quality of life of children with SCID following successful treatment, although much has been written on the quality of life of those children who have had bone marrow transplants for disorders other than SCID. Of particular note is the seminal work by the child psychologist Carien Pot-Mees (1989) who looked at the psychological profiles of children who had had bone marrow transplants for most of the disorders for which bone marrow transplantation is the accepted treatment, with the notable exception of children with SCID. Consequently, as there had been successful haploidentical bone marrow transplants for SCID for the past ten years when this research was commenced, it was considered that the time was right for this study, particularly as, in 1997, the oldest surviving child of a mismatched haploidentical bone marrow transplant for SCID had left the care of the paediatric immunologists and had been transferred to an adult immunologist for future followup. Consequently, it was being accepted that these children had been ‘cured’ of their SCID and that they could look forward to a normal span of life.

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Quality of Life Issues: Previous and Present Considerations The central question of the study became: What is the long-term quality of life for children who have survived bone marrow transplants for severe combined immunodeficiency syndrome (SCID)? According to Zamberlan (1992 p. 172), ‘Quality of life is a subjective global characterisation made by the child about the physical and emotional functioning, symptoms of disease and treatment, peer and family relationships and interactions, and the child’s current overall assessment of well-being according to stated life-satisfaction’. For the purposes of this study, ‘Quality of Life’ was deemed to be concerned with the way in which the lifestyle of the child is affected as a result of the initial disease and by the subsequent treatment. In order to identify the quality of life experienced by the children in this study, it was necessary to pose several additional questions. This would allow for a more focused discussion of quality of life. The questions to be asked were an amalgam of dimensions which came forward during the undertaking of the pilot study, as well as dimensions which emerged following a reading of the literature. Whilst there were several Quality of Life Scales at the time of the study, for example Derogatis (1983), most of these were concerned with adults, and none were felt to be suitable in their entirety for the children in this study. From a review of the relevant literature at that time, Meeburg (1993) teased out four critical attributes of quality of life which were considered to be common: 1. A feeling of satisfaction with one’s life in general. 2. The mental capacity to evaluate one’s own life as satisfactory or otherwise. 3. An acceptable state of physical, mental, social and emotional health as determined by the individual referred to. 4. An objective assessment by another person that the individual’s living conditions are adequate and not life-threatening. Whilst these were acceptable as a starting point, they were considered to be rather nebulous, and so the work of others in the field was accessed in order to devise a set of questions which could allow the children and families to give accounts of their quality of life after a successful bone marrow transplant.

Elements Involved in Quality of Life According to Neff and Dale (1992), there are three elements of individual growth and development included as outcome measures of quality of life, namely physical, social and psychological.

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Severe Combined Immune Deficiency

r Physical development encompasses biological factors, sexuality and body image.

r Psychological development encompasses cognitive development, r

emotional and intellectual development, the climate of the home, and religion. Social development includes cultural patterns, family constellation and peer relationship.

Zamberlan (1992) used two major dimensions in her study of the quality of life of children following liver transplantation. These were:

r Psychosocial adjustment at school and relationship with peers and family members.

r Changes in physical appearance and physical function. One of the measures that can be used to test quality of life is the Satisfaction with Life Domains Scale by Baker et al. (1992) which was also used by Baker et al. (1994) in their study of long-term bone marrow transplant survivors. Major life domains identified by them include health, relatives, friends, body appearance, daily activities, leisure time and eating. Another scale in use, by Molassiotis et al. (1995), was Derogatis’s (1983) Psychosocial Adjustment to Illness Scale (PAIS-SR) which examines seven domains of adjustment as well as overall psychosocial adjustment. These seven domains are:

r Health care orientation (the nature of the respondent’s health care r r r r r r

position and whether it will function to provide a positive or negative adjustment to illness or treatment). Impact of treatment on sexual development. Impact of treatment on extended family relationships. Impact of treatment on psychosocial distress. Impact of treatment on vocational environments. Impact of treatment on the domestic environment. Impact of treatment on social environments.

Finally, when asked what quality of life means to them, adult bone marrow transplant survivors in Ferrell et al. (1992) identified nine themes as quality of life parameters. In descending order of popularity, these were: 1. 2. 3. 4. 5. 6. 7. 8. 9.

Having family and relationships. Being independent. Being healthy. Being able to work. Having financial success. Having a heightened appreciation for life. Being alive. Being satisfied and fulfilled with life. Being normal.

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Psychological well-being

Physical well-being and symptoms

BMT

Social well-being

Spiritual well-being

Figure 2.1 How bone marrow transplants may impact on the dimensions of the quality of life of people who had received such a transplant (Ferrell et al . 1992). Source: Cancer Nursing 1992. Adapted with permission from Lippincott Williams & Wilkins.

When asked how bone marrow transplantation has affected their quality of life, again nine themes emerged from the adult survivors (Ferrell et al. 1992): 1. 2. 3. 4. 5. 6. 7. 8. 9.

Occurrence of side effects. Infertility. Fear of relapse. Decreased strength and stamina. Limited work and activities. Being given a second chance. Being given the opportunity to improve their quality of life. Increased spirituality. Increased appreciation of life.

From these and other studies, Ferrell et al. (1992) developed four domains of quality of life into a conceptual model (Figure 2.1), which was developed further by Whedon and Ferrell (1994). In Figure 2.1, the four domains of quality of life are capable of being broken down into a variety of factors that possibly may contribute to the quality of life experienced by a person who has had a bone marrow transplant. Physical well-being and symptoms may include:

r r r r r r r

strength and stamina functional activities visual problems recurrent colds and other infections infertility coping with chronic graft-versus-host disease nutritional problems

Psychological well-being may include:

r r r r

anxiety fear of recurrence of the disease depression difficulties with attention

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Severe Combined Immune Deficiency

r difficulties with understanding r changed priorities, including living with the feeling of having been given a second chance

r coping with survival r trying to live a normal life Social well-being may include:

r r r r r r r r

physical appearance financial problems added burdens for the caregiver new/old roles and responsibilities problems with giving and receiving affection concerns over sexual function leisure activities returning to work

Finally, spiritual well-being may include:

r r r r

a strengthened (or weakened) belief in religion and/or self hope – increased or lessened despair inner strength

Physically, as well as the original disorder (cancer, SCID etc.) the transplant procedure itself can weaken the patient, due to the drugs that they receive, both before the transplant as conditioning, and afterwards to prevent graft rejection, graft-versus-host disease, and infections. There are also concerns regarding fertility afterwards (again due to the drugs given to combat the cancer as well as to ‘condition’ the patient to receive the transplant). In addition there are concerns regarding the ability to have a normal sexual life afterwards – this encompasses both physical and psychological concerns. Social well-being can also be a major concern (as it certainly was for many of the children and their families in the SCID study), particularly in terms of roles and relationships, and this may be linked to actual or perceived issues around appearance. Spiritual well-being is not something that people automatically think of as being important to quality of life, but if ‘spiritual’ is deemed to be more encompassing than just being ‘religious’, then it becomes apparent that this is a very important dimension to consider for both the religious and the non-religious person alike.

Measuring Quality of Life Measuring quality of life is a matter of considerable debate, and it is difficult, if not impossible, to evaluate what the future may bring – particularly with new and ever improving therapies. Why do we need to measure quality of life? The reasons why quality of life is measured are:

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r Medical professionals are increasingly accountable for their perforr r r r r r r

mance and practice. (Jenney & Campbell 1997). To consider resource allocation. To evaluate interventions. To commission programmes of care. To explore patient preferences and expectations. For public health data collection. To allow us to understand each patient’s perception and to be able to therefore practice holistic care. To assess the outcomes of new treatments (Eiser & Morse 2001).

There are many challenges that we have to face when considering measuring the quality of life of children with a chronic illness.

Quantitative Quality of Life Tools If, as defined earlier, quality of life is accepted as being an individual perspective, then the ultimate challenge in child health is the identification of a tool that will elicit that perspective. When considering a tool for finding out from the children with a chronic disease what they consider their quality of life to be, there are a number of points to consider with the use of structured generic tools that can quantify the degree of life quality as experienced by children. According to Eiser (1997), whilst there are many disease-specific quality of life tools, there is a dearth of generic quality of life tools. In addition, there are disadvantages to using these generic tools. These disadvantages reflect the fact that generic quality of life tools lack sensitivity and do not reflect the specific impacts of treatments on quality of life. Jenney and Campbell (1997) supported this conclusion, by doubting whether a single generic quality of life instrument for children would ever be appropriate. In 2001, Eiser reviewed 137 papers concerning quality of life tools for children with chronic ill health, and she came to the conclusion that there remain many problems in measuring quality of life in children with different diseases. In 2001, Eiser and Morse made a list of the problems that can arise with the use of quantitative tools that purport to measure quality of life in children, namely:

r r r r r r

Confusion about the definition and measurement of quality of life. Limited availability of disease-specific measures. Discrepancies between parent and child ratings. Limited availability of measures for self-completion by children. Lack of precision about the content domains of quality of life. Cultural appropriateness of measures used in the UK.

Other problems that have been identified include:

r Inclusion of direct questions in cancer-specific quality of life measures (Varni et al. 1998).

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Severe Combined Immune Deficiency

r The child’s concept of health as well as their experience of health and illness (Bibace & Walsh 1980).

r A child’s developmental stage – there are relatively few quality of life measurement tools available for pre-school children.

r The child with learning difficulties may be unable to address any issues of preference. In spite of the concerns discussed above, researchers still use quantitative research tools to explore the quality of life of children (and adults). One such research tool is the SF36 questionnaire. SF36 is perhaps the bestknown questionnaire used by experts in measuring quality of life and health status. The ‘SF’ stands for ‘short form’, and the ‘36’ means that it is made up of 36 questions. There are two reasons for the popularity of the SF36 questionnaire as a tool for identifying health status and quality of life. Firstly, because it was so carefully developed in the first place, the results it produces are accepted as being statistically reliable and valid. Secondly, the issues it explores using these 36 questions apply to people having many different types of treatment, and in all the different states of health, from good to bad. It is particularly invaluable in measuring the changes in health that follow medical interventions. This tool is for use with adults over 18 years of age. For young children, a quantitative tool for health-related quality of life in common use is the Child Health Questionnaire. The Child Health QuestionnaireTM (CHQ) is a family of generic quality of life instruments that have been designed for children aged from 5 to 18 years. It measures 14 unique physical and psychosocial concepts. The parent form is available in two lengths – 50 or 28 items. Scores can be analysed separately, the CHQ Profile Scores, or combined to derive an overall physical and psychosocial score, the CHQ Summary Scores. In a recent study from the Netherlands (Buysse et al. 2007), the SF36 questionnaire was used alongside the CHQ to investigate the long-term healthrelated quality of life of children, some years after their illness, and their parents following the successful treatment of meningococcal septic shock. The researchers compared the quality of life scores of the children and their parents with those for the normal Dutch population (also obtained using these two tools). In terms of physical factors, survivors had significantly poorer health-related quality of life scores than the normal population, which lead Buysse et al. (2007) to suggest that this may be because the present health status as well as the earlier illness had a negative impact on their perception of their physical health status. Interestingly, the psychosocial aspects that were measured by the CHQ were significantly better in those older than 12 years. They suggest that this ‘might indicate that adolescents and young adult who survived a severe illness in childhood may be more inclined to enjoy life’ (Buysse et al. 2007 p. 1572). This is very much at odds with the adolescent children in the SCID study (Vickers 1999b), where the tools used to collect the research data were qualitative ones. Using the SF36 questionnaire, the parents in the Buysse et al. (2007) study, however, were found to have better health-related quality of life

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scores than the normal Dutch population. According to the researchers, this might indicate that the child’s illness did not have a negative impact on the parent’s future quality of life. They rationalised this by theorising that ‘Parents are under great stress at the time their child is struck by life-threatening disease. But if their child survives, especially when there are only minor sequelae, they will leave the stress behind’ (Buysse et al. 2007 p. 1573). In the interviews, the parents in the study reported that their experiences with their children’s illnesses made them stronger and allowed them to appreciate life more fully. Again this is in marked contrast to the parents in the SCID study, and Buysse et al. (2007) do postulate that there might be some degree of denial or overcompensation in the parents’ responses and attitudes. Thus, it is possible that the quantitative quality of life tools may not provide the complete answer as to quality of life status, and we should be looking at other methods – the qualitative research data collection tools, such as interviews.

Qualitative Quality of Life Data Collection Tools Because of all the difficulties in devising and using a quantitative tool to measure quality of life in children, then using a qualitative method which is situated within a phenomenological philosophy will give more pertinent information, and allow children with chronic diseases the opportunity to explore, identify and express their own thoughts and feelings into the quality of life that they are experiencing. Phenomenology is concerned with an individual’s experience of an event or happening, and more importantly, their own perspectives of that experience. Therefore, a phenomenological exploration of the child’s experiences and their personal perceptions of their experiences of living with a chronic disease will give a truer picture of their quality of life. Historically, children have been perceived as passive recipients of health care rather than active participants. Gradually, there has been a shift in emphasis and children are now viewed as active participants. Thus, in order to understand the meaning of quality of life for a child with a chronic illness, it is imperative that the voice of the child be heard, and there are several ways in which this can be achieved. Perhaps the simplest (although the hardest to do well) is by means of face-to-face interviews with the child. Other methods which have been successful, depending on the age and cognitive development of the child include the use of diaries, drawings and paintings, videos and play. Yet another method of ‘listening’ to the child with a chronic disease is the use of quality of life questionnaires, although the child would have to be capable of understanding the questions and of being able to write down the answers. However, Hill (2002) identified that completing a quality of life questionnaire gave patients many new insights into the multidimensional aspects of their lives which often prompted a positive life review. Surprise at how different the nurses’ view of quality of life differed from

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Severe Combined Immune Deficiency

that of the patients’ were expressed by the nurses participating in this study. However, the benefit of this quality of life questionnaire was that:

r awareness of the issue of quality of life was increased for the nurses r reflection occurred within the nursing work force who took part in the study

r the nurses were encouraged to seek new knowledge and understanding to enhance their practice

The SCID (Vickers 1999b) Study From all the studies reviewed, and from the results of the pilot study, the following questions were posed in the SCID research project in order to elicit information about the quality of life as experienced by the children and their families in the study. 1. Has the child’s physical growth been affected, and if so, has it affected the quality of life? 2. Does the health of these children improve as they get older, and is it better, worse or the same as could be expected for children who have never had SCID? 3. Is the diet/nutritional intake of the children normal, or does it remain as poor as it was immediately after the bone marrow transplant? 4. Is the psychomotor development of the children delayed, as compared to the normal population, due to the combination of early ill health, treatment and isolation? 5. Is the psychosocial functioning of the children in the present study adversely affected, and are there differences in the psychosocial functioning of children having bone marrow transplants for SCID, children having bone marrow transplants for other diseases, and healthy children? 6. Is the subsequent educational potential of the children in the study affected? 7. If there are effects, as outlined above, are any of these effects caused by/related to:

r type of bone marrow transplant and particular type of immunodefir r r r

ciency age of child at isolation and length of isolation period age at bone marrow transplant type of pre-transplant conditioning culture

Methods of Investigation The questions posed above were investigated by a combination of strategies, namely:

Study on Children with SCID and Their Families

r r r r r r

17

medical/nursing records of the children parent interviews parent questionnaires teacher questionnaires drawings/discussions with the children personal knowledge of the children and their families

Using a combination of investigative methods, such as those above, allows them to act as a cross-check on each other, as well as invoking the process of ‘triangulation’ or ‘mixed method’ – a process in which one is able to get a better view of things by looking at them from more than one direction (McNeill 1990).

Quantitative and Qualitative Research: Reasons for the Use of Both Methods in This Study In addition, using the particular investigative measures displayed in the previous section allows for both a quantitative and a qualitative study. ‘Quantitative research is a formal, objective, systematic process in which numerical data are used to obtain information about the world. This research method is used to describe variables, to examine relationships among variables, and to determine cause-and-effect interactions between variables’ (Burns & Grove 2005 p. 23). A quantitative study is essential for discerning trends within the population or group, whilst qualitative research has been defined by Blenner (1995 p. 87) as being concerned with the ‘preservation of the holistic, subjective experience of individuals’. The main theme of the study was, as stated above, the quality of life of these children and their families posttransplant. As quality of life hinges on the perceptions of the individuals concerned, then it is important that the researcher who wishes to look at the quality of life of any individual, needs, as Holloway (1991) discusses, to use research methods that allow for the discovery of that individual’s perceptions and interpretations to events and actions. Knafle and Howard in their paper (1984 p. 17), which is acclaimed by Dickson (1995 p. 417) as a ‘landmark article’, state that ‘Qualitative research is equated with those methods or data-gathering techniques which generate narrative as opposed to numerical data’. They note that qualitative data can be in the form of verbatim interviews and/or transcripts of field notes. In addition, they make the point that there are four strands to qualitative research:

r firstly, to describe a phenomenon that occurs r secondly, to explain to others that phenomenon in order for them to understand the experiences of the participants

r thirdly, to develop research instruments and methods r fourthly, to create a model that explains these experiences

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Severe Combined Immune Deficiency

Other researchers, such as Wilson (1992), suggest that qualitative research can explore and describe phenomena/experiences as complementary to discovery and explanation. Dickson (1995 p. 417) also stresses that ‘qualitative methods are a family of ways to collect and analyse phenomena of interest’. Qualitative research is an umbrella term that covers a variety of styles of social research, drawing on a variety of disciplines such as sociology, anthropology and psychology. However, there are some common elements to these approaches that begin to give some sense to the term ‘qualitative research’.

r A concern with meanings and the way people understand things: Human

r

activity is seen as a product of symbols and meanings that are used by members of the social group to make sense of things. One of these symbols and meanings that can be analysed is ‘text’. A concern with patterns of behaviour: The focus is on regularities in the activities of a social group, such as rituals, traditions, relationships and the way that these are expressed.

Qualitative data, whether words or images, are the product of a process of interpretation. The data only become data when they are used as such. The data do not exist ‘out there’ waiting to be discovered, but are produced by the way they are interpreted and used by researchers. Qualitative research can be part of an information gathering exercise and useful in its own right. Alternatively, qualitative research can be used as the basis for generating theories. In neither case, however, are its descriptions ever ‘pure’ – they are always the outcome of an interpretation by the researchers. Researchers are often the catalyst for the collection of data – and sometimes are the ‘data collection tool’ itself. Their importance cannot be overestimated because they need the skills to be able to allow the research participants to deliver, often very personal, information. In particular, they need to be skilled communicators. In the end, the data that is generated in qualitative research is only as good as the researcher is skilled in the arts of interviewing and observation. Morgan and Smircich (1980), in arguing that the dichotomy between quantitative and qualitative methods can be considered to be rough and oversimplified, determine that qualitative research is an approach rather than a particular set of techniques, and that its appropriateness depends upon the phenomena/phenomenon being explored. Every qualitative researcher has to be aware of the methodological doubts which exist concerning qualitative approaches; for example, Sandelowski (1986) questions the rigour of qualitative research as opposed to quantitative research, and the difficulties of auditing qualitative data. However, according to Sandelowski (1986 p. 33), ‘A study and its findings are auditable when another researcher can clearly follow the ‘decision trail’ used by the investigator in the study’. They designate this ability to replicate the results as the concept of ‘trustworthiness’. In fact, this trustworthiness, made up of credibility, transferability, dependability and confirmability, has, amongst qualitative researchers, gained acceptance as a concept

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of validity and reliability, and goes some way to answering the criticisms of those researchers involved in quantitative studies (Dickson 1995). Thus, this study used a mixture of quantitative and qualitative methods to identify relevant data, the use of which allowed for a more complete understanding of the phenomena experienced by the children and their families involved in the study – particularly in terms of their psychosocial and emotional development.

Parameters of Quality of Life Used in This Study A closer look at the questions posed at the beginning of this chapter will show how each of the methods of investigation contributed both quantitatively and qualitatively to the study. 1. Physical Growth: Using the medical records of the children and the parent interview, the height and weight of the children were ascertained before and after the transplant, up to the date of the interview. These were then compared with the normal population by plotting them on centile charts. 2. Health Status: The data were taken from medical records, the parent interview and the parent and teacher questionnaires. Comparisons were made between the health status around the time of the bone marrow transplant of the children and their present status. In particular, the incidence of infections was investigated. 3. Diet and Nutritional Status: Information relating to dietary and nutritional status was taken from the medical records, parent interview and both questionnaires. Once again, comparisons were made between the period at the time of the bone marrow transplant and subsequent years. For many of the parents and children this aspect was of considerable concern in the months following a successful transplant, particularly once the child had returned home, and was an area of much potential conflict. 4. Psychomotor Function: The development of psychomotor function was addressed in the parent interview. Three milestones of psychomotor development were discussed, namely crawling, walking and talking. These three milestones were selected for two reasons. Firstly, they are crucial milestones in a child’s development. Failure to reach the latter two in particular, or a major delay in doing so, can lead to many problems of psychosocial function. The second reason for selecting these three milestones was that they are also very important for all parents so they are usually the milestones that, almost without exception, they are able to remember the age at which they were attained. The psychomotor functions of the children in the study were compared with the general population, as discussed by Lansdown (1984) and Sheridan (1975). 5. Psychosocial Functioning: This was made up of behaviour and of social adjustment. Three methods were used to obtain most of the information for the study.

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Severe Combined Immune Deficiency

(a) Rutter Scales: In order to ascertain the psychosocial functioning of the children, the Rutter A Scale for Psychosocial Assessment (for parents) and the Rutter B Scale for Psychosocial Assessment (for teachers) were inserted into the two questionnaires (Rutter et al. 1970). These scales were chosen for two reasons. In the first instance, both these scales had been used in several studies in the UK and their reliability and validity had been reported as satisfactory (Bradford 1994, Fergusson et al. 1985, McGee & Silva 1982, Rutter et al. 1970, 1975a, 1975b). The second reason for their use in this study was that they were used by Pot-Mees in 1989 when she researched the psychosocial effects of bone marrow transplants on children, other than those who had bone marrow transplants for SCID. This allowed for comparisons to be made between children in the SCID study and those in the Pot-Mees (1989) study. The two scales consist of a number of statements mainly focused on behaviour, for example, ‘often appears miserable, unhappy, tearful or distressed’. In addition, the Rutter A Scale also has items on health and habits. These statements are then rated by the parents or teachers as No (does not apply), Sometimes (applies at times) and Yes (certainly applies). These ratings are allocated scores; No = 0, Sometimes = 1, Yes = 2. Children who score more than 13 on the Rutter A Scale or 9 on the Rutter B Scale are identified as suffering from a behaviour disturbance. In addition, the behaviour disorders are allocated into one of four classifications – neurotic, antisocial, hyperactive and depressive behaviours. (b) Interviews: Complementary to the Rutter Scales, questions about behaviour and social adjustment were asked of the parents during the interview. This allowed them to expand and to discuss feelings and fears. In particular, the child’s reactions to certain situations, such as attempting something new, were discussed to elicit some ideas on their coping mechanisms. (c) Children’s Drawings: Initially, attempts were also made to talk to the children, but most were loathe to talk freely and generally only answered in monosyllables. This problem, however, had been anticipated, so the children were also asked to draw pictures which could then be assessed using criteria as suggested by DiLeo (1983) and Oster and Gould (1987). According to O’Malley and McNamara (1993), the use of drawing as a means of assessment and communication with children has been widely recommended. The drawings of children have been used to evaluate their emotional state and physical and emotional development by such researchers as McLeavey (1979), Sturmer (1980) and Scavnicky-Mylant (1986). For further information on children’s drawings and their application in this study, see Appendix A. 6. Education: Teachers were asked in their questionnaire to identify any educational problems the children may have, in addition to any health or behavioural and social adjustment problems that may be affecting their schoolwork.

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The Children Involved in the Study First, all those children still living, who had had a successful bone marrow transplant for an immune deficiency disorder in the UK and Germany up to 1992 were considered for inclusion in the study. These two countries were chosen because I had lived in both countries, and so spoke English and German. Eventually, 36 children were selected. In order to be as inclusive as possible, the criteria for their selection were: 1. The child had to have had a bone marrow transplant for SCID, including adenosine deaminase SCID (ADA SCID), reticular dysgenesis (RD), Omenn’s disease, combined immunodeficiency syndrome (CID) and Bare lymphocyte syndrome (BLS), but excluding Wiskott–Aldrich syndrome and DiGeorge syndrome. 2. The child had to have survived the bone marrow transplant for a minimum of 12 months. 3. The child had to have been transplanted in either the UK (at the Hospital for Sick Children, Great Ormond Street, London, or Newcastle General Hospital) or in Germany (die Universit¨atskinderklinik, Ulm). At the time, these were the only specialist centres in the two countries. 4. The child and family had to have maintained regular contact with the transplanting hospital since the bone marrow transplant. Not all the children who had survived bone marrow transplants for SCID between 1982 and 1992 could be included in this group for a variety of reasons, the main one being that the parents’ command of English or German was poor. In particular this meant the exclusion of all the Turkish children in Germany and some of the Asian children in the UK. Other reasons for the exclusion of children from the in-depth study included any reluctance on the part of either the parents or children for taking part in the interview, non-return of the forms agreeing or not agreeing to be interviewed, families moving too far away to be visited and themselves no longer visiting the transplanting hospital, and recent loss of contact with the family. The group of 36 children was made up of 20 German children (15 boys and 5 girls) and 16 UK children (9 boys and 7 girls). Of the 36 interviews that took place with the families of the children, 31 occurred in the child’s home and the other five, for the convenience of the parents, took place at the transplanting hospital when the child returned for a check-up. The age range of the children involved in both groups at the time they and their families were interviewed was from 3 to 15 years, and altogether they lived in a total of nine countries, but were treated in two countries. Those treated in England lived in England, Wales, Scotland, Northern Ireland, the Republic of Ireland and Malta, whilst those treated in Germany lived in Germany, Switzerland and Austria. As can be seen in Table 2.1, several different types of SCID were represented in the group of children in the study, the largest group being represented by the diagnosis of SCID. SCID at that time was a ‘catch-all’

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Severe Combined Immune Deficiency

Table 2.1 The Different Types of SCID Represented in the Study SCID disorder Germany Girls Boys UK Girls Boys

SCID ADA SCID Omenn’s disease SCID ADA SCID Reticular dysgenesis

2 2 1 12 2 1

SCID ADA SCID Omenn’s disease SCID ADA SCID Reticular dysgenesis

3 3 1 7 1 1

term to indicate children with SCID that did not fall into one of the other types, such as ADA SCID. Since the research study took place, there have been tremendous improvements in diagnostic techniques, and so the group initially diagnosed as just having SCID would now have a more definite diagnosis. ADA SCID is the next most common diagnostic group in the study, with 8 out of 36 (22%) children having this disorder. Omenn’s disease was only found in girls within the group, although boys also can be diagnosed with the disorder. Similarly, reticular dysgenesis – the most severe form of primary immunodeficiency was only diagnosed in boys within the group. Following submission to research ethics committees, the research proposal was accepted, and informed consent sought. In order to maintain confidentiality, as promised to the children and families, each of the children had been given a pseudonym.

Reference Groups Because of the many variables within the SCID group, in terms of age, gender, country of origin, country of bone marrow transplant and experiences pre- and post-transplant, it was not considered feasible to have a matching normal control group. Therefore, for certain parts of the study, reference groups have been used. With the Rutter A and B Scales, the children in the SCID group have been compared to the three groups reported in the Pot-Mees (1989) study, namely bone marrow transplant children, children with cardiac problems and a normal group. In addition, using just the Rutter A Scale, the SCID group are compared with the group of children with liver disease studied by Bradford (1994). In order to have an up-to-date reference group of children without any serious health problems, the Rutter A Scale was also used with a sample of 50 children with good health in 1997. The age range of these children

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was from 3 to 15 years, with an equal mix of boys and girls, and they came from York, Hull, Sheffield, London and Luton. The children with SCID are compared with this group. Some of the health criteria were also compared with the liver disease children in the Bradford (1994) study. Physical growth and psychomotor functioning were compared to the normal populations using centile charts for growth, and the data in Sheridan (1975) and Lansdown (1984) for psychomotor development.

Methods of Data Collection As stated previously in this chapter, several strategies were involved in the collection of data for this study. The medical and nursing records of all the children from 1980 until 1997 were accessed for relevant medical information. Interviews took place with the children and their families from August 1994 until June 1997. The format of the interview was derived from the author’s personal knowledge, the results of a pilot study, discussions with parents and professionals and also some of the items used for the interviews with parents in PotMees’s (1989) study. The interviews were conducted in either English or German, and took place mainly in the family home. Occasionally, it was more convenient for the interview to take place in hospital when the child came for his/her annual check-up. Most of the interviews were undertaken by myself, but a few were carried out by two other researchers (nurses) who were trained in using the same instructions for the interviews. Following the interview, the parents were requested to fill in a questionnaire and return it by post. Once again, the questionnaires were an amalgam of personal knowledge, the pilot study evaluations and discussions with parents. As with the interview schedule, the questionnaires for both parents and teachers were translated into German for use in Germany, Switzerland and Austria and were piloted. The same timescale for the questionnaires and the children’s drawings operated as for the interviews, namely from August 1994 until June 1997. Of the 36 questionnaires given to the parents, only three (all from German families) were not returned, giving a response rate of 92%. The response from teachers was nowhere near as good; only 17 out of 29 returning their questionnaires – a response rate of 59%.

Hospitals Involved in the Study There were three hospitals involved in the study. These were the specialist centres for the treatment of children with SCID in the UK and Germany. 1. Die Universit¨atskinderklinik, Ulm: This hospital is situated on a hill overlooking the city of Ulm in southern Germany. It was a specialist children’s hospital attached to the university with one consultant and a

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dedicated unit. Initially, the children were nursed in mini-isolators, but quite quickly the unit changed over to large isolation tents and since 1991 the unit has been situated in a purpose-built isolation unit with five laminar air flow cubicles. Children with haematological problems requiring bone marrow transplantation were also nursed in the unit, but the treatment of SCID remained the main purpose of the unit. The first successful mismatched bone marrow transplant for SCID took place here in May 1982. 2. The Hospital for Sick Children, Great Ormond Street, London: This hospital is situated in the centre of London and is a specialist children’s hospital. The first few children with SCID were transplanted in the same unit as children with haematological disorders, but from 1984, they were transplanted in the specialist isolation unit within plastic tents with filtered air. Most of the children from this hospital in the research study were transplanted in this second unit (12 out of 18 = 67%). For the last few years, children with SCID have again been transplanted in the same unit as children with haematological and other oncology disorders. For most of the time during the period covered by the study, 1980–1992, there were three consultants involved in the care of these children. The first successful mismatched bone marrow transplant for SCID at this hospital occurred in March 1982. 3. Newcastle General Hospital, Newcastle-upon-Tyne: This hospital is situated in an inner city area of Newcastle-upon-Tyne in the North of England. Compared to the other two hospitals involved in the study, Newcastle General Hospital had only comparatively recently been involved in the treatment of SCID; the first successful mismatch bone marrow transplant taking place in May 1988. Newcastle General Hospital is not a specialist children’s hospital, but since 1993 has had a purpose built unit for SCID, with six laminar air flow cubicles, and is now a very busy unit. Initially with only one consultant for SCID, there are now three employed. The unit now also transplants children with haematological problems alongside children with SCID, but it still remains dedicated to children with immunological problems. The unit now caters for all children with SCID from Birmingham northwards, including from Ireland and Scotland. Children with SCID who live south of Birmingham are treated at Great Ormond Street.

Results from the Study Throughout the study various themes were identified and explored, all of which have a bearing on the quality of life experience, as perceived by the children and their families. Whilst this book is concerned with the psychosocial problems that were identified as a result of the children having SCID and surviving a bone marrow transplant, it is interesting to look at some of the results that were found when the other parameters linked to quality of life were explored, particularly the physical one of health status.

Study on Children with SCID and Their Families

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The present health status of the children was discussed and the continuing ill health of many of the children in the study was demonstrated. Notable problems, such as deafness, speech defects and poor co-ordination were identified in children who had ADA SCID or reticular dysgenesis, and their overall health status was noted as problematical. Problems were also present in children with other types of SCID. For example, skin and hair problems (including two children with alopaecia and mottled skin), warts, poor teeth and jaw development, difficulties in maintenance of healthy teeth, and upper respiratory tract infections. In addition, all three children who had Omenn’s disease were found to have developed Hypogammaglobulinaemia due to a failure of B-cell reconstitution post-transplant. It was noted that better health profiles were achieved overall by the German children in the study than by the UK children, and possible reasons for this were advanced, including cultural and sociological reasons. For example, the close integrated family life apparent in many of the families in Germany may reduce stress and provide greater support, thereby allowing for a more positive perception of ill health present, in addition to the reality of continuing ill health. The physical development of the children in terms of weight and height was explored, along with their dietary and nutritional status. Nutritional intake and the mechanics of eating were found in many of the families to be major sources of conflict between the children and their parents in the early years post-transplant, but in the main this was said to be no longer the case by the time the child started to attend school. With regards to growth, the children did experience very poor growth before, during and immediately after the bone marrow transplant. In time this poor growth improved as far as the boys were concerned, so that during their school years, they had normal growth patterns and spanned the whole range of centiles for both height and weight. Of note, however, was the poor growth of the girls in the study compared with the boys, as well as the better growth patterns achieved by the German children as a whole compared with the UK children. Consideration was given to the psychomotor development of the children, using the three developmental milestones of crawling, walking and talking as parameters. With the children in this study, there was some marked delay in achieving both walking and talking, but not crawling, and possible reasons for this included the length of confinement and lack of oral stimulation. In addition, variables such as age at isolation and length of isolation were used for comparative purposes and this research demonstrated that differences in the age of attainment of these milestones may be linked to these two variables, as well as to gender, country of transplant and type of SCID. Apart from those children isolated at birth, children isolated before six months of age had delays in walking and talking which were statistically significant when compared with the norm. Children isolated after six months of age had no such problems with the development of walking, but did have major problems with delay in language acquisition. There were no such problems with those children isolated at birth.

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In terms of length of isolation, there were no differences in achieving crawling skills, apart from those children who were isolated for a length of time in excess of 12 months, in whom there was much delay. Within this group of children there were similar problems with the development of walking skills and with language acquisition. Children isolated for a period of up to four months in length achieved parity with the norm for attainment of these three parameters. With reference to the effects of gender – a third variable – on the attainment of all these three milestones, the girls consistently experienced more delay than did the boys, and in the acquisition of walking and language skills, these delays were statistically significant. This runs counter to the norm where, in the general population, compared to girls, boys are considered to be developmentally retarded on a number of counts, including walking and language acquisition (Lansdown 1984). A comparison of the children from the two countries (UK and Germany) and their age at the attainment of the three developmental milestones demonstrated that the German children were close to the norm, whilst the UK children were significantly delayed. Finally, children with ADA SCID were compared with all children with SCID as well as the norm. It was found that children with ADA SCID experienced significant delay in the acquisition of the three milestones compared with both the norm and all SCID children, but this delay was entirely due to the four UK ADA SCID children, thus mirroring the situation with SCID children in general. Reasons for the occurrence of all these phenomena included particularly the high incidence of hearing problems in children with ADA SCID, as well as cultural/sociological resources available. One important aspect of the study is the psychosocial functioning and development of the child and family. Rutter et al.’s (1970) Behaviour Questionnaire and Scales were used to explore the many facets of psychosocial development following bone marrow transplantation. The children in this study tended to experience many problems, such as anger, depression and lack of peer friendship, as well as many antisocial and neurotic problems. Results from this study were compared with a reference group of well children who had not had major ill health, a group of children following bone marrow transplantation for diseases other than SCID, children with cardiac problems and a group of children with severe liver disease. The children with SCID were found to have similar problems to those of the liver disease group, but to have significantly more problems than the bone marrow transplant, cardiac and reference groups. Possible reasons for this situation are advanced and debated elsewhere within this book (Chapter 8) so will not be discussed here, except to summarise that, once again, there were many differences between the UK and German children, with the UK children experiencing many more psychosocial problems. Differences in age at isolation and length of time spent in isolation, along with differences in the quantity and quality of the support afforded the family and child at the time of the transplant may explain some of these differences, but cultural and social variations almost certainly have a role to play as

Study on Children with SCID and Their Families

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well. Gender difference elicited one particularly interesting phenomenon, namely that the boys experienced more psychosocial problems than did the girls. Types of problems experienced varied between the genders as well, with boys often displaying neurotic and depressive symptoms whilst the girls exhibited symptoms of aggression and anti-social behaviour. This was the opposite of the experiences of the children in the reference group. The educational progress of the children in this study was also explored. Most of the children were found to be very capable when placed in normal classes within normal schools. Some children, however, particularly those with ADA SCID and reticular dysgenesis, were found to have special learning needs and to require education in special centres. In many cases, these needs were linked to their severe hearing problems. A major problem for many of the children within the educational setting, as within the home situation, however, was a very poor concentration span. This possibly was linked to their high levels of energy. Particular problems that children with ADA SCID, reticular dysgenesis and Omenn’s disease have to cope with were identified. These three disorders are variants of SCID and children with them have been noted as being responsible for many of the health and behaviour problems identified in this study. Therefore, their results were extrapolated and commonalities determined. The majority of the children with ADA SCID experienced progressive severe hearing loss and speech problems, as well as co-ordination problems. These problems may be linked to damage to nerve cells caused by the same toxins which damage the cells of the immune system. Consequent to this, many of the ADA SCID children exhibited severe behavioural problems. All the children with reticular dysgenesis were discovered to have speech problems, and two out of the three children were known to have severe hearing problems. All three children with Omenn’s disease have hypogammaglobulinaemia following their bone marrow transplants, possibly as a result of a poor engraftment. Regarding physical growth, children with ADA SCID, reticular dysgenesis and Omenn’s disease generally had poor growth patterns when compared to their peers (both with and without SCID), although there are a very few exceptions.

Conclusion On the whole, the results from the study do appear to support the hypothesis that, following a successful bone marrow transplant, these children may well develop a chronic physical, psychological or social disorder, and so questions as to the best way to treat and care for these children need to be posed and answers found. In addition, because in a few of the cases the quality of life appeared to be so poor, particularly for many of the children with ADA SCID and reticular dysgenesis, questions as to the best ways to treat these children need to be considered.

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Following the study, recommendations were made as to the future care of these children. Earlier and more comprehensive support of the child and family during and after the transplant, perhaps even for many years after, was considered to be vitally necessary, as well as on-going counselling. Shorter isolation periods, within the bounds of safe practice, along with a fuller understanding of the stresses these children and their families experience, would all help in the goal of attaining healthier, happier and more stable children. This study was one step towards that aim. However, further research into the various problems identified in this study was considered essential for the future, and a major research by Titman and colleagues has just been completed and is about to be written up. This study has found similar problems in these children as were found in my research study, and will move the debate forward (Titman P., 2007, personal communication).

3

Severe Combined Immunodeficiency – A Brief Overview

Introduction A five year old boy was seen in the Walter Reed Army Hospital, Washington DC, USA. He presented with a history of recurrent infections, namely varicella, rubella complicated by pneumonia, epidemic parotitis, gastroenteritis, otitis media, mumps, and pneumococcal sepsis. In the next four years he was to have 15 episodes of sepsis. Pneumococci were isolated from blood cultures on seven occasions. During these four years he was also to have two episodes of pneumonia, otitis media three times, and mumps twice. None of the available drugs appeared to be of any help in controlling these infections. (Bruton, 1952:722) The above report from over 40 years ago concerns a five-year-old boy who is acknowledged as the first child ever to be diagnosed as having a primary immune deficiency disorder, in this instance agammaglobulinaemia; it presents a classic description of an immune deficiency disease – early onset, recurrent infections and the ineffectiveness of most drugs in fighting these infections (Stiehm et al. 2004). Primary immune deficiencies are so-called because there are no extrinsic factors present, such as an infection or chemotherapy, which could cause the immune deficiency. Some of the immunoglobulin disorders can cause such mild problems that many people who have them are unaware that they have such a disorder, or even that anything is wrong with them, for example, in some cases of IgA deficiency. At the other end of the spectrum, the severe combined immunodeficiency (SCID) disorders are uniformly fatal within, at the most, 18–24 months of life unless appropriate treatment is given. Even then there are great risks attendant to the treatment. Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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The prognosis for a child suffering from a primary immunodeficiency disorder can vary not only upon the particular immunodeficiency disorder, but also upon such factors as how soon the child is diagnosed and treated, and also how well that child manages to avoid infections. The earlier a child is identified as having one of these disorders, the greater the chance of successful treatment and improvement in their quality of life. Combined immunodeficiencies are immunodeficiencies with low, but not absent T-cell lymphocytes (Buckley 2000). One of the problems that occurs when T-cell lymphocytes are missing, not functioning properly, or are only present in low numbers is that the rest of the acquired immune system also cannot function properly. This is because it needs to be stimulated by one type of T-cell lymphocyte, namely the T-helper cells (also known as cells with CD4 receptors). Thus, children with combined immunodeficiencies also have some degree of B-cell deficiency as well as the primary T-cell deficiency. Severe combined immunodeficiencies occur when there is an absence or lack of functioning of T cells, plus or minus other lymphocytes, such as the B cell or natural killer (NK) lymphocytes (Buckley 2002, Fischer & Notarangelo 2004). SCID and combined immunodeficiency (CID) include all immunodeficiency disorders in which there is an absence or severe decrease in the production of T- and B-cell lymphocytes (Amman 1987). CID is often used as a generic term for all combined immunodeficiencies and includes SCID, Omenn’s disease, reticular dysgenesis and ataxia telangiectasia, although it is also sometimes used to denote a rather less severe form of SCID. There are a number of disorders which are embraced by SCID/CID (see Box 3.1). The most severe of these disorders is undoubtedly reticular dysgenesis which is a failure of both lymphocytes and neutrophils to function and which often results in death within the first two to three months of life. DiGeorge syndrome, whilst being a failure of T-cell lymphocytes to mature and differentiate, due to the absence of the thymus, induces a state of combined immunodeficiency because of the regulating nature of the T-cell lymphocyte arm of the immune system. Without the T-helper cells stimulating the B-cell lymphocytes to mature, proliferate and differentiate, the affected child is unable to mount an adequate B-cell response to infection. This situation also applies to other T-cell immune deficiencies. The basic treatment for both types of immunodeficiencies is generally the same, so for much of the time it is possible to talk about both types of immunodeficiencies together.

The Immune System Unless you are a specialist in immunology, much of what has been written so far in this chapter may not mean much to you. Therefore, in order to better understand the effects of SCID on children, it is important to have some knowledge of the immune system, so that the effects of the

Severe Combined Immunodeficiency – A Brief Overview

Box 3.1

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Combined Immune Deficiencies

Severe combined immunodeficiencies: (a) X-linked Lymphopaenic Agammaglobulinaemia (b) Swiss Type Agammaglobulinaemia (autosomal recessive) Omenn’s syndrome SCID and eosinophilia with severe skin breakdown Reticular dysgenesis Lymphopaenia and neutropaenia Enzyme deficiencies: adenosine deaminase deficiency (ADA) SCID and short-limbed dwarfism Wiskott–Aldrich syndrome CID with severe eczema and thrombocytopaenia Ataxia telangiectasia CID with progressive neurological, vascular and endocrinal defects DiGeorge syndrone (congenital thymic aplasia) CID which also embraces cardiac and other midline defects

pathophysiology of severe immune deficiencies can be discerned in the health status of the individual child (you may also want to look at the glossary). To put it simply, the immune system is an intricate combination of tissues, blood cells, enzymes and proteins which work together to protect the body by making it resistant, i.e. immune, to infection by micro-organisms (bacteria, viruses, fungi) and larger organisms, such as worms and flukes, and other parasites. In addition, the immune system possesses other functions, for example tumour surveillance, which are now being understood much better. There are two types of immune defence, known as non-specific (innate) immunity and specific (acquired) immunity.

Non-Specific Immunity This is innate (or inborn) immunity. In other words, this is the immunity that we are born with. There are many components involved in non-specific immunity, including:

r skin r the secretion of bactericidal enzymes such as lysozyme in tears, saliva and mucosal membranes

r hydrochloric acid found in the stomach r a sequence of enzymes collectively known as the complement system r thrombocytes (which help in blood clotting)

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r certain of the white blood cells known as myeloid cells, which consist of polymorphonuclear leukocytes, monocytes and tissue macrophages, eosinophils and basophils. All of these cells, with the exception of basophils, are involved in phagocytosis, which is the engulfment and destruction of foreign matter (including micro-organisms) which is achieved by biologically active molecules, such as enzymes and toxins, which are found inside these cells The activities of all the white blood cells in the innate immune system can be divided into three categories:

r Phagocytosis – the devouring and destruction of non-self matter. r Cytotoxicity – a means of killing infectious organisms by damaging their outer membranes.

r Inflammation – the body tissue response to infection and inflammation, or to damage. These white blood cells interact with other components of the non-specific immune system, such as the complement system (as well as components of the specific immune system) to protect the body from invading microorganisms.

Specific Immunity This is the part of the immune system that is particularly affected when a child has SCID. This is known as acquired immunity. Although the cells involved are present from birth, their functioning as part of the specific immune system only develops after birth following exposure to infecting micro-organisms. In fact, for the acquired immune system to function, it needs to come into contact with infectious micro-organisms, so that it is activated. It is called specific immunity because the protection this system gives to the body is specific only to organisms with which it has already come into contact. This system is made up of certain white blood cells, known as lymphocytes, of which there are two classes – the T-cell lymphocyte and the B-cell lymphocyte.

T-Cell Lymphocytes The immunity given by T-cell lymphocytes is also called cell-mediated immunity because the cells themselves are involved in the actual destruction of invading micro-organisms. T cells mature and differentiate in an organ called the thymus, which is situated in the chest. There they differentiate into four types of cell, namely:

r r r r

T-cytotoxic cells T-helper cells T-suppressor cells T-memory cells

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Whilst in the thymus, as well as differentiating into one of the above types of T-cell lymphocyte, the T cells also learn to distinguish self from non-self matter by means of recognition molecules on their surfaces. T-cytotoxic cells play an important role in defending the body against viral infections in particular. They are able to recognise cells which have been infected by viruses (and which hijack the cell to force it to make hundreds of new viruses) and to destroy them, thus preventing the release of these newly-manufactured viruses. In addition, they are implicated in the rejection of organ transplants because the cells of organs from another person do not carry the same recognition molecules on their surfaces as do the cells of the host body. T-memory cells are the cells which give this branch of the immune system its specific defence against micro-organisms, because, in simple terms, when an infectious micro-organism has been destroyed by the T cells, in conjunction with the rest of the immune system, these cells are then able to keep a record (or memory) of the recognition molecules of that particular micro-organism, and so any subsequent infections by that same type of micro-organism are instantly recognised. This means that the immune system can be immediately mobilised against it. The invading micro-organisms are then able to be quickly destroyed, often before they can cause any of the symptoms of infection. T-helper and T-suppressor cells together form the lynch pin of the specific immune defences, and much of the non-specific immune defence as well. When the T-helper cells come into contact and interact with an antigen (something that will cause an immune response), they release substances which stimulate the appropriate T-cytotoxic cells or B cells, as well as the phagocytic cells of the non-specific immune system, to proliferate and react to that particular antigen, be it a micro-organism or any other antigen in the body. By contrast, the role of the T-suppressor cells is to ‘switch off’ this proliferation and reaction in the other cells once the invading antigens has been destroyed. It is the balance between the actions of the T-helper and T-suppresser lymphocytes which forms the basis of our normal specific immune response. Too much T-suppresser action and too little activity from the Thelper cells means that the body cannot mount a satisfactory immune response. The reason the human immunodeficiency virus (HIV) is so dangerous is that the cells it attacks and destroys are mainly the T-helper lymphocytes, thereby reducing the ability of the body to mount a decent specific immune response. Thus, the T-cell lymphocytes have many functions, the main ones of which are summarised below.

r r r r r

They are of great importance in fighting viruses. They regulate the immune system. They fight fungal infections. They help to destroy bacteria. They are the body’s major defence against tuberculosis and other intracellular pathogens.

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r They reject non-self matter in the body, including, unfortunately, tissue transplants.

r They help to protect the body against the formation of tumours. B-Cell Lymphocytes The type of immunity given by B-cell lymphocytes is known as humoral immunity, because the effective components (the immunoglobulins – more commonly known as antibodies) are soluble in tissue and plasma fluid. This part of the immune system has nothing to do with being funny, it comes from the old English word for the fluids of the body, namely the four humours of blood, phlegm, black bile and yellow bile, and it was believed that for good health to occur these needed to be held in balance. B cells consist of two types of cell: r B-memory cells, which work in the same way on the B cells as do the T-memory cells on the T cells. r Plasma cells are formed from B cells which have been stimulated by the T-helper cells to proliferate and undergo this change. These plasma cells then manufacture specific antibodies, properly known as Immunoglobulins (Ig). During an infection, these immunoglobulins are released and become attached to the receptors on the cell surfaces of micro-organisms. Once attached to the cell surface of the infecting micro-organism, the other end of the immunoglobulin then becomes attached to receptors on the T-cytotoxic cells and also phagocytic cells. This means that the attached micro-organism is unable to escape from the cells of the immune system, and is held there until destroyed. In addition, some of the immunoglobulins (in particular, IgM and IgG – see below) are concerned with the stimulation of the complement system into action. Following the destruction of the infecting micro-organism, T-suppressor cells suppress the manufacture of the plasma cells, until the next infection occurs.

Immunoglobulins There are five classes of immunoglobulin (Ig) which are identified by the molecular weight of their heavy chain molecules. r Immunoglobulin G (IgG): This is an extremely important immunoglobulin. Over 70% of human immunoglobulins in serum (plasma) are IgG. It has the smallest molecular weight of all the immunoglobulins, so it is found in both intravascular and interstitial spaces. IgG is the major immunoglobulin involved in the secondary response to infections, and is also able to activate the complement system. Of particular importance is its ability to cross the placental barrier. It is the only immunoglobulin small enough to do this, and this importance is centred on the fact that when born, a baby has a very immature immune system and is initially unable to manufacture sufficient immunoglobulins for its defence. Because of the ability of IgG to cross the placental barrier,

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at birth the baby has a huge store of maternal IgG and all the protection that carries. The half-life of these maternal antibodies is from three to four months, by which time, unless the child has one of the B cell or combined immune deficiencies, he or she is able to produce sufficient IgG for his or her own defences.

r Immunoglobulin M (IgM): This immunoglobulin has the heaviest molec-

r

r

r

ular weight of any of the immunoglobulins and is constructed in the form of a pentamer (five immunoglobulin M molecules linked together). As a result of its size it is only found in the large intravascular spaces (i.e. the blood and lymph vessels). Following birth, IgM is the first immunoglobulin to be produced in sufficient quantities, and it is particularly important during the first few days of a primary immune response to an infection. Immunoglobulin A (IgA): This is another very important immunoglobulin. There are two types of IgA and they are found in two different environments within the body. Serum IgA is found in body fluids, such as blood, and secretory IgA, as its name suggests, is found in the body’s secretions. Secretory IgA is the major immunoglobulin found in all the secretions the body produces, such as saliva, tears, colostrum, breast milk, and the mucosal secretions of the nose, mouth, respiratory tract and gastro-intestinal tract. Consequently, secretory IgA has a very important role to play in protecting the body from infections of these areas. For example, it appears to be an essential part of the defence against the absorption of pathogenic micro-organisms that may be present in food. Because of the presence of secretory IgA in colostrum and breast milk, breast feeding a baby is a useful way of giving that baby some defence against pathogenic gut micro-organisms before the baby’s own immune system is capable of giving sufficient protection. IgA is the last immunoglobulin to be produced in the developing child, with full levels not being achieved until around puberty. Immunoglobulin E (IgE): There is still much to be discovered about this immunoglobulin, but it is known that levels in the body are higher in people who have an atopic disease (e.g. asthma and eczema), so this immunoglobulin is concerned with allergic reactions. Normally it is only found in small amounts in the body, but the levels are also raised following parasitic infections, therefore it is seen as an important component of the immune defence against parasites. Immunoglobulin D (IgD): Even less is known about this immunoglobulin, but it is thought to be active against food antigens. Again, only very small amounts of this immunoglobulin are found in the body.

Thus the workload of the B-cell lymphocytes can be summarised as:

r They are important in fighting bacteria. r The immunoglobulins help to bind phagocytes and other cells to immunogens (this is known as opsonisation).

r They trigger off the classical pathway of the complement system.

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Although this has been a very brief and simplistic account of the immune system, it is important to have a knowledge of the essentials in order to understand the effects of SCID in terms of the actual deficiency disorders which come under this term ‘SCID’ and the symptoms that are present with the disorders.

Severe Combined Immunodeficiency The following section will consider SCID itself.

History The history of primary immunodeficiency disorders is actually quite short, but eventful. In fact, primary immunodeficiency disorders were not really acknowledged until after the introduction of antibiotics because before antibiotics were developed, morbidity and mortality from infections were so high, that children with immunodeficiencies could not be identified within the population as a whole. It was only once antibiotics had been developed and introduced for general use, with the consequence that children recovered from infectious diseases such as pneumonia, meningitis, and measles, that it was possible to identify a population of children with immunodeficiencies (Stiehm et al. 2004). However, even before the widespread use of antibiotics, a few immunodeficiency syndromes had been described. These included:

r Ataxia telangiectasia (Syllaba & Henner 1926) r Mucocutaneous candidiasis (Thorpe & Handley 1929) r Wiskott–Aldrich syndrome (Wiskott 1937) In 1952, Ogden Bruton in the USA had a paper published in which he described a boy who had congenital agammaglobulinaemia, or an inability to produce immunoglobulins (antibodies). He not only described this particular primary immunodeficiency disorder, but he also described how he had successfully treated it, by infusing immunoglobulins into the boy. Bruton’s (1952) report has often been seen as marking the inception of the branch of medicine known as Immunology. Since this first description of a primary immune deficiency disorder, there have been more than 140 others that have been identified and described. From these early beginnings, the science and specialties of immunology and immunodeficiency took off. Although the first reporting of a primary immunodeficiency disorder is usually attributed to Bruton in 1952, an earlier description of an immune deficiency disorder had been given by Glanzmann and Riniker (1950) under the title ‘Essentielle Lymphocytophthise’ in which they discussed the cases of two children who were seen in the Universit¨ats-Kinderklinik, Bern, Switzerland, in 1948 and 1949, respectively. The course of the illness was summarised as consisting of out-of-the-ordinary infections, rampant

Severe Combined Immunodeficiency – A Brief Overview

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thrush, sepsis, pneumonia, convulsions and death. Both children had severe failure to thrive and died at the age of five and a half months. The autopsy summary read as follows, and even ignoring the technical jargon, it provides an insight into the complex and wide spread damage that is caused to children by the disease): The essential signs of idiopathic lymphocytosis are loss of lymphocytes in the gut, in the lymph nodes, and in the spleen, various degrees of proliferation ex vacuo in the reticular stroma which later on may show hyaline or fibrous degeneration. The disappearance of the Peyer Plaques may leave sharply defined bare patches on the mucous membrane which microscopically looks like cleared ulcers. The shape and size of the lymph nodes are present, in spite of the complete absence of the lymphocytes, through the widening and proliferation of the cortical and medullary sinus. The lymphocytes are also absent in the spleen. The bone marrow became excessively aplastic towards the end. The megakaryocytes persisted for a long time. Besides the constitutional factor leading to the lymphocytosis, disturbance of intestinal resorption on account of an infection with monilia albicans producing necrosis has to be made responsible for the lethal course of the illness. (Glanzmann & Riniker 1950 p. 31 – trans. Vickers 1999b) With hindsight, the account of the clinical picture and course of the disease, the autopsy results and the family medical histories leads to the conclusion that this is undoubtedly the first reported account of children suffering with an immunodeficiency disorder – in this case SCID. ¨ Later, in 1958, Hitzig et al., at the Kinderklinik, in Zurich, described two baby boys with agammaglobulinaemia and alymphocytosis with atrophy of the lymphatic tissue. They summarised their cases, thus: The case reports of two male infants are given, who displayed an extraordinary low resistance against infection. This is explained by a two-fold defect, namely of humoral antibodies (agammaglobulinaemia) and of cellular elements (alymphocytosis). The clinical course is therefore more severe and unmodified by therapy than is simple agammaglobulinaemia. The following criteria are emphasised: r Early onset after the end of the first trimester. r Severe diarrhoea which barely improved with therapy and which led to a profound cachexia. r Persistent low lymphocyte counts with inconstant changes of total leukocyte counts. r Very low gammaglobulin levels, lack of specific antibodies and natural isoagglutinins. r In spite of this, allergic reactions may be produced as in normal persons. r One patient developed a fatal haemolytic-uraemic syndrome with all the characteristic features of the generalised Sanarelli-Schwartzmann phenomenon. r One patient presented with a severe neurological disorder (right hemiplegia).

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The whole lymphatic system was atrophied and, in the remainder, most of the lymphoid tissue was replaced by giant, clear reticulocytes. (Hitzig et al. 1958 pp. 583–584 – trans. Vickers 1999b) Once again with hindsight, following the description of the clinical course, the autopsy results, and the family histories, these two babies could be rediagnosed as having SCID.

r It is interesting to note that two of the most common types of SCID are known as Swiss-type agammaglobulinaemia and

r X-linked lymphopaenic agammaglobulinaemia

This is a tribute to Switzerland’s lead in identifying the syndrome of SCID. More than 20 years ago, Dr John Soothill of the then Hospitals for Sick Children, Great Ormond Street, London, coined the term severe combined immunodeficiency syndrome (or SCIDS) for this condition which was appearing with increasing frequency in the medical literature about children (Rosen 1997). In the ensuing years we have learnt a great deal about both combined and severe combined immunodeficiencies, although there is still much more to discover. We now accept that SCID is a paediatric emergency and needs to be diagnosed and treated as soon after birth as possible (Rosen 1997). Box 3.1 gives a list of some of the more common and better-known combined immunodeficiencies that have been identified. The majority of severe combined immunodeficiency disorders are genetic in origin, either autosomal recessive or X-linked recessive (as discussed more fully in the section below on the causes of SCID), so if one child in the family is affected, there is a chance of other siblings also being affected or being a carrier. One particular primary immunodeficiency disorder (congenital thymic aplasia or DiGeorge syndrome) is thought not to be genetic in origin, but rather occurs due to a failure of mid-line development in the early stages of embryonic life. The abnormalities associated with this disorder appear to be a result of interference of normal embryogenesis between the sixth and tenth weeks of embryonic life (Amman & Hong 1989). For some other disorders, a cause has not yet been found.

Incidence of SCIDS Since the early 1960s, there have been many cases of SCIDS reported, and although it is impossible to put an accurate figure on the incidence of SCIDS, it has been guestimated at approximately 1:30 000 live births, in the United Kingdom, i.e. 30 cases or so diagnosed a year, but there is no hard statistical evidence for this. An Australian study in 1983 put the figure at 1:66 000 (Robertson et al. 1983) but this is now assumed to be a gross underestimate. Even the UK figure could be an underestimation because of failure to diagnose the condition, although following several initiatives in the early 1990s, the rate of diagnosis is much improved. The problems of diagnosis were brought to the fore by the mother of one of the girls

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in the study. Talking about the three of her children who died whilst still babies, she said ‘The diagnosis was, errh . . . cystic fibrosis. They have since recognised that it was SCID. They looked through the notes again and said that’.

Cause of SCIDS SCIDS, with the exception of one or two particular classifications (e.g. DiGeorge syndrome), are disorders of genetic origin (Amman 1987, Amman & Hong 1989). They are transmitted in one of three ways: 1. X-linked recessive 2. autosomal recessive 3. spontaneous genetic mutation Some of these genes have been mapped, for example, X-linked SCID on chromosome Xq13, and ADA deficiency SCID on chromosome 10q13-ter. PNP SCID (Purine nucleoside phosphorylase SCID – caused by an enzyme defect and similar to ADA SCID) has also been mapped on 14q13.1 (Buckley 1994). X-linked SCID is believed to be the most common form of SCID in the USA (Conley 1991, Conley et al. 1990) and this immunodeficiency is caused by the gene encoding for the Interleukin 2 (IL-2) receptor, which plays a key role in intracellular signalling in T cells and, consequently, in the function and regulation of the immune system. Once genes underpinning the other types of SCID have been mapped, there may well be similar causes of these diseases identified, although it is thought that the genetic faults of some of the others encompass more than a single gene, and so the cause and subsequent treatment of these diseases may be even more complicated.

Signs, Symptoms and Diagnosis of SCID Whatever the particular cause of the severe immune deficiency, the major signs and symptoms of the disorder are all similar. The onset of symptoms usually occurs well before six months of age when most of the maternal IgG has disappeared and the baby has not been able to make any of its own as replacement. There are four cardinal signs and symptoms which should lead to a suspicion of a severe immunodeficiency disorder. The first is infection, both chronic and recurrent, especially of the upper respiratory tract, otitis media (infection of the middle ear) and lungs. These are particularly suspicious if the infections are caused by unusual micro-organisms and if they do not respond well to the usual antimicrobial therapies. The other three highly suspicious presenting signs and symptoms are failure to thrive, diarrhoea and intractable oral thrush. Other features which are potentially diagnostic of SCID are skin rashes and hepatosplenomegaly (enlargement of the liver and spleen) as shown in Box 3.2.

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Box 3.2

Signs and Symptoms of SCID

Onset of symptoms by six months of age Recurrent and chronic viral, bacterial and protozoal infections Infection by opportunistic micro-organisms Inadequate clearing of infections by antimicrobial drugs Failure to thrive Chronic diarrhoea Oral candidiasis Skin rashes Occasionally hepatosplenomegaly Absence of both T- and B-cell immunity

SCID: Pre-Transplant Disorders and Infections The above signs and symptoms were displayed by most of the children in the research study. In the early months of life, prior to a bone marrow transplant (BMT), the children in the study generally suffered major health problems, as shown in Table 3.1, which details the problems that the children were suffering from prior to diagnosis of SCID, in addition to their underlying immunodeficiency disorder. Only some of the children who were diagnosed at birth and treated within three months escaped serious life-threatening illnesses prior to their first BMT. For those children in this study with other serious problems, outside of SCIDS, failure to thrive was the most common complicating symptom with 17 out of the 55 children (31%) being reported as failing to thrive at the time of their SCIDS diagnosis. This is followed by chest infections with 13 children (24%) being affected. However, if the 23 children who were diagnosed at, or shortly after, birth are excluded, remembering that Table 3.1 Some of the Combined Immunodeficiency Disorders Medical problems at diagnosis of SCIDS Diagnosis Failure to thrive Chest infections Maternal–fetal GvHD Gut problems BCG-itis Skin problems Agranulocytosis Sepsis Pneumocystis carinii pneumonitis Hepatitis B Stridor Muscle hypotonia

Number of children (n = 55)

Percentage

17 13 10 10 9 4 3 3 3 2 1 1

31 24 18 18 16 7 5 5 5 3 2 2

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Table 3.2 Signs and Symptoms of SCID Pre-transplant disorders Disorder Failure to thrive Skin problems Poor general condition Anaemia Gut problemsa Inguinal hernia Lymphadenopathy Othersb

Number of children (n = 32)

Percentage

20 13 12 5 4 2 2 1

62.5 41.0 37.5 16.0 12.5 6.0 6.0 3.0

a

Includes both chronic malabsorption and food intolerance. Includes the remaining disorders each of which affected just one child. They were: iron deficiency, agranulocytosis, stridor, graft-versus-host disease, hydrocele, hypernatraemia, hypogonadism, muscle hypotonia, fibrotic lung disease, ‘crumbling’ nails, poor hair growth, hepatosplenomegaly, intraventricular haemorrhage, cerebral palsy with spastic hemiplegia, patent duct arteriosus, cardiomyopathy, talipes equinovarus and developmental problems. b

problems usually only start to occur from the third or fourth month of life when maternal immunity is on the wane, then the percentage of children appearing at diagnosis with failure to thrive is 53%, and with chest infections is 41%. Other medical problems arose or were diagnosed in the period following the initial SCID diagnosis and the BMT. These disorders are detailed in Table 3.2. As well as the pre-transplant disorders, pre-transplant infections were also a major problem. As some of the children were diagnosed at birth and nursed in an isolation cubicle from that point until a successful transplant had taken place, only 35 of the 55 children were recorded as having infections pre-transplant. These infections are listed in Table 3.3. These figures do not include repeat infections in the same children. As can be seen, chest infections, gastro-intestinal tract infections and upper respiratory tract infections are the most common infections. BCGTB occurred as a result of the children being given the live BCG vaccine shortly after birth. This occurs more frequently in Germany (where it is known as BCG-itis) than in the UK; amongst the German children in this study, 7 out of 16 children were affected, whilst in the UK, the incidence was only 3 out of 19. These figures result from the more widespread German practice of immunising all children against TB within a few days of birth, whereas in the UK, only certain areas or groups are targeted. Whilst the disorders depicted in Table 3.2 were present before, or shortly after, the initial diagnosis of SCID, but were not initially picked up, the infections mentioned in Table 3.3 all occurred following the diagnosis of SCID. The sheer numbers and types of infections make a very strong case for the early diagnosis and treatment of children with SCID before the children become weakened by these multiple infections, perhaps even succumb to them and die before they are able to have a BMT.

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Table 3.3 Other Problems Prior to SCIDS Diagnosis Pre-transplant infections Infection Chest infection GIT infection URTI BCG–TB Candida Septicaemia Skin infection Ear infection Eye infection UTI Chicken pox Hepatitis B Alveolitis Umbilical infection Necrotising enterocolitis Tonsillitis Others (not stated)

Number of children (n = 35)

Percentage

19 15 13 10 10 8 7 5 4 4 1 1 1 1 1 1 4

54 43 37 29 29 23 20 14 12 12 3 3 3 3 3 3 14

If, following clinical examination, plus the family medical histories, the initial suspicions of a severe immunodeficiency disorder are strong enough to warrant further investigations, then blood samples are taken for immunological diagnosis. If SCID is to be confirmed, then these tests would have to show a complete or partial absence of T- and B-cell lymphocyte immunity, although not necessarily an absence of T- and B-cell lymphocytes themselves.

Treatment of SCID Once the primary immunodeficiency disorder has been identified, treatment can be initiated. This could range from very simple and basic, yet vitally important care, that ensures adequate nutrition that maintains body protein levels (Westwood 1997), to Bone Marrow Transplants BMTs, and, more recently, gene replacement therapy (Vickers 1990a). Box 3.3 summarises the treatment possibilities for SCID.

r Palliative therapy: The prognosis of an infant with SCID is very poor, and most will die as a result of severe overwhelming infections long before they are a year old. Palliative treatments including replacement immunoglobulin infusions, antibiotic, antiviral and antifungal drugs are of limited use and can still at best only marginally prolong survival (Fischer 2000). Isolation (reverse barrier isolation) can also help to prolong life, but it will not be too long before the infant succumbs to an infection.

Severe Combined Immunodeficiency – A Brief Overview

Box 3.3

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Treatments for Immune Deficiencies

For all defects: Recognition Prevention of infections Immunisation (only using killed vaccines) Prophylactic antibiotics Symptomatic treatment – of infections, anorexia etc. For special defects: Immunoglobulin replacement therapy (where B cells are affected) Fetal thymus transplant – particularly for DiGeorge syndrome Bone marrow transplant – for serious disorders, particularly where T cells are involved Fetal liver transplant – has been used for SCIDS Enzyme replacement therapy – has been used for ADA SCID Gene replacement therapy – has been used for ADA SCID and X-linked SCID Peripheral blood stem transplants – now being used more and more in place of bone marrow transplants

As with other immune deficiency disorders, avoidance of live vaccines, such as bacillus Calmette-Guer`en (BCG), and live polio vaccine are essential to prevent infection from these vaccines. Luckily, the latest immunisation schedule from the Department of Health (2006) has substituted an inactivated polio vaccine for the live vaccine, but until recently children with undiagnosed severe combined or combined immunodeficiency have died as a result of receiving these vaccines.

r Enzyme substitution: This treatment has been used for children with adenosine deaminase (ADA) SCID for several years now. It consists of once-weekly subcutaneous or intramuscular injections of PEG-ADA, or to give it its full name, polyethylene glycol-modified bovine adenosine deaminase (Buckley 2002, Fischer 2000). This usually results in normal levels of the enzyme in the blood cells, and the actual numbers of T cells rise within a few weeks of commencing treatment, along with evidence of specific T-cell immune responses (Hershfield et al. 1993, Hirschhorn et al. 1996). Unfortunately, although successful in approximately 90% of cases, some patients develop antibodies to this bovine PEG-ADA, and so the dose has to be gradually increased. In addition, the quality of T-cell immunity may decline with time (Fischer 2000).

r Thymic transplants: Although infants with partial DiGeorge syndrome require no immunologic treatment, those with full DiGeorge syndrome have had successful reconstitution of immunologic function following

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r

the transplantation of cultured mature thymic epithelial cells (Buckley 2002, Markert et al. 1999). Bone marrow/stem cell transplants: Over the past 35 years or so bone marrow and stem cell transplants have been used to treat several haematological and immunological malignancies such as leukaemia, reduced myeloid cell production such as aplastic anaemia and immunodeficiency disorders such as SCID (Vickers 2005). Stem cells are obtainable from bone marrow, peripheral stem cells and cord blood. Cord blood transplants particularly have received much publicity from the media.

For many years, the standard, and only, curative treatment for SCID was an allogeneic BMT as described by Gatti et al. (1969). Bone marrow is harvested from a matched donor, i.e. one who has identical human leukocyte antigens (HLA) to the recipient, ideally an identical twin. HLA consists of the genes that code for our own identity cell receptors. If the HLA does not match then rejection of transplant, or host, will occur, as the body’s T cells recognise the cells with a different HLA as foreign and so try to destroy them. Children with SCID have very few functioning T cells, and so the problem is more likely to be the T cells in the transplanted bone marrow destroying the child’s cells (the opposite to graft rejection). To combat this complication, the harvested bone marrow is then purged of almost all of the mature T cells, because these are the cells that may cause graft-versushost disease – GvHD – a case of the graft rejecting the recipient’s cells which was often fatal in the early days. The purged bone marrow is then transfused into the recipient. There is however one problem with allogeneic BMTs for SCID and that is the fact that because SCID is a genetic, inherited condition, it is often rare to find anyone else within the family who has the same HLA as the recipient and has not died as a result of having SCID. So, very few infants with SCID survived until in the early 1980s when a medical procedure was developed to overcome this problem. This development was the use of haploidentical (or mismatched) BMTs usually using one of the parents as the donor. They were mismatched, because only half the HLA were identical (humans inherit two sets of HLA genes – one from the mother and one from the father, and unless the parents are closely related, the two sets of HLA genes are usually totally different, so in haploidentical transplants, there is only half a match). This was achievable because the recipient with SCID does not have functioning T cells, so there is no, or very minimal, myeloablation or immunosuppression required in order to attain engraftment (Buckley 2000, 2004, Vickers 2005). In the absence of GvHD, by 1999, the survival rate of infants with SCID receiving haploidentical stem cell transplantation was as high as 78% (Buckley et al. 1999). There have also been a few successful haploidentical stem cell transplants in utero on fetuses that have been diagnosed as having SCID (Flake & Zanjani 1997). There is probably no more sterile environment than the uterus, and personal experience has certainly shown that the younger the

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infant is when transplanted, the better the result because of the immaturity of the infant’s immune system. Children diagnosed at or before birth, isolated immediately, and transplanted within a very short time have much better survival chances (Vickers 1999b). In more recent years, the development of peripheral stem cell transplants and cord blood transplants have also been used. A matched HLAidentical cord blood transplant is rarely available, but with the use of cord blood banks, a matched sibling cord blood transplant could be used in the future (Stiehm et al. 2004). Following successful engraftment, there have, to date, been no relapses, and it is hopeful that the treatment is permanent, but it is still early days – although the first successful transplants took place nearly 40 years ago (Stiehm et al. 2004, Vickers 1999b).

r Gene replacement therapy: At the moment, only two types of SCID have been successfully treated with gene replacement therapy – ADA SCID and X-linked SCID. For gene replacement therapy to be used, and successful, it is, of course, important for the identification and cloning of the abnormal or absent gene causing the disorder to be possible (Fischer & Notarangelo 2004). This has occurred in the above two SCID disorders. The isolated correct gene is first of all inserted into peripheral white blood cells or bone marrow stem cells from the child using a viral vector (a vector is a vessel for carrying something – in this case the cloned gene). The cells are then infused back into the infant. (Fischer & Notarangelo 2004). The first ever attempts for gene replacement therapy were undertaken with infants with ADA SCID, but were only partially successful, as the children needed to continue with PEG-ADA (Blaese 1993, Fischer & Notarangelo 2004). However, three years ago, there was successful gene replacement therapy in an infant with ADA SCID without the continuation of PEG-ADA (Aiuti et al. 2002). In addition, there have been several cases of infant boys with X-linked SCID being successfully treated with gene replacement therapy (Buckley 2004, Cavazzano-Calvi et al. 2000, Hacein-Bey-Abina et al. 2002). Unfortunately, two of the boys developed leukaemia as a result of the position within the chromosome that the cloned genes occupied. Both boys have since been successfully treated for leukaemia, but it did mean the suspension of gene replacement therapy until the problem was addressed (Buckley 2004, Hacein-Bey-Abina et al. 2002). Happily, the programme of gene replacement therapy has since successfully restarted. Although there is still much work to be done in this area, there are possibilities of gene replacement therapy being used to treat other primary immunodeficiencies in which the gene defect has been identified, particularly where the defect is caused by a single gene rather than by multiple genes (Vickers 2005).

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Conclusion In the first 50 or so years following Bruton’s diagnosis and reporting of agammaglobulinaemia, research has brought an increased understanding of the immune system and there has been a marked increase in the reporting of additional immunodeficiency disorders. The last 25 years, however, have also seen great advances to both the identification of the causes and to the treatment of primary immunodeficiencies (Fischer 2000). Historically, however, for the great majority of children with SCID, the only choice has been between a BMT and death. With SCID there is no grey area, either the infant is successfully treated or death occurs within a very few months. All the children in this present study received a BMT, and before, during and after the transplant, specialist nursing and medical care had to be initiated and vigorously maintained (Amman 1987, Fonger et al. 1987, Gurevich & Tafuro 1985, Vickers 1990b, 1990c). Aggressive treatment of current infections by means of intravenous antimicrobial drugs, and prevention of future infections by strict isolation using reverse barrier nursing techniques, as well as attention to strict infectious precautions are crucially important. There have been a variety of methods utilised in achieving this strict isolation, for example miniisolators, laminar air flow rooms and positive air pressure tents, but in all cases the type of isolation used needs to be supported by scrupulous and intensive attention to detail by all members of the transplant team in order to prevent the child suffering a serious, and possibly fatal, infection. Allied to this is the fact that a child with SCID may need to be isolated for the best part of a year or even longer – as has already been mentioned, one of the children in the study was not released from her isolation cubicle until more than two years of living in a very strict isolation environment had elapsed. Never letting standards slip in these cases is dependent upon a very dedicated and knowledgeable transplant team as well as a knowledgeable family. The child’s nutritional status will need to be improved (Cunningham et al. 1983, Westwood 1997). This almost invariably means the use of total parenteral nutrition (TPN) administered via a central line. Many weeks of this type of feeding are necessary, but if TPN is not in situ, the hope is to maintain the child on special oral feeds as soon as possible, and for as long as possible, in order to try and prevent the physical and psychological problems caused by oral deprivation. The children during this period, and usually for some time following a successful BMT, will all need to receive intravenous immunoglobulin (gammaglobulin) infusions regularly so as to replace their own missing antibodies. These infusions consist of immunoglobulin G and help to give some protection to the child against infection whilst they are themselves deficient in antibodies. Another precaution that is essential is the irradiation to 4000 rads of all blood products (Amman & Hong 1989). This is to try and prevent

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GvHD occurring due to the absence of functioning T-cell lymphocytes in the child’s blood. Their lack of immunity means that the children often succumb very quickly to sepsis, so continuous round-the-clock vigilance is once again seen to be a vital component of their care. The very fact that children with SCID have very little immunity means that they do not always react to infections in the normal way. Throughout the many seemingly endless months, regular monitoring of electrolyte balance, of weight to check nutritional and hydrational status, of post-transplant blood cultures, of bacterial and viral sampling, and of testing for immunological function also takes place. The maintenance of excellent oral hygiene is a high priority, both to prevent infections and also to aid the child in continuing with oral feeding and stimulation for as long as possible. Along with this, specific skin care needs to be religiously carried out to prevent it from drying, cracking and eventually breaking down. Once this does occur, it is very difficult to remedy, and is painful for the child, there can be loss of valuable fluid and proteins through the skin, and most importantly it can provide another ready access point into the body for any pathogenic micro-organisms. It can be seen that for children with SCID, certainly the ones in the study, the early months (and years) are very precarious, requiring the utmost dedication, not only from hospital personnel, but also from the child’s family.

4

Effects of Early Hospitalisation on Children

Introduction The risk of upset and even disturbance of the social and emotional development of young children as a result of hospitalisation has become a cause of widespread concern since the 1950s (Fahrenfort et al. 1996). According to Vernon et al. (1966), children between the ages of six months and four years are most likely to be upset following hospitalisation. ‘Toddlers and preschool children have been identified as being at risk for post-hospital behavioral sequelae, especially when confronted with an unanticipated intensive care (ICU) hospitalization’ (Small & Melnyk 2006 p. 226). This was not always understood to be so, as can be testified from my own personal experience. As discussed in the first chapter, in 1951, at the age of eight years, I was admitted into hospital in the early hours of a Sunday morning following a serious accident. My parents were told by the doctors that I would not survive. In spite of that my parents had to leave me at the entrance to the ward and find their own way home at 2 a.m. – not easy in 1951 Manchester (so no care for the family in those days). Despite my not being expected to survive, my parents were not allowed to see me until the next visiting period which was for one hour on Saturday afternoons. So, they left me and did not see me for another week at a time when they were expecting to be told that I had died. There was no thought given as to what effect this sudden separation would have on me, nor whether my development would be affected – or even whether I was happy, upset, coping with a severe head injury and being away from home etc. This situation had completely changed, I was pleased to see, when I started as a children’s nurse in the early 1980s. So, how did these changes come about? Since the 1970s, there have been tremendous changes in the way in which children are cared for in hospital, not least from a 48

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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psychosocial position, and it all really started the same year that I had my own personal experience of the care of children in hospital. Some of these changes that have improved child coping mechanisms during hospitalisation include parents being able to stay with the child in hospital, and the complete liberalisation of visiting hours in an effort to minimise the problems caused by parental deprivation (Dougherty 1998, Melnyk et al. 1997, 2004, Small & Melnyk 2006, Wise 2004). The first person to start to make a difference was John Bowlby who, in 1951, started to look at the effects that hospitalisation, particularly early hospitalisation, could have on children. From this, changes started to be made to the system of caring for children in hospital. Until we have now reached the present system of family-centred care.

A Brief History of Studies into Early Hospitalisation As early as 1943, Edelston, after having reviewed many clinical cases, concluded that although some young children were not particular disturbed by being in hospital, others responded with:

r r r r r r r

screaming nightmares sleep disturbances fear of separation from the mother clinging behaviour docile and spiritless behaviour obstinate and negative behaviour

He said that, as a general rule, the younger the child was at admission to hospital then the more profound were the disturbances. He felt that the determining factor as to whether or not the child would be distressed appeared to be the degree of rejection or insecurity that was felt by the child. Indeed, in the year that I was born, he strongly supported liberal visiting policies as a major way of ameliorating these effects. Eight years later, short weekly visits were still the norm. Bowlby (1951) claimed that early life experiences may have serious effects on intellectual and psychosocial development. Since then work by Rutter and others has backed this claim up and has demonstrated that, in certain circumstances, early experiences can and do have important effects on their development (Rutter 1972, 1979, Rutter and Madge 1976). In addition, Bowlby (1951) dwelt on the problems associated with maternal deprivation in the early years of life. However, Rutter (1980) made the point that severe maternal deprivation in the first two years of life does not necessarily inevitably lead to intellectual, emotional and psychosocial development. Rutter (1980) also debates that the long-term effects of early deprivation do depend very much on whether the deprivation is of short duration, or whether it continues for some time. However, of great importance was the work of Bowlby (1960) whose work on anxiety separation

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led to much further research into this problem by people such as Rutter above, and Robertson (1958) who did so much to highlight the problems that young children encounter when hospitalised. However, Robertson will always be remembered for his 1952 film ‘A two-year-old goes to hospital’ which has been rightly acclaimed. In this film, which is still available, a two-year old is admitted for a routine operation. The film shows how her behaviour dramatically changes from distress, crying and violent objections to withdrawal and depression over the course of her ten-day stay in hospital. Over 50 years later, this film remains an emotionally demanding experience and is still very difficult to sit through, but should remain as required viewing for all involved with children in hospital. Truly is one picture worth a thousand words. Anna Freud (1953), after seeing the film and being very positive about it and its merit in challenging practice at the time, did chide Robertson for calling the little girl’s operation for repair of an umbilical hernia ‘minor’, as she insisted that to the little girl it was anything but minor, and that the child’s own internal reality of the experience is the only reality that counts. As King and Ziegler point out in 1981, what would Freud’s reaction be to the typical hospital procedures that were current then (and now) and indeed, even in the early 1950s. The concept of the development of children being affected by early hospitalisation has been well researched in the past, particularly by Rutter (1980), Bowlby (1951, 1960) and Douglas (1975). The Platt Report (Ministry of Health and Central Health Services Council 1959) on the care of children in hospital also needs to receive an honourable mention here because of its influence on paediatric nursing in the last 35 years. Douglas (1975 p. 476) reported that his study had provided ‘strong and unexpected evidence that one admission to hospital of more than a week’s duration or repeated admissions before the age of five years (in particular between six months and four years) are associated with an increased risk of behaviour disturbance and poor reading in adolescence’. In addition, he reported that nervous, shy or withdrawn behaviour in children does not positively correlate with early hospital admissions. This research was confirmed in 1976 by Quinton and Rutter, who replicated Douglas’s findings that single hospital admissions of children for up to a week carry no increased risk of later emotional or behavioural disturbance, but that there is a positive correlation between repeated hospital admissions and disturbance in adolescence. Rutter (1980) comes to the conclusion that the effects of early hospitalisation on adult behaviour is minimal, unless the deprivation caused by early hospital admission continues in the long-term. Maier (1978) states that children learn to think and understand by building all new experiences (like bricks in a wall) block by block on to previous experiences. So, early childhood becomes a time of ‘trial-and-error’ learning as experiences analysed (either subconsciously or consciously) and lessons learned from them – whether well or not is, of course, the important point here. The child experiments with different coping mechanisms and solutions as they encounter each new experience. This is an important concept to consider in relation to children who spend a lot of time in

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hospital for, as Wells et al. (1994) point out, these children may have few opportunities for such experimentation. Since the 1970s, research has shown that following hospitalisation, negative emotional and behavioural changes have occurred in up to 80% of children (Dearden 1970). And it has not improved much since the 1970s. Bonn (1994), Hart and Bossert (1994) and Melnyk et al. (2004) have found that behavioural and emotional problems are still commonplace following a child’s stay in hospital, with these problems being found more frequently and more long-lasting in children younger than seven years of age compared to the older children (Chelsa 1996, Haslum 1988, Zieglar & Prior 1994). Something that has changed over the past 30 years has been the length of stay in hospital that children experience (however, it has to be noted that children in hospital tend often to be much sicker than a generation ago). Inguinal hernia repair is an example of how much shorter hospital stays are. When I started nursing in the early 1980s, children having an inguinal hernia repair spent two weeks in hospital. Now they are in and out of hospital in one day. (Actually, in one hospital in the 1980s in which I worked, where there were two consultant surgeons who undertook inguinal hernia repair on children, we had the situation where one surgeon kept them in for two weeks whilst the other surgeon, with children on the same ward, kept them in hospital only for two to three days. It was very confusing for the children, their mothers, and, of course, the nursing staff.) It would make sense to presume that the shorter stays plus the improved contact between parents/family and child would have meant that the problems inherent in early hospitalisation would have disappeared – or at the very least been ameliorated. According to Small and Melnyk (2006), there is evidence that these shorter hospital stays may actually exacerbate the problems. Teichman et al. (1986) and Wright (1995) have suggested that short hospital stays of two to four days are more likely to result in more behavioural problems than are hospital stays of five to eight days. Small and Melnyk (2006) do assert that, with the short hospital stays prevalent now, it is very important that we can identify factors that could predict problems that children may have in adjusting to their hospitalisation. As they say, ‘psychosocial assessments and care of pediatric patients must be deliverable in an expedited fashion that corresponds with the rapidity of medical care delivery and in a manner that does not impinge on nurses’ abilities to provide quality, prioritized physiologic care’ (Small & Melnyk 2006 p. 623).

The Effects of Early Hospitalisation on the Development of Children Almost 40 years ago, Schaffer and Callender (1959) studied 76 infants undergoing hospitalisation. The infants were all aged less than 12 months and underwent short-term hospitalisation. They found evidence for the existence of two main syndromes in these children. The first syndrome

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was found in children over seven months of age. These infants appeared upset when admitted to hospital, as well as experiencing a period of disturbance after they had returned home. Both these problems centred on the need for the physical presence of the mother. The second syndrome appeared in the behavioural patterns of babies under seven months of age. Here, separation was not followed by any observable disturbance. Such young babies were able to make an immediate adjustment to new people. On returning home, however, there was a marked change in behaviour which only lasted for a very brief period. Therefore, the authors concluded that a baby’s separation from his mother was more traumatic after six months of age than if he or she was separated from his mother before that age. However, Freedman et al. (1976) pointed out that it is just possible that the problems occurring as a result of traumatic infant experiences may not manifest themselves until much later in life. This belief is related to their study of a boy with severe combined immunodeficiency who, at the time of publication of the paper, had, since his birth been living in a reverse barrier nursing environment for 52 months, with no physical contact other than via plastic gloves built into his isolator. Douglas (1975) suggested that there was no evidence for a causal relationship between early hospital admissions and later behaviour. Rather he discussed the social model of Stacey et al. (1970) which, at some level, is persuasive. Simplified, this model states that a child’s expectations of behaviour are formulated within the child’s own social network, and that within that network, e.g. home, he behaves in certain ways because that is what is expected of him. However, once he is admitted to hospital he will modify his behaviour to encompass the new environment (a more formal and hierarchical environment than home) and expected behaviours that he is presented with in hospital, whilst at the same time trying to make sense of that environment within the known expectations of behaviour that he experienced at home. Once the chid is discharged from home he has to fit back into the expected modes of behaviour that are required in his home environment, but he finds it difficult because he has learned new behaviours that allowed him to cope with the hospital environment. In other words, he returns home with changed expectations – expectations of which his parents and siblings are unaware. Douglas (1975) points out that this can cause problems, particularly if the child has repeated hospitalisations, which can introduce a discontinuity to his behavioural development. In addition, not only does the child who has been in hospital have to make adjustments, but also so do his parents and siblings. However, they may find this difficult to do because they do not have any understanding of the experiences that the child has because they did not experience them themselves. This can lead to confrontations and a change of expectations within the family, which may lead to behavioural problems particularly in the child and siblings. In other words, as Douglas 1975 p. 474) puts it, ‘the children who have repeated admissions to hospital in early childhood find it difficult to adjust to the conflicting demands of home and institution (hospital), and suffer for it’.

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Douglas (1975), however, believed that there was a much simpler interpretation of the phenomenon of altered child behaviour following hospitalisation. He believed that it is down to whether or not the child is insecure at the time of admission to hospital. If the child is insecure when admitted to hospital then he is particularly likely to have long-term behavioural problems present. Douglas (1975 p. 475) gives the following as examples of situations in which the child could be insecure when hospitalised:

r Being highly dependent on mother and not used to substitute care. r Has had a recent disturbing experience such as the birth of a new sibling or the mother returning to work.

r Having to compete with an older sibling. Douglas (1975) concludes his paper by stating that the risk of altered and disturbed behaviour could be reduced or eliminated if children who fit into one of the categories above, or were insecure for any other reason, were either treated at home or had their hospital admission postponed. Whilst this may seem sensible, of course not all children and families have such choices. The desperately sick child these days requiring very high medical and nursing standards of care and treatment needs to be in hospital where they can access that care and treatment, whether they are insecure or not. In addition, the very sick neonate may not have left hospital since birth because of the seriousness of the medical condition. So not all children who require hospitalisation would fit into Douglas’s explanation and suggestion, and this particularly applies to the critically ill child. In a follow-up study to Douglas’s (1975) study discussed above, Quinton and Rutter (1976) confirmed most of the negative findings. In particular, they did confirm that single hospital admissions lasting no more than one week were not associated with any form of emotional or behavioural dysfunction later on. However, they did confirm that repeated hospital admissions were significantly associated with later emotional and behavioural dysfunction.

Surgery and Hospitalisation Research by Ludman et al. (1992) explored the effects of major neonatal surgery on full-term babies who were consequently hospitalised in the first six months of life. This research obviously added a new dimension to the hospitalisation debate by including the traumatic event of surgery. This is important, because unlike the reports by Douglas (1975) and Quinton and Rutter (1976), this research took place at a time when children and infants generally were only hospitalised for serious illness, so all infants and children at that time, and since, can expect to undergo various traumatic events such as surgery or other treatments associated with critical care. What Ludman et al. (1992) found was that for full-term babies who were undergoing major neonatal surgery, hospitalisation in the first six months

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of life did increase the risk of behavioural problems as well as difficulties in the mother–child relationship by the age of three years. Within this group of neonates, they found that a long first admission was certainly associated with increased parenting difficulties by the time the child was three years old. In addition, they found the behavioural difficulties were increased in those children with lengthy first admissions as well as amongst those who had repeat hospital admissions. Ludman et al. (1992) found that there were certain family factors, or should we say maternal factors, which appeared to contribute to this association of behavioural difficulties and increased parenting difficulties. These maternal factors were said to be:

r the age of the mother r the mother’s educational attainments r whether or not this infant was the mother’s first baby However, when there was a long first admission but not repeat hospital admissions, none of the children had behavioural problems. In addition, it was said that only 20% of the mothers of this group were rated as having poor parenting skills. It was also suggested that more of the mothers of infants in the group who had a lengthy first admission, but not followed by repeat admissions, were supported by good relationship with their partners. This suggestion that, as with children in isolation, the support of the mother’s partner is of importance is something that is self-evident, but has not really been looked at in these earlier papers. Thus, Ludman et al. (1992) concluded that the findings demonstrated that it is a combination of risk factors – both family and social factors, rather than any single one, which is important when looking at whether or not there would be an increased risk of short-term or long-term adverse consequences as a result of early hospitalisation. A conclusion from this research was that the mothers had benefited from psychological support at the same level as medical and surgical care that the children received. Again, it is interesting to note the absence of fathers in this research. Once again, the role of the father in the care of young children is downgraded to just one sentence in the whole report, and that in terms of supporting the mothers. Another research study, this time in 1996, by Fahrenfort et al. also looked at the potential for emotional disturbance three years after early hospitalisation. The findings backed up those of the earlier report by Ludman et al. (1992) by demonstrating that scores on the Behaviour Checklist were statistically significantly raised in this group of children who had had an early hospitalisation as opposed to a control group without early hospitalisation. The behaviours that they identified as being troublesome included poor concentration, inappropriate attention seeking, poor bowel control and fearfulness/anxiety. They also found that those children who had early hospitalisation and surgery had the highest behavioural and emotional disturbances. Thus, taking this research and the research by Ludman et al. (1992), it can be seen that it is not just being hospitalised at an early age that causes emotional and behavioural difficulties later on, but also any accompanying trauma – in this case the trauma of surgery.

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This link between surgery and hospitalisation in children is expanded upon in a review by Caldas et al. (2004). What they discovered was that surgery in children, even routine and minor surgery, along with everything that goes with surgery such as analgesia, anaesthetics, hospitalisation, etc., may be experienced as distressing life events by children and their parents. These distressing life events cause anxiety, and this anxiety has an impact not only at the time that the surgery takes place but also on the time afterwards – the recovery period. Those patients who have lower levels of anxiety appeared to recover more quickly (Quilles et al. 2001). In addition, they also appeared to show fewer emotional and behavioural problems following discharge (Lumley et al. 1993). On the other hand, those patients who experience high level of anxiety have an increased possibility of negative psychological consequences (Caldas et al. 2004), such as:

r r r r

continuing anxiety depression irritability aggressiveness

Along with these behavioural problems, Quilles et al. (2001), point out that there are also medical consequences for patients with high levels of anxiety, and these can include:

r r r r r

more frequent pain worse scarring lowered defences to infection increased need of analgesia/sedation lack of cooperation with health professionals

Caldas et al. (2004) also point out that the anaesthetic drugs (even for a relatively short operation) can themselves have an affect on the child’s cerebral functioning, which may in turn bring negative consequences to the normal development of the child that affects emotional, social, behavioural, cognitive and academic levels, in either the short-term, the medium-term or the long-term. Anxieties that the parents have may also affect perioperative problems in their children (Caldas et al. 2004). However, Caldas et al. (2004) also put forward their concerns regarding present health care practices and policies and their role in fostering problems concerning emotional and behavioural development following surgery and hospitalisation in children. As Caldas et al. (2004 p. 911) state ‘the trends in health care management aimed at reducing waiting lists for surgery, may ignore, or relegate to second place, prevention and negative social and psychological consequences of anesthesia, surgery and hospitalization. These strategies may result in medium and long-term economic and social costs from lack of such preventive measures’. In further exploring the effects of major surgical trauma and hospitalisation, Meyer and Kieran (2002) demonstrated that following neurosurgery for brain tumours, there were significant problems found in both the short-term and long-term which were related to behaviour, as

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well as adaptation to schooling. In terms of schooling, Wray et al. (2001) discovered that, following heart or heart and lung transplantation, there were a significant number of children who had problems at school in relation to both behaviour and learning. Completing this very brief look at the effects of surgery on behaviour in children, Schwering et al. (1997) looked at children following liver transplantation. Using the Achenbach Child Behavior Checklist, they found that although the behaviour of children aged between 4 and 16 years was within the normal range, those children who had transplants at an earlier age did show increased scores relating to aggressiveness and social problems, while scoring lower on activity and competence scales. In addition, they showed that as the years since the transplantation increased, so did the scores on anxiety and depression, social activity and competency.

Neonates and Hospitalisation Without a doubt, the hospitalisation of a newborn infant is highly stressful for the parents (Miles et al. 1991, Seideman et al. 1997). Klaus and Kennell (1970) were amongst the first researchers to demonstrate the harm that could be done to the mother–infant relationship by early separation, and this helped to change hospital practice towards the family-centred care that is prevalent today in neonatal units (Rautava et al. 2003). Unfortunately, in spite of this, there is still no getting away from the fact that hospitalisation remains a disruptive and disturbing threat to the closeness of a mother and infant that is necessary for good mother–baby bonding to be achieved (Rautava et al. 2003). Allied to the problems of hospitalisation of neonates, there is also a factor that for a neonate to be admitted into hospital, there is usually a medically serious condition, often life-threatening. For example, a study by Feldman et al. (1999) compared a group of infants with low or very low birth weight who were in a life-threatening situation and required prolonged hospitalisation to a group of preterm infants who did not require intensive care. They found that the thoughts and behaviours of the mothers following birth were very different between the two groups. Studies of preterm infants have also demonstrated similar behavioural and psychosocial problems to those already mentioned in this chapter (Singer et al. 1999). Difficulties in disturbances to parent–infant relationships have also been demonstrated in several research studies, including Jeffcoate et al. (1979) and Minde et al. (1983), as well as problems of functioning within the whole family (McCain 1990, Stjernqvist 1992). The study by Rautava et al. (2003) demonstrated that a critical illness in a newborn child affected the family and child for several years, even though the medical outcome may well have been very positive. They also stated that the finding of later behavioural problems in this group of newborn infants following a life-threatening illness and early hospitalisation was consistent with the concept of ‘vulnerable child syndrome’ (Green & Solnit 1964). Rautava et al. (2003) determined that whilst the hospitalisation

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itself did not appear to be harmful because of present-day family-centred care provision (e.g. allowing free access for the families to the neonatal units as well as positively promoting parent and infant interaction), they did consider that the potential loss of a newborn was a factor that caused long-lasting consequences to the parents and families. They found that families with newborn children who were hospitalised but did not have a critical medical condition coped slightly better than did the control group of families with newborn infants who were healthy. Interestingly, in the light of what has been said above regarding the role of fathers with hospitalised children, the study by Rautava et al. (2003) made the point that the fathers in the study had appeared to be affected more than the mothers by the critical illness of the newborn. They acknowledged that this finding, to their knowledge, has not been reported in other studies – as can be testified from the studies discussed in this chapter. In this study, it was found that the fathers of critically ill neonates reported maladaptation to their children, and they also reported that they needed more help and support during the first year following the birth of their infant when compared with the other two groups of low-risk infants and healthy infants. The findings from the study by Rautava et al. (2003) were consistent with those from the study by Minde et al. (1983), and that they both demonstrated that the degree of illness in preterm infants did correlate with the skills of the mothers to interact with their babies for up to two months after the expected date of birth. Another study (Feldman et al. 1999) is also consistent with this study by showing that a potential loss of the newborn baby did affect the mother’s attachment capabilities during the first few weeks following birth. One of the many interesting points to emerge from the Rautava et al. (2003) research study was that the families with children who were hospitalised but whose infants were at low risk medically, coped even better than the control group of families whose babies were not hospitalised. This finding was echoed in the studies of Singer et al. (1999) and Trause and Kramer (1983). This they attribute to the fact that with hospitalisation comes the provision of extra support for the families and help for them to adapt to the child following discharge from hospital. Rautava et al. (2003) also make the surprising comment that the mothers of the healthy infants in their study had a significant amount of adaptation problems themselves. In fact they appear to experience the same amount of problems as did the mothers of the infants who were hospitalised with critical illnesses. What of the future for these extremely low birth weight infants who were extremely sick in hospital, often for several months? There have been several large research studies (Hille et al. 2001, Stjernqvist & Svenningsen 1999) that have shown that behavioural problems are more commonly found at school age in infants who were born with an extremely low birth weight than are found with their peers. However, in the Rautava et al. (2003) study, it was found that the behavioural and family problems had disappeared by the time the child was 12 years old.

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Conclusion In 1994, Wells et al., looking at the effects of children growing up in hospital, point out that they are growing up and developing in an environment that is ‘. . . over-stimulating, inconsistent, full of pain or discomfort, and “artificial” at best’ (p. 66), and that a child growing up in hospital will miss many of the early life experiences that other children experience, which in turn may lead to the delayed development of many psychosocial skills. An interesting study by Danilowicz and Gabriel (1971) states that emotional reactions to hospitalisation are completely normal, and that it is only bizarre and extreme reactions that should be thought of as being abnormal. In their study of 67 children in hospital, they put the children into various categories:

r Overtly anxious, fearful, withdrawn, or restless (42% of the 67 children r r r r r

displayed these reactions). Overtly anxious and combative (15%). Totally compliant (10%). Actively co-operative (24%). Abnormal (6%). Unable to classify (3%).

They also make the point that the only children about whom they considered the appropriateness of their response was the group of children that they classified as ‘compliant’. In other words, they turned the accepted wisdom on its head by stating that it was not the child who causes problems who has inappropriate problems in hospital, but rather it is the child who causes no problems – whom every nurse and doctor wants to look after – who may be said to have an abnormal response. When one thinks about this, there may be some merit in it. After all, being within a hospital environment is an abnormal situation for most children, and therefore it should only be expected that their behaviour will change and possibly become ‘difficult’. There may be fear and distress linked to leaving their normal safe home environment. They may want to show that they are ‘grown-up’ and can cope with the change of environment, and also their medical treatment (which can be painful). How many young children going into hospital are told to ‘be brave’ or ‘be grown up’? For some of them the only way that they can show that they are grown up is by being angry and loud and generally badly behaved, because they have no concept about what being grown up means. So, there can be an inner conflict taking place within the child. On the one hand they are scared and in pain, but on the other hand they are being told to be brave and to be grown up. No wonder their behaviour deteriorates once they are in hospital. Then again, there may be problems with fitting in with a lot of strangers – and not just all these strange adults, doctors, nurses, physiotherapists and other adults caring for them, but also suddenly having to live with a lot of strange children. There will be problems of roles within their peer groups, and conflicts as they try to sort these out, leading to poor behaviour. As Oremland

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and Oremland (1973) state, it is important to give due consideration to the developmental levels of children in relation to their capacity to deal with the hospital experience as this undergoes changes with the growth of ego, the establishment of defence mechanisms, and the mastery of these experiences and any further complications. So to add to all these conflicting concerns about being in a strange environment with strange people, there is the added stress of the illness and medical treatment to cope with as well! Perhaps we should cease to be concerned about the causes of the behaviour of children in hospital (because these are well known) and concentrate on the ways in which we can ameliorate the problems and hence the behaviour of the children in hospital, and try to prevent those becoming chronic problems after discharge from hospital. That is the real concern that we need to do something about – the long-term effects of hospitalisation, and the detrimental effects that these have on the quality of life of the child – and of the family. Certainly, we can take heart from the results of the study by Rautava et al. (2003) who demonstrate that whilst a critical illness of a neonate affects the family and child for years following discharge from the hospital, these problems appear to disappear by the time that the child is 12 years old, and also that whilst the separation of the newborn from the close contact with its mother may occur in neonatal intensive care units, there are no apparent consequences for the family and child in the long-term. As they say, this is comforting and encouraging for the thousands of parents who go through this experience. Perhaps we should end this chapter with a quote from one of the early pioneers in this field who did so much to help us to vividly see the problem and to cause us to look for solutions. ‘Separation from the mother, particularly when coupled with the stresses of illness, medical investigations, and surgical interventions, can be traumatic and detrimental to mental health’ (Robertson, 1958 p. 127).

5

The Effects of Isolation and Bone Marrow Transplantation on the Child and Family

Isolation According to the Concise Oxford Dictionary (Allen 1991 p. 629), to isolate means to ‘place apart or alone, cut off from society’, whilst isolation is the ‘act or an instance of isolating; the state of being isolated or separated’. Within the hospital environment, there are several types of isolation. The simplest type of isolation environment is the individual cubicle which is used for patients with actual or potential infections, and whose purpose is to prevent the patient from infecting others. These cubicles may have the same air pressure as the surrounding environment, or they may have a negative air pressure – that is the air pressure in the cubicle is lower than that outside of it. The purpose of this is to prevent the outflow of infected air from the isolation cubicle to the external environment when the door is opened. Instead, ‘clean’ air enters the cubicle from the outside. At the other extreme, are to be found the reverse barrier environments in which the purpose is to prevent a patient with a compromised immune system from being infected by other people. There are many varieties of these, but the simplest were the ‘bubbles’ experienced in the early days by the children and parents with SCID (severe combined immunodeficiency). These consisted of a plastic bubble that was situated within an isolation cubicle, so having a double protective ‘skin’ or barrier protecting the immune-deficient patient from infections coming from the outside. The area between the ‘bubble’ and the cubicle outer wall was the area where the nurses, doctors, other necessary health personnel and any visitors, put on the protective gowns, gloves, etc., not to protect themselves from the patient, but to protect the patient from any infectious organisms that they may be carrying. The air pressure in these isolation environments is positive – it is at a higher pressure than the external air pressure. This means 60

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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that when a door is opened, the air in the ‘bubble’ will be forced out of the cubicle, so preventing any infected air coming in from the outside. In addition, because the ‘bubble’ is air-tight, filtered air (filtered to prevent even viruses from entering the isolation environment) needs to be pumped into the ‘bubble’ at a high pressure. As can be deduced, these ‘bubbles’ are very isolating, and frustrating for the patients and the families, not forgetting the nursing and medical staff. The later types of reverse barrier environment are not as isolating, whilst being just as effective in preventing infections. These are known as laminar air beds, and work on the basis of high air pressure that is filtered being forced around the bed that is in an isolation unit. Thus there is an ‘invisible’ wall of clean, filtered air surrounding the actual area that is occupied by the patient, but other people can go into the room – as long as they do not cross into the area reserved for the patient. This reduces some of the isolating stressors for the patient, whilst still protecting them from the risk of catching infections from their visitors. Obviously when a child is in a reverse barrier isolation environment, there are many restrictions on the child as to what they can do, where they can go (nowhere outside of their protective environment), and who they can meet, touch and talk to. These isolation environments, along with the child’s illness and the treatments that he or she has to experience, underpin the psychosocial and behavioural aspects of children in isolation that this chapter will discuss.

Introduction to the Effects of Isolation and Bone Marrow Transplants ‘The effect of mother–child separation, followed by isolation and subsequent deprivation, on the psychological development of children has been a longstanding concern’ (Hollenbeck et al. 1980 p. 3). Indeed, it has been concluded that young children who are admitted to institutional care (including hospitals) respond with acute distress which in turn is followed by a slow process of adaptation to the new environment (Hollenbeck et al. 1980, Robertson & Robertson 1971). One of the major problems with writing this chapter on the effects of isolation and bone marrow transplantation on the child and family is that, as Koller et al. (2006a p. 135) point out, referring to pioneers of the problems of hospitalisation in children (see Chapter 4), ‘despite Bowlby’s and Robertson’s work, there exists a paucity of recent research regarding the psychosocial effects of isolation on children’. Koller et al. (2006a) go on to explain that this relatively recent lack of research on the problems of children in isolation is of particular concern because some studies have reported that children with chronic ill health experience more isolation than do their peers in good health. Krulik and Florian (1995) reported that they had found that chronically ill children shared more stories about loneliness, fear and unpleasant emotions than did children who were not chronically ill. Koller et al. (2006a), point out

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that the isolation of children who are already ill may exacerbate or even intensify these pre-existing feelings of loneliness and alienation. Whilst hospitalisation at an early age may well cause psychosocial problems to all children (and their families) because of the disruption of normal family life, as discussed in the preceding chapter, infants and young children who require treatment in hospital that necessitates them being isolated are at risk of experiencing even greater stressors that will affect their psychosocial development. This in turn increases the stressors that will affect the parents and the family as a whole. Add to this the extra stressors that a bone marrow transplant causes, as well as the stressors of critical illness, and these stressors and their effects are multiplied many times. This chapter, whilst bearing in mind the stressors caused by early hospitalisation, explores these further stressors and their effects on the psychosocial development/functioning of the children, and the psychosocial functioning of the families. This chapter is divided into two parts, namely:

r The effects of isolation on the development of children. r The effects of bone marrow transplantation on children.

The Effects of Isolation on the Development of Children ¨ According to Kohle (1979), the whole experience of being treated in an isolation environment is associated with extreme dependency on the hospital staff by the patient, which also means limitations, and a reduction, in the contact of the patient with the family. Mirand and Mirand (1983) state that extreme changes in human behaviour have often occurred in people during prolonged periods of isolation, such as can occur with explorers, prisoners in solitary confinement, shipwrecked sailors and hospitalised patients. There are many categories of isolation behavioural patterns which are related to that portion of a person’s normal environment from which he or she is isolated. Mirand and Mirand (1983) identify three types of isolation as: (a) Perceptual isolation – separation from one’s normal sensory and perceptual environment. (b) Social isolation – separation from other people and from society. (c) Cultural isolation – separation from one’s own culture, such as arises from being in an alien cultural environment.

Variables Associated with Isolation In addition to the isolated environment itself, there are other variables which may intensify individual responses to the state of isolation. In their paper of 1983, Mirand and Mirand summarised the variables that they considered to be the most important, leading to the development of the Mirand and Mirand (1983) model of isolation in general. These important variables, according to Mirand and Mirand (1983) are:

Effects of Isolation and BMT on Child and Family

(a) (b) (c) (d) (e) (f) (g) (h) (i) (j) (k) (l) (m) (n) (o) (p) (q) (r) (s)

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type (category of isolation exposure) size and shape of the confinement chamber degree of restriction of movement duration of confinement social environment vocal and visual contact with others physical condition of the isolated person amount of sensory deprivation and/or sensory overload intensities of sensory stimuli response requirements and their relationship to mission success variability and redundancy of sensory stimuli and response requirements work–rest ratios, fatigue and boredom interactions with physical stressors, such as lack of food, water or air interactions with psychological stressors, such as fear, anxiety and aggravation interactions with environmental stressors, such as high temperature, poor ventilation, noise etc. motivation and interest personality, temperament and emotional stability prior experience and training age of individual

Children in Isolation The above variables are all present during the treatment of a child with SCID in an isolation cubicle, and will affect the behavioural and psychosocial functioning not only of the child, but also of the parents (and even, to some extent, of the nurses and others caring for the children). With relevance to the infant or child who is nursed within some degree of hospital isolation, the variables above can be matched and linked to the children, and to their parents/family members, whether the isolation be necessary for an immunodeficient child, a child with cancer having a bone marrow transplant, as a result of a child of newborn being in intensive care, or a child with an infection.

Types of Isolation Environment The type of isolation, as well as the size and shape of the isolation environment, can have a strong bearing on the psychosocial consequences. Too small an isolation environment can lead to frustration and feelings of claustrophobia in both the child and parents, whereas a large isolation chamber may allow the child and the parents opportunities to move around the chamber, and possibly to personalise it, thereby giving them some degree of ‘ownership’ of their environment. Luckily, the isolation environments of the children with SCID that I encountered in the UK and

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in Germany in the study, and with which I was involved as a nurse, were the size of a small room. However, they were not always like this, and certainly the first isolation chambers were actually much smaller, and would only be suitable for a baby (Barnes et al. 1969). Freedman et al. (1976 p. 594) described the case of one infant who was isolated in these mini-isolation chambers. ‘Gloves are built into the walls of his isolator to allow him to be picked up, handled, and played with. However, all these activities are carried out under the constraints imposed by the plastic barrier between him and his caretakers. This circumstance interferes with his ability to receive some forms of sensory stimulation which are inevitable elements in the experience of youngsters who are reared under less atypical circumstances. He has never, for example, felt another person’s skin, smelled another person’s breath or body. The process of moulding his body to that of a caretaker is hampered and the opportunity to use his arms in order to embrace another person or engage in mutual ventral/ventral clinging is unavailable to him’. As Freedman et al. (1976) comment, these stimuli were considered to be critical experiences for good early psychological development by Bowlby (1969). Indeed, as late as the year 2000, in some European countries these small isolators were still in use, as I witnessed during a visit to one European bone marrow transplant unit. Although the staff felt that there were no psychosocial problems with the children, afterwards, parents I spoke to disagreed with this, and explained to me that many of the children could take two or three days to gain the courage to leave their isolators once they had had a successful treatment. Even with the full-size isolation cubicles, once the children were allowed to go outside, they were often afraid of simple everyday experiences such as birds flying near them, or even the wind lightly touching them. These personal observations are backed up by Kellerman et al. (1976) who reported that discharging the children from their isolation unit had presented a challenge and that they had to set aside at least one day following successful treatment in which the door to the isolation unit was open and the child was free to enter and leave as they wished. This allowed the child to establish his or her own pace in re-entering the outside environment, and during this period they took part in role reversal games with the children in which the children would often pretend to be the nurses. Children who survived treatment for SCID, however, being still young infants did not have this opportunity, and great sensitivity was needed at this crucial time to allow them to come to terms with the outside environment. This had to be set against the very understandable desire of the parents to remove their children from the isolation cubicle as soon as possible because this was the proof they needed that their child had survived.

The Effect of Time Certainly, the duration of time spent in isolation can affect the psychosocial development of children as is evidenced by the study of children with SCID who spent many months, if not a year or more, in isolation. It can be difficult enough for an adult if he or she was to be isolated for just a few

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weeks, but for a child those few weeks or months can be a major amount of their lived experience at the time, and the younger the child, the more important is that length of time spent in isolation and lacking normal social and cultural interactions.

Perceptual Isolation All three types of isolation detailed by Mirand and Mirand (1983) as discussed earlier in this chapter, namely perceptual, social and cultural isolation, are present for children in hospital isolation environments. Isolation in hospital, whether it be in an isolation cubicle, a reverse barrier nursing environment, an intensive care cot or bed, or just a busy ward, can lead to a separation from that child’s normal sensory and perceptual environment. This is particularly difficult for the child or infant who has experienced life at home before going into hospital isolation, where he or she has already come into contact with normal sensory experiences. However, even the newborn infant who is isolated from birth will experience perceptual isolation because the normal sensory experiences are often missing within this environment. Concerning children with cancer, Kellerman et al. (1976), by means of interviews, attempted to explore the subjective perceptual experiences undergone by the children once they had left the isolation environment, which in their words was an environment that was ‘relatively perceptually bland, due to a limited input of sound, movement, touch, and taste’ (p. 24). They reported that several of the children had the perception that things seemed to be moving faster and that sounds were louder. They also noted the ambivalence of the children with regard to the isolation unit in that while they did not like being confined for several months and also be subject to many unpleasant procedures, they had also come to perceive the isolation room as a place where they received a lot of attention as well as much stimulation, and in particular they perceived the isolation room as a place where they developed several close relationships. Indeed, whilst some of the children were very happy to leave isolation, Kellerman et al. (1976) reported that others appeared to be sad at the prospect and that some of the children even said that they wanted to stay in their isolation room and did not want to go home. With some children, this attachment to the isolation environment reappeared when they were hospitalised again at a later date.

Social Isolation Social isolation is also present because, whilst the infant or child may not be separated from his or her parents, they may well be separated from all other significant individuals within their experience, such as siblings, grandparents or just friends within the home or within their social milieu. For the newborn infant, the social isolation experienced may well take the form of isolation from a close attachment to the parents, particularly the mother.

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Life in an isolation cubicle can become very boring, as well a disorientating, and this can impact upon the young child or infant – as indeed it can impact upon the parents. The lack of relevant sensory stimulation, particularly if the child is not feeling too unwell, can affect their behaviour. Similarly, the opposite of sensory deprivation which may occur in isolation is that of sensory overload. Anyone who has been in a neonatal or children’s intensive care ward will have experienced sensory overload, with all the noises of the alarms and the equipment, such as the artificial ventilators, as well as the lights that are generally on all the time and the heat which can become very debilitating. This sensory overload can certainly affect the children and make them withdrawn – as they try to withdraw from all the noise and heat and light that surround them and dominate their environment. More recently, the concerns following the various outbreaks of severe acute respiratory syndrome (SARS) has led to re-evaluations of the effects of children being isolated. Koller et al. (2006b) found that with regards to infection control precautions it was issues concerned with the social isolation that were predominant. They found that the experience of SARS had a direct impact on the very basics of family-centred care within a child nursing environment. This is because the children and their parents (as well as hospital personnel) experienced drastic immediate and immense changes in the environment of care. It was found that one of the many challenges to implementing family-centred care in this situation (as indeed with all isolation situations) was a struggle to establish and maintain communication.

Cultural Isolation In terms of cultural isolation, the hospital environment is to the child (and often also to the parents) an alien cultural environment, with its own rules, and which is populated by people with specific roles, which may not include their parents, or at the very least significant other family members or friends. The infant or child is also propelled into a cultural alien role space – that of a patient (very often a passive role), whilst the parents are forced to take on the role of visitors to their child. Within this state/environment of isolation, sensory alteration, perceptual alteration, sensory deprivation, sleep deprivation and sensory overload appear to be especially important because these occur in conditions in which a person is exposed to marked changes in the quantity and variety of sensory inputs – in effect the conditions that arise when a child is isolated in hospital.

Psychological Disturbance The work of Mirand and Mirand (1983) is complemented by that of Kellerman et al. (1977) who reported that patient care in strict reverse barrier isolation may increase the risk of psychological disturbance precisely because there is a reduction in both social contacts and sensory stimulation. Twelve months earlier, Kellerman et al. (1976) had reported that they had not observed any serious debilitating psychological disturbances in

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children who were being treated within a ‘protected environment’. However, they did point out that there had been temporary periods of psychological disturbance, including:

r r r r r

regression apathy anorexia depression intense anxiety

They also stated that the children and the parents had exhibited periods of anger, with the children being angry with the hospital staff and their parents, and the parents being angry with the hospital staff. In addition, they reported that, at times, the children who were in isolation ignored and rejected their parents, and this was particularly apparent following prolonged periods of separation from their parents. They also noted that some of the parents had found it difficult to enter the isolation room wearing all the sterile protective clothing, but did not explain why this was so, although personal experience of nursing children with SCID was that the parents found it time-consuming when all they wanted to do was to be with their child, and at the same time felt that the protective clothing also acted as a barrier between them and their child. Some of the psychological reactions described by many of the researchers and workers in this area include:

r r r r r r r r r r r

anxiety and depression regression withdrawal boredom developmental delay dependency inhibition of motor activity emotional lability manipulation changes in patterns of play sleep disturbance (Kellerman et al. 1976, Kohle et al. 1971, Koller et al. 2006a, 2006b, Loranger 1992, Peterson 1987, Pot-Mees 1989, Pot-Mees & Zeitlin 1987, Simons et al. 1973, Susman et al. 1980a, 1980b, Zerbe et al. 1994).

Studying pre-school children who required isolation because of respiratory problems, Casey (1989) found that, in addition to desperately seeking human contact, these children demonstrated behaviours such as irritability, sadness and withdrawal. She reported that these children were very demanding of the time of both their parents and the nurses. The parents of these children themselves reported that they felt frustrated because of the isolation barriers. They also attributed their children’s behavioural changes to the isolation and environment. Campbell (1999) in a study of children being nursed in protective isolation due to their receiving chemotherapy discovered that these children

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found it difficult initially to come to terms with their feelings of being ‘shut in’. They also had to learn to cope with the experience, which included knowing what to expect and developing relationships with the health care personnel. Campbell also reported that the children’s major coping strategy was the maintenance of contact with the outside world. Holland et al. (1977), in answer to the question that they posed in their paper as to whether psychological stress might be a limiting factor to the potential use of isolation units, came to the conclusion that the psychological status of people in isolation environments was most strongly influenced by their actual physical status. They noted also that patients described that the most significant psychological deprivation that they received during their time in isolation was the loss of the ability to touch and to be touched by others, which in turn generated feelings of loneliness; sometimes very acute and intense loneliness. Further evidence for this comes from Hare et al. (1989) who make the point that such essential physical and psychosocial needs that the child requires, in particular the need for human touch and interaction, are usually inhibited within the isolation environment, and consequently, because the child is living in an ‘altered state of existence’, loneliness may occur to such a degree that it could overwhelm the child. This is certainly a possibility, again according to Hare et al. (1989), because isolation usually occurs at just the time that the child feels most sick and is at his or her lowest point physically and psychosocially. And it is whilst the child is in this state of vulnerability that any fears may assume gigantic proportions – fears that can affect their psychosocial functioning and development. Powazek et al. (1978), in their paper on the emotional reactions of children with cancer to isolation, reported that both the children and their parents had overall high levels of hospital-related anxiety and depression which varied with the patient’s chronological age. They reported that patients aged between 7 and 12 years reported just normal levels of acute anxiety, whilst older children were found to be much more anxious (acute anxiety was defined in their study as the reactive anxiety exhibited by the children to the immediate hospitalisation experience). However, they found that all of their patients, no matter what their age, had normal levels of chronic anxiety. In addition, the parents of the pre-school children were found to have more negative opinions towards isolation than did the parents of the older children, although there was universal concern about experiencing further separation from their hospitalised children by them being in isolation. The age of the children in isolation appears to be crucial in terms of their psychosocial development and behaviour, as reported by Powazek et al. (1978). Hollenbeck et al. (1980 p. 11) also stress this in their paper, and conclude that ‘the effects of separation, isolation, and illness appeared to be more extreme in younger children than in older children, possibly because the young children have not developed strategies to cope with these situations’. In 1975, Clancy and McBride proposed a new diagnostic category to describe a pattern of abnormal behaviour in children – the isolation

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syndrome. They hypothesised that this syndrome can occur in several situations, namely:

r an institutional setting r the separated child r the child in a family with severely abnormal relationships They then put forward their findings that the child’s ‘isolated’ state manifests itself in:

r defective social interaction and communication between mother and child

r disorders of perceptual function and motor skills r stereotyped behaviour r general developmental retardation, especially in language Certainly many of the children who had had successful treatment for SCID exhibited the first two manifestations above to varying degrees for many years (up to at least 13 years according to the Vickers 1999b SCID study) following the end of their isolation. However, there was no evidence of the latter two manifestations of the Clancy and McBride (1975) isolation syndrome in the children who participated in the Vickers 1999b SCID study.

Effects on the Family However, whilst accepting that children experiencing isolation can have resulting psychosocial problems, there is also a need to look at the effects of isolation on the primary family caregivers, and their subsequent effect on the children in isolation. This is of particular importance when considering the treatment of children with SCID as one, or both, parents are usually with their child the whole of the time they are awake, and also often present when their child is sleeping. Parents in this situation can experience the same psychosocial problems as their children, which in turn can affect their ability to care for, and nurture, their children, as well as causing them to exhibit highly volatile and often negative emotions which, in turn, can be picked up by even very young children and babies. Little work has been undertaken on this aspect, although Hare et al. (1989) did address the effects on the family of a child having a bone marrow transplant, including the period in isolation, and in 1994, Heiney et al. identified post-traumatic stress disorder in parents of children who had had a bone marrow transplant. The importance of the parents/family is stressed by Simons et al. (1973), Drotar et al. (1976), Kutsanellou-Meyer and Christ (1978) and Tamaroff et al. (1986). All these studied children who had SCID as well as examined the role of the parents, particularly in relation to the care of the children. Kutsanellou-Meyer and Christ (1978) comment upon the near impossibility of mothers caring for their infants with any degree of normality within the isolation environment, and the stresses placed upon them because of the nature of the illness and its genetic implications. They also highlight

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the difficulties caused to the mothers by a lack of emotional and physical support from their husbands. Before leaving this theme concerning the effects of isolation on the development of children, it is important to note that several researchers have stressed that isolation per se is not incompatible with normal development. Rather it is a combination of factors, including isolation, medical treatments, mother/parent and child interactions and severity of the illness, which may determine the developmental outcome (Drotar et al. 1976, Freedman et al. 1976, Gordon 1975, Kamphuis 1979, Kutsanellou-Meyer & Christ 1978, Loranger 1992).

The Effects of Bone Marrow Transplantation on the Development of Children There is some evidence as to the effects on children who have undergone bone marrow transplants for disorders other than SCID. Perhaps the most comprehensive report is that by the psychologist Carien Pot-Mees in 1989. In her study, Pot-Mees looked at children from four centres in England who were undergoing bone marrow transplants. These children underwent bone marrow transplants both for acquired disorders – mainly leukaemia, lymphoma and aplastic anaemia – and for some of the inherited/genetic disorders, such as Hurler’s syndrome and Gaucher’s disease. The one group excluded from her study was children with SCID. In terms of psychosocial development and behaviour, Pot-Mees (1989) noted that the children in her study who received a bone marrow transplant were really quite noticeably different as regards to their behaviour, when compared to their healthy peers. For a start, their, often long, history of ill health had already had an effect on the quality of their lives by the time they entered hospital to commence their bone marrow transplant. She discussed that these differences occurred as a result of them missing so much more schooling than their peers, as well as the previous long hospitalisations. This had led to them experiencing more life disturbances than their peers in the months prior to the bone marrow transplant. Pot-Mees also found that, because of their illnesses, they, along with the children with congenital heart disease in her study, could be considered as being chronically ill, and all these children exhibited similar problems in adjustment as do other chronically ill children, including raised anxieties, solitary behaviour, eating difficulties and extreme dependency on their parents. Whilst not having the same problems pre-transplant because of their extreme young age, it is interesting to note that the children in the SCID study, often demonstrated such behaviours in the years following their bone marrow transplant. Another point that Pot-Mees (1989 p. 63) made about the children in her study was that, concerning the cognitive status of the children, the younger children demonstrated a ‘significant delay in developmental skills in comparison with their normal, healthy peers’, whilst the older children in her study were found to have significant delays in their school attainments, although functioning within the normal range of intelligence.

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The mean age of all the children in the Pot-Mees (1989) study was 6.6 years, covering an age span of 3 months to 16 years 8 months, of whom 41.3% were below 5 years of age, 44% between 5 years and 11 years and 14.7% over 11 years of age. The children stayed in hospital for an average period of 66.5 days (or 9.5 weeks). An average of 35 days was spent in isolation. Twenty-three per cent of these children were reported by the nurses to have exhibited severe behavioural problems during their hospital stay. Almost all of the children also experienced severe pain and discomfort. However, none of the children demonstrated a behaviour pattern suggestive of a psychiatric illness, but rather demonstrated a pattern which included depression and withdrawal, prolonged refusal to take medication or food, aggression towards staff and parents, hyperventilation due to anxiety and fear and regression (for example, returning to bottle feeding). This is in keeping with other reports, particularly with regards to emotional lability, anxiety and depression (Brown & Kelly 1976, Gardner et al. 1977, Patenaude et al. 1979). These findings offer useful comparisons with the SCID children in the Vickers (1999b) study. Other researchers have noted an overdependency on parents and a regression of behaviour on the part of the child having a bone marrow transplant, which is exacerbated by the added stresses of isolation and a fear of death (Brown & Kelly 1976, Popkin et al. 1977). The loss of any desire to eat and drink, and even full-blown anorexia, is a feature of the behavioural problems of children undergoing bone marrow transplants. Often this is caused by the side effects of the drugs given as part of the treatment protocol, particularly the cytotoxic drugs, and by the subsequent gastrointestinal problems. This problem is also sometimes identified as an attempt by the child to have some control over his or her own life (Gardner et al. 1977, Patenaude et al. 1979, Pot-Mees & Zeitlin 1987). Children’s reactions are seen to differ according to age and stage of development. Children under five years of age tend to become withdrawn and to lose skills already gained, particularly during the actual period of isolation. Older children, on the other hand, tend to demonstrate anger, aggression and depression (Pot-Mees & Zeitlin 1987). There are other variables which can affect the well-being of the children and influence their reactions and coping abilities. Amongst these are the medical condition and the length of time spent in hospital and, by projection, the length of time spent in isolation (Patenaude et al. 1979). The 1989 study by Pot-Mees demonstrated that there were significant increases in behavioural problems six months post-transplant (approximately three months post-discharge from hospital). These problems were, in the main, made up of depressive behaviour patterns. There was also an increase in the frequency and severity of temper tantrums and eating difficulties, and shortened concentration spans. It was concluded that the actual procedure of bone marrow transplantation had potentially harmful psychological effects. This corresponds with observations from other researchers (Freund & Siegel 1986, Gardner et al. 1977, Patenaude et al. 1979).

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Nevertheless, 12 months post-transplant, most of the children in the Pot–Mees study showed an improvement in their psychosocial behaviour and 81% had started to integrate into normal life. There were, however, still some problems in their general behaviour, such as anxiety and problems in integrating into normal life again. General cognitive function, however, was not affected, but there were effects on their school attainments. At six months post-transplant, these children were experiencing difficulties when exposed to academic pressure, probably as a result of lack of schooling and their emotional problems. After a further six months, there was some improvement, but the children were still not reaching their pre-transplant levels. The problems of school and coping with peers at school for children following a bone marrow transplant with the concomitant isolation was summed up by the father of one of the UK children in the SCID study, He . . . he’s a withdrawn child, and very quiet . . . lacks confidence and needs praise. He has no confidence whatever in doing things for himself, and that’s why, even at school with his education, we have to push him harder. But he’s shy, he’s shy. He’s very shy when it comes to meeting other kids. He’ll even walk down the street with us, and one of his friends from his own class will pass and say ‘Hello’, and we’ll always say, ‘say hello’, but he won’t. There was a stage when he wouldn’t go out of the door. The under-five year olds in the Pot-Mess 1989 study also had similar problems to the older children at the six months post-transplant time. Eating problems, social difficulties, lethargy, developmental regression and loss of self-help skills were all in evidence. These had all improved, however, after a further six months. Children under one year of age at the time of transplant, however, had normal developmental progression, and did not appear to be affected by the transplant procedure. This is a particularly important point as the SCID children in the Vickers (1999b) study all had their transplants when they were less than one year old. It must be stressed, however, that children undergoing mismatched haploidentical bone marrow transplants for SCID differ to a great extent from those children described by Pot-Mees in terms of age of diagnosis of illness and isolation/transplant, the number of infections they suffer, and the length of time they spend in isolation. Hollenbeck et al. (1980) studied four pre-school children who were in protective (reverse) isolation whilst receiving cytotoxic chemotherapy for cancer. They reported that the children’s behaviour followed a sequence from agitation to behavioural depression; a finding supported by the work of Popkin and Moldow (1977) who investigated the stressors and responses during bone marrow transplantation. Hollenbeck’s work is also supported by the work of Clancy and McBride (1975). Other studies concerning partial sensory deprivation and social isolation, other than that which occurs within the strict reverse barrier nursing isolation environment, appear to show that when there are alterations or restrictions on the sensory informational and social inputs to the child,

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areas of cognition, affect and perception are negatively affected (Dennis & Najarian 1957, Goldfarb 1945, Spitz 1945).

The Effects of Isolation and Bone Marrow Transplantation on the Child with SCID To turn now to the studies which have focused particularly on children in isolation from a very early age as a result of having SCID. Simons et al. (1973), who studied one set of twins who lived in a reverse isolation environment from shortly after their birth, stated that at assessments at 28 and 33 months, the children displayed signs of emotional disturbance, impairment of intellectual ability and evidence of a degree of learning disorder whilst they were still in isolation. The research team concluded that this was mainly due to their actually being in reverse isolation, rather than any other cause. However, another important factor suggested was that these children did not always have their parents present to help establish long-term stability in the feeding programme. Conversely, Freedman et al. (1976) found none of the problems described by Simons et al. (1973) when they assessed at 52 months, a single child who had been isolated from birth. It was suggested by them that reverse isolation is ‘not incompatible’ with normal development. Dalton (1981), who also studied a child in reverse isolation from birth, demonstrated that even when deficits in motor, cognitive and language function had been noted in an assessment at 15 months, the situation was somewhat reversed by means of a programme of intervention. Fonger et al. (1987) stated that, because of the use of masks, gowns, other clothing paraphernalia and the isolation routine commonly used in nursing children with SCID, the children were unable to differentiate medical/nursing staff from their other visitors, and this led to alienation from all visitors. The lack of direct touch and contact with people may lead to a degree of sensory deprivation, which in turn could lead to emotional distress in the children, with consequent infantilism and depressed behaviours. They also stated that both the age of the child and the severity of the illness influence the emotional states of both the children and their families more than does the degree of isolation. These findings mirror those of Powazek et al. (1978). Drotar et al. (1976) presented a single case study of a baby with ADA SCID who was isolated during his first year of life. This baby was then released from isolation following successful treatment with enzyme replacement therapy. This child did not undergo a bone marrow transplant. Developmental testing was carried out at 14 months of age and again at 16 months which revealed near age levels for social and intellectual functioning. The authors felt that this was due not only to the parent’s involvement in the child’s care during hospitalisation, but also to optimal nursing care using care plans which included surrogate mothering when the parents were away, as well as the child’s adaptive capacities which permitted rapid adjustment to normal life following the prolonged isolation.

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When the infant eventually returned home, It was reported by the authors that there was some screaming and clinging to his mother, but that within a few weeks this phase had passed, so that at 14 months of age, the mother described him as an active, curious child who preferred novel stimuli and strangers, but displayed no unusual habits or fears. At the 16 months assessment, it was found that, despite delays in social imitation and language skills, his intellectual development was within normal levels. In conclusion, the feeling was that ‘. . . the absence of severe deficits attests to the human infant’s remarkable capacity to compensate for severe environmental restrictions’ (Drotar et al. 1976 p. 677). Another report was by Tamaroff et al. (1986), who studied four infants with SCID. These children eventually had successful bone marrow transplants, but their time in isolation spanned a range from 10 to 52 months. The conclusions from this study were that deficient self-generated activities, including motor and motor-based cognitive skills, such as expressive language were found in two of the children studied. These deficits were also observed to be at least moderately reversible either upon discharge or in relation to an inpatient intervention programme. This finding is similar to that of Dalton (1981) discussed previously. The two children who displayed no evidence of major psychopathology or developmental delays were the children who had no major feeding problems, as well as having adequate mothering, and with a supportive father in each case. Tamaroff et al. (1986) concluded that the children who had the most prolonged oral deprivation and the least adequate parenting had, psychosocially, the poorest outcome. They believed that oral deprivation is related not only to medical factors, but to the quality of parental involvement in the feeding process. Although there are some dissenting voices (Freedman et al. 1976, for example), there is accordingly a substantial body of literature and research which shows that severe ill health, isolation and hospitalisation at a young age can cause emotional and behavioural problems. Additional support for these findings has been given by Douglas (1975), Rutter (1980) and Corter (1976). It was against this background that the Vickers (1999b) study into the physical and psychosocial affects of mismatched bone marrow transplants on children with SCID was undertaken.

Conclusion The psychosocial ramifications for a child in isolation (and the family) can be seen as an exacerbation of the psychosocial effects of hospitalisation at a young age. These appear to be centred mainly on the separation from the main caregivers along with the disruption of normal daily routines, living arrangements and the social isolation which is very acute in an isolation unit.

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The age of the child appears to be of paramount importance in terms of the acuteness and the chronicity of the psychosocial effects of isolation, with the younger child/infant being most at risk of long-term psychosocial problems and abnormal behaviours. Whilst the actual disease that causes the child to be in isolation may in itself not be an important factor, there is certainly a body of evidence to show that the severity of physical symptoms allied to the isolation environment is a particular factor that can exacerbate the problem. There is also some evidence to suggest that the type of isolation (particularly in terms of size of the isolation environment) can have an effect upon the psychosocial development during and after the isolation episode. Parents of children in isolation find the situation very frustrating, and not a little scary, and this again can be enhanced by the severity of ill health that their child is experiencing. Thus, parents of babies and children in intensive care are far more likely themselves to suffer from negative psychosocial effects than parents of children who may be isolated because they have an infection and are only isolated to prevent others from getting that infection. Parents of children with cancer or with SCID, for example, have a ‘triple whammy’ to contend with in that they have a child who is very sick, who requires treatment that may make them even more sick (such as cytotoxic chemotherapy and/or a bone marrow transplant/stem cell transplant), and are also in isolation for possibly many months. This longterm, yet acute, physical, psychological, emotional and social isolation environment can lead to many negative feelings and actions – although it needs to be mentioned that these negative feelings may well turn out to be positive coping strategies, and maybe a way of helping the parents to come to terms with their situation and the situation of their child. What is surprising, however, is the relative lack of recent research into these phenomena. With the onset of more aggressive therapies and treatments, then more children will be experiencing these situations, whereas previously they may well have died without any treatment being possible. This is certainly the case with the bone marrow transplants. For example, 25 years ago there were only half a dozen bone marrow transplants for SCID and other similar immunological disorders taking place in the UK each year, but now there are more than ten times that number taking place annually. Similarly, the number of intensive care units for children and neonates has increased dramatically compared to what was available 25 years ago. There have been similar exponential increases in the treatment of childhood (and adult) cancers, which again has increased the pressure for the facilities in which treatment can take place, including isolation units. The time is ripe for further research into the effects of present-day isolation environments, and the reasons for isolation, to mirror these increases. Finally, Freedman et al. (1976 p. 595) give further insight into the care of an infant with SCID undergoing isolation, ‘during the period of his hospitalisation he has not had a single relatively constant caretaker; rather a succession of nurses and attendants, as well as members of the medical team have been involved in his daily care. Included in the group are some individuals with whom he has had frequent contact throughout his life – his

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mother when he is at home, and some members of the medical team – as well as a larger number (especially nurses and attendants) who have been relatively transient but who at any given time have been active participants in his care. In addition he has, of course, been subjected to repeated painful interventions, such as blood drawing and other procedures which are relevant to the monitoring of its immunologic status’. The experiences that the children undergo during the time in isolation in hospital can leave a devastating mark on them and can affect their future psychosocial behaviour for many years afterwards, as can be seen from this comment by the mother of this UK girl who had survived a bone marrow transplant for SCID, ‘When she was about three, she used to cry every time we drove up to the hospital for her therapy. Every time we drove up there, she used to burst into tears. She didn’t want to go in. she used to scream and . . . not out loud, but sort of whimper . . . all the way in. And she didn’t want this thing done to her. Now I think she’s learnt to accept what the problem is. But I think it’s accepting because she has to accept, not accepting because she wants to’. As can be seen in this chapter and the preceding chapter (Chapter 4), it is very difficult to assign blame for the many and varied psychosocial problems that may affect children in isolation. Where exactly can the blame be pinned for the behavioural problems of children with SCID (or indeed children who have undergone similar experiences to those with SCID)? Hospitalisation at an early age, as was discussed in Chapter 4, can cause many problems linked to separation. Will a young child in isolation in a hospital have more severe psychosocial and behavioural problems or fewer problems? The evidence appears to demonstrate that a child in isolation in a hospital has more behavioural problems during and after treatment, than does a child who is in hospital but not isolated. However, the majority of children who require some form of isolation, whether it is to have cytotoxic chemotherapy, or a bone marrow transplant, or to have intensive care, or whose condition is so poor that they are at risk of catching infections from other children whilst in hospital, are often very sick. So to what extent does the severity of the illness affect the present and future psychosocial and behavioural problems? Again, it is impossible to say, but the severity of the illness does appear to play a role in future behavioural development, although to what extent has yet to be determined. Then there is the age of the child, with the evidence appearing to show that the younger the child the more the risk of behavioural problems developing. Although not discussed in this chapter, as will be seen in the later chapters (Chapters 8–13) the gender of the child may also have a bearing on whether behavioural and psychosocial problems occur, and if so, what the exact nature of these problems may be, for example whether the child be more prone to develop depressive behavioural symptoms or aggressive behaviour of symptoms. There are also the effects of the various treatments to be taken into consideration, although once more, this may be linked to the severity of illness experienced by the child, because often the more severe the illness, the more aggressive and extreme the therapy. When looking at the potential for the development of such behavioural

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and psychosocial problems, perhaps two of the most important factors to consider are the child’s personality both pre-illness and during the treatment itself, and the family environment. From my own experience, it is perfectly possible that two children of the same gender with exactly the same problem, and having exactly the same treatment at the same age can develop totally different psychosocial outcomes. This may well be down to the individual child and family, or perhaps there is still some mysterious factor X. that is yet to be discovered. Thus, it can be seen that the potential for the development of aberrant psychosocial and behavioural problems in children with SCID, and also in children with similar problems, is dependent on many factors.

6

Children’s Perceptions of Illness and Death

Introduction This chapter will consider how children and adolescents perceive ill health and death. The chapter will first of all look at what is meant by health and the importance of personal narrative illnesses. This will be followed by a brief discussion of children’s perspectives of health, before discussing children’s concepts of ill health. The concluding part of this chapter will discuss children’s concepts of death. Without a doubt, the first thing to point out in this chapter is that ‘being ill from a child’s perspective has not been often investigated’ (Forsner et al. 2005a p. 314). In the study by Forsner et al. (2005a), the five adolescent participants, who had a short-term illness, were asked to discuss the experience of being ill between the ages of 11 and 18 years. They reported that their illness disrupted their daily lives and made things unrecognisable. From their discussions with these participants, Forsner and associates concluded that being ill at the age of 11–18 years generates many feelings, such as being lost, of hurting, and of being in need of comfort not only from others but also from themselves. Illness comes as a disruption to daily life and the adolescents in Forsner et al.’s (2005a) study found that their way of ‘being’ became unrecognisable because they felt that they had lost their normal life – they were lost in the physical sense because they felt that they did not seem to recognise their body as their own because it had changed – it was not as it used to be – and in addition they were very tired and had no energy. These adolescents were also lost in their feelings because when they were ill they were vulnerable – their psychological defences were weakened making them depressed and worried. When referring to ‘hurting’, they were not only referring to themselves physically hurting because of their illness and the therapies associated 78

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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with their illness, but because of the vulnerability of their experience of illness they were psychologically hurting as well. As Forsner et al. (2005a) reported, it hurt them to be lonely because they could not share the experience with their close relatives and friends and it hurt them psychologically when their parents could not understand what the illness meant for them. Only they knew what they were going through and because they could not get anyone else to understand their experiences, they felt vulnerable, lonely and lost, and easily hurt by comments and perceived slights, as well as loss of dignity and privacy. Because of the discomfort caused by their illness and their hospitalisation, the adolescents looked for comfort not only from others, but also from themselves. They looked inside themselves to find what comfort they could from their situation – previous expectation of ill health assisted them in seeking comfort. However, just as important, they desired comfort from others – from their parents, their families and friends, and of course from the nursing and medical staff. Sometimes, this comfort was not forthcoming. For example, in another study, this time by Sartain et al. (2000), children with chronic ill health said that they were not listened to when they were in hospital; similarly, the adolescents in Forsner et al.’s (2005a) study were hurt/upset when they were not listened to by health professionals and when they believed that they were not treated with the respect that they felt was due to them. Another study involving teenagers with cancer detailed how their friends tended to ‘disappear’ the longer the illness continued (Enskar et al. 1997), a view also echoed by the teenagers in the study by Forsner et al. (2005a).

What is Health? ‘Overall, health seems to be an abstract concept, multi-dimensional and relative by nature, hard to measure and even harder to define’ (Almqvist et al. 2006 p. 277). Of late, the general concept of health has changed from what was previously considered to be a biomedical model, in which health and illness were seen as direct opposites, to a more holistic concept that takes psychosocial and behavioural attitudes and actions into consideration (Almqvist et al. 2006, Gochman 1997, Susman et al. 1992). Antanovsky (1987) defines health is being a continuum with absolute health and complete illness being at either end, and that anyone’s position on that continuum occurs as a result of interactions between various daily stressors and coping strategies of that person (see also Chapter 7). Early studies (Lasky & Eichelberger 1985, Natapoff 1978) demonstrated that young children (less than eight years of age) see health and illness as two totally separate concepts. In other words they fail to conceive of a person being partly healthy or partly sick – this could then make it difficult to place health and illness on a continuum. Almqvist et al. (2006) deduce that,

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if this is so, then there is an implication that young children are unable to understand that if they, or someone else, is sick, then they could also be healthy in other ways. If, as the WHO (2001) describe, health and illness can be viewed as two different dimensions, then health and illness could be present at the same time and therefore health is not something that a person has or does not have, but instead it is as a result of a continuing process (Almqvist et al. 2006). In the International Classification of Functioning, Disability and Health (ICF) different dimensions of people’s health are classified (WHO 2001). The main dimensions put forward by the ICF are:

r body structure and function (positive and negative body functioning –

r r r

positive body functioning being the structural integration and functioning of both physical and psychological dimensions, whilst negative body functioning being linked to structural loss and impairment) activity (a person’s ability to perform a task or an action) participation (a person’s ability and desire to engage in his own ‘life situation’) environment

These are all described in the ICF model as being important factors in relation to health (Almqvist et al. 2006). Looking for a moment at dimensions of adult illness, Goldman et al. (1991) state that amongst adults, five dimensions have been identified as important influences on health behaviour, and these are:

r identity (a determination of what the illness is – including ‘labels’ and symptoms)

r consequence (the evaluation of the short-term and long-term effects of the illness)

r timeline (the duration of the course of the illness) r cause (the factors that led to the illness) r cure (an assessment of how to assist in the recovery from the illness) Other researchers have come to the same conclusions as Goldman et al. (1991), including Lau et al. (1989), Bishop et al. (1987) and Meyers et al. (1985). Goldman et al. (1991) found that, as well as adults, children also understand these five dimensions of illness, and in addition, they revealed that there were associations among these five illness dimensions with the concepts of nutrition (in terms of healthy or unhealthy eating), as well as the children’s knowledge about routine medical examinations. Nettleton (1995), during her discussion on the social basis of illness, points out that some authors distinguish between the concept of disease (which is, as Kleinman in 1988 explains, a physiological occurrence) and the more social concept of illness. Again, Kleinman (1988) refers to illness as a concept which is concerned with how the sick person, as well as members of the patient’s family (or even the patient’s wider social network), perceive and respond to the symptoms of illness, as well as any disabilities that are encompassed by the particular illness. Alongside this perception of illness, Kleinman’s (1988) concept is concerned with how the patient, and

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the important people in his or her life, live and cope with the illness in its total and holistic manifestation. Illness is a very personal concept and state. Many things can affect our perception of illness, as indeed they can affect our perceptions of the environment in which we find ourselves at any time, for example being in hospital. Davey (2002 p. 12) explains, ‘people experience an illness or disability not as an isolated state, but in a social context. The same apparent condition may carry quite different meanings in different times and places’. Dingwall (1976) argues that illness as a stand-alone concept does not exist – it only exists through the meanings and interpretations of the total experience (past and present) of that patient and that illness.

Personal Illness Narrative ‘To be ill is a unique personal event and the person affected may find it difficult to communicate the experience to others. The illness narrative provides information about personal and cultural meaning’ (Forsner et al. 2005b p. 314). How we perceive an illness can be linked to our past experiences, because if we have experienced an illness once and we then experience that same illness again at a later time, then, it is no longer something that is ‘unknown’, but rather something that we can understand and can cope with through the various stages of the illness because of our previous experience. However, if we are experiencing that illness for the first time, then fear of the unknown comes into play, and our perceptions are coloured by what we do not know rather than what we do know. Even pain can be affected by past experiences, for if we were unfortunate enough to break an arm today and we have broken an arm in the past, we know what is happening and we have experienced the pain associated with a broken bone previously. We have experience of the amount, the type and the duration of the pain, as well as experience of coping with our arm in a cast, and knowledge of how long the healing process will take. In other words, we already have a personal ‘narrative’ of living with a broken arm and that narrative is one that we can turn to any time we suffer a similar injury. Kleinman (1988) explains that it is the interpretation of the illness itself that contributes to how it is experienced, rather than just the physical signs and symptoms, although undoubtedly they play a part as well, and allow the patient to come to terms with the whole ‘illness experience’. Another important factor that determines how we perceive an illness is age. As we get older, we experience many illnesses (and accidents), and therefore build up many narratives, or rather layer upon layer of a single narrative (almost like a book). Cognitively, as we get older, we can also have a better understanding of what is happening to our bodies during illness, and this again will alter our perceptions. When we are very young, we have usually neither had the time nor the experiences to develop such narratives in relation to ourselves, as well as not having the knowledge to

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understand what is happening to our bodies. We are much more reliant upon other people’s ‘narratives’ of ill health; particularly our parents, friends and possibly the health professionals with whom we come into contact during our illness. These narratives are very important to us, because we construct them in order to make up a meaningful story that can explain the illness that we are experiencing. These narratives that we develop during episodes of ill health are known by sociologists as ‘personal illness narratives’. Davey (2002) explains that the stories or narratives that we develop are unique to us individually and are attempts to make sense of our illness so that it becomes an understandable event that occurs at that particular point in our life. However, as Davey (2002) goes on to discuss, our personal illness narratives are more than just a collection of facts, rather they also deal with our feelings and perceptions of our illness, as well as those of everyone else who is involved in our care. Further developing her theme, Davey (2002) states that the narrative that emerges from our illness (which does not just encompass the symptoms, but also the treatment that we require for that illness) is unique because the ‘ingredients’ that come together to make narratives do vary between individuals. In addition, they tend to include past events in that person’s life (their personal history) which may have a bearing on the present illness, as well as on how effectively they can cope with it. Everybody’s personal account/narrative is an ongoing story because it describes the dynamic state of illness which can change from day to day (sometimes, as for example with children who have SCID [severe combined immunodeficiency], or who are in intensive care, it can change from hour to hour, if not from minute to minute). In addition, these personal illness narratives consist of a variety of individual strands such as the information that is already known, or that is given during the course of the illness, as well as a wealth of detail that the sick person considers to be important factors in their experience of that illness. Nettleton (1995 p. 79), being a sociologist, takes a slightly wider view of these narratives and states that ‘how people make sense of their illness is within the context of their personal biographies and in turn this must invariably be influenced by, and mixed in with, the cultural values of the society in which they live’. Because we are partly a product of society in which we exist, then Nettleton (1995 p. 78) stresses that all personal biographies are very much linked with our own society’s ‘biography’, and cannot be seen in isolation from it. She goes on to make the point that ‘when people have spoken about their experiences of illness it becomes evident that these are woven into their biographies’. It is important to remember that it is not just a person who is experiencing that illness who will construct a personal illness narrative. As well as the sick person, every one closely connected to that person, such as parents, will also be producing their own personal illness narratives which may be very different to the narrative produced by the patient. This is because it will include all their unique knowledge, experience and feelings around that illness, as well as their links with the person who is ill.

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There are very important reasons why we need to construct our personal illness narrative. For example, constructing narrative may allow us to feel that we have some degree of control over the events surrounding us and our illness, particularly if we can understand why it has occurred. It may also help us to prevent the illness occurring in the future (for example, if we know that falling off a motorbike was the reason why we broke our arm, then we may decide that we are going to sell the motorbike and so reduce the risk of it happening again). In addition, by constructing our narrative we may be able to reduce the impact that the illness is having on us at that time. Other reasons put forward by Davey (2002 p. 17) for the importance of constructing our own personal illness narrative are that it may ‘. . . help us to counter accusations of blame for our ill health; or it may reinforce our faith in (for example) medicine or religion, which will sustain us in difficult times ahead if the condition worsens’. According to Kleinman (1988 p. 49) a personal illness narrative is a story told by the patient, and retold by significant others (for example parents) in order to give some coherence and understanding to the ‘distinctive events and long-term course of suffering’. Kleinman (1988) also points out that the structure of a personal illness narrative, just like the structure of a written story, requires its own plot lines and imagery that are taken from both cultural and personal models in order to allow the experiences that are interwoven within the narrative to be arranged in ways that are meaningful, and that can be effectively communicated. However Kleinman (1988) also suggests that anyone’s personal narrative is not just a device for reflecting the illness experience alone, but instead it contributes to the perceptions of the symptoms and suffering that are inherent within the illness – these being modified positively or negatively by personal and societal experiences. Finally, Nettleton (1995) makes the point that it is important to understand ‘illness action’ rather than just observe ‘illness behaviour’. ‘Illness behaviour’ is concerned with the way that people behave whilst in the presence of the symptoms experienced, whilst ‘illness action’ is concerned with how people make sense of and interpret their illness experience. So, it is not just a matter of relating the experiences encountered, and lived, during the illness, but it also involves some interpretation of the illness experienced, to enable the patient to make sense of what it means to him or her. As Almqvist et al. (2006) point out; experience of illness is a prerequisite for a child’s understanding of the perception of health, and that individual variations in a child’s understanding of health are dependent on earlier experiences of health and ill health, as well as the child’s characteristics and ability to cope with health/ill health. That does not necessarily mean that the child has personally had to have been ill before starting to understand what health and ill health are, rather it can include the observations and experiences that they may have had with others who have been ill. Of course, as soon as a child becomes ill, he or she will start to construct their own personal narrative of illness. It is rather like the difference between writing a story and having a blank page before you with no idea of what you are going to write, and continuing a story that you

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have already begun, where many of the characters already have a ‘back story’.

Children’s Concepts of Health Miller (2006) poses the question, how do children view health? She partly answers this by discussing that it is difficult for some children to define ‘health’ because it is such an abstract term. However, at the same time, she notes that even young children have clear ideas about the factors underlining health and behaviour. Age and cognitive development appear to be crucial in understanding health – although it is often defined (even by adults) as an absence of ill health. It appears easier to conceive of ill health than it is of health. Most early studies focus on the relationship between cognitive development and the child’s conceptual understanding and knowledge of the concept of health (Almqvist et al. 2006). It has been hypothesised for some time that an understanding of many complex concepts, including health and illness, develops gradually throughout childhood (Goldman et al. 1991, Natapoff 1982, Natapoff & Essoka 1989, Tinsley 1992). Gochman (1997) demonstrated that the perceptions that children hold are more concrete, egocentric and less abstract than are the perceptions of adults, but that with increasing maturity children gradually develop more adult-like perspectives. Susman et al. (1992) make the point that a child’s early concept and understanding of health run parallel to his attitudes to health and patterns of health behaviour, and this affects the well-being of that child when he is an adult. An understanding of ill health and its causes and prevention, as well as its treatments and therapies can differ and vary considerably at various ages and stages of development from infancy through to adulthood (Perrin & Gerrity 1981). A study by Normandeau et al. (1998) showed that children aged from 5 to 12 years of age had a complex perception of health that was formed around three dimensions:

r everyday functioning r healthy lifestyle r mental health (including a more general sense of well-being as well as their relationships with significant others)

The answers that were given by the youngest children in the study (those aged from five to six years) were mostly related to:

r being outside r having tasty food to eat r good friends

These younger children perceived health in relation to situations in their everyday life and their environment and that this engagement with their life and environment can be seen as both a perception and an expression of health by young children (Almqvist et al. 2006). Indeed, a study in Canada (Canadian Association of Occupational Therapists 1997) demonstrated the

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importance of interaction with the environment when they found that children who spent some considerable time interacting with their environment delivered a wider behavioural competence than did children who spent less time engaging with their environment (perhaps a concern in this age of the computer). This study also showed that those children who did interact with the environment participated more often in the development of peer- and age-appropriate activities. Miller (2006) noted that from a young age, children are aware of the importance of lifestyle, particularly in its relation to health, and a study by Oakley et al. (1995) of children aged nine and ten years identified the factors in life concerned with health and ill health that were considered important by this group. They did this by asking the children to draw pictures of healthy and unhealthy factors that impinged on their lives. The two major factors that were implicated by the children in their drawings were factors associated by diet (the most important factor) and factors associated with exercise and sport. In the same year, James (1995) asked a group of four- and five-year-old children in London to draw and discuss factors and issues that impacted on their health. These were all negative and included a lack of a safe outdoor space for them to play in, a fear of being bitten by dogs and of being abducted by strangers, and a concern about the noise and the pollution from traffic. A rather sad commentary on our world today, and in marked contrast to my own early childhood 60 years ago. A discussion on the relation between environment and ill health appears later in this chapter. Finally, in this section, Almqvist et al. (2006 p. 276) discuss the difficulties of defining health because it is such a complex concept, and that for the child, so much depends upon their age, cognitive development and their experiences. As they say, ‘The manners in which children perceive multi-faceted concepts such as health probably vary with personal characteristics, child development and earlier experiences’.

Children’s Concepts of Illness ‘Effective communication between health care professionals and patients is a complicated process when two adults are the only participants. When the patient is a child, developmental differences in basic understandings of how the world works, and of health and illness, may further impede effective communication’ (Perrin & Gerrity 1981 p. 841). A child patient will have personal knowledge of his or her illness that is derived from experience, and whilst it has to be acknowledged that this information is both subjective and emotional, it is important that we focus on the patient’s perception of their illness as opposed to the professional’s perception because, as Forsner et al. (2005b) point out, professionals tend to see illness from the medical perspective and they base their knowledge on the symptoms that are easy for them to measure and to deal with. Miller (2000) stresses that parents and other adults cannot really give a

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valid view of the child’s experiences; it is only the children themselves who can accurately report their own experiences (Carter 2002). Much of the literature that has been written on children’s understanding of illness has been concerned with the emotional effects of their illness and also their hospitalisation (Perrin & Gerrity 1981). According to Almqvist et al. (2006) young children perceive health as being a multi-dimensional construct that is largely related to being engaged with things/people/environment. Almqvist et al. (2006 p. 275) define ‘being engaged’ as being ‘able to perform wanted activities and participate in a supportive every-day context’. Children’s concepts of illness vary according to the age of the child, and their stage of development (Bibace & Walsh 1981, Bird & Podmore 1990, Gochman 1997, Goldman et al. 1991). Similarly, Toombs (1992) and Eiser (1985) believe that the meaning of illness changes throughout childhood and the changes are related to the child’s cognitive development, leading to both their understanding of the reasons for their illness and their future expectations. Forsner et al. (2005b) know that assumptions are made that at around the age of 11 years a more mature reasoning of illness and health can emerge (Bibace & Walsh 1980, Natapoff 1982). This maturation in understanding illness and health may be linked to previous experiences of illness (Crisp et al. 1996). Eiser (1990) explains that for many people, a child’s concept or understanding of his or her illness is related to knowledge about the illness and the treatment. She says that research into children’s knowledge of their illness is ‘usually justified on the grounds that some knowledge is necessary in order that children can assume a degree of responsibility for controlling their own health’ (Eiser 1990 p. 81). However, knowledge about the disease and its treatment will form only part of the child’s personal illness narrative, because it excludes the whole range of experiences, both personal and societal. Eiser (1990), discussing the work of Bibace and Walsh (1981) as well as Perrin and Gerrity (1981), links children’s concepts of illness as being related to a shift from pre-formal to formal operational thought. Between the ages of four and seven years, children’s beliefs and concepts around illness are very much linked to thoughts of punishment for a misdemeanour (no matter how minor), or even magic (linked to fairy tales – witches, wizards, and ogres etc.). Between the later ages of 7 and 11 years, children start to accept that there are actually a number of causal factors linked to an illness, although their understanding of what these causal factors are and how they work may be limited. At about the age of 11 years, children start to realise that illness could well occur as a failure of a particular part of the body, and around the age of 14 years, there is a recognition by some children that disease may also occur as a result of psychological stress (Eiser 1990). Other studies (Cook 1975, Gellert 1961, Simeonsson et al. 1979) found that concepts of the causes of illnesses progressed and changed with the increasing age of children. Young children were more likely to blame themselves for their illness (Gellert 1961) and in general at a young age children

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are more likely to explain the cause of illness with egocentric concepts (Perrin & Gerrity 1981). However, all these studies showed that, in line with Piaget’s (1929) theoretical framework of intellectual development, the children’s conceptions of illness became more objective and rational, and better articulated, the older they were. In addition, the more mature children became increasingly more aware of the many different interacting factors that could cause disease and ill health. Children who are in hospital have been known to believe that the cause of their illness was somehow due to them disobeying their parents and that they are therefore incarcerated in hospital as a punishment for this disobedience or because they had been rejected by their parents due to their perceived disobedience (Bergmann 1965, Langford 1961, Perrin & Gerrity 1981). Arguing against this, Brodie (1970) in a study of the perceptions of healthy children towards illness, found no evidence of illness being seen as a punishment in any way. However, in a paper presented in 1961, Gellert found that two thirds of the children in the study attributed the causes of ill health as being ‘transgressions against rules or to admission of specified actions on the child’s part’ (Perrin & Gerrity 1981 p. 847). Perrin and Gerrity (1981) also argue that the emotional effects of separation from their parents, along with other aspects of being in hospital, such as the medicines that they have to take (along with other therapies) can be confused in the mind of the child with their own conceptions of their illness and its cause. Also of note, Perrin and Gerrity (1981) discuss that children who are in hospital generally gave more immature explanations of what illness is than did children who were healthy. However, children as young as four or five years of age have shown that they have an understanding of the concept of health and also the consequences of their own health behaviour and the health behaviour of others (Almqvist et al. 2006, Flaherty 1986, Lasky & Eichelberger 1985). In 1988, Clunies Ross and Lansdown conducted a study into the concepts of death, illness and isolation found in children with leukaemia. One of the areas they studied concerned the children’s beliefs of the causes of illness, and they found that the notion discussed earlier in this chapter of children believing that ill health was caused by them doing something wrong did not appear to apply to this group of children. As they reported, the ‘postulated notion of eminent justice was not found to be common’ (p. 373). So, as Almqvist et al. (2006 p. 276) conclude, there are ‘still some contradictory findings to what extent young children can interpret their own or others’ actions as causes for certain health outcomes’. The young children, aged seven to ten years, in the study by Forsner et al. (2005b) found it hard to distinguish between reality and imagination – for them, reality and imagination seem to coexist. Whilst they had knowledge of their illness that was based both on previous experiences and also from what they had been taught, their thinking about their illness seemed to be coloured by their imagination. Their descriptions were often contradictory in their experiences both within the same child as well as between all the children in the study. They could be worried and scared, or they could be confident. They could be sad or they could be comfortable and cosy. And

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whilst they could be hurting and in pain, they also found the experience can be fun and comforting. In addition, the children described how being ill and being in hospital was like being caught in a trap and wanting to escape, and that the illness seemed to have occupied their body and caused the many distressing symptoms that they were experiencing. They also appeared to be of the belief that their illness would disappear if they ignored it and pretended that it did not exist, and a very interesting point that was made by them is that they felt that if they did not tell their parents how they were feeling then perhaps the illness would go away. At the same time, as Runeson et al. (2002) confirmed, when the children were anxious and having a bad time, they wanted their parents nearby. They wanted to feel safe and to feel safe they needed to be with the things and people with which they were familiar. Conversely, in the Forsner et al. (2005b) study, having their parents with them did not always help with the fears, because the children in the study who experienced such fears said that even with their parents by their side all the time they were still frightened. Linking to the five dimensions of illness discussed by Goldman et al. (1991) and mentioned in the section headed ‘What Is health?’ above, namely cause cure, identity, consequence and timeline, there are suggestions that children’s models of illness also guide their emotional and behavioural responses in addition to their coping strategies in response to ill health (Goldman et al. 1991). An example of this, by Johnson (1982), refers to children with diabetes. Johnson (1982), cited by Goldman et al. (1991), found that those children with diabetes who perceived their illness as being a destructive force in their lives (the consequence) tended to have poorer control of the diabetes. Similarly, children in hospital with chronic ill health who do not understand why they require medical treatment (the cure) regard doctors and nurses more negatively than those who do understand why they need to have medical treatment (Brewster 1982). As mentioned above, there are some children who see their illness as being a punishment for misbehaviour (the cause), feel guilty because of this, and therefore tend to have more negative emotions that are linked to their illness and their symptoms (Kister & Patterson 1980). Goldman et al. (1991) also found that a relatively high number of children did have some cognition of the process of treating the cause of an illness, although children aged four to six years did not appear to have an understanding of the cause of illness, and that same age group appeared to lack an understanding of how they could play a part in both preventing and ameliorating an illness. Earlier, Parcel and Meyer (1978) also found that children’s beliefs about their control over their own health and recovery are not related to their beliefs about the cause of their illness. As has been mentioned previously, there is the possibility that active experience with aspects of an illness can lead to a child’s cognitive understanding of that illness. Turning to the SCID study that is central to this book, the parents of the children who had survived bone marrow transplantation for SCID were asked if they thought that their children considered themselves to be ‘unwell’ in any way. Ten out of 34 parents of the children (29%) felt

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that their children did consider themselves unwell, and consequently had ‘unhappy’ lives, and gave the following reasons:

r r r r r r r r r r r

Aware of his illnesses, complains of pain. Parents consider their son to be a ‘hypochondriac’. Knows he has been ill and still has to take medicines. He is a ‘hypochondriac’. Aware of his illnesses, his deafness and poor eyesight. Aware of his illnesses. Complains of pains. Often thinks he is going to die because of his health problems. Knows he has been ill. Complains of pains, has severe deafness, still has serious health problems Still has to take medicines – considered by parents to be a ‘hypochondriac’.

Personal observations of some of these children supported the parents’ knowledge of their children’s perceptions of their lives as not very happy because of their actual or perceived ill health. This actual (or perceived) ill health impinges on, and often defines, their whole lives, so that they continually feel different from their peers (and sometimes from their siblings also). This is exacerbated by their parents’ attitudes and comments, for it must be remembered that just as some of the children in this study can consider themselves as prisoners of their previous/present illness, so some of the parents likewise can feel themselves to be prisoners of the disease. And indeed, some of the siblings are also deeply affected by the illness and treatment, so that they can consider that their lives have been blighted by their sibling’s illness. Acute and/or chronic ill health does not just affect the person who is ill. The ripples of that person’s illness spread out in ever expanding circles, touching and encompassing close family members, extended family members, friends of the family and even members of the wider society. To illustrate the point made above concerning children experiencing ill health, one German boy in the SCID study, when asked to draw a picture of himself, drew himself vomiting blood (Figure 6.1). Although in the picture the sun is shining (and even smiling), the boy in the picture is very unhappy. At that particular time this boy was feeling very depressed as he had influenza, but it is rather frightening to think that this is how he saw his life at that time – several years after the transplant. However, a diametrically opposite finding in several of the children in the SCID study was a state of denial. As far as they were concerned, they were fine, and sometimes they even denied that they had ever been ill – echoing to some extent the response of a very few fathers in the study to their child being desperately ill in hospital (see Chapter 11). This state of denial echoes studies by Burns and Zweig (1980) and McCully (1963) who explore the fact that a male child may use denial in order to ease their own anxieties as well as to try and keep them functioning in a world that,

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Figure 6.1 How one boy felt on the day the family was interviewed (aged ten years). [Previously published in Vickers P.S. (2006) Communicating with children and families. In: Peate I. and Whiting L. (eds.) Caring for Children and Families, Chichester, John Wiley and Sons, pp. 25–48 (p. 36).]

according to Burns and Zweig (1980 p. 188) has been ‘shaped to fit the needs of a healthy child’. McCully (1963) interpreted his findings as supporting the notion that the ill child would use both denial and fantasy in order to help deal with his or her awareness of the seriousness of the illness and the future. So, according to Burns and Zweig (1980) the sick child’s attempt to be similar to other children by denying his or her illness is one way of coping with the illness, particularly in chronic ill health. As Burns and Zweig (1980 pp. 188–189) conclude, ‘Denial, repression, and hope can be undoubtedly used by chronically ill children and their families to maintain a compromise between ego-ideal and realistic self concept’. Pidgeon (1966 p. 21) agrees with McCully (1963), and she makes the point that ‘the child’s concepts of procedures, and the extent to which they are reality-based or fantasy-dominated, will influence his capacity to cope with the stress of his illness and hospitalisation’.

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A child’s psychosocial behaviour can be seen as an outward manifestation of his perceptions, concepts, understanding, and knowledge of not only his illness, or even of his therapy and treatment, but also of how he fits within his own environment, the environment occupied by his family, friends and neighbours (whether ill as he himself is or healthy as others are), his past experiences, and the expectations both of himself and of those around him. In particular, it is determined to some extent by his stage of cognitive development, for without cognition, he will have difficulty in understanding what is happening to him and why, and hence his perceptions of both him and his illness will be radically different to what it would be if he did have understanding.

Environment and Ill Health In recent years, as our knowledge about the interrelationship between children’s biology and their early experiences and environments has advanced considerably, so an understanding of their experiences has also advanced (Shonkoff & Phillips 2000). As Irwin et al. (2006) point out; we now have got a much greater understanding of the social and emotional development of children, alongside their cognitive, language and physical development. Hertzman (2000) explains that how a child develops in all of these facets influences their health, well-being and competence throughout their life. For example, children who experience multiple or ongoing risks in their life and their varied environments are more likely to experience some degree of health problems (Willms 2002). The problem to be addressed, therefore, is that whilst some children who experience these risks within the course of their life experience various health problems, why do others who experience similar risky circumstances do much better in terms of health (Irwin et al. 2006)? Some people (Grotberg 1995, Werner & Smith 1982) have suggested that it is the children’s own perspectives and understanding of the context in which they are existing that play an essential role in determining their present, past and future health status. However, Irwin et al. (2006) argue that whilst the child’s perspective plays an important role in establishing a connection between their living conditions and their understanding of health, we still, as yet, do not fully understand the role of the child’s own perspectives in resolving the dichotomy that exists between the child’s living conditions and his or her health outcomes. Various studies (Brooks-Gunn 1995, Feldman et al. 2000, Kohen et al. 2002, Kotchick & Forehand 2002) have discerned several factors that can cause a higher risk of poor health outcomes in children, including:

r r r r r r r

living in poverty living in a neighbourhood with high crime living in environments with overcrowding living in unstimulating family environments negative parenting styles low maternal education maternal health problems

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In addition, of interest to the theme of this book, living in isolation has also been shown to be one of the risk factors for poor health outcomes in children (Irwin et al. 2006). Most of the risk factors mentioned above are linked to the child’s home, but Irwin et al. (2006) make the point that once children reach school age then there is an increase in the child’s interactions and experiences within different contexts and environments as their horizons are expanded both by the school and the surrounding neighbourhood. Irwin et al.’s (2006) study was concerned with children’s perspectives of health, rather than their perspectives of ill health. However, their findings and conclusions are relevant to this chapter insofar as our perceptions of health will have a bearing on our perceptions and concepts of ill health. If we know what it is like to be healthy, then, we can have some concept of an absence of health. There cannot be any one who has not experienced ill health at some stage in their own lives (particularly as, for example, each one of us experiences approximately 12 infections in the first year of life whilst our acquired immune system is learning to make us immune to common infections). For most children in the developed world, fortunately, these are experiences of minor episodes of ill health, but they are still an experience for them of an absence of full health. Bearing this in mind, but returning to Irwin et al.’s (2006) study, in discussing the perceptions that children have of health, they conclude that it is context that shapes children’s perceptions of health, and they make the point that professionals need to pay close attention to just how context shapes children’s perspectives of physical health, and in particular the child’s connection to ‘place’ and their feelings of safety in determining their perceptions of good health. This context of environment that can shape children’s perceptions of health and ill health is particularly pertinent when considering the context in which children experience chronic ill health. In particular, as regards to the child’s connection to place as well as his or her feelings of safety, we need to look at the environment, both physically and psychosocially, with which a child with acute or chronic ill health is familiar. In terms of place, there may well be restrictions as to their environment, whether in hospital or at home, and the overriding concern is often the safety of the child. For example, if a child has asthma then the parents may well become overprotective and make their child aware of the potential dangers linked to their asthma. Similarly, with diseases such as diabetes, cystic fibrosis and immunodeficiencies there are seen to be limitations that the child has to accept. Whilst it is sound practice to make a child with a chronic disease aware of potential problems, it can take away from the child his or her feelings of safety. This may lead to the child being physically or psychosocially isolated. James (1993) makes the point that children’s play provides an important function in terms of socialising, because it is during play that children learn about their, and other people’s, identity, as well as the various social subtilties involved in relationships, and more importantly they can learn to negotiate the social mores that are so essential to the smooth

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functioning of society. Without the opportunity to play and socialise with their peers, children will miss out on many of the normal socialisation experiences and structures that help to make a person feel safe and comfortable within their environment, as well as missing out on a way of communication – another essential tool for feeling comfortable in the company of others (Vickers 2006). As Bruce (1996) suggests, play helps children to become complete people who are physically healthy as well as being psychologically healthy. Indeed, Wong et al. (1999) refer to play as being one of the most important experiences in a child’s existence, whilst Donnelly and Robinson (2006 p. 155) forcefully make the point that play is essential for children and young people because there is a relationship ‘between play and cognitive, social, psychological and emotional development’. In order to enhance socialisation however, play has to be a cooperative activity rather than a solitary one, and the danger for the child with longterm ill health, or long-term and/or frequent hospitalisations is that they will miss out on cooperative play, and hence could have difficulty in social situations, particularly with their peers (see Chapter 12). These children can then start to see their lives as being deficient in many ways due to the fact that they are ill, and it is this continuous or long-term interruption in their normal existence, leading to such problems as isolation from their peers, allied to the pain and discomfort, that is going to colour their perceptions of ill health, as much, if not more so, than the actual disease. The effects of this isolation were visually depicted by children in hospital in a study by Pelander et al. (2007), who noted that the children’s drawings of their rooms in hospital showed very few people, where they had expected the drawings to include more people, especially parents, although contrary to the drawings in Pelander et al.’s 2007 study, Van Andel (1990) reported that children prefer places where there are other children. Some of the children in the SCID study also drew pictures of their time in hospital – or rather their perceptions of their time in hospital. The first picture below (Figure 6.2) shows the boy from the UK who drew this picture of himself lying on a bed in hospital with a nurse at the foot of the bed. Because his time in hospital was mainly spent in isolation, then it is probably not surprising that there are no other patients in the picture, but the absence of the parents, who were able to go into the isolation cubicle, is noteworthy, and to some extent supports the study by Pelander et al. (2007). In this picture, the boy who painted it mainly used the colour red, which is often associated with blood and danger. The second picture below (Figure 6.3), also drawn by one of the UK boys in the SCID study, again shows a child lying on a bed, this time completely alone. The fact that he has drawn bars around his bed is an indication of how he perceived his isolation during his treatment, and perhaps an indication of how he still perceived his state (i.e. one of still being in some form of isolation). Pale red was also the principal original colour of this drawing. In addition, an analysis of drawings of children’s families in a study by Clunies Ross and Lansdown (1988) did support this hypothesis that sick children do perceive themselves to be isolated in hospital. In all the drawings of their families, or a least in all the drawings where it was

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Figure 6.2

Boy, with a nurse, in isolation in hospital (aged seven years).

possible to distinguish facial features, the children drew everybody smiling. However, in drawings of themselves in hospital, over 30% of the children drew themselves with sad faces or crying.

Children’s Perceptions and Concepts of Death Some of the children in the SCID study sometimes had intrusive thoughts of their own death. Kane (1976) demonstrated that a child’s perception of death differed with the child’s age. For example, she suggested that by the time they are 3 years old, children often have a realisation of death, but it is not until about the age of 12 years old do they have an accurate idea of what a dead body would look like. Kane’s conclusion was that it is not until they are seven years old do children acquire a more or less complete concept of death. A moment’s thought will show that this is rather a simplistic concept of children’s perceptions of death, because it does not take account of experience. Whilst it may now be usual that children in the developed world in 2007 rarely come into contact with dead people, for previous generations this was quite a common occurrence. For example, during World War II children in the UK often came into contact with death as a result of air raids – and that is not including children in the occupied countries who were directly in the line of fire and for whom death was an ever-present companion. Even after the war, certainly in the geographical area and social environment in which I was born, the bodies of family members and neighbours who had died were put on display in an open coffin in their home for all people, including children, to visit and to pay

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Figure 6.3

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Boy in isolation in hospital (aged nine years).

their last respects. As a very young child I can still remember seeing and touching my grandmother who was lying in a coffin in her front room. And children in earlier generations, along with children in many parts of the world, were/are very accustomed to the sight of death. So, it is only in quite recent times and only in certain parts of the world, that children have been shielded from death. Experience, along with age and cognitive development, play a large part in formulating a child’s perception of death. Indeed, several people have specifically related children’s explanations of death to their stage of cognitive development (Kastenbaum & Costa 1977, Koocher 1973, White et al. 1978). Eiser (1990) makes the point that pre-school children have been reported as regarding death as a reversible process and deny their own personal mortality, but this is possibly also linked to their cognitive development and experience, rather than just their age. This is supported by Bluebond-Langner (1978) who maintained, as has been discussed above, that age on its own will not predict a child’s understanding of death. In her book on children who were dying, BluebondLangner (1978) suggested that children who are terminally ill have to pass

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through a series of stages and experiences in order to understand the nature of their own illness, as well as to gain an insight into what ‘death’ actually means. She went on to argue that, as long as they have passed through the various stages of their illness, along with understanding and experience, then even the children as young as four years of age are able to understand both the finality of death and the separation of death. In addition, she maintained that these young children gain an insight into, and a realisation of, their own imminent death. Kane (1976) concluded that there were a number of components that together constituted the concept of death. Initially she maintained that there were ten components, but then later she reduced them to nine and these components ranged from the functionality of a dead person to the location of the body (Clunies Ross & Lansdown 1988). In 1985, Lansdown and Benjamin took seven of Kane’s nine components as the starting point for their own study of children aged five to nine years with leukaemia. These seven components were:

r r r r r r r

separation universality causes of death irrevocability appearance of a dead body insensitivity of a dead body cessation of bodily function after death

In their study, Lansdown and Benjamin (1985) reported that 60% of the five-year-old children who participated in the study had a complete, or an almost complete, concept of death at that age. Clunies Ross and Lansdown (1988) followed up this research by Lansdown and Benjamin (1985) by looking at the concept of death of 21 children with leukaemia who were aged between four and nine years of age, with the addition of the children’s own ideas about the causes of illness and an analysis of drawings and the children’s families. In this study, Clunies Ross and Lansdown (1988) found that whilst the concepts of death held by children with leukaemia were not significantly different to those held by healthy schoolchildren, the youngest children with leukaemia demonstrated a better understanding of some of the above components which make up the concept of death than did the healthy children (a finding that reinforces the hypothesis mentioned above that experience, rather than just age, can add to a child’s concept of death). There were two particular components of the concept of death that the young children with leukaemia scored relatively higher than did the healthy children of the same age, and these were cessation of bodily function as well as irrevocability. According to Clunies Ross and Lansdown these two components are features of death which have a particular relevance and importance to people who may be facing death. Of interest is that in this study, the most frequent cause of death listed by children is violence or murder. Perhaps there is some denial at play here with these children – a denial

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that, although they understand death, they want to believe that they can only be killed by a violent action, rather than by the disease that they have (leukaemia in this case). This finding confirms the finding of a much earlier study by Schilder and Wechsler (1934), although in this earlier study, the children were shown pictures of violent deaths and it was concluded by the researchers that children often associated death with violence. However, Koocher (1981) felt that these findings were of questionable validity because of the methodological bias (the violent stimuli presented to the children). In their discussion at the end of their paper, Clunies Ross and Lansdown (1988) report that 30% of parents refused permission for the researchers to discuss the concept of death with their children. Some of the reasons given by the parents for refusing permission are quite interesting. One mother apparently told the researchers that ‘He has been talking about death and dying recently and I don’t want him to be reminded about it’ (Clunies Ross & Lansdown 1988 p. 383). Another mother said that ‘For us the treatment time and the questions about whether she would live or die are so long ago that there is no point in digging them up again’ (Clunies Ross & Lansdown 1988 p. 383), whilst a father pointed out that ‘Clinic days are bad enough without talking about that’ (Clunies Ross & Lansdown 1988 p. 383). Whilst from these quotes above it appears that the parents are trying to protect their children, in actual fact they are probably trying to protect themselves, which in itself is very understandable. For parents, the idea of the death of a child that will precede their own death is an unimaginable horror – although as mentioned, the survival of so many children is quite a recent phenomenon, and is not the case in so many parts of the world even today. However, for parents in the developed world in 2007, the death of a child is a relative rarity, and so some degree of denial in the parents is understandable, and I have had much experience of this in my professional life as a children’s nurse. However, at the same time, having nursed many children who were dying, I have found that the children themselves are often not afraid of dying, but are very concerned about their parents, and I have even known some older children not want their parents to be told that they are dying because they want to protect them. Bluebond-Langner (1978) and Spinetta and Deasy-Spinetta (1981), for example, believed that refusing to talk about issues of death and dying with children who have diseases that are life-threatening (such as leukaemia) actually makes it difficult for the children to come to terms with the stresses of the situation and so be able to find coping strategies from within themselves. As Clunies Ross and Lansdown (1988) point out, it is just possible that the children whose parents refuse to allow them to take part in their study may well have been the very children who were actually most in need of being given an opportunity to talk about death, and their own death in particular. They further argue that in families where it is possible to talk about death, and where the parents are able to talk with their child about the possibility of that child dying as a result of their illness, there is more likelihood of that child being able to form an accurate concept of death.

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In the SCID study, there was little talk of death, apart from recollections of the past, when other children of the parents died with the disease – including two parents who had lost three children before the successful treatment of their fourth. Parents were also prepared to talk about their feelings at the time of treatment when their child’s life was continually in danger, and they talked about children of other parents who died in the hospital, at the time their child was having transplant. But they did not talk about the possibility of death at this stage, several years posttransplant, although a common question that was asked of me by the parents was concerned with how other children were getting on with their lives following the bone marrow transplant, and there was often a very definite feeling of relief when assured that no one who had survived the initial treatment had since died. The children in the study did not mention death, and of course, as the interviewer, it was not ethical for me to ask them, or their parents, about this subject, because the whole focus of the research was on the survival of the children. However, one mother did mention her child who did get depressed often. This boy – an adolescent in Germany – had very negative feelings towards the state of his health in particular, and his life in general. As his mother explained, ‘He worries. He thinks that he is going to die. If he has some pain, he has a headache, or he is poorly, or if he is dizzy, or if he has one of his many bad colds, or if he has to have blood, he says, “Oh God, I’m going to die”’, but it was said with a smile as if she was confident that everything would be fine. Bluebond-Langner (1978) describes well the loneliness and feelings of isolation that are caused to the terminally ill child by the societal taboo placed upon the open discussion of death. Some children use different ways to try and express their knowledge of, and their feelings and questions about their presumed or imminent death (Burns & Zweig 1980). For example, play with dolls, play with other children, or what appear to be general conversations with their parents, but which are actually desperate attempts for understanding, discussion, answers and explanations – and not least, reassurance. Burns and Zweig (1980) point out that this failure on the part of parents to recognise their child’s awareness and needs at this stage probably parallels their own denial and difficulties in facing up to the possible or imminent death of their child. Apart from increasing their apprehension and fear, this refusal by parents to talk to them about death, may leave these children with the inability to really come to terms with it themselves, and as one child once said to me ‘I can’t say my goodbyes because nobody lets me talk about dying’. Another child – a young teenager – who had had a short but fulfilling lifetime with a major disability, and knew that he was going to die soon, told me, ‘I am ready to die, I want to die, but my parents won’t let me’. Fortunately, following discussions with his parents, they agreed that they didn’t want him to die because they couldn’t face losing him, even though they knew that he was now in so much pain and discomfort, and that it was for their own ‘selfish’ reasons. They agreed to let him die, and went to him to give him ‘permission’ to die. Several hours later he died contentedly, peacefully

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surrounded by all his family, and after having had the opportunity to say his goodbyes to them all.

Conclusion The concept of illness is a very personal concept, one that each of us builds up as we go through life experiencing our own states of ill health and those of others around us. Each person’s experience of illness becomes a very personal narrative – a narrative that is constantly being written and updated as we live. Consequently, our own illness narrative can differ in so many ways from those of our friends and neighbours, or even from those of our own families. Children have an illness narrative that is usually very short and often, to some extent, is very much unformed because their experience of ill health is often not very great. However, there are some children whose illness narratives can be longer and more intensive than the illness narrative of an 80-year-old, because they have experienced very intense ill health at a very young age. Children with leukaemia are one example, as are children with SCID, children who have been in intensive care, children with cystic fibrosis or asthma, or indeed children with any number of chronic diseases. Children with chronic illnesses often learn to develop coping strategies, and one such strategy is ‘denial’. As Hester (1987) found, diabetic children often saw themselves as being well, and illness was not a prominent theme in discussions with them. This led Hester to conclude that children have not internalised illness as part of their self-concept. However, Wichowski and Kubsch (1997) put forward a possible explanation to the phenomenon discovered by Hester (1987). They feel that because of the child’s age, chronic illness or disease will not have had time to have an effect on the child’s own perception of themselves, and if the child’s condition was an hereditary one or was acquired very early in life, then that illness will have always been part of the child’s life, and therefore will have been a stable state that is linked to their perception of themselves. This is important because Wichowski and Kubsch (1997) found that the less perception of their illness that a patient had, then the less compliance to preventative measures and to therapies occurred. This lack of perception of their own illness that children with chronic ill health may have is one of the interesting findings from a study by Male et al. (2000) where they discovered that asthmatic children who presented to hospitals in a hypoxic state tended to perceive themselves as less breathless than did children who were non-hypoxic. This can have serious consequences, because as Male et al. (2000) point out, this could predispose to a future life-threatening attack. Similarly, children with diabetes who lack a perception of their illness and think they are well (as opposed to healthy) may not be able to control the diabetes because they lack compliance with both treatment and prevention strategies. This finding is supported by Goldbeck and Bundschuh (2007) who note that both

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health-related and illness-related cognitive understanding of children with asthma (and of their caregivers) is relevant for patient education and for understanding and complying with the necessary interventions (i.e. treatments). These understandings of ill health and all that surrounds ill health (treatments and modified behaviours, for example) are necessary parts of the experiences that allow the children to develop their own personal illness narratives – if they accept them. For children to develop their personal illness narrative requires some degree of cognitive development so that they can make sense of, and edit, their narrative. Otherwise, the narrative can be only an experiential concept, with no rationalisation or understanding – and this occurs with children who are in neonatal intensive care or those children with SCID who are treated at a very young age. These experiential and unformed narratives may take on so many negative connotations just because of the lack of understanding and rationalisation – in other words they can become real horror stories. Nowhere is this seen more clearly than the feelings and suppositions of many children who feel that they are sick because they have done something naughty. As they get older and become more developed cognitively, they begin to learn that there are causes of ill health that are nothing to do with whether they have misbehaved or not, and whilst their illness can be no less frightening, they are at least able to make some rationalisation of the causes of their illness and not just to blame themselves. With this rationalisation and understanding of the causes of their illness, children can then start to understand why they have to undergo the various medical procedures they experience, or why they have to take medicines, and why there may be restrictions on what they can do. Their personal narrative hopefully thus becomes less horrific and they can enter a stage of acceptance, rather than just anger and/or denial. For some of these children with a life-threatening illness, their personal illness narrative may well come to a sudden stop as they die. Even young children can be aware that they are dying and are prepared to accept it, as long as they have the opportunity to talk about it and to rationalise what is happening now and what is going to happen in the future. It is at this point that many of them reach a brick wall because of the reluctance of members of their family and even of health professionals to discuss the possibilities of death with them. They may have to encounter such strong emotions that culminate in states of denial from their parents and from health professionals. For many children, death is not something to be afraid of – they see it as a stage in their life in which they will still exist, but will no longer have the pain and disability that they had in life. They are also confident that they will one day be reunited with their families. They want to know that they will still be loved, and that they will be missed, but that they will remain a part of the family until they are once more reunited.

7

Stressors and Coping Mechanisms

Introduction This chapter will explore the potential stressors that are associated with any childhood chronic (and acute) illness, although it has to be emphasised that these stressors may not be a problem for every child and family. However, there are many children who will face severe difficulties because of these stressors. Faced with any stressor in life – whether associated with ill health or not – people learn to develop coping strategies to allow them to reduce or overcome the effects of these stressors. Consequently this chapter will also address possible coping strategies. Although some of the stressors and coping strategies are generic and common, many are individual and dependent upon a whole range of external and internal factors. To give some structure to this whole discussion of stressors and coping strategies, several theoretical models have been developed in the past few years to facilitate our understanding of them, and this chapter will explore just a few of them. Many stressors, such as temperature changes, do not necessarily cause a physiological stress response if psychological factors are minimised. Stress itself is not an independent entity but a system of interdependent processes that are moderated by the nature, intensity, and duration of the stressor and the perception, appraisal, and coping efficacy of the affected individual, all of which in turn mediate the psychological and physiological response to stress (McCance & Huether 2002 p. 284). Chronic ill health impinges not only on the physical status of the patient, but also the mental, psychosocial and spiritual well-being of the patient (and the patient’s family – particularly where children are concerned). Consequently an understanding of stress and coping in relation to children with severe combined immunodeficiency (SCID), and indeed with any child who has a serious and/or chronic illness, is of vital importance because valuable insights leading to changes in therapeutic practice can Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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come from a knowledge of how the children cope with their illness, and how parents come to terms with their child’s illness, which in turn will have a bearing on their ability to provide a healthy environment not only for the child, but also for any siblings and other family members (Eiser 1990). According to Varni and Wallander (1988), stressors are difficult situations and events that require appropriate decision-making processes, solutions and actions to relieve, or at least ameliorate, the difficulties. In addition, they believe that coping with the stressors necessitates an individual’s competence, which is defined as the effectiveness of the coping responses to a difficult situation. How an individual is able to develop and utilise coping strategies when faced with difficult situations will determine how that individual will be able to function physically, psychologically and socially. There are many theories which have been put forward in the past concerning the concept of stress and coping, but what exactly do we mean by stress and coping. When discussing the psychosocial effects on children and families of ill health, the terms ‘stress’ and ‘coping’ often are widely used, but as Keil (2004) points out, the significance of these two terms in relation to ill health has changed throughout time, and there continues to be a lack of clarity as to what exactly the meaning of these terms and their practical use may be.

Stress Obviously, coping is a response to stress, but the important thing is to define what we mean by stress, and that will help to define what we mean by coping. Many have defined stress as being a set of conditions that is being coped with (Heth & Somer 2002, Keil 2004, Lazarus & Folkman 1984, Sheu et al. 2002, Tyson et al. 2002), but this leads us to a ‘chicken and egg’ situation, as to a definition. The Shorter Oxford Dictionary (Allen 1991 p. 1206) gives many definitions of stress, depending upon the context, but for the purposes of stress and ill health, there is one that is relevant, namely ‘a demand on physical or mental energy’. Ill health certainly places a demand on both physical and mental energy, and Keil (2004) points out that the use of stress as a form of psychological term linked to both hardship and/or coercion has a long history, being in use from as far back as the fourteenth century. So, in terms of ill health, what is stress? Colloquially, there are two different meanings:

r An unpleasant or potentially harmful external force which places one under pressure.

r One’s response to unpleasant or potentially harmful events – something going on in the body.

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The Biology of Stress and the Stress Response The fundamental purpose of the stress response is to enable organisms to cope swiftly and effectively with life-threatening challenges – the ‘fight or flight’ response. This involves a rapid switch of priorities from longto short-term survival. Biological resources are channelled to systems that might need to cope with imminent challenges. Your body will need extra energy, and quickly. Many of the biological changes that accompany the stress response are designed to mobilise the body’s fuel reserves, convert them into a form suitable for immediate use and transport that fuel, together with the extra oxygen required to burn it, to the organs most likely to need it – mainly the brain and major muscles. This is done at the expense of other biological systems such as growth and reproduction. The physical symptoms of acute stress include:

r r r r r r

a dry mouth sweaty palms pounding heart irritable bladder cold feet stomach pains and flatus

This is an acute/short-term stress response, and it obviously entails a rapid and quite serious change in our physiological state. Short-term stress response is mediated mainly by the sympathetic nervous system. The pulse, blood pressure and respiratory rate are increased to boost the supply of available energy, which leads to the pounding heart. In addition, more blood is pumped around the body with each heartbeat, and the bronchial tubes dilate to allow extra air with each breath. Blood vessels that supply the muscles expand, and the palms of hands and the soles of feet perspire which accounts for the sweaty palms. This means that the surfaces of the palms and soles are moist, possibly to provide less traction to slow the person down when running away from danger. Blood is shunted away from the extremities towards the muscles, heart and brain, whilst the peripheral blood vessels constrict (leading to the cold hands and feet). The pupils of the eye dilate. This lets in more light and improves vision, whilst at the same time, the improved vision and the increased blood flow to the brain means that mental alertness improves, and reaction times speed up. If things become really terrifying, then involuntary urination and defecation can occur – an empty bladder and bowels makes you lighter and more alert. At the same time, biological functions not vital for short-term survival are shut down, whilst long-term energy reserves (in the form of stored fat) are broken down into fatty acids and glycerol, which can be metabolised immediately, and carbohydrates stored in the liver are converted into glucose (for more energy).

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Energy-consuming digestive processes (including the production of saliva) are shut down, which in turn leads to the dry mouth, decreased appetite, churning guts and flatus, mentioned above. Finally, the libido is reduced so that sexual functions are put on hold, because in the short term, they are not a necessity for survival of the individual. The biological mechanisms underlying the stress response are very complex, but there are certain crucial principles:

r The stress response originates in, and is coordinated by, the brain. r Many different parts of the brain play a role in processing all of this information – including the cerebral cortex and other higher centres.

r An important point to mention is that conscious thought need not be

r

involved – hence we do things subconsciously without thinking about them. This saves time, because even a simple thought followed by the action determined by that thought can take up a millisecond, and that millisecond can, in extreme circumstances, be the difference between life and death. When the brain perceives stress, the hypothalamus is activated. The hypothalamus (in the forebrain) is the source of many of the primary electrical and chemical signals that trigger the full stress response.

The Psychological Consequences of Stress So far, we have looked at stress from a biological/physical point of view, but as was mentioned earlier in this chapter, stress has a psychological aspect as well. Scientists use ‘psychological stress’ to encompass both external disturbances and the individual’s response to those disturbances, and according to Martin (1997), we can define psychological stress as the state arising when the individual perceives that the demands placed on them exceed (or threaten to exceed) their capacity to cope, and therefore threaten their well-being. Stress affects us psychologically as well as physically. In particular, stress affects our senses, our memory, our judgement and also our behaviour (Martin 1997). In essence, it changes the way in which we perceive the world – often in a totally different way in which we might perceive it when not stressed. This is linked to the release of a particular stress hormone – cortisol. The release of large amounts of cortisol is caused by being in a very stressful situation, and a high cortisol level reduces our ability to detect weak stimuli in our environment. In other words, it reduces our sensory acuity. All the senses, including taste, smell, hearing and even balance, are affected by high cortisol levels. As Martin (1997 p. 129) explains, ‘someone with a high cortisol level may not be able to detect the presence of, say, a very faint sound, but their ability to tell two slightly different sounds apart will be enhanced’.

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Any stressful situation that anyone finds themselves in can cause damage – that is mental, emotional and physical damage (particularly in terms of post-traumatic stress disorder which will be discussed more fully in Chapters 13 and 14). Similarly, their mental, emotional and physical states also can be drastically altered, either temporarily, or even permanently. So far, much of what has been written in this chapter has given the impression that stress is a negative state, leading towards biological illness, or towards neurosis, or even towards social dysfunction (Dirkzwager et al. 2002, Keil 2004, Shephard 2000), and later chapters in this book will support this impression. However, it is important to redress the balance by pointing out that stress per se is not in itself a negative state, but it can also have positive results. These positive aspects have been implied in the discussion on the acute stress response, namely that it prepares us, in the short term, for the ‘fight or flight’ response. In other words, the physiological changes caused by being in a stressful situation help us to either fight the danger, or flee from the danger. So, undoubtedly, in the short-term stress has a very positive impact – even, at times, a life-saving impact. Whilst in the short term, the psychological consequences of stress are important for our safety under stressful conditions, for example, in a dangerous situation, the ability to be able to ignore all extraneous sensory inputs to concentrate on the life-threatening problem is obviously an advantage. However, when the stressful situation continues for a long time, as in the case of chronic ill health – or even some stressful jobs, then this sensory discrimination can overwhelm us, leading to an inability to concentrate or to think clearly. This is because, as Goleman (2006 p. 268) points out, under stressful conditions ‘the thinking brain gets sidelined for the duration’. As our thinking brain opts out of decision making, we lose the ability to think clearly. Indeed, the more intense the pressure or stress, the more our thought processes and our overall psychological functioning and performance will suffer (Goleman 2006). The greater the anxiety we suffer as a result of stress, the more our cognitive functioning suffers, leaving us poorly able to understand, to work out problems, and to make decisions. As our anxiety increases, so our ability to take in new information and to generate fresh ideas decreases. Focus is lost and motivation disappears. We thus become ‘depressed’ individuals, unable to function at a high level, and often only able to perform the very basic functions – and those not often well, either. Eating and basic hygiene may well suffer, along with our memory, personal interaction, and even sexual desire. Eventually we can end up in a catatonic state, unable to rouse ourselves to perform the most basic of functions to keep us alive. Selye (1978) developed the concept of stress to mean the reaction of the organism to environmental factors, rather than the external factors themselves (Keil 2004). In other words, it is how we react to these external stimuli that determine the amount of stress that we feel, rather than the external stimuli themselves. Although there was initially quite a bit of resistance to this stress theory (Helman 2001, Keil 2004), interestingly, it was taken up by the American military establishment and one of the

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reasons, if not the reason, for this was, as Viner (1999) suggests, that stress was seen by the military as an antidote to fear on the battleground. McCance and Huether (2002 p. 284) explain that ‘extreme physiological stressors, such as severe burn injury, represent a predictable stimulus for the stress responses’. In ill health, obviously the greatest fears are those of dying, disability, pain and discomfort, separation and the unknown. Each of these, or a combination of several of them, can lead to stress. If the illness is of short duration and not too severe, then the stress response can often help the patient to get through without too many ill effects, but if the illness is a severe one, and particularly if it is of long duration, and hence a chronic, or lifelong illness, then the stress response to these fears and environmental challenges can, without appropriate and effective coping mechanisms, exacerbate and prolong the problems. These coping mechanisms are discussed below, but to put it simply, each of us has different coping abilities and coping strategies that we can utilise and bring to stressful situations, so one individual can respond completely differently to another individual who experiences exactly the same stressors, which may not necessarily be intense stressors. Even a less severe and defined occurrence or situation can be a stressor for one person but not for another (McCance & Huether 2002).

Stress and the Immune System Of great importance concerning health is that stress can seriously affect the immune system, particularly if stress is experienced for a long time. Indeed, as Segerstrom and Miller (2004) believe, the most pressing question that needs to be addressed in future research is the extent to which any stressor-induced changes in immunity have meaningful implications for disease and ill health in otherwise healthy humans. They point out that work in the field of psychoneuroimmunology has been going on for the past 30 years, and in that time, many studies have established that stressful experiences do alter features of the immune response, which in turn can cause health problems where the immune system is involved in protecting the body. However, there is still concern that these studies have not yet determined whether these immune system changes are the mechanism through which stressors have increased susceptibility to disease onset. The immune system integrates with other systems during physiological stress. Indeed, many immune-related conditions and diseases are associated with stress, although the specific stress-induced mechanisms that cause these illnesses are not as yet clearly defined (McCance & Huether 2002). As Segerstrom and Miller (2004 p. 617) discuss ‘The immune system, once thought to be autonomous, is now known to respond to signals from many other systems in the body, particularly the nervous system and the endocrine system. As a consequence, environmental events to which the nervous system and endocrine system respond can also elicit responses from the immune system’.

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According to McEwan (2002), the immune system plays an important role in helping us to deal with stress. This is because the immune system needs to get ready to deal with injury to the skin, muscles and tissues, as well as to fight any infections that might result following this injury. He points out that acute stress can enhance the response of the immune system, causing it to send white blood cells to where they are needed. However, he does concede that chronic stress can suppress the immune system, so making the body more prone not only to infectious diseases, but also to inflammatory disorders (e.g. allergies), asthma and autoimmune disorders. To sum up, it is possible to say that, ‘Stress is a system of interdependent processes that are moderated by the nature, intensity and duration of the stressor and the coping efficacy of the affected individual, all of which in turn mediate the psychological and physiological response to stress. Many studies have linked psychological distress with altered immune function, and evidence now strengthens the association of stress with potential for others in humans’ (McCance & Huether 2002 p. 287).

Coping According to Keil (2004), the use of the word ‘coping’ can be problematic, because it refers to a collection of abstract ideas which can range between the affective (i.e. emotional) and behavioural. Keil (2004) also points out that nurses use the term ‘coping’ in different settings for essentially diagnostic purposes, and that the criteria that they have used for this term and for its practical use, have been intuitive rather than systematic. In other words, we can make ‘coping’ mean whatever we want it to mean in any given circumstance. Turning to the Concise Oxford Dictionary for a definition, we find that ‘to cope’ means to manage successfully or to deal with a situation or problem (Allen 1991 p. 254). So, in the context of ill health, coping with the various stressors that are built within that phenomenon of ill health, means to be able to successfully manage the accompanying stressors and to be able to deal with the situation or problems of that ill health. Keil (2004 p. 660) attempts to rationalise the meaning (and practice) of coping when she points out that ‘“coping” implies dealing with adverse or stressful or difficult circumstances, where one can “manage!” an entirely benign situation’. Thus, Keil is arguing that, in terms of semantics, we cope with problems but manage non-problematical situations. A moments thought would show us that Keil has the terminology correct, because whilst we say that we can manage difficult situations and problems, to manage something means to be proactive and to be able to anticipate elements of the situation or problem and therefore we are not really ‘managing’ the difficulties and problems, rather we are ‘coping’ with them, because to cope with something means that one is tending to be reactive, which is more the situation that occurs in relation to ill health. So whilst, in general terminology it is possible to

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say that we are managing the stressors of ill health, in actual fact for most of the time we are really just coping with them. Lazarus and Folkman (1984) point out that there are two major aspects of coping:

r to change the situation for the better (if possible, to solve the problem or difficult situation/event

r to manage the physical and psychological components of stress-related emotions As Bradford (1997) explains, these two aspects (or functions) of coping can be either opposing functions, or they can be supporting functions. In other words, solving the problem and managing the problem can at times be opposite functions, whereas at other times they can support one another. To return to the definition that was made at the beginning of this chapter, namely that coping is a response to stress, the direction of our practice should be on identifying the stressors inherent, or acquired, in the situation of ill health, and then seeking ways in which those stressors can be modified, because as Keil (2004) states, the process of coping needs to contain elements of modification, or even of alteration, which may include either the modification of external factors, or an internal adaptation to them – or even a combination of both. Both these elements are concerned with the reduction of stress, either by the act of modifying, or even removing, external stressors linked to the situation (externalised response), or by being able to accommodate them, to learn to live with them (internalised response), so that whilst the stressors still remain, they have less effect upon the individual than if the individual had not made this accommodation (Keil 2004). Shaw (1999) points out that in practice, most coping strategies, or responses, to stressful situations, such as ill health, will use a combination of these internalised and externalised approaches.

Models of Stress and Coping Over the years, there have been many attempts to produce models that allow us to understand the cycle of stress and coping. The first model was probably the one that was initially developed by Selye (1956) which defines stress in terms of a non-specific response in the body to any demand that is made upon it (Pot-Mees 1989). This model concentrates on the physiological response of the individual, rather than, as we have discussed earlier in this chapter, the physical, psychological, emotional and social responses. Selye’s (1956) model proposed that the physiological stress response of an individual represents a universal pattern of defence reactions which does not depend upon the nature of the stressor (Pot-Mees 1989). It was Mason (1971) who cogently raised the major criticisms against this model, namely the non-specificity of the stress response and, pertinently from what we have already discussed, the psychological impact of the stressor.

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Since Selye’s (1956) model, there have been many models that have been put forward to try and make sense of these phenomena, and a few of these will be briefly discussed below.

Pless and Pinkerton’s Model of Child Adjustment to Chronic Disease (1975) Pless and Pinkerton (1975) developed their model by suggesting that adjustment is a dynamic process which is influenced by feedback loops. These feedback loops demonstrate that the child’s adjustment is influenced by the dynamic interplay of various factors included within the model, such as genetic, social and family factors which help to determine an individual child’s temperament, personality and intelligence. These attributes then interact with the characteristics of the disease and the reactions and attitudes of others who are important to the child. Pless and Pinkerton suggest that it is a combination of all the above that determines a child’s coping style and adaptability to ill health, and that these feedback loops reflect the child’s current life situation along with their past experiences and their past, present and future expectations (Figure 7.1). In this model, the coping responses to illness and the stress of illness are determined by three factors. The first factor involves the family characteristics which feed into the intrinsic attributes or characteristics of the individual child experiencing the stressful event as well as the nature of the stressful event – in which the family characteristics can lessen or exacerbate the stressful event. Similarly, the second factor, which is the social environment within which the individual child experiencing the stressful event (in this case ill health), can have an impact on the intrinsic attributes of the individual as well, like the first factor, as affecting the stressful event itself. Finally, the third factor involves the individual intrinsic attributes Adjustment (normality)

Influences of: Patient illness and/or stress of illness

1. patient experiences 2. family

Coping Abilities and Strategies

Psychological functioning

3. society

Maladjustment (abnormality)

Figure 7.1 Pless and Pinkerton’s (1975) model of child adjustment to chronic disease. Source: Pless and Pinkerton (1975).

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and character of the individual child experiencing the event. All of these three factors impact upon the coping style and coping resources, as well as the self-concept of the individual child (i.e. how he or she sees himself or herself, and their ability to cope). The family and society also have an important part to play in the individual child’s response to the stressful situation, i.e. the illness, because they can quite dramatically influence the coping strategies of the individual, by means of support, explanation and fears and expectations. The coping strategies for the individual child that are made up of all these various aforementioned factors, and that child’s self-concept, and determine the psychological functioning of that child in the wake of his or her experiencing of the stressful situation/illness. The resultant adjustment to the situation, or lack of adjustment to it, depends upon how the child has coped not only with the illness, but with all the resultant family, societal and self-pressures. Throughout this model, Pless and Pinkerton have integrated a timeline, with the time-zero being the actual illness and its stressors, and time-one being the child’s response to illness. However, they take this further, by discussing the idea that, as regards long-term stressful situations (i.e. a chronic illness) functioning in early childhood sets the template for functioning as an adolescent and later as an adult. In their timeline, adolescent functioning as a result of the child’s response to the illness is denoted as time-two, whilst the adult functioning is denoted as time-three. Over time, the loops which feed back to the child are constructed, and the ability of the child to adjust to ill health is the result of the dynamic interplay of all the above factors. Pless and Pinkerton define adjustment as freedom from physical, emotional and psychosocial abnormality. Whilst they also assume that functioning in early childhood determines later adjustment in adolescence and adulthood, Bradford (1997) has a problem with this model because, as he states, the idea that adjustment is an unalterable state cannot be substantiated by various studies that have examined the influence of psychological treatments on the particular difficulties that are associated with chronic disease as well as distress during medical procedures (Jay 1988, La Greca 1988). The concerns of Bradford are also backed up by Rutter (1985) who suggests that the Pless and Pinkerton concept of pathways from childhood to adult adjustment does not stand up to testing. Rutter points out that there are a number of transition points which can affect later outcomes, such as developing social supports (friendships), break up of families etc. A further concern with this model is that, whilst as Bradford (1997) concurs, adjustment to ill health, or any stressful situation, is certainly influenced by self-concept and coping responses, there is, however, difficulty in determining which factors may impact on adjustment, and the degree to which any individual factor can impact psychological functioning. In this, he is supported by Moos and Schaefer (1984) who make the point that the actual coping strategies that people use are in themselves not adaptive or maladaptive, but that it is the skills with which individuals approach, and use, their coping styles and strategies, and the situations in which individual coping strategies are utilised, that need to be taken into

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account. They point out that coping skills may be effective in one situation, but not necessarily so in another. To this, could be added the thought that even the same stressful situation may require different coping strategies at different times, because of the influence of so many intrinsic and extrinsic factors, including age, friendships, family dynamics and environmental changes. This is particularly important in terms of chronic ill health, where an individual may have to live with that ill health for the whole of his or her lifetime. In addition, the magnitude of the stressors involved with chronic ill health may change, and there could well be crisis points throughout the lifetime of that individual when the chronic ill health becomes exacerbated for one reason or another. Whilst this model does allow for changes in adolescent functioning and adult functioning, as the child grows up, it does not appear to allow for these crises in health, so consequently it is possibly too simplistic, because the changes will require different coping strategies at different times.

Lazarus and Folkman’s Model of Stress and Coping (1984) Yet another model to briefly consider is that of Lazarus and Folkman who in 1984 hypothesised that stress and coping continuously interact with each other and involve adjustment between individuals and their environment. Stress occurs when demands on a person exceed resources or coping ability. Coping is defined by them as the problem-solving process which is used by people to manage the discrepancy between demands made and resources available. This model, whilst being first proposed over 20 years ago remains one of the most well-known and quoted models of stress and coping in existence. In the Lazarus and Folkman model (Figure 7.2), there are two major concepts involved in understanding the responses to stress, namely cognitive appraisal and coping. Cognitive appraisal is concerned with assessment of the threat and the resources to cope with that threat. Lazarus and Folkman designate two types of appraisal – primary and secondary. Primary appraisal is a process of perceiving the actual threat involved in the stressful events/situation, and is concerned with assessing whether a particular event is irrelevant, benign or stressful. On the other hand, secondary appraisal, which is a process of considering a response to that threat, determines what resources are available for coping and the likely consequences. The primary appraisal (which is made as to the level and the magnitude of any potentially or actual stressful situation) can determine both the coping mechanisms that need to be called upon in order to counter the situation, as well as the efforts and resources that are needed to be put into these coping responses. If the situation is appraised as being either irrelevant or benign, then often either coping mechanisms are not needed or they are only of a limited magnitude and duration.

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Influence of: personal, family and/or societal coping resources

Seen by patient as:

Stress (illness)

Patient appraisal of stress

1. No problem (positive/benign) 2. Stressful for patient 3. No stress

1. a challenge 2. a threat 3. harm and/or loss occures

Second appraisal – what can be done?

Response to stress

Third appraisal – of response

O U T C O M E

Figure 7.2 Lazarus and Folkman’s (1984) model of stress and coping. Source: Lazarus and Folkman (1984).

However, if the primary appraisal determines that the event is stressful, then, according to this model, there is a further appraisal as to the type and level of stress. Lazarus and Folkman then say that from this further appraisal, three possible scenarios can ensue. The first scenario of harm – loss is an appraisal of the amount of damage (physical, psychological, emotional or social) that has already occurred. The stressful event may be seen as a challenge to be overcome (a positive response), and is seen as the opportunity to achieve growth and mastery in spite of, or because of, the stressful event/situation. Alternatively it may be seen as a threat (a negative response) leading to the expectation of future harm (Bradford 1997). Once the primary appraisal process is complete, and the appropriate scenario has been selected, a secondary appraisal takes place in which potential appropriate coping mechanisms are appraised and a coping strategy can be employed. Again, Lazarus and Folkman distinguish two major factors of coping, namely solving or managing the situation. These two factors can oppose or support each other. The model also designates five categories of coping resources. 1. 2. 3. 4. 5.

Utilitarian, e.g. socio-economic status Social networks Health, energy or morale, e.g. pre-existing physical or psychiatric illness General and specific beliefs, e.g. self-esteem Problem-solving skills, e.g. intellectual ability, analytic skills

The first two categories of coping resources can be lumped together, according to Lazarus and Folkman, as socio-ecological coping resources, whilst the latter three categories, can be combined under personal coping resources. Once the coping mechanisms are in place, then a tertiary appraisal takes place – this time of the effectiveness of the coping strategies employed. This will determine the outcome of the stress and coping experienced. This is a cyclical model in which the outcome of the response to a stressful situation can determine the levels of stress within that stressful

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situation, and hence can exacerbate or ameliorate the actual situation. So, if the individual is able to utilise all his or her coping resources to their maximum, then the actual stressful event or situation can, following a further primary appraisal, be downgraded to an irrelevant or, perhaps most likely, a benign event or situation. If the coping resources of an individual are not sufficient to manage the initial stressful event or situation, then rather than downgrading this event, the situation becomes more exacerbated, and a downward spiral of ever increasing stress levels and ever decreasing coping resources can occur. The beauty of the Lazarus and Folkman model is its simplicity, whilst still managing to encompass the major events in the coping process. However, a fault with it is that it is not predictive, but then no model can really be predictive because of the complexity of humans.

Stress Caused by Ill Health that Is Both Chronic and Acute In relation to children with SCID, as well as all children who have illnesses that are chronic with acute exacerbations, or that are initially acute that may lead on to a chronic condition; the challenge is to produce a model that encompasses both chronic and acute ill health. The theoretical model used as a framework for the study of children with SCID and their families is based on two existing models (Vickers 1999b). The first is put forward by Wallander et al. (1989) which is well reviewed by Bradford (1997), whilst the second is put forward by Pot-Mees (1989), which itself is based upon work by Lazarus (1976) and later incorporated into the model by Lazarus and Folkman in 1984. The decision not to just use one of the models reviewed above is that they deal with chronic ill health and I wanted to produce a model that catered for both acute and chronic ill health in the same person. The theoretical model used in the Vickers (1999b) study places particular emphasis on the role of stressors and the ability of the individual to manage them, i.e. the responses. Varni and Wallander (1988 p. 215) define stressors as events in which ‘problematic situations requiring a solution or some decision-making process for appropriate action’ occur. Responses to these stressors occur when a person is confronted with the stressors. An example of stressors and (admittedly negative) responses to bone marrow transplantation was presented by Popkin and Moldow in 1977. These are: Stressors:

r r r r

r

Decision for bone marrow transplantation The disease itself ‘Illness’ syndrome Environmental stressors e.g., restricted physical contact highly regimented routine intense nursing care Physical stressors e.g. major weight loss

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r r r

fever sepsis bleeding abscess formation alopaecia lassitude sleep dysfunction anorexia The transplant procedure Fears of failure, rejection, graft-versus-host disease Fears of death

Responses:

r r r r r r r r r

High anxiety Phobic preoccupations Intrusive thoughts and feelings Regressive behaviours Emotional lability Despondency/depression Guilt Denial Ritualised obsessional patterns

It must be pointed out that the responses to stressors may not necessarily be all negative as there are positive responses which may ensue, such as acceptance, compensatory attitudes and behaviours, positive aggression and optimism.

Pot-Mees’s Model of Stress and Coping (1989) Pot-Mees (1989) developed a theoretical model (Figure 7.3) to underpin her research into the psychosocial effects of bone marrow transplantation in children. This model was based to a large extent on the work of Lazarus (1976) in which there is a continuous interaction between the individual and his environment, and in which an imbalance in this interaction gives rise to stress in an individual. Lazarus (1976), supported by Pot-Mees (1989), identifies three key concepts which can be said to be influential psychological processes in an individual’s response to stress, namely cognitive appraisal, coping and social support. By cognitive appraisal, Pot-Mees (1989) is referring to the cognitive activities, such as judgement and evaluations, which can be used to interpret and to guide the interaction with a stressful event or situation. The first point to make here is that whether or not the situation is actually experienced as threatening, depends on the way that the person perceives the event, and also whether that person believes that he or she has the coping ability to deal with it.

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Coping is the second of these three processes identified by Lazarus, and this, as can be seen in Figure 7.2, is referring to the efforts made by the individual child to master conditions of harm, threat or challenge (Lazarus 1982). The third of these processes is social support, and according to Lazarus and Cohen (1977) social support has an important role to play in coping with stress. Pot-Mees (1989), Bloom (1982) and Cobb (1976) have all demonstrated that the presence of a social support system and a social network can help when dealing with crisis situations. Pot-Mees (1989 p. 23) points out that ‘a social network can be described as a person’s framework of different relations (friends, colleagues, relatives etc.), who provide different types of social support’. It is both the quantity and the quality of the support relationships that are important (McFarlane et al. 1984, Morgan et al. 1984). As Pot-Mees (1989) reports, it is, as expected, the parents who are unstinting in giving close emotional and social support to their child. Although, as anyone who has worked with families and children can testify, this is not always the case – although it usually is. However, the nature and the quality of the support given and received depend on the age of the child and also on the emotional relationship that exists between the child and his or her parents, and possibly upon the ‘emotional age’ of the parents. In terms of chronic illness, there are said to be two aspects of support that parents give that are particularly important. These are the quality of communication between the parent of a child, and the effect which parental mental health can have on the child’s ability to cope. This is important because parents can find it very difficult to maintain their normal parenting skills following the diagnosis of their child’s lifethreatening disorder, because this makes it very difficult to treat the child as normal (Lavigne & Burns 1987, Pot-Mees 1989). Eiser (1985) has reported that the previous parent–child relationship may become affected, and even broken, by the parents’ reaction to such a diagnosis, leading to parental over-indulgence and over-protectiveness, feelings of hostility,

CHILD Biographic

Stressor (BMT)

Medical-diagnostic

APPRAISAL

Hospital

School

Personality

RESPONSE

Imbalance = Stress

FAMILY

Social environment

Figure 7.3 Pot-Mees’s model of stress and coping (1989) – simplified version. Source: The Psychosocial Effects of Bone Marrow Transplantation in Children 1989. Adapted by permission of Eburon Academic Publishers.

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rejection, depression or even a disruption in attachment (see Chapter 12). Again, this is important, because, as Freeman (1968) has suggested, when parents have a positive and accepting approach to their child, following a diagnosis of a life-threatening illness, their child is more likely to adjust well to chronic illness (Pot-Mees 1989). Similarly, as Rutter (1981) points out, parents who are functioning well themselves have a better ability to support their child through such stressful situations, than parents who are themselves struggling to function normally. In all this discussion about parents supporting their children, it must not be forgotten that extra-familial relationships (i.e. from outside of the family) can also be very important for these children, not least by supporting the family, to enable them to support their child better (Dow 1965) – although the children’s friends, as well as outside agencies can do much to help the child to cope with both acute and chronic ill health. Within this model, there are many variables that have been identified that will feed into the child’s ability to appraise the stressful situation, in this case having a bone marrow transplant for a life-threatening disease (Pot-Mees 1989). Under biographic variables can be included:

r Age and gender of the child – age is important, because it is often linked

r

r

to cognitive and emotional developmental level, which in turn determines how much the child will understand of the illness, and also the ability to cope with it (Erikson 1956, Pot-Mees 1989). In addition, Rutter (1970, 1981) has suggested that boys are more vulnerable in their response to most types of stress, than are girls. Intelligence – intelligence plays an important part, along with cognitive development, on the understanding that any child has of their illness and the treatment regimens that he or she will have to undergo. Shure and Spivack (1979) suggest that children of above average intelligence have greater problem-solving capabilities. This may mean that they have possibly better coping strategies. Place of residence at the child/family – Rutter (1973) and Rutter et al. 1975a, 1975b) have suggested that children in inner cities are more risk of developing behavioural problems than children from rural areas (PotMees 1989).

Medical-diagnostic variables include:

r The diagnosis – the illness that the child has that is causing him or her to have a bone marrow transplant, e.g. leukaemia, SCID. Many reports have suggested that children who have chronic ill health have a greater risk of developing behavioural and emotional problems than do children without such chronic ill health (Roghmann & Haggerty 1970, Rutter et al. 1970). Stubblefield (1974) posits that one of the explanations is that chronic illness undermines normal development of the child and also reduces the child’s sense of self-worth which can impact upon the child’s ability to attain independence and achievement. Against this,

Stressors and Coping Mechanisms

r r

r

117

several reports show that chronically ill children are not very different from normal healthy children in their development (Kellerman et al. 1980, Tavormina et al. 1976). In addition, certain diseases and illnesses necessitate frequent and painful therapies over a long time, along with a slow decline, and this can adversely affect the ability of the child to cope with their illness. Age of the child at the time of diagnosis – Douglas (1975) and Quinton and Rutter (1976) have suggested that children who are diagnosed at a young age have problems with psychosocial adjustment in later life. The interval of time that has elapsed since the diagnosis before the bone marrow transplant is attempted – the effects of a long drawn out illness, with many hospitalisations and treatments can interfere with the educational and social life of children, so causing them behavioural problems and problems with coping. The number and duration of previous hospitalisations – a report by Quinton and Rutter (1976) suggested that repeated and lengthy hospitalisations could have a possible negative effect on later psychosocial adjustment.

Variables linked to personality consist of:

r The child’s self-concept – according to Coopersmith (1967) self-perception

r r

r

refers to one’s personal judgement of self-worth, and is expressed in the attitudes and opinions that the person has towards himself or herself. Ill health, particularly chronic or life-threatening illnesses, can have a negative effect on this self-concept of the child (Pless & Pinkerton 1975), whilst a positive self-concept is seen as essential in establishing personal happiness and effectiveness in life (Pot-Mees 1989). The psychological state of the child – if there are present or past psychological problems, then children can have difficulties in dealing with present and future stressful events (Brown & Kelly 1976). The number of occurrences of stressful events in the child’s life to date – there is some doubt as to the influence of these on the child’s ability to cope. Some reports suggest that they make it difficult for the child to cope (Pless & Pinkerton 1975), whilst others suggest that they help to make the child more resilient (Rutter 1980). The child’s own coping strategies – whether they are effective or not, whether they are positive or negative and their influence on the child’s psychological, emotional and social behaviour.

The stressors that have been identified that accompany bone marrow transplantation are (Pot-Mees 1989, Vickers 1999b):

r Pain and physical discomfort – the intensity of pain and physical discomr r

fort attached to the illness can have a detrimental effect on the child’s ability to cope. Psychological adjustment – if the child is psychologically well adjusted prior to the illness, than they will often be able to cope better than a child who had psychological problems. Conditioning for the bone marrow transplant, including total body irradiation and cytotoxic chemotherapy – causes pain and discomfort.

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r The degree of acute or chronic graft-versus-host disease – again linked with pain and discomfort.

r The length of time spent in hospital, and the duration and degree of isolation – r

can affect the psychosocial and development behaviour of the child, leading to problems such as depression, anger and alienation. Facing death – during the time of the bone marrow transplant procedure (including the conditioning regimen), and afterwards if the procedure does not work.

The family variables may well include: Demographic Factors:

r Size and gender makeup of the family – it has been suggested by Rutter r r r r r

et al. (1970) that family size and birth order can have an effect on the ability of the child to psychologically adjust to the illness Birth order of the child requiring a bone marrow transplant within the family Occupation of the father (and these days, of the mother as well) – McGee et al. (1984) Socio-economic status of the family – McGee et al. (1984) The age of the parents – McGee et al. (1984) The psychiatric history (if any) of members of the family – Christ and Adams (1984)

Parental Functioning:

r The mental state of the parents – Weissman and Prusoff (1984) and Richman et al. (1975)

r Marital relationships – Barbarin et al. (1985) Coping Aspects:

r The locus of control – the locus of control is the way in which people

r r

believe that they have influence on the outcome of events in their lives, and this shapes how they will perceive and cope with stressful events (Pot-Mees 1989, Rotter 1966) Coping styles and strategies Amount and type of social support

The Pot-Mees 1989 model is an excellent model for the acute stage of an illness, but as many acute illnesses can develop into chronic (either physical or psychological) ill health, then a study of these conditions and the effects caused by a chronic condition require a broader framework. For example, after the highly stressful situation of the actual bone marrow transplant for SCID, there then follow years of worry and care for the families and children as they continue to cope with the aftermath of the transplant. This appears to be so much the case that it was the contention of the study that forms the focus of this book (Vickers 1999b) that, far from being just an acute disorder, SCID can lead to chronic ill health. Consequently, it is necessary to look at a model that will take into account the stressors (and

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hence the coping strategies) that are inherent in chronic ill health – such a model is the one put forwards by Wallander et al. (1989).

Wallander et al .’s Model of Child and Maternal Adaptation to Chronic Conditions (1989) Adaptation to chronic ill health is well covered by the 1989 model of Wallander et al. (Figure 7.4). This model offers an explanation of how a combination of disease factors linked with the independence of the child (i.e. in terms of mobility, development, thought, bladder control etc.) and psychosocial stress (both those as a result of the disease and those outside the parameters of the disease) make up the risk factors. Seven variables are considered by Wallander et al. (1989) to be particularly important. These are:

r r r r r r r

the child’s diagnosis severity of the handicap occurrence of medical problems visibility of the disorder lack of bladder and bowel control whether or not cognitive functioning is impaired whether or not the condition is associated with neuropathology

There are two major classifications of resistance factors – intrapersonal and socio-ecological which contribute to the coping abilities of the child and family. It is the balance and interplay between these risk factors and resistance factors which underpin the stress processing ability of the child, namely the cognitive appraisal and subsequent coping strategies. Finally, the risk factors, resistance factors and stress processing factors intertwine RISK FACTORS Disease/Disability parameters Functional independence Psychosocial stresses

RESISTANCE FACTORS Intrapersonal factors

Socio-ecological factors Stress processing

ADAPTATION

Figure 7.4 Wallander et al.’s (1989) model of child and maternal adaptation to chronic conditions – simplified version. Source: Journal of pediatric Psychology 1989, by permission of Oxford University Press.

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to determine the child’s and the family’s adaptive capabilities in terms of mental and physical health and social functioning. Regarding risk factors in the model above (Figure 7.4), risk factors that are included by Wallander et al. as comprising the disease and disability parameters are:

r r r r r r r r

diagnosis severity of any disability medical problems any bladder and/or bowel incontinence the visibility of any disabilities or of the illness – for example, skin problems, facial disfigurements etc. motor functional problems cognitive functioning neurological involvement

At the same time, the risk factors that can be included under psychosocial stressors include:

r disability related problems r major life events that can impinge on psychosocial function r the everyday, often minor, hassles encountered by the child Turning to the resistance factors in the above model by Wallander et al., under intrapersonal factors can be included:

r r r r

the child’s temperament the child’s competence the child’s problem-solving skills the child’s motivation

And under socio-ecological factors can be considered:

r the family environment r social support to the child (and the family) r the effectiveness, or otherwise, of the family members adaptation to the child’s chronic illness

r utilitarian resources that are available for the family to access – and of course their willingness (and ability) to access them Finally, successful adaptation in this model consists of:

r good mental health r successful functioning within the family and also within society at large r optimum physical health In this model (Figure 7.4), there are three variables that act as risk factors, namely disease and disability, the child’s level of functional independence and psychosocial stressors. However, the impact of these three variables on the child’s ultimate adaptation to his or her chronic ill health can be moderated by three variables of resistance factors, namely the child’s intrapersonal functioning, the socio-ecological factors that involve both the

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family and society and the stress processes (or coping resources such as cognitive appraisal and coping strategies). According to Bradford (1997 p. 143), Wallander et al.’s model attempts to ‘integrate the above ideas into a unified conceptual theory of adaptation to chronic illness and handicap’. One major assumption of this model is that having a chronically sick or handicapped child may not necessarily mean there are adverse consequences for the family, but rather this situation may lead to an increased number of potentially stressful situations which may lead to adverse consequences, but not necessarily so if they are successfully managed. Wallander et al. (1989) themselves argue that their model can be used with children who have any chronic disorder. In fact, they believe that there are commonalities between illnesses that, despite different physical signs and symptoms, are greater than any differences between them in terms of stressors and coping strategies to deal with the stressors. They conclude that children and their families face common challenges in trying to achieve a successful adaptation to their chronic disease, not matter what the particular disease is (Bradford 1997). Bradford (1997) argues that Wallander et al.’s 1989 model offers a much more complex and detailed explanation of parenting difficulties as compared to those models mentioned previously in this chapter, with the exception of the one by Pot-Mees (1989). Whilst borrowing several concepts from these previous models, Wallander et al.’s model clarifies much more the interdependence of risk and resistance factors and how they operate. In addition, they contend that it is a consequence of having a child with a chronic disorder that causes families to be presented with an increasing number of potentially stressful situations. If not successfully managed, then these situations can lead to problems for the family, both within the family and in society. Bradford (1997) proceeds by stating that this model is much more multi-factorial because it includes concepts not included in the other models, and he also argues that, because of this, it presents the responses to stress as being something more than coping skills or a child’s self-concept. In Bradford’s words, ‘This model represents the most up-todate and, some would say, the most sophisticated and coherent, theoretical framework to emerge so far’ Bradford (1997 p. 146). In spite of Bradford’s glowing quote above, following Wallander et al.’s testing of their own model, Bradford (1997) states that there are indications that several of the predictions made by this model have totally, or partially, been unsubstantiated, and there is a need to reformulate the model in order to strengthen its predictive validity. However, it has to be acknowledged that no one model can completely satisfy all the elements that are inherent in an appreciation of the stressors and coping strategies linked to acute and chronic ill health. Even with that caveat, this model does allow us to develop some understanding of what children and their families experience in situations of ill health, what coping strategies they may be able to utilise in dealing with the stressors, and also, perhaps more importantly from our point of view, what we can do to aid the children and their families in their attempts to cope with the multifaceted physical, psychological and social ramifications of their disease.

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The Theoretical Model Developed from the 1999 SCID Study As briefly discussed earlier, because the nature of SCID and bone marrow transplantation is now seen to be a series of initially acutely stressful events, followed by chronic ill health, as demonstrated in this study, it was felt that by adapting and combining Pot-Mees’s (1989) model (dealing with the more acute relatively short-term stressful situation) and Wallander et al.’s (1989) model (dealing with the stress that is induced by longerterm, chronic ill health) a theoretical model could be produced to fit the experience that children with SCID and their families initially undergo. Bone marrow transplantation is a major traumatic event in which many of the stressors and responses as described by Popkin and Moldow (1977) are in evidence. Working through, and coping with, these form a major part of Pot-Mees’s (1989) model, whilst the subsequent chronic ill health stressors are dealt with in Wallander et al.’s (1989) model. This adaptation (Figure 7.5) takes the essential elements from the two models (Pot-Mees 1989 and Wallander et al. 1989.) and combines them so that the effects of both an initial major stressful event, plus the effects of subsequent chronic stressors are taken into account. Whilst there is interaction between the two types of event, each can be viewed as a discrete entity, the influence of which is dependent upon the extent to which each is seen as a problem and the perceived dominance of one situation over the other. In the Vickers (1999b) model, the child and the family are, to a large extent, integrated, because of the age of the children. Whatever stressor affects one, affects the other. This is particularly so in the chronic situation CHRONIC

ACUTE Feedback loop

Family stressors

Family stressors Family variables

Appraisal and response

Family variables

CHILD FAMILY

Child variables

Appraisal and coping strategies

ADAPTATION

Child variables

Child stressors

Child stressors Time line ACUTE ILLNESS

CHRONIC ILL HEALTH

Figure 7.5 Model of child and family adaptation to acute and subsequent chronic ill health (Vickers 1999b).

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because of the age of the child and the dependence of the child upon the family. The stressors and the factors which help to determine the response to these stressors feedback to one another. For example, the occurrence of crises (stressful events) can affect the fears of the family, and hence their appraisal of the situation and their response to it. At the same time, these fears can affect the ability of the family to accept professional support, whilst separation in an isolation unit, or even in a hospital if the family have difficulties visiting because of distance and work commitments, can affect the amount of family support available. In this model, the stressors and variables are similar to those for the PotMees (1989) and Wallander et al. (1989) models, although the inclusion of the family into the model for reasons discussed above, namely the age and dependency of the children, means that there are adult stressors that do not impinge upon the child. For example, there are fears that the child may not, because of age, experience, knowledge, cognitive ability, or emotional faculty, be aware of. Examples of adult stressors include:

r r r r r r r

marital state fear of relapse and dying support from others than parents genetic concerns in some diseases financial concerns concerns over other children in the family – family needs effects – physical, emotional and psychosocial – of caring for the child

There are also the stressors that siblings of the other child may face, such as:

r r r r r r

separation from parents and sick child uncertainties as to outcome of the illness affecting their sibling schooling concerns how the illness affects them fears about how they are/will be affected (e.g. genetics) possible social stigma

In this model (Figure 7.5), there is also a two-way affect and feedback mechanism between the responses to stress and the factors, both individual and social/environmental. Psychological responses, for example, can feed back to affect the coping styles available to the individual, or even the family support available or acceptable. Once the acute crisis is over (in the case of the children in the SCID study, once they have a functioning immune system following a successful bone marrow transplant), then the stressors of chronic ill health begin to come into play. However, the influence of the stressors associated with chronic ill health is dependent to some extent upon the responses that were made to the acute crisis. Because of the long-term nature of chronic ill health, as well as the fact that the child and family are now no longer physically, medically or emotionally separated by barriers, there is much more interaction and cross-influence between those stressors and factors affecting the child and those affecting the family.

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Both appraisal and coping strategies are dependent upon the stressors and factors which affect the child and family, but the coping strategies and appraisal techniques used can, in turn, affect some of the factors, such as motivation and problem-solving skills. Finally, adaptation by the child and family, using the parameters of physical health, mental health and social functioning, as the end result of all these stressors and factors, is also dependent upon the stressors, factors, appraisal and responses to the acute crisis, to a lesser or greater degree. Thus, for children with SCID and their families, the process leading to adaptation to chronic ill health is a very complicated one, with so many variables involved, and there needs to be a universal acknowledgement that the child may develop chronic ill health. This model also takes into account the fact that for the many acute illnesses that develop into chronic illnesses (as well as for the many primary chronic illnesses) the chronic state is not always a stable state. Throughout the course of a chronic disease, there could be many acute exacerbations, for example children with cancer could have relapses, children with diabetes could develop diabetic ketoacidosis and become very sick indeed, and children with asthma can have acute attacks which become emergencies. When an acute crisis occurs within the course of a chronic disease, then the stressors and the coping strategies needed to deal with these stressors revert to those of an acute ill health episode. Consequently, in this model, the timeline does not continue to progress forward in an unbroken and unchanging way, but rather can reverse backwards to an acute stage. Once that acute stage has passed, then the timeline moves forward again until such time as another acute episode occurs. Thus, the stressors involved in a chronic illness can continually be changing, as can the coping strategies required by the child and family, and all those who work with the children and their families should be aware of this and be able to provide the necessary treatment and support as and when required. The importance of this model lies in the health professionals being aware of the potential stressors faced by the individual children and their families and by means of forward planning (being proactive rather than reactive) helping the child and family to find within themselves any externally positive coping strategies that they can utilise to minimise the effects of the stressors on themselves and on others within their social and physical environment, such as family and friends. Given that children with chronic disorders also have moments of crisis and acute exacerbations, the model can allow for the stressors to be anticipated during these crises, and so coping strategies can also be planned and put into place ready for these stressors.

Coping with Chronic Ill Health – Children Previously in this chapter, mention was made of the theory by Wallander et al. (1989) that chronic diseases have many commonalities in terms of

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their stressors, and this theory was supported in a study by Spirito et al. (1995) who found in their study of children and adolescents with different chronic illnesses of varying severity that the chronic illness itself did not determine the identification of a disease-related problem, but that the chronically ill children in the study encountered a common set of diseaserelated problems that, to them, were more important than disease-specific characteristics. Non-painful, disease-related stressors were the problems most commonly reported by the chronically ill adolescents in the Spirito et al. (1995) study. In terms of coping strategies, Spirito et al. (1995) found that it was the adolescents in their study who tended to use resignation as a coping strategy, and the younger children who tended to use ‘blaming of others’. This finding echoed the finding by Gil et al. (1991) that passive adherence was used frequently by all the children. Spirito et al. (1995) also found that gender-affected coping strategies in the children and adolescents, with boys reporting using cognitive restructuring and self-blame more than the girls as a way of coping whilst the girls reported using emotional social support more than the boys. A further message that came from this study by Spirito et al. (1995) was that in crises, or highly stressful situations, it is best to reinforce the child’s preferred coping style rather than to attempt to change to other coping strategies. Van De Ven et al. (2007) also looked at adolescents – in their study they concerned themselves with the role of coping strategies in the quality of life of these adolescents who had asthma. Previous reports had demonstrated that increased asthma severity is associated with poorer quality of life in children and adolescents (Sawyer et al. 2001, Somerville et al. 2004, Warschburger et al. 2004). In their study, Van De Ven et al. (2007) found that the most reported coping strategy of adolescents with asthma was a positive reappraisal of their condition (an active and positive coping strategy), followed by hiding the asthma and ignoring the asthma (two much more passive and negative coping strategies). As previous studies have found, there were gender differences in coping, with girls tending to use all coping strategies more often than the boys. This supports a study by Wilson et al. (2005) which demonstrated that female adolescents in their study also used all coping styles (avoiding coping, problem-focus coping, and emotional coping) more often than did their male counterparts. Van De Ven et al. (2007) also reported further coping strategies from this group of adolescents, namely:

r leading a restricted lifestyle (reduces quality of life) r worrying about asthma, particularly during and after a severe asthma attack (reduces quality of life)

r information seeking (improves quality of life) Non-compliance is yet another (negative) coping strategy particularly favoured by adolescents (Eiser 1993). There are many reasons for this, but, outside of the results of hormonal and physiological changes, the need for them to take charge of their own illness, and to be treated as an

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equal partner in managing their chronic disease is a major reason for this. This finding is supported by research by Bossert (1994). Chesler and Barbarin (1987), in their book on coping and stresses linked to cancer and families, identified a wide range of stressors that are associated with childhood cancer. These included the:

r understanding of the diagnosis, prognosis and the treatment r adapting to the treatment and the side effects that are caused by the treatment

r interacting and relating with medical and nursing staff (and also paramedical and support staff with whom they may come into contact)

r maintaining all present relationships as well as forging new ones with their family and peers

r dealing with two worlds, rather than the one that they had inhabited previously. Chesler and Barbarin (1987) note that these two worlds are illness/specialness and health/normalcy So, as Koocher and O’Malley (1981) explain, children with cancer have to cope not only with the many and varied cancer-related stressors, but they also have to deal at the same time with the demands of everyday stressors – both major and minor. In a very interesting study by Bull and Drotar (1991), children with cancer were asked about the stressors and coping strategies they used. In terms of stressors, these were divided into two types, cancer-related stressors and non-cancer-related stressors. There were seven cancer-related stressors that were described by the children, and in descending order of report by the children, they were: Stressor r Handicap r Treatment r General r Self-concept r Family r Peer r School

Number of children reporting 8 6 6 4 1 1 1

There were 13 non-cancer-related stressors

r r r r r r r r r r r

School Sibling Parent Peer Social Worries Work Death Illness (not cancer) Miscellaneous Sports

38 20 18 17 14 11 9 8 8 7 7

Stressors and Coping Mechanisms

r Self-concept r Trauma

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2 2

What is particularly striking about this are the relatively few reports of cancer-related stressors (27) as opposed to the number of reports of noncancer-related stressors (161). This again supports the theory by Wallander et al. (1989) regarding the commonality of disease stressors, if so few of the stressors are disease-specific as in this case. There may be a fatalism (manifesting as an acceptance) in the children to there being nothing that the children can do about their cancer, but just hope that the doctors can sort it out. What does really stress out the children, however, are day-today, routine concerns. Whilst these would be regarded by many as minor concerns, to the children they are concerns and worries that they can, in some way, influence and try to have put right. Again it is down to management of situations, and possibly feelings of control. The children accept that they cannot control the cancer – that is left to somebody else (or to God) – but they can try to have a measure of control over the daily routines of their lives. The high number of reports identifying school, siblings, parents and peers, as stressors may be because they see these people (including the people who are represented by ‘school’) as somehow not accepting the children as themselves, but rather as ‘sick’ or even ‘dying’ children, and therefore they do not behave ‘normally’ with the child with cancer as they used to do. The children with cancer may then be grieving for the ‘lost/changed’ relationships and ‘lost/changed’ lives. In the same study (Bull & Drotar 1991), five coping strategies are reported. These are:

r Information seeking – not just about the cancer, but also about why the r r

r

r

cancer is affecting their social life. Again, there seems to be an element of trying to maintain some form of control over their own lives. Support seeking – this is linked with changes of relationship with peers and family. They need to feel that they have the support of others around them to try to live as normal a life as possible. Direct action – again, taking control over their lives. Accepting the changes but seeking ways to minimise the effects on them and on others, for example wearing a hat to hide the loss of hair due to the cancer treatment. Inhibition of action – trying to minimise the effects of other people’s behaviour and of painful or debilitating treatments by becoming passive, and just waiting for something to happen, or for the procedure to be over as quickly as possible. Intrapsychic – internalising the situation and peoples’ responses to them and their cancer.

Turning now to age, Bull and Drotar (1991) found that compared with the school-age children, the adolescents in their study used more emotionmanagement and less problem-solving coping strategies when faced with the cancer-related stressors than they did when faced with the non-cancerrelated stressors.

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With reference to gender, Bull and Drotar (1991) found differences between the coping styles of the boys and those of the girls, with the girls using more emotion-management and less problem-solving strategies than did the boys when coping with cancer-related stressors, although the caveat for both these findings must be the small number of reports of cancerrelated stressors. It was Lazarus and Folkman (1984) who distinguished these two major functions of coping, namely problem-focused and emotion-focused. Problem-focused coping has been said to include strategies that are directed at changing the environment or at changing one’s own behaviour, whilst emotion-focused coping has been defined as the efforts made to regulate (i.e. to decrease or increase) emotional states that are caused by, or are related to, stressful events. In addition, emotion-focused coping involves changing one’s perception of a situation without altering the situation itself (Bull & Drotar 1991, Compas et al. 1988, Lazarus & Folkman 1984).

Conclusion A child’s exposure to multiple stressors, namely stressors associated with their chronic or acute disease as well as general stressors and life stressors, places incredible and almost unimaginable demands on the child (and also on the family). To survive, and to maintain something approaching a normal life, requires the child and family to dig deep into their reserves of strength and internal qualities in order to come up with coping strategies that allow them to deal with the stressors. As Eiser (1990) points out, parents caring for a chronically sick child take on an extremely demanding role – a role that is demanding both physically and emotionally. But the way in which the family copes with these difficulties, will also have a bearing on how the child copes with these difficulties and responds to the illness and all the stressors that are linked with the illness and the treatment of that illness. This chapter has explored models of stress and coping, as well as looking at stress and coping in children with chronic and/or life-threatening illnesses. Whilst not an exhaustive review, it does identify what people who care for the children and their families need to be aware of in terms of the stressors that they are experiencing and what possible coping strategies can be brought to bear on those stressors. The child and the family require much support, but the support has to be informed support, so it is important for the professionals caring for them to ascertain what stressors they are experiencing and how best they feel they can cope with them. Particularly, it is important to identify those who are not coping well with the stressors, or who are using negative coping strategies, because these situations will only lead to greater problems in later years.

8

Psychosocial Effects on the Children

Introduction She makes a ‘poker-face’. She attempts to show no feelings, although I believe she is often in much torment because she is so small. (Mother of German child) The theoretical perspective of the study was concerned with stress and coping in children and their families following a diagnosis of severe combined immunodeficiency syndrome (SCID) and treatment by means of bone marrow transplantation, as well as the stresses and coping abilities associated with the long-term after-effects. As has been discussed, stressors that occur at the time of treatment include those of hospitalisation and isolation. As Pot-Mees (1989 p. 14) wrote concerning the effects of strict isolation, ‘It has been noted that this may lead to psychological disturbance, due to the reduction of social contacts and sensory stimulation. Psychological reactions include anxiety, depression, extreme dependency, inhibition of motor activity, sleep disturbance and change in play. Particular risks are noted for children under 4 years of age as they are not yet able to generate their own sensory stimuli’. Andrykowski (1994 p. 364) in his review of the psychosocial factors in bone marrow transplantation observes that ‘. . . some BMT patients do “better” or “poorer” than might have been anticipated solely from consideration of disease and treatment-variables’. Following this statement, Andrykowski states that he feels that the contribution of psychosocial factors such as personality, confidence, social support, mood or coping styles is worthy of further examination. Pot-Mees (1989 p. 74) reported that 12 months post-bone marrow transplantation, 21 out of 26 children (80.8%) in her study were ‘. . . physically fit, experienced no limitation and were able to participate in normal activities Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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with friends and siblings’. She went on to report, however, that there were psychosocial problems in evidence. Twelve months post-transplant, the children in her study had significantly more problems in concentrating than before their transplant, and there was also a significant increase in anxiety levels. In addition, compared to the matched group of normal, healthy children, the bone marrow transplant (BMT) children showed ‘. . . significantly more neurotic and depressive behaviour, indicating problems in settling, fussiness and overparticular behaviour. They had more anxieties and problems with their peer group, also in the school situation’ (Pot-Mees 1989 p. 95). These findings were also borne out by those of Leigh et al. (1995), who, in their study of adult BMT survivors, found that significant psychosocial morbidity was present six to nine months post-transplant. The major problems encountered with these adults were neurotic depression and anxiety. In her study, Pot-Mees (1989) points out that depressive symptoms, which were suggestive of post-traumatic stress disorder, were the most prominent behavioural problems at six months post-transplant. In addition, there was an increase in the frequency of temper tantrums as well as eating problems, again at six months. However, by the 12-month posttransplant stage (when her study finished), these problems had decreased, with the children increasingly reintegrating into normal life. The effects of early hospitalisation, isolation and bone marrow transplantation on children are more fully explored in Chapters 4 and 5, in which all these aspects are well documented, but not in so far as children with SCID are concerned. A major theme of the Vickers (1999b) study was that SCID, far from being just an acute disorder requiring early hospitalisation, isolation and bone marrow transplantation, could also be perceived as a chronic disorder, as evidenced by the ill health, disabilities and other physical factors that many of the children in the study were left with. One other factor to consider is that chronic ill health is not only concerned with the physical well-being of the child. Pot-Mees (1989) agrees that, concerning the BMT children in her study, the physical cure following bone marrow transplantation should not be equated with a total cure, because a total cure should also involve the psychosocial well-being of the child, including emotional and psychological well-being, plus satisfactory social integration. Pot-Mees’s study showed that at one year after treatment, the children demonstrated deficiencies in their mental and social health. She concluded by stating that her study indicated that there were ‘. . . special needs which chronically ill children tend to have after having received their potentially curative treatment’ (Pot-Mees 1989 p. 95). If one accepts the proposition that many SCID children go on to suffer chronic ill health following bone marrow transplantation, then, according to Bradford (1997 p. 62), discussing the work of Pless and Nolan (1991), ‘It is well established that children who have a chronic disease are a population at risk of developing emotional and behavioural problems, with a two- to

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threefold increased rate of psychological difficulties in comparison to their healthy peers’. SCID children, however, suffer a threefold insult, namely their acute, life-threatening illness, their hospitalisation, isolation and bone marrow transplantation, and also their chronic ill health post-transplant. Thus, the risks of psychosocial morbidity are increased greatly. Consequently, this section will examine the patterns of stress and coping and the psychosocial functioning of the children. Because of the many variables within the SCID group, in terms of age, gender, country of origin, country of BMT, and experiences pre- and posttransplant, it was not feasible to have a matching normal control group. Therefore, other groups were used for comparison purposes. Using the Rutter A and B scales (see Chapter 2), the children in the SCID group were compared to two groups reported in the Pot-Mees (1989) study, namely:

r BMT children r children with cardiac problems In addition, using just the Rutter A scale, the SCID group were compared with the group of children with liver disease studied by Bradford in 1994. There was also a reference group of children without any serious health problems for the SCID study involving the Rutter A scale. This was a sample of 50 children with good health, in which the age range of the children was from 3 to 15 years, with an equal mix of boys and girls, and they were tested in York, Hull, Sheffield, London and Luton. The children with SCID were compared with this group. The use of these other groups allows for a comparison of the SCID group with well children, with children who have a chronic disease and with children who have also received a BMT for reasons other than SCID. This chapter gives an overview of psychosocial functioning in the SCID children.

The Rutter A Scale A total of 21 items from the Rutter A scale was presented to the parents within their questionnaire. For the first 18 items on this list, the parents had to grade specific behaviours as not occurring in their children, sometimes occurring, or occurring a lot. The parents were presented with a total of 21 statements, but to simplify the presentation of the tables in this chapter, headings have been used. The list of headings for the first 18 items and their related statements (as presented to the parents) are given below. Heading 1. Restless 2. Fidgety 3. Destructive

Statement Very restless. Often running about or jumping up and down. Hardly ever still. Squirmy, fidgety child. Often destroys own or others’ belongings.

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4. 5. 6. 7. 8. 9.

Fights Disliked Worries Solitary Irritable Unhappy

10. 11. 12. 13. 14.

Tics Sucks thumb Bites Nails Disobedient Unsettled

15. Fearful 16. Fussy 17. Lies 18. Bullies

Frequently fights with other children. Not much liked by other children. Often worried, worries about many things. Tends to do things on his/her own – rather solitary. Irritable. Is quick to ‘fly off the handle’. Often appears miserable, unhappy, tearful or distressed. Has twitches, mannerisms or tics of the face or body. Frequently sucks thumb or finger. Frequently bites nails or fingers. Is often disobedient. Cannot settle to anything for more than a few moments. Tends to be fearful or afraid of new things or new situations. Fussy or overparticular child. Often tells lies. Bullies other children.

For the last three items below (19–21), the parents were asked to state whether these behaviours occurred: (a) (b) (c) (d)

never in the last year less than once per month once a month or more once a week or more

and they were then given the score of 0, 1 or 2 accordingly. Heading 19. Temper 20. Tears 21. Truants

Statement Has temper tantrums. Had tears on arrival at school or refused to go into the building. Truants from school.

Once all the 21 items for each of the children had been scored, a mean score (index) was arrived at. The groups that were compared with each other were:

r r r r r

SCID group – post-transplant Reference group BMT group – 12 months post-transplant (Pot-Mees 1989 p. 153) Cardiac group – 12 months post-transplant(Pot-Mees 1989 p. 153) Liver disease group – (Bradford 1997 p. 69)

Note that, in the tables in this chapter, an index of 2.00 would mean that all the children displayed that behaviour all the time, and that in all the tables in this chapter, Refer. = reference group.

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The Post-Transplant Behaviours of the Children Treated Successfully for SCID The first table (Table 8.1) depicts the scores on the Rutter A test for the children successfully treated for SCID and the references group for comparison. The SCID children were assessed from 14 months to 14 years post-transplant. Given that an index of 2.00 indicate that all the children would display that behaviour all the time, and an index score of 0.00 would mean that none of the children ever display that behaviour, it can be seen from Table 8.1 that certain behaviours stand out as being a problem in many of the children – although no single behaviour is exhibited by all of the children all the time. One behaviour, with an index score of 0.96, that stands out as being exhibited by many of the children for a lot of the time is that of worrying and being anxious. As Eiser (1990) points out, anxiety and uncertainty are inevitably a result of childhood disease. Although Eiser was discussing the parents of children with chronic disease, this can also affect the children themselves. These children are aware that they had a life-threatening disease, and that they are seen as ‘special’ (and possibly delicate and ‘at risk’) because of this. In addition, many of the parents reported that they worried continually over their children and that they could never relax – even Table 8.1 Rutter A Scale – Index Scores of SCID Children and Reference Group Rutter A scale Item Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

SCID (n = 32)

Refer. (n = 50)

0.72 0.69 0.22 0.38 0.19 0.96 0.89 0.67 0.48 0.15 0.38 0.67 0.81 0.70 0.82 0.50 0.22 0.22 0.88 0.25 0.06

0.34 0.26 0.06 0.20 0.14 0.54 0.46 0.30 0.16 0.04 0.28 0.30 0.50 0.12 0.36 0.18 0.15 0.17 0.46 0.18 0.06

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when their children were well. This worry can easily transfer itself to the children. Linked to this theme of worry and anxiety can be such behaviours as being fearful or afraid of new things and situations (index score of 0.82), unhappy (0.48) and bites nails (0.67). The next most common problem (with an index score of 0.89) exhibited by these children is that they are solitary. This, along with all the other behaviours where there are serious problems, will be discussed more fully in a later chapter, but it appears to be linked to problems with attachment. Solitary behaviour is very closely followed by temper tantrums (index score of 0.88). This may be due to feelings of frustration, possibly linked to restrictions and personal appearance problems that some of the children continue to have. Linked to temper tantrums is the problem of disobedience (with an index score of 0.81), restlessness, being unsettled, and being fidgety (0.72, 0.70 and 0.69, respectively), along with, to a lesser extent, fighting (0.38) and being destructive (0.22).

Comparison of SCID Children and Children of the Reference Group These compare quite markedly with the children in the reference group of well children, whose members are 50% less likely to be worried and anxious, fearful of new situations, or to bite their nails and 33% less likely to be unhappy. Again the children in the reference group are 50% less likely to be solitary children (according to Rutter’s definition) and 50% less likely to have temper tantrums, to fight other children, or to be restless children. Finally, the children in the reference group are even less likely to unsettled, fidgety or destructive children. For statistical comparisons, in order to assess whether the results between the two groups are significant, a z test was performed for each of the items, this time using the SCID group as the index group for comparison with the reference group. Significance level for the comparison of the results was at the 5% level (p < 0.05; z value = 1.96). Thus, any z value that is more than 1.96 means that there was a statistically significant difference between the two groups for that behaviour. In addition, as shown in Table 8.2, the z values are given as either negative (−) or positive (+) to indicate whether the SCID group had a better or a worse score than the reference group, i.e. a negative z value means that the SCID group exhibited that behaviour to a greater degree than the group with which it is compared. Similarly, a positive z value means that the SCID group had less of a problem with that behaviour than did the reference group. From Table 8.2, it can be seen that the children of the reference group had lower levels of behavioural problems on every one of types of behaviour included in the Rutter test, and that for 13 types of behaviour, there was a statistically significant difference between the two groups. These were:

r restlessness and being fidgety r being destructive and having temper tantrums r being unsettled (very significant difference between the two groups)

Psychosocial Effects on the Children

r r r r r r

135

being disobedient being irritable being worried, unhappy and fearful biting their nails being solitary children being fussy children

The use of this behavioural scale shows that, compared to children who have not had SCID, the children who have survived the treatment do have more prominent psychological problems following the treatment – even many years after BMT. These findings will be explored in more detail in later chapters. Table 8.2 Rutter A Scale – Comparison of Items Between SCID and Reference Groups Rutter A scale Item Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

Refer. −3.04** −3.07** −3.20** −1.50 −0.50 −3.00** −2.87** −2.64** −2.67** −1.38 −0.77 −2.18** −2.58** −4.14** −3.07** −2.67** −0.78 −0.50 −3.61** −0.64 −0.02

Values marked with a double asterisk (** ) are values which are significant at the 5% level (p < 0.05).

Comparison of SCID Children with Children from the Reference Group and from Groups of Children with Other Disorders In order to see whether the psychosocial and behavioural problems of the SCID children, as shown using the Rutter A scale, are unique to them, or whether they are a manifestation of all children who have undergone

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the traumas of early hospitalisation and acute treatment, such as a BMT, the children in the SCID group were compared with children from other groups, as mentioned earlier. The children had either had a BMT for a disorder other than SCID, had had open heart surgery for serious heart disease or had serious liver problems. These groups were chosen because their post-treatment psychosocial behaviour had previously been assessed using the Rutter A scales (Bradford 1997, Pot-Mees 1989). Table 8.3 shows the comparisons between the SCID children and the other three groups of children who had had hospital treatment as well as the reference group From Table 8.3, it can be seen that the SCID group of children approximate mainly to the children with liver disease in terms of their behaviour. As can be seen from Table 8.3, the children in the SCID group exhibit worse behaviour traits with every one of the items as compared to the children in the reference group and the children with cardiac problems, and with all, bar one of the behaviours compared to the BMT children. This is ‘sucking the thumb’ which is only a minor problem with all the groups. The group of children with liver disease is the only group which has more than one positive score compared to the SCID group. In fact the Table 8.3 Rutter A Scale – Comparison of Items Between the SCID Children and Four Other Groups, Using z Test Rutter A scale Item Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

Refer. −3.04** −3.07** −3.20** −1.50 −0.50 −3.00** −2.87** −2.64** −2.67** −1.38 −0.77 −2.18** −2.58** −4.14** −3.07** −2.67** −0.78 −0.50 −3.61** −0.64 −0.02

BMT −2.56** −3.14** −4.40** −0.67 −1.90 −2.93** −4.27** −1.43 −1.08 −1.88 +1.31 −3.06** −3.25** −1.79 −3.47** 0.00 −1.89 −1.70 −3.26** −1.36 −0.10

Cardiac −1.12 −2.21** −1.40 −2.83** −1.10 −2.21** −4.40** −1.50 −1.42 −0.88 −0.85 −2.59** −3.25** −2.50** −2.13** −2.25** −0.78 −1.40 −3.61** −0.55 −0.52

Liver +2.24** +0.14 +3.20** +0.83 +0.50 −2.43** −2.13** +0.64 −0.17 +4.63** −2.15** −1.12 −0.42 −0.07 −1.33 −1.75 +1.78 −0.30 −0.57 −1.00 +0.31

Values marked with a double asterisk (** ) are values which are significant (p < 0.05, z value = 1.96).

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liver disease children have a total of nine items in which their behaviour is worse than that of the SCID children. In addition, the z test demonstrates that, as was apparent from the raw data, the nearest comparable group to the SCID group in terms of behavioural problems is the group of children with liver disease, with only 6 out of 21 behaviours (26%) showing significant differences between the two groups. This compares with the number of behaviours in which there is a significant difference between the SCID group and the reference group (13 behaviours = 62%), the BMT group (9 behaviours = 43%) and the cardiac group (10 behaviours = 48%). Whilst the results are to be analysed in more detail a little later in this chapter, some points are worth highlighting at this stage. As was seen from the index values in Table 8.1, the SCID children have more psychosocial problems than do the children in the reference group, significantly so in over half of the items (13 out of 21 = 62%) at the 5% level of significance (p < 0.05). With no types of behaviour do the SCID children have better behaviour profiles in comparison with the reference group, and in only one item, ‘truancy’, do they reach anything like parity. Comparisons with the Pot-Mees (1989) BMT group show a similar trend, albeit not quite as striking. There are nine items (43%) in which there are significant differences (p < 0.05). As with the reference group, the SCID and BMT children almost achieve parity as regards ‘truancy’. In addition, there is one behaviour – ‘frequently sucks thumb or finger’ – in which the SCID children demonstrate this behaviour to a lesser extent than do the BMT children, but the overall numbers exhibiting this behaviour are actually very small. In terms of the items in which significant differences at the 5% level emerge, both neurotic/depressive and antisocial/aggressive behaviours are included, and every one of the nine behaviours included in this list is also to be found on the list of behaviours which show significant differences between the SCID children and the children in the reference group. One other point – the reason that there is no difference between the amount of fussiness present in the SCID children and in the BMT children, whilst there are major differences between the SCID group and the other three groups for this behaviour, may be linked to the fussiness exhibited by parents, nurses and others during the BMT, in terms of cleaning the transplant area and other precautions, which do sometimes reach quite obsessive proportions. These precautions are often carried out for some time when the child and family return home, certainly as far as the SCID families are concerned, and this attitude may have seeped into the family consciousness and remain with them and the children. There are significant differences between the SCID children and the children with cardiac problems in a total of ten behaviours (48%), and with no behaviour do the SCID children have fewer problems than the cardiac children. Even with truanting, there is a greater difference between the SCID children and the cardiac children than is seen in the previous two groups.

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The picture, however, changes dramatically when comparisons are made between the SCID children and the children who make up Bradford’s (1994) group of children with liver disease. There are only six behaviours (29%) in which significant differences at the 5% level occur, half of which are positive differences, i.e. the SCID children exhibited this behaviour to a lesser extent than the children with liver disease. In addition, another six items (29%) also display, to a lesser extent, a positive difference. These positive differences generally occur within the antisocial/aggressive range of psychosocial behaviours, whilst the negative differences (i.e. where the SCID children display the behaviour to a greater extent) tend, in the main, to be within the neurotic/depressive behaviours. Thus, whilst there are more similarities between these two groups (i.e. more behaviours with a z value less than 1.96) than occurs with the SCID children and the other groups, there are clear differences between the types of psychosocial behaviour exhibited by the children in the two groups. Whilst one would expect the SCID children and the BMT children to be the most similar in their behaviours, the fact that this is not so, and that the children with liver disease and those with SCID are the two groups with the most similarities may, in part, be due to the ‘chronic’ nature of both these disorders. From these tables above, it is possible to conclude that all children undergoing early hospitalisation and acute treatment – BMT, liver disease, open heart surgery (all of which require a certain degree of isolation) are very much at risk of long-term psychosocial behavioural problems when compared to children who do not have these experiences, there are differences between the quality and quantity of behaviours displayed by the children, as well as they type of behaviour. This may be as a result of the type of treatment, the preparation beforehand (e.g. the SCID children do not have time for any preparation before hospitalisation), the type of disease, and the age of the child when the treatment commences. Or there may be differences in support that the child and/or families receive during and after treatment. There are also the problems with whether there are any long-term complications, such as chronic ill health, that may well play an important part in the types and scale of behavioural problems displayed by the children. What, however is not in any doubt, is that the children who survived BMTs, early and lengthy hospitalisation and isolation, are very much at risk of developing many psychosocial problems in the long-term – although, as can be seen from the tables above, not all of them do develop such behavioural problems. Some of them are happy well-adjusted children. Consequently, the challenge is to work out why some children develop psychosocial problems following successful treatment for SCID whilst others do not. If the answer to that can be found, then it may be possible to put supportive measures in place to at least reduce the impact of the disease and its treatment in the long-term, even if such behavioural problems cannot be completely eliminated. Consequently, the next step is to look at certain variables to see if they were involved in any way with the longterm outcomes, psychosocially, of the children. The variables to look at are the gender of the child and the country of origin.

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Table 8.4 Rutter A Scale – Comparison Between the Boys and Girls of the SCID Group Using a t TwoTailed Test Rutter A scale Item

t – value

Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

−0.63 +0.69 +0.50 +0.45 +0.78 −1.10 +0.84 −0.38 +0.49 +0.94 −0.63 +1.24 +0.63 +2.22** +0.25 +0.85 −0.08 −0.11 +0.14 +1.10 −1.00

Value marked with a double asterisk (** ) is significant (p < 0.05).

The Importance of Gender The first question to consider is to what extent the gender of the SCID children can possibly be linked to psychosocial behaviour. First of all, the mean scores for boys and girls from the SCID group and the reference group for each of the 21 types of behaviour were abstracted. Comparisons were then made between the SCID children and the children of the reference group. Table 8.4 displays the t values following a t two-tailed statistical test where significance is placed at the 5% level (p < 0.05) of the children making up the SCID group, whilst Table 8.5 compares the boys and the girls of the reference group. In Tables 8.4 and 8.5, positive (+)t values are indicative of greater problems experienced by the girls in that behaviour. Although there are differences between the SCID girls and boys in the study (Table 8.4), only one type of behaviour, namely that concerned with poor concentration, is significant at the 5% level (p < 0.05). This is actually quite similar to the pattern for the differences between the boys and girls in the reference group (Table 8.5), in which there are only two items with significant differences, namely restlessness and fussiness. What is surprising, however, is that the differences between the boys and girls in each of the two groups follow different paths. With the SCID children, the antisocial/aggressive behaviours, such as being destructive, fighting and

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Table 8.5 Rutter A Scale – Comparisons Between the Boys and Girls of the Reference Group Using a t Two-Tailed Test Rutter A scale Item

t – value

Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

−2.28** −0.68 −1.82 −1.96 −0.72 +1.92 +1.04 +0.83 +1.89 +1.00 +1.05 +1.87 −1.44 −1.62 +0.87 +2.45** +0.91 −1.58 +0.55 −1.26 −1.82

Values marked with a double asterisk (** ) are values which are significant at the 5% level (p < 0.05).

disobedience, whilst not being significant at the 5% level, demonstrate differences between the boys and girls, in which the girls are seen as being more aggressive and antisocial, whereas in the reference group, the opposite is true. In the reference group, the boys are seen as the more aggressive, antisocial group. On the other hand, the SCID boys display more neurotic and depressive behaviours, such as worrying, than do the girls, whilst, again, the opposite is true of the children in the reference group (Tables 8.4 and 8.5). In a total of 14 out of 21 items of behaviour (67%), the girls in the SCID group exhibit psychosocial behaviours greater than the boys, whilst with the reference group, the items are more evenly distributed between the two genders, with 11 out of 21 (52%) of the psychosocial behaviours being displayed more by the girls than the boys (Tables 8.4 and 8.5). Meanwhile, Table 8.6 investigates the comparisons between the SCID boys and the boys in the reference group, and Table 8.7 does the same for the girls in the two groups. In Tables 8.6 and 8.7, positive (+) t values are indicative of greater problems experienced by the girls with that behaviour. In Table 8.6, a positive t value indicates that there are more problems with that behaviour for the boys in the reference group, whilst a negative

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Table 8.6 Rutter A Scale – Comparison Between the Boys of the SCID Group and the Boys of the Reference Group Rutter A scale Item

t – value

Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

−1.06 −1.31 −0.35 0.00 0.00 −3.74** −2.45** −3.07** −3.03** −1.45 −0.91 −2.65** −0.97 −1.64 −2.41** −3.03** −1.90 0.00 −2.45** +1.22 +0.39

Values marked with a double asterisk (** ) are values which are significant at the 5% level (p < 0.05).

t value indicates greater problems for the SCID boys, whilst in Table 8.7, a positive t value indicates that there are more problems with that behaviour for the girls in the reference group, whilst a negative t value indicates greater problems for the SCID girls. When comparing the boys in the SCID group with the boys in the reference group (Table 8.6), the SCID boys exhibit 16 of the 21 types of behaviour (76%) to a greater extent than do their counterparts in the reference group, with the differences in 8 of the 21 items (38%) being significant at the 5% level. The other five types of behaviour include three in which there is no difference at all between the mean scores of the boys in these two groups (Table 8.6). Looking at the types of behaviour in which there is a statistically significant difference between the boys of the SCID group and the boys of the reference group, with the exception of temper tantrums, the types of behaviour generally fall in the depressive/neurotic category. This lends credence to the theory that the boys who survived treatment for SCID tend towards the more neurotic and depressive behaviour symptoms. A similar pattern emerges when comparing the girls in these two groups. In only two of the types of behaviour (9.5%) do the girls in the SCID group have fewer behavioural problems than do the girls in the

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Table 8.7 Rutter A Scale – Comparison Between the Girls of the SCID Group and the Girls of the Reference Group Rutter A scale Item

t – value

Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

−1.81 −2.20** −1.41 −1.57 −1.07 −0.43 −2.14 −1.22 −1.23 −1.02 +0.66 −2.22** −2.21** −4.62** −1.56 −1.51 −0.54 −1.04 −1.55 −1.07 0.00

Values marked with a double asterisk (** ) are values which are significant at the 5% level (p < 0.05).

reference group. However, in only four of the behavioural items (19%) are the differences between the two groups statistically significant at the 5% level. None of the girls from either group have played truant from school. It can be seen now that the SCID girls are closer to their counterparts in the reference group than are the SCID boys with their reference group, but there is still quite a gulf existing between them (Table 8.7). Thus, it can be seen that the gender of the child is an important variable in the quality and type of psychosocial behaviours exhibited by the SCID children, with the girls exhibiting more of the aggressive/antisocial types of behaviour whilst the boys tended to exhibit more of the neurotic/depressive types of behaviour. In addition, it can be seen that both boys and girls who have had SCID have more psychosocial problems than their respective peers in the reference group.

The Importance of the Place of Treatment/Place of Origin The second variable to consider is that of the country in which the children received their BMT. Table 8.8 displays the mean scores for the 21 items for the UK SCID children and the German SCID children.

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Table 8.8 Rutter A Scale – Mean Scores of Items for UK SCID Children and German SCID Children Rutter A scale Item Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

UK SCID (n = 15)

German SCID (n = 17)

0.87 0.67 0.33 0.60 0.47 1.20 1.40 1.07 0.67 0.33 0.40 1.13 1.27 0.73 1.13 0.80 0.40 0.40 1.20 0.27 0.13

0.59 0.71 0.12 0.24 0.06 0.88 0.65 0.53 0.35 0.00 0.24 0.65 0.53 0.77 0.59 0.29 0.19 0.18 0.59 0.12 0.00

A first glance at the data in Table 8.8 elicits the fact that there does appear to be a difference between the SCID children transplanted in Germany and those transplanted in the UK, with the UK children appearing to experience more psychosocial problems than do the German children. Table 8.9 bears out that initial impression, by displaying the t values for the German and UK children, using a t two-tailed test, with the significance level at 5% (p < 0.05). A negative sign before a t value means that the UK children exhibit that behaviour more than the German children, whilst a positive sign before a t value indicates that the German children have more problems with that behaviour than do the UK children. What can be ascertained immediately from Table 8.9 is that, although there are only four types of behaviour (19%) with significant differences between children from the two countries at the 5% level, namely having a temper, being solitary, being disobedient and being fussy, there are only two types of behaviour (being fidgety and unsettled) in which the German children demonstrate worse behaviour than do the UK children. Possible reasons for this will be explored later in this chapter. The second point to make about the above two tables is that the German SCID children do parallel the psychosocial behavioural profile of the UK SCID children, but with not the same severity of problem. Compared to the children in the reference group, the UK SCID children demonstrate greater problems in all behavioural aspects, with 11 out of 21 behaviours

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Table 8.9 Rutter A Scale – Comparison Between the UK and German SCID groups Using a t Two-Tailed Test Rutter A scale Item

t – value

Restless Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Disobedient Unsettled Fearful Fussy Lies Bullies Temper Tears Truants

−1.08 +0.13 −1.06 −1.44 −2.03 −1.09 −2.63** −1.99 −1. 30 −2.09 −0.62 −1.43 −3.73** +0.11 −1.86 −2.20** −0.85 −1.05 −2.94** −0.69 −1.00

Values marked with a double asterisk (** ) are values which are significant at the 5% level (p < 0.05).

(52%) demonstrating significant differences at the 5% level, whilst the German SCID children demonstrate only two behaviours (9.5%) where the differences between them and the children in the reference group are significant at this level, and in five behaviours (24%), namely tears on going to school, being disliked by other children, sucks thumb, has facial tics, and truants from school, actually exhibit that behaviour less often than do the children in the reference group (Tables 8.8 and 8.9). Turning to comparisons with the Pot-Mees BMT group, it can be seen that the UK SCID children have more psychosocial problems overall than the BMT children, with again the number of items in which there are significant differences at the 5% level showing a variation between the UK SCID and BMT children and the German SCID and BMT children, with the UK children experiencing significant differences in ten items (48%), as opposed to only three items (14%) where the German SCID children are concerned. In only one of the items (sucks thumb) do the UK SCID children have a better mean score than the BMT children, whereas the German SCID children have a better mean score in three of the items – fights, sucks thumb and fussy (Tables 8.8 and 8.9), although a weakness

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of the study is that all the reference group were from the UK, and this undoubtedly, given the differences between the SCID children from the two countries, affects the findings. Overall, therefore, it appears from these statistics that the UK SCID children have more psychosocial problems than the German children as well as the children in the other groups used for comparison purposes. Possible reasons for this will be explored shortly.

Psychosocial Functioning: A Comparison with Other Children Using the Rutter B Scale In addition to the parents filling in the Rutter A scale, the teachers of those who were attending school were requested to fill in the Rutter B scale. This scale is very similar to the Rutter A scale, with just a few different items of behaviour being included. There are four extra items in the Rutter B scale. These are:

r r r r

Absent Steals Soils Pain

Tends to be absent from school for trivial reasons Has stolen things on one or more occasions Has wet or soiled self at school this year Often complains of pains or aches

There is one item not included in the Rutter B scale but which forms part of the Rutter A scale, namely temper. Altogether 18 out of 30 teachers replied and returned the completed Rutter B scale, a reply rate of 60%. Table 8.10 gives the mean for each item for the SCID children, as well as for the two Pot-Mees (1989) groups, namely the BMT and cardiac children. In the Bradford (1997) study of children following liver transplantation, no Rutter B sale was used. Unfortunately, this is not comparing like with like because the children in the two Pot-Mees groups were assessed by their teachers 12 months posttransplant/treatment, whilst he children in the SCID group were approximately four to six years post-transplant. In addition, the children in the two Pot-Mees groups were assessed by their teachers before the transplant, and actually the results were better post-transplant than pre-transplant for these two groups – possibly due to the fact that they pre-transplant the children had lots of physical and psychosocial problems linked to their illness, whilst post-transplant they were, in many cases, ‘cured’. However, Pot-Mees (1989 p. 80) did point out that in spite of this, the rate of behaviour disturbance in school ‘was still higher than could be expected from children in the normal population’. From the mean index scores in Table 8.10, significance testing was done at the 5% level, i.e. p < 0.05, z value = 1.96, using a z test. These results are displayed in Table 8.11. Positive z values indicate items of behaviour where the children in that group experience more problems with that behaviour than do the SCID children.

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Table 8.10 Rutter B Scale – Mean Scores of the SCID Children and Children in the Other Groups (Pot-Mees’s 1989 BMT and Cardiac Groups) Rutter B scale Item Restless Truants Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Absent Disobedient Unsettled Fearful Fussy Lies Steals Soils Pain Tears Bullies

SCIDS (n = 18)

BMT (n = 22)

Cardiac (n = 28)

0.50 0.00 0.83 0.17 0.22 0.28 0.72 0.50 0.28 0.33 0.28 0.28 0.44 0.00 0.17 1.00 0.61 0.39 0.22 0.00 0.00 0.22 0.17 0.06

0.35 0.00 0.29 0.06 0.18 0.12 0.53 0.29 0.35 0.06 0.06 0.12 0.18 0.00 0.24 0.29 0.12 0.06 0.06 0.06 0.00 0.18 0.12 0.12

0.37 0.04 0.26 0.00 0.11 0.07 0.52 0.30 0.11 0.22 0.19 0.07 0.19 0.04 0.08 0.27 0.35 0.04 0.00 0.04 0.00 0.19 0.23 0.04

The four items ‘Absent’, ‘Steals’, ‘Soils’ and ‘Truants’ are not scored as no SCID children are recorded by their teachers as exhibiting any of those behaviours. From the results it can be seen that similar patterns emerge with the Rutter B scale as with the Rutter A scale, although there are differences in the scale of the problems demonstrated. In only three behaviours (12.5%) – being irritable, naughty and a bully – do the BMT children have worse scores than the SCID children, whilst there are significant differences (p < 0.05) in four behaviours (17%), namely fidgety, unsettled, fearful and fussy. In only one type of behaviour (crying on arrival at school) do the SCID children exhibit fewer problems than the cardiac children, whilst there are significant differences in three behaviours (fidgety, unsettled and fussy). From these statistics, it can be seen that the psychosocial profile as evidenced by the teachers’ responses to the Rutter B scale parallels the parental responses in terms of demonstrating that the SCID children do have many psychosocial problems, although the teachers assess their problems as being not as severe as the parental assessments.

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Table 8.11 Rutter B Scale – Comparison Between the SCID Children and Children in the Other Two Groups (Pot-Mees’s BMT and Cardiac) Using the z Test Rutter B scale

z – values

Item

Restless Truants Fidgety Destructive Fights Disliked Worries Solitary Irritable Unhappy Tics Sucks thumb Bites nails Absent Disobedient Unsettled Fearful Fussy Lies Steals Soils Pain Tears Bullies

BMT (n = 22)

Cardiac (n = 28)

−0.94 — −3.38** −1.22 −0.31 −1.07 −0.91 −1.31 +0.47 −1.69 −1.47 −1.07 −1.44 — +0.78 −3.74** −3.06** −2.36** −1.23 — — −0.31 −0.42 +1.20

−0.81 — −3.56** −1.89 −0.85 −1.40 −0.95 −1.25 −1.13 −0.69 −0.60 −1.40 −1.39 — −1.00 −3.84** −1.63 −2.50 −1.69 — — −0.23 +0.50 −0.40

Values marked with a double asterisk (** ) are values which are significant at the 5% level (p < 0.05).

Behaviour Disturbance in Individual Children According to Pot-Mees (1989), the presence of behaviour disturbance is identified by using the number of points scored on the Rutter A scale as the criterion. Children with a score of 13 or above are deemed to have a behaviour disturbance. Table 8.12 summarises the prevalence of behaviour disturbance, according to this criterion, in the SCID children and also the Pot-Mees BMT children at their 12 months follow-up. The percentage of all the SCID children with a behaviour disturbance is virtually identical to that of the BMT children. However, there is a great difference between the German and the UK SCID children in that, using this criterion, far fewer German SCID children have a behaviour disorder. A similar, but not as extreme, difference occurs between the German SCID children and the BMT children. However, the group with the least number

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Table 8.12 Percentage of Children (SCID, BMT and Reference Groups) with a Behaviour Disturbance, Using the Rutter A Scale Children with a behaviour disturbance

n %

SCID UK (n = 15)

SCID Germany (n = 17)

SCID all (n = 32)

BMT (n = 20)

8 53

3 18

11 34

7 35

Refer. (n = 50) 2 4

of children with a behavioural disturbance is the reference group, with only two children – one boy and one girl – having a behaviour disturbance. These two children actually only just reach the category with the minimum 13 points each. Interestingly, Pot-Mees found that by six months post-BMT, the rate of behavioural disturbance had risen from 15% pre-transplant to 40%, but that by 12 months post-transplant, it had dropped slightly to 35% of the children having a behaviour disturbance. She pointed out that this was certainly higher than would be expected in the normal population (Rutter et al. 1970). Others, such as Howarth (1972) and Garralda et al. (1988) had also identified similar behavioural disturbance rates. Howarth (1972), looking at the psychiatry of terminal illness in children identified behavioural disturbance in 40% of patients with leukaemia and cystic fibrosis, whilst Garralda et al. (1988) identified psychiatric disturbance in 32% of children with severe renal failure and who required haemodialysis. Pot-Mees (1989) also found that at 12 months post-transplant, 5 out of 7 children (71.4%) from her BMT group displayed a fresh behaviour disorder – i.e. one that had not been present previously. She presumed that these new behaviour disorders occurred as a result of the treatment the child received, and as a result of the BMT in particular, because none of these children had been previously identified as having a psychiatric illness. She also pointed out that these children were actually medically fit at this time and had no serious complications following their BMT. She felt that these new behaviour disturbances could therefore not be explained by the presence of medical problems at that time, by restrictions – such as isolation (as the children were now free to lead normal lives) – nor by any other special precautions – as there were none necessary. The same could, to some extent, apply to the SCID children. Many of them were quite medically fit, and certainly their SCID had now been cured, although some of the children did have long-term problems as a result of the initial SCID problem and the subsequent treatment. There was now no longer any excuse for any special precautions to be taken (although there had been for the first few years following the successful transplant – at least until they had had all their vaccinations and immunisations. Using the same criterion of 13 points, a similar, but again not as extreme a picture emerges between the groups when the teachers assessed the children using the Rutter B scale, as shown in Table 8.13. The children in the Pot-Mees BMT sample were assessed at their 12 months follow-up.

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Table 8.13 Percentage of Children (SCID and BMT) with a Behaviour Disturbance, Using the Rutter B Scale Children with a behaviour problem

n %

SCID UK (n = 7)

SCID Germany (n = 11)

SCID all (n = 18)

BMT (n = 17)

3 43

0 0

3 17

4 23.5

As with the parents’ assessment, the teachers’ assessment shows that the UK SCID children have a higher percentage of children with a behaviour disorder than do the other two groups, with the German SCID children having the lowest, in this instance none. In the Pot-Mees (1989) study, the teachers felt that the children with behavioural problems tended to have problems with concentration and adaptation in the classroom, and also had problems responding to discipline. These findings were to a large extent replicated in the SCID group. Looking at individual children highlights rather large differences between the children in the SCID group. The maximum number of points that the children could receive from all the items in the Rutter A and Rutter B scales are 42 and 46, respectively. The higher the number of points a child scores, the more behaviourally disturbed he or she is. Table 8.14 below tabulates the individual scores of all the children for the Rutter A, and, where, available the Rutter B scales. In addition, the types of severe immunodeficiency are also recorded in Table 8.14 so that comparisons can be made between the type of severe immunodeficiency disorder and the presence and extent of behavioural problems. The children are placed in order of severity of behavioural problems as defined initially by the Rutter A scale, and thereafter by the Rutter B scale. In the table, RD stands for reticular dysgenesis. Box 8.1 gives a little more information about the disorders mentioned in Table 8.14. As can be seen from Table 8.14, some of the children demonstrate a great deal of behavioural disturbance. One UK boy, for example, only failed to score on one item out of 21 – that of being disliked (although anecdotal evidence suggests that he could have scored on this item as well) and received a score of 37 out of a possible maximum of 42, whilst one UK girl only failed to score on two items – truancy and sucking her thumb – and had a total of 36 out of a possible 42. The question at issue here is that of the possibility of any common factors possessed by the children with a score of 13 or more. The first immediately noticeable fact is that there are a high number of children with ADA SCID in this group – a total of 5 out of 11 (45.5%). In addition, there is one child with reticular dysgenesis (RD) in this group, but only one. However, three children with ADA SCID are not deemed to have a behaviour disorder, and in fact two of them have the lowest scores in the group. In addition, the second child with RD falls far short of having a

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Box 8.1

Types of SCID Mentioned in Table 8.14

Types of SCID in Table 8.14

r ADA: Adenosine deaminase deficient SCID. A metabolic disorder

r

r

r

in which other cells of the body are affected as well as the cells of the immune system. Includes the absence of lymphoid tissues (vital for the immune system), infections and failure to thrive. In addition, the underlying metabolic disorder cause skeletal, liver and/or neurological abnormalities that may be severe (Hershfield 2004). Omenn’s: Omenn’s disease. An autosomal recessive severe combined immune deficiency, with scaly dermatitis, alopaecia, hepatosplenomegaly, severe respiratory infections, diarrhoea and failure to thrive (Fischer & Notarangelo 2004). RD: Reticular dysgenesis. A rare form of SCID which also includes an absence of other white cells of the immune system as well as lymphocytes. RD becomes apparent within the first days of life and can lead to failure to thrive, diarrhoea, vomiting and localised infection. Without successful treatment, death occurs within the first three months of life (Fischer & Notarangelo 2004). SCID: This category includes all other forms of severe combined immunodeficiency not mentioned above.

behaviour disorder on this criterion, as does the eighth child with ADA SCID. A closer examination of the children with ADA SCID with a behavioural disorder show that all five of them have hearing problems, some of them having very severe hearing deficits indeed. To counter this, however, the one child with RD who has a severe hearing disability is the one not included in the group of children with a behaviour disorder. The preponderance of children from the UK in the first half of the table is very striking and does back up the point made previously that the UK children, for whatever reason, do have much more serious psychosocial problems following their successful treatment for SCID. The next question to pose, concerns the possibility that the other children may have some common factor which could predispose towards a behaviour disorder, other than that of having SCID. Two of the UK children come from a single parent family and so may not have received as much family and social support as the others, although both mothers do come from quite supportive families. In addition, one UK boy’s parents certainly felt very isolated, as they discussed with me on my visit to them. Other than that, the children all seemed to be part of functioning two-parent families. One possible common factor is that, with the exception of one of the German boys, all the children with a behavioural disorder have some ongoing health or disability problems which may affect their behaviour. It may be,

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Table 8.14 SCID Children – Individual Scores from the Rutter A and Rutter B Scales Individual children – behavioural problems

UK boy UK girl UK girl UK boy UK boy German UK girl UK girl German UK boy German UK girl German German UK girl UK boy German German German German German UK boy German German German UK boy UK boy German UK boy German German German

boy boy boy boy girl boy boy boy boy girl boy girl boy boy boy girl boy

Parents

Teachers

Type of SCID

37 36 23 20 17 16 15 13 13 13 13 11 10 10 10 10 10 9 9 9 8 8 7 7 7 7 6 5 5 4 2 2

— — 27 — 7 10 19 23 7 1 1 — 10 4 1 — — — — — 10 — 8 4 — — 0 3 — 0 2 —

ADA ADA ADA SCID SCID ADA ADA SCID SCID RD SCID Omenn’s SCID SCID SCID SCID SCID SCID SCID SCID ADA SCID RD Omenn’s SCID SCID SCID SCID SCID SCID ADA ADA

therefore, that the children with a behaviour disorder are responding to chronic ill health and disability, but then so many of the SCID children also appear to suffer from long-term ill health and disability and they, according to the Pot-Mees criterion, do not have a behaviour disorder. It must, however, not be forgotten that, although only 11 out of the 32 SCID children are deemed to have a behaviour disorder, almost all the other 21 SCID children display behaviour that is considered a greater problem than that displayed by children in the other groups, particularly the children in the reference group. Thus, there does not appear to be any common factor running through this group which might explain their behaviour disorders, other than there does seem to be a link between ADA SCID and deafness and a behaviour

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disorder. However, neither ADA SCID nor RD are automatic criteria for the development of a behaviour disorder, nor is deafness per se. In terms of gender, seven of the children with a defined behaviour disorder are boys (30% of all boys) and four are girls (33% of all girls) so gender as a variable does not appear to be important. Country of transplant is seen as a variable because eight UK children (53%) and only three German children (18%) are represented in this group. Possible reasons for this have already been discussed in this chapter. The mean age at isolation for the children with behaviour disorders was 2.6 months, so this should fit in with the lower level of behavioural problems, as evidenced by the German children’s statistics presented earlier in the chapter, and the mean length of isolation in hospital was 9.6 months. Thinking back to the earlier statistics on the length of time spent in isolation and behavioural problems, this could be considered a factor, but for the fact that 5 of the 11 children were actually isolated for six months or less! As with so much of this study, the answer to the question of why certain of the SCID children have a behaviour disorder and others do not, will probably be a combination of factors, including severity of ongoing health and disability problems, family and social support, internal and external coping systems and strategies and trauma encountered at the time of transplant, along with the length of time spent in isolation.

Summary of the Psychosocial Problems as Indicated by the Rutter A and B Scales From all the foregoing statistics in this chapter, various points can be elicited. The SCID children generally exhibited more and worse psychosocial problems than the children from either the reference group or the two PotMees groups (i.e. children who had BMTs for disorders other than SCIDS, or who had cardiac problems). In contrast, the psychosocial behaviour of the SCID children approximates more to that of the children with liver disease (Bradford 1997). As the children with liver disease are perceived to have a chronic illness (Bradford 1997), then the likelihood is that the SCID children could also, from their psychosocial profiles, be deemed to have a chronic disorder. Significant differences occur between the SCID group of children and the children in the reference group in both the aggressive/antisocial range of behaviours (restlessness, fidgeting, destructive, disobedient, unsettled and having temper tantrums) and the neurotic/depressive range of behaviours (worrier, solitary person, irritable, unhappy, bites nails, tearful and fussy). In other words, the SCID children have significantly more problems over the whole range of behaviours. In only one behaviour, that of sucking the thumb or finger, do the Pot-Mees BMT children exhibit a worse behavioural trait than do the SCID children. As with the children in the reference group, the items in which there are significant differences between the children in the SCID and BMT groups encompass the whole range of behaviours, including

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aggressive/antisocial behaviours (restlessness, fidgeting, destructive, disobedient and temper tantrums) and neurotic/depressive (worrier, solitary child, bites nails and fearful). Why there should be such a difference between the two groups is hard to fathom, as both have had the core treatment of cytotoxic chemotherapy and bone marrow transplantation. Indeed, most of the BMT children also received radiotherapy as well as more and higher doses of cytotoxic chemotherapy. So, one would actually have expected the BMT children to have more psychosocial problems. The answer may lie partly in age of the children at isolation. The children in the Pot-Mees study received their BMT at a mean age of 8.3 years for acquired disorders, such as leukaemia and other cancers, and at 4.9 years for genetic disorders, such as Hurler’s syndrome. By contrast, the SCID children in this study received their BMT at a mean age of 4.6 months. There is some evidence (Douglas 1975, Freedman et al. 1976, Ludman et al. 1992) that children hospitalised and isolated at a very early age may have severe psychosocial problems at a later age. A second factor concerning isolation is the length of time spent in isolation. For the BMT children in Pot-Mees’s 1989 study, the time spent in isolation on average was 35 days. Compare this to the SCID children in this study who spent an average of 8.6 months in isolation (range 0–30 months), often with a much stricter regime of isolation in place. A combination of early hospitalisation and a longer period of isolation may be a major reason for the apparent differences in psychosocial behaviour between the children in the BMT and SCIDS groups. Another factor which may negatively affect the psychosocial functioning of the SCID children could be the ability of the parents/families to come to terms with all the traumas surrounding the diagnosis and treatment of the respective disorders, as well as the support they get from each other, the family, society, support groups and professionals. For many years, there has been much more awareness of the needs of children with leukaemia and other disorders requiring bone marrow transplantation than has been the case for children with SCID and other severe immunodeficiencies, and support strategies and funding for children with leukaemia have been in place for some time. The fact that both the SCID and BMT children rate highly in the Rutter A scale for ‘fussiness’ may be linked to the almost obsessional nature of the care, particularly in relation to their cleaning of the isolation cubicle. Certainly with SCID children, this obsessional behaviour and outlook continues for a long time once the children are discharged from hospital. Similar patterns to the Pot-Mees BMT children are found with the children in the Pot-Mees cardiac group. Because the cardiac problems that the children have are congenital, then these children, like the SCID children would have been hospitalised at a very early age. The fact that the cardiac children have fewer psychosocial problems than the SCID children leads one to suspect that it is the length of isolation rather then the early hospitalisation which is a major determining factor in the psychosocial profile of the SCID children. Comparisons with the children studied by Bradford (1994) who had liver disease present a totally different picture. In 9 out of the 21 types

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of behaviour listed (43%), the children with liver disease displayed more problems than did the children with SCID. These nine behaviours are mainly from the aggressive/antisocial range of behaviours, which may be linked to feelings of anger concerning their situation and feelings of helplessness. However, because there are far more similarities in psychosocial functioning between the children in the SCID group and the liver disease group, one comes to the conclusion that similar coping mechanisms are at play here between the children in these two groups, and therefore, they may both be experiencing chronic ill health, whilst at the same time having had, or still have, or at least perceived to have, an acute disorder. When attention is turned to one of the variables within the SCID group of children which may affect psychosocial behaviour, namely gender, one is struck by the apparent role reversal of the boys and girls in the group in relation to types of psychosocial behaviour, compared to the children in the reference group. In the reference group, the girls tend to display more neurotic/depressive behaviours, whilst the boys display more of the aggressive/antisocial behaviours. However, where the SCID children are concerned, the opposite applies. As yet, no evidence-based explanations are forthcoming for this apparent role reversal, although the aggressiveness of the girls may be linked to the chronic ill health scenario, as discussed above, whilst the boys may become neurotic and depressed because they feel that they are unable, or perhaps are not allowed by their parents, to come up to the image that is required of them as boys in today’s society. Other possible explanations may come forward once this phenomenon has been further researched. What is obvious, however, is that the SCID children generally are great ‘worriers’ and ‘loners’, and many of them are considered to be unhappy children by both their parents and their teachers. What exactly their worries are was not ascertained, although the feeling that came from visiting the families is that many of them feel alienated from their peers, and in some cases, from their families. For example, when many of the children drew pictures of their families, they drew themselves on the periphery of the group, or with the members of the family cut off from each other, or even, occasionally, with themselves missing. As far as the boys are concerned, with the exception of temper tantrums, all the types of behaviour in which there are statistically significant differences (p < 0.05) between the SCID boys and the boys in the reference group are concerned with the neurotic/depressive behaviours, i.e. worrier, solitary child, irritable, unhappy, bites nails, fearful and fussy. In other words, the boys in the SCID group are much more depressive and neurotic than are the boys in the reference group. There are fewer significant differences between the girls in the SCID group and those girls in the reference group – only four in effect. The extent of the poor concentration amongst the SCID girls is very striking, but overall the differences between the girls in these two groups are not as significant as between the boys from the same two groups. With only one type of behaviour, however, do the girls in the reference group appear to have more of a problem than the SCID girls, and that is sucking thumbs or

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fingers. Therefore, one can be assured that, on this evidence, the SCID girls have more psychosocial problems than the girls in the reference group. Thus, although not as dramatic as with the boys in the two groups, a similar pattern has emerged between the girls from the two groups. Gender does appear to play a part in the quality, quantity and type of psychosocial problems that SCID children exhibit, with the boys having more problems than the girls, and also tending to be really quite unhappy and depressed, as opposed to the aggressiveness and antisocial behaviour of the girls. This, as has already been pointed out, is in complete contrast to the norm as evidenced by the children in the reference group. Turning to the second variable that has been examined – the country in which the BMT took place, what is very striking is the fact that, apart from being more fidgety and having more problems with poor concentration, the UK SCID children experience more problems in all the behaviours than do the German SCID children. The differences may partly be explained by the age at which isolation commences and the length of time the children spend in isolation. The UK children were isolated at a mean age of 3.5 months, whilst the German children were isolated at a mean age of only 2.5 months. A greater disparity is demonstrated with the length of time spent in isolation. The UK children spent on average 11.7 months (range 3–30 months) in isolation, whilst the German children, on average, only spent half that time in isolation (6.0 months, with a range of 0–11 months). Certainly, if length of time spent in isolation can affect the psychosocial functioning of the children (see also the discussion earlier comparing the SCID children in this study with Pot-Mees’s BMT children) then this could offer an explanation for the German children having less psychosocial problems than the UK children. In addition, there are differences in the care of the children at the time of the transplant. In Germany, whole families can stay close to the children by staying in ‘Family Houses’ which are situated very close to the hospital, whilst in the UK, the ability to stay close to the children is usually restricted to parents only. The German families are able to live a restricted, but reasonably normal life during the transplant, a situation which, with the UK families in the study, was not an option. The use of an ‘Oma’ figure in the German transplant centre – a ‘granny’ carer to look after the children in their isolation and thereby giving the parents/families opportunities for breaks away from the stresses and claustrophobia of the unit, was also cited often by the German parents as being an important factor in enabling them to get through this very traumatic period. This attempt to give some normality to the situation for the parents and families, I feel, goes some way to explaining the better psychosocial profiles of the German SCIDS children. There was very little of the above available for the UK families, although attempts have been made to rectify this. This same level of support for the families and their children also continued after discharge, although not necessarily from the professional staff, which leads to another possible explanation for this difference between the UK SCIDS children and their German counterparts, namely the family/social systems within the two countries.

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Although there are obviously exceptions to this rule, in general, German families tend to be closer together, both physically and socially, than do families in the UK. There is not as much movement away from one’s own area in Germany as there is in the UK. Thus, many parents of SCID children in the UK felt very isolated, unlike families in Germany. As the mother of one UK child explained, ‘We had no family there, and when we came here, no friends, you know . . . no family. We were just stranded’. Many of the UK parents were so isolated that they were unable to talk about their experiences, their worries and expectations, and only when I visited them in connection with this study did they feel able, often for the first time in many years to talk about the experience and their feelings about it then and now. For some UK parents, the interview took on the role of a cathartic experience, a release of emotion and tension. In Germany there was not the same sense of isolation, except in one family, where, as with many of the UK parents, these parents felt they did not have much support from their family. Otherwise, I would often find other members of the extended family, particularly grandparents, at the house or flat in Germany when I visited the parents. In addition, certainly within the small villages in Germany and Switzerland, there was support from the community as well. It may not necessarily be the case that more family/social support actually makes the children’s behaviour better, but rather that the behaviour is perceived by the parents as being good or normal because they themselves are not under such strain and can therefore take a more positive and optimistic view of their child and the future. This does not mean to say that all in the garden is rosy for the German children, for indeed the German children do have more psychosocial problems than all the other groups of children with the exception of the UK SCID children, according to the Rutter A scale, but these problems have possibly been ameliorated, when compared to the UK SCID children, because of the system of family and social support to be found in Germany. In addition to the Rutter A scale for the parents, the Rutter B scale, paralleling the Rutter A scale, was used by the teachers of those children at school. One advantage of using the teachers as a second source of information on the children is that they are able to give a more objective assessment of the child. A disadvantage, however, is that they only see the child for limited periods, and often only see one side of the child’s character and behaviour. It was plain to see, however, that the teacher’s assessment did parallel that of the parents in that, as far as the teachers are concerned, the SCID children do have more psychosocial problems than the children in the Pot-Mees BMT and cardiac groups. The SCID children are particularly fidgety, restless and lack concentration. In addition, they are seen as fearful and fussy. However, the teachers do not rate the children as excessively antisocial or depressive, but they do rate them as having more antisocial and depressive tendencies than do the children in the other groups. One factor at play here may be the greater discipline in schools and less freedom that children may have to display their true characters, because of greater discipline from the teachers, school organisation and pressure from their peers. The assessments from the teachers are particularly useful because

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one of the criticisms of using the parents’ assessment is that they only have a handful of children to base their comparisons on. Using assessments from the teachers overcomes this criticism as they have many more children to base their comparisons for assessment on. Consequently to see the same patterns emerging with the teachers’ assessments as with the parents’ assessments gives cause for confidence in the overall assessment strategy. To sum up, it can safely be stated now that the statistics point to a group of children with many and profound psychosocial problems which, because of the similarities, must be linked to some event(s) in their lives. As the one connecting event is severe combined immunodeficiency and bone marrow transplantation, then one can point to this event as being at the root of their problems.

The Present Since this study was completed, there has been a follow-up study, and whilst the final report has still to be written, the results are very similar to the results from this study (Titman 2007, personal communication) This latest study followed up a cohort of children with immunodeficiencies who were treated by human stem cell therapy/BMT at the Hospital for Sick Children, Great Ormond Street, London. This cohort included several children from the first study. The background to this study included a study by Kramer et al. (1997) who looked at cognitive and adaptive behaviour following BMT, Phipps et al. (2000) who looked at cognitive and academic functioning in children following BMT, and Rogers et al. (2001) who looked at cognitive and behavioural abnormalities in children with ADA SCID. Kramer et al. (1997) started on the premise that children receiving a BMT are at risk of neurophysiological late effects because of the potentially neurotoxic drugs and total body irradiation. This, however, is not a problem with children who have BMT for SCID because they do not have total body irradiation and the drugs that they have are in much smaller doses and less toxic. From their study, they found that the children’s IQ and adaptive behaviour were lowered at the 12 month’s post-transplant follow-up. This study did include a cohort of children with SCID and despite the caveat above regarding the treatment, several of the SCID children in the study did have total body irradiation. Consequently, there was a lowering of IQ and adaptive behaviour, but by not as much as was found in children with other disorders who received the total body irradiation. Phipps et al. (2000), however, came up with a completely different result from their study. They concluded that BMT treatment, even with total body irradiation carries only minimal risk of late neurological problems in patients older than six years of age at the time of the BMT, but those younger than this, and particularly those children less than three years of age at the time they receive their BMT are at some risk of cognitive decline following successful treatment. The third paper underpinning the Titman (2007, personal communication) study looked particularly at children with ADA SCID (Rogers et al.

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2001). These children had been identified in the first study (Vickers 1999b) as being the group causing most concern as regards their behaviour. Their conclusions were that the cognitive function of these children is seriously affected and that they do have significantly high levels of behavioural abnormalities following bone marrow transplantation. However, they make the point that it is not so much the BMT procedure that causes these defects, but rather the metabolic problems that underpin this condition. For her study, Titman (2007, personal communication) used various Wechsler scales to measure intelligence, but to assess behavioural problems, she used the Goodman Strength and Difficulties Questionnaire (SDQ), which over the past few years has superseded the Rutter scales (Goodman 1997, Goodman et al. 2000). This is very similar to the Rutter A scale (Rutter et al. 1970) used in the study above. Titman (2007, personal communication) found that SCID children following BMT generally had lower than average scores on the measures of cognitive function and that they had lower IQ scores than did their non-affected siblings. Other findings were that there were:

r Differences between ADA and other types of SCID children, particularly in terms of behaviour.

r No relationship between age at transplant and cognitive outcome. r No significant relationship between the type of pre-transplant conditioning (to prepare the child for the transplant) and cognitive outcome.

r Higher rates of psychological difficulties compared to the general population.

r Specific difficulties in areas of peer relationships compared to children with other types of chronic ill health. The last two points in particular bear out the results from the first study, even though totally different data collection tools were used.

Conclusion This has been a look at the behavioural difficulties of children who have been successfully treated for SCID by means of a BMT and which included months in very strict reverse barrier nursing environments in hospitals. This was the first ever study to look at these children in such depth, and it highlighted many problems that the children still had to cope with. Although there were several children who still had long-term health problems, many of them were fit and healthy. However, the major problems identified by this study were psychosocial and behavioural ones. Before this study, no one had imagined that there could be these problems, but research since the first study has backed up these findings. It must be stressed that not all children who have a BMT for SCID will end up with behavioural problems – there are many mitigating factors that could alter this, but too many of the children do have behavioural problems, as described in this chapter, that it cannot be dismissed as a minor problem.

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Work continues to identify the problems, and hopefully to pinpoint the causes, so that therapies can be put in place to prevent, or at the very least, ameliorate them. Indeed, discussion with Penny Titman (2007, personal communication) has shown that attempts are being made to do just that. The point to bear in mind is that, although this was a study of one group of children, the findings may be relevant to other children’s diseases and therapies, where the conditions are similar – particularly the early hospitalisation and isolation for some length of time. The effects of hospitalisation and isolation could be more common than we imagine, and whilst mention has been made of studies into the psychosocial and behavioural patterns following treatment in other disorders, such as leukaemia, liver disease and cystic fibrosis, such studies need to be carried out with children in other similar situations, such as neonatal and paediatric intensive care. The remaining chapters will look at particular concerns that arose from this study, but will not be concerned with statistics. Rather they will look at how these psychosocial problems affect the children and their families – much of it told in their own words and pictures.

9

Anger, Aggression and Antisocial Behaviour in Children

Introduction This chapter is concerned with some of the behavioural problems that came to the fore during the discussions with the families and also from the drawings made by the children together with their comments. The key issues raised were anger and aggression. By means of statistics, the previous chapter has shown the extent of the psychosocial problems of the children. This chapter will consider how these problems affect the quality of life of both the children and their families. He’s a bit of a screw loose.

(UK mother of one child with ADA SCID)

Antisocial and Aggressive Behaviour One of the major issues that was identified from the study was that of bad behaviour – a response to the stressors inherent in the initial disorder and treatment, which can, in turn, create even more stressors. As was seen in the previous chapter, the Rutter A scale for parents (Rutter et al. 1970) produced the following mean scores for items associated with antisocial and aggressive behaviours in the SCID (severe combined immunodeficiency) children (out of a possible maximum of 2.00): Naughty Temper Destructive Fights

0.81 0.88 0.22 0.32

When compared with those mean scores for the children in the reference group, using a z test, the following z values were produced: 160

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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2.58* 3.61* 3.20* 1.50

* The first three items (indicated by an asterisk) are all significant at the 5% level (p < 0.05), whilst all four of them indicate that the SCID children have greater problems with anger and aggression than do the children in the reference group. These differences are echoed when the SCID children are compared with two of the other groups referred to in the previous chapter, namely the Pot-Mees (1989) BMT (bone marrow transplant) and cardiac groups. However, when compared with the Bradford (1994) group of children with liver disease, the differences between the mean scores – with the exception of destructive behaviour – are generally much less and are not statistically significant. There is a significant difference (p < 0.05), between the incidence of destructive behaviour between the children with SCID and those with liver disease, but in this instance, the children with liver disease are significantly more destructive than the SCID children (Table 8.2, p. 135). Turning to the Rutter B scale for teachers, the z values for three of the four types of behaviour present a rather different picture (‘temper’ is not included in the Rutter B scale for reasons discussed in the previous chapter). It has already been hypothesised that the differences between the Rutter A and the Rutter B scales in this aspect of behaviour may be due to tighter discipline and peer pressure at school, although, Pot-Mees (1989) reported that in actual fact there is not generally a close correlation between the two scales. Again this may be due to the factors mentioned above, namely discipline and peer pressure. We become different people in different spheres of our life and perhaps can only truly be ‘ourselves’ in our own home, where we feel comfortable. For example, given that many SCID children are more prone to being worriers, and they tend to be solitary children who are fearful and fussy (as shown by the statistics in Chapter 8), it is not surprising that in the rough and tumble of school life, they should become more withdrawn than at home where, because of the past experiences, they may well assume a more favoured and special position. This point was made by the mother of one UK girl who, when asked if her daughter thinks that she is special, replied ‘Everyone tells her she is. Every single person says, “oh, you’re so special”. You get to the point where you’re so used to hearing it. I mean, it’s always . . . she goes coy, but she knows it. And she’s got everybody where she wants them anyway’. This aspect of the position of SCID children within the family is also beautifully summed up by another UK mother, ‘I think she feels a lot more confident when I’m around. Mind you, the teachers in school say she’s very confident. Like her behaviour is very good. She’s not naughty or cheeky to them. Saves it all up for me, yes. They have no problems with her, you know’.

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This aspect of the child being ‘special’ at home was discussed by the mother of one of the UK boys in the study who explained, ‘Yeah, well he knows, like, when he was in hospital an’ that, especially when he’s the only one. I’ve always said to him, ‘you’re special’, an’ so he does believe he’s special’. Even siblings are aware of this favoured state of the SCID children, as the sister of one German boy pointed out, ‘He knows . . . he knows if he does something . . . he knows he’ll get away with it’. Outside of the Rutter scales, during the interviews with the parents, they were asked about two aspects of bad behaviour – whether their child had a temper and whether they were naughty. Each of these behaviours will now be examined more closely.

Disobedience The parents were asked if their child was ever disobedient or naughty. Thirty-one out of 34 (91%) said that their child was naughty at times, but most of them accepted this as being normal for children. For example, the mother of one UK child said ‘She’s fourteen. She gets up to the horrible things that they do at that age, but basically she’s quite a good girl’. This point was made by the parents of other children in the study when they were asked if their child was ever disobedient of naughty, for example,: ‘Yes, I think he’s totally normal. Like all children, no?’ (Mother of a German boy) He must be just like all the other children, but it rarely happens. (Father of a German boy) Apparently the headmaster doesn’t think she’s any worse than any other year six eleven year old. (Mother of a UK boy) Yes, a lot naturally, (but) he is fine.

(Mother of a German boy)

This father of a UK child was really quite proud of the behaviour of his son, ‘We’ve caught him doing things like that . . . I mean, doing the usual things as kids do . . . peeing up against other people’s walls as I walked round the corner. He realised straight away he was doing wrong. As long as he’s doing things like that, I’m happy. I would hate to think he was always a little angel with a halo above his head’. Even siblings accept that the naughtiness of the child who has survived the treatment for SCID is not particularly different from the expected norm, as was shown in this conversation about a German boy, when the family was asked if he was disobedient or naughty. His mother laughed and just said, ‘Both’, whilst his sister chipped in with, ‘Like every child’.

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Some parents, however, do have some concerns about how far their child’s behaviour can deteriorate, even though they are not always naughty. He’s not always an angel, I have to say. He can misbehave, especially when he has to do something. (Father of UK child) Most of the time it’s backchat. But if then, like, if he’s going to do something an’ I told him, ‘No’. He’ll do it knowing that he’s going to get in trouble, you know. And then once I’ve told him off for doing it, because I’ve just told him not to do it, that’s when I get the kicking the walls an’ all that again, you know. (Mother of UK child) She becomes cheeky – She can be devious.

(Mother of German child)

This UK mother has mixed emotions about the behaviour of her daughter, her heart wanting to believe that it is just because of her age, but her mind accepting that her behaviour may have other causes: In a thirteen year old, it’s not disobedience or naughtiness, you know. It’s a different thing. A different phase. If you were saying, is she naughty . . . on that basis I would say probably a little more than the average child, but not excessively naughty and disobedient. Natural rebellion in a thirteen year old. Some of it you could say, ‘Oh yes, it’s because of — in the past’. But when you talk to others, you have to remember to put these things back into perspective, you know. You think it’s because she’s —, but in fact, if — hadn’t had a transplant . . . . . oh, it’s just because she’s thirteen. At the moment she has this thing about boys which never worries girls . . . worries us tremendously . . . and wanting to mix with the wrong crowd. It was a big problem earlier in the year . . . she was part of the wrong crowd, which was not good for her. She really couldn’t cope. That was — . . . immature I think. Other parents tried to explain away their children’s behavioural problems by looking for external causes: Me an’ her, when we’re on our own, oh she loves it, you know. Just peace an’ quiet, an’ just me an’ her. Everythin’ is, you know, hunky dory. It’s just when other people come an’ they interrupt me an’ her being me an’ her. She doesn’t like it, you know. She just doesn’t like it at al. (Mother of UK girl) Sometime he starts now, yes. If his mother tells him something he responds to her, yes. And boom, boom, yes! He picks words from children from school, you know. Sometimes not dirty words, but things that I don’t like, you know. Things like . . . he’ll tell you ‘you are like a pig’, you know, ‘you are a pig’! That’s the only thing for him. (Father of UK boy) But when she doesn’t get her own way, she’s just like a two-year old really. Like . . . tantrum, and totally self-centred behaviour. She’s very good as well. You know we have to follow her brothers around . . . playing tennis and

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as choristers . . . and she’s awfully good. She just sits through a cathedral service and she’ll sit through the concerts. And you’d expect her to be naughty, but she’s not. She’s only really naughty when she’s very miserable about something. (Mother of UK girl) Finally, there are some children whose behaviour is very worrying to the parents, for example, these three children, as related by their mothers: I’ve gotta take him though, say, like when we was through in outpatients there, an’ a wee girl was sitting on a sort of horse . . . that wee girl was going after the toy — had been playing with, so — stopped his horse and went up and grabbed it. Actually I’ve gotta take him out of the situation. I’ve had to take him out of the toy, like, an’ sit him down an’ talk to him. So then when he goes out he’ll say sorry an’ play quite happily. He just loses it, so I’ve just gotta get him away, talk to him an’ bring him back. (Mother of UK boy) Awful naughty . . . very naughty. Horrendous. She does horrible things to the dog to get my attention, you know. She’ll literally pull his tail, you know . . . horrible things. Hides his food and things like that. Poor dog gets the end of it. You tell her to do something and she does the complete opposite, you know. Tell her to wash her teeth and she’ll go up and mess with the water and have the floor wringing wet. Tell her to go to her own room and she’ll go to the girl’s room and rob all their make-up, you know. Things like that. She’ll do the complete opposite. (Mother of UK girl) She won’t do what you want her to. She just starts being naughty. She’s just naughty as in . . . it’s not a problem . . . it’s just attention seeking. She’ll just get louder and louder. Not screaming. We’ll say, ‘Come, will you do this for me?’ ‘Alright mum, I’ll do it.’ You come back ten minutes later and say, ‘Have you done this?’, and she’ll say ‘In a minute’. You can carry on like that all day, and she’ll just sit and ‘alright then’, but she won’t do it. And then, I’ve been doing the beds this morning, and she’s cut a little hole out of her duvet. She’s cut a brand new pair of curtains. Her dad fixed the rail, and she’s actually pulled the nails out of the rails. The duvet – she’ll get into trouble because she’s not long had it, and she’s been saying for ages that she wants a new one. And I think that’s probably why she’s done it, because she wanted new curtains and a new duvet, and the new curtains weren’t what she wanted. So, I think that’s probably why she cut those. The duvet just had a hole cut out of it. I’d say she’s naughty, but not . . . naughty but expensive (gives a rueful laugh). They’re expensive to replace at some stage, but I know some people who’ve got children who throw things around and are violent, or whatever. (Mother of UK girl) The child with the most aggressive and antisocial problems, however, is one of the children with ADA SCID who gave much cause for concern. When asked if her son was ever disobedient, this UK mother’s short, but heartfelt reply was, ‘Every day! Every day!’ She then went on to try and explain her concerns over him: ‘He’s got a weird sense of humour.

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He laughs, doesn’t he? He laughs when he’s done something naughty. You know — (his brother) is in there. He’s thrown a cupful of water over — (his brother), and laughs. He’s got a good sense of humour alright. If we bring him out now and you’re sitting there, and there’s a glass of beer on the table – he’d throw it over you and break his heart laughing. He’d think that’s funny’. There is a much darker side to this boy’s behaviour, however, as first of all his father, and then his mother confirmed: Father: ‘He’s got a tendency for pulling your hair out’. Mother: ‘He’s got a fascination for pulling your hair . . . strangling you . . . stabbing you with pens’. The tone of the discussion turned even darker, as this mother proceeded to enlarge upon his behaviour: Errm . . . naughty. When he does things . . . he sees no danger in anything. Errm, you know, knives and things like this. Errm, generally he sees no danger, I mean, he sees no danger. It’s more he can’t help it, Peter. It’s not deliberate . . . it’s just his mind doesn’t . . . I think it’s opening up, he’s just in . . . at the moment it’s a matter of him twisting around . . . Anything could set him off. He can go for hours and can be very good, but something . . . he’s twisting . . . I mean, upstairs now, he was reading a book, and I went up and he was . . . everything . . . trying to plug into the socket in the wall. At one stage in the interview, this mother came out with her main worries over his behaviour: My son’s behaviour is very worrying. You feel like you’ve got, errm . . . the next axe murderer in the future (laughs ruefully). That is the truth . . . he’s quite dangerous. I don’t know. He picks up knives, doesn’t he? It’s very worrying. He seems to be inspired by anyone he sees. Like he was watching one morning here. Errh . . . no . . . his mind’s twisted a little bit at the moment. Errh . . . like he was watching a section, errh . . . childbirth on the tellie one morning, and they were doing a section on the lady, and he came in and he was just sitting there and said, ‘you’ve got a baby girl in your tummy’, and he took a knife out of the kitchen drawer . . . just wanted to cut. You see, his mind . . . his mind when he’s watching something, doesn’t go in right. He takes too much in. This particular boy, it must be stressed, is an exception, as can be seen from the above quotations. Although the behaviour of SCID children can be a problem, in the main, most of it is the sort of behaviour that other children indulge in, such as backchatting, being disobedient, sibling rivalries and throwing things. However, this boy had a particular type of SCID with metabolic problems, and most of the children with this type of SCID appeared in the study to have severe behavioural problems. This finding has been supported by the recent research undertaken by Dr Titman and colleagues at Great Ormond Street Hospital, London (personal

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communication 5 July 2007). Consequently, it appears that this particular SCID predisposes to aggressive behaviour as a result of the physiological abnormalities produced in this disorder. Although, in common with all other types of SCID, the effects of the treatment and the environment in which they live for the early part of their lives, also need to be taken into account. Scenarios that the parents described concerning the children being naughty, ranged from it being ‘normal behaviour’ to mixing with the wrong crowd, but the most common cause was the child not getting his or her own way and not getting enough attention. Mostly the children appeared to pester for attention, although some of them did lose their temper, become very naughty, or become very stubborn. Quite a few of the children can become very stubborn and uncooperative for a variety of reasons. For one German boy, it was his reaction to stress, ‘Uncooperative, yes. No longer any enthusiasm – stubborn, yes’ (Mother). For one UK girl it was her determination to order her life as she wants which made her appear stubborn (a possible hangover from the total lack of control she had over any aspect of her life whilst having her BMT and the time spent afterwards in hospital, plus her having to have regular monthly intravenous immunoglobulin infusions). As her mother discussed, ‘She’s quite constructive in her arguments, and if you won’t give in to her, she’ll go and get it herself. She’s always trying to do things herself, but she still wants you there. She’ll try for herself first. She’ll start it off, or she’ll, ‘I want to do it!’. She likes to choose her own clothes to wear. She can be quite stubborn in that. You have to ask her quite a lot of times to do it. You have to keep at her. If she wants to do it, she’ll be out and she’s gone and done it before you . . . . . .’ The same applies to this German boy, who according to his mother was ‘. . . very determined and hates being beaten by anything’. The picture that has emerged from the parents is one that shows that, although their children may exhibit more poor behaviour than do children who have not had SCID, their behaviour, with one or two exceptions, was not extreme. Again, the difference between the UK and German SCID children showed that the German parents considered their children to be, for the most part, normal in their behaviour, unlike the UK parents who tend to be more critical. This may be due to the fact that the German parents were mainly more optimistic about their children, for the reasons discussed in the previous chapter.

Loss of Temper During the interviews, the parents were asked if their child ever lost his temper. Thirty-one out of 35 parents (89%) answered in the affirmative. Again, for some of them, this was considered to be normal, as mentioned by these four mothers (all German mothers):

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She is completely normal. Yes, but not extremely so. I think it’s normal. I wouldn’t say losing her temper is a problem. She is actually good-tempered with me; she is also the same in school. Definitely she is a good-tempered child. Most of the parents, however, did perceive their child’s bad temper as a problem, as the mother of this UK child confided, ‘When I tell him to do something, he . . . stomps up the stairs, slams the door. He’s shouting and he’s shouting at me; screaming and then storming upstairs into the bedroom’. The mother of this German child was really quite concerned about her son’s temper tantrums, ‘He is very destructive. Mostly he throws things against the wall, against the door’. (Making a throwing gesture) Shouting and making a lot of noise seemed to be the most common manifestations of the children’s temper, as described by the parents of these children: She will ‘slam the door, shout, stamp’.

(UK mother)

Then she breaks everything. Anything is a possibility. She always wants to be the centre of attention. Loud! A loud voice, very good voice. (German father) Everything is possible. Her shouting, but her crying is not so . . . not crying as such. Crocodile tears. No emotion. (German mother) He shouts at people.

(UK mother)

I’m surprised you don’t hear her wherever you are. She has awful tantrums, but she’s getting better. (UK mother) He shouts, he slams doors, stomps about and growls. Sometimes in real anger he cries. (German mother) These UK parents described other manifestations of their children’s temper tantrums, including throwing themselves and other objects around. He throws himself on to the floor, or he kicks things. I usually try to just ignore it. In the beginning I just had to ignore it. He throws things. She’ll throw things. She’ll slam the door, or else she’ll lock the bathroom door and let nobody in. If he’s doing something an’ he’ll lose his temper, right, he’ll just throw it away. He loses the head very quickly, but then, errm . . . he doesn’t lie down

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an’ throw his feet . . . things like that, an’ scream an’ shout. He just loses the head, like, throws . . . throw it to the side an’, you know, he doesn’t do it a . . . maliciously. He doesn’t, like, throw it at anybody else an’ that. He’ll just, like, throw it to the side an’ walk away from it, you know, sort of thing. Other reports of temper manifestations were reported by other parents: If you say ‘no’ to him, he’ll cry. He will get bad-tempered, but not to the extreme that he would throw himself to the floor. (Mother of UK child) He gets the hump and storms off. He’s very argumentative. (Mother of UK child) — is so pale with it (temper). He gets so pale. (Mother of German child) Reasons given by the parents for their child losing his or her temper were mainly a combination of frustration and not being able to do what they want, as for example, with this mother from the UK when describing her daughter’s behaviour when she is in a temper: ‘Not getting her own way, being told off. Really she’s got a temper and sulks to get her own way.’ Other reasons for their children were given by some of the other UK parents, with frustration being a major cause: ‘When he doesn’t get what he wants.’ ‘When other boys tease him . . . when he wants something. Frustration, yes . . . when he wants something.’ Not being allowed to do what he wants to do. And, errm . . . he’s watching something and when we ask him to sit up at the table he loses his temper, or if he’s been playing in the garden and it’s time to come in, we might tell him to clear the toys away . . . he loses his temper. You know, anything that’s not enjoyable really. Being made to do things about the home. The tantrums were awful for years . . . enormous frustration. Frustration! Sometimes the children lost their temper when they felt that they had not been treated fairly, as described by these two UK mothers: Only when she’s accused of doin’ somethin’ she hasn’t done, which any kid will do. If she feels that she’s been hard done by . . . that will make her become angry. She’ll tell you it’s bravado because she’ll get into trouble.

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Whilst, in terms of how their episodes of bad temper manifest themselves and the reasons for them occurring, SCID children really do not differ much from other children, except perhaps in the number of times that a loss of temper occurs, some children do have particularly bad problems. For example, one UK mother summed up her son’s behaviour quite succinctly as, ‘Wild! Totally wild!’. But even that boy’s problem temper pales by comparison with temper tantrums of the one UK boy. When asked if her son ever lost his temper, his mother replied, ‘Oh yes . . . fifty times a day . . . oh, about fifty times a day, yeah’. When this particular boy lost his temper, he would scream and throw things. In answer to the question concerning the causes of his loss of temper, his mother told me, ‘It doesn’t have to be anything specific . . . just if he wants to. It doesn’t have to be anything specific to make him lose his temper. Sometimes you wonder what you’re rearing’. Loss of temper appears to be a major concern of the parents, possibly because it impinges on the quality of life of the family, and also leads to awkward social situations when out of the house. Most of the temper tantrums appear to be the result of frustration – of the children not getting their own way; of not being allowed to do what they want. Because many of them are brought up to consider that they are special (as indeed they are to have survived their illness and the treatment for their illness), and because they have been ‘spoiled’ for many years (again understandably so), then many of them they feel that that gives them carte blanche to do as they want. This, allied to their poor socialisation skills as a result of the disorder and their long-term hospitalisation and isolation, leads to much frustration when they feel that they are being thwarted, and this in turn leads to a loss of control of temper.

The Children’s Drawings The children, whilst not putting their anger into words, showed through their drawings some of the frustrations, the aggressive feelings, the anger that they had. One has to be careful when interpreting children’s drawings, but there is evidence of anger and of aggressive impulses being suppressed which run as a common thread throughout many of the drawings, no matter the age of the child. For example, fingers that are enclosed by a loop can indicate a wish to suppress aggressive impulses. In addition, many of the drawings were executed in a very aggressive, rapid manner, with no apparent care taken with them, which in itself may indicate either a suppressed anger or an impulsive and uncaring nature. The following are just a few of the drawings which serve to reinforce both the parent’s words and my own perceptions of the anger the children often felt. It has, however, to be pointed out that this is not unique to children who have been successfully treated for SCID, but rather is quite common with children who have a chronic disorder.

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Figure 9.1

Drawing by UK boy of his family (eight years old).

Several factors stand out from this drawing of the family (Figure 9.1). The first is that all the people have very small arms, which are just depicted as ‘blobs’. This could indicate feelings of inadequacy or helplessness, both of which could lead to anger. In addition, this boy has drawn himself with the same amount of hair that the rest of his family have, whilst in real life, he had no hair at all, a fact that he acknowledged when he drew a picture of himself alone. Another interesting phenomenon is that he drew a tree, as he also did in his self-portrait, even though he was not asked to do so. Trees, according to Oster and Gould (1987 p. 37) are believed ‘. . . to be associated with one’s life role and one’s capabilities in obtaining rewards from the environment. Generally tree drawings have been considered especially rich in providing insights concerning “life content”, that is, displaying accurate biographical situations and/or offering personal characteristics of the person being examined. The tree seems to reflect longstanding, unconscious feelings towards the self and these feelings tend to reside at a more basic, primitive level. The tree also appears to be an easier object than the person or house on which to project negative self-feelings . . .’ This boy has put exaggerated emphasis on the tree’s trunk which may represent emotional immaturity. The fact that there is no ground line present shows that he is vulnerable to stress, and the excessively dark and reinforced shading represents hostile defences and/or aggressive behaviours. Finally, the very immature drawings show a degree of impulsiveness and uncaring, whilst there is an aggressive ‘feel’ to the picture (as there was to his self-portrait) which suggests a hidden anger. All this is in line with comments about this boy made by his parents and his teacher.

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Figure 9.2 Drawing by German boy of his family (11 years old). Previously published in Vickers P.S. (2006) Communicating with children and families. In: Peate I. and Whiting L. (eds) Caring for Children and Families. Chichester: John Wiley and Sons, pp. 25–48 (p. 42).

This very sophisticated drawing below shows the anger that this boy, who is deaf, may be feeling (Figure 9.2). First of all, the boy himself (to the left of the picture) has drawn himself with one arm across his chest which can represent hostility and suspicion. The large enclosed fingers may show a wish to suppress his aggressive impulses, whilst the eyes staring off to the left are indicative of a watchful and wary nature. Of particular interest is the drawing of his mother. She is dressed in red which is often thought to be associated with anger or danger. She also has a big red mouth, which indicates immaturity as well as oral-aggressive feelings. In addition, she has what appear to be deep red claws/talons, although they could possibly just be painted nails – they could, however, indicate aggression and hostility. He has also placed his mother furthest away from himself, so he may have had particular hostile feelings towards his mother, or have felt most alienated from her. Although, in terms of draughtsmanship, this was one of the better drawings of the children in the study, there is still a feeling of energy and anger, something it has in common with the majority of the children’s drawings. This drawing (Figure 9.3) came about when the girl was asked to draw herself doing something that she enjoys. However, she decided that she wanted to draw me (the interviewer) instead. This drawing, unlike the

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Figure 9.3 Drawing by German girl of the interviewer (six years old). Previously published in Vickers P.S. (2006) Communicating with children and families. In: Peate I. and Whiting L. (eds) Caring for Children and Families. Chichester: John Wiley and Sons, pp. 25–48 (p. 42).

ones she drew of herself and her family, has facial features, and there is an overall feeling of anger, aggression and hostility. This is particularly the case with the pointed teeth and the staring eyes that indicate overall fear. The ears are emphasised and have eyes in them, which can be indications of suspiciousness and paranoia. During the interview with her parents, at which she and her siblings were present, this girl came across as a delightful, bubbly child, so it was quite a shock to see her drawings – especially this one. In her general behaviour there was no hint of this anger and hostility, so perhaps she associated me (as a nurse) with her treatment and her general condition. It is interesting to note that she now appears to be quite healthy, albeit with a severe speech problem. Her aggressive and hostile fears, for now apparently latent, are similar to those displayed by many of the SCID children and which will need to be resolved at some stage by working their fears out within their families, or with professional help. These three drawings depict some of the anger and hostility that these children were striving to hide, but which manifested as temper tantrums. As already stated, caution must be the watchword when attempting to analyse children’s drawings, but knowledge of the children, their families and their backgrounds, can help to give confidence that the interpretations are appropriate and correct.

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Conclusion This chapter has considered how the children who have survived bone marrow transplantation, early hospitalisation and long-term isolation for SCID express their anger and frustration afterwards, as described by their parents, and also by the children themselves in their pictures. Anger against parents, professionals, themselves and their situations are common feelings amongst children who have a chronic disorder; this goes some way to showing that SCID should be perceived as a chronic disorder. Frustration can occur for many reasons, but, as many parents pointed out during the interviews from this study, it can be a catalyst for outbursts of anger and for temper tantrums. Thus, frustration can be born of an unconscious belief that they have missed out on a normal infancy and early childhood through ill health and through the isolation – remembering that these children may be prevented from mixing with children outside of their family until they commence Kindergarten or school (once they have, of course, successfully had all their childhood immunisations). There are also frustrations that arise because they are unable to relate to their peers, and even to members of their own family – feelings that can develop so they see themselves as ‘outsiders’ in their world (see the chapter on attachment and alienation). There are also the frustrations that many of them feel because of their continuing ill health, and because they are always being reminded of their early childhood (many of the parents find it difficult to accept that their children are now fit and well. These frustrations can lead to many outbursts of anger and aggressive behaviour, resulting in antisocial activities in the children. Turning to other situations of early hospitalisation and isolation, several researchers have explored the possible future antisocial behaviour risk factors of perinatal complications and environmental adversity, including Arsenault et al. (2002), Piquero and Tibbetts (1999) and Raine et al. (1997), although there was some inconsistency with the results as reported by both Arsenault et al. (2002) and Laucht et al. (2000). Beck and Shaw (2005) felt that this inconsistency of results may arise because these studies focused on the influence of perinatal complications on the development of serious antisocial behaviour in adulthood, i.e. some years later, when it is always difficult to identify the source of the causes. In order to overcome this, Beck and Shaw (2005) studied a cohort of boys from birth to ten years of age (see below). There was earlier precedence for this, because several researchers (Broman et al. 1975, Drillien 1964, Werner et al. 1971) had found that, within the context of family adversity, an increased risk of child antisocial behaviour was linked to high levels of perinatal complications. In their study of 2002, Arsenault et al. (2002) demonstrated that a combination of perinatal complications that posed imminent harm to the baby/infant led to higher rates of physical aggression at the ages of 6 years and 17 years. In terms of adult antisocial behaviour following perinatal complications, Raine et al. (1994, 1997) found that it was boys who experienced perinatal

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complications as well as rejection by their mothers who were most likely to become violent offenders and adults. There is much research looking at the quality of early parental care and the development of early-onset antisocial behaviour, particularly parental unresponsiveness and rejection (Campbell et al. 2000, Shaw et al. 2003). Other studies (Bost et al. 1998, Wakschlag & Hans 1999) have shown that the responsiveness of parents to their infants, particularly in relation to their emotional availability and to the sensitivity of the parents to their infants’ social cues are associated with positive social competence and behaviour in their children, whereas other studies (Shaw et al. 1994a, 1998b, Wakschlag & Hans 1999) have demonstrated that a lack of parental responsiveness during this period of infancy is associated with more negative traits, such as antisocial behaviours in the children at a later date. Other studies (Campbell et al. 1996, Dishion 1990, Dodge et al. 1994, Younge et al. 1996) have linked the development of antisocial behaviours at a later date with a combination of parental rejection/parental unacceptance of the child and controlling parenting practices. These parental difficulties, themselves, can arise due to a combination of ‘family stressors’ which include the problems with bonding with the child, financial problems, parental separation (including when a child is in hospital), sibling concerns, coping abilities etc. All of these could have been present with the children in the SCID study, but they can also apply when the infant is in neonatal or paediatric intensive care, where there is chronic and serious ill health in the early years of life, as well as where there is early and long-term isolation. These situations can lead to lack of acceptance of the child by the parents and other family problems centred on the sick child; there have been many studies that have demonstrated that as the number of these family stressors increase, so do the problems and difficulties of the child being able to adjust socially and psychologically to their situation (Deater-Deckard et al. 1998, Rutter et al. 1975a, 1975b, Sameroff et al. 1987, Shaw et al. 1994b, 1998a). Beck and Shaw (2005), looking at healthy boys’ antisocial behaviour, point out that antisocial behaviour can result from a negative psychosocial environment. They cite the work of Raine (2000) who proposed a biosocial model that states that both biological and environmentally related risk factors can contribute to the development of antisocial behaviour. In particular, Beck and Shaw were interested in the influence of complications after birth (in the perinatal period) and the environment that such complications necessitate, or are caused by, as risk factors for the development of antisocial behaviour in boys. The actual risk factors that Beck and Shaw were interested in, as well as the actual perinatal complications themselves, were the effects of parent rejection and family adversity. From their findings, Beck and Shaw (2005) suggested that perinatal complications are related to antisocial behaviours in boys, but only when there were increased family adversity problems. They found that there was a significant three-way interaction that led to antisocial behaviours in boys, namely perinatal complications, parental rejection, and family adversity (or problem stressors). In addition, as it has been hypothesised (Hawkins

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& Trobsta 2000, Raine 1997) that perinatal complications are believed to lead to neurological impairment in the frontal lobe (the part of the brain believed to be involved in executive functioning) this may also have a part to play in the development of antisocial behaviour. This is because deficits that occur in the executive functioning have been associated with problems with impulsivity, verbal expression, as well as an understanding of normal socialisation (Raine 2000) – all of which are part of the antisocial behaviour (Beck & Shaw 2005). Executive functions of the brain are those neurological capacities (most commonly linked to the frontal cortex of the brain) that guide and help to control a person’s complex behaviour – it is linked to the planning, decision-making and response control roles of the brain. Beck and Shaw (2005) conclude, from their study, that there are indications that children who experience severe perinatal complications are the children who are most vulnerable to the effects of an adverse home environment. In addition, as Martin (1997) points out, it is now acknowledged that the mother–offspring relationship (using mother as shorthand for the main carer in the life of that child) is pivotal for all babies and young children in terms of physical and mental development, then it is not surprising that any disturbances in this close relationship can have widespread repercussions, not only for the child as an infant, but also much later in life. This study by Beck and Shaw (2005) above was only concerned with boys, and this is possibly because generally males are much more likely to display more overt aggression than females (Lansdown 1984). Indeed, Lansdown says that between the sexes, this is the most often noted behavioural difference. He goes on to point out that boys not only show aggression, but that they also act as a catalyst and cause aggression much more in others, as well as retaliating aggressively far more than do girls. This, of course, was not the finding from the study of children with SCID (previous chapter) where girls were found to have more aggressive tendencies than the boys. It was earlier hypothesised that this may be due to the boys not being able to fulfil their perceived role and so their frustration may lead to depression and anxiety as opposed to aggression and antisocial behaviour. Although, as discussed above, there are some notable exceptions to this, particularly where children with ADA SCID are concerned. However, with that caveat, it is still apparent that the children with SCID do run counter to the accepted thinking in relation to behaviour of the sexes – this is just one of the many interesting themes to develop from the study that will require further exploration and research at some later date. Dr Titman’s study with a larger group of children with SCID (personal communication 2007) found that there were many examples of challenging behaviour from the children and that there were higher rates of psychological differences when compared to the general population. This is supported in the research by Rogers et al. (2001). This latter study (Rogers et al. 2001) of children with ADA SCID detailed higher than normal levels of hyperactivity and attention deficits as well as abnormal patterns of behaviour, particularly aggression and social problems. The authors of this paper (Rogers et al. 2001) also note that the behaviour of the ADA SCID

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children was more abnormal in the older children, which may be due to the progressive nature of the condition, but also suggest that it may be due to the parents having different expectations and perceptions as the child becomes older. In other words, this poor behaviour may be acceptable, and accepted in the younger children because of all they have just gone through with the transplant, but that the parents perceive it to be much more of a problem when the child is older and bigger. Interestingly, Pot-Mees (1989), in her seminal study, found that, of the children having BMTs for a multitude of problems (but not SCID), seven children were classed as having a behaviour disorder post-transplant, five of them had emotional and neurotic behaviour disorders (such as school phobia, depression and misery, problems with peers, overdependent behaviour and sleep disturbance), whilst only two of them displayed antisocial behaviour disorders (including aggressive, disobedient and rebellious behaviour). Unfortunately, Pot-Mees did not look at the gender of the children in each of these groups, so it is impossible to say if they followed the same pattern of aggressive versus neurotic behaviour problems as normally expected, or the pattern shown to occur with the SCID group of children. In conclusion, aggressive and antisocial behaviour was seen to be a problem for quite a few of the children in the SCID study, especially some of the girls, but this seems to contradict studies of other children undergoing similar experiences in terms of initial acute ill health, early hospitalisation and long-term isolation, followed by continuing chronic physical and/or psychological ill health.

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Introduction Apart from the antisocial behavioural aspects of psychosocial functioning discussed in the previous chapter, the children with SCID (severe combined immunodeficiency) also scored highly on both the Rutter A and Rutter B scales for the depressive and neurotic behaviours (Table 10.1). For example, on the Rutter A scale, a comparison of the children from the SCID group with the children from the four other groups – the reference group, Pot-Mees’s BMT (bone marrow transplant) and cardiac groups and Bradford’s liver disease group – a z test, showed significant differences (p < 0.05) between them for most of the items of behaviour. In almost every case the SCID children demonstrated more serious problems with these behaviours than did the children in the four other groups (Table 10.1). Indeed, compared to the children in the reference group, only two of these behaviours are not significantly different – being disliked and sucking their thumbs and fingers. Sucking thumbs or fingers is the only behavioural trait which the children in the Pot-Mees BMT group have a worse mean score than the SCID children, although the children in both these groups are equally fussy. Possible reasons for this have already been put forward in Chapter 8, namely the cleaning and infectious precautions that take place in these units from the beginning to the end of the hospital stay In only 4 out of 11 types of behaviour are the differences not significant at the 5% level between the SCID children and the children in the cardiac group, but again, in none of these behavioural types do the cardiac children have more problems than the SCID children. As always, there are closer correlations between the SCID children and the children with liver disease than there are between the SCID children and the children in the other three groups, but even here, within this Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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Table 10.1 Rutter A Scale – Comparison of Depressive and Neurotic Items Between SCID Children and Children from the Reference Group, the Pot-Mees BMT and Cardiac Groups, and the Bradford Group of Children with Liver Disease, Using a z Test Rutter A scale Item

Reference

BMT

Cardiac

Liver

Worries Disliked Solitary Unhappy Bites nails Fearful Fussy Sucks thumb Unsettled Restless Fidgety

−3.00 −0.50** −2.87 −2.67 −2.18 −3.07 −2.67 −0.77** −4.14 −3.04 −3.07

−2.93 −1.90** −4.27 −1.08** −3.06 −3.47 0.00** +1.31** −1.79** −2.56 −3.14

−2.21 −1.10** −4.40 −1.42** −2.59 −2.13 −2.25 −0.85** −2.50 −1.12** −2.21

−2.43 +1.50** −2.13 −0.17** −1.12** −1.33** −1.75** −2.15 −0.07** +2.24 +0.14

Items marked with a double asterisk (** ) are those behaviours in which the difference between the SCID children and children from the other groups is not significant (p < 0.05).

depressive and neurotic range of behaviours, there are only three types of behaviour in which the children with liver disease demonstrate more problems, namely being disliked, being restless and being fidgety. So, from Table 10.1, it can be seen that the SCID children do have major depressive and behavioural problems as compared to children in the other groups, and particularly when compared to the children in the reference group. However, this chapter is not focused on just the statistics; rather it is more concerned with letting the children and the parents express themselves in words and pictures. The umbrella term ‘neurotic behaviour’, as can be seen from Table 10.1, can encompass many varied individual behaviours including, depression, obsessive behaviours and anxieties. Similarly, there are many varied reactions to these behavioural states in terms of both experiencing them oneself and to witnessing to them in others. This chapter attempts to gain some understanding of what these disorders mean to the children and families.

Neurotic Behaviour As with the antisocial behaviours discussed in the previous chapter, the parents of the SCID children were asked about their child’s behaviour within this range of depressive and neurotic behaviours. The main question asked was, ‘Does your child ever get worried or anxious?’ Twenty out of 33 parents (61%) replied that their child did become worried or anxious.

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The ways in which the children manifested their anxieties and worries were many and varied, including becoming withdrawn and uncommunicative, sleep problems, biting their nails, crying and becoming aggressive, for example one UK child was said by her mother to be a ‘nervous wreck’. Many reasons were also given for their children becoming worried, including:

r r r r r r r

parental problems being alone in the house being ill memories surfacing novel situations people and objects (the most common reason) concerns at school

As one UK mother said to me, ‘Yes, it really is to do with school, isn’t it? Those are the kinds of things that worry him’. Amongst the German families, one mother was particularly concerned about her daughter. As she reported, ‘Then I believe, no I am completely positive, she actually never laughs, never has fun, always she is so worried. She is very nervous with new people. If she goes to other people she worries. She worries when she is at the doctors. I have to always support her. She is nervous about going to sleep. She has a great deal of anxiety, and she also worries that she cannot do anything’. According to the mother of another German child, he had anxiety when he was trying something new, as well as when he was with other children: ‘He is anxious being with other children’. Another mother of a German child believed he was anxious much of the time, but particularly when he was ill: ‘Worried, cautious, yes. Overall he is rather . . . rather anxious and cautious, especially when he is ill’. Surprisingly enough, another German boy’s worries were not centred on himself, but rather on his baby sister, as his mother explained when asked if he ever got worried, ‘About her, yes (indicating his baby sister). He grabs her and he carries her around. Or he comes if he can’t reach her and gets his Mom or his Dad . . .“Come on!”’. Perhaps this child was concerned that his sister may have to go off to hospital like him, or, because he was watched over very carefully, perhaps he thought all children should have the same attention. The worries of one of the children in the UK were also centred upon someone else, in this case his parents, as this little excerpt from the visit shows. His mother was asked about him being worried or anxious, she replied, ‘Well it’s sort of, like, we’re splitting up, and since then . . .’. Her son then chimed in with ‘Second time!’ His mother came back with, ‘I told you he’d tell you. He has been very anxious lately. He always wants to know when his dad’s coming in and what’s happening, and just crying a lot. And he’s very clingy to me as well’. The drawing of his family that this boy produced illustrates well his feelings about his parents (Figure 10.1). He has drawn himself between his

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Figure 10.1 One UK boy’s drawing of his family (eight years old). Previously published in Vickers P.S. (2006) Communicating with children and families. In: Peate I. and Whiting L. (eds) Caring for Children and Families. Chichester: John Wiley and Sons, pp. 25–48 (p. 37).

parents as if he feels he is the one thing that will hold them together, and his use of interconnecting lines crossing one another shows that he wants to keep the family together. During the visit, this boy was very chirpy because his father arrived for part of the interview, but, according to his mother, ‘It’s just like, he sort of gets something on his mind, he won’t let go. He’ll pester and pester, and he wants to know the truth about everything’.

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Worries Concerning Novel Situations One of the older survivors of SCID in the group, a UK girl, herself admitted that when faced with something new, ‘I’m nervous’, she said, and her mother continued the theme, ‘She’s very worried about doing anything, or going anywhere on her own. I don’t think she’ll ever leave home. I don’t think she’ll ever. . . . Not home, home, but I don’t think she’ll ever leave the area. I’ll be very surprised if she does. The only time she’ll leave home is when she finds herself a husband. (laughs) No; but she wouldn’t, would she?’ Another of the older children from the UK also found new situations difficult to cope with as her mother related when asked what made her daughter anxious, ‘Changes of circumstances is possibly top of the list. I mean, she just doesn’t like a challenge. She wants things to stay the same’. This girl’s mother then went on to enlarge upon this theme – ‘She’s an internal worrier, I feel. She doesn’t tell me of her problems. All of a sudden it’ll come out, you know . . . major misdemeanours, because . . . I think she internalises her worries and her anxieties. I can usually tell, you know, because she can be, say, awkward’. Another of the girls from the UK, whilst apparently not accepting the disability that she is left with as a result of her immunodeficiency and treatment, is, according to her mother, ‘. . . quite frightened to try something that she feels she doesn’t want to do, or she’s not prepared to put herself in that position’. For this same girl, it is a loss of routine which makes her very anxious, as her mother described, ‘She gets anxious about things like . . . errm . . . ballet the next day. Her ballet clothes have to be put out the night before her school. She loves the routine and she’s anxious if the routine isn’t in place. She’s anxious about things like . . . it’s the summer holidays and she’s not going to school, and it’s different to a weekend, and it takes her a while to adjust to that’.

Worries Over Hospitals Yet another UK girl had worries that appeared to be centred on hospitals. As she has hypogammaglobulinaemia due to an incomplete BMT reconstitution, she required intravenous immunoglobulin infusions every three to four weeks, and, understandably, she had something of a needle and hospital phobia. Her mother described how ‘when she was about three, she used to cry every time we drove up to the hospital for her therapy. Every time we drove up there, she used to burst into tears. She didn’t want to go in. She used to scream and . . . not out loud, but sort of whimper . . . all the way in. And she didn’t want this thing done to her. Now I think she’s learnt to accept what the problem is. But I think it’s accepting because she has to accept, not accepting because she wants to’.

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This girl went on to receive home immunoglobulin replacement therapy, with her mother cannulating her and gives her the immunoglobulin, so the problems and worries had eased somewhat, but she still hated needles. Another of the UK children – in this case a boy – also had anxieties over hospitals according to his mother. However, not all children find hospitals terrifying places, as parents of one of the children from the UK explained in this little excerpt from the interview: Father: ‘She keeps on telling us that she wants to go back to the hospital when she’s been naughty’. Mother: ‘And the reason is because Mummy never shouted at her whilst she was in hospital’. Father: ‘She learned blackmail from an early age’. This little girl was isolated in hospital for over two years, so, for her, hospital was the only home she had known until she was three years old.

Worries Connected to School School was the apparent major cause of worry and anxiety for one of the UK boys, as this UK father related in the interview when asked about his son and his anxieties – ‘The same like his sister, you know. When given him homework, you know, and he try, you know. He worry if he are going to make it wrong, you know. Alright, his mind is very good, you know, but he’s afraid that he get it wrong and he like for somebody to go and give him a hand, yes. She, she becomes very shaky and it’s come terrible when she comes in the exams, you know. She comes very worried about is the same that. She becomes very shaky and dizzy, and I’m afraid that character as (his sister), yes . . . But she is studying, yes. And if I shout . . . sometimes I shout, you know, if I shout he feels it . . . he feels it, you know. Yes, yes, he’s very sensitive’.

Worries Over Sleep and Night-Time in General Night-time appears to be a major occasion when worries and anxieties surface. Two of the boys from the UK, for example, had nightmares: He had these nightmares. Really bad. He just screamed. He didn’t wake up . . . he was awake, but not awake . . . I don’t know. He just screamed for hours. He does have nightmares. At me mother’s . . . me daddy was out cutting with the chain saw and he had nightmares about that.

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Several of the children found it difficult being on their own at night-time, as the examples of two of the boys in the study – the first one transplanted in Germany and the second one transplanted in the UK: At night-time he comes . . . he comes every . . . every night he comes to our bed. And he is like . . . if the moon, that is a full moon . . . the full moon, he is sometimes so wide awake. First of all he goes downstairs, and when sleepy he comes into our bed . . . . . . . . . . Four and five o’clock he comes to our bed. This comes from the time in hospital . . . he was alone, and so he comes now to our bed. He is dependent. But you know, he needs somebody, me or his mother, mostly his mother, just to go to sleep. He wake sometimes before. He was sleeping with the daughters. He can sleep well. After that, I don’t know why, you know, he likes somebody to sleep with him. Sometimes, you know, because he watching television and he has seen, there are, you know, people go out in houses and stole, and he afraid somebody is coming to stole him. That’s the reason he wants somebody to go to sleep with him. But he makes two years . . . two years he was sleeping alone, yes, and he make everything like that. But I tell you what, sometimes I send him to my daughters and he sleep. But the reason is that, he, because he hears from television that people go houses and stealing and so and so, and he brings it back in the mind and he start, you know, afraid of the night. This second child’s fear of being stolen may stem from his isolation, not only in an isolation cubicle in a hospital, but also in another country (although he received his BMT in the UK, he was born and lived in another country), so that he did not see his mother and sisters for over six months. Although he was only a baby, it is possible that some of that time is subconsciously imprinted on his mind. This girl from the UK is another child who at times needed company at night: Her mother told me ‘She goes off to sleep when she’s happy. She sometimes gets a bit unnerved on her own at night, and she’ll, errm . . . take herself off to the spare bed in her sister’s room’. According to the mother of a boy from the UK, he normally slept well, but if he woke up he ‘. . . just comes down an’ kicks us until we wake up an’ let him in’. On the other hand, some children have difficulty going off to sleep, as the fathers of these next two children from the UK volunteered: He’s a worrier, and he will . . . it affects his sleep and he’ll lie awake worrying. Gets quite agitated. We’ve found it very difficult not to make him worried, and we try and sort something out. Nine o’clock, ten o’clock, eleven o’clock, it doesn’t matter because he’s still awake. You could put him down to sleep at half-seven. He’s still banging about his bloody room at ten o’clock. He comes down the stairs with eyes like piss-holes in the snow, like.

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One child even sleepwalks, as this boy from Germany volunteered in this excerpt from the interview: Mother: ‘He wakes often during the night’. Son ‘I go walking’. Mother: ‘Sleepwalking, yes, he goes walking. He walks in the night’. Fortunately, this boy was very much an exception and most children did not have problems at night-time, particularly the German children.

Worries Linked to Other People Many of the children spent a lot of time on their own – whether from choice or because they found it difficult making friends is difficult to assess, but as the mother of one of the UK children told me, ‘He hasn’t got a lot of friends’. On the other hand, the mother of another child from the UK reported that ‘He likes to spend time on his own. He’s happy playing in his room’. The same theme arose with several of the German children, as these four mothers explained: He can occupy himself well. I believe that that comes from his time in hospital when he had to play on his own. But he knows how to play by himself. He also likes to sit quietly. He’s fine on his own. She likes to watch the television on her own. Occasionally the opposite can be true, as with this German girl, She is unable to play on her own. This latter theme will be explored more fully in Chapter 12.

Lack of Confidence Linked to the above, one other aspect to consider in this group of behaviours is that of confidence. A major theme put forward by the parents is the lack of confidence their children generally had, which leads to them often living rather solitary lives. One father explained, ‘He . . . he’s a withdrawn child, and very quiet . . . lacks confidence and needs praise. He has no confidence whatever in doing things for himself, and that’s why, even at school with his education, we have to push him harder. But he’s shy, he’s shy. He’s very shy when it comes to meeting other kids. He’ll even

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walk down the street with us, and one of his friends from his own class will pass and say “Hello”, and we’ll always say, “say hello”, but he won’t. There was a stage when he wouldn’t go out of the door’. According to this mother of a German child, her daughter was ‘selfconscious’, and she also lacked confidence, for example, when attempting something: – ‘“I cannot” she says, “I cannot”. She cries and . . . . . . . she regresses’. Not all children, though, were lacking in confidence. In fact a major worry for some of the parents was that their child was unable to see the dangers of going off with just anybody, as the mothers of these three children from the UK confided: She’s quite good really, but you know, she’s good to the extent that it’s almost inappropriate. She will take to a strange bus driver and go on a bus. She’s very trusting of people . . . very accepting. He’ll run away from you. He doesn’t yet understand that. I’ll try an’ say, , there’s bad people there. You’ve ‘Look, when we go into town an’ things, got to stay with your mummy. You can’t go running wild, you see’, but he will. He doesn’t yet understand ‘You’ve got to stay with me’. If he sees something he wants, he’s go straight to it. He doesn’t say, ‘Mummy, I’m going there’ or ‘Mummy, I’m going there’. He just goes straight to it, an’ next thing, right . . . he’s got hold of the pram. I’ll look an’ turn back, an’ it’s not there, you know. An’ then I’m frantic, you know. He did it yesterday when we went to town, you know, an’ like you say, ‘There’s bad people out there, you don’t know who’s . . . you don’t know who’s bad, you know’, but he just says, ‘oh, I’m okay’, you know, ‘you’ll come an’ get me’. He does not yet understand the concept, you know. But it’s a bit scary. You think, ‘Oh please, son’. She’s very much more forward in the way she communicates with people (than her brothers), and I think it’s because of what she went through when she was little. And the boys; they went through a stage when they were quite clingy and that. If someone wanted to pick them up, they would cry, or they wouldn’t go off with . . . not strangers, but, like, people that we know quite she well, who I would not want them to go off with. The boys wouldn’t. knew . . . she was gone. Anyone could go off with her. That was when she was little. I was worried and had to be quite careful with her. Even when she was young, when she went to playschool, you know, she went to everybody. And we would go shopping . . . she’d sort of wander off. She seems to have extra confidence. That’s what it’s given her; that’s what she’s got. This may be due to their early days in hospital when they were constantly meeting new people (nurses, doctors etc.) who did intimate things to them with their parents’ approval. Consequently, some of the children were unable to see that outside of the hospital, adults are not necessarily always going to look after them, and may even harm them. However, the interesting thing is that this confidence and independence is not seen as quite the same problem in Germany that it is in the UK. For example, one

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Figure 10.2

UK girl – self-portrait (aged 13 years).

boy, according to his mother, ‘. . . goes with other people, with other children in order to play. That is not at all a problem’, whilst another mother said of her son, ‘He is very independent. He comes home on his own. He goes shopping’. When asked to draw a picture of herself, one of the older UK children, who at the time of the research study was requiring therapy for depression, drew the picture (Figure 10.2). It is a very immature and small picture placed in the very centre of the paper. She then proceeded to colour the surrounding area completely black. The small figure may have indicated her feelings of inadequacy; the fact that the hands and feet were omitted is considered to be indicative of a lack of independence, whilst the largish head is considered to be an illustration of a degree of preoccupation with a fantasy life. The very large mouth is thought to be suggestive of immaturity and oral-aggressiveness, and the squared shoulders may be linked to her being overly defensive and hostile towards others (Oster & Gould 1987). The surrounding black is possibly suggestive of very deep depression.

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Figure 10.3

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UK girl – amended self-portrait (aged 13 years).

It is interesting to note that in the original drawing, the ratio was such that the figure was much smaller in comparison to the rest of the paper. Once she realised that the drawing was going to be taken away, she became worried and would not let the interviewer take it until she had cut all the black away, just leaving a very tiny figure (Figure 10.3). Quite a few of the other adolescent children who had SCID were also depressed, although not all of them to the same extent as this girl. It is acknowledged that adolescence is generally a very difficult time emotionally, but in the SCID children it appeared to be exacerbated. One’s thoughts turn to one older German boy, for example, whose depression was encapsulated by his mother, so ‘He worries. He thinks that he is going to die. If he has some pain, he has a headache, or he is poorly, or if he is dizzy, or if he has one of his many bad colds, or if he has to have blood, he says, “Oh God, I’m going to die!”’ One UK adolescent had depression that was so severe because of his problems – both medical and social – that his parents were concerned that he was suicidal. These drawings and excerpts from the interviews provide merely a snapshot of the behavioural problems encountered by the children that

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are linked to neurotic and depressive behaviours. The next part of this chapter will discuss these behaviours in more detail.

Discussion We tend to think of the neurotic and depressive behaviours (as indeed we think of the aggressive and antisocial behaviours) as being undesirable. However, emotions such as fear, anxiety and sadness, along with other more physical feelings, like pain and nausea, have a vital biological function and, in actual fact, are actually beneficial to us as they can help to protect us from physical or emotional harm (Martin 1997). Whilst pain can stop us doing physical damage to ourselves, emotions such as anxiety and depression help us to cope with potential emotional harm, and so prevent this from further affecting us – often physically. Williams and Nesse (1991) suggest that too little anxiety can leave us open to physical danger. Similarly, sadness or mild depression is a very common reaction to life events like emotional upheavals such as bereavement (in its many guises), losing a job, or the break up of a relationship. This depression can then give us the space (and the incentive) to work through the problem and eventually find a resolution. However, the problems occur when these emotions and behaviours get out of hand or when they go on too long. When this happens, sometimes very serious problems can occur – both physical and psychological. Severe depression and anxiety disorders ruin millions of lives because in these cases, the individual is unable to function normally. According to Martin (1997), it has been calculated that approximately 2–3% of the population of most industrialised societies suffer from a serious depressive illness at any one time. This depression drives thousands to kill themselves every year (Martin 1997). So, it is natural that children who have been acutely ill, who have been separated from their normal environment, and, more especially, have been separated from their families, should suffer some depression. This allows them to cope with their feelings of isolation and sometimes of betrayal. For example, Bradford (1997) noted that in the short-term, children who had had liver transplants were quite likely to experience changes in mood, particularly experiencing some of the neurotic and depressive behaviours, such as unhappiness, crying and mild depression. This is to be expected – the child has lived with a life-threatening disease for months, if not years, waiting for a liver to become available. Then there have been the, to them, quite horrendous and painful experiences during the time of the transplant, followed by the uncertainty afterwards as to whether or not it has been successful; then there is the realisation that it was not a ‘one-off’ cure because the child will have to take life-long drugs to prevent rejection and infection (the latter as a result of the anti-rejection drugs). Then there is the knowledge that for there to be a liver available for the transplant, someone has probably died – in other words, someone has died to give them life. Finally, there is the realisation that there is no guarantee that at some later

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stage the new liver will not fail or be rejected by the body’s immune system. Is it any wonder that there is depression following a liver transplant? Indeed there would be more concern if there was no emotional reaction to all this. However, what is then necessary is for the child to work through his or her feelings in order to be able to accept what has happened so that he or she can start enjoying this new opportunity of life. Concerns only need to be raised if the child continues to be depressed, or if the depression is so debilitating as to seriously affect the child’s functioning. This belief that these emotions, such as fear, depression or worry, should not automatically be assumed to be bad or abnormal is also discussed by Lansdown (1984) who points out that fear and worry are not abnormal in themselves. Instead, what should cause concern is the extent and cause of the worry and fear, as well as the amount of distress experienced and displayed by the child. Lansdown (1984 p. 172) explains that anxiety develops from the emotions of fear and worry, and that it is ‘. . . an uneasy state concerning impending or anticipated ill, a characteristic being that the individual cannot escape’. It differs from worry because worry has objective origins in that it is concerned with specific topics and concerns, for example exams, whereas anxiety has subjective origins and is concerned with more general topics and concerns, for example, relationships, or life itself. Using the Rutter A behavioural scale, Pot-Mees in her 1989 study of children who had BMTs for all sorts of medical conditions except for SCID, came to the conclusion that six months after the transplant, there was a rise in behaviour problems of the children that were related to depressive symptoms, so echoing the conclusions of Bradford’s (1997) study of children following liver transplants. Pot-Mees (1989 pp. 69–70) noted that the symptoms of depression exhibited included ‘. . . a depressed mood state, social withdrawal and poor peer relations, impairment of school work, irritability, complaints of fatigue, sleep and eating difficulties’. These were the same symptoms that McConville and Bruce (1985) described as meeting the criteria for depressive symptomatology. Pot-Mees (1989) also reported that there was only a small rise in the parental reporting of ‘misery’ in their children but this she explained as possibly being due to the fact that parents found it difficult to admit that their child was depressed – at least when putting it down on paper. When it came to the interviews with the parents at the six months period, Pot-Mees (1989) found that the parents reported a significant increase in the incidence of lethargic and passive behaviour in the children, caused partly by the intense physical tiredness that the children felt at this stage after their transplant, as well as their increased feelings of misery and depression In addition, the parents reported an increase in their child’s anxieties, that were particularly related to their child’s illness and the possibilities of dying. It is very difficult and unrealistic, if not unnatural, for young children to have to come face to face with the notion of their own mortality. Indeed many much older people struggle to come to terms with their own mortality, so it is not surprising that these young children became anxious and depressed. The anxieties were also linked to the fact that many of these children were still worried about

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the possibility of infections or of the recurrence of problems that they had experienced in hospital. Pot-Mees commented that these symptoms have also been found amongst other groups of children with chronic illnesses who have experienced long-term hospitalisation (Eiser 1985, Susman et al. 1980a). Pot-Mees also found evidence that at this stage of their convalescence, some of the children displayed symptoms of post-traumatic stress disorder; this will be addressed in detail in Chapter 14. The parents of these children reported that their children tended to ‘. . . feel down and miserable’ and that ‘. . . they were insecure and displayed lethargic behaviour’ (Pot-Mees 1989 p. 81). These parents also reported that their children were emotionally up and down (labile) and that they were easily agitated. In addition the children had eating problems and also tended to withdraw themselves from other members of the family as well as from their peers. This latter observation will be explored more fully in Chapter 12. When Pot-Mees (1989) went back to these children after another six months (12 months post-transplant) she found that generally there was a reduction in the scale and incidence of these depressive and neurotic behaviours, with two notable exceptions. First of all she found that the children had much greater problems in concentrating on anything for any period of time than they had had before their BMT, and this, she concluded, indicated that they were now more restless and nervous than before their treatment. She also found that there was a quite significant increase in anxieties compared to their pre-transplant behaviour. Again, these anxieties were centred on the same issues as they were six months previously, namely the possibility of infections and the recurrence of problems encountered in hospital during their transplant, as well as illness in general and possible readmission to hospital. There are many similarities between these concerns and those of the children in the SCID study. Some of the other worries and anxieties of these children at this stage post-transplant were focused on their appearance and body strength, whilst other children worried about school and their workload (after having missed so much time due to their illness and the BMT), and they also worried about mixing with other children (linked to their periods in isolation and the risk of infections). In terms of the seven children diagnosed with having a behaviour disturbance at 12 months post-transplant, 75% of them were diagnosed as having emotional and neurotic behaviours, whilst the other 25% displayed antisocial and aggressive behaviours.

Conclusion The two main studies mentioned above (Bradford 1997, Pot-Mees 1989) considered the psychosocial behaviours of young children in the shortterm following either a BMT or a liver transplant, whereas the study with children who had SCID researched the psychosocial behaviours of the children in the medium-term to long-term. The problems that Pot-Mees and Bradford encountered were still present in many of the SCID children

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some years later – even 15 years later in some cases, and so it seems acceptable to theorise that these same problems can persist in any child for many years (if not for life) if the initial stimuli were severe enough and no therapy is put into place to counteract these psychosocial behavioural problems (Vickers 1999b). Indeed, there has been much research in recent years into the extent to which early behavioural problems are predictive of longer-term developmental outcomes (Cot´e et al. 2001, 2002, Fergusson et al. 1997, Fergusson & Lynskey 1998, Goodwin et al. 2004, Hofstra et al. 2002, Scholte et al. 2002), although, according to Goodwin et al. (2004 p. 874), ‘. . . relatively little attention has been given to the life course outcomes associated with anxious/withdrawn behaviour tendencies in childhood’. There have been a few such studies, including one by Verhulst and Koot (1991) that studied the links between anxious and/or withdrawn behaviour in young children and the subsequent development of anxiety and depression in older children and adults. Pine et al. (2001) in their study of 717 teenagers in the USA, concluded that those who, at 14 years of age, reported having fears had increased risks of depression in subsequent years, whilst in 2000, Prior et al. reported that ‘shy’ infants and young children had increased risks of anxiety problems by the time they were 13 years old. Finally, from their research study in 1996, Caspi et al. discussed the fact that young children who had been described as being reticent or fearful when they were three years old were found to be at an increased risk of experiencing depression by the time that they were 21 years old. In addition, laboratory studies have supported these field studies that behavioural inhibition in children (including shyness, inhibition and fear of new situations) is associated with anxiety disorders during later childhood and adolescence (Biederman et al. 1990, 2001, Hirshfeld et al. 1992, Rosenbaum et al. 1988, Schwartz et al. 1994). So, according to Goodwin et al. (2004 p. 875), ‘the weight of this evidence clearly supports the view that early anxious, shy or reticent behaviours may be precursors of later anxiety and depression’. Interestingly, Goodwin et al. (2004) found that the link between early anxious and withdrawn behaviour and later depressive and anxiety symptoms could be mediated by common childhood, social and family factors. They concluded that because early anxiety and withdrawn behaviour were associated with such factors as conduct and attention problems, family stressful life events, child abuse, parental separation, maternal education and parental problems, then these can feed into the child’s emotional and behavioural development and potentially prevent, improve, cause or exacerbate early anxious and withdrawn behaviours. Not all those who exhibited anxiety and withdrawal symptoms in early childhood went on to develop depressive emotional behaviours in later life, but, according to Goodwin et al. (2004 p. 880), ‘this does not preclude the possibility, however, that for some individuals who exhibited early anxiety/withdrawal, the onset of internalising disorder (e.g. depression) may occur after age 21’. Goodwin et al. (2004) do go on to make the point that treatment and other intervention programmes for children with early anxious and withdrawn behaviours is warranted, and that this is especially relevant because it has

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been estimated that by the year 2020, major depression will be the third leading burden of disease in the world (Murray & Lopez 1997). So, to return to the children with SCID who survived BMTs, early hospitalisation and long-term isolation, for many of them depression, anxiety and other neurotic behaviours are an ever-present complication to their quality of life. The study on them (Vickers 1999b) has shown that this can be present for many years afterwards, and this has reinforced research into other acute and chronic diseases, such as cancer, BMTs and other transplants, such as liver transplants (Bradford 1997, Pot-Mees 1989). In addition, personal experience and observations of nursing many children, over a long period of time, with other chronic–acute disorders, such as cystic fibrosis, early-onset diabetes, children in paediatric and neonatal intensive care units, as well as children with disabilities following accidents or disease, also supports these findings. Coyne (2006) similarly reports from her study about children’s experiences of hospitalisation, and concludes that the unfamiliar setting of a hospital and the uncertainty about treatments appear to create feelings of anxiety within the children, although, as she pointed out, most of the children had previous experience of hospitals as patients. These fears included the health professionals themselves, as well as the procedures the children have to endure – investigations and operations (particularly in relation to the possibility of harm, mutilation, pain and even the possibility of dying whilst in hospital). In summary, depression and other neurotic symptoms are expected and indeed are natural (and desirable) in the short-term. The problems occur when these symptoms become more severe and long-term. To echo Goodwin et al. (2004), treatments and intervention programmes need to be in place to identify and help with these emotional behavioural problems at an early stage, because later on, it can become much more difficult to combat them.

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Psychosocial Effects on the Family

Introduction ‘Although infants with SCID and their mothers constitute a small population, they jointly experience the effects of a catastrophic illness and dramatic intervention under conditions of prolonged, pervasive social and physical isolation. This study was conducted to illuminate mother’s experiences of the protective isolation setting and of their infants’ care trajectories’ (McKeever et al. 2002 p. 1022). So far in this book, the emphasis has been on the child with SCID (severe combined immunodeficiency), but, as was discussed in a previous chapter, what affects the child affects the family and what affects the family affects the child, so this chapter is concerned with the effects on the family and the stresses they have to cope with. As with the previous two chapters, much of this chapter is devoted to the words of the families as they relived their experiences. McKeever et al. (2002) make the point that the potentially damaging physical, psychological, social and sensory effects on adult and child patients of even a relatively short period of isolation are well documented, for example, Ross-Kerr & Wood (2001), but that virtually nothing is known about the experiences of parents of extremely sick infants and children who have to cope with many months of isolation. At the time of writing their paper, McKeever et al. (2002) pointed out that the only research, other than anecdotal reports, into this aspect of nursing children with SCID was by Vickers (1999a), and that was an abstract from a presentation at a conference. Vickers (1999a) revealed that parents experienced as traumatic their children’s treatment for SCID and that some felt alienated from their own communities and families as a result. In 1978, there was an anecdotal report from Kutsanellou-Meyer and Christ that claimed that mothers of infants with SCID found mothering to be difficult as a result of the lack of progress they discerned and lack of freedom of movement within the hospital. Beyond those two reports, McKeever and colleagues found only Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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two studies specifically linked to the experience of mothers with children in protective isolation. The first one (Powazek et al. 1978) found that mothers who could only communicate with their children in isolation by means of an intercom device displayed more anxious and aggressive behaviours and those who were able to actually enter the isolation room. The second one by Kronenbeger and colleagues in 1998 found that 25% of mothers whose children were being prepared for bone marrow transplants (BMTs) were clinically distressed. It was against a backdrop of these very few reports that McKeever et al. (2002) undertook their study. ‘A child’s emergency admission to the paediatric intensive care unit (PICU) can strike fear and feelings of helplessness into the hearts of parents who only hours earlier had been in control of their lives’ (Russell 2007). Indeed, as Russell (2007) then goes on to explain, any acute critical illness can seriously threaten the ability of the parents to fulfil what they consider to be their familiar and vitally important role of protecting and providing for their child. Whether they have been parents for some years, or whether they have just become parents (as is sometimes the case in neonatal intensive care – NICU – and special care baby units – SCBU), their perceptions of themselves as parents can change dramatically because they see someone else taking over their roles of protection and nurturing. Helplessness, fear and frustration are common feelings for these parents. In many cases even ‘fear’ does not describe their emotions – perhaps ‘terror’ is a better word. Not only is the present compromised by their child’s illness, but so is their future. This helplessness was picked up by Aite et al. (2003) in their study on the impact of maternal anxiety when a baby is born with a congenital anomaly requiring urgent surgery. Amongst the reasons for the high levels of anxiety in these mothers, such as the initial shock of learning the diagnosis, the lack of contact at birth, and the fear of their baby dying during the operation, Aite et al. (2003) pick out the mother’s sense of helplessness when the reality of the intense and specific care of their baby struck them. They mentioned that the first reaction of the parents once apprised of the situation was ‘It was as if the world was falling apart’ (Aite et al. 2003 p. 576). The PICU, NICU, SCBU setting, or indeed any acute hospital setting such as isolation units and transplant units, can quickly come to undermine the sense of competence, control and stability of even the most dedicated parents (Meyer et al. 1998). As Russell (2007) put it so succinctly, acute admission for babies and children – whether emergency or planned – represents a major violation of parental expectations and hopes, as well as beliefs. This is because parents are no longer in charge of their children’s lives at a time when their child is in danger, or even dying. The decisions are mainly being made by the professional staff and parents may feel that they have no role to play and that the child, no longer belongs to them. One mother in the SCID research project said that when her child was in hospital, she felt shut out from all of the major and the minor decisions that were made about her daughter, and that it felt as if her daughter belonged to us (the staff on the unit) rather than her and her husband. It was not

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until her daughter went home that she felt they were truly a family again, and that her daughter was now really her daughter, and not just some stranger that she was supposed to be fond of. It was also only at this time that she and her husband felt that they could start to think of, and plan for, the future. All the time her daughter had been in hospital, she and her husband had felt that their lives were on hold, and that they could only really survive by living in the present – often from day-to-day (sometimes even from hour-to-hour) at critical periods during their daughter’s illness and transplant. The past was too painful to contemplate, and the future was too vague and untrustworthy. It was the present, painful and terrifying and uncertain as it was, that was the only reality that they could attempt to cope with – often by closing down all their feelings and becoming emotional ‘zombies’. There are quite a few studies that focus on the psychosocial health of parents during the early hospitalisation period when their babies are in NICU which demonstrate that mothers with preterm infants experience more severe levels of psychological distress in this period than do the mothers of healthy full-term infants (Brooten et al. 1988, Doering et al. 2000, Gennaro et al. 1993, Singer et al. 1999). In addition, there have been a few studies that have shown that the addition of a preterm infant into a family has negative repercussions for the family (Cronin et al. 1995, Ong et al. 2001, Saigal et al. 2000, Taylor et al. 2001), whilst there also have been a few studies that disagree with this (Lee et al. 1991, McCormick et al. 1986, Tommiska et al. 2002). Because of these contrasting results, and because there is little information in the research literature on how parents of NICU children adapt psychologically to the demands of their caregiving beyond the initial hospitalisation period, Klassen et al. (2004) compared the psychological health of family caregivers of children who were admitted at birth to an NICU with that of family caregivers of healthy children. What they found was that, beyond the initial hospitalisation, there did not appear to be any difference in parental psychosocial health between the two groups. This is in line with the study by Singer et al. (1999) which demonstrated that after the neonatal period, the mothers of low-risk infants who had been hospitalised in NICU did not differ from mothers of full-term infants, and that by the age of three years, these mothers actually had lower levels of stress, which led Singer et al. (1999) to suggest that this may be due to the relief of the mothers following an initial period of fear and anxiety. In contrast to this, however, Singer and colleagues (1999) found that mothers of high-risk infants had more symptoms of distress when their children were two years old, more negative family impact at two and three years of age and more parental strains and illness stressors at three years. However, by three years, the reported psychological distress of these mothers with high-risk children did not differ from that of mothers of full-term children, and this Singer and colleagues (1999) put down to the fact that by two years of age, infant developmental scores are predictive of later outcomes, and so by this time many mothers of high-risk infants have relinquished their hopes of the children being the same as healthy

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‘normal’ children, and that there is then some psychological adaptation taking place, despite the parents acknowledging that there were greater family and parenting stresses. Klassen et al. (2004) were not able to confirm this latter point in their study, but felt that it is possible that the parents of both the high-risk and low-risk infants who had been hospitalised in NICU in their study could have adjusted over that period of time. Russell (2007) pointed out that the alteration of parental roles has been identified in many studies; in particular Meyer et al. (1998) discuss that parents feel they are no longer in charge of their child’s life at a time when their child is in danger. Continuing her discussion, Russell (2007) notes that parents have to relinquish much of the care of their child to another person – a stranger – and they, themselves, have to take up the role of a sick child’s parent, which is a totally different role – and certainly within intensive and critical care environments in hospital, is a more passive role. In a qualitative study by Carnevale (1990) five categories of parental stressors within paediatric intensive care units were identified, namely:

r r r r r

parental role conflict concern for their child concern for their other children concern linked to the intensive care environment concern for their friends

It was the conflicts that arose within the parental role that were the major stresses identified by the families in this study, along with concern for their child. From her review of the literature, (Russell 2007) found that four main areas were highlighted that are known to cause stress in parents in these situations. These were:

r r r r

the child the parent’s role environmental stressors the staff

According to Morrison (2004), parents often experience increasing stress, not just the stresses of having a desperately sick baby or child (acute and mind-numbing though that may be), but also the stresses involved in adjusting to life in the hospital and the stresses involved with their attempts to define their new role(s) as parents within the hospital community and environment, along with trying to negotiate their own role(s) with the role(s) of the staff and the alien environment into which they are peremptorily and brutally (as they perceive it) thrust along with their desperately sick child. Parental reactions to such acute illness in children, whether an emergency, or an exacerbation of a chronic complaint, include overwhelming shock, helplessness, anxiety and guilt (Carnevale 1990). However, as Russell (2007 p. 28) summarises from all the literature, ‘Parental stress is a

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normal, inevitable response and the identified loss of a well child and parenting role, transition to an alien environment, and staff communications are well highlighted’. Helseth and Ulfsæt (2005), describing the parenting experiences during paediatric cancer, point out that the main task of parenting is to give care and to protect the children and make any adverse situations as secure and normal as possible for them. Thus, a diagnosis of paediatric cancer, which will bring insecurity and worries into the child’s life (Helseth & Ulfsæt 2003), presents a challenge to the parents in trying to fulfil one of their primary roles. This challenge can be multiplied many times if the parents see that this role is being usurped by hospital staff. Parenting has three dimensions, according to Lamb (1987), namely engagement, accessibility and responsibility. However, when a child or baby requires intensive or high dependency care, then it becomes difficult for the parents to fulfil these roles. For example, thinking of the child with SCID or with cancer, who is in an isolation environment, engagement becomes difficult because of barriers – physical barriers, such as an isolation cubicle, or an intensive care bed/cot, as well as barriers that can occur caused by not knowing what is happening or what to do about it. Helseth (1999, cited in Helseth & Ulfsæt 2005) points out that caring for a child implies both the interpretation of the child’s needs and the ability and desire to respond to these needs, which, in turn, implies knowledge of their needs. Mayerhoff (1971) states that knowledge is absolutely essential when one is trying to understand what the needs of another person may be. Who, in the situation of a child with an acute and/or life-threatening illness has this knowledge? Hopefully the hospital staff, but very rarely the parents (although often before the end of their stay in hospital, parents pick up a great deal of knowledge about the disease and, of course, it is the parent who has the knowledge and understanding of their own child outside of the illness). Similarly, accessibility becomes difficult because the parents cannot just pick up the child spontaneously and cuddle it – there are precautions to be taken (protective clothing, help with tubes and drains etc.) each and every time, and often this can only occur with the ‘permission’ of the hospital staff. There is also a lack of accessibility for other members of the family, so further isolating the parents. Finally, responsibility: this has already been discussed above, but the families do feel that they lose all responsibility for their own child. The combined effects of the failure to be able (or to feel able) to carry out these three dimensions of parenting is that the parent starts to lose confidence and trust in his or her ability to look after their child, and this can take some time to be rediscovered, even after the child, has gone home. In a study by Van-Dongen-Melman et al. (1998), parents reported that their child having cancer was the most overwhelming experience in their life. They had to cope with many changes, and overwhelmingly, these changes were negative, with the major changes being labelled as losses. Even if their child survived, the parents still had to deal with the loss of their usual way of life. Many of them reported that they could never be the same person again – relaxed and happy. They had lost the experience

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of joyfulness and keenly felt a loss of positivity towards life. The parents also felt more vulnerable when faced with traumatic events and found it difficult to cope with new stresses – they felt a loss of invulnerability. As can be seen, there is a lot of literature that discusses the effects on parents of children in NICU, PICU and cancer wards, and this serves to point up the paucity of such literature that is concerned with the effects on the family of having a child with SCID, who often has to experience such stressful situation for much longer and at a very intensive level.

Problems Faced by Parents The hospitalisation of a child is a stressful situation and a stressful time both for the child and also for members of the family (Simon 1993). The pressures of looking after a very sick child in hospital, as well as when the child came home, were almost unbearable for many of the parents who took part in the SCID research project. For example, these UK parents found the situation affected them immensely – as the mother explained, ‘I think that it did affect me an’ him (her daughter’s father) immensely, yeah. Just the institution like, yeah, I think it did’. Two of the stresses that have been consistently identified by parents of children in NICU/PICU or of children with cancer are the changes that are made in their role(s) as parents and the disruptions to the child and parent relationship, including physical separation from their child, limited opportunities to care for their child and the handing over of responsibility for the care of their child to strangers (Aldridge 2005, Russell 2007). The father of one UK boy who was born with reticular dysgenesis (perhaps the most serious of all the SCID disorders with death normally occurring within a few weeks of birth unless the child is successfully treated by a BMT) so movingly put into words the effects of having a child with this immunological disorder, who had become very ill within three weeks of his birth: There’s a psychological aspect of all your family aspirations. The preparation . . . of building up your family – getting all your . . . buying all your stuff for your first child. The nursery and everything like that. Then, all of a sudden, you’re a couple of weeks into . . . into fatherhood and motherhood, and somebody pulls the carpet from underneath your feet. And it’s like that then for the next couple of years. So everything that you planned in your mind, for that couple of years, goes. And nobody ever seemed to consider . . . nobody seems to consider the relationship . . . the loving relationship between the mother and father. The bond that should have occurred as parents . . . stepping from husband and wife into parents. All your aspirations as to how you’re going to cope as parents go out the window as well. Nobody ever steps in to deal with that. Stress can begin for the parents even before a diagnosis has been made. These stresses occur when the baby or child is obviously sick, but no

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one knows what the cause is. This was certainly the case with SCID in early days, because very few health professionals – even senior paediatric consultants – had any knowledge of what SCID was or how to diagnose it. Consequently, many of the families in the SCID research project had experienced several weeks of uncertainty before the disease that their child was suffering from was finally diagnosed (for some unfortunate parents, the diagnosis often came too late to save their child). Hilton (1992) defines uncertainty as a cognitive state that is created when an event cannot be adequately defined or categorised, and in terms of ill health, it is a major factor influencing expectations about treatment and about the prognosis of the disease (Swallow & Jacoby 2001). The longer the wait before a diagnosis is made, the greater the uncertainty, and the greater the psychological damage to the parents (and to the child), so consequently once a diagnosis is made, initially there is often a feeling of relief that the parents, the child and the medical staff are now dealing with something that is known rather than unknown (Bury 1982, Swallow & Jacoby 2001). However Mishel (1983) points out that, although the eventual diagnosis of a disease may get rid of the uncertainty around the cause of the disease, it may then create further uncertainties around the course of the disease, treatment and prognosis. Once diagnosis is made, then stresses arise that are linked to decisions that have to be made about treatment. For the parents of children diagnosed with SCID there was always a stark choice given to them. To put it simply and bluntly, they were told that their babies were dying and that they only had a few months of life left. They were then told that there was an experimental treatment (as it was in those days) of a BMT using one of the parents as the donor. However, it was stressed that this was experimental and that there were no guarantees, that their baby might die even sooner as a result of the treatment, and that, in addition, their baby could become very sick indeed during the treatment. They were then asked to think about it and to make a decision, but the sooner the treatment began the slightly better the chances would be of the child surviving. Although, the pros and cons of the decision that they would have to make were spelt out at the initial meeting, it was obvious that for almost all the parents after they had heard that their child was dying, they heard nothing more. So, after giving them a little time to grieve and to comfort one another, I would take them away to somewhere private and allow them to discuss it with me, and to ask me lots of questions. Although to an outsider, the decision to be made was a very simple one because without treatment the child would die, but to the parents it was not so simple. There was no guarantee of the success of the treatment, and the treatment could even hasten the death of their baby. Almost without exception, however, the decision was to go ahead and try the treatment – although not without continuing misgivings, which would be exacerbated if their baby died during the treatment. The one exception concerned the parents of a baby with a different immune deficiency disorder, where they were told that their child could live without treatment until she was in her early teens (although by then she would be blind and deaf). In this case, the parents decided to defer

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treatment for some years in the hope that the treatment itself would improve and become less risky. For other disorders, the decisions about treatment would be different, but always it was the parents who had to make that decision, and they knew that they would have to live with their decision for the rest of their lives. If everything turned out fine, then that would be no problem, but if the opposite occurred then they would have a terrible burden to live with. For the parents of children requiring a BMT for causes other than SCID, there are still the same decisions to make after diagnosis. Hare et al. (1989 p. 30) summed it up as Families are confronted by these possible outcomes: (1) without transplantation the patient’s early death is likely; (2) transplantation procedure may result in death and shorter survival than without transplantation; (3) after transplantation, the patient may suffer a very grave illness and any of the following: (a) early death, (b) remission with later relapse and death, (c) survival with prolonged disability secondary to the side-effects of transplantation, and (d) remission and lifelong cure. Such decisions are often taken out of the hands of parents of babies and children requiring intensive or high dependency care because of the emergency nature of such care. However, once care has commenced, there are still decisions to be made by the parents, particularly if the baby or child is not responding to the treatment. These decisions are along the lines of how long efforts should be made to treat their child, and, if there is no improvement, then the decision that every parent dreads – the decision whether or not to turn the life-support systems off. Once the decision had been made, whichever way it has been made, then the parents, and the rest of the family, face further stressors and have to come up with ways of coping with them.

The Environment The effects of the environment are deemed to be a major stressor for the parents (Aldridge 2005). The first stressor that the parents are conscious of, outside of the knowledge that their child is very ill and may be dying, is possibly the appearance of their child – very sick, often with tubes and drains going into and coming out of various parts of the body (particularly distressing in babies, as babies can seem so tiny next to these tubes and drains) and all the other essential apparatus that may be needed to keep their child alive, such as ventilators. There are also the noises that can seem alien and frightening, particularly if there are alarms going off all the time. Intensive care units and isolation cubicles can be very claustrophobic and often seem too warm for comfort. At the same time, there is no privacy for the parents or the child. As one mother explained to me, she felt as if she was living in a goldfish bowl throughout the 13 months her child was with us in isolation. An early paper by Kutsanellou-Meyer and Christ (1978)

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reported that mothers of infants with SCID found that their mothering role became difficult partly because of the lack of privacy that they encountered in the transplant unit. In their more up-to-date study, McKeever et al. (2002) found that the physical characteristics of the isolation cubicle loomed very large in the recollections by mothers of their infants’ hospitalisation for bone marrow transplantation for SCID. Some of the mothers in the McKeever et al. (2002) study stated that they found the physical and social isolation of the room unpleasant, and one mother went so far as to describe the isolation cubicle as a jail in which she and her son were imprisoned. This feeling was supported by comments from the parents in the SCID study in both the UK and Germany in which many parents, whilst understanding, and being grateful, that the isolation cubicle was helping to protect their child, still found it very restricting and claustrophobic, and during the isolation period, several of the parents just wanted to take their child out of the cubicle and home – some even threatened to do so. Fortunately none carried out this threat, but a few did require a great deal of persuasion not to do so. The protective clothing that the parents (and the hospital staff) had to put on when entering the cubicle, and remove when exiting it, also caused frustrations and problems. In McKeever et al.’s (2002) study, they discussed how the effort to put on and remove this protective clothing made it difficult for them, and all of the mothers in the study described how they avoided, or at least delayed, such basic needs as eating, drinking and going to the toilet in order to minimise the number of times that they would need to exit and enter the isolation cubicle, with all its cumbersome and time-consuming putting-on and taking-off of protective clothing. However, as McKeever et al. (2002) reported, in spite of all the constraints of the room and the ‘goldfish bowl’ nature of the experience, all the mothers accepted that this was the price they had to pay for the possibility of their child’s survival, and therefore it was worth it. There was a belief amongst the mothers in the study that the isolation room was a safe sanctuary, which, however, led to another concern. Once their child had survived and plans were being made for their discharge home, the great joy of these mothers was tempered by anxiety and fear just because the isolation cubicle had been a ‘safe sanctuary’ for them and their child. This was reinforced by the knowledge that even after discharge, until their child’s immune system was fully functioning and they had received all their immunisations (and these had all ‘taken’), their children were still at some risk of further serious infections. Consequently, many of the mothers in the study recreated many of the isolation cubicle’s physical and social barriers within their own homes. For children other than babies, there are also psychological effects as a result of them having been treated in an isolation room or cubicle. As Hare et al. (1989) discuss, for the patient in isolation, there is an additional concern linked to essential needs such as those needed for human touch and interaction, and in this altered state of existence it is possible that the patient may be overwhelmed by loneliness, and if this is linked to the fact that the patient may be unwell, makes them very vulnerable to

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many fears and anxieties. As one of the patients in Hare et al.’s (1989 p. 31) study said, ‘All I really want right now is to be held, really held – with nothing separating me from someone else’. These are also the needs and feelings that have been expressed by the parents of babies and children in isolation. Although things have improved much more since the early days of BMTs in that there is less protective clothing necessary, and gloves in particular are often discarded (as long as handwashing and sterilisation is very strictly enforced), and this means that there can be skin to skin contact, this feeling of loneliness in the isolation cubicle for parents can still exist.

Death and Bereavement For parents, the diagnosis of a life-threatening and critical disease in a child is considered to be their worst nightmare come true. Until the illness is resolved one way or the other, parents often feel that ‘Death’ is looking over their shoulder. They spend the next few days, weeks, months or even years living with the possibility that their child may die, whilst at the same time trying to appear positive and reassuring. They can never relax, as was found to be the case with the parents of the children in the SCID research project, even for those who to all intents and purposes knew that their child was cured. In many instances, stresses linked to the potential death of a child for the parents in the SCID study arose even before the birth of their child. Because SCID is a genetic disorder many of the parents had already had to deal with the death of at least one child. When asked how many children she had, the mother of one German boy told me that, ‘. . .we have three, the first one died, no? The first one died, [in a whisper] three children, two alive’. Other parents told a similar story: The first one died, and now there is this boy . . . well then, two, yes. (Mother of German boy) We now have five children alive. There were two children who died in . . . (former home country). (Father of German boy) Another German mother, when asked how many children she had, just simply replied, ‘Six children. Three have died’. Yet another German mother had also lost three children, as well as having had several miscarriages, whilst one of the UK mothers had lost three children, but they were initially wrongly diagnosed, and only later were rediagnosed as having had SCID. The parents of one German girl had not only to cope with that child’s sickness, but had also experienced the death of three children in addition to several miscarriages. As the mother explained,

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I wasn’t prepared to have an ill child . . . sick child. That was the . . . So, we lost three child . . . children, but I didn’t . . . I hoped she wasn’t sick, and then she was sick, and my family . . . there’s no one sick from the . . . And I said, ‘Okay, I am an older mother. I am forty . . . it is . . . with this child everything will be well . . . I have patience’. Yes, I’m very . . . I have so much love. Well, next I begin to think that I am crazy, no? I exist only for this child. Consequently, for many of these parents, the discovery that they are pregnant again can be both a joyful and a frightening, anxious-ridden time. And it is not just the parents who have already had a child with SCID who has died who have these concerns. Because SCID is a genetic disease, then relatives of those parents may also have concerns when they become pregnant. This is especially so in families where first cousin marriages are the norm. On one occasion, the whole extended family of parents with a child who had SCID came for testing because first cousin marriages were the norm within that extended family. There were two cousins of parents of a SCID child who came for testing because they were supposed to be getting married to one another and wanted to check whether or not they carried the abnormal gene before going ahead with their wedding – unfortunately they did. Over 30 members of the family were tested, and many of them did carry the abnormal SCID gene, many of whom were married to others who carried the gene including one couple who were pregnant with their first child (which subsequently was found to be affected, and died in spite of treatment). So, many of the families have faced death and suffering already. For many of them now there is the opportunity of pre-natal diagnosis and the option of a subsequent termination of pregnancy if the fetus is found to be affected. This in itself, of course is very traumatic, and brings further stressors, and even if the pre-natal diagnosis gives the ‘all clear’, this is still not the end of the worry as one mother informed me in a letter. ‘They’ve told me it is a little boy from an ultrasound, but they can be wrong, can’t they? One thing they can tell me is that this one isn’t going to be a SCID, which is reassuring, but still at the back of my mind there’s always this nagging doubt that there will be something wrong. I’ll just have to keep my fingers crossed and hope for the best’. The effects of having a child who had already died affected other families, as well as the family of the girl above. For example, these parents below had to cope with the diagnosis of their first child as having SCID, and then his subsequent death shortly afterwards, whilst his mother was several months pregnant with their second child, who was also diagnosed as having SCID at birth, and who had a successful BMT. They discussed the problems this brought (Box 11.1). In the hospital itself, death is ever present. Even if your own child survives, other children may not be so fortunate. In such a close-knit community as exists in the hospital environment, with at least one parent of each child living there 24 hours a day, seven days a week, for months on end, parents get very close to each other – almost like an extended family, but a family held together by powerful negative emotions of survival, of death, of shared pain and fears, of knowing that each day could be the last

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Box 11.1 Discussion Between Two Parents About Their Feelings Following the Death of Their First Son and the Birth of Their Second Son (Also with SCID) a Few Months Later

Mother: ‘It did affect us, didn’t it. Not our relationship. I’ve been through depression, which has been quite long-term, hasn’t it? I suppose it started just after I had my second son, so I’m still not right.’ Father: ‘One of the problems is it’s not just the transplant. I suppose it’s anyone with a child with a long-term illness. That upsets your lifestyle, and then you get into a routine, and then it’s a bit of a shock when you actually return to normality, and it takes a long time to adjust. That’s one of the aspects. Another is what actually happened with my two sons. Both to me overlap, and I know that I wouldn’t talk about my first son for a long time.’ Mother: ‘Which caused problems with me because I needed to talk about him, and every time I mentioned him, I felt tremendous guilt because I knew my husband didn’t want to, so it, it . . . you know, it was difficult wasn’t it.’

one for their child. Gold et al. (1986) talk about such emotions linked to children undergoing solid organ transplants and their families as having prolonged feelings of powerlessness (there is that helplessness again), fears of death, and continues guilt that result in an overall weakening of defence mechanisms and emotional adaptation. In all these situations – BMT, solid organ transplant, NICU, SCBU, PICU, neonatal/paediatric surgery – when one child dies it affects everyone, including hospital staff. But particularly it affects the other parents, although there is usually a rollercoaster of emotions for the parents to cope with. There is obviously sadness for the family of the child who has died, as well as for the child, but there are also feelings of relief, and even joy, that it was not their child who had died. With this, there is often guilt for feeling so happy and relief that their own child is still alive. There is also a ‘trade-off’ mentality, described by one mother to me as if there was some sort of quota system – as if someone had to die, and if it was another child who died, then ‘my child would stand a better chance of surviving’. Then the guilt kicked in, because these families had got very close to one another in the long days and even longer nights in hospital, and the thought of their relief (and happiness) that their child was still alive whilst another one had died seemed obscene to them. Gold et al. (1986) reported that many of the parents in their study described feelings of vulnerability and fear as they reflected that it could easily have been their child who had died.

Guilt In the previous section, parents discussed the guilt they felt when another parent’s child died whilst their own child was still alive, but guilt can arise

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in parents for various reasons. The fact that SCID is a genetic disorder can produce more stress, including that of guilt, particularly for the mother if the mode of transmission is X-linked. This German mother found this to be the case. ‘I had this guilt in the beginning, but my husband told me that I had given him life. I felt guilty, but he just said, well it’s okay’. One mother felt tremendous guilt because her son had SCID caused by the X-linked gene that she carried. In spite of long counselling sessions, she could not lose the guilt that she felt, and this affected her quality of life for many years afterwards. There is also the guilt that many parents felt, usually without foundation, that they had delayed seeking treatment – even if they only delayed as little as half an hour in the case of children requiring emergency care. Alongside this, is the guilt that some parents carried because they felt that they had not pushed the doctors hard enough to make a definitive diagnosis and so start treatment sooner than they had done. Guilt must never be underestimated because it can gnaw away at it the feelings and emotions and quality of life of those suffering from it.

Separation and Support When a child becomes sick with a life-threatening critical illness or accident, many parents expect that they will go through this terrifying ordeal together and that they will share the same feelings and hopes/aspirations, doubts and fears (Van-Dongen-Melman et al. (1998). However, in actual fact, parents of the same child might possibly experience the illness differently and so have to face different stressors linked to the illness, and learn to cope with it in different ways. As Van-Dongen-Melman et al. (1998 p. 190) discuss, ‘The illness sets limits to the expectation and experience of togetherness in the relationship and this can create loneliness within the marriage and sometimes great marital friction’. In the study by Gold et al. (1986), of psychosocial issues in paediatric organ transplantation, they reported that parents felt an increasing feeling of isolation which affected their ability to cope. This was exacerbated by both parents usually not being able to remain at the hospital whilst their child was recovering from the organ transplant. Reasons for this include financial problems, care of other children in the family, as well as general job responsibilities. This was also often the case with the parents of children with SCID. With these parents, the problems were often exacerbated by distance. Because there were only two specialist centres in the UK and one specialist centre in Germany, then parents often lived several hundred miles away from the centre. As at the time of the study, in the UK there was only accommodation for one of the parents available on site (usually the mother), then this often lead to separation of the parents for many months. In Germany, the situation was much improved because they had a system of Familien Hausen (Family Houses) in which not only both the parents, but the whole family (including grandparents) could live and

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spend time together and still be close to their child. This obviously reduced the accommodation problems that resulted in parental separation, but of course could not alleviate financial and job problems, nor the problem of looking after other children in the family when they had to go to school. These family houses in Germany were available not only for the families of children with SCID, but for the families of all children undergoing bone marrow transplantation in the hospital. When the parents/families undergo such physical separation, then problems arise caused not only by the distance, but also by experience. The parent who remains with the child undergoes a totally different experience to that of the parent who remains at home. The parent with the child has continual contact, which is good in one respect, but can also be negative in that they may well find themselves on an emotional rollercoaster as they live every single minute of that child’s treatment. The parent at home, however, may find it possible to block out what is happening with their child by carrying on their normal day-to-day life and by immersing themselves in their work and any other children they may have. However, the parents at the hospital often have tremendous support from the hospital staff, including such people as psychologists, social workers, and councillors, and the nurses and doctors on the isolation/transplantation unit, as well as great support from other parents going through the same experiences, whilst the parents at home have no such support – they have no one with them who can really understand what they are going through, and yet at the same time they have to be a ‘rock’ and a support for any other children and the rest of the family. So great can be the stresses during and after the transplant, that several partners came close to splitting up, as explained by one father, It was a terrible time for us. We were on the point of splitting, and I dare say, had I not left the . . . , it would have happened. We had a lot of support off of the family, but in respect that it . . . it was verbal support. There was no ‘hands-on’ support, because nobody understood . . . and no-one in the family could help us, because they didn’t know what they were doing. In . . . in their hearts, they wanted to help. There’s not one member of my family or my wife’s family . . . not one of them, and I can honestly say hand on heart, believed for one minute that we would still be together. Everyone of them thought that we would have split up. We came through, but I would hate to see any other couple have to go through it that same way as what we did. You’ve got to work through and build it up yourselves. You need support to do that. This German mother also explained one of the problems of separation because of the transplant. ‘I stayed with him (her son) in Ulm I was always with him. I was always there. But I forgot about my husband’. Problems with separation dogged the parents of this German girl, as with many others, but this was exacerbated by their daughter’s behaviour, as her mother explained,

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Her father, who had very . . . constantly driven to . . . . every week, always returning. And he was very conscientious. But he was always on his own. He always came to . . . . from . . .. . .. . . But we already had a problem. Sometimes we don’t do so well on our own . . . it depended upon the course of her illness, because there was also a story of tyranny (from her). It was a persistent problem we had to bear. She was a little tyrant, and it was difficult being with her. But her life was also very difficult. For some parents, however, the stresses brought them closer together: I was with him all the time. My husband visited us. I’d say it brought us closer, my husband and I. It goes either . . . or, I know. I heard this. But then we were lucky too. We were fortunate he only was there maybe a short time, so it wasn’t that bad. I think it would have been a different thing if we’d stayed a year or . . . like other parents, almost two years. (German mother) That was a complicated time for both of us. We had to leave one child here. She had just started school. It was very difficult. It was, I think, a very good year for us two. It brings the family together. We found it brought us together. Oh, we had arguments, we still do, but you have to pull on your strength together. (Mother and father of a German boy) His father and I had a bit of a hard time, you know, like, because of the separation an’ that . . . but, I mean, you know, it’s just like we had to be there for each other. (Mother of UK boy) It’s brought us together more. We’ve been quite lucky . . . we’ve been okay. (Mother of UK girl) I suppose now when you sit back and think, you think, ‘oh how did we ever survive that!’ But I think we’ve obviously got a very strong relationship anyway. And I think if it saw us through that, it’ll see us through anything’. (Mother of UK girl) In terms of relationships within an isolation environment and the role of the parent within the isolation cubicle, McKeever et al. (2002) in their study of mothers of children with SCID described the mothers’ relationships to the room and their roles within it as being complex. They found that the mothers did not feel at home in the room, but were cast by themselves and by the staff as ‘working visitors’. This idea of the mothers as being the visitors was reinforced by the fact that every evening, the mothers had to leave the isolation cubicle, which many of them found very difficult to do as it was against all that they believed in as being a mothering role. These mothers also experienced problems and fractures in their normal support systems and relationships, which they found became of a secondary nature with their family and became almost non-existent with their friends. In other words, as well as the physical isolation that they

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encountered within the hospital and the isolation unit, they found themselves, often at their own insistence, in a social isolation environment. In their study of parental psychosocial outcomes in paediatric liver and/or intestinal transplantation, Tarbell and Kosmach (1998) found that although a majority of the parents in their study showed evidence of clinically significant psychological symptoms, on average, fathers presented with greater distress symptoms than did the mothers, which contrasts with previous studies on parents of children with disabilities or chronic illnesses in which mothers have consistently scored higher than fathers in terms of psychological distress (Eiser & Havermans 1992, Goldberg et al. 1986, Kazak & Marvin 1984, Timko et al. 1992, Thompson et al. 1996). The reason for this was felt by Tarbell and Kosmach (1998) to be due to the fact that all the fathers in the study actually stayed at the hospital with their children and were actively involved in their child’s care, so disproving the suggested wisdom that mothers are more emotionally involved with their sick children than are fathers. It may be that the fathers just need to be able to take an equal role in the care of their children, and to feel welcomed at the bedside of their children. Perhaps that is something that hospital staff need to be aware of and factor it into their care and social constructs. This point was supported by Hueur (1993) who found that fathers rated their overall intensive care experiences as being more stressful than the mothers rated their experiences. Russell (2007) on commenting on this fact thought that this may be due to the fact that most fathers returned to work within 24 hours after their child was stabilised, and so was related to their lack of parental participation, as well as their access to supporting mechanisms and a supporting environment. In this chapter, much has been made of the problems of the parents adapting to new roles. It has been mentioned above that fathers often have more difficulties in mothers in coping with the pressures and stresses when their child is in hospital with a life-threatening, or an acute, illness. This point does need labouring somewhat, because most of the literature on the stresses that parents looking after their child in an intensive care environment or a high dependency environment, such as an isolation and/or transplantation unit, is almost entirely concerned with mothers. They have been the focus of most of the research that has taken place in this field. This probably came about because, in these situations, most fathers appeared to be absent because they are continuing at home looking after the other children or going out to work and helping to keep the family financially solvent – and this is a crucial role that they have to take on, because whether the child in hospital lives or dies, the family will need to go back home and to pick up their lives, as much as possible, from where they left off. However, that does not mean that the father is not affected by what is going on in the hospital. In fact, in many ways they may be more affected because they are not present throughout the whole experience, as has been discussed earlier in this section. It is incumbent upon hospital staff to be aware of this, and to make every effort to involve the father in the experience as it happens, whether he is in hospital or at home.

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One of the first children I ever nursed was suffering from a severe head injury and was not expected to survive. Throughout the long period he was with us, his mother and grandmother were in his room every single day for the whole day and a large part of the night, talking to him holding him and just generally caring for him. His father, on the other hand, being a farmer and having to run the farm on his own just could not spare the time to stay with his son, because there were crops to get in and animals to be looked after. After some weeks, reports came back to the hospital that he was drinking heavily, and there was much condemnation of him. But no one at the hospital had taken the time to actually look after him. He felt cut off from his son and his wife, and felt that he had no part to play in the care of his son, even though he was experiencing all the stressors that have been mentioned in this chapter. As what you do not know can be more frightening than what you do know, all his fears, his anxieties, his concerns were magnified because he had no support, and consequently found support in alcohol, as the only means that he could get through each day. Luckily, after a few months his son came out of his coma, and was discharged home, where his father could once more take part in his care. His drinking ceased immediately and family life was resumed. However, it is a salutary lesson that hospital staff should not forget the carer who is at home just because they are out of sight. Two other interesting points came out of the study by Tarbell and Kosmach (1998). The first was that higher stress was apparent in those parents where a younger, rather than older, child was undergoing transplantation, which may be related to the generally high demands of caring for a younger child. The second interesting point was that greater parenting stress was found in those parents whose son was undergoing a transplant. According to Lamb and Laumann Billings (1997) and Frey et al. (1989), male gender has been associated with increased parenting stress in previous research on disabled children, with fathers being more affected in a negative way by having a son with a disability than were the mothers (Tarbell & Kosmach 1998). Occasionally, some of the stress could actually be brought about because the parents were together and not separated, as this discussion between the parents of this UK boy with SCID demonstrated when they talked about the stresses on the family (Box 11.2). This latter point is supported by Gold et al. (1986) who discuss the fact that in those families where both parents remain with their child at the hospital, a different set of stresses can occur. They point out that the fact that both parents are together virtually 24 hours of the day, seven days a week for several weeks, if not months, then additional burdens are placed on the relationship. These extra burdens, combined with the fact, as has been discussed a little earlier in this chapter, that each parent will use different (and sometimes conflicting) coping mechanisms to deal with their anxieties and their fears and all the other stressors that they are encountering, can often result in increased problems in the relationship between the two parents.

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Box 11.2

Discussion on Separation Between the Parents of a UK Boy

Mother: ‘But we got on each other’s nerves because we were there together. We were with each other all day.’ Father: ‘We were here full time. Normally I’d be at work all day and usually come home in the evening.’ Interviewer: ‘What about when . . . (father) went home to go to work?’ Mother: ‘I found that hard at first, but I coped. I think it was more a relief that he’d gone home.’

Denial One UK boy’s parents had difficulties, as his mother told me, partly because of separation during the period of isolation, but also because of denial on the part of his father, ‘It was very difficult. His father wouldn’t accept how sick his son was. He went to work and I was stuck at the hospital. We didn’t have rows or such, just things were very different and very difficult’. The problems brought about by this denial on the part of the father, as well as the separation, also came to the fore when the mothers of these two UK children were reminiscing about the time of their children’s transplants: He would never admit to it, but especially when his son was in hospital, I found out that he wouldn’t admit that he had a son, and I found it was all on me, you know. And then when he came out, it seemed there was so much attention, like, around . . . (child), there wasn’t any time for us, you know what I mean. I mean, I don’t know if you remember, but he was working and I was up at the hospital, and I found that when he come to pick me up, he couldn’t even go into the bubble. And you must remember what he was like with changing nappies. He went in there . . . he wouldn’t change a nappy (laughs). But I did feel like it was all . . . there was a lot of pressure’. (First mother) Stress! There was lots of stress all around everybody. I think you end up blaming each other and the family. End up . . . I didn’t think he did his fair share, and he wanted to do a lot of running from it. But he convinced himself he was going to hospital . . . he wasn’t going to hospital. You know that, don’t you? This man here convinced me he was going every day to the hospital, but he wasn’t. He couldn’t . . . He really convinced himself in his mind that he was, but he wasn’t. (Second mother) One father, according to his wife, even went so far as to deny to outsiders that he even had a child, let alone that that child was desperately ill in hospital!

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Effects on Other Members of the Family

Siblings ‘Sibling bonds are intense, complex and of an infinite variety. Siblings share a common cultural background and outlook and they share their parents’ time, interest and love. Emotional relationships between siblings are seldom static, rather, they display varying degrees of loyalty, companionship, rivalry, love, hate, jealousy and envy’ (Walker 1988 p. 208). There are other people within the family to consider as well as the parents, for example the siblings. All siblings of children who have a very severe or life-threatening disease, no matter what the actual disease involved, must experience changes in their daily lifestyles due to the upheaval of normal family life (Byrne 1994). In their study from 1990 on the effects of childhood cancer on the whole family, Martinson and colleagues concluded that the siblings, whether the child with cancer survived or died, shared feelings and memories of isolation and separation from the child and from the parents. Those siblings of children who were still living recalled missing them during their periods in hospital whilst the group whose sibling had died all said that they missed them, with the notable exception of one boy who denied the existence of his younger sibling. However, both groups of children were worried about the possibility of them becoming sick themselves. Those children whose sibling had died suddenly only knew that the cancer could end in death when their sibling was actually dying. The effects of having a sibling with cancer consist of both physical and psychological problems. Byrne (1994) also noted that as well as their personal distress, siblings of children with cancer are also affected by other family events, particularly the financial burden that occurs within the family/household as a result of the illness. According to Burns (1982) healthy siblings appear to experience the stresses, to an equal or greater extent, that are similar to that of the sick child. These same siblings have identified a lack of knowledge and a fear of the unknown as the main cause of distress (Byrne 1994). Because of the genetic nature of the disease, many of the SCID children have no siblings, and those who do have siblings tend to have much younger ones, as parents often waited until they knew, or felt, their child was going to survive before thinking about other children. Therefore, most of the siblings were not affected because of the actual transplant, although they may be affected by the continuing chronic nature of the ill health and psychosocial functioning. During my visits to the families of children with SCID, I never became aware of serious problems between the children of the families, as does happen when children have BMTs for reasons other than SCID. For ex¨ ¨ ample jealousy, which was reported by Blumke (1994 p. 1) in ‘Punktchen Magazin’ with the headline, ‘Eifersucht am Krankenbett: “Schade, daβ ich ¨ nicht krank bin . . .”. Was gesunde Kinder uber ihre kranken Geschwister

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Box 11.3 1999b)

P¨unktchen Magazin Jealousy at the Bedside (Translation Vickers

Jealousy at the bedside ‘“My brother has it great”, says Moni. Her brother Sebastian, 4 years old, has leukaemia. At this moment he is once again in the hospital. It is a hard time for the family. Their mother is often with Sebastian. Moni spends much of her time alone – and she is annoyed with her brother. “He can play all the time with mummy. Why can I not be sick?”’ ‘The parents of Moritz are also in the hospital every day. When Moritz dies, his sister thinks, “What luck, now they will have more time for me.”’

denken’. [‘Jealousy at the bedside: “What a pity that I am not sick . . .”. What healthy children think about their sick siblings’] (Box 11.3). Indeed, far from this being the case, many of the siblings of the SCID children are quite protective, as for example the siblings of this German child, as reported by the mother ‘In the first year they were naturally protective of their brother, and everything was fine. But, God be praised, that time came to an end. But the first two or three years, it was all about their brother, everything centred around their brother’. This attitude of caring and protecting their sibling was also a major theme of the mother of a UK girl who explained that her sons have ‘. . . the patience of a saint with her. They’re terribly gentle and calm with her’. One sister in Germany, when asked how she felt about her brother, who had survived treatment for SCID, replied, ‘I’m very careful with him’. Other than being more careful, there tends to be the normal sibling rivalry. As described by these three UK mothers: I honestly think they’re just normal kids. They fight like crazy, they do . . . even at this age. They squabble like any other children. But if one’s hurt, or if one’s getting told off, the other one’s at their defence. They have a very good relationship, actually. We’re quite fortunate. Could be more difficulties, but I think, on the whole, they have a good relationship. They do fight. They fight just the same. They don’t treat her any different. Just one parent expressed concern in this area. This UK mother reported, I’ve found they’re very jealous of their sister and can cause jealousies which weren’t there before. They kind of gang up on her if she gets too much attention. One sister loves her. She loves her, kind of thing, but at the same time she can only take her for so long. She can’t take her company for more than an hour. Another of her sisters, on her good days can stand her for

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twenty minutes. Her other sister was very, very . . . she was very young and had been the baby for seven years when I had this daughter with SCID, and it caused immense problems. And with the result that her jealousy turned into tantrums. And I think she lost confidence in herself for a few years. Her brother has more patience than any of them with her. He’ll play with her. Now he loses his rag after a while because she doesn’t really listen, and he’s not a very clear speaker, either. Kind of mumbles his words, you know. He’s not a great communicator, so therefore she’s not really listening to him at all, and he’s get frustrated at her. But he does have more patience than the girls. The problems with this child’s siblings occurred because they were quite a bit older than her and could appreciate the loss of their mother for many months whilst she was in London with the girl. This mirrors the situation as found with children with leukaemia as described in the piece from ¨ ‘Punktchen Magazin’ above. If the siblings of other SCID children had been around at the time of transplant, then they may also have had the same feelings. Interestingly, one of the UK SCID boys in the study had a younger sister who was more recently treated for SCID. This boy drew a picture of his family, as all the children in the study were requested to do. His picture (Figure 11.1) shows himself at a distance from his parents who are holding his sister and talking to her, whilst apparently ignoring their son. He was old enough when his sister had a BMT to feel the absence of his parents, although they do not live too far away from the transplanting hospital. Particularly he must have felt the isolation of realising that all their attention now appeared to be focused on someone else. Perhaps sibling jealousies might have come to the fore more if the siblings had also been asked to draw a picture of their family. According to Simon (1993), the first reported findings that hospitalisation could affect the siblings of the hospitalised child were noted by Sipowicz and Vernon (1965). In 1985, Craft et al. described the emotional and behavioural changes that occurred in siblings of hospitalised children, and found that the number of behavioural changes was significantly related to sibling age, sibling relationship, the nature of any explanations given to the sibling, the sibling’s fear of getting the illness, where the sibling was living at that time and changes in parenting. Simon (1993) found that the siblings of hospitalised children perceived the event as a stressful event, and indeed she found that the degree of stress and anxiety that these siblings experience was similar to that of the child who was in hospital. Interestingly, she found that children who stayed with neighbours whilst their siblings and parents were at the hospital had lower perceived stress, possibly because they were removed from the situation and from people intimately concerned with the situation – although this does contradict the findings of Craft et al. (1985) and Knafle (1982). On the other hand, Simon (1993) found that children who visited their sibling in hospital every day demonstrated a high degree of perceived

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Figure 11.1 Drawing of his family by John. Previously published in Vickers P.S. (2006) Communicating with children and families. In: Peate I. and Whiting L. (eds) Caring for Children and Families, Chichester: John Wiley and Sons, pp. 25–48 (p. 40).

stress, which she suggests shows that intense contact may be more anxietyproducing than it is anxiety-reducing for children. She does, however, concede that there may be other factors that influence the level of anxiety, such as preparation for the visit, the actual time spent in the hospital, and the explanation given to them of their sibling’s condition. In addition, the sights and sounds of the hospital itself might well be anxiety-provoking in these children who are not ill themselves, but only visiting their sick sibling. Cornman (1993) reported from her study that siblings were most affected by illness-related variables in patients, such as stage of disease, pain or discomfort level, as well as visible signs of illness in their siblings. In addition, she found that the amount of stress that the majority of parents as well as siblings reported represents a potential risk that cannot be overlooked and she concluded that it was important that support groups for each family member, whether parent, patient or sibling, were identified to ‘. . . help in his or her unique struggle’ (Cornman 1993 p. 1565). The study by Cornman (1993) reinforces the paper by Siemon (1984) who identified the siblings of children with special health needs as a focus of concern: she also makes the point that because of the advances in medical technology that allow children to survive and to live, but often with long-term special health needs, then children now have to confront their relationships with their ill or disabled siblings much more than previously, and given that siblings play such an important and such an influential role in each other’s lives, then one child’s illness or disability will affect their

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siblings to some extent. Because families are a social system, then distressing one member affects both the system and each member within it. In addition, Simon (1993) makes the point that there is evidence that particular children are especially vulnerable to difficulties when their siblings are ill and disabled. These children who are vulnerable to difficulties include children who were in the midst of a developmental transition and have a lowered ability to tolerate other stresses and frustrations. This group, for example, include children starting school as well as children going through puberty. Other children vulnerable to the difficulties of living with a very sick or disabled sibling include those children who have few, or poor, coping skills and who will be more easily overwhelmed by such stressful experiences, including very young children or children with poor support systems. Children with poor support systems include those with poor relationships with their parents or who receive inadequate care from their parents. Finally, Simon (1993) notes that older siblings who are closest in age to a sick child are most likely to be affected by, and have the most difficult adjustment to, a child with special health needs. A study by Gallo et al. (1992) supports Simon (1993) in regards to the fact that sibling adjustment may be selective and may vary depending upon specific characteristics and family environment influences. They found that, as a group, well siblings of sick children do not experience a significantly higher incidence of behaviour and social competence problems than normal, but that siblings within certain groups may experience such problems. Lavigne and Ryan (1979) also report that some siblings of children with chronic or life-threatening illnesses may be more at risk of problems than others. For example, they discuss that younger siblings may incur more adjustment problems than do older siblings because the younger ones are uprooted and displaced from their position within the family as their own brother or sister assumes a more dependent role within the family. However, they are at pains to point out that although most studies have emphasised the negative impact of illness or disability, it is important to note that there are also positive effects as well. Hunt (1973) noted that some siblings responded favourably to the added responsibilities that they were given. At the same time, Lavigne and Ryan (1979) state that because some positive effects are seen to occur, then the presence of adjustment problems among siblings must not be assumed, although, like the previous researchers, they do consider that collectively, siblings of children with chronic illnesses do seem more likely to experience adjustment or behavioural problems than do siblings of healthy children. One of the points made by Lavigne and Ryan (1979) is that when a sibling takes notice of physical illness in their brother or sister, they may possibly undergo a change in their own ‘body image’, perception closely followed by subsequent changes in behaviour as a result. Another cause of changes in sibling behaviour could be changes in interactions within the family. Simon (1993) also found that children who identified more changes in their parents’ behaviours had higher levels of stress than those who identified fewer changes. She suggests that the more changes in the parents’

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usual behaviour, the more stressful the situation may be for the children. This may well be linked to the loss of familiar behaviours and relationships and the difficulty that the siblings have with coming to terms with these changes. This, she found to be similar to the findings from Craft et al. (1985). Nolbris et al. (2007) note that when a child or adolescent in the family is being treated for cancer, then the situation for the family, including any healthy siblings, is changed. This also involves the pre-requisites to maintaining a sibling relationship also changing. Previous studies have shown that siblings do not feel included in what is going on with the sick child, and in these circumstances they feel that their needs are poorly met ¨ et al. 2005, Freeman et al. 2003, Murray 2002, Nolbris & Hellstrom ¨ (Bjork 2005, Scout-Findlay & Chalmers 2001, Woodgate & Degner 2003). In their study, Nolbris et al. (2007) found that the cancer affected the everyday life of the healthy siblings, leading to new very labile daily life experiences with many ups and downs. Von Essen and Enskar reported in 2003 that when a child with cancer felt well and was happy, then their siblings had no worries. Certainly, the siblings in the Nolbris et al. (2007) study experienced anxiety about losing the child with cancer when they first heard about the diagnosis, and all the time there was a feeling (sometimes more, sometimes less) of the possibility of threatening changes in their everyday life, together with existential thoughts about life and death in general. An interesting finding from the Nolbris et al. (2007) study was that the cancer diagnosis appeared to make the sibling relationship clearer and closer than it had been before and there was an awareness amongst the siblings of their being a special, strong and close relationship with their brother or sister. The siblings trusted and stood up for each other and would make contact with each other, often by telephone, as well as providing company as and when needed. The siblings felt that it was important to be loyal to their sick brother or sister, although this could cause some problems in their daily life when there was conflict with loyalty concerning their role with school and friends as against their brother or sister and their needs. There were feelings of fury and envy when the sibling felt that the sick child got everything and yet was still not satisfied, and there were moments when the siblings did not want to meet their sick brother or sister. However, Nolbris et al. (2007) reported that the feeling of love between the siblings was always there, even in the difficult moments. Finally, Nolbris et al. (2007) concluded from their study that it was very obvious that the bond between the siblings became strong, close and very clear after the diagnosis of cancer. The normal everyday life of the siblings was changed to a new everyday life. The healthy siblings admitted to a responsibility that they felt they had to protect and act as an advocate for their brother or sister to people outside of the family, and they always tried to see and appreciate the needs of their brother or sister, but felt that they did not always succeed and when that happened they felt bad about themselves. There were periods of joy in the relationship between the siblings, but also an awareness from the siblings of any sudden changes to the condition

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and behaviour of the sick child, and they found that watching their brother or sister with cancer having doubts about the future was hard for them to cope with.

Grandparents As regards other members of the family, such as grandparents, the overwhelming opinion from the parents is that their children were spoiled by others in the family. According to her mother, this UK girl’s grandparents, who live with the family, spoiled her terribly. ‘Her grandmother does, her granddad does. They spoil her rotten. She gets away with murder. She can say what she wants; she can do what she wants with her grandparents. Granny’ll take her side. Whatever my daughter says is right. She’s spoiled. If she asks for it, generally she gets it. If I don’t give it to her, then there’ll always be somebody around to give it to her. I think it’s everything to do with the fact that at home, my mother-in-law several times says “Oh don’t, she’s ill”, but with the boys . . . It’s a case of “She’s only got another fourteen years at home, then she’ll be gone”, you know, “you’ve got to be nice to her”, and whatever, and “Oh don’t, she’s skinny”.’ To which the girl immediately countered with, ‘I’m not skinny, I’m fat’. The above comment to her only having another 14 years at home is to do with the fact that in Asian families, when the girl marries she leaves the home and goes to the in-laws home, whilst the boys bring their bride home, and is not a comment on how long they think the girl will live. Incidentally, whilst several of the UK parents mentioned that their children who had had SCID were spoiled, these comments were unique to the UK parents. No German parent thought that there child was spoiled – possibly because most German children are, to UK eyes, spoiled, so it is nothing special to spoil a child in that society. In the UK, however, somehow there is a feeling that children should not be spoiled or else they feel it will bring out the worst in their children. Certainly this theme cropped up many times in the interviews, for example, another UK mother said of her daughter, ‘I think they all spoil her rotten. They’re always giving in to her’. There are similar feelings that grandparents have to those of parents when considering a grandchild with a critical or life-threatening illness – namely that everything in the world is topsy-turvy, and unfair. Parents often feel that their children should bury them, not the other way round, and for grandparents this sentiment is even keenly more felt. Several of the grandparents whom I met during the course of the research felt that they would be happy to have given their life if it meant that there grandchild would survive (the ‘trade off’ mentioned earlier). One regular comment from grandparents alluded to the fact that they ‘had had a good life’. In addition, it must not be forgotten that, as well as grieving for the sick child, the grandparents may well also be grieving for their own child (the sick child’s parent). Sometimes, because of the alienation that can occur in these situations (see Chapter 12), grandparents feel that, as well

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as losing their grandchild, they are also losing their own children. This was discussed in a recent phone call with the grandfather of a child with SCID, who felt that he and his wife were excluded from the grandchild and the parents of that child because they were alienated from them as a result of the illness and treatment. This grandfather, as well as wanting to understand and help his son and daughter-in-law, also felt a sense of loss – a loss of the previous relationship he had had with the parents of the child, as well as with the child. As well as this grandfather, some grandparents did feel excluded from the whole experience of having their grandchild desperately ill in hospital. Partly this was due to distance, as has been alluded to previously, in that with only one or two specialist centres in the two countries, then they were bound to feel distant from the whole experience. In addition, the very strict rules in terms of who could or could not visit the children in their isolation cubicles, meant that even if they were living in the vicinity, the likelihood of there being able to be with their grandchildren, even briefly, was very negligible indeed. Even though, in Germany at the least, grandparents could stay with the parents time in the Family Houses, there was still restricted access to the isolation unit, but a least these grandparents could better help support their own children. The third possible cause of this feeling of exclusion that many of the grandparents had was down to vocabulary. SCID is a very complex disorder and the treatment is also very complicated. Parents, after a time, because they are in the hospital so much, learned to pick up the jargon and to understand what was going on. Outsiders, on the other hand, find it very difficult to understand. One of the many comments from parents in the SCID research project was that no one other than their partner and the hospital staff and the specialist unit had any understanding of the disease, its treatment, and particularly of what they experienced in their time there. Similar problems to these also occurred with other members of the extended family, such as aunts and uncles, and cousins, as well as friends.

Going Home To many of the parents who were interviewed for the SCID research study, being discharged from hospital and going home was both a longed-for event, but also a frightening one. It was frightening because there was a lot of concern over whether or not they could cope once they were back home on their own. This is similar to the feelings of many parents whose children have been in hospital for intensive care or because they have life-threatening illnesses. Parents in NICU in particular, especially those who are first-time parents are very concerned about having to look after their baby at home, when up till then the responsibility has been that of the hospital staff. They need much reassurance that there will be health professionals who will be able to help them back in the community.

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Another problem that needs to be considered by health professionals, certainly those involved with children with SCID, before discharge, is that many of the parents have unreasonably high expectations of what they and their children will be able to do once they are at home. Many, in my experience, felt that once home their child would be cured and there would be no restrictions on what they could do. In actual fact, this is not so, because although the BMT will have been seen to have been successful before they are allowed to go home, the children will still not have a fully functioning immune system. Their immune system may not be fully functioning for another two, three or even four years, and so precautions, although not as strict as when the child was in hospital, will still need to be taken to prevent the child from picking up infections that could prove to be very serious indeed, even normal run-of-the-mill childhood infections. Usually, all precautions against catching infections can cease once a child has had all its childhood immunisations and they have been found to be working. Returning home can also exacerbate parental relationships which may have been subsumed whilst the child was desperately sick in hospital, particularly if there has been separation with one parent staying with the child in hospital all the time whilst the other parent has had to try and get on with a normal life at home, possibly looking after other children and continuing to work to prevent financial problems. Some parents explained that they needed to have to get to know one another all over again because of the time spent separated, but also because each of the parents had had different experiences over the past 12 months or so, with one parent experiencing all the labile emotions of being with their child all the time, of experiencing life in an acute hospital environment, and having made different friends amongst the hospital staff and the other parents on the unit, whilst the other parent at home had had totally different experiences to cope with, and some of them said that they felt resentful of their partner who had been with the child all the time. This also led to different needs of the parents once they were united again as a family unit with their child/children. The parent who had been away with the child often felt that they just wanted to get on with a normal life, but of course they could not because their child still required special care and attention, whilst a parent who had stayed at home needed to get to know their child again. Often the child that they had last spent any time with would have been just a baby of a few weeks, but now he or she could be 18 months old – or even older. In that intervening time, as well as the child experiencing so much, the child will also have progressively developed, and will certainly not be a baby any more, and the parent at home will feel that they have missed out on seeing their baby develop to that stage. This problem of different needs and expectations of the parents was addressed by this UK father talking about his daughter, particularly when he talked about her going home after nearly two and a half years in isolation in hospital,

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I think when you’re both at hospital, you have a common goal which is effectively trying to get the child home, and so, well . . . the illness itself is obviously going to generate a lot of stresses. Quite often it’s a bit like revising for an exam. Once you’ve actually taken it, it’s a question of where do you go from here. So . . . a lot of it, I think, is really down to the success, because you get a degree of anticlimax afterwards. You’re going to have exactly the same problem of course if you lose the child. You’re working as a team until some result is obtained, and then you lose that bond. There is a sort of common aim. The sort of problem that we got, I suppose, at home is that we’re not both as cautious, and I think that you’ll find that most partners . . . there are differences. If you get a child home, then those differences can become much more polarised because feelings are going to be much more high. There’s going to be an air of caution. One person is going to be much more cautious than the other. So this is . . . this can create differences. Of course, as has been mentioned previously in this book, whilst the child may be discharged home, there may still be long-term medical problems to cope with, for example the child may still need certain drugs, or they may need a certain diet. This UK mother still has problems to cope with many years post-transplant, as she explained, I have to wake her up (every night) to give her her (growth hormone) injection, you see . . . that can only be given at night when she’s (going to go to) sleep. She has been quite a good sleeper other than her bed wettin’ quite a lot. It’s very tirin’ for me, you know. When I get time to myself, it should be my time, an’ I have to go up an’ change, you know, change what I’ve just been explainin’ . . . three or four times a night, an’ it’s not her fault, you see. I have to wash, wash, wash, an’ . . . oh. There’s nothin’ worse, ‘cos all you want to do is sleep. But it can’t be nice for her either. In addition, the worry of relapse and of serious illness is never far from the surface for many of the parents in the years following discharge from hospital: Of course, it (illness) still causes us to have very many worries. It’s very clear that it gives us problems. (Mother of German boy) We’re more careful. If he runs a fever we always . . . we’re always scared. I guess that will be always . . . we’re always scared. We’re always afraid he might die because he had this thing. With other parents . . . we talk to each other, and they always feel the same, so they give medication, you know, if they have a fever for two days. (Mother of German boy) To put it plainly, I’m rather concerned about my son when he is poorly. (Mother of German boy) So, although being discharged is obviously a wonderful experience for the family, and the child, there is still a long way to go for that family and child before they can really feel comfortable with themselves, and

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this can be a very worrying situation. In fact, the return home can be an overwhelming experience – particularly for the parent who has been at the hospital with the child. She (or occasionally, he) may feel a stranger in her (his) own home, and also feel a stranger to other family members and friends. Many feel that they will never be comfortable with the situation, and some of them are still tortured by what they went through with their child in hospital. But at least, once at home, the psychological healing process can begin.

Conclusion Having, in a professional capacity, supported parents expecting another child after losing one with SCID, even to the extent of attending antenatal clinics with them, I have personal experience of the fears and stresses that those nine months can engender. Even after the birth, and during the subsequent transplantation, the stresses are manifold. Figley (1983) identified several problems experienced by families of a chronically ill child, which are also, according to Hare et al. (1989) recognizable in the child undergoing BMT and the family. These include:

r r r r r r r r r

strained family relationships modifications in family activities and goals burden of increased tasks and time commitment increased financial burden need for housing adaptation social isolation (discussed in Chapter 12) medical concerns differences in school experiences to the child patient grieving associated with possible death

To these can be added the possibility of the development of a posttraumatic stress disorder which can be present from the time of the return, or can occur some time after the return home (Chapter 14). One of the many questions that I was often asked when working with children who had SCID, and having to deal with all the emotional highs and lows, and particularly with the many deaths that occurred in the early days, was where did I get my support from, particularly when people saw me ensuring that parents of the child who had died, other parents on the ward, as well as the ward staff all had means of support following a death. At first I was stumped by this question because I had never really given it much thought, but then it came to me. Just as I gave support to the parents, so they gave support back to me. It was a mutual thing. One of my memories is of an evening when a child who had been on the ward for over 12 months died, and I had spent some time ensuring that all the parents on the unit felt supported, and that none of my staff was going to go home to empty accommodation that evening, but would have someone they could go to. Suddenly, once I had handed responsibility over to the night staff and was about to go home myself, two sets of parents of children

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with SCID on the unit came to me and asked what I was going to do for support. Not having an answer, they just told me that I had to go with them to the nearest pub and we would have a drink or two. They had been as concerned about me as I had been about the other people on the unit, and that made me realise that care and compassion was not all one way – from professionals to patients and families, but worked both ways, and that we were all in the situation together, professionals and families and we all needed each other. It was a salutary lesson for me. To conclude, parents of children who are critically ill and are being nursed in such intensive environments have completely different needs of those parents whose children are not critically ill, but still being nursed on an intensive care unit (Russell 2007). A need has been defined by Buskist and Gerbing (1990) as a requirement at a particular point in time, either a collective requirement in the individual requirement, and Russell (2007) found that from her literature search, three main needs emerged, namely the need for information, the need for hope and the need to be with their child. Perhaps the hardest of these three main needs for the parents and the family, to obtain and to hold on to, is the need for hope.

12

Attachment and Alienation

Introduction As has been seen from the study into children with SCID (severe combined immunodeficiency) who are isolated for many months, or even years, from birth or shortly afterwards, the children tended to be of a solitary nature. Indeed, a major theme to emerge from this research was ‘alienation’ – alienation of child from family, family from the extended family and society and child from peers. The dominating factor leading to this state of alienation is the isolation of the children, and consequently of the family, from normal experiences at this particular time in their lives. For the research project these feelings of isolation were explored by means of parent and child interviews, parent and teacher questionnaires and the children’s drawings. Reasons for these feelings of isolation, and actual isolation, are put forward in this chapter and include poor parental and child bonding, isolation of children during very important stages in their development and play and the traumas experienced by parents and children. Children in other groups with similar experiences, for example those who were very premature and required treatment and nursing in a Special Care Baby Unit are also reported as having equivalent problems.

Alienation Alienation can be defined as an inability to develop positive relationships with others, and alienation exists between parent and child, between the parents, parents and extended family, child and nuclear/extended family and parent/child/family from society as a whole. In previous chapters, the topic of the children tending to be of a solitary nature was mentioned, and obviously, the question needs to be asked as to why these children are solitary children. Undoubtedly some children liked to be on their own, but, as one German mother said, about her daughter, Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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‘Her ability to mix socially with her own peers is quite a problem for her’. This theme recurred throughout the interviews with the parents, particularly the parents in the UK, although some German parents reported this phenomenon as well, for example, this mother, ‘He is anxious being with other children’. A mother in the UK was perhaps more brutal, or more honest, in her assessment of her daughter – ‘She can’t relate to other children’. Not all children, particularly in Germany, were reported as having this problem. According to one mother, her daughter ‘. . . has no problems with other friends. She has so many friends’. Other German parents echoed this with comments of their own, such as ‘Yes, she has other friends’, ‘She likes to ride, and also, she plays with children and with friends’, ‘He has many friends, so, playing football’. When this mother in Germany was asked if he had many friends, her unequivocal reply was, ‘Oh definitely, yes, one could say that’. Some of the German children, however, did have problems with children of their own age, as one mother explained, ‘She seeks out the little children. Well, she only ever plays in the Kindergarten with the little children. In August of this year, new children arrived. She plays with them. With the larger, the older children, she does not play’. For this boy in Germany, the problem also appeared to be relating to boys, as his mother told me, ‘He gets along better with girls than with boys. Little older girls, like about five year old, six year old girls’. For another German boy, it was larger groups of children that were the major problem, as discussed by his parents: Mother: ‘He is not so good with many children. With one or . . . it’s also exactly the same in the Kindergarten’. Father: ‘It is difficult when he is in a big group. It is easy for him to play with one or two . . . boys or girls. It’s a little difficult for him in a big group’.

The majority of German children, however, according to their parents, fell into the category of sociable children, as summed up by the mother of this boy who reported, ‘He is with children every day. He likes this, yes. And he is very popular with the children. The older, the younger, and those of the same age all treat him as equal’. As already stated, a different picture emerged with the UK SCID children. Even when the parents told me that their child had friends, there were often caveats, as in the case of this boy, whose mother told me, in response to my question concerning his relationships with other children, ‘On the whole he’s very, very good, you know . . . considering, like, the way he’s been brought back into the community’. With some children there was a delay before they made friends: ‘Once he gains confidence, e.g., befriending another child or person, then he acts

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normally and is not withdrawn’ (father of UK boy). ‘He is shy at first, but then he’s usually okay. He likes them, and doing things with other people’ (mother of UK boy). Some children often had no insight into their problems in relating to other children, as, for example, one girl living in the UK, whose mother explained, She would say to you that she gets on well with other children. But from watching other children – their reaction to (her daughter) is – they’re not keen. So she’s not aware of the effect she has – her behaviour has on others. ‘Oh no, it’s !’ I’ve seen kids do this as she comes down the road. Sad isn’t it! is very immature – and you know what girls of thirteen are like. They want to be seen as normal. ’s not like that, because – I think they find her a bit embarrassing as much as anything. They can’t really cope. Those who’ve since junior school are able to cope with her, and most of sort of known the friends she has at the moment are friends from junior school They’ve adjusted to and her little wiles. Other girls are just not prepared to go on with . As mentioned earlier, the SCID children could often relate to other children, but not children of their own age, as this little girl’s mother confided in me when I asked her if her daughter got on well with children of her own age, No, she doesn’t . . . there’s no children of her own age that play with her around here. They’re much more grown up. Younger children . . . she’s much better off with about the seven to eight age group. She communicates fine with them. That’s about her level. Older children have no patience and are way ahead in what they play with. She’s not shy or anything, and she doesn’t lack confidence. It’s just people get bored with her. They push their bikes and run away from her. One of that girl’s problems was her deafness, and that, as her mother told me, does cause difficulties with other children because . . . children don’t look at her . . . now we’ll tell kids that you have to look at her when you speak and then she’ll understand you . . . but kids forget, you know. They’re busy playing their own games and they’re too up in it . . . into it. They forget to say, ‘we’re going down to such and such a place thinks and we turn that corner and we go around the field’, you know. they’re not playing with her because she didn’t understand what they were saying, and she comes home crying. Another girl, who was also deaf, had similar problems, as her mother explained to me when I visited the family,

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An’ she couldn’t make friends, an’ all the children used to be, you know, nasty to her, because she had such a loud voice. Kids are just kids, aren’t they, at the end of the day. But she really had no friends, you know. The kids used to really, really ridicule her, an’ then she had her hearing’ aids, an’ in the end I used to have to replace the hearing’ aids myself ‘cos she threw the hearing’ aids that often, ‘cos the children used to call her deafhead . . . deafhead. She used to throw them on the floor. How can a kid be expected to be . . . I mean, it’s hard for an adult to cope with that, but at the age of five, six an’ seven, it’s an extremely large, you know, thing to take on, an’ it would give you this type of defensive attitude that she’s now got, you see, towards other children. You’d be wanting’ to be like them, wouldn’t you, you know what I mean, an’ she definitely isn’t, because of her size an’ whatever problem she’s got, an’ she never will be, but she would love to be an’ that’s all what matters to me, you know. Yet another girl in the UK had problems because of her speech and general behaviour. According to her mother, She is quite funny at times, but . . . children of her own age she used to avoid completely . . . she’s now getting better. She does stand out a bit because of her height, and her speech has been bad . . . and because she is very heavy. I mean, even despite her height, she’s terribly solid. If she was clumsy, which she has tended to be, they would be frightened of her because she might hurt them. Again, as with so many of the SCID children, that girl got on well with much younger children as well as with much older children, as her mother explained, ‘. . . wonderful with the little babies and children much younger than her. Very good with the boy’s friends, and they are amused by her. One of them said she’s like something out of a Disney movie. Children of her own age group now are tending . . . they tend to be asked to help her . . . and so they understand and sympathise with her. And so there’s at least a relationship building up, even if it isn’t a peer relationship. Her teacher also commented on her poor relationship with children, and speculated on a possible reason for this, other than her poor speech. She reported that she had ‘. . . very poor interaction with other children. Reasons? Considerable adult input at home’. Many of the children were their own worst enemies when it came to making friends because of their behaviour and attitudes towards other children, as the mothers of these four children in the UK mentioned:. I wouldn’t say he’s the most sociable of children, but he’s fairly . . . choosy is the wrong word. He does show a certain amount of intolerance. Whether that’s his age or not, I’m not sure, but he’s got a particular friend who

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he’s quite intolerant to . . . things that Justin does. But he hasn’t got lots of friends. Well, he won’t share. He’s very covetous of his own things. You know (his cousin) would share a toy, whereas he’d do anything but give his cousin a toy. He’s not so bad now, but when he first came home . . . but then, I suppose that’s partly our fault because we didn’t let anybody else play with his toys. He tends to, like, take over. He tried to throw his weight around a lot, especially when they were in his set. He behaves very well, but he’s quite . . . They have to be quite tolerant of him, ‘cos he pulls hair. He grabs and does stupid things. This friend he had, , she was very tolerant of him. The teachers were amazed. I mean, he nearly used to kill her, but she was very tolerant. Although not in the same league as the last of the children mentioned above, one little UK girl also had problems relating to other children. Of interest is the fact that this girl was isolated for over two years in hospital, and, as her father reported, ‘She has a very adult relationship to other children. I think she’s been so used to being treated by adults, and talked to by adults, that she employs exactly the same treatment with other children. She talks in sound bites. She asks them how they are, what their names are. A bit like a doctor does, and then goes off and does something else’. Whilst one of the UK girls was having problems with her relationships now she was in her early teens, this father of a UK boy could only see problems ahead for his son in this area at the same age, as he said, ‘Relationships is gonna be a problem when he starts realising that for a ten year old he’s alright, but that after the age of ten, kids are maturing earlier. Eleven year olds . . . it’s gonna be boyfriend/girlfriend relationships. He’s gonna be the one that’s gonna be alone at school’. This lack of friends of their own age is reflected in the drawings that the children produced. In the study of children with SCID (Vickers 1999b), when both UK and German children were asked to draw themselves doing something that they enjoyed, only 5 out of 26 (19%) drew a friend, and then in each case only one other child is involved. This boy, for example, drew himself playing football with a friend (Figure 12.1). Upon analysis, this picture does show that the boy is striving for security (big, long feet), is overly defensive and has some hostility to others (square shoulders), and has controlled anxiety (type of shading). The interpretation of this drawing is also valid for his other drawings of his family and of a house. Apart from a few of the SCID children, in spite of what the parents told me, other children do not appear to loom large in the lives of these SCID children. This can be demonstrated by examining the list of activities in which the children took part (Table 12.1). The majority of them are solitary pursuits, although they may take place within the social environment of

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Figure 12.1 with a friend).

Drawing by UK boy of himself doing something he likes (playing football

the family. Some of them, as can be seen from Table 12.1, are perfectly normal activities that all children at some time another may indulge in, whilst a few of them do appear to have a flavour of obsession of behaviour about them, for example running on the spot. Obsessional behaviour has Table 12.1 Favoured Activities of the Children in the Vickers (1999b) Research Study Children’s favourite activities 1. Solitary or family activities Mental activities Playing with toys inside Painting and drawing Walking Listening to music Running Jumping Walking with the dog Playing on a swing

Playing outside on own Running on the spot Reading Helping parents Building things (models) Watching TV on own Writing Sewing Fantasy play

2. Group activities are very much in a minority Sport Playing football Playing table tennis Scouts and guides Karate Drama Fighting 3. There are also a group of activities which could be group or solitary activities, but which rarely require cooperation Cycling Sledging Skiing Riding Gymnastics

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been mentioned in previous chapters in relation to the fussiness of the children, which in turn may be linked to the degree of fussiness concerning infectious precautions and obsessional cleaning behaviours taken by their parents, both in the hospital and when at home again. Table 12.1 was drawn up from conversations with the parents and the children themselves. Thus, it can be seen from Table 12.1 that most of the children do appear to gravitate towards activities which do not necessitate cooperation and/or teamwork. The question to be asked at this point is what the cause of this alienation is.

Causes of Alienation There are many causes of these feelings of alienation and they all stem from the disease and the stay in hospital, particularly if it is a long stay in hospital and some degree of isolation is practised at a very early age. However, it is impossible to isolate just one factor and say that that is the reason, because of the multitude of potential factors and the fact that they interact so closely with one another. Factors that could lead to alienation are given in Box 12.1. Box 12.1

Causes of Alienation

Separation Physical isolation Linguistic/vocabulary isolation ‘Being special’ Problems with bonding and attachment Guilt and blame Stresses involved in the disease and treatment Lack of support Chronic ill health Lack of opportunities for play with others Jealousy

As can be seen, many of the causes are underpinned by separation as well as the disease and treatment of the disease.

Separation Separation can be a major factor of this alienation, particularly for the children with SCID and their families, because the separation can be so acute, long-lasting and at a very early age (sometimes from birth itself). The separation may occur long before the parents and other family members

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have actually got to know the child, and so the child can remain a stranger for many months, if not years, following birth. Other factors may exacerbate the effects of separation, and these may include previous history between, for example the parents, or the parents and the extended family. If there are problems between the parents or within the family before the birth of a child with SCID and the subsequent hospitalisation, separation and treatment, then usually any such separation as occurs in this situation is only going to magnify the problems, and this has been found in parents who have separated because of the stresses involved, usually only for a period around the time of greatest stress caused by the illness, but sometimes permanently. Another factor that can exacerbate the problems of separation for the parents and the family is the behaviour of the child. Several of the parents in the SCID study discussed how their children were manipulative and played one parent off against the other, or how their behaviour at times is so poor that it caused problems within the parents and the family, particularly once a child has returned home after treatment and there is no third party in the form of nurses and other health professionals who could act as a buffer between the child’s behaviour and the parents’ emotions. This latter point is also linked to the withdrawal of the very strong parental support systems that they would have experienced within the hospital, with the support coming from the health professionals. Other factors that can exacerbate the problems that occur with such separations are the different anxieties, needs and expectations that the parents (and family) will have regarding their child, the treatment, the prognosis, as well as the stresses involved in all these situations. Sometimes parents have unrealistic expectations that once they get home everything will be fine. Unfortunately, the reality is that their child will still need some degree of isolation as well as medical/nursing care that they themselves may have to deliver, and the parents and family may never get back to the relationships that they had before the birth of their child with SCID. These factors may all combine together to magnify and exacerbate the effects of separation that were discussed in earlier chapters in some detail.

Isolation Previous discussion in this book has been about the effects of physical isolation on the child, the parents, the family and the relationships between these entities. However, there are other types of isolation that can occur which is not confined to the physical barriers between the child and the parents, or the child and the family. For example, there is the isolation that occurs through distance. One of the problems that parents who have a child with SCID encounter is that in each country there are so few specialist centres that can deal with them. For example, in the UK there are just two centres, whilst in Germany there is just the one. This means that for a child with SCID to be treated, that child and the parents will have to be uprooted

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from their family home, from all their friends and relations, from all that they know and are comfortable with (and even sometimes from others of their children) and have to go to a place often several hundred miles from their home where everything is strange and they know nobody and often feel that they know nothing. It is certainly not a ‘comfortable’ experience for them. This isolation through distance can be made even worse if, for economic or social/family reasons, one of the parents has to remain at home whilst the other one goes with the child to the specialist centre. This situation, as has been mentioned before, can last for many months, and even for more than a year, and often very quickly, the distance between the parents, or the parents and family can become just as strong a physical barrier as if walls were separating them. It must be pointed out that this situation is not unique to families who have a child with SCID; it can happen with children who require neonatal intensive care, or who have cancer, or indeed children who have any illnesses that require specialist treatment. Another form of isolation that can occur in these situations is psychological isolation. Psychological isolation occurs when, as a result of the separation, one parent can have no conception of what the other one is going through during this period. For example, a common scenario is the mother who is staying with the child who feels that, because of her close proximity with her child, then she is experiencing all the highs and lows of the extreme lability of her child’s condition and treatment. Whilst the father, who for whatever reason – work requirements or care of other children in the family – is at home and therefore does not have to go through what the mother is going through. The father at home, however, may feel that what he is doing is of lesser worth than the mother, that he is excluded from everything to do with his child and has no part to play in supporting or caring for his child, and that he is, as one father in the SCID study explained, ‘out of the loop’ in terms of knowing just how his child is. In addition, the father has to cope with all this without the support systems that are present in hospitals and which are able to give great support and comfort to the mother. This lack of understanding of what the other parent is going through, or a lack of understanding from the family as to what the parents are going through, and vice versa, can drive a wedge through the relationships of the people concerned, and so cause them to become alienated from each other because of a complete lack of understanding of the psychological traumas that each of them are going through. The third type of isolation that can be found in these situations is that of linguistic or vocabulary isolation. This occurs as a result of the parent or parents who are present with the child in hospital picking up a lot of the medical and scientific vocabulary (otherwise known as ‘jargon’) that is used by the health professionals in the hospital about their child’s condition, illness and treatment. Parents who are with their child in hospital can become very confident about using this jargon and knowing just what it means. However, parents (and/or families) who remain at home do not get an opportunity to become familiar with this jargon, and this can lead

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to great difficulties once a child returns home afterwards with a parent(s) who were with them during the months in hospital. The parent or family who remained at home will have no idea what is being said and will feel alienated from the whole experience because they do not have the vocabulary to discuss it, or even to make sense of it, whilst a parent with a knowledge of the jargon will feel that the other parent or the family are not making an effort to understand. All these various types of isolation combine to drive a wedge between those who were at the hospital and those who were not, and so can make everyone feel alone, unappreciated and excluded from what is probably the most important and emotional experience of their lives.

Being Special One of the many words that were constantly repeated during the interviews with the parents, and sometimes siblings, of the children with SCID, was that these children were special because of what they had been through, and particularly that they had survived the experience (Vickers 1999b). Occasionally, the parent who had stayed with their child throughout the period of the treatment was also considered to be ‘special’, again because of what they had been through. Whilst most of the siblings appeared to be happy with the idea of their sibling who had survived treatment for SCID being special, with some of them it obviously rankled, and whilst they were in many ways protective of their sibling, some of them did feel that within the family structure they were not considered as important as their sibling who had been sick and had survived against all the odds (as some of them were told ad nauseam, or so they perceived). Consequently, there was some degree of alienation between siblings, particularly if the sibling was old enough to appreciate their own worth. Admittedly, there was only one family that was interviewed for the SCID study where this became a real issue, but in families who underwent similar experiences to other diseases, such as cancer, this can become a real problem is evidenced ¨ by the article by Blumke (1994) discussed in a previous chapter.

Bonding and Attachment This is one of the most important factors leading to alienation. As Butterworth and Harris (1994) discussed, the notion of ‘attachment’ when it is related to separation from caregivers may not be conducive to the formation of trusting relationships. This separation from the caregiver could consist of either one separation of long duration, or it could occur as a series of repeated separations, each of which could be of short duration. According to Harris and Butterworth (2002), in line with Bowlby’s (1969) theory, when infants feel attached to somebody important in their lives – such as their primary caregivers, they feel safe and secure in that particular relationship.

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Whereas an attachment that is not secure, can lead to feelings of dependency and the fear of rejection in the infants. Bowlby (1969) argues that the biological basis of attachment in infants is self-interest leading to selfpreservation, and that it is a way of ensuring physical closeness to the main caregiver – which in itself aids the infant in its goal of self-preservation. An interesting study by Ainsworth and Bell (1970) found that 66% of infants could be classified as secure in that separation from their mothers, whilst distressing at that time, leaves no long-lasting effects. A further 20% of the infants in the study were classified as being insecure, but not externally anxious, and that upon separation from their mothers there were very few overt signs of distress and when the mother did return she was often ignored. However, there was a third small group (12%) who were highly distressed by separation from their mothers but were not easily pacified when reunited with their mothers. Although initially seeking contact with their mothers, they then showed resistance and anger to them. Finally, there was a small group of infants (2%) who were categorised as insecure and disorganised, and who showed confused behaviours when reunited with their mothers. Ainsworth and Bell (1970) came to the conclusion that it was not the separation per se that caused the differences in infants, but that it was down to the style of parenting. So, for example, the largest group of infants (66%) who were considered to be secure were said to have a carer who was available, sensitive and supportive, whilst the smallest group of infants (2%) who were insecure and disorganised, were said to have a carer who was inconsistent and who may also abuse the infant. The other two groups (insecure and also not displaying anxiety – 20%, and insecure as well as being anxious and resistant – 12%) were said to have carers who were rejecting and inconsistent respectively. Like Ainsworth and Bell (1970), van IJzendoorn and Bakermans-Kranenburg (2004) also feel that maternal sensitivity has a causal role to play in the formation and development of infant and mother attachments, as does Stevenson-Hinde (2005). This experiment by Ainsworth and Bell (1970) was a one-off separation of shortish duration, but it can perhaps give some indication of the problems involved in the separation of the children with SCID, and other children who are in hospital with some degree of isolation during this crucial time of infancy. Because of the physical barriers set up in these situations, parents can be perceived by the infant as rejecting them and as being inconsistent, just because there is not the closeness with a primary caregiver that is a pre-requisite for attachment in order to make the infant feel safe and secure. Another thing to consider is that the trust. Lansdown (1984) points out that from birth to 18 months, the infant is acquiring a sense the basic trust which is the foundation of all subsequent development and which enables new experiences to be easily accepted. Eiser (1990 p. 59) emphasises and expands this by stating that during infancy, ‘the main developmental task is related to the development of basic trust’. How does the infant gain this trust? Trust comes about from feeling safe and secure, which in turn, as has been mentioned above, comes about from forming a secure attachment

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to a main caregiver – usually the mother, but could be the father. With the separation that comes about due to hospitalisation in isolation, then this attachment may not develop fully, leading to a lack of this development of trust within the infant. Because of the unnatural situation that the children find themselves in, with multiple, often strange, caregivers (i.e. nurses and other health professionals), the infants may never get an opportunity to build up such close attachments (otherwise known as bonding). This could then lead to a life-long suspicion of other people, including (and maybe particularly) their parents and other family members, which can turn to feelings of alienation from other people – their family and peers.

Guilt and Blame As mentioned previously, many parents have feelings of guilt when their children become critically ill or have a very serious genetic disorder, such as SCID. The feelings of guilt sometimes spring from the fact that the parents feel that they should have done more to prevent their child from becoming sick, as in the case of a child with cancer. For some parents it is a case of ‘what could I have done to prevent this happening to my child’, or ‘why did we have to go there’? They often begin sentences with the two words ‘if only’. For parents who see their roles as protecting and nurturing their children, the fact that a child becomes desperately ill is somehow due to their faults – they have failed to protect their child. These feelings of guilt can gnaw away at the parent, and they may well turn away from their partner or from the rest of their family because they do not feel worthy of them. In the case of a child with a severe genetic disorder, such as SCID, this feeling of guilt can be magnified, and I have known parents split up as a result of this. Of particular note is the guilt that is felt by the mother who has a child with an X-linked genetic disorder, because in that case only the mother is a carrier of the faulty gene and the father has a normal gene. This is completely different to the situation of the child who has an autosomal recessive genetic disorder in which both parents are carriers, and therefore can give mutual support. The corollary of this is that if one parent takes all the guilt on themselves, it becomes easier for the other parent to assuage their guilt by assigning blame to their partner. Thus, alienation can have its roots in this guilt/blame ethos. Sometimes even the child can feel guilt – guilt that they have let down their parents, their siblings and their family. Or they may want to find somebody to blame, because they cannot accept what has happened. This searching for someone to blame, and this guilt can turn them in on themselves emotionally and psychologically, so causing them to become alienated from those who would only be too happy to help them.

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Stressors The stress that all of the parents experience in situations where their child is desperately ill, and particularly where the child may be dying, is unimaginable to those who have never been through the experience. Apart from having to cope with feelings of guilt and blame as mentioned above, parents also have to watch their child receive treatment that may make them worse (hopefully only initially). They may have to watch their child receiving painful and/or traumatic treatments such as receiving injections, having chemotherapy being mechanically ventilated, etc., and they themselves may even have to take part in such treatments by holding them. There are also the stresses of fears of long-term damage to their child – or even of death. Even if the treatment is successful, there are fears of relapse, or insufficient improvement. There are uncertainties as to the future, as well as how they are going to be able to cope with any physical care needs that will be required for their child afterwards. Again, as with guilt and blame, the stressors will make it more difficult for the parents to pick up their normal lives afterwards, and they may possibly have a tendency to shrink within themselves, often as a way of coping with the stressors. The children also have stressors that can affect their socialisation, including of course the isolation in itself. But the stressors can involve physical pain, their own fears of dying, or of never being a completely healthy child afterwards. They will also have their own fears and uncertainties as to the future. They may feel that they cannot do all the things that children do because of physical weakness (sometimes exacerbated by overprotective family members). In addition, the children may well have missed crucial social developmental phases and experiences because of their time spent in isolation in hospital, and consequently they may feel ill at ease with their peers who they see as having much more confidence in themselves and in their abilities.

Lack of Support The importance of support was highlighted in the study of children with SCID and their families (Vickers 1999b) and can be seen by the comparison between the German families and the UK families. In Germany, where there tended to be much more support from the extended family and from society, although problems similar to those in the UK do exist, they were perceived by the parents in Germany a not being a serious as they were by the parents in the UK, who because of the different societal environment that exists in the UK often tended to live some distance from their extended family. In Germany, grandparents in particular often lived very close to the family. Consequently, the German parents felt they were more supported than did the UK parents, and this lead to a feeling of having greater control over the situation.

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Good support from within themselves, the extended family and society as a whole (not forgetting professional support) can help the parents and the child to feel less isolated, both in hospital and at home. Once a child and parents realise that they are not on their own, but that there will always be people to help and support them, they can often find the strength to cope with their stressors (or as one child thought of them – their demons).

Chronic Ill Health Although much of this work is concerned with the acute and critical illness, it has to be accepted that for some children this critical illness is only a precursor to a lifetime of chronic ill health. This chronic ill health may arise as a result of the disease itself, the acute treatment, or the long-term treatment and drug therapy. Long-term ill health does have an isolating effect, because the child may be unable to do the things that children of his or her age are doing, or to partake in activities involving other children of their age. As Eiser (1990 p. 112) pointed out ‘There are indications in the literature that chronically sick children can appear socially isolated’. In addition, long-term and chronic ill health in their children can lead to feelings of isolation and alienation, as well as actual physical isolation, in the parents. They may have fears about leaving their child in case he or she gets sick, or they may just be too tired to socialise themselves.

Lack of Opportunities to Play with Others This has already been discussed in some of the sections above, but just to reiterate that children who were hospitalised and isolated at a very young age – often for a long time, may well have missed out on some of the important socialisation phases such as cooperative play, in which they can learn to work with others of their age, and to enjoy the company of others of their age.

Jealousy Again this has been mentioned above, but it is not just a case of other children being jealous of the perceived ‘specialness’ of the child who was sick, but sometimes the child who was sick may be jealous of their siblings and other children of their age, because they see them doing things that they feel that they cannot do, or they perceive them as being comfortable with themselves and with others and at ease in other’s company.

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The Extended Family and Alienation So far, this chapter has concentrated on alienation concerning the child and the parents, however, one of the major themes that came out of the study of children with SCID and their families was the effects of the extended family and social support on the coping abilities of the parents, and ultimately, the child. Differences between the social and family support that the German families and the UK families generally receive have already been discussed in relation to the coping abilities of the child (Chapter 7). However, a lack of support can seriously affect the functioning of the families. For example, a lack of support seems to predispose the parents to be more pessimistic and judgmental over their child’s present and future capabilities and functioning. As has been mentioned already, there appeared to be greater family and/or social support forthcoming for the German parents, which goes some way towards explaining the overall better functioning of the German children. This, however, is not universal. One mother in Germany felt quite unsupported by her own family, and she discussed with me why she thought this was so, ‘My parents have a . . . so, a picture which agrees with “our healthy family” . . . yes? So not like from Hitler, not so . . . not . . . they have their own ideas with regard to a German child, a German family, German grandchildren not being healthy. But now this child is sick. My parents were unhappy’. Consequently, this mother appeared to have a more pessimistic outlook than any of the other German parents I visited. This contrasted with, for example, another German mother, who had much more support, as she explained to me when I visited the family, ‘With my mother-in-law here, my husband came to Ulm every weekend, the whole time. And his sisters spent fourteen days in Ulm and fourteen days here, so they did not miss their mother. That was the correct way’. The parents of one UK boy felt that they, like the first German mother mentioned above, did not have the support they needed. However, unlike with that German family, this was not because their families did not want to support them, i.e. social distance, but was due to physical distance – their families lived a long way away. Although the parents of one UK girl had a lot of support from their families, who lived very close, they felt isolated from the community. As her mother reminisced about how other people in her village would cross over the road to avoid meeting her whilst her daughter was so seriously ill in hospital, Like when she came out of hospital, to take her for a walk was magical . . . it really was magical. And as she got ill, she was so little. It was, errm . . . everybody knew I had a baby, and even now people say to me, you know . . . ‘we knew you had a baby, but we didn’t want to say anything, just in case.’ And these were people you thought were your friends, whatever. And just

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the fact, I mean . . . they obviously felt guilty. We’d probably have done the same if we were in their shoes . . . just that feeling of being left out. As well as physical isolation, there was the isolation that comes from not being able to talk about the illness or the transplant. One UK mother explained what this is like, ‘They just say you mustn’t talk about that, , because at the end of the day, you know, you can’t play too much on her medical condition. Everyone thinks that’s all you wanna talk about, an’ you want sympathy for your child, an’ it frustrates me to a point where I actually get irate with . . . .’

Relationships with Adults Whilst there appeared to be problems with many of the SCID children in terms of their relationships with other children, this was certainly not the case where relationships with adults were concerned. In Germany, one boy’s mother told me ‘He gets along better with adults’, whilst another German boy’s mother told me that he also had no problems – ‘He has no problems with adults. He speaks to everybody, and behaves himself very well with them’, as did this German boy’s mother, ‘He is cheerful. He’s fine with them as well’. Meanwhile, in the UK, one boy was ‘very good with adults’ according to his mother, as was a second boy, whose mother replied, when asked how he got on with adults, ‘Brilliantly! He really gets on with older people’. One UK girl was in her element when she was with adults, particularly older adults, as her mother reported, Oh she loves adults, she does. She goes up an’ they’ll ask her to sing for them, an’ she’ll sit there an’ sing for them. I’ll say, ‘oh . . . . . , for God’s sake get out’ you know. I walk up the shop an’ there she is sittin’ with some old woman on the steps, singin’ her head off. Are . . . bloody hell, are they laughin’ at her, or do they really . . . but they love her . . . they love her. But whether it’s the attention. I mean, the adults give her attention, you see. But, errm . . . children . . . no. Adults, yes. Sometimes, as has been mentioned, previously, this easy-going relationship that some of the children had with adults could cause problems, as this extract from this UK girl’s mother’s interview demonstrates: She does get on very well with most people, like that. She has no problems. She’s very friendly to adults . . . actually too friendly. Now she’s like that with everybody. We have a couple of new priests come to Mass, and she has to give them a hug and a kiss, you know. ‘But I like giving people hugs and kisses’ (she says), you know, so I don’t want to change her personality. She might be an outward, giving person, you know, but at the same time I’m trying to warn her that there is dangers, you know. It’s quite hard to

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get that through to her. She can’t see that. Even if (her father) speaks to them, ‘they’re alright’, she thinks, but it might not necessarily mean that you can speak to strangers, and she wouldn’t know the difference. She can’t differentiate between two people like that. So, as I say, she gets on very well with most people. She can be just over-generous with some people, and give herself too much to strange people, which is very hard to explain to her. Some of the children were initially shy of adults, but then got on well with them, as for example this UK boy, whose father told me, ‘He doesn’t talk to strangers. He’s real shy. Someone comes, he’ll hide round the corner. Even our friends he’s known for a year or two’. Some children were said to be suspicious of strangers – ‘He is relaxed with those he knows. This is not so with strangers’ (German mother) and ‘He’ll still be wary of strangers that he didn’t know. He’d back off them a bit; wouldn’t let them lift him until he’s got to know them’ (UK mother). One UK girl who was still in her very early teens actually saw herself as an adult, which in itself could cause problems, as her mother explained, She relates a lot better to adults than to her peers. She thinks of herself on an equal footing with an adult . . . which of course is what causes her so many problems. She doesn’t seem to understand that she is thirteen. There’s no natural understanding of this, you know. There’s something in there that’s not functioning right. Even as a small child, she preferred to be with adults. She didn’t want other children to play with. She’s more comfortable. She likes to be around adults. I think they found her difficult to deal with. They are a bit flummoxed at times, but they do understand. One German girl’s mother did not find her relationships with adults a problem at that time, but she was concerned about the future as she felt she would become totally alienated from her peers – ‘This isn’t a problem (at the moment). This is a typical only child . . . only adults. She talks with adults. This will only cause difficulties for her later’. Some children did have problems with adults, however, as demonstrated by comments from these two mothers. According to the mother of a German boy, he was ‘restrained with adults’, whilst the mother of a UK boy just stated baldly that he was ‘rude to them’. Meanwhile, one of the UK boys, who had severe problems in his relations with other children, also had severe problems with adults. His mother responded to the question of his relationships with adults by saying, ‘He doesn’t trust anybody! He doesn’t even like having a different man . . . he goes by minicabs to school . . . and they’ve had to give him one driver because he doesn’t want to go with others . . . so they’ve given him one driver. Very suspicious (of adults)’. The seeds of the different relationships between the SCID children and other children, and between the SCID children and adults were undoubtedly sown at the time of their bone marrow transplant (BMT). Months in isolation at a very early age, never ever seeing another child, let alone interacting with one, for many months (in some cases for years) meant that

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crucial steps in play – cooperation, etc., were missed out, and so many of the children do not gain experience of being with other children. It is not just stimulation per se that the children need whilst in isolation, but stimulation akin to what they would experience normally. This is similar to a case I came across when I was a teacher in a school for children with severe behavioural difficulties in the 1960s. One little boy had been hidden away for the first five years of his life, so much so that most of the people living in the street had no idea that there was a child in that house. He had never come across other children and his life had been dominated only by adult females (his mother, aunt and grandmother). When he first arrived at the infant school at the age of five years, the trauma of seeing all those other children, just like him, was too much and he became very disturbed and violent, trying to stab other children in the eye with a sharpened pencil. Eventually he was excluded from school at the age of five years. He was totally unable to relate to other children. This same situation, although, fortunately, not quite so extreme, occurred with the SCID children. So many of them appeared never really to become comfortable with other children of their own age. Younger children, they could control, and so did not perceive them as being a threat. In addition, because they had been exclusively in the company of adults for many months or years, they did not have the same feelings of awe and apprehension towards adults that other children may have, just the opposite in fact, particularly as most of the adults were there to help and nurture them (if one forgets the injections and other traumatic practices in hospital, as well as the effects of the drugs they received from adults), so the children could relate to them, albeit at a superficial level. Even when they started to go to school, the SCID children still felt more comfortable in the company of adults and so tended to gravitate towards them rather than to the children in the school. As this UK mother said of her son’s relationship with adults, ‘He’s very good, communicates well . . . comfortable’. This drawing by a UK teenage boy (Figure 12.2) illustrates how SCID children feel more comfortable with adults. A member of the Scouts, this teenager chose to draw a picture of a scene from his activities with the scouts during a sailing session as his picture of himself doing something that he liked. In spite of all the other boys in the Scout troop, he actually drew himself with his scoutmaster, i.e. the person he felt most comfortable with. All the above does not mean to say that there were no problems between adults and the SCID children. Just as some of the children attempted to manipulate their parents, so some of them attempted to do the same to other adults outside of the family, as one UK girl’s teacher testified, ‘she is extremely manipulative against adults, plays one off against the other. Always tests a new person’. In particular, one UK boy appeared to embody all the problems of the other SCID children on his own. His behaviour was summarised by health professionals as having a wide range of behavioural problems including aggressive behaviour towards his younger sibling and other children, refusing to go to bed and frequently disturbing his parents during the night,

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Figure 12.2 Drawing by UK teenager of something he likes doing. Previously published in Vickers P.S. (2006) Communicating with children and families. In: Peate I. and Whiting L. (eds) Caring for Children and Families, Chichester: John Wiley and Sons, pp. 25–48 (p. 41).

a marked fear of going to hospital, general difficulty in interacting with peers and a fear of being alone.

Summary and Conclusions The problems caused by early hospitalisation and by isolation have been well explored in the past (Bowlby 1951, Douglas 1975, Loranger 1992, Rutter 1980, Wells et al. 1994, Zerbe et al. 1994). These studies demonstrated that early hospitalisation and isolation were associated with increased behaviour disturbance, particularly in adolescence. This was also found to be the case in the Vickers (1999b) study concerning children treated for SCID. However, the notion of alienation has not been explored in previous studies. The problem of alienation in children with chronic ill health has been mentioned in a paper by Sartain et al. (2000), but was incidental to the main theme of the paper and so was not explored at all. A suggestion has been made that this state of alienation is very similar to, if not the same as, reactive attachment of infancy or early childhood (American Psychiatric Association 1994). Indeed, there are similarities between the two conditions, such as disturbed and developmentally inappropriate social relationships; they both begin before the age of five years and are

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associated with grossly pathological care. In the case of children with SCID, this would link to the long-term isolation and treatment. However, there are several differences. For example, in the reactive disorder of infancy or early childhood, two types have been identified. These are inhibited type and disinhibited type. In the inhibited subtype, there is a persistent failure to initiate and respond to social interactions in a way appropriate to the person’s development. With the disinhibited subtype, there is indiscriminate sociability or a lack of selectivity in the choice of people to whom they will attach themselves. Some of the children treated for SCID do exhibit some of the symptoms associated with one or other of the subtypes, but there are many who straddle both. Some children are often very specific in regards to with whom they do not associate, namely members of their peer group, whilst being able to socially interact with people who are younger or older. In addition, there is the alienation expressed by the parents that must also be taken into account. This parental alienation is not linked to their early childhood, but rather to their experiences with their children in hospital. The long-term effects of this alienation, on the children and their families, particularly in terms of lasting relationships with peers and other members of their families have yet to be ascertained, but some of the adolescent survivors continue to show signs of difficulties in forming relationships but at least one survivor has in the past 18 months got married and given birth to her own daughter (who does not have SCID). Without help, these problems will persist for a long time, as they already have for many years. The challenge is not only to save the lives of these children, but also to ensure that each and everyone of them, and their families, are helped to achieve optimum potential and optimum mental, as well as physical, health. It must be stressed, once more, that not all the children in the Vickers (1999b) research study displayed signs of alienation, but sufficient did so to warrant concern. On the positive side, children transplanted for SCID since this research was undertaken are now showing signs of not having such major problems as many of the SCID children in this early cohort exhibited. This is undoubtedly due to a greater awareness of the increased risk of such problems for these children, which has been highlighted by this research. Much is being done in terms of better psychosocial awareness and care throughout their stay in hospital, as well as different, less toxic ways of treating these children, such as matched unrelated donors for BMTs, gene therapy and cord blood transplantation. This chapter has looked at the causes and the effects of alienation in children, who have experienced early hospitalisation and long-term isolation, and their parents. It can be seen that the causes can be many, but the three underpinning factors appear to be isolation and hospitalisation at an early age, and severe ill health/trauma. In terms of the SCID study, this was a dramatically clear finding that does appear to be of concern.

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Trauma to the infant, almost without exception, involves a relative degree of physical or emotional separation from the mother. Sometimes the original stressor is augmented by physical pain, illness or previous trauma, but fear of separation from the main caregiver – regardless how brief – is the primary factor. (McKenzie & Wright 1996 p. 76)

Introduction Traumatic stress has, in recent years, emerged as a useful framework for understanding aspects of individual and family responses to the experience of children’s ill health, serious injury and the subsequent medical treatment (Kassam-Adams 2006). This has sparked greater attention by paediatric psychologists to the use of a post-traumatic stress framework for child and family responses to children who have cancer, burns, injuries and other serious illnesses, or who require a bone marrow or solid organ transplant. What has emerged from all this interest in a post-traumatic stress framework related to children’s medical conditions and treatment is that traumatic stress reactions are extremely common and that they should be considered as normal following such a traumatic event (Kassam-Adams 2006). Indeed, it is thought by many that some traumatic stress reactions occur as a result of the person experiencing the trauma adapting to it rather than not adapting to it (Kassam-Adams 2006). This chapter will consider children who experience severe trauma as a result of their ill health. Pot-Mees (1989) suggested that the children as well as parents could also suffer from Post-traumatic Stress Disorder (PTSD). According to the Diagnostic and Statistical Manual of Medical Disorders (American Psychiatric Association 1994) specific features associated with PTSD, where children are concerned, include: Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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r r r r

a diminished interests in significant activities a marked change in orientation towards the future nightmares various physical symptoms, such as intrusive images, flashbacks and nightmares about the event

Pot-Mees (1989) concluded that, six months post-bone marrow transplant, the children in her study exhibited behavioural symptoms which corresponded to those associated with PTSD as seen in adults. Given that there are some similarities in their psychosocial functioning between the SCID (severe combined immunodeficiency) children and those bone marrow transplant children in Pot-Mees’s (1989) study, it is possible to hypothesise that the SCID children may also develop PTSD. There is an extensive body of knowledge and opinion demonstrating that infants who have traumatic experiences may later have varied psychological disorders, including PTSD. However, excluding all the other traumas inherent in the course and treatment of SCID, McKenzie and Wright (1996) point out that trauma from infancy possesses one common denominator – this is a threat of physical or emotional separation from the mother as experienced by the infant. They also go on to say that people who have serious mental disorders often have early traumatic experiences and that there is a positive correlation between drugs and alcohol dependency and with prolonged stress during infancy. Sigmund Freud (1894) was one of the first to attribute the causes of neuroses to trauma during childhood, whilst Bettelheim (1969) and Mahler (1979) proposed that autism and childhood psychoses were linked to traumatic experiences in the first 18 months of life. Another Freud, Anna Freud (1963) found that a single uninterrupted mother figure, instead of the continual rotation of caregivers (as occurs in hospitals for example), could play a great part in reducing these childhood problems. This fear of separation from parents/mother figure is, according to McKenzie and Wright (1996) probably the greatest fear in infancy and early childhood and Kaplan and Sadock (1994) make the point that children who are dying from cancer fear the separation of death more than the pain of death. A little earlier, MacLean (1985) described early separation as the most painful experience to be found in all the mammalian species and this can be seen in the cry response which, according to MacLean is the infant’s desperate attempt to bring the mother back to his or her side. This loss of an important relationship has been recognised as one of the most profound of all life’s stressors (Meisner 1977, Williams & Siegal 1989) and early separation of the infant from the mother in particular, has been noted by many, especially by people such as Bowlby (1969, 1973, 1984), Cichetti (1984) and MacLean (1985) who noted that there were profound effects from this early separation, particularly in relation to the stress experienced by the infant (see Chapter 4, the effects of early hospitalisation). McKenzie and Wright (1996 p. 6) put forward the theory that the mechanism of a shift from a past-perceived trauma to the present situation, where there may be a perceived trauma, is a survival mechanism in which

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flashbacks occur that are linked to the same ‘mind/brain/reality/feeling/ behaviour/chemistry and physiology’ that existed at the time to take place the first trauma, and which enabled survival of the first time round. As the initial trauma is a highly emotionally charged situation leading to fears of abandonment and death, as well as feelings of pain, sadness, overwhelming anxiety and despair, all these feelings can occur at a later date if they are re-awakened (e.g. as occurred during the interviews of parents for the research on children with SCID). These flashbacks can also be caused by being in a hospital environment again, and they can be precipitated by the doctors and nurses, as well as everyday objects such as scissors and bandages.

Signs of PTSD in Children Whilst the standard criteria for PTSD are clearly spelt out (American Psychiatric Association 1994), how can it be recognised in the child? PTSD is a disorder that can seriously disable the child leading to severe interference in the child’s development and ability to adjust to changing circumstances for many years (Yule 1999). Girls are generally more susceptible to PTSD than boys, whilst children who are less able both academically and cognitively are more badly affected than are the more able children (Yule 1999). This may be because the less able children have more difficulty in trying to make sense of the traumatic event than do the more able children. PTSD can manifest itself in children as:

r a continual need for comfort and reassurance r clinging to parents, other family members and/or favourite inanimate objects

r intrusive images of the traumatic event that can recur at any time and that can be very vivid to the child, and which the child can replay over and over again r in particular, those intrusive images can be accompanied by repetitive memories involving any of the senses, such as sound, smell, touch and motion r flashbacks in which the child can sometimes believe that the event is happening all over again r dreams and nightmares that may make the child afraid to go to sleep r tiredness and irritability which can lead to anger r a lack of concentration r development of fears, especially those concerning the stimuli that were present when the traumatic event occurred r depression – sometimes even becoming clinical depression (particularly in adolescents, some of whom may have suicidal thoughts) r a loss of faith in the future (the ‘life is fragile’ concept) r the priorities of the child may change (Janoff-Bulman 1985) (Yule 1999).

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Prevention and Treatment of PTSD in Children According to Yule (1999), the severity of the trauma is directly proportional to the stress reaction that results from the trauma, but subjective factors can mediate the effect and can either make the effects on the child who has experienced the trauma more or less serious. It is necessary to take both the objective severity of the trauma and the mediating subjective factors into account when formulating any intervention to assist the child. There is little evidence that drug treatment has anything other than a peripheral role to play in PTSD (Yule 1999). Rather, the focus of treatment is on behavioural therapy that can help the survivor to make sense of what happened as well as to help them to master their feelings of anxiety and helplessness (feelings that are very much to the fore in PTSD). Following any traumatic event, children sometimes experience tremendous pressure to talk about what happened. When they are put under this pressure they often find it difficult to talk about the experience; however, at the same time children can have a need to talk repeatedly about the event (but in their own time and in their own way). It has been suggested that they often do not want to talk to parents or friends for ‘fear of upsetting them’; this can lead to them keeping their feelings to themselves, which can then exacerbate any future PTSD. Therefore, there is the need for someone else to find the time to listen, but not to belittle or disregard their fears. To the child, these fears are very real – frighteningly so in many cases. Group support techniques have now been adapted to use with children following traumas (Dyregrov 1991). The aims of these groups should include the sharing of feelings, the boosting of the children’s sense of coping, having a mastery of the situation and their own feelings and sharing ways of solving common problems (Yule 1999). Often children, particular younger ones, need to ‘play out’ the traumatic event repetitively, as well as draw it time and time again (Galante & Foa 1986, Pynoos & Eth 1986). This playacting and drawing of the event often helps the child to recall both the event and the emotions they experienced, and are often good ways of starting to talk with the child about the experience in a non-threatening way, since it fosters a feeling of initiation of the talk, which in turn leads to feelings of self-control.

Flashbacks What is a flashback? Flashbacks are partial or complete returns to the initial trauma (McKenzie & Wright 1996). The flashback that can cause an individual to return to that initial traumatic event is fundamental to the development of PTSD. However, McKenzie and Wright (1996) point out that whilst a flashback to a specific moment during infancy (when there is no conscious

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recollection of the trauma) is not as widely reported and is less obvious than a return to a traumatic event that can be consciously remembered by the individual, the time of infancy is a time when the individual is in his or her most delicate and vulnerable state of all. The infant is totally dependent upon the mother figure (who could also possibly be the father). Research studies have shown that the younger the individual is at the time the traumatic event occurs, the more susceptible that individual is to suffer from PTSD, and the more intractable the PTSD becomes (Eth & Pynoos 1985, Hyman et al. 1988, Janet 1919, Kilpatrick et al. 1985, McKenzie & Wright 1996, van der Kolk 1988). All these studies demonstrate that there is a very clear pattern in which the younger someone is, when trauma and stress is experienced, the more susceptible that person is to PTSD. As a result, infants are much more likely to be at risk of PTSD than older children or adults (McKenzie & Wright 1996). Spitz (1975, 1983) discusses that the earlier and more prolonged the separation from the mother experienced by the infant, the more stressful is the trauma. According to McKenzie and Wright (1996 p. 41), the trauma of early separation (or indeed any situation that causes the infant to fear that separation may occur) may possibly be the ‘maximal stress known to mankind’. Continuing this theme of the importance of the mother to the infant (as opposed to any other person with whom the infant may live), McKenzie and Wright (1996) put forward the suggestion that whilst an infant prefers his/her mother to meet his/her needs, this may not always be possible. Whilst they concede that the mother is very difficult to replace, they also point out that mother substitutes are often not satisfactory. According to Spitz (1945), it is only the mother who is really able to stimulate her infant – far more than anyone else, whilst Dorman (1984) and Pearce (1985) argue that early stimulation is crucial for mental growth and development. Once again is important to consider the situation of the child with SCID (or any child who is hospitalised with any serious/critical illness); this infant may be able to have access to his or her mother, but at the same time there are barriers put between the mother and the infant, including the need for specialist care that can only given by professionals, as well as physical restrictions that may occur in isolation nursing, for example, or when the infant is mechanically ventilated.

Early Traumas What is meant by early traumas? Various early traumas have been identified, some of which may not have appeared traumatic to the adult, but to the infant can be very significant, and may include:

r maternal emotional upset – identified by the infant r birth of a sibling – leading to feelings of separation r moving home – separation from all that was familiar and safe

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r changing parental (and particularly maternal) priorities, including r r

death or serious illness within the family, the loss of the parent’s job, the loss of the family home emotional and physical separation from the mother (or the mother figure) – as may occur during hospitalisation pain – as may occur when hospital treatment is necessary, may be the catalyst for hospital treatment (leading to further trauma – that of parental emotional upset and separation)

An important point made by McKenzie and Wright (1996) is that whilst early separation from the mother can cause PTSD, this can be exacerbated if the infant is situated in a new environment with caregivers who are strangers. This describes exactly the situation when an infant is hospitalised. This early trauma can be cumulative, and the various factors may lead, in certain circumstances and in certain individuals, to PTSD. These various factors include:

r r r r

previous experiences of trauma (Burgess & Holstrom 1979) early emotional problems (Helzer et al. 1987, Hough et al. 1990) repeated traumas – cumulative trauma (Pynoos & Nader 1988) anxiety (Freud 1926)

So, it can be accepted that early trauma occurs and can have a serious effect on future mental and psychological development.

Children with Cancer and PTSD Most of the work with children who have illnesses and PTSD has been done with those who have cancer. Butler et al. (1996) point out that childhood cancer does meet the traumatic event diagnostic criteria of PTSD because it typically involves a definite threat to life, as well as many invasive and painful medical procedures. Lee (2006) discusses that children with cancer (and their parents) experience uncertainty and psychological distress. Concern is focused upon whether or not the child will survive, as well as the probability of relapse and other later effects of the cancer and treatment, for example secondary cancers, cognitive impairment, physical impairment and psychological distress. Lee was very interested in this idea of uncertainty and examined the relationship between uncertainty and post-traumatic stress symptoms. Lee (2006) points out that an understanding of this relationship will help in the development of intervention strategies that could reduce the incidence and severity of psychological distress. Foa et al. (1992) concur with this, and state that they consider that the traumas most likely to result in PTSD are characterised by two factors, namely controllability and unpredictability. Meanwhile, Mishel in 1988 and 1990 reported that adults manage uncertainty and chronic illness by developing a new view of uncertainty and the situation which is supported by psychosocial structures, such as

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education, social support and credible authority roles. Without this, Mishel (1988, 1990) determines that post-traumatic distress symptoms can develop. Lee (2006) takes this idea further by stating that the uncertainty that goes with experience of serious childhood illness can lead to the development of symptoms similar to those of PTSD. To examine this theory, Lee (2006) carried out a study to understand the levels of uncertainty and posttraumatic stress symptoms and also to examine their relationship in young adult survivors of childhood cancer. This study showed that there was evidence that uncertainty was positively correlated with post-traumatic stress syndrome is in this group, and this supported the Uncertainty in Illness Theory (Mishel 1988, 1990) which states that people with chronic uncertainty in illness may adapt post-traumatic stress symptoms as a way to manage their uncertainty when they lacked sufficient resources to cope with the challenges of survival (Mishel 1988, 1990). As Lee (2006) discusses, when the support mechanisms for a gradual evolution (growth and development) in the ability for appraisal of the experience/situation and the understanding of the inherent uncertainty involved is lacking, or when illness and the normal demands of living become too great, then post-traumatic stress symptoms can become the norm so that survivors are able to manage their emotional distress and avoid an awareness of the uncertainty with which they are confronted. Therefore to improve and reduce post-traumatic stress symptoms in these situations there need to be sufficient support resources, including education as well as credible authority roles within the therapy situation. Lee (2006) further makes the point that psychological distress, including post-traumatic stress syndrome, can be minimised through reducing uncertainty by:

r providing information about the occurrence and severity of any late effects of cancer and cancer treatment

r clarifying any ambiguity about the illness r decreasing the complexity of the health care system r increasing the ability to exert some control over the unpredictability of life in terms of its quality in the future and levels of functioning Added to this is the hypothesis by Butler et al. (1996 p. 503) that ‘adequate emotional support over the course of an extended period of trauma will act as an inoculation against eventual emergence of PTSD’. Hobbie et al. (2000) looked at symptoms of post-traumatic stress in young adult survivors of childhood cancer. In their study of 78 young adults who had survived childhood cancer, they found that 20% of them met the criteria for a diagnosis of PTSD associated with anxiety and other psychological distress symptoms. They found that with survivors of childhood cancer, it was their perceptions of the treatment and its effects that were more highly associated with post-traumatic stress, as opposed to actual objective medical data. They identified that the participants who were diagnosed with PTSD reported a higher perceived life threat at that present time, more intense treatment histories, and having clinically significant levels of psychological distress than did those participants who

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were not diagnosed with PTSD. This was significantly different to earlier studies (in the 1980s and early 1990s) that appeared to demonstrate that child and adolescent survivors of cancer were generally well adjusted, although there was a small subset who had difficulties in adjustment (Kazak 1994). Hobbie et al. (2000) suggested that the incidence of PTSD in survivors of childhood cancer would make sense when one looked at the possible causes of PTSD in collaboration with the hospitalisation of children with cancer. For example, once diagnosed with cancer, the following factors occur:

r r r r

painful, invasive procedures frequent hospitalisation separation from family and friends fear of the unknown

In addition, after treatment, the following may be reminders of the event – may signal possible future medical problems:

r r r r

infertility problems with growth cardiac dysfunction cognitive changes

Interestingly, in studies by Kazak et al. (1997) and Barakat et al. (1997), it was the parents of these children who had survived cancer who, when compared to a control group, experienced a greater incidence of PTSD, rather than the children themselves. However there were some symptoms of post-traumatic stress reported in the children who had survived cancer, and these children did have certain common factors present:

r They were characteristically anxious. Children with anxiety had inr

creased levels of post-traumatic stress (but not full-blown PTSD) than did those children who were not anxious. Perceptions of treatment intensity and life threat were predictive of post-traumatic stress, as opposed to the more objective ratings made by health care providers.

Anyone familiar with phenomenological philosophy will not find this second point strange because the underlining tenet of phenomenology is that what we personally experience is the truth as far as we are concerned. This echoes the mantra which all nurses are taught concerning pain, namely that pain is what the person experiencing the pain says it is (McCaffrey 1972). Therefore, the perceptions of treatment intensity and life threat are more real to those experiencing them than the more objective account given by health professionals. One point made by Hobbie et al. (2000) is that the high levels of PTSD found in their group of young adults who have survived childhood cancer may be so high because they are at an age when they are undergoing developmental changes. Young adulthood is a time of increased vulnerability to stress when people start to face different life challenges such as

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negotiating interpersonal relationships (including intimacy and forming families), as well as educational and employment decisions and achievements, which may require them to focus, perhaps for the first time, on the medical, social, cognitive and psychological effects of the cancer and the treatment that they have had. Lee (2006) agrees with Hobbie et al. (2000) that these problems become greatly exacerbated as there is a move from childhood to adulthood, and there is a necessity to assume much higher levels of social independence, integration through friendships, marriage, higher education and employment or career development. To reinforce this, Butler et al. (1996), from their research, suggest that PTSD symptoms coexist with socialisation difficulties in children. Hobbie et al. (2000) conclude that these adult survivors of childhood cancer may therefore be at risk not only of increased distress, but also of finding difficulty in facing the challenges outlined above. In addition, they have to face all these challenges whilst trying to integrate with their present responsibilities and needs their unique childhood experiences and the realisation, and perhaps understanding for the first time, of the potential late effects of the treatment that had saved their lives. As Erickson and Steiner (2001) explain, being diagnosed with a lifethreatening illness is included as a qualifying traumatic event for PTSD (American Psychiatric Association 1994). In 1996, Stuber et al. demonstrated that there was a substantial subset of paediatric cancer survivors who experienced significant PTSD-related symptoms up to 12 years after treatment had been successfully completed. They worked out that the prevalence of severe PTSD symptoms in this group of childhood cancer survivors was 12.5%, and claim that although most survivors do not meet the diagnostic criteria for full-blown PTSD, and although the majority of childhood cancer survivors appear to be functioning well, they do have relatively high levels of intrusion (re-experiencing of the traumatic event), avoidance (persistent avoidance of circumstances that may be reminders of their experiences), and general anxiety symptoms, and clearly this is a cause of concern. Erickson and Steiner (2001) conclude that five years posttreatment, childhood cancer survivors still do have relatively high levels of current trauma-associated symptoms, including symptoms of PTSD, avoidance and personality problems. A 1996 study by van der Kolk et al., although not limited to cancer, showed that exposure to extreme stress could result in a range of traumarelated psychological disturbances, including symptoms of PTSD. They found in this study that whilst the symptoms apply to most adults with a history of trauma, it was actually adults who had experienced childhood trauma who had the highest level of the symptoms. Work by Canning et al. (1992) and Phipps and Srivastava (1997) showed that, whilst children with cancer appear to have relatively low levels of reported distress such as depression and anxiety, they do have high levels of ‘restraint’ (repression). Erickson and Steiner (2001)also discuss this and explain that because there appears to be a difficulty in recognising distress in these children, despite both physiological and behavioural evidence that contradicts this, then the children have a ‘repressive adaptive style’. In other words, the children

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are taking a defensive attitude and are coping by repressing their feelings, although this repressive adaptive style is not a conscious reaction. They pursue this argument by stating that this reaction occurs outside of the ‘awareness’ of the child and that this repression involves a process of selfdeception rather than just a deception meant for others. Consequently, Phipps and Srivastava (1997) found that the children with cancer reported significantly lower levels of depression and anxiety (distress) but higher levels of defensiveness when compared with a control group of healthy children. Repressive adaptation has been associated with a variety of negative health outcomes, including hypertension, asthma, cancer (Jamner & Leigh 1988, Schwartz 1990, Steiner et al. 1987) and psychosomatic disorders in adolescents (Steiner & Harris 1994). Erickson and Steiner (2001) postulate that the children with high defensiveness scores – the ‘repressors’ – are characterised by a high desire to appear acceptable to others, along with an inability to recognise and express subjective distress. These children who have survived cancer manage their lives by using high levels of restraint, repressive defensiveness and denial.

Other Conditions and PTSD This chapter has concentrated on children with cancer and PTSD because, as was stated earlier, most of the research appears to have been conducted in this area. However, hopefully it can now be seen that any early hospitalisation for any severe illness can lead to the development of PTSD in children. For example, Shemesh et al. (2000) studied post-traumatic stress in paediatric liver transplant recipients which they linked with non-compliance and non-adherence to medical treatment in the patients. They found in their sample that clinically significant non-compliance/ adherence was associated with the full spectrum of PTSD symptoms, and hypothesised that it may be that traumatised patients were avoiding taking their medications following a successful transplant because they served as painful reminders of the disease. Shemesh et al. (2006) therefore concluded that screening for, and management of, these PTSD symptoms may improve compliance. It was also suggested by Shemesh et al. (2006) that this conclusion may be applicable to other patient populations. Looking at another group of patients who are hospitalised at an early age, McKenzie and Wright (1996) discussed the traumas that premature babies may have to deal with – particularly in terms of separation anxieties. These babies may well be separated from their mothers initially as a mother may not be fit enough after the birth to be with her baby, and possibly also from their fathers. Allied to this are all the traumas associated with their treatment, such as drug therapy (injections), physiotherapy and mechanical ventilation. Even the very environment can be traumatic to an infant who has just spent the last nine months in a quiet, calm, warm, and darkened environment and who is now suddenly thrust into an environment

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where noise and light can often appear to them to be at hyperintensive levels. Anyone who has spent time in neonatal intensive care units will be well aware of just how noisy and invasive the environment can be. For the neonate, the noise and light will be so much more intrusive. It could be suggested that the neonate would be far too young to have any memory of the time spent in an intensive care unit. The same argument could be made in relation to the children with SCID who survive successful bone marrow transplantation as infants. Indeed, there is evidence within the behaviour of the SCID children, who took part in the study, to suggest the presence of PTSD, and therefore it is important to be conscious of the age at which the SCID children experience their major traumas – the disease, the isolation and bone marrow transplant. A comparison of the Pot-Mees (1989) study and the study with children who had survived treatment for SCID reinforces this argument. In Pot-Mees’s (1989) study of children who received bone marrow transplant for all diseases other than SCID, the mean age for the children to receive their bone marrow transplant was 8.3 years for acquired disorders and 4.9 years for genetic disorders. The children with SCID were all babies and infants – all were certainly younger than 12 month of age. Thus, one could argue that the children with SCID would have no memory of their traumas and treatment and therefore would not experience PTSD. This, however, may not be so. Bradford (1997 p. 85), discussing work by Nelson (1986), whilst not discussing babies as such, makes the point that ‘research evidence highlights the fact that from an early age children develop “scripts” or stories in their minds about everyday events. The importance of this is that children who have been exposed, for example, to frequent hospital procedures may well develop quite a clear appreciation of the sequence of events involved in treatments. It is wise not to assume, therefore, that a child’s development level is the only important factor that influences knowledge acquisition; both social context and experience appear to have a role as well’. One must not dismiss the ability of the baby to be aware of their environment. From as early an age as two months, babies are becoming more aware and interested in their environment (Weller 1990). Butterworth and Harris (1994 p. 110) state that ‘it could be that infants’ perception and understanding of the permanence of objects is well in advance of their ability to put into effect sequences of action in such tasks’. They are stating that an infant’s ability to perceive and understand something occurs far sooner than their ability to utilise that perception and understanding. Butterworth and Harris (1994 p. 110) then argue that ‘When the emotional attachment of the infant to the parent is considered, it becomes apparent that the baby may already have accumulated a great deal of perceptual experience both of the physical and social world’. Lansdown (1984 p. 115) also accepts that babies are much more able, in all sorts of ways, than was once thought, when he writes ‘Not only do they have a functioning sensory system, albeit not fully functioning, they appear to possess some pre-programmed powers of discrimination

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and preference. There is even some evidence of a degree of co-ordination between the senses in the neonate’ (Lansdown’s emphasis). It must also be taken into account that, although the children with SCID may be very young when they first encounter their traumas, they may be much older before they are free of trauma, as exemplified by the girl who was over two and a half years old before she even left her isolation cubicle in hospital. In addition, many children were isolated at home until they were even older than that, sometimes until they were five years old and preparing to go to school. Thus, there is a possibility of the babies and infants with SCID having some perception of the traumas they are undergoing. What they cannot do, however, is conceptualise their experiences at this stage. Conceptualisation comes much later in their development. This perception without conceptualisation may be one of the factors underpinning the behavioural problems of the children with SCID as opposed to Pot-Mees’s (1989) bone marrow transplant children who, being older when experiencing their own particular traumas will have some internal conceptualisation to help them try and make sense of their experiences. A more advanced age and development at the time of experiencing trauma may not lead to a child having increased psychological and behavioural problems, far from it. It is more likely that the younger, less-developed child will have greater problems because of a lack of conceptualisation and past experiences.

Conclusion This chapter has briefly considered PTSD and children who have undergone hospitalisation, often as infants, for serious and possibly lifethreatening illnesses. Hospitalisation itself carries the risk of future psychosocial problems, including PTSD or post-traumatic stress symptoms if full-blown PTSD is not present. On top of that, serious and possibly life-threatening treatments for them can add to the traumas. McKenzie and Wright (1996) talk about combination traumas, where there is pain and discomfort, allied to separation from the family as well as separation from familiar surroundings. This can lead to multiple traumas in which first of all the pain or the sickness intensifies the need for the infant’s mother or mother figure. With the pain and sickness comes fear which in turn makes a child more vulnerable to any separation. In addition, the child is separated from his or her home environment, from familiar surroundings. Then there is the anxiety provoked by strangers (doctors, nurses and other health professionals) being with the child, and sometimes causing the child pain. All this greatly increases the amount of trauma that the infant is experiencing. Is it any wonder that some of the children do go on to develop posttraumatic stress symptoms or even PTSD in later years? Even if PTSD or post-traumatic stress symptoms do not develop, there must surely be some psychosocial problems allied to all this pain, fear and anxiety. However, if hospital staff and family work together, then there is every possibility that the severity of the psychosocial problems will be decreased.

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This chapter began with the quote, and will end with one. A traumatic stress framework can provide medical and nursing staff with a useful perspective and understanding of the impact of traumatic events on themselves and an appreciation to the experiences of patients, families, and colleagues. (Kassam-Adams 2006 p. 337)

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Post-Traumatic Stress Disorder in Families

Introduction One of the abiding memories of many of my visits to families in the course of the research into children with SCID (severe combined immunodeficiency) was of interviewing parents who, for the first time for many years, felt that they were being allowed to talk freely about their experiences. For so many of the parents, particularly the UK parents, this situation has resonance with people suffering from a post-traumatic stress disorder (PTSD). In addition, the themes which came out of the actual interviews had very striking parallels with PTSD. For the majority of the parents, their child’s diagnosis, illness, bone marrow transplant (BMT) and supportive treatment were assessed as elements of a traumatic event. Without appropriate support and counselling, however, they had not had an opportunity to work through their psychological problems. It was obvious that there was much re-experiencing of the event, along with intrusive thoughts and a variety of emotional and cognitive responses.

What Is PTSD? PTSD is defined in the 10th edition of the International Classification of Diseases – ICD-10 (World Health Organisation 1992) as a disorder that people may develop in response to one or more traumatic events. These traumatic events can include deliberate acts of violence on the person, severe accidents, disaster or military action. In addition, they can include people who have experienced threat to their own life (or to others) whilst undergoing medical care (NICE 2004). PTSD was first recognised by the American Psychiatric Association as a discrete syndrome in 1980 (APA 1980, later updated in 2000). 256

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The disorder can occur at any age from infancy to old age. However, a much stricter diagnosis for PTSD is defined in the 4th edition of the Diagnostic and Statistical Manual of Mental Disorders – DSM-IV (American Psychiatric Association 1994). In addition, DSM-IV distinguishes between acute stress disorder (ASD) which lasts for up to one month, and PTSD which lasts for one month or longer (NICE 2004). The diagnosis of PTSD is restricted to people who have experienced exceptionally threatening and distressing events, and according to DSM-IV, a traumatic stressor (that could lead to PTSD) usually involves a perceived threat to one’s own life or to the life of another person, particularly one close to the person experiencing the traumatic event – often, but not always, a family member. If not a threat to life, traumatic stressors also include threats to physical integrity, as well as feelings of intense fear, helplessness and/or horror. People with PTSD also have intense feelings of guilt, shame, anger and/or emotional numbing (NICE 2004). As NICE (2004) points out, whether or not people develop PTSD depends not on objective facts of the event, but much more on their subjective perception of that event as being traumatic. The most characteristic symptoms of PTSD are:

r The re-experiencing of symptoms – including flashbacks in which the r r r

person affected acts or feels as if the event were recurring, nightmares, as well as repetitive distressing intrusive images. Avoidance of reminders of the trauma – including people, situations or circumstances resembling the event or associated with it. Hyperarousal – including hypervigilance for threats, intense and exaggerated ‘startle responses’, irritability, sleep problems and concentration difficulties. Emotional numbing – including the inability to have any feelings, feeling detached from other people, giving up activities that previously were very important and amnesia (NICE 2004).

Symptoms of PTSD usually commence in the first month after the traumatic event, although according to McNally (1993), a small minority (15%) may not start to develop the symptoms for months or even years after the event. However, a large number of people with symptoms of PTSD may spontaneously recover from them without any treatment, many of them within the first year (Kessler et al. 1995), but at least a third of them will continue to have the symptoms for three years or longer (NICE 2004). Of these people who continue to have symptoms of PTSD, many may develop further, secondary disorders, including:

r Substance use disorders – including alcohol, drugs, caffeine or nicotine – as a means to cope with their symptoms.

r Depression – including an increased risk of suicide. r Other anxiety disorder – including panic disorder (NICE 2004). According to Kessler et al. (1995), the risk of developing PTSD after a traumatic event is 8.1% for men and 20.4% for women (NICE 2004).

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Parents and PTSD As early as 1977, Gardner et al. reported that parents of children who are undergoing BMTs encounter many psychological problems, particularly, but not exclusively, guilt, misgivings and anxiety. In 1979, Patenaude et al. pointed out that bone marrow transplantation is a crisis situation that severely taxes the coping ability of any family. These psychosocial problems that parents encounter following their child’s BMT and that they have to work through include:

r concerns about leaving the ‘protective’ hospital environment once their child has had a successful BMT

r ‘magical thinking’ about creating a safe home environment for them r anxieties as to whether or not they will be able to keep their child well once they are living at home again

r expectations that are quite possibly unrealistic that the transition from hospital to a home would be a smooth process (Atkins & Patenaude 1987, Freund & Siegel 1986, Heiney et al. 1994). In addition, Freund and Siegel (1986) warned that parents would have to cope with negative consequences to their marital relationship as well as with the negative effects that the whole experience will have on their personalities. As has been seen in previous chapters, anxieties and all the psychological problems mentioned above are very prevalent in parents of children who are undergoing all sorts of traumatic medical procedures. So what distinguishes PTSD? The American Psychiatric Association (APA 1980 p. 247) defined PTSD as a reaction to a ‘psychologically traumatic event that is outside the range of usual human experience’. In this seminal work, PTSD was classified as being an anxiety disorder. This anxiety disorder is made up of three main groups of symptoms which were perceived as being characteristic of the syndrome, namely:

r distressing and recurring recollections of the traumatic event r avoidance of stimuli associated with the trauma r a range of signs of increased physiological arousal (APA 1980, Yule 1999). According to Green et al. (1985), the APA definition above includes reactions to any traumatic, overwhelming life experience, or indeed to any traumatic stressor. These traumatic stressors that can lead to PTSD can include a serious threat or injury to oneself or to people close, such as family members, the sudden destruction of home or community, or even witnessing mutilation or violent death (Green et al. 1985). Those who actually have PTSD are those people who experience intense psychological distress and recurring unbidden intrusive thoughts about the event – the

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idea of ‘flashbacks’ that can be emotionally debilitating and can lead to many physical, psychological and psychosocial symptoms. Some of these symptoms, accepted by many experts in the field, include:

r the constant re-experiencing of the event r avoidance tactics – not wanting to discuss the event, or even to conr r r

sciously think about the event (this obviously excludes the unbidden thoughts) detachment from the event and from people involved in/with the event (alienation) physiological arousal, particularly the physiological responses to stress in addition, there are a variety of emotional and cognitive responses to the experience or event, including depression, guilt, grief and anger (Heiney et al. 1994, Spitzer & Williams 1988).

The APA (1980), Mejo (1990) and Wilson et al. (1985) have isolated several factors that can contribute to the development of PTSD in anyone who has encountered traumatic experiences as outlined above. Of particular importance is the role that an inadequate social support network can play in exacerbating the development and the symptoms of PTSD, along with the role played by the survivor in the experience. Two other factors contributing to the development of PTSD include the environment in which the event occurred and the origin of the stressor, i.e. whether it was man-made or natural. The experiences encountered by the parents of children with SCID parallels those discussed above by Heiney et al. (1994) who described the elements of a child’s BMT experience which can lead to parents experiencing PTSD. With all families there was a great and urgent need to talk about their experiences with their child. Almost without exception both German and UK families were desperate to know how their child compared to other SCID children, or wanted to know how other SCID children whom they had come across in hospital were doing. So great was this need for information, for reassurance often many years after the transplant that not only could the actual interviews themselves with the parents be emotional and draining, but also the time before the interviews – a period when the interviewer and the family became comfortable with one another. This experience was paralleled a few years ago when the immunodeficiency unit at one hospital arranged for the parents of past SCID children to spend a weekend together, with no professionals in attendance. The feedback from the parents was first of all how successful it was, secondly that the parents talked non-stop to one another – often talking to people with whom they had nothing in common apart from the fact that their child had had a BMT for SCID, and thirdly, that there was a need for more opportunities to meet, to reassure each other, to explore, to compare their lives now and to gain strength for the future. Heiney et al. (1994 p. 834) have described the characteristics of a child’s BMT experience that may precipitate a PTSD in parents of children with a

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haematological disorder, such as leukaemia. They state that ‘When viewed from the PTSD (post-traumatic stress disorder) framework, parental reactions to a child’s BMT offer striking parallels that include assessment of the event as traumatic, re-experiencing the event, intrusive thoughts, and a variety of emotional and cognitive responses’. They conclude that using the framework of a PTSD may aid health professionals to devise strategies to help the families cope with the traumatic experience. According to Wilson et al. (1985), cited in Heiney et al. (1994), researchers have isolated seven factors that increase the likelihood that an individual will develop a PTSD. These are:

r r r r r r r

degree of life threat duration of the trauma degree of bereavement or loss of significant others displacement from the home community potential for recurrence of the trauma role in the trauma exposure to death and destruction

The question to ask is ‘do the parents of children with SCID have experiences which fit into these seven categories?’

r Degree of life threat: The parents of the children with SCID were told,

r

r

quite simply, that their child was going to die unless a BMT took place, but that the success rate was only about 60% for a mismatched transplant (which was what most would require), therefore not all children would survive the transplant, even if they survived long enough to have a transplant. Because their child was so ill, the threat of death was always present throughout the treatment. Duration of the trauma: Children with SCID are very sick – they are under sentence of death. This death sentence would be in place for many months, if not years. Some parents never lose that fear, even many years post-transplant, when all the signs indicate that their child is doing very well. Degree of bereavement or loss of significant others: Many parents had already lost children as a result of them having SCID, and most of them come across other children who died during their own child’s treatment and transplant. By the very nature of this disease and treatment, the parents tend to become very close and feel it deeply when another child dies. One mother in the UK explained to the interviewer that when she was in the hospital with her daughter, another of the babies in the unit died. She had become very close to that baby’s mother, but she felt that the staff ignored her and that she was left for a long time wondering what had happened to the baby and that she was given what were for her unsatisfactory answers to her requests for information. She pointed out that, despite her knowledge of the code of confidentiality, the parents of the children in the unit had known each other for a long time and they found it difficult to distance their feelings and thoughts from what was happening to people who had become almost

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r

r

r

261

‘family’. Another mother, this time in Germany, thought about the guilt that she felt because her child survived, but all the other babies, who were on the unit at the same time as her child, died. Displacement from home community: As there are only three centres in the UK and Germany that were able to offer this treatment, then most of the families had to travel great distances for their child’s treatment. In Germany, the families in the study came from all parts of Germany, Austria, Switzerland and even one family from Spain, whilst in the UK the families came from all the four home countries, from Ireland, and one family came from Malta. Often only one parent was able to accompany their child, and so separations were unavoidable. In addition, in the UK, most of the families in the study who accompanied their child, had to share bunk rooms with other mothers, thus they were unable to establish temporary ‘nests’. For many of the German families, at least, there were the ‘Elternhausen’ (family homes) which allowed for a degree of privacy and intimacy, which may be one of the reasons for the differences apparently existing between the psychosocial functioning of children and families from the two countries. One father, in particular, found the situation in the UK very difficult because he needed to have ‘time away’ in order to have a period of normality, although he didn’t want to leave his daughter and her mother in the hospital whilst he went home. According to Heiney et al. (1994 p. 845), this situation of displacement ‘parallels the displacement experienced by victims of natural disaster’. Potential for recurrence: Many parents in the research project stated that, even many years later, they had a fear of recurrence/relapse, either in the child who had been successfully treated for SCID or, due to the genetic nature of the disease, in any children they may have in the future. There were also concerns centred on future grandchildren. Role of parents in the trauma: Because of the highly technical/medical nature of the care of the child during the BMT period, many parents felt unable to, or afraid to, care for their child, even though basic care, as for any infant, is an essential part of the overall care of these children during their treatment. Many felt that their own role as parents was displaced by nurses and other staff. For some of the parents, this period came after a few months of looking after their baby in a normal way as any parent, and during this period of transplantation and hospitalisation they lost confidence in their parenting skills. Conversely, other parents, whose child had been diagnosed at birth and isolated in hospital immediately, felt that they had never had the opportunity to gain these skills. As Heiney et al. (1994 p. 845) report, concerning parents of children with leukaemia, ‘Parents began to feel inadequate in carrying out care routines at which they once felt proficient. Additionally, they were unable to be as supportive to their child because their own high levels of stress exacerbated feelings of helplessness. The parents, because of their reaction to the stressors of BMT, also must be considered victims of the trauma’. Of interest, in Heiney et al. (1994) it is reported that the stress levels were so high in the parents that they felt unable to

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r

be as supportive to their child as they felt they should have been. This exacerbated their own feelings of helplessness. These days an important part of the pre-transplant assessment involved a consideration of how the procedure will affect the psychosocial functioning of the parents of transplant recipients (Young et al. 2003). The concern is that at a time when the parents might experience tremendous emotional stress, the parents’ own emotional health, and their ability to provide the necessary care for their child might very well be compromised (Young et al. 2003). Exposure to death and destruction: Although not seeing their child deliberately and maliciously tortured and killed, nevertheless the parents did see their child undergo much invasive, sometimes painful treatment and saw them suffering from all the side effects. In addition, during their sojourn on the transplant unit, parents may well have been witness to the conditioning/treatment regimes hastening the death of other infants on the unit, which was a source of great stress to them as they tried to assess their own child’s chance of survival. Heiney et al. (1994) reported that parents described the horror of seeing their child vomit tissue and blood. They also felt particularly unprepared for all the severe side effects that their children encountered.

So the answer to the question as to whether or not the parents of children with SCID go through the experiences which fit the seven factors leading to an increased likelihood of developing a PTSD has to be in the affirmative. At the debriefing session following the interview with one mother, the interviewer reported back on several points. One of which was that . . . a lot of things made me feel we didn’t take parents’ feelings into consideration as much as we thought, and either didn’t have the time or the expertise to counsel them through the experiences. Perhaps parents need more or different ‘after-care’. I appreciate the difference in the amount of ‘blocking’ and/or acceptance of information by parents, and I wonder also how much parental coping depends upon the age and experience of the parents. Another point she made was that she felt that this mother ‘. . . found the interview, and in particular talking before and after the official taped interview, a good way of releasing her feelings about things that happened during their stay in hospital. Sadly, it seemed as though after nearly eight years, she was only just able to talk about them’. This interviewer’s experience was not unique either in the UK or in Germany. By the end of all the interviews there was obviously some concern about the mental state of some of the parents following the reopening of painful experiences. Having said that, without fail, during the meetings and interviews with the parents, they confided just how glad they were that they had at last had the opportunity to talk about things that they had kept hidden for many years. Turning now to parents of children undergoing BMTs for diseases other than SCID, a report by Manne et al. (2004) found that approximately

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20% of 111 mothers of children who had undergone haematopoietic stem cell transplantation (HSCT) had clinically significant distress reactions, namely:

r r r r

generalised anxiety disorder major depressive disorder panic disorder post-traumatic stress disorder

However, as they report, if subthreshold PTSD was included in the total, almost 33% met the criterion for distress. HSCT as a treatment for blood disorders and cancers is a demanding procedure involving intensive chemotherapy or radiation (or both) prior to an infusion of bone marrow or peripheral stem cells (either from a donor from the patient). It also involves lengthy stays in hospital and lengthy periods of recovery. Although it can lead to a cure of these diseases, it is still associated with significant levels of further ill health, and even death. According to Manne et al. (2004 p. 1700) ‘the significant medical risks along with the difficult experience of witnessing one’s child undergoing an aversive medical procedure such as HSCT can result in both acute and persistent psychological distress reactions among parents’. Manne et al. (2004) also looked at the quality of life that this distress brought about, and they found that mothers with at least one of the above distress reactions said that they had a significantly lower quality of life. So, it is obvious that PTSD can have a negative and important impact on general quality of life. Candilis et al. (1999) have also reported that people with psychiatric disorders experience a disruption in their quality of life. Manne et al. (2004) found that mothers diagnosed with one of the four above distress reactions at 18 months post-HSCT were younger and had had higher anxiety and depressive symptoms at the time of the HSCT. Thus, they were able to conclude that there is a subset of mothers of children who survived the HSCT who may require psychological intervention. One of the concerns expressed by Manne et al. (2004) is that there had been relatively few studies which have led to the long-term distress reactions among parents of children who have survived HSCT, and most of this has been qualitative or clinical descriptions, for example the work published by Atkins and Patenaude (1987) and Freund and Siegel (1986), although they do acknowledge that Sormanti et al. (1994) did conduct one of the earliest long-term follow-up studies. Sormanti et al. (1994) interviewed parents of children who had undergone bone marrow transplantation from one to ten years prior to the study. Their aim was to identify sources of stress which could lead to PTSD and other similar disorders. What they found was that the parents reported as their sources of stress concerns about the possibility of the disease recurring, insurance (it was an American study), finances, as well as their own physical and mental health. More recently, Manne et al. (2002) have concurred with previous studies that parents of children who have survived HSCT may experience post-traumatic stress (PTS) reactions.

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Fathers and PTSD One interesting, and possibly contentious, comment was made by Manne et al. (2004), who stated that they were focusing their research on the distress responses of mothers because previous studies of parents of paediatric cancer patients had shown that the mothers report more distress than do other family members (Ostroff et al. 1997). This could be contentious, because the perceived idea that mothers report more distress is often down to the fact that mothers are given more opportunity to report distress. Many years’ experience of looking after children with severe lifethreatening diseases as well as this research with parents of children with SCID has shown that the fathers can experience as much distress as the mothers. However, it is the mothers who are often the parent who is with the child during the transplant. Therefore, they are supported and often allowed to give voice to their distress. Fathers, on the other hand, often have to stay at home, continuing to work and trying to keep the rest of the family together. They feel that they have, to use a very British term, to maintain a ‘stiff upper lip’. As mentioned in previous chapters, fathers often find tremendous difficulty in coming to terms with their child’s illness and the fact that they may die. Because they do not have the support that the mothers often have, some fathers take to alcohol as a means of coping with their distress, whilst others may deny that their child is so desperately ill and may die. When fathers are given the opportunity to express how they feel in what they perceive to be an emotionally safe environment (often with sympathetic male nurses, doctors, social workers etc.) giving them permission, time and understanding to really express their feelings, then their feelings can be as strong, jagged, all-encompassing and devastating as are those of the mothers. This is something that all who care for sick children need to accept, appreciate and allow for. After all, as Boscarino pointed out in 1995, one of the most well-documented resources to aid recovery from, and to prevent, the development of, distress reactions to traumatic life experiences is social support (Manne et al. 2004). As has been reported by Nelson et al. (1997), Rodrigue et al. (1997) and Sormanti et al. (1994), parental stress during and after the HSCT has also been shown to be associated with the presence or lack of social support, as well as the quality of it.

Mothers and PTSD However, to return to the report by Ostroff et al. (1997), they state that mothers report more psychological distress than do other members of the family. One reason for this, is that mothers are typically the primary caregivers for their children during treatment therefore they may carry more parenting stress and witness a greater number of upsetting medical events than do the fathers (Streisand et al. 2000, Uzark et al. 1992). The flipside is that often fears are worse when what is happening is left to the imagination, which is often the position that fathers are in who are not at

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the hospital during this time. To be able to confront one’s fears, to have them explain to you, and to be given the support and comfort, not only of medical and nursing personnel, but also of other parents going through the same emotional experiences, is to at least allow one to try to put them into perspective, but more importantly, to be able to express them in a safe and nurturing environment. As Manne et al. (2004), with reference to work by Lang (1985) as well as Tait and Silver (1992), note, from a theoretical perspective, the way that people processed traumatic experiences can be linked to the perceptions and reactions that occur during the ‘exposure’ to the traumatic or stressful event, and the psychological vulnerability factors, as well as the resources that the person brings to the experience. These, according to Manne et al. (2004) are key predictors of later psychological reactions. Work by Koopman et al. (1994) has demonstrated that emotional reactions during the event are predictive of later PTSD. This is backed up by a report published by Maercker et al. (2000). Manne et al. (2004) concur and point out that these severe emotional reactions at the time of the stressful event are risk factors for PTSD in mothers because they are likely to indicate greater appraisals of their fear as well as a greater perception of the threat to the life of their child. In addition, more severe emotional reactions at the time of the event (in this case the HSCT) may also indicate a greater vulnerability to psychiatric problems. So, according to this theory, the stressful event can cause severe emotional reactions, but if these reactions are too severe, then it is supposed that the mothers are more likely to have psychiatric problems, even though any reaction to a stressful event can cause severe emotional reactions. Manne et al. (2004) do point out that Tedstone et al. (1998) have reported that one of the potential psychological vulnerability factors which may predict a mother’s long-term distress reaction is their psychiatric history before the event. Any psychiatric history that is present before the stressful event is likely to be a key predictor of long-term emotional distress. In addition, they point out that there is a large body of literature which considers that exposure to previous traumatic events are also a critical vulnerable factor in the development of stress reactions, depression and panic disorders (see also Faravelli 1985, McFarlane & Yehuda 1996, Resnick et al. 1993, Zlotnick & Bruce 2003). So, traumatic experiences before the HSCT may sensitise the mother by adding to her perception that life is both difficult and traumatic. Other risk factors for PTSD developing in mothers following HSCT are demographic ones, and according to Manne et al. (2004), the following demographic characteristics may contribute to the development of longterm distress reactions in parents:

r r r r r r

gender of the child age of the child age of the parent maternal ethnicity education parental income

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In their study published in 2004, Manne et al. found that there were two factors that were predictive of later psychiatric disorders among the mothers in their sample. These were maternal age, and anxiety and depression at the time of the HSCT. Regarding maternal age, they found that the younger mothers were the ones who were at greatest risk of later psychiatric disorders. They hypothesised that this was possibly because the younger mothers will have had less experience of coping with difficult life events, and therefore they may be more likely to have severe persistent distress reactions. Turning to the presence of anxiety and depression at the time that the HSCT was carried out, Manne et al. (2004 p. 1706) felt that this was constant with suggestions that previous psychiatric history is associated with later psychiatric disorders, as suggested by Bromet et al. (1998) and Wittchen et al. (1994). However, Young et al. (2003) in their research study on parents of children undergoing transplantation of solid organs found that PTSD severity was not related to:

r r r r r

age or gender of the child age of the parent educational level of the parent racial or ethnic background of the parent type of insurance carried by the family (financial burden?)

So obviously, there is a dichotomy present between the findings of these two research studies. Young et al. (2003) found that the parental report of the child’s overall physical health did significantly contribute to self-reported levels of PTSD, with poorer child health related to more severe parental PTSD symptoms. It is not necessarily the actual health of the child undergoing a transplant, but rather the parent’s perceptions of the health of the child that can affect the severity of the parental PTSD symptoms. The strongest predictors of PTSD, found by Young et al. (2003), were:

r the parental perceived impact on family and social functioning r the parental perceived benefits of the experience r parental attitudes towards health care in general These are consistent with previous research studies undertaken on the social stressors present in the post-transplant phase (Gold et al. 1986, Meltzer & Rodrigue 2001, Rodrigue et al. 1997), and suggest that these social stressors that are secondary to the actual transplantation procedure may lead to PTSD symptoms being present for a long time after the transplant. Indeed, Young et al. (2003) discussed that parents who reported feeling that family and social functioning (e.g. travelling, participating in social events, visiting with friends and relatives) was negatively affected by the transplant experience, also tended to have more severe self-reported PTSD symptoms. The first two factors above that can influence levels of PTSD (parental perceptions of impact on family and social functioning as well as on the benefits and experience) may be self-evident, but the third (parental

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attitudes towards health care in general) need some explanation. Young et al. (2003) hypothesised that this may be linked to the possibility that the parental perceptions of health care service providers and facilities may be seen by some parents as negative and essentially hostile to the parent. This could increase the perceived threat to the parent and child, and may therefore increase the possibility of more, and more severe, PTSD symptoms present in the parent. Kazak et al. (1998) looking at PTS symptoms in parents of survivors of childhood cancers found that symptoms of PTSD were significantly associated with:

r r r r

trait anxiety perceptions of whether the child could still die perceived intensity of the child cancer treatment social support

They also reported that their findings applied to both mothers and fathers and were consistent. For Kazak et al. (1998), the importance of trait anxiety as a predictor for PTSD is consistent with other research (Kupst et al. 1995). Its importance lies in the fact that parental anxiety is an important predictor of adjustment to traumatic situations, both in the long-term and short-term. Acknowledging this can lead to attention being paid towards assessing an understanding of parental anxiety in the situations. As Kazak et al. (1998) point out, within a paediatric environment, often the primary focus in terms of confronting anxiety is focused upon reducing the child’s anxiety and on providing parents with information so that in collaboration with medical and nursing staff they can play a role in their child’s care. Kazak et al. (1998) believe that it is also important to address parental anxiety, not only during the treatment, but also, because of the long-term impact that this anxiety can have on parental functioning, specific interventions should take place after treatment is concluded. Finally, looking at the factors present during treatment with lifethreatening illnesses, such as HSCT, Manne et al. (2004) state that mothers of children who have to have more risky transplants and which have a more difficult post-transplant and recovery period, are themselves more likely to develop long-term psychological reactions such as PTSD. This is because a more risky and difficult procedure/treatment regime would mean that there is greater exposure to potentially severe emotional and physical experiences, as well as a greater potential threat to life in the child. These, according to March (1993), are factors that can increase vulnerability to long-term psychological problems. In contrast to the research undertaken by Manne et al. (2004), Young et al. (2003 p. 725) looking at PTSD and solid organ transplants in children found that although the caregivers of children undergoing transplantation did not report raised levels of depression or anxiety, they did report raised levels of symptoms of PTSD. In their study, Young et al. (2003) found that an analysis of their findings revealed that PTSD symptoms were more strongly associated with parental reports of:

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r the perceived health (or otherwise) of the child r impact on the family of the transplantee r attitudes towards medical caregivers, such as doctors and nurses They did conclude that PTSD appeared to be relatively common in parents of children receiving solid organ transplants, and that this may be largely the result of how parents perceive and interpret the whole experience surrounding the transplant. Young et al. (2003) do, however, point out that unlike with the children with SCID or some of the blood and oncological disorders, with the arrival of much safer and more effective immunosuppressive drugs, the survival rates for children requiring solid organ transplants have dramatically improved. In fact, they have improved so much that now solid organ transplantation is seen not so much as a ‘cure’ for a particular end-stage disease, but rather it is perceived as a transition from an often chronic life-threatening disease to a second chronic condition, namely living with, and caring for, a transplanted organ (LiBiondo-Wood et al. 1992). The rise in the number of children surviving solid organ transplantation has meant that more attention has been given to the functioning of the child after the transplant, in particular there is now much attention being given to looking at psychosocial functioning, quality of life and adjustment to living with the transplant (Almond et al. 1991, DeBolt et al. 1995, Uzark et al. 1992, Wray et al. 1992, Zamberlan 1992). Reports by Gold et al. (1986), Meltzer and Rodriquez (2001) and Rodrigue et al. (1997), although descriptive in nature, do suggest that parents undergo a series of stressors throughout the transplant process from the preoperative stage up to several years post-transplant (Young et al. 2003). These stressors include:

r the wait for a suitable organ donor – leading to apprehension helplessness and worry (Gold et al. 1986, Meltzer & Rodrigue 2001)

r the uncertainty and fear of rejection/infection post-transplant r the increased responsibilities of caring for their child without the constant support network from the hospital (Young et al. 2003) According to Young et al. (2003 p. 725) citing Gold et al. (1986) ‘many parents describe this phase as an “emotional roller coaster” marked by feelings of anxiety, hypervigilance, and helplessness’. In addition, several studies have demonstrated that many parents score highly on self-reported measures of psychological and emotional distress (Douglas et al. 1998, Rodrigue et al. 1996, Tarbell & Kosmach 1998). Various studies have looked at factors with a possible relationship to parental distress in these situations. Tarbell and Kosmach (1998) found that family conflict significantly predicted a mental health status of parents during this period, whilst Sormanti et al. (1994) found that the distress that the parents experienced with the whole process of their child having a solid organ transplant was frequently centred upon financial problems as well as social relationship problems.

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Other studies have suggested that these coping resources are essential for ameliorating the stressors that parents face in caring for a child who has had an organ transplant (Meltzer & Rodrigue 2001, Rodrigue et al. 1997). The two major coping resources that have been identified are family cohesion and social support, echoing the findings by Mejo (1990) and Wilson et al. (1985). Gold et al., as early as 1986, noted that the post-transplantation period often necessitated an adjustment of the family dynamics in dealing with increased caregiving responsibilities and increasing financial burdens. Once home after the transplantation, the parents may feel happiness, triumph and relief that their child has survived the transplant. However, these positive feelings can soon be overcome by negative feelings as, without the constant support of the transplant unit personnel, the burden of looking after the physical, medical and psychosocial needs of their child can quickly lead to exhaustion, uncertainty, anxiety and fear, as they take on all the roles that previously they had shared with the personnel in the transplant unit. The father, who may not have had much involvement to child in hospital because of continuing to work and possibly looking after other children in the family, now has to become involved. It is about this time also that, with their child once more at home, the enormity of what they have all been through as a family can cause an emotional and physical ‘rebound’ following on from the enormous ‘high’ that they will have experienced at actually having their child at home again. This can exacerbate the anxiety and fear, as well as lead to depression. This is often the time when they do need strong social support, but they may find that extended social support networks are not as available now that there is the superficial appearance of normality in terms of the family being together at home and the child having had a successful transplant. As Young et al. (2003 p. 726) point out ‘without the acute crisis of hospitalisation and surgery, friends and relatives may not be as spontaneously and readily supportive to parents’. In addition, if there has been a long separation with the mother and child in hospital and the father and the rest of the children at home, then the family – and the parents in particular – will have to build up and develop their relationships once more. This may be difficult, particularly if there are other children in the family, because jealousy can appear. This was certainly the case with one of the families where the child had a BMT for SCID. When the child and the mother returned home, the brothers and sisters of that child were very jealous of her because, as they perceived it, she had taken their mother away from them for many months. Similarly, the girl who had had the BMT jealously guarded her relationship with her mother. On this point, what is interesting is that a study by Rodrigue et al. (1997) found that generally, parental stress tended to be higher in the immediate post-transplant period, and that by the sixth month post-transplant (as opposed to one month after the transplant), parents reported:

r greater family conflict r less social support r less family interaction

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r less optimistic perceptions r greater financial burdens r increased caregiver burdens. These levels of parental stress were found to be higher than they had been before the transplant. According to Young et al. (2003), it is the whole total transplant experience that is a traumatic event for the parents as opposed to any one particular acute traumatic event during the procedure. The research study by Young et al. (2003) suggested that whilst, on the whole, parents of children undergoing solid organ transplants did not appear to report clinically significant levels of depression or anxiety (although some did report such high levels of depression and/or anxiety), they did report more severe symptoms of PTSD than is to be found in the general population. Such severe symptoms of PTSD are also found in parents of childhood cancer survivors. Young et al. (2003) found that more than 50% of the parents in their study reported moderate to severe symptoms of PTSD, and 27.1% reported symptoms that met the DSM-IV diagnostic criteria for PTSD. These results are also similar to the prevalence of PTSD as has been reported by parents of children with cancer (Kazak et al. 1998). Interestingly, the more severe symptoms that were reported by the 46 parents in Young et al.’s (2003) study considered as having PTSD were those of ‘avoidance’, such as feeling distant or cut off from people around. This obviously has a resonance with the notion of ‘alienation’ discussed earlier in the book. Manne et al. (1998) examined PTSD in mothers of children who have survived cancer. They looked at the co-morbidity (meaning that both disorders are present together) of anxiety and depressive disorders as well as the prevalence of subclinical PTSD. Their results from the study showed that just over 6% of the sample diagnosed was found to have current PTSD, while 20% had subclinical PTSD. In addition, 25% of the sample had a comorbid diagnosis of an anxiety and depressive disorder. Many researchers (Butler et al. 1996, Heiney et al. 1994, Kazak et al. 1997, Stuber et al. 1994a, 1996) have found that between 10% (Kazak et al. 1997) and 40% (Stuber et al. 1996) of parents of children who survived cancer reported significant levels of, the stress symptoms. However, according to Manne et al. (1998), these studies did not formally assess the presence of a PTSD diagnosis using the DSM-IV criteria. Pelcovitz et al. (1996) did formally assess the occurrence of PTSD in parents of children who had survived cancer, and although the sample was a very small one of 24 mothers, the results indicated that 25% of these mothers were diagnosed with current PTSD. Further to this, Manne et al. found that 25% of the mothers in their sample with PTSD had a co-morbid diagnosis of an anxiety and depressive disorder. They felt that not only should symptoms of PTSD be looked for in mothers of childhood cancer survivors, but also symptoms of co-morbid diagnoses of anxiety and depressive disorders. Santacroce (2002) found in her study that the level of anxiety in parents of children who have been recently diagnosed with cancer was comparable to that found in hospitalised patients with

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anxiety disorders. She also found that the levels of symptoms of posttraumatic stress (PTS) found in her sample of parents of children who had recently been diagnosed with cancer were higher than those reported in parents of childhood cancer survivors. Her final finding was that there was a significant relationship between anxiety and symptoms of PTS, and therefore all who work with children with cancer should offer parents interventions to relieve these high levels of anxiety and symptoms of PTS at the stage of diagnosis. Of interest is the point made by Manne et al. (1998) that in studies of other people who have undergone different traumatic events, partial PTSD is more common than is PTSD (Carlier & Gersons 1995, Weiss et al. 1992). Still looking at children with life-threatening disorders, Kazak et al. (1998) looked at PTSD in parents of survivors of childhood cancers. Their findings support the findings of other researchers that have been discussed above. They found that for both mothers and fathers, the strongest predictor of PTS symptoms was anxiety, along with:

r perceived threat to life r perceived treatment intensity r social support They also found that there is evidence that substantial distress continues after the successful completion of the treatment, including surgery, transplantation, chemotherapy and radiotherapy. Kazak et al. (1998) wanted to answer the question as to what constitutes traumatic stressors in illness. It is easier to determine this with a single traumatic event such as an accident, but it is much harder with an illness, because it can last over a long time, and there can be many traumatic events during it. With reference to childhood cancer, from the very beginning the diagnosis of childhood cancers is potentially traumatic for the parents. They understand that their child will die without treatment, but they may die with treatment. This mirrors the situation with parents of children diagnosed with SCID, except that children with SCID would die without treatment. Similarly, the treatment r´egimes themselves are potentially traumatic. They consist of repeated painful invasive procedures, hospitalisations, life-threatening complications, disruptions of family life, feelings of helplessness, recurrent reminders of the child’s vulnerability to relapse and/or death. Again, these are the situations that face the parents of children with SCID. Even after the treatment has finished and the child has survived, potentially traumatic events can still occur, including dysfunction of vital organs, relapse and secondary tumours. Once again, this parallels the experiences of parents with a child with SCID – although for many of them, these are perceived concerns, rather than actual problems. Of the various factors that could lead to PTSD in parents of children who survived cancer and its treatment, one of the strongest factors is that, even after successful treatment, there is a risk that the child could still die. Although these children are largely considered to be ‘cured’ of their cancer,

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and specialists in the field know that the risk of dying from cancer related to long-term treatment effects is very low at this stage, many mothers still felt that death was a possibility more than five years after the end of treatment (Kazak et al. 1998). Another of the factors related to an increased risk of PTSD is the perceived intensity of the treatment. This is linked to the importance of somewhat subjective views of illness and treatment. As Kazak et al. (1998) point out in their research paper, the objective variables concerning medical data were not significantly associated, for either mother or father, with the symptoms of PTSD. Research by Compas et al. (1996) supports this phenomenon of subjective perceptions of illness having more of an effect on psychosocial functioning than were the actual objective aspects of ill health. In their study, Compas et al. (1996) found that subjective appraisals of how stressful and how serious an illness might be were stronger and more important predictors of psychological outcomes in close family members than were the actual objective disease characteristics. The third factor, namely social support (in particular the size and closeness of the social support network) was also found to be inversely predictive of PTSD. This reinforces other studies in terms of social support. Parents of chronically ill children have consistently been found to have smaller social support networks than do the parents of children who do not have chronic ill health (Kazak 1987). In effect, the smaller the social support network the greater the risk of PTSD occurring, and conversely, the larger the social support network the smaller the risk of PTSD being present. As Kazak et al. (1998) point out, although the size of the social support network is a simple measure of social support, it does tend to represent a reliable assessment of the extent of isolation that a person or a family may experience. As has been discussed in the chapter on alienation, Kazak et al. (1998) found that many parents were eager to talk about their cancer-related experiences. However, unfortunately they did not often feel that they had others in their social support network who understood what they had been through or their ongoing distressing reactions. The fourth and final factor that was discussed by Kazak et al. (1998) concerned family variables. In the research study, these were found to be concerned with family cohesion, family adaptability, family communication and family satisfaction. These were not found to be significantly directly associated with PTSD. Rather, it was family function that was significantly associated with parental anxiety, and therefore was considered to indirectly predict symptoms of PTS. The researchers were of the opinion that the data from their research suggested that family variables could be a ‘background resource’ that affected levels of anxiety, and therefore indirectly affected the reactions that were specific to the child’s illness. Kazak et al. (1998) found in their study that most families appeared to be functioning competently, and this reflected a relatively neutral role for family variables in the understanding of PTSD. Because there is always a risk of a late relapse or of secondary tumours for children who survived cancer, Kazak et al. (1998) believe that parents

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can develop strategies for coping and adapting with this uncertainty – but at a price. They believe that the potential psychological implications of acting as if one’s child could die could have serious mental health implications. In addition, Kazak et al. (1998) point out that parents and health care professionals may view the intensity of the treatments in different ways. An example that they give concerns the fact that health care professionals might not realise that some events that are actually quite short in duration (but may be very intense) are actually important to parents in the long-term and they may provoke memories and distressing and recurring recollections of that event. They suggest that health care professionals consider offering parents some of the classic intervention techniques for PTSD, such as exposure and cognitive–behavioural approaches. Interventions that directly address the development of supportive relationships, as with the maintenance of these relationships, can also benefit the parents. In a later study, Kazak et al. (2004) explained that as well as the parents, it is likely that any member of a family which contains a child who has survived cancer could be experiencing intrusive memories, arousal, or avoidance that is specific to the cancer treatment of that child. In this latest study, Kazak et al. (2004) found that post-traumatic distress was common across families with a child who survived cancer, but that it tended to affect one particular member of the family rather than multiple members within a single family. They found that in their sample, the likelihood of PTSD being present in mothers since the child’s illness was 30%. They also reported that due to different gender patterns, differences in exposure, or family patterns, fathers did not report such high levels of PTSD. However, they did point out that both mothers and fathers remember many of the events around the diagnosis and treatment of the cancer, they recall their psychological reactions vividly, and are likely to have a clear understanding of the circumstances – including the possibility that the child could die. They also noted that fathers, although sometimes seen as more peripherally involved in the care of their child than mothers, were similar to mothers in terms of current symptoms of PTS, and that this demonstrated that the experience has long-lasting effects on them as well as the mother. They felt that their research suggested the importance of evaluating all family members for symptoms of PTS rather than the supposed primary caregiver – normally the mother, otherwise other family members with PTSD or symptoms of post-traumatic stress could be missed, with consequent long-term psychological sequelae. This welcome emphasis on considering other members of the family, particularly the fathers, is still quite new in terms of research into PTSD in parents of children with severe life-threatening illnesses. To turn to another life-threatening traumatic event, Hall et al. (2006) looked at the incidence of PTS symptoms and parents of children who had acute burns. They found that approximately 47% of the parents reported experiencing significant PTS symptoms three months after the burn had occurred. They looked at three independent pathways to PTSD symptoms, namely:

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r parent–child conflict r parents’ dissociation r children’s PTSD symptoms What they found was that parental anxiety predicted an increased parent–child conflict, whilst conflict with the extended family and the size of the burn predicted parental dissociation, and the size of the burn and children’s dissociation predicted children’s PTSD symptoms. Studies by several people (Byrne et al. 1986, Mason 1993, Rizzone et al. 1994) have shown that burns in children may be among the most stressful events for a parent to experience. Hall et al. (2006) explained that parental stressors associated with this traumatic event may include:

r r r r r

a parent’s own burn injuries witnessing their child’s painful surgeries and medical procedures being away from home other family members and social support contending with a serious impact of the burn on the child’s future

From this, it can be seen that the parental stressors for children with burns vary little from the parental stressors in any of the traumatic illnesses discussed previously. Thus, the contention is that, although there may be some differences in the stressors that parents experience when their child has a serious life-threatening traumatic illness, basically the concerns are the same, as are the overall criteria that lead to PTSD.

Post-Traumatic Stress and Siblings Often forgotten and marginalised when a child is hospitalised because of a life-threatening disease or following a traumatic event, siblings can also be severely psychologically affected. Whilst there has been much research into the effects on parents of children with cancer and other life-threatening diseases, there is very little that has been written about the siblings of these children in relation to PTS, particularly in the long-term (Alderfer et al. 2003), although there is a body of literature looking in a more general way at the concerns of siblings of childhood cancer survivors – and other chronic/acute disorders – in the short-term (Barbarin et al. 1995, Horwitz & Kazak 1990, Houtzager et al. 1999, Martinson et al. 1990, Sahler et al. 1994, Shannon et al. 1994a, 1994b, Sharpe & Rossiter 2002, Van-Dongen-Melman et al. 1995). In their research project, Alderfer et al. (2003) investigated whether or not adolescent siblings of children who had survived cancer experience PTS, by looking at 78 siblings who were adolescents. They were asked to self-report on their anxiety and PTS, as well as perceptions of the whole experience of living with a sibling who had cancer. What they found was quite worrying in that they found that 49% of the siblings in their sample reported mild PTS, whilst 32% demonstrated moderate to severe levels of PTS. They also discovered that 25% of them thought that their brother

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or sister would die during the treatment, and that more than 50% of the siblings found the cancer experience ‘scary and difficult’. Alderfer et al. (2003) explained that these latter perceptions were related to PTS. They also compared this sample of siblings of children who had survived cancer with a reference group of non-affected adolescents, and what they discovered was that, although there were similar levels of general anxiety between the two groups, the siblings of cancer survivors had raised levels of symptoms of PTS. (This also demonstrates that anxiety is a somewhat common trait in all adolescents!) From their research, Alderfer et al. (2003) were able to conclude that levels of PTS are elevated for siblings of children who survived cancer, in particular for female siblings (Foa & Street 2001). This they attribute to the possibility that this may be related to the increasing family responsibilities that may be given more to female siblings as opposed to male siblings of the sick child (Shannon et al. 1994a) which may expose the girl more intimately to the various traumatic stressors surrounding a child with cancer. They also refer to a paper by Eisenberg and Lennon (1987) by noting that this environmental and role difference may be exacerbated by gender differences because female siblings may have higher levels of empathy than male siblings. However, one could argue this point and hypothesise that it may also be explained by the fact that girls are allowed to express their empathy more than boys who are expected to cope more silently than their female siblings. Alderfer et al. (2003), citing Houtzager et al. (1999), do, however, point out that no matter the gender of the sibling, it is those siblings who are more emotionally linked to their sick sibling who may be more vulnerable to the traumatic stressors that accompany any diagnosis, treatment, and outcome of paediatric cancer. A report by Punam¨aki et al. (2006) also looked at symmetry and asymmetry in family responses to traumatic stress – this time the stress of war. In this paper, they show that families who are undergoing traumatic experiences can show asymmetry in their responses to it when, in their words, ‘there is “a share of work” in expressing vulnerabilities and strengths’ (Punam¨aki et al. 2006 p. 386). This is linked to individual family members’ coping efforts, adaptation styles and shared expressions of the pain that they are experiencing (Punam¨aki et al. 2006 p. 386). Thus, returning to the situation of a very sick child with a life-threatening illness, if one family member is excluded from the coping efforts of all the others (discussing the situation and experiences, caring for the child, being allowed to share feelings, etc.) then that person is going to experience different, but no less intense, feelings, which can lead to the development of the symptoms of PTS. Punam¨aki et al. (2006) also discuss the development of ‘parentification’ which is an extreme form of change of family roles in which children take on some of the caring and supporting tasks of the parents, where they are, for whatever reason, unable to fulfil them themselves (Chase 1999, ZahnWaxler & Radke-Yarrow 1990). In the case of the child with cancer or other life-threatening disorder, this may be because the parents are spending so much time at hospital with the sick child, or they are too upset themselves to be able to cope with anything but dealing with the sick child.

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In attempting to explain these findings, Alderfer et al. (2003 p. 281) pointed out that these siblings may well be witness to the ‘physical and emotional pain of an ill brother or sister, along with the parents’ distress’. They explain that for those siblings cognisant of the threat to life of their brother or sister, the uncertainty of the future may be disturbing. This may be disturbing not only in terms of their sibling having no future, but also concern over their own futures, or the future of the family relationships and dynamics. In addition, due to the necessity for hospitalisation and some degree of isolation of the sick child for treatment purposes, they may also have to come to terms with sudden and extended separations from the child with cancer and at least one of their parents. There may also need to be a renegotiation of family roles and responsibilities (Shannon et al. 1994b) which will again add to uncertainties of the future for the sibling, as well as increasing the anxieties. As is explained in more detail in the section above on PTSD and the family, the person not intimately and continually concerned with the care and treatment of the sick child can be left much more to their imagination as to what is happening. There is more time to dwell on the sick child and the possibility of a future without that child. In the short-term, there are many psychological consequences which are prevalent in siblings of children with cancer during the time of treatment, including (Barbarin et al. 1995, Martinson et al. 1990):

r Guilt – mainly centred around the fact that they themselves are well,

r r

r

r

r

whilst their brother or sister is so ill, and may even die. In addition, there is the guilt they may feel if they have had arguments/fights, etc. with their sibling (common occurrences amongst siblings in any family). Powerlessness – including the inability to be of help to their brother or sister. This is why most siblings jump at the chance to be bone marrow donors if there is the possibility of a transplant as treatment. Loneliness – even though the healthy sibling may have many friends outside of the family, there is no getting away from the fact that the siblings have known each other for longer than anyone else and they have a (sometimes unacknowledged) ‘bond’ between themselves. Anxiety – self-explanatory in that there is great anxiety, not only about how their sick brother or sister will fare, but also about how they themselves will cope – particularly if their sick sibling dies. There is also anxiety about how they will cope with their sibling if they survive – how will they be able to relate to them, etc.? Depression – brought on as a combination of the above, but magnified by the fact that, because of their youth, they have less experience of coping with difficult events, or even of ill health, and certainly less experience of coping with the possibility of someone dying with whom they are so close. Anger – anger and frustration that they feel that there is nothing that they can do to help their brother or sister. But also anger that their brother or sister has been so ‘selfish’ as to be so ill and therefore put them in this position. There is anger at possible lost opportunities for

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the future, and there is also anger that they have to change their life for the brother or sister just as they are struggling to find their own role in life. Jealousy – of all the attention that their brother or sister is getting, particularly if one, or both, of the parents are spending so much time (and money) with their sick sibling. This jealousy was brilliantly expressed by an article in a German magazine under the headline: Jealousy at the bedside: ‘What a pity that I am not sick . . . What healthy children think ¨ about their sick siblings’. Blumke (1994) reported on one child called Irma, whose brother Tomas, four years old, had leukaemia. Tomas was once more in hospital, and it was a difficult time for the family. Their mother was often with Tomas, so Irma spent much of her time alone – and she became annoyed with her brother, and used to say ‘my brother has it okay. He can play all the time with mummy. Why am I not ¨ sick?’ Blumke also discussed the case of Johan. The parents of Johan were also in the hospital every day. When Johan died, his sister then ¨ thought, ‘what luck, now they will have more time for me’ (Blumke 1994 p. 1 – translated by Vickers 1999b).

In addition, according to Barbarin et al. (1995) and Sahler et al. (1994), some of the siblings experience poor academic achievement as well as difficulties in social relationships, mood swings and behavioural problems (Alderfer et al. 2003). Also, Horwitz and Kazak (1990) reported that other siblings exhibited evidence of enhanced socialisation by taking on the roles of helping both their sick sibling and their parents. Returning once more to the study by Alderfer et al. (2003), the authors point out that although these siblings of children with cancer may not experience the same levels of exposure to the sick child and the whole ‘acute hospitalisation event’ as their parents (particularly the mother) who are constantly (if not living there for the duration of the treatment) at the hospital with the sick child and are involved in decisions of care and treatment – as well as undertaking some of that care themselves, they still experience many of the traumatic aspects of cancer. They are often exposed to both the physical and emotional suffering of their sick sibling, but at the same time, as mentioned earlier in this chapter, like many of the fathers, they are often not able to take an active participation in the physical and emotional care of their sibling, so leaving them to deal with ‘the unknown’ – a potent facilitator of fear, anxiety and depression. Another point to consider discussed by Alderfer et al. (2003) is that, unlike most survivors of childhood cancer, siblings are often isolated from the main source of support and comfort for them at this time – their parents. As Alderfer et al. (2003 p. 284) comment, ‘Shifts in family role responsibilities, possible long parental absences, and the intense distress of parents may interfere with successful adaptation of siblings to this severe stressor’. Finally, the report by Alderfer et al. (2003) discovered that children older than six years of age at the time of the diagnosis appeared to experience more symptoms of PTS than did those who were aged six years or less. This they attribute to a better memory of the experience and a greater

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understanding of its ramifications. In addition, older children at the time of diagnosis and initial treatment may be included more in the family discussions about the disease and its possible effects on their siblings, themselves, and the family as a whole (Shannon et al. 1994a). In the meantime the younger child is excluded to ‘protect them’ from anything that could distress them, not realising that this ‘shutting out’ from the discussions of their sibling’s illness and possible outcomes, and the concomitant secrecy, can be much more distressing than it would be if they were involved and allowed to express their feelings on the situation. Thus there is an, admittedly small, body of research and knowledge that puts forward the case for the development of PTSD and symptoms of PTS in siblings of children undergoing traumatic experiences. Much of this discussion has been about the effects on siblings of children with cancer because this is where the research has been focused. However, it is easy to make the leap to say that the same situation occurs for siblings of children with other life-threatening and life-altering conditions, for example children in neonatal and paediatric intensive care units, children with diseases such as cystic fibrosis, diabetes and life-threatening infectious diseases, as well as those who have had serious accidents, particularly where head injuries are involved. In the same way, this situation could apply where children have siblings who were born with severe combined immunodeficiency disease, although there are differences, the main one being that because these immunodeficiency disorders are genetic in origin, often the affected child is either the first child to be born in that family, or that the affected child is the only one to have survived. Although there are some families where there are siblings, very little research has been done on the potential problem of PTSD in these children.

Conclusion Whatever the reason, whatever the traumatic event, it is important to recognise that parents (both mothers and fathers) and possibly siblings will be profoundly and psychologically affected by it, and it is incumbent upon health care professionals working in all areas where children will require medical and nursing care to be aware of this, and to know how to recognise the symptoms occurring in the parents, and be able to do something about it – even if all they do is refer them to somebody who can work with the parents to relieve the symptoms. Ultimately, however, it has to be accepted that parents are going to suffer psychologically from seeing their child in life-threatening situations and traumatic events, for as Manne et al. (1998 p. 362) say, ‘There are few life experiences as horrifying and difficult as coping with the diagnosis and treatment of cancer in one’s child’.

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Summary of Psychosocial Themes within this Book Whilst this book has attempted to show the psychosocial problems that the children with SCID (severe combined immunodeficiency) and their families experience both from an intensely personal angle, using their own words and pictures, and from the perspectives of many experts; it has also discussed the parallels with children who have other disorders requiring hospitalisation at an early age, and where there is with some degree of isolation involved. Without a doubt, the SCID children experienced more problematical behaviours than did their peers, even those who had also had bone marrow transplants for reasons other than SCID (Vickers 1999b). What they did display were behaviours and attitudes akin to those of children with a chronic disorder, as discussed in the works of Susman et al. (1980a, 1980b) and Eiser (1985, 1990). These problems are also to be found in children who have experienced similar environmental and psychosocial stressors as the children with SCID, including those children with cancer and those who have experienced the intensive care environment. A major theme to emerge from both the children and the parents who took part in the SCID research study (Vickers 1999b) was that of alienation. For the children, this often took the form of alienation from other children of their own age, and even, in some cases, a feeling of alienation from their own families. This alienation may initially result from their many months of isolation, but it is also exacerbated by the continuing ill health and problems with psychosocial functioning experienced by many of these children. As has also been demonstrated by the literature, many children with similar chronic/acute disorders are at risk of experiencing post-traumatic stress. Pot-Mees (1989) believed that the bone marrow transplant children in her study suffered from a post-traumatic stress disorder. She cites Pynoos et al. (1987) who produced a systematic description of post-traumatic stress disorder in children. As Pot-Mees (1989 p. 73) reported ‘Based on a study of children’s reactions after witnessing a sniper Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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attack, he (Pynoos) reported symptoms which included: lessened interest in play or other usually enjoyable activities, estrangement, emotional constriction, sleep disturbance and difficulties with concentration’. Given the similarity with the psychosocial functioning of the children with SCID, there are grounds for investigating the children with SCID, as well as their parents, for post-traumatic stress disorder. Certainly, as far as the parents of the children with SCID were concerned, the psychosocial functioning of several of them was affected because they appeared to be suffering from a previously unreported post-traumatic stress disorder which had never been addressed. Like the children in the SCID study, several of the parents detailed their own feelings of alienation (not necessarily from the children – although a few did feel that there was some distance between them and their children) but more from their extended family, their friends and even occasionally from society. There are other problems that the family, particularly the parents, may have to face. Pot-Mees (1989 p. 16) described the family system as ‘. . . . being in a state of disequilibrium with role changes for all members’. This comment is also supported by Cohen et al. (1977) and Wiley et al. (1984). Pot-Mees (1989), discussing previous work by Patenaude et al. (1979), also pointed out that other factors such as previous illness, geographic dislocation and other emotional problems, can influence the relationships and functioning of the family, as was apparent with the parents of children with SCID. Separation, which for the parents of the SCID children can last for a year or more, can affect the marital and sexual relationships of the parents (Freund & Siegel 1986, Kamphuis 1979, Pot-Mees 1989). The resident parent may experience feelings of claustrophobia, sleep disturbance and increased mental and physical fatigue (Kamphuis 1979, Pot-Mees 1989, Pot-Mees & Zeitlin 1987). According to Artinian (1982) and Pot-Mees (1989), some parents experience difficulties in maintaining effective support as a consequence of their fears linked to the possible death of their child. An example of this was the reluctance of some parents to touch their child for fear of provoking a possibly fatal infection in him or her. Siblings, because of their age, generally have no problems with the children who have SCID because most were not born until long after their sibling had had a successful bone marrow transplant. However, as has been demonstrated, this is not universally the case with children who have other similar disorders, mainly because siblings of children with other disorders are often older than are the siblings of children with SCID. Other members of the families, particularly grandparents, spoil the children with SCID, as is usually the situation with most grandparents. From the psychosocial functioning of many of the children with SCID and their parents in this study, as identified and discussed in this book, one has to query whether they actually do have effective coping strategies in place. The high incidence of behaviour problems in some of the children and the problems and behaviours of their parents would certainly seem to suggest this to be so. Because of this, health professionals have a duty

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to step in and aid the families in developing and maintaining appropriate and effective coping strategies. As a first step to this, the problems have to be identified and acknowledged by parents and health care professionals.

Quality of Life and the Children/Parents in the SCID Study (Brief Summary) At the very commencement of SCID research study, the following question was posed as being central to the study: What is the long-term quality of life for children who have survived bone marrow transplants for Severe Combined Immunodeficiency Syndrome (SCID)? The research study answered the question by demonstrating that for some of the children, the quality of life they were experiencing was very good indeed. Minimal or no long-term ill health or disability, good psychosocial functioning and development, and good educational prospects all contributed to this state of affairs, along with parents who were ‘comfortable’ with their own and their child’s role in life. By far the largest group of children in the study could be said to have a quality of life that could have been improved, but that what quality of life they did have was acceptable to them and their parents. The children in this group tended to have some degree of ill health or disability, but they were able to cope with it and so did not have too many psychosocial problems, although generally they did have more than their peers who did not have SCID. In addition, their parents were able to be reasonably positive and supportive of their children. However, there was still a need for further help for these children and their families. Finally, there was a group of children within this study, particularly many children who were living in the UK, who appeared to have a poor quality of life. Some of the causes of this poor quality of life were identified in the study as:

r severe ill health or disability r poor, or no, coping strategies r the parents and/or the children still suffering from post-traumatic stress disorder

r the parents and/or the children who had other severe psychosocial problems In addition, age seemed to have a role to play in determining the quality of life of these children because, for some of them, adolescence appeared to exacerbate the psychosocial problems, leading to even more feelings of alienation and depression. Depression, alienation and anger appeared to be the main problems for children following bone marrow transplantation for SCID. These resulted from many variables and situations, including length of time in

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isolation, age when commencing isolation, changes in carers, routines and management throughout the long period of their treatment (i.e. a lack of constancy), and no peer/family (excluding parents) interaction for many months, often stretching to several years. There was also the fact that the children were in the ‘spotlight’ for so long – in other words, there was so much emphasis on that one child that the process of that child interacting with others at a later stage could be a difficult and psychologically painful process. The child often had problems because of his/her fears and uncertainties for the future as well as for the present. Finally, there was the possibility to consider post-traumatic stress syndrome in the child following a successful bone marrow transplantation. Considering the parents, the following factors could all lead to problems within the family, and in particular between the parents:

r feelings of loss of identity, control and their role as parents r the break-up of the family (however, temporary this may be) due to the need for one parent to be with their child

r a lack of privacy r a sense of guilt and of failure (again in their role as parents and protectors) Two major problems that were found in the parents of the children with SCID were:

r the development of post-traumatic stress disorder r a feeling of isolation, including isolation from each other, from the extended family or from the rest of their society

Concluding Remarks This book has taken the study by Vickers (1999b) into the effects on children who had survived bone marrow transplants and long-term isolation and hospitalisation for SCID and their parents as a focus for the exploration of the psychosocial issues linked to the effects of early hospitalisation and isolation on children with many different disorders and their parents. Although the many disorders mentioned in the book are different in terms of signs and symptoms, and treatments, there are also many commonalities, particularly in terms of the psychosocial stressors that the children and their families have to cope with in order to have a good quality of life during and after the illness. Lessons need to be learnt from those children who had SCID with a good quality of life, as well as from children and families of children with other chronic disorders, and also those who have had bone marrow transplants for reasons other than SCID. The present state of affairs concerning the quality of life of children with SCID in general is not unmanageable, and it may be possible to reverse some of the effects already in place by means of counselling and support and, above all, understanding of the problems, and their causes, of these children and families. The quality of life of most

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German children, and some of the UK children, gave cause for optimism that much could be done to improve the quality of life of all the children with SCID. Whilst striving for further improvements in the medical treatment of children with SCID, and indeed of children with all serious and lifethreatening disorders, there also needs to be an emphasis on the improvement of psychosocial care at all stages of treatment, and for a long time afterwards. To sum up the problems and experiences of families who are having to cope with a child with chronic ill health following an acute, life-threatening illness, I can do no better than to conclude with a snippet from one of the interviews from the SCID study (Vickers 1999b). These parents were talking eight years after their child had had a bone marrow transplant for SCID, but he had been left with some degree of chronic ill health. Father: ‘The biggest problem that we now experience is lack of aftercare. Nobody gives . . . and I don’t care what any agency says . . . everybody’s trying to support their own job. I know that because I work for part of the public service. The speech therapist would say they’ve got . . . he’s gonna be getting this, that and the other. What they say and what we get is two totally different things. And I think that would be a great thought to go home with. The main thing that we are needing is after-care. Somebody to make contact with, because we lose contact with the guys at hospital – the consultants. We lose contact with the doctors because they move on. And this is what we need. Someone like yourself, as we said, to make contact. Since you , we’ve never had anybody. left – since we left Talking makes a big difference, but to talk to people, they’ve got to . . . I . . . I’m of the mind that problems we’re identifying now . . . there is a big problem . . . a volcano in there waiting to explode – to coin a phrase. We are anticipating that now, and have done so for a couple of years now. We’re trying to cope with that, as I was saying to you earlier, but again, it’s still . . . it’s still gonna happen. It’s still gonna happen, and it’s gonna be down to us to deal with it. I can’t see anybody coming in to deal with it, and even if they did want to come in, the question is . . . is it gonna be of benefit for the child psychologist, to a six year old child who’s already undergoing awful problems, to make a bigger issue out of what he believes to be a problem? I would probably suggest the answer to it is – don’t confront the child; confront the parents. Teach the parents. Don’t do it with the child ‘cos it will cause problems. Do it with parents; let the parents . . .. . .’. Mother: ‘Cos they’ve got the understanding’. Father: ‘Yeah. The parents know the child better and can then deal with it as there’re going through. They try and deal with it on their own – which is what they suggested to us’. Mother: ‘Especially when I moved here, Peter. I was just left – then you get the social people coming up – the social workers coming up and , you know, what type of saying . . . they said to us when we were in

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help do you want? We had no family there, and, when we came here, no friends, you know – no family. We were just stranded. I had a child that couldn’t go outside – couldn’t mix with the other people . . . . . . . . . ’. Father: ‘But you were gone, you see. We couldn’t tell anybody about it. There’s not one member of my family or (mother’s) family, either fathers, or sister, uncles, aunties, and what have you. Not one of them – and I can honestly say, hand on heart – believed for one minute that we would still be together. Every one of them thought that we would have split up. My father said that no woman, with the exception of (mother’s) personality, would be able to come through. And that’s their individual personal beliefs. But we came through. But I would hate to see any other couple have to go through it the same way as what we did’. Mother: ‘If we’d had family with us around then, we wouldn’t be together. We had to rely on one another, ‘cos that’s all we had. If I’d had my mum around, and he’s had his dad there . . .. . .’. Father: ‘Which begs the question as well. All the interference and all the support might do more harm to the individual couple’s relationship . . .. . .’. Mother: ‘Family-wise’. Father: ‘Family-wise – yeah’. Mother: ‘Other people-wise, no. I mean, you were the only person we seen, Peter . . . on Sundays, wasn’t it’. Father: ‘If you’ve got the family there, taking all the problems off your back, (But if you haven’t) you’ve got to work through and build it up yourselves. You need support to do that. Become a team between yourselves, because your child won’t look at you, Peter . . . My child wouldn’t have looked at you for support, would he? He was looking at his parents for support’. All health professionals have to be sensitive to individual family needs (and the needs of individuals within families), and all members of a family need to be sensitive to the needs of others within the family. Above all, families need support – not for a few months after discharge, but possibly for years afterwards. Not everyone requires the same types and levels of support, but their needs have to be sensitively assessed and met. Can we afford not to give this support?

A

Children’s Drawings

Over the past 50 years or so, there has been much interest in the use of children’s drawings, particularly of the human figure, as an investigative tool in the evaluation of cognitive ability, personality and emotions (DiLeo 1983, Goodenough & Harris 1950, Hulse 1952, Johnson 1990a, Koppitz 1966, Machover 1951, O’Brien & Patton 1974, O’Malley & McNamara 1993). As Johnson (1990a p. 11) explains, ‘Assessment of children often presents a difficult challenge to the pediatric nursing professional. Accurate identification of physical and psychosocial problems in the pediatric patient is often hindered by self-concept of the child, development level and psychosocial relationships within the child’s family unit. Projective techniques are often the method of choice used by pediatric health professionals in an effort to obtain information which the child cannot or will not verbalise’. Johnson (1990a) argues that children may speak to us more clearly and openly through their drawings than they are willing, or able, to do verbally. Kelley (1985) found that children were able to express their experiences and feelings in drawing and painting, even though they were unable to verbalise them. She also found that the act of drawing decreased children’s anxiety because it provided them with a motor activity at a highly emotional time (i.e. they could lose themselves in the actual manual activity). Skybo et al. (2007 p. 15) discuss this idea further and point out that ‘drawing is a fun, inexpensive and easy-to-administer method’ that can be used to allow children a ‘projective means of expressing attitudes and emotions’. Skybo et al. (2007) echo Melnyk et al. (2003) and Ryan-Wenger (2001) by pointing out that children with emotional and behavioural problems, including attention deficit, aggressive behaviours and stress disorders that are now considered to be childhood ‘morbidities’, often go unrecognised by health care and educational providers because of difficulties in finding the time and educational preparation for the diagnosis of such problems. As was discovered during this research study, children may be unwilling to discuss their emotional status, or if they are willing, they may lack the vocabulary to do so (Skybo et al. 2007). Ryan-Wenger (2001) explains Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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that the very basic holistic nursing skills often used by children’s nurses to defuse their anxieties in hospital, such as playing with dolls and other toys, storytelling, or drawing, are also reliable and valid projective techniques for measuring emotional status. This is particularly so with the drawings of human figures which are quick, inexpensive and non-threatening techniques for children to use (Skybo et al. 2007). According to Koppitz (1984), when children are asked to draw something – as opposed to purely spontaneous drawing – the act of drawing (and the actual drawing) is used by the child as a method of communication that represents that child’s self-concept, anxiety, attitude and/or conflict. Wesson and Salmon (2001) undertook a study to examine the effectiveness of drawing (along with reenactment) as a means of facilitating children’s verbal reports about emotional and significant events that they had experienced or encountered. They found that drawing and re-enactment of the occurrence had elicited a greater number of items of descriptive information than had the verbal interview. Similarly, DiCarlo et al. (2000) found that one way to access the strengths and challenges of street children in Honduras was through the children’s drawings. Echoing Donald and Swart-Kruger (1994), DiCarlo et al. (2000) hypothesised that the content of the drawings of children would reflect the reality of their daily life on the streets of Tegucigalpa, Honduras, including the children’s attitudes and values. Their conclusion was that ‘along with other more traditional methodologies, social service providers might look to the richness of the drawings of street children to provide a window into the children’s circumstances, their social worlds and their aspirations’ (DiCarlo et al. 2000 p. 119). Teichman (2001) also used human figure drawings by children to explore the development of ethnic attitudes in Israel by examining the drawings of Jewish children of themselves and of Arabs. Interestingly, much of the work with children’s art has been undertaken by art therapists and psychotherapists (Vickers 2006). For example, Linesch (2002) worked with a group of children of various ages from the kindergarten to the eighth grade (from 4 to 14 years) the day following the World Trade Centre destruction by terrorists on 11 September 2001. Linesch had been assured that the children were unscathed by the terrorism because the children were unaware of details. However, as she wrote in her paper, ‘the art that emerged spoke from aching hearts and minds to attending hearts and minds’ (Linesch 2002 p. 156). The children had all drawn images from the destruction of the twin towers – some of them quite horrific, but she felt that this was the way in which the children had tried, within their own minds, to make sense of what had occurred, and had tried to work through their feelings about it. Another example of the power of children’s art and the way that it allows children to express their thoughts and feelings was seen recently in Berlin, Germany (Paterson 2005). In this exhibition of school children’s art from the Nazi era, the children at that time had illustrated in detail what they chose – or were ordered – to draw whilst they attended art classes at Munich schools during the 1930s and early 1940s. Again the images are very powerful, but whilst those of the American children in 2001 demonstrated horror,

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uncertainty, and fear, the paintings from the Germany of the Third Reich depicted a glorification of German might and brutality (Vickers 2006). Again, Sengupta (2006) reported on drawings by children in Darfur, Africa, following the terrible events experienced by the people who finally found their way into refugee camps. These drawings, although concentrating in the main on the violence that they had experienced and encountered, some, according to Sengupta (2006 p. 26) ‘feature a wistful hope of a better life’. Consequently, it can be seen that the use of drawings by children now has a history of providing explanations, communication and catharsis for them. Children’s drawings develop in an orderly fashion from basic scribbles towards consistent symbols. Kellogg (1970) has shown that even by the age of two years, children’s drawings, rather than being meaningless scribbles, can be differentiated into 20 types of markings, such as dots, lines and circles. Kellogg believes that every child can make these markings and those who are unable to do so are disabled in some way. According to Whaley and Wong (1987), children’s drawings are usually of themselves, those who are significant to them (such as family and friends) and their experiences. Most children enjoy drawing and so will co-operate with an adult when asked to do so, particularly if they are asked to draw themselves or their family. Thus, drawings have become an excellent source for measuring current functioning and for expressing present concerns and conflicts. For example, someone with depressive symptoms will produce drawings which include limited colour, more empty space, greater constrictiveness, disorganisation, incompleteness and execution with minimal effort. There are, however, three caveats to bear in mind when using children’s drawings in any assessment situation. The first caveat is that it is important to remember that there are real differences in drawings as a result of development and maturation. The second caveat is to remember the basic fact that children draw things as they see them, which is often very different from the way things appear to the adult (Johnson 1990a). The third caveat is that drawings by children should not be considered as diagnostic because they are not. Rather, they provide strong cues for follow-up interviews with the children and their parents (Skybo et al. 2007). In the present study, discussion of the children’s drawings is based upon the interpretations which have been collated by Oster and Gould (1987) from previous studies by such luminaries in the field as Buck (1948), Koch (1952), Machover (1952), Harris (1963), Hammer (1967), Koppitz (1968), Burns and Kaufman (1970), Jolles (1971), Bolander (1977) and DiLeo (1983). As Oster and Gould (1987 p. 24) state, the list of emotional indicators given can be used ‘. . . with some assuredness that theoreticians and researchers in this field have concurred on the interpretation of that sign.’ According to Oster and Gould (1987), human figure drawings can be used as projective instruments which can be analysed for emotional indicators. The emphasis on the drawings can then be centred on the child’s emotional conflict and attitudes rather than just on the milestones of development. These emotional indicators can be grouped into three categories.

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1. Overall quality of the figure – line quality, proportions and shading. 2. Untypical specific features – large/small head, teeth and crossed eyes. 3. Typical features – eyes, nose, feet and neck. In order to produce more useful information from this non-threatening approach, alternatives to the original human figure are now used, and the children are asked to draw houses, trees and their own families, amongst others. The reason for these alternatives is the assumption that each drawing will tap into various segments of the child’s personality. A house, for example, is thought to be concerned with family ties and with conflicts surrounding home life. This is also true of family drawings in which the child’s perceived position within the family hierarchy may be ascertained. For example, if children feel important, they will usually place themselves next to their parents. Children who feel themselves isolated or different may be at the side of the picture, or physically separated from them, for example, by a line, by a table etc. Drawing a tree is meant to reflect deeper, and possibly more unconscious, feelings about themselves. This is because a tree is emotionally more removed from them for the reason that it is perceived by the children as an inanimate object. This allows the children to safely express less desirable personal traits, whilst drawing a person allows the children to express real life feelings more directly. In the SCID (severe combined immunodeficiency syndrome) study, the children were all provided with the same size drawing paper and exactly the same set of felt-tip pens (that they could keep afterwards). They were asked to draw themselves, their family, themselves doing something they enjoyed, their house, and, occasionally, depending upon time, a tree. Once again, it must be stressed that the drawings can only be interpreted on the basis of age, maturation, emotional status, social and cultural background and any other relevant history of the child.

Interpretations of Drawings The main interpretations of drawings that were used in the SCID study (Vickers 1999b) are listed below, all of which are taken from Oster and Gould (1987).

Colour Colour can be highly subjective in meaning, for example:

r The excessive use of the colour ‘red’ may be associated with anger. r Dark colours if used primarily may indicate depression. r An excessive use of multiple bright colours may possibly be linked to manic tendencies.

Appendix

Figure A.1 1999b).

289

Self-portrait by an 8-year-old boy from the UK in the SCID study (Vickers

r The use of light, barely visible colours could indicate that the child is attempting to hide his or her true experiences. This child (Figure A.1) was identified by his father as having ‘manic’ tendencies.

Drawing a Person Human figure drawing portrays an unconscious projection of the way the child actually perceives him or herself.

r Hostility and anger may be indicated in children’s drawings by the use of glaring eyes, bared teeth, sneering lips or giving the figure weapons.

r Claw-like hands suggest aggressive tendencies. r Where there is poor depiction of parts of the figure, this can indicate low frustration tolerance and impulsivity.

r Shading is important because it can indicate anxiety (the greater the

r r

r r

shading, the more intense the anxiety). For example, shading of the face can indicate that the child is seriously disturbed with a poor selfconcept, whilst shading of the arms can portray aggressive impulses. The drawing of a figure that is leaning at an angle grater than 15◦ may be an indication of instability/mental imbalance. If the drawing is of a tiny figure, this may mean that the child has extreme insecurity, withdrawal, depression and/or feelings of inadequacy, whilst a drawing of a large figure can indicate expansiveness, with poor inner controls. Teeth may indicate aggressiveness (orally related). Arms are important in drawings of the human figure because they can be used to change or control the environment, so, for example, arms

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r r r

r

r

r

r

r r

r

r r

folded over chest are an indication that the child is hostile or suspicious, and arms held behind the back indicate that the child wants to control his or her anger, and if arms are omitted from the drawing, the child may have feelings of inadequacy or helplessness, or it could be a sign of guilt over hostility or sexuality. The drawing of short arms on a figure may illustrate that the child has a tendency to withdraw, a turning inward, or an attempt to inhibit impulses. The drawing of long arms may indicate that the child has ambition for achievement or for acquisition, and is capable of reaching out towards others. Hands can be important as well as arms in drawings, with the depiction of large hands indicating that the child displays acting out behaviour, whilst a drawing with the hands cut off is often a sign that the child is troubled and/or feels inadequate. Turning to fingers, long and spike-like fingers are an indication of aggression and hostility, whilst fingers that are enclosed by loop or are drawn in a single dimension may indicate a wish to suppress aggressive impulses. Shoulders may also be important indicators of the child’s psychological state – if they are unequal, this could be a sign that the child is emotionally unstable, if they are large, the child may be preoccupied with the perceived need for strength, and if they are squared, the child could be overly defensive and hostile towards others. Just as important in a child’s drawing are feet that indicate a degree of interpersonal mobility, with long feet possibly meaning that the child is striving for security, tiny feet being a sign of dependency or blunted feelings, and the omission of feet being a sign of a lack of independence. Legs that are absent in a child’s drawing of a human figure may indicate that the child feels constricted, and if they are of different sizes, this could be a sign that the child has mixed feelings regarding independence. Long legs on a figure demonstrate that the child is striving for autonomy, whilst short legs could be a sign of emotional immobility. A large head on a figure drawing could mean that the child is preoccupied with a fantasy life, and if it is small, this may mean that the child has obsessive–compulsive tendencies, or has feelings of intellectual inadequacy. An overemphasis on the mouth may suggest immaturity and/or oral aggression in the child, and a very large mouth is considered to be orally erotic, whilst drawing teeth may also denote aggressiveness in the child. Drawings of the ear are important because an overemphasis on them appears to equate to a degree of suspiciousness and paranoia. Drawings which include staring, blank eyes may equate to overall fear, whilst a sideways glance is indicative of suspicion and paranoid tendencies.

Appendix

Figure A.2 1999b).

291

Self-portrait by a 9-year-old girl from the UK in the SCID study (Vickers

r If hats appear in the drawing, this could be indicative of a person who is exerting much energy to control angry feelings in order to reduce the fear of becoming overwhelmed by them. Reinforced marks around a belt in a drawing are suggestive of sexual concerns. If the figure is drawn facing away from the viewer, then it is possible that the child displays paranoid or schizoid tendencies. In terms of the overall drawing of the figure, if the individual body parts are out of proportion, then the child may possess poor inner controls and have a low frustration intolerance, whilst a lack of defined shape could be a sign that the child has fearful thoughts concerning the body. If the drawing is impulsive, this could mean that the child has poor inner controls and a low frustration intolerance, and if the drawing is composed of shading and scratchy lines all within clearly demarcated boundaries, then this may be a sign of controlled anxiety within the child.

r r r

r

This girl (Figure A.2) has many psychosocial problems including much anxiety, poor body self-image, hostility, immaturity and a need for security. All these are evident in her self-portrait above.

The House r The details of a house are important, and can be divided into essential and irrelevant.

r Essential details should include at least one door, one window, one wall, a roof, a chimney (considered to be in a normal drawing), whilst

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r r

r

r r

r r r

r

r

r r

r

irrelevant details (e.g. shrubs, flowers, walkway) indicates a need for the child to structure his or her environment more completely, which is sometimes associated with feelings of insecurity or needing to exercise control in interpersonal contact. The chimney is a symbol of warm intimate relations, although it is sometimes associated with a phallic symbol of significance. However, an absence of chimney can be a sign of a lack of psychological warmth or of conflicts with significant male figures, whilst if the chimney is exceptionally large, there may be an overemphasis on sexual concerns on the part of the child, or the child may possibly have exhibitionist tendencies. Smoke is also important, with profuse smoke exiting the chimney indicating inner tension or internal anger, and a single line of smoke coming from the chimney may signal a perception of lack of emotional nurturance in the household. If the roof is drawn as one-dimensional (just a single line connecting two walls), this may demonstrate that the child is either unimaginative or emotionally constricted. The roof is particularly linked to fantasy, with an overly large roof indicating that the child seeks satisfaction in fantasy, and an emphasis on the roof indicating an attempt on the part of the child to control an inner fantasy. Gutters on the roof may indicate suspiciousness on the part of the child, whilst a lightning rod on the roof may indicate the child’s need for structure and control. Window(s) can often give clues to the state of mind of the child who is drawing a house. An absence of window(s) may indicate hostility or a withdrawn personality, whilst if they are present on the ground floor of the house but not the upper storey, then the child may be displaying a gap between reality and fantasy. If the windows are bare, then the child’s behaviour may mainly be blunt and direct, but if there are curtains at the window, then the child may have behaviour that is reserved and controlled, whilst shades on the window are a sign of suspiciousness and paranoia. If there are shutters at the window, and they are closed, this could be an indication of extreme defensiveness and withdrawal, whilst if they are open, this may indicate an ability to make sensitive interpersonal adjustment. The absence of a door may be a sign of extreme difficulty in allowing accessibility to others, whilst a very large door may mean that the child is overly dependent on others. An open door may denote a strong need on the part of the child to receive warmth from external world, but if the door has a lock or hinges, then the child may be indicating a degree of defensiveness. The inclusion of a peephole in the door is often a sign of suspiciousness and paranoia on the child’s part.

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r If the door is situated in the drawing of the house above the baser

r

r r

line, without steps, then this may denote a feeling of interpersonal inaccessibility. A fence around the house may indicate a need for emotional protection, and if there is a wall around the house, then the adequacy of this wall may be directly associated with the degree of ego strength of the child. A very long path up to the house may be a sign of lessened emotional accessibility, but if the path is narrow at house and broad at the commencement, then this may show that the child is superficially friendly. Finally, in terms of the actual drawing, if the house is drawn at the bottom of the paper, this could be a sign of basic home or intimate insecurities. If the perspective of the house that has been drawn is shown from below, this could mean either a rejection of the child’s home or feelings of an unattainable desirable home situation, and if the perspective is from above, then there may be some rejection of the home situation here.

Figure A.3 1999b).

Drawing of her home by an 12-year-old boy in the SCID study (Vickers

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r A hasty drawing may be a sign that the child is impulsive and uncaring, heavy outlines around the exterior of the house may indicate a need for structure and control, whilst shading is often a sign of anxiety on the part of the child. Several points can be made about this drawing (Figure A.3).

r There is no chimney present, indicating a lack of psychological warmth, or conflicts with significant male figures.

r The inclusion of flowers, etc., shows a need to structure his environr r

ment more completely, which is sometimes associated with feelings of insecurity, or needing to exercise control in interpersonal contact. The fence around the house indicates a need for emotional protection. The long path to the front door indicates lessened emotional accessibility and a need for structure in his environment.

All the above points do parallel the information on this boy gained from the interview, personal knowledge and the questionnaires.

The Tree ‘The drawing of a tree is believed to be associated with one’s life role and one’s capabilities in obtaining rewards from the environment. Generally, tree drawings have been considered especially rich in providing insights concerning “life content”, that is, displaying accurate biographical situations and/or offering personal characteristics of the person being examined’ (Oster & Gould 1987 p. 37). The tree appears to allow the child to bring out long-standing and subconscious feelings towards themselves at a more basic, primitive level. In addition, it is often considered a ‘safer’ object to draw than a human figure, because it is a neutral and less threatening object as opposed to the human figure which can be too personal to the child. This, in turn, allows the child to project their feelings onto the tree more openly and honestly than they might otherwise.

r The size of the tree is important, with an extremely large tree being

r

indicative of aggressive tendencies; expansiveness of behaviour, and a very small tree being indicative of inferior, and feelings of insignificance. Exaggerations of different parts of the tree can have significance, with an exaggerated emphasis on the trunk possibly indicating emotional immaturity, exaggerated emphasis on the crown of the tree being linked to an emotionally inhibited and/or analytical personality, exaggerated emphasis on roots being a sign of shallow emotional responses and/or limited reasoning, and an overemphasis on the roots as the enter the ground possibly linked to feelings of insecurity.

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r Imperfections in the tree, such as a scar, knothole, or a broken branch are r

r r r r

r

often associated with trauma (e.g. accident, illness, rape), and knotholes themselves have sexual symbolism. If the branch structure is abruptly flattened at top, then this may indicate an attempt to reject or deny painful fantasy life, whilst broken or dead branches may indicate the presence of past physical trauma and loss of satisfaction in life, and hidden branches are often a sign of impulsive and/or aimless behaviour. If the drawing includes the bark on the tree, and this bark is inconsistent, there may be signs of anxiety being displayed. Where the tree is drawn, using just two lines for the trunk and a loop for the crown, there are suspicions that the child is impulsive and has a changing personality. If the tree is seen coming out of a ground line, but there are no roots visible, then there are indications of repressed emotions, but if no ground line is present, then the child may be vulnerable to stress. If shading on the tree is excessively dark or reinforced, this may indicate hostile defences or aggressive behaviours in the child, but if only faint lines are used to draw the tree, then the child may be displaying feelings of inadequacy, indecisiveness. Fine, broken lines in the drawing of the tree are possibly indicative of overt anxiety, as is scribbling on the drawing.

The drawing below by this boy in the UK of a tree (Figure A.4) illustrates several aspects of his psychosocial profile.

Figure A.4 (1999b).

Drawing of a tree by a 6-year-old boy from the UK in the SCID study

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r This is a drawing of an extremely large tree which is an indication of aggressive tendencies.

r The exaggerated emphasis on the trunk indicates emotional immobility.

r The exaggerated emphasis on the crown of the tree is suggestive of this boy being inhibited emotionally and being analytical.

r The excessively dark shading suggests hostile defences and aggressive behaviours.

r The scribbling can denote anxiety. Finally, it must be noted that, as Oster and Gould (1987) stress, care must be taken with all children’s drawings and their interpretations. They need to be supported and validated by other observations, tests and research tools. On their own they are rather meaningless and must not be relied upon to give a diagnosis. In addition, context is all important.

Glossary

ACQUIRED IMMUNITY. Immunity to infections which is learned (acquired) by the body’s immune defences as they come into contact with specific infectious agents. Involves the lymphocytes and is also known as Specific Immunity. ADA. Stands for Adenosine Deaminase. Adenosine deaminase is an enzyme necessary for the conversion of adenosine to inosine in the cell. An absence of this enzyme causes toxins to remain in the cell, particularly lymphocytes, and thus causes a type of SCID. AGAMMAGLOBULINAEMIA. This is also known as hypogammaglobulinaemia. A lowering, or absence, of immunoglobulins (antibodies) in the body. ALLOGENEIC BONE MARROW TRANSPLANT. A transplant in which the bone marrow is obtained from another person. AMINO ACID. Sub-units of proteins. ANTIBODY. This is also known as Immunoglobulin. These are proteins which are produced by plasma cells (a type of lymphocyte) and are released into the circulation. These antibodies bind to antigens and hold them in order to allow other cells of the immune system to attack the antigen. Part of specific immunity. ANTI-MICROBIAL THERAPY. Drug treatment used against microbes. These drugs include antibiotics, anti-viral and anti-fungal drugs. AUTOLOGOUS BONE MARROW TRANSPLANT. A bone marrow transplant in which bone marrow is taken from a person, leukaemic cells disposed of and then returned to that same person. AUTOSOMAL DOMINANT GENETIC DISORDER. A genetic disorder in which only one copy of a gene (i.e. from mother or father) is necessary to cause a problem. If a parent is a carrier, there is a one in two chance Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

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of each child being affected. All who carry the disorder are also affected by it. AUTOSOMAL RECESSIVE GENETIC DISORDER. A genetic disorder in which both copies of a gene (i.e. from mother and father) is necessary to cause a problem. If both parents are carriers, there is with each child a one in four chance of being affected and a one in two chance of being a carrier. It is possible to be a carrier but not be affected. This is the situation when only one copy of the gene is faulty. BONE MARROW. The soft tissue which fills the cavities within bones. Most blood cells are formed here, including lymphocytes, in the form of stem cells. CELL-MEDIATED IMMUNITY. Immunity provided by T-cell lymphocytes in which the cells themselves destroy invading micro-organisms, such as viruses and bacteria. CHEMOTHERAPY. Another name for any drug therapy (i.e. chemical therapy). CHROMOSOME. The hereditary material within cells; chromosomes contain all genes and control genetic activity. In humans there are normally 46 chromosomes (23 pairs). These are made up of 44 autosomal chromosomes, or 22 pairs (numbered 1–22), and two sex chromosomes known as X and Y. A boy has XY and a girl XX. CID. Stands for Combined Immunodeficiency Syndrome. This name is a generic term for all the Severe Combined Immunodeficiency Disorders, although sometimes the two terms are considered interchangeable. Sometimes used for slightly less severe forms of SCID. See also SCID. CMV. Stands for Cytomegalovirus. An infectious micro-organism of the blood which causes few problems in the immune-competent person, but which can be fatal for the child with SCID, or, indeed, anyone with lowered immunity no matter the cause. COMPLEMENT SYSTEM. An enzymatic system of serum proteins which interact with one another in a cascading sequence. When the first enzyme is activated, it triggers off the next one, and so on. In addition, each reaction is amplified and enlarged. This system plays a very vital role in several immunological reactions, including phagocytosis and opsonisation, as well as the destruction of invading micro-organisms (cell lysis). CONDITIONING. The name given to the pre-bone marrow transplant drug regimen which prepares the body to receive the new bone marrow. CONGENITAL. Something that has been present since birth. CYTOTOXIC. The ability to damage, or destroy, cells of the body and other cells, such as invading micro-organisms (i.e. cyto = cells; toxic = poisonous to).

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ENGRAFTMENT. The situation when a bone marrow transplant has been successful and the donor’s bone marrow has been accepted by, or grafted into, the recipient’s body. ENZYME. A substance (protein) which speeds up the rate of chemical changes/reactions within the body in order to make them effective. FTT. Stands for Failure to Thrive. GENES. The sub-units of Chromosomes, consisting of lengths of DNA, which control the hereditary characteristics of organisms. Inherited in blocks from both parents. GRANULOCYTE. White blood cells which form part of the immune system. Under a light microscope they show what appear to be granules in their cytoplasm, but in fact these are vesicles which can contain toxic enzymes. Granulocytes are the major cells involved in phagocytosis, and they include neutrophils, basophils and eosinophils, and form part of the non-specific immune system. GvHD. Graft-versus-Host Disease. A possible complication of bone marrow transplants in which the cells of the transplanted marrow recognise and attack the cells in the recipient’s body. Can be fatal, but there is now a battery of drugs and monoclonal antibodies which are capable of preventing too severe a reaction, or even of preventing a GvHD in the first instance. HAPLOIDENTICAL. A haploid is one of a pair of genes. Thus, a haploidentical bone marrow transplant means that only one of the genes in the MHC match. In other words, the bone marrow comes from one of the parents and there is thus only half the genes that will match with the recipient’s own genes, hence ‘mismatched’. HAPLOTYPE. Means ‘half a genotype’ and refers to the complete set of MHC and closely associated genes inherited from one parent. Each cell, other than for the X or Y chromosomes, will have two haplotypes, one from the father and one from the mother. HEPATOSPLENOMEGALY. Enlargement of the liver and spleen. HGG. These initials stand for hypogammaglobulinaemia (see also agammaglobulinaemia). HLA. Human Leukocyte Antigen. The group of genes which code for the recognition molecules of major immune system cells of the human body. HISTOCOMPATIBLE. Sharing transplantation antigens HUMORAL IMMUNITY. The branch of the specific immune system which is concerned with antibodies/immunoglobulins, the cells of which are called B-lymphocytes and plasma cells. HYPOGAMMAGLOBULINAEMIA. See agammaglobulinaemia and HGG. Ig. Short for immunoglobulin (see also antibody)

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IMMUNOGLOBULIN. There are five classes, or types, of immunoglobulins, namely IgA, IgD, IgE, IgG and IgM (see also antibodies). INFLAMMATION. A physiological response to injury or infection, which helps to protect the body from further harm. The four cardinal signs of an inflammatory response are heat, swelling, pain and redness. INNATE IMMUNITY. Also known as non-specific immunity. This is immunity to infection which is innate, or inborn. This is present from birth and does not have to be learned. Includes white blood cells other than lymphocytes as well as many chemical and physical forms of defence. LAMINA AIR FLOW. A way of preventing an immune deficient person contacting air-borne infections. Filtered air is regularly and frequently blown into an isolation room at a high pressure whilst air already in the room is drawn out. It is a form of regular air exchange of filtered air for used air which may contain infectious organisms. LEUKOPAENIA. Absent, or decreased numbers, of white cells, usually of granulocytes, in the blood. Someone with leukopaenia is very prone to infections LYMPHOCYTE. One of the types of white blood cells which help to make up the immune system. These form the major part of the specific immune system. There are two major classes of lymphocyte – B cell and T cell. MENDEL. An Austrian monk who lived in the nineteenth century and is considered the ‘father’ of genetics. He first identified the different modes of genetic transmission from parent to off-spring, namely recessive and dominant. MHC. Major Histocompatability Complex. The collection of genes coding for the major histocompatability antigens. In humans, these are known as Human Leukocyte Antigens (HLA). MMR. The live attenuated vaccine against measles, mumps and rubella. MUD. Matched Unrelated Donor. Someone from outside of the family of a bone marrow transplant candidate whose bone marrow matches that of the would-be recipient, i.e. the HLAs of both people are a full match. NON-SPECIFIC IMMUNITY. See innate immunity. OMENN’S DISEASE. One of the specific types of SCID which is characterised by severe skin problems as well as the symptoms of severe immunodeficiency disease. OPSONIN. Substances found in blood and other body fluids. They bind to antigens and make them more susceptible to phagocytosis by the cells of the immune system. Antibodies and complement are the two main opsonins. PATHOGEN. An organism which causes disease.

Glossary

301

PEG-ADA. Polyethylene glycol-modified adenosine deaminase. The regular administration of this enzyme is another form of treatment for ADA SCID, other than bone marrow transplantation or gene replacement therapy. PHAGOCYTE. A white blood cell of the immune system which ingests and destroys micro-organisms, dead cells and other debris in the body. Many granulocytes are phagocytic cells. PNP. Purine Nucleoside Phosphorylase. An enzyme similar to adenosine deaminase, the absence of which also causes a severe immunodeficient disorder. PRECURSOR. A ‘forerunner’, e.g. a cell not fully developed into its mature form. PROPHYLACTIC. Preventative, e.g. a prophylactic medicine is one that is given to try and prevent a disease rather than one given to treat a disease. PURINE NUCLEOSIDE PHOSPHORYLASE. See PNP. RETICULAR DYSGENESIS. A severe congenital cellular and antibody (humoral) deficiency associated with agenesis of the granulocyte precursors of the bone marrow. Causes the most severe form of SCIDS. SCID. Severe Combined Immunodeficiency. A genetic failure of both parts of the specific immune system – the B- and T-cell lymphocytes. Without treatment, death from overwhelming infection occurs within a few months. SPECIFIC IMMUNITY. See acquired immunity. SPONTANEOUS GENETIC MUTATION. A mutation of genes which can occur spontaneously and can cause genetic disorders, even though neither of the parents are carriers. STEM CELLS. Immature blood cells found in bone marrow. These will eventually develop into one of the different blood cells such as lymphocytes, red blood cells or neutrophils. SYNGENEIC BONE MARROW TRANSPLANT. A bone marrow transplant between identical twins. The most successful type of transplant. THYMUS. An organ situated just behind the sternum (breast bone). It is in the thymus that T-cell lymphocytes mature, differentiate and learn to recognise cells of the host body. This organ is large in the very young but atrophies (shrinks) with age. TUMOUR SURVEILLANCE. Describes the role of the T-cell lymphocytes which recognise slightly abnormal recognition molecules on the surface of embryonic tumours and destroy them before they have a chance to grow and multiply. URTI. Upper Respiratory Tract Infection. Infection of the upper airways, including nasal passages. UTI. Urinary Tract Infection. Infection of any part of the urinary tract, including bladder, ureters and urethra.

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X-LINKED GENETIC DISORDER. Genetic disorder which is carried on the X-chromosome. Males are affected because they only have one Xchromosome, whilst females are usually only carriers. Females are not affected because they possess a second X-chromosome with a normal gene on it. This blocks the action of the abnormal gene. Males do not have this second blocking gene. There is a one in two chance of any girl born to a carrier mother being a carrier herself. Similarly, there is a one in two chance of any boy born to a carrier mother being affected.

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Index

Note: Italicized page numbers refer to boxes, figures and tables Achenbach Child Behavior Checklist, 56 acquired immunity, 32, 297 acute anxiety, 68 acute ill health, 113–14, 122 f acute stress disorder (ASD), 257 adenosine deaminase, 31, 297 adenosine deaminase SCID (ADA SCID), 21–2, 43, 150b adolescents with asthma, coping strategies in, 125 adult stressors, 123 agammaglobulinaemia, 36–8, 297 age, 116 aggressive behaviour, 55–6, 137–8, 160–62, 169–72 alienation, 223–9 causes of, 229–36 being special, 232 bonding and attachment, 232–4 chronic ill health, 236 guilt and blame, 234 isolation, 230–32 jealousy, 236 lack of play, 236 lack of support, 235–6 separation, 229–30 stressors, 235 and extended family, 237–8 and relationship with adults, 238–41 SCID (Vickers 1999b) study, 279–80 allogenic bone marrow transplant, 297 334

alymphocytosis, 37 amino acid, 297 anaesthetic drugs, 55 anger, 276–7 antibody, 297 antigens, 297 anti-microbial therapy, 297 antisocial behaviour, 160–62 anxieties in children, 181–4 hospitals, 181–2 novel situations, 181 people-related, 184 school-related, 182 sleep and night-time, 182–4 appraisal, 109–11 asthma, 125 ataxia telangiectasia, 31b attachment, 232–4 autologous bone marrow transplant, 297 autosomal dominant genetic disorder, 297–8 autosomal recessive genetic disorder, 297–8 bacillus Calmette-Guer`en (BCG), 43 Bare lymphocyte syndrome (BLS), 21 B-cell lymphocytes, 30, 34 behaviour disturbance, 147–52 Behaviour Questionnaire and Scales, 26 bereavement, 202–4, 260–61 birth order, 118

Severe Combined Immune Deficiency: Early Hospitalisation and Isolation. Peter S. Vickers © 2009 John Wiley & Sons, Ltd. ISBN: 978-0-470-31986-4

Index

blame, 234 blood pressure, and stress, 103 B-memory cells, 34 bonding, 232–4 bone marrow, 297–8 bone marrow transplant (BMT), 44 and development of children, 70–73 effects on child with SCID, 73–4 and isolation, 61–2 stress and coping model, 114–18 biographic variables, 116 family variables, 118 medical-diagnostic variables, 116–17 personality variables, 117 stressors, 117–18 as treatment for immune deficiencies, 43b bone marrow transplant survivors behavioural disturbance in, 147–52 depression in, 130 effects of isolation on, 73–4 parents of, 88–9 post-transplant behaviours of, 133–4 post-traumatic stress disorder, 253 quality of life, 10–11 bovine PEG-ADA, 43 Bruton, Ogden, 36 bubbles, 60–61 CD4 receptors, 30 cell-mediated immunity, 297–8 chemotherapy, 297–8 Child Health Questionnaire (CHQ), 14 childbearing, 3 children adjustment to chronic disease, 109–11 concepts of death in, 94–9 concepts of health in, 84–5 concepts of illness in, 85–91 coping with chronic ill health in, 124–8 children in isolation, 63–74 bone marrow transplantation, 70–74 cultural isolation, 66 effect of time, 64–5 effects on family, 69–70 perceptual isolation, 65 psychosocial disturbance, 66–9 social isolation, 65–6 types of environment, 63–4

335

children with diabetes, 88 children with leukemia, 87, 96 children with SCID, 7–28 anxiety in, 181–4 behavioural problems in, 160–69 aggressive behaviour, 160–62 antisocial behaviour, 160–62 disobedience, 162–6 loss of temper, 166–9 concepts of death in, 94–9 effects of bone marrow transplantation on, 73–4 effects of isolation on, 73–4 health perceptions in, 93–4 lack of confidence in, 184–8 neurotic behaviour in, 177–80 parents of, 281–4 post-transplant behaviours, 133–4 psychosocial behaviours, 135–45 vs children of other disorders, 135–9 vs children of reference group, 134–5 gender factor, 139–42 place of origin, 139–42 place of treatment, 142–5 quality of life, 281–2 children’s drawings, 285–96 aggressive and antisocial behaviour in, 169–72 colour, 288–9 and effects of isolation, 93–4 house, 291–4 interpretations of, 288–96 person, 289–91 tree, 294–6 use in psychosocial effects studies, 20 chromosomes, 297–8 chronic disorders, 8 child adjustment to, 109–11 child and maternal adaptation to, 119–21 psychosocial effects of, 130 chronic ill health, 113–14, 122 f , 124–8, 236 civil partnerships, 3 cognitive appraisal, 114 colour, in children’s drawings, 288–9 combined immune deficiencies, 30 combined immunodeficiency syndrome, 297–8 complement system, 31, 297–8

336

Index

conditioning, 117, 297–8 confidence, lack of, 184–8 congenital thymic aplasia, 31b, 38 coping, 107–8. See also stress causes of, 104 with chronic ill health, 124–8 functions of, 108 models, 108–24 appraisal, 111–13 child adjustment to chronic disease, 109–11 chronic and acute ill health, 113–14 resources, 112 strategies, 127 cord blood transplants, 45 cortisol, 104 crisis, 123 cultural isolation, 62, 66 cytomegalovirus, 297–8 cytotoxicity, 32 data collection, 23 death, 202–4 children’s concepts of, 94–9 exposure to, 262 denial, 89–90 depression, 130, 276 destruction, exposure to, 262 diagnosis, 116–17, 198–200 Die Universit¨atkinderklinik, 23–4 diet, 19 DiGeorge syndrome, 21, 30, 31b, 38 direct action, 127 discharge from hospital, 218–21 disobedience, 162–6 displacement, from home community, 261 domains of adjustment, 10 DSM-IV, 257 Dutch population, 14–15 early hospitalisation, 48–59 effects on children’s development, 51–3 history of studies into, 49–51 of neonates, 56–7 and post-traumatic stress disorder, 252–3 and surgery, 53–6 early traumas, 247–8 education, 20, 27 elderly population, 3

emotional disturbances, and hospitalisation, 54–5 engraftment, 299 environment effects of, 200–202 and ill health, 91–4 enzyme, 299 enzyme deficiencies, 31b enzyme replacement therapy, 43b enzyme substitution, 43 extended family, 3, 237–8 failure to thrive (FTT), 39–41, 299 Familien Hausen, 205 family, 2–6 definitions of, 2–3 importance of, 4–5 structure of, 3–4 family functioning, 4 family-centred care, 5–6 fantasy in children, 90 fathers occupation of, 118 and post-traumatic stress disorder, 264 fetal thymus transplant, 43b flashbacks, 246–7 Folkman, S., 111–13 Freud, Sigmund, 244 Gaucher’s disease, 70 gender, 139–42 and coping, 116, 128 and post-traumatic stress disorder, 266 and psychosocial behaviours, 139–42, 155 gender makeup, of family, 118 gene replacement therapy, 43b, 45 genes, 299 German children with SCID, 139–42 family support, 237–8 neurotic behaviour in, 179 relationships with adults, 238–41 studies, 21–2 graft-versus-host disease (GvHD), 118, 299 grandparents, psychosocial effects on, 217–18 granulocyte, 299 group support, 246 guilt, 204–5, 234, 276

Index

haematopoietic stem cell transplantation (HSCT), 263 haploid, 299 haplotype, 299 health, 79–81 children’s concepts of, 84–5 definition of, 79 dimensions of, 80 and environment, 91–4 health behaviour, influences on, 80 health status, 19 hepatosplenomegaly, 39, 150, 299 Hospital for Sick Children, 24 hospitalisation, 56–7, 117 hospitals, children’s worries over, 181–2 human immunodeficiency virus (HIV), 33 human leukocyte antigen (HLA), 44, 299 humoral immunity, 299 Hurler’s syndrome, 70 hurting, 78–9 hypogammaglobulinaemia, 299 hypothalamus, 104 idiopathic lymphocytosis, 37 ill health, and environment, 91–4 illness, 79–81 children’s concepts of, 85–91 and environment, 91–4 personal narratives, 81–4 stressors, 113–14 immune system, 30–36 non-specific immunity, 31–2 specific immunity, 32–6 and stress, 106–7 immunodeficiencies, 30 immunoglobulin(s), 34–5, 297, 300 immunoglobulin A (IgA), 35 immunoglobulin D (IgD), 35 immunoglobulin E (IgE), 35 immunoglobulin G (IgG), 34–5 immunoglobulin replacement therapy, 43b infants, 56–7 attachment behaviour in, 232–4 hospitalisation of, 56–7 and parental stress, 195–6 perception of trauma in, 253–4 post-traumatic stress disorder in, 244 inflammation, 32, 300

337

information seeking, 127 inhibition of action, 127 innate immunity, 300 intelligence, 116 intensive care units, 200 interviews, 20 intrapersonal factors, 119 intrapsychic strategy, 127 isolation, 60–63 and bone marrow transplants, 61–2 as cause of alienation, 230–32 and development of children, 62 effects on child with SCID, 73–4 in treatment of SCID, 42 types of, 62 variables, 62–3 isolation room, 200–201 isolation syndrome, 68–9 jealousy, 211–2, 229b, 236, 277 lack of support, 235–6 lamina air flow, 300 laminar air beds, 61 Lazarus, R.S., 111–13 Lazarus–Folkman model, of stress and coping, 111–13 leukopaenia, 300 libido, and stress, 104 life threat, degree of, 260 live polio vaccine, 43 liver transplant recipients, 10, 56, 146, 188–9, 252 locus of control, 118 loneliness, 61–2, 68, 201–2, 276 loss of temper, 166–9 lymphocytes, 32–4, 300 lymphopaenia, 31b major histocompatibility complex (MHC), 300 marital relationships, 118 marriage, 3 matched unrelated donor (MUD), 300 Mendel, Gregor, 300 mother–child separation, 61 mother–infant relationships, and hospitalisation, 56 mothers, and post-traumatic stress disorder, 264–74 myeloid cells, 32

338

Index

natural killer (NK) lymphocytes, 30 neonatal intensive care unit (NICU), 194–6 neonatal surgery, effects of, 53–6 neonates, hospitalisation of, 56–7 neurotic behaviour in children, 177 hospitals, 181–2 lack of confidence, 184–8 novel situations, 181 people, 184 schools, 182 sleep and night-time, 182–4 neutropaenia, 31b Newcastle General Hospital, 24 night-time, 182–4 non-specific immunity, 31–2 nutritional status, 19 occupation, of parents, 118 Omenn’s disease, 21–2, 25, 31b, 150b, 300 opsonin, 300 otitis media, 29, 39 paediatric cancer patients parents of, 196–7 post-traumatic stress disorder, 248–52 paediatric intensive care unit (PICU), 194 pain, 117, 248 palliative therapy, 42 parental stressors, 196 parent–infant relationships, and hospitalisation, 56 parents age of, 118 mental state of, 118 post-traumatic stress disorder in, 258–63 psychosocial effects on, 198–200 death and bereavement, 202–4 denial, 210 environment, 200–202 guilt, 204–5 separation and support, 205–10 quality of life, 281–4 role in trauma, 261–2 pathogens, 300 perceptual isolation, 62, 65 peripheral blood stem transplants, 43b, 45

personal illness narratives, 81–4 phagocytes, 301 phagocytosis, 32 phenomenology, 15 physical development, 10 physical discomfort, 117 physical growth, 19 physical well-being, 11 Pinkerton, P., 109–11 place of origin, 139–42 place of residence, 116 place of treatment, 139–42 plasma cells, 34 plastic bubbles, 60–61 Platt Report, 50 play, 236 Pless, I.B., 109–11 Pless–Pinkerton model, of child adjustment, 109–11 polyethylene glycol-modified adenosine deaminase (PEG-ADA), 301 post-transplant behaviours, 133–4 post-traumatic stress disorder, 279–80. See also stress in children with cancer, 248–52 in children with SCID, 243–55 early traumas, 247–8 flashbacks, 246–7 prevention and treatment of, 246 signs of, 245–6 definition of, 256–7 and depression, 130 factors in development of, 260 in families, 257–78 fathers, 264 mothers, 264–74 parents, 258–63 siblings, 274–8 symptoms of, 243–4 Pot-Mees, C., 114–18 Pot-Mees’s model, of stress and coping, 114–19 powerlessness, 276 precursors, 301 pre-school children, 66–9 primary appraisal, 109–11 primary immune deficiencies, 29–30 problem-solving skills, 112 protective clothing, psychosocial effects of, 201

Index

psychological well-being, 11–12 psychomotor function, 19 psychosocial adjustment, 10, 117 Psychosocial Adjustment to Illness Scale (PAIS-SR), 9–12 psychosocial behaviours, 152–7 psychosocial development, 10 psychosocial disturbance, 66–9 psychosocial effects, 129–59, 193–222 on children with SCID, 129–31 behavioural disturbance, 147–52 gender factor, 139–42 vs other disorders, 135–9 place of origin, 142–5 place of treatment, 142–5 post-transplant behaviours, 133–4 vs reference group, 134–5 on family, 193–222 death and bereavement, 202–4 denial, 210 discharge from hospital, 218–21 environment, 200–202 grandparents, 217–18 guilt, 204–5 parental stressors, 198–200 separation and support, 205–10 siblings, 211–17 post-transplant behaviours, 133–4 Rutter A scale, 131–3 Rutter B scale, 145–7 psychosocial functioning, 19–20 pulse, and stress, 103 purine nucleoside phosphorylase (PNP), 301 purine nucleoside phosphorylase SCID, 39 qualitative research, 16–19 quality of life, 9–16 critical attributes of, 9 data collection tools, 15–16 elements of, 9–12 measuring, 12–13 parameters of, 10, 19–20 quantitative tools, 13–15 quantitative research, 16–19 recurrence, 261 reference groups, 22–3 relapse, 261 resistance factors, 119 respiratory rate, and stress, 103

339

reticular dysgenesis, 21–2, 30, 31b, 150b, 301 reverse barrier isolation, 42, 60–61 risk factors, 119–20 Rutter A scale, 20, 131–3 gender factor, 139–42 neurotic behaviour, 178 post-transplant behaviours, 133–4 psychosocial behaviours in, 152–7 vs Rutter B scale, 145–7 SCID children vs children with other disorders, 135–9 SCID children vs reference group, 134–5 summary of psychosocial problems, 152–7 Rutter B scale, 20, 145–7, 152–7 Satisfaction with Life Domains Scale, 9–12 schools, as cause of worry, 182 SCID (Vickers 1999b) study, 16–27 children involved in, 21–2 children’s favourite activities in, 228t data collection, 23 hospitals involved in, 23–4 methods of investigation, 16 qualitative research, 17–19 quality of life, 19–20, 281–4 quantitative research, 17–19 questions, 16 reference groups, 22–3 results, 24–8 stress and coping model, 122–4 types of SCID, 22t secondary appraisal, 109–10 secretory immunoglobulin (IgA), 35 self-concept, 117 separation, 280 as cause of alienation, 229–30 and support, 205–10 serum immunoglobulin (IgA), 35 severe acute respiratory syndrome (SARS), 66 severe combined immunodeficiency syndrome (SCID), 36–45 cause of, 39 in children. See children with SCID as chronic disorder, 8 definition of, 301 diagnosis, 39 history, 36–8

340

Index

severe combined immunodeficiency syndrome (SCID) (cont.) incidence of, 38–9 overview of, 29–30 pre-transplant disorders and infections, 40–42 signs and symptoms of, 39–41 treatment of, 42–5 SF36 questionnaire, 14 short-limbed dwarfism, 31b siblings birth of, 247 and post-traumatic stress disorder, 274–8 psychosocial effects on, 211–17 significant others, loss of, 260–61 single-sex relationships, 3 size of family, 118 social development, 10 social isolation, 62, 65–6 social networks, 52, 112, 115 social support, 115, 120, 156, 237 social well-being, 12 socio-ecological factors, 119–20 socio-economic status, 118 Soothill, John, 38 special care, as cause of alienation, 232 special care baby unit (SCBU), 194 specific immunity, 32–6 spiritual well-being, 12 spontaneous genetic mutation, 301 stem cell transplant, 44–5 stem cells, 301 Strength and Difficulties Questionnaire (SDQ), 158 stress, 101–28 biological mechanisms of, 103–4 coping with, 107–8 definitions of, 102 and immune system, 106–7 models, 108–24 adaptation to chronic condition, 119–21 appraisal, 111–13 bone marrow transplantation, 114–18 child adjustment to chronic disease, 109–11 chronic and acute ill health, 113–14 SCID (Vickers 1999b) study, 122–4

physical symptoms of, 103 psychological consequences of, 104–5 stress processing factors, 119 stressors, 113, 123, 196, 235 support, lack of, 235–6 support seeking, 127 surgery, and hospitalisation, 53–6 Swiss-type agammaglobulinaemia, 31, 38 syngeneic bone marrow transplant, 301 T-cell lymphocytes, 30, 32–4 T-cytotoxic cells, 33 teenage pregnancies, 3 temper, loss of, 166–9 T-helper cells, 30, 33 thrombocytes, 31 thumbsucking behaviour, 177 thymic transplants, 43–4 thymus, 301 T-memory cells, 33 total parenteral nutrition (TPN), 46 trauma duration of, 260 role of parents in, 261–2 T-suppressor cells, 33 tumour surveillance, 301 Uncertainty in Illness Theory, 249 upper respiratory tract infection (URTI), 301 urinary tract infection (UTI), 301 Wallander, J., 119–21 Wallander et al.’s adaptation model, 119–21 Wiskott–Aldrich syndrome, 21, 31b World War II children, 94 worries in children, 181–4 hospitals, 181–2 novel situations, 181 people-related, 184 school-related, 182 sleep and night-time, 182–4 X-linked genetic disorder, 302 X-linked lymphopaenic agammaglobulinaemia, 31, 38, 39