RESPIRATORY DISORDERS
CP Cavendish Publishing Limited
London • Sydney
TITLES IN THE SERIES
ACCIDENT AND EMERGENCY CARDIOLOGY CLINICAL CARE DENTISTRY EAR, NOSE AND THROAT GENERAL PRACTICE GENITO-URINARY GYNAECOLOGY MEDIATION AND ARBITRATION NEPHROLOGY NEUROLOGY ONCOLOGY OPHTHALMOLOGY PSYCHIATRY RESPIRATORY DISORDERS UROLOGY VASCULAR SURGERY
RESPIRATORY DISORDERS Norman McI Johnson, MD, FRCP Consultant Physician and Director of Research and Development, The Whittington Hospital, London Honourary Senior Lecturer in Medicine, University College London Medical School Member of the Institute of Expert Witnesses
SERIES EDITOR Dr Walter Scott, LLB (Hons), MBBS, MRCGP, DObstRCOG
CP Cavendish Publishing Limited
London • Sydney
First published in Great Britain 1999 by Cavendish Publishing Limited, The Glass House, Wharton Street, London WC1X 9PX, United Kingdom. Telephone: +44 (0) 171 278 8000 Facsimile: +44 (0) 171 278 8080 e-mail:
[email protected] Visit our Home Page on http://www.cavendishpublishing.com
© Johnson, N McI 1999
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, scanning or otherwise, except under the terms of the Copyright Designs and Patents Act 1988 or under the terms of a licence issued by the Copyright Licensing Agency, 90 Tottenham Court Road, London W1P 9HE, UK, without the permission in writing of the publisher.
Johnson, Norman McI Respiratory Disorders – (Medico-legal practitioner series) 1. Respiratory organs – Diseases 2. Respiratory organs – Diseases – Treatment – Law and legislation I. Title 616.2’0024344
ISBN 1 85941 427 3
Printed and bound in Great Britain
FOREWORD When I first conceived the idea of the Medico-Legal Practitioner Series in the Summer of 1994, I had been preparing reports for lawyers on cases of alleged medical negligence for about five years. I had also been looking at other doctors’ reports for the same length of time and it was becoming increasingly apparent to me that one of the lawyers’ most difficult tasks was to understand the medical principles clearly. To be fair to the lawyers, there were some doctors who did not always make matters very clear. This, coupled with the difficulty which many doctors have in understanding the legal concept of negligence and related topics, merely served to compound the problem. A little more than two years have now passed since I wrote the foreword for the initial launch of the series and, already, the number of titles available in the series has reached double figures with many more imminent. Therefore, this seems to be an appropriate moment to take stock of our efforts so far and to assess the way in which matters are likely to unfold in the future. Since the publication of the first books in the series, there have been some exciting developments in the medico-legal scene and there can be no doubt that this is becoming an increasingly specialised field. That trend is likely to continue with the establishment of legal aid franchise firms of lawyers. Such firms will find it more and more necessary to identify strong cases and eliminate weak ones in an economical fashion and with as little risk as possible. One important feature of the more recent titles in the series is the inclusion of case studies which are placed adjacent to the relevant parts of the text and are listed in a table for ease of reference. Most chapters have several examples of cases which have either settled in the plaintiff’s favour or have fallen away because, perhaps, they were considered to be weak on negligence or causation. These studies give the reader a ‘feel’ for the work of the clinician and the difficulties which face him. The patient’s expectations do not always correlate particularly well with the doctor’s treatment plan, for example, in relation to consent, and such issues as this are often highlighted by the case studies. The other interesting development in some of the newer titles is the coverage of areas that do not relate to clinical negligence. With the series becoming more comprehensive, we have felt able to expand into other medico-legal areas. Examples include this volume, Respiratory Disorders, which deals with industrial lung disease, and Psychiatry, which covers testamentary capacity and the defence of insanity to criminal charges. So much, then, for the latest developments in the Medico-Legal Practitioner Series. Our aim remains as it was at the outset with regard to uniformity of approach and clarity of presentation. In this way, I hope that our readers, mostly the practitioners who are engaged in unravelling the complexities of the medical evidence that is the subject of so much litigation, will continue to rely on us as an invaluable source of reference. Walter Scott Series Editor Slough v
PREFACE The role of this book is to provide the reader with a readily accessible and, hopefully, easily understandable reference work of those areas of respiratory medicine which, in my personal experience, have tended to be those areas in which litigation occurs. The style I have chosen is designed to facilitate easy access to the facts for busy people whose time is valuable. The book is not supposed to be a fully comprehensive respiratory text – there are already many excellent examples of these. Nor is it meant to obviate the need for the respiratory expert’s opinion, rather, it is meant to enable the reader to understand the medical problem and enable greater understanding of the issues raised by clients or within experts’ reports. By greater understanding of the medical issues, so the lawyer will be able to ask the ‘right’ questions of their experts. By necessity, this volume is relatively compact. Where there are British nationally accepted guidelines for management of common conditions, these are referenced and the reader is advised to obtain reprints of these to refer to in greater depth alongside this book. Around 20% of all acute medical admissions are for respiratory illnesses. Regrettably, respiratory experts manage the minority of these, as there are limited numbers of respiratory consultants. Thus, although respiratory physicians are rated as low risks by the defence organisations, this is not necessarily the case for the respiratory problems managed by other specialists. The illustrations are chosen as examples of their kind. A word of warning should be made here to the non-medical reader. Chest X-rays are viewed as if looking at the patient. Thus, when the caption refers to, for example, a legion on the right side, this refers to the patient’s right side, which is, of course, to the left as the reader sees the illustration. Norman Johnson December 1998
vii
ACKNOWLEDGMENTS I am indebted to a large number of people for help with the preparation of this book: Bernice Walker for her rapid and accurate typing; Blackwell Science for permission to use illustrations from my book, Pocket Consultant – Respiratory Medicine, which were produced by Mr R Phillips and his staff in the Department of Medical Photography at the Middlesex Hospital; Lydia Malim, who drew the illustrations in that book, which are reproduced here; Clement Clark, for permission to reproduce endoscopic pictures; Dr Walter Scott, who has worked hard and patiently to keep me up to the mark on deadlines and whose idea this book was; and, lastly, my wife, Penny, for her patience throughout the preparation.
ix
CONTENTS Foreword
v
Preface
vii
Acknowledgments
ix
Table of Cases
xxv
Table of Figures
xxix
Table of Abbreviations
xxxv
1
THE WORK OF THE RESPIRATORY PHYSICIAN
1
WHAT IS RESPIRATORY MEDICINE? Introduction What is a respiratory physician? The workload of a respiratory physician The firm or team Training in respiratory medicine Typical working week for the respiratory physician Support in out-patients Staffing Investigational support for out-patients Follow-up in out-patients? Referral to other specialists Professional societies and standards Postgraduate continuing medical education for the trained physician Management involvement Medical complaints procedure Legal financial settlements Source of respiratory experts for medical reports Instructing a respiratory physician – when? General medicine Respiratory medicine What type of respiratory specialist? Practising or retired respiratory physicians? Standard reference books Textbooks of general medicine Standard reference for prescribing Standard specialist reference books Specialist journals on thoracic medicine General journals and sources Guidelines and protocols
1 1 2 2 3 3 5 6 6 7 7 8 9
xi
9 10 10 10 11 11 11 11 12 12 13 13 13 14 14 14 14
Respiratory Disorders
2
3
Common types of problem over which clients wish to take advice as to whether to sue their doctor Sources of experts Availability of experts and instructions Suggested obligations of instructing solicitors Obligation of respiratory physician to instructing solicitors
15 17 17 18 19
STRUCTURE AND FUNCTION OF THE LUNGS AND LUNG FUNCTION TESTING
21
ANATOMY The lungs The mediastinum The pleura Bronchial tree Chest wall Nerve supply Purpose of respiration Tests of respiratory function Introduction Peak expiratory flow rate Spirometry Patterns of disorder Gas transfer – diffusing capacity (DLCO) Overall lung size – total lung capacity (TLC) Histamine challenge test Allergy skin tests Other respiratory tests Blood gases Oxygen saturation Medico-legal points Failure to perform the correct respiratory function test Failure to measure oxygen status
21 22 22 22 23 24 24 25 25 25 26 37 28 30 31 31 32 33 33 36 36 37 37
COMMON CHEST SYMPTOMS AND THEIR INVESTIGATION
39
CLINICAL APPROACH
39
INVESTIGATION OF SYMPTOMS AND GENERAL POINTS Age In younger patients (up to 30 years) In older patients (over 50 years of age) Occupation Racial origin
39 42 42 42 42 42
xii
Contents Drug addiction Sexual orientation Smoking history
4
43 43 43
TAKING THE PATIENT’S HISTORY Past medical history Family history Social history Current medications and any recent changes Allergies
43 43 43 44 44 44
PRESENTING SYMPTOMS
44
COUGH Common causes of cough Acute cough – less than two weeks Chronic cough – more than two weeks Minimum acceptable standard for investigation of a chronic cough
45 45 45 45 46
HAEMOPTYSIS (COUGHING OF BLOOD) Minimum acceptable investigation for patients with haemoptysis
48
SHORTNESS OF BREATH Common respiratory (lung) causes of breathlessness Cardiac causes of breathlessness The minimum expectation for investigating breathlessness Wheeze Stridor
49 52 52 53 56 57
CHEST PAIN Angina pectoris Pleurisy Retrosternal pain – discomfort localised behind the breast bone Other causes of chest wall pain (intercostal nerve distribution) Chest pain: minimum investigation Features associated with chest pain Significance of fever in respiratory illness Poor appetite and weight loss Ankle swelling
57 57 57 58 58 58 59 59 59 60
ASTHMA
61
INTRODUCTION
61
CLINICAL FEATURES
61
xiii
46
Respiratory Disorders USUAL SYMPTOMS
62
ASSESSMENT OF THE SEVERITY OF AN ATTACK
62
INVESTIGATIONS OF PATIENTS WITH ASTHMA Respiratory function tests Variability of lung function Exercise test Domiciliary measurement Workplace measurement Allergy tests Skin tests Blood tests Allergic bronchopulmonary aspergillosis (ABPA) Chest X-rays Sputum Blood oxygen
63 63 64 65 66 66 66 66 66 66 67 67 68
RECOGNITION OF CAUSATIVE FACTORS
68
PREVENTION OF ASTHMA
69
DESENSITISATION
69
DRUG AVOIDANCE
70
INDUSTRIAL AND OCCUPATIONAL ASTHMA
70
TREATMENT OF ASTHMA
72
BRITISH GUIDELINES ON ASTHMA MANAGEMENT
72
ASTHMA THERAPY Beta agonist bronchodilators Anticholinergic drugs Antiallergic compounds Corticosteroids
73 73 73 73 73
MANAGEMENT OF CHRONIC ASTHMA Steps
74 74
TREATMENT OF ACUTE SEVERE ASTHMA Immediate Subsequent management Indications for transfer to intensive care unit
74 74 75 75
TREATMENT OF ACUTE SEVERE ASTHMA IN GENERAL PRACTICE
75
MEDICO-LEGAL ASPECTS OF ASTHMA MANAGEMENT
76
xiv
Contents Death/near death Poor long term supervision and control Inadequate assessment and recognition of severity of attack Inadequate treatment Delay in referral to hospital Delay in arrival of the emergency services Delay of therapy or failure of recognition of severity in hospital Inadequate treatment in hospital Case studies: general Case studies: primary care Case studies: secondary hospital care Steroid therapy Possible pitfalls Symptoms Therapy Comment Inadequate steroids Occupational asthma: medico-legal aspects 5
76 76 76 77 77 77 77 77 78 78 80 81 82 82 82 83 83 83
PNEUMONIA
85
INTRODUCTION
85
COMMON CAUSES OF PNEUMONIA Causes of community-acquired pneumonia Causes of hospital-acquired pneumonia
86 86 87
CLINICAL FEATURES
87
ASSESSMENT
88
CAUSES OF CAVITATING PNEUMONIA Infectious causes Non-infectious causes
89 89 90
PRINCIPLES OF PNEUMONIA MANAGEMENT Hospital admission Antibiotics Oxygen therapy Bronchodilators Intravenous fluids Physiotherapy Assisted ventilation
90 91 93 94 94 94 94 94
PROGNOSIS IN PNEUMONIA
94
xv
Respiratory Disorders MEDICO-LEGAL ASPECTS OF PNEUMONIA Failure of diagnosis 6
7
95 95
CHRONIC BRONCHITIS AND EMPHYSEMA
101
INTRODUCTION Causes Symptoms Clinical features Complications Acute exacerbations (attacks) Pneumonia Right heart failure (cor pulmonale) Respiratory failure Secondary polycythaemia Lung cancer Pneumothorax Investigations Routine out-patient referral Therapy Routine out-patient visits Drugs to be avoided The acutely ill patient warranting hospital assessment/admission Therapy of acute exacerbation Alpha 1 antitrypsin deficiency
101 101 101 102 102 102 102 102 102 103 103 103 103 103 104 104 105
MEDICO-LEGAL ASPECTS Management problems Inadequate assessment Inappropriate use of drugs Artificial ventilation Co-existing diseases Lung cancer Heart attacks Ability to work Life expectancy
107 108 108 108 109 109 109 110 110 111
PULMONARY EMBOLISM AND DEEP VEIN THROMBOSIS
113
INTRODUCTION
113
CLINICAL FEATURES Massive pulmonary emboli Smaller pulmonary emboli
113 113 114 xvi
105 105 106
Contents Predisposing factors
8
114
DIAGNOSIS AND INVESTIGATIONS Essential investigations Chest X-ray PA Blood gas estimation or oximetry Ventilation perfusion scan Investigation of limb veins
114 115 115 116 116 120
PREVENTION
122
TREATMENT Massive life-threatening pulmonary embolism Non-life-threatening pulmonary embolism Recurrent thromboembolism Anticoagulant control
122 122 123 123 123
MEDICO-LEGAL ASPECTS Prevention Failure to treat Failure to warn patients of the effects of therapy Failure to make the correct diagnosis
124 124 125 125 125
LUNG CANCER AND OTHER TUMOURS
127
LUNG TUMOURS The natural history of lung cancer Clinical presentation Diagnosis of lung cancer and staging of the disease Classification (shortened) Tests Chest X-ray Cell types Therapies and prognosis Practicalities of lung cancer care
127 128 128 129 129 129 130 133 134 135
MEDICO-LEGAL ASPECTS Missed diagnosis Slow diagnosis and treatment
135 137 139
OTHER LUNG TUMOURS Alveolar cell carcinoma Clinical features Investigations Bronchial adenoma Clinical features
139 139 140 140 140 140
xvii
Respiratory Disorders
9
Hamartoma
140
TUBERCULOSIS Introduction Types of disease
141 141 142
POPULATION AT RISK Common symptoms and signs Glandular tuberculosis Rarer symptoms and signs Diagnosis and investigation Therapy Routine follow-up Tuberculosis contacts
143 143 144 144 145 145 147 147
MEDICO-LEGAL ASPECTS
148
10 SARCOIDOSIS
155
INTRODUCTION
155
BACKGROUND
155
CLINICAL FEATURES Acute self-limiting sarcoidosis Chronic forms of the disease Pulmonary sarcoidosis Sarcoidosis of the skin Sarcoidosis of the eyes Reticulo endothelial sarcoidosis Skeletal and neuromuscular sarcoidosis Other metabolic and endocrine problems Cardiac sarcoidosis
156 157 157 157 158 158 158 158 159 159
INVESTIGATION AND DIAGNOSIS OF SARCOIDOSIS Plain chest X-ray Lung function tests Haematology Biochemistry Bacteriology Gallium scans Kveim skin test Histology
159 159 159 159 159 160 160 160 160
THERAPY IN SARCOIDOSIS
162
PROGNOSIS IN SARCOIDOSIS
163 xviii
Contents MEDICO-LEGAL ASPECTS OF SARCOIDOSIS Co-existing sarcoidosis – its effect upon personal injury claims 11 COMMON CONDITIONS AFFECTING THE PLEURAL CAVITY
163 163
169
PLEURAL EFFUSION Introduction Causes of transudation (protein < 30 g/litre) Causes of exudation (protein > 30 g/litre) Haemothorax – the collection of blood in the pleural cavity Empyema – the collection of pus in the pleural cavity Chylothorax Clinical features of pleural effusions Symptoms Physical signs Imaging Management and investigation of pleural effusions Pleural aspiration and biopsy Pleural fluid – analysis Complications of pleural effusions and drainage
169 169 170 170 171 171 171 172 172 172 172 172 174 175 176
PNEUMOTHORAX Introduction Common causes of pneumothorax Clinical features of pneumothorax Symptoms Physical signs Investigations Tension pneumothorax Management of pneumothorax Drainage techniques used Simple air aspiration Intercostal tube drainage Air aspiration versus tube drainage Tension pneumothorax Complications of pneumothorax Prognosis and indications for further surgery Prevention of pneumothorax
177 177 178 178 178 178 179 179 179 179 180 180 181 181 181 182 182
MEDICO-LEGAL ASPECTS Pleural biopsy Placing of intercostal tubes
182 182 183
xix
Respiratory Disorders
12 PULMONARY FIBROSIS
185
INTRODUCTION
185
CAUSES OF PULMONARY FIBROSIS Industrial exposure Connective tissue disorders Miscellaneous
186 186 186 186
CRYPTOGENIC FIBROSING ALVEOLITIS Clinical features of cryptogenic fibrosing alveolitis Investigations of cryptogenic fibrosing alveolitis Complications of cryptogenic fibrosing alveolitis Therapy for cryptogenic fibrosing alveolitis Prognosis for cryptogenic fibrosing alveolitis Medico-legal aspects of cryptogenic fibrosing alveolitis
187 187 187 188 188 188 189
EXTRINSIC ALLERGIC ALVEOLITIS Clinical features of extrinsic allergic alveolitis Investigations of extrinsic allergic alveolitis Pulmonary function tests Blood gases Skin tests Blood tests Treatment of extrinsic allergic alveolitis Acute episodes Chronic disease Compensation Prognosis
189 189 190 190 190 190 191 191 191 191 191 191
13 INDUSTRIAL RELATED RESPIRATORY DISEASES
193
PNEUMOCONIOSIS Diagnosis
193 194
COALMINERS’ PNEUMOCONIOSIS Clinical features Therapy Assessment of damage and disability Small opacities Large opacities Clinical features of pneumoconiosis Disability
194 194 195 195 195 195 196 196
RHEUMATOID PNEUMOCONIOSIS
xx
Contents (CAPLAN’S SYNDROME) Differential diagnosis of pneumoconiosis and other disorders Bronchitis and emphysema
196 197 197
ASBESTOS RELATED DISEASES Asbestosis Asbestos bodies Clinical features of asbestosis Chest X-ray appearances Lung function test Diagnosis of asbestosis Asbestosis or not?
197 197 198 198 198 199 199 199
MALIGNANT MESOTHELIOMA Presentation of mesothelioma Prognosis of mesothelioma Asbestos related lung cancer
201 202 203 203
SILICOSIS Symptoms and signs of silicosis
203 204
TALC AND KAOLIN
204
BYSSINOSIS Clinical features of byssinosis
205 205
OCCUPATIONAL ASTHMA Differential diagnosis of occupational asthma Skin tests Blood tests Lung function tests Bronchial provocation tests Clinical features Prevention and treatment
205 206 206 206 206 207 207 208
EXTRINSIC ALLERGIC ALVEOLITIS Farmer’s lung Clinical features Examination and investigations
208 208 209 209
CONDITIONS WHICH ARE CAUSED BY INHALATION OF OXIDES OF NITROGEN Clinical features
209 210
CADMIUM Features of acute cadmium poisoning Features of chronic cadmium poisoning
210 210 211
xxi
Respiratory Disorders BERYLLIOSIS
211
HARD METAL DISEASE
211
14 RARE RESPIRATORY DISEASES
213
ASPERGILLUS IN THE LUNG
213
DISEASES CAUSED BY ASPERGILLUS FUMIGATUS Simple extrinsic asthma Allergic bronchopulmonary aspergillosis Aspergilloma Common clinical features Investigations Therapy Invasive aspergillosis Investigations Therapy
213 213 213 216 217 217 218 218 218 218
ASPERGILLUS CLAVATUS
218
PULMONARY EOSINOPHILIA With asthma Without asthma Clinical features of pulmonary eosinophilia
218 219 219 219
CONNECTIVE TISSUE DISORDERS AND THE LUNG Rheumatoid arthritis Systemic lupus erythematosus Therapy Polyarteritis nodosum Therapy Systemic sclerosis Ankylosing spondylitis Clinical features affecting the lung Kyphoscoliosis Therapy Prognosis Cystic fibrosis Clinical features Investigations Therapy Prognosis Goodpasture’s syndrome Clinical features Therapy
219 219 220 220 220 220 220 220 221 222 222 222 223 224 224 224 225 225 225 225
xxii
Contents Wegener’s granulomatosis Diagnosis Therapy
226 226 226
15 MISCELLANEOUS CONDITIONS AFFECTING THE LUNGS
227
SICKLE CELL DISEASE Clinical features of sickle cell disease Investigations Therapy for sickle cell disease
227 227 227 228
ADULT RESPIRATORY DISTRESS SYNDROME – SHOCK LUNG Causes of adult respiratory distress syndrome Clinical features of adult respiratory distress syndrome Therapy in adult respiratory distress syndrome
229 229 229 230
SLEEP APNOEA Obstructive sleep apnoea Central apnoea Causes Clinical features Investigation Therapy
231 232 232 232 232 233 233
THERMAL INJURY (SMOKE INHALATION) Treatment of smoke inhalation Diagnosis and quantification of damage where smoke has been inhaled
233 233 234
CARBON MONOXIDE POISONING Clinical features of carbon monoxide poisoning Acute heavy exposure Chronic exposure Investigations Therapy
234 234 234 234 235 235
HIV INFECTION AND THE LUNG Respiratory diseases in HIV infection Minor symptoms Major symptoms Clinical features of respiratory involvement in HIV infection – pneumocystis pneumonia Investigation of patients with HIV who may have developed lung involvement Therapy
235 236 236 237
xxiii
237 238 238
Respiratory Disorders Complications
238
16 SURGICAL PROCEDURES IN RESPIRATORY MEDICINE
239
BRONCHOSCOPY Indications Samples
239 239 239
THORACOSCOPY
240
MEDIASTINOSCOPY Causes in the superior mediastinum Causes in the anterior mediastinum Causes in the middle mediastinum Causes in the posterior mediastinum Common presentations of mediastinal masses Investigations
240 240 240 241 241 241 241
OPEN LUNG BIOPSY
242
LOBECTOMY AND PNEUMONECTOMY
242
COMPLICATIONS OF SURGICAL PROCEDURES ON THE LUNG
242
MEDICO-LEGAL ASPECTS
243
Useful References
245
Index
249
xxiv
TABLE OF CASES Case 1:
asthma – sudden severe attack and death caused by hairspray . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .78
Case 2:
asthma – sudden death from pneumothoraces . . . . . . . . . . . . . .78
Case 3:
asthma death – inadequate appreciation of severity of attack – delay in referral to hospital . . . . . . . . . . .78
Case 4:
asthma – respiratory arrest – brain damage – delay in GP visit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .79
Case 5:
asthma death – inadequate therapy . . . . . . . . . . . . . . . . . . . . . . . .79
Case 6:
asthma – respiratory arrest – brain damage – delay in ambulance service . . . . . . . . . . . . . . . . . . . . . . . . . . .80
Case 7:
asthma death – inappropriate prescription . . . . . . . . . . . . . . . . .80
Case 8:
asthma death – allergy to non-steroid anti-inflammatory drug . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .81
Case 9:
pneumonia –failure to diagnose leading to death . . . . . . . . . . . .95
Case 10: pneumonia death – complicating asthma and pneumothorax – whose responsibility? . . . . . . . . . . . . . .96 Case 11: pneumonia death – inadequate assessment . . . . . . . . . . . . . . . . .97 Case 12: pneumonia – failure to recognise severity of illness and inadequate therapy . . . . . . . . . . . . . . . . . . . . . . .98 Case 13: pneumonia – decision not to ventilate . . . . . . . . . . . . . . . . . . . . .99 Case 14: pulmonary embolism – poor anticoagulant control . . . . . . . . .126 Case 15: lung cancer – wrong diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . .136 Case 16: lung cancer – missed diagnosis – incurable mediastinal spread . . . . . . . . . . . . . . . . . . . . . . . .138 Case 17: lung cancer – missed diagnosis – inadequate lung function . . . . . . . . . . . . . . . . . . . . . . . . . . .138 Case 18: tuberculosis – delay in diagnosis – ignored X-ray . . . . . . . . . .148
xxv
Respiratory Disorders
Case 19: tuberculosis – delayed diagnosis – ignored X-ray report . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148 Case 20: tuberculosis – delay in diagnosis – no chest X-ray . . . . . . . . . .149 Case 21: tuberculosis – delayed diagnosis – the value of audit? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .149 Case 22: tuberculosis – delay in diagnosing meningitis . . . . . . . . . . . . .149 Case 23: tuberculosis – unnecessary lung resection? . . . . . . . . . . . . . . . .150 Case 24: tuberculosis – unnecessary surgery – ignored sputum result . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .151 Case 25: tuberculosis – all that cavitates is not tuberculosis! . . . . . . . . .151 Case 26: tuberculosis – failure of contact tracing . . . . . . . . . . . . . . . . . . .153 Case 27: tuberculosis – side effects of anti-tuberculous drug – visual disturbance . . . . . . . . . . . . . . . . . . . . . . . . . . . .153 Case 28: tuberculosis – side effects of therapy – hepatitis and death . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .154 Case 29: sarcoidosis and occupational asthma . . . . . . . . . . . . . . . . . . . . .164 Case 30: sarcoidosis – end-stage disease or industrial accident? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .164 Case 31: sarcoidosis – the wrong diagnosis . . . . . . . . . . . . . . . . . . . . . . . .165 Case 32: sarcoidosis – negligent Kveim testing . . . . . . . . . . . . . . . . . . . . .165 Case 33: sarcoidosis – delayed investigation? . . . . . . . . . . . . . . . . . . . . . .166 Case 34: sarcoidosis – pneumothorax post-transbronchial biopsy . . . . . . . . . . . . . . . . . . . . . . . . . . .167 Case 35: sarcoidosis – the wrong patient? . . . . . . . . . . . . . . . . . . . . . . . . .167 Case 36: pleural effusion – intercostal nerve damage with pleural biopsy? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .182
xxvi
Table of Cases
Case 37: pneumothorax – death from intercostal drain cardiac puncture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .183 Case 38: pneumothorax – intercostal drain causing splenic rupture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .184 Case 39: unnecessary pneumonectomy complicated by breathlessness and chronic chest sepsis . . . . . . . . . . . . . .243
xxvii
TABLE OF FIGURES CHAPTER 2
STRUCTURE AND FUNCTION OF THE LUNGS AND LUNG FUNCTION TESTING
Figure 2.1:
surface anatomy of the lungs . . . . . . . . . . . . . . . . . . . . . . . . . .21
Figure 2.2:
bronchial tree . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .23
Figure 2.3:
normal valves for peak expiratory flow rates according to age, sex and height . . . . . . . . . . . . . . . . . . . . . .26
Figure 2.4:
spirogram tracings obtained from a vitograph . . . . . . . . . . .28
Figure 2.5:
typical patterns of abnormal lung function tests . . . . . . . . . .29
Figure 2.6:
forearm showing positive allergy skin prick tests . . . . . . . . .32
Figure 2.7:
typical patterns of blood gas abnormalities . . . . . . . . . . . . . .33
Figure 2.8:
oxyhaemoglobin dissociation curve . . . . . . . . . . . . . . . . . . . . .34
Figure 2.9:
report form for blood gas analysis showing normal ranges and shifts with acid base disturbances . . . . . . . . . .35
CHAPTER 3
COMMON CHEST SYMPTOMS AND THEIR INVESTIGATION
Figure 3.1:
normal chest X-ray (postero-anterior) . . . . . . . . . . . . . . . . . . .40
Figure 3.2:
diagram of a postero-anterior chest X-ray . . . . . . . . . . . . . . .41
Figure 3.3:
clinical features of common causes of haemoptysis . . . . . . .47
Figure 3.4:
causes and features of breathlessness . . . . . . . . . . . . . . . . . . .50
Figure 3.5:
associated features of breathlessness . . . . . . . . . . . . . . . . . . . .51
Figure 3.6:
physical signs in chest disease . . . . . . . . . . . . . . . . . . . . . . . . .54
Figure 3.7:
common causes of abnormal physical signs in the chest . . . . . . . . . . . . . . . . . . . . . . . .55–56
xxix
Respiratory Disorders CHAPTER 4
ASTHMA
Figure 4.1:
peak flow chart showing recovery from an acute asthma attack . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .62
Figure 4.2:
changes in the arterial PaCO2 with increasing severity of an asthma attack . . . . . . . . . . . . . . . . . . . . . . . . . . .63
Figure 4.3:
simple exercise test to diagnose exercise-induced asthma . . . . . . . . . . . . . . . . . . . . . . . . . . . . .65
Figure 4.4:
oxyhaemoglobin dissociation curve . . . . . . . . . . . . . . . . . . . . .67
Figure 4.5:
some causes of occupational asthma . . . . . . . . . . . . . . . . . . . .71
CHAPTER 5
PNEUMONIA
Figure 5.1:
right lower lobe consolidation (some consolidation on left, also) . . . . . . . . . . . . . . . . . . . . .86
Figure 5.2:
active pulmonary tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . .88
Figure 5.3:
tomogram of figure 5.2 confirming cavitation and showing area of calcification . . . . . . . . . . . . . . . . . . . . .89
Figure 5.4:
antibiotic therapy for home management of community-acquired pneumonia . . . . . . . . . . . . . . . . . . .91
Figure 5.5:
antibiotic therapy for in-patient management of community-acquired pneumonia . . . . . . . . . . . . . . . . . . .92
Figure 5.6:
antibiotic therapy for hospital-acquired pneumonia . . . . . .93
CHAPTER 6
Figure 6.1:
CHRONIC BRONCHITIS AND EMPHYSEMA
large bilateral basal bullae due to emphysema caused by alpha 1 antitrypsin deficiency . . . . . . . . . . . . . .107
xxx
Table of Figures CHAPTER 7
PULMONARY EMBOLISM AND DEEP VEIN THROMBOSIS
Figure 7.1:
multiple pulmonary infarcts . . . . . . . . . . . . . . . . . . . . . . . . . .115
Figure 7.2:
ECG showing acute pulmonary embolism . . . . . . . . . . . . . .116
Figure 7.3:
perfusion radioisotope lung scan showing multiple perfusion defects compatible with pulmonary emboli . . . . . . . . . . . . . . . . . . . . . . . . . . . .117
Figure 7.4:
normal ventilation scan of the same patient confirming the perfusion defects to be due to emboli . . .118
Figure 7.5:
multiple pulmonary emboli . . . . . . . . . . . . . . . . . . . . . . . . . . .119
Figure 7.6:
pulmonary angiogram of the same patient confirming the occlusion of many major pulmonary vessels due to emboli . . . . . . . . . . . . . . . . . . . .120
Figure 7.7:
venogram showing extensive venous thrombosis in the left femoral vein . . . . . . . . . . . . . . . . . . . . . . . . . . . . .121
CHAPTER 8
LUNG CANCER AND OTHER TUMOURS
Figure 8.1:
carcinoma of the left upper lobe bronchus, appearing as a left hilar mass and causing left upper lobe collapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . .127
Figure 8.2:
view of normal bronchi through a bronchoscope . . . . . . . .130
Figure 8.3:
carcinoma of the bronchus . . . . . . . . . . . . . . . . . . . . . . . . . . . .131
Figure 8.4:
carcinoma of the bronchus – chest wall (rib) and vertebral invasion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .132
Figure 8.5:
carcinoma of the bronchus – liver metastases . . . . . . . . . . .132
Figure 8.6:
CT scan of the brain showing a large intracerebral tumour, a secondary from a bronchial carcinoma . . . . . . . .133
Figure 8.7:
cumulative percentage surviving five years by clinical and surgical (including pathological examination of specimen) TNM subsets . . . . . . . . . . . . . .134
xxxi
Respiratory Disorders CHAPTER 9
TUBERCULOSIS
Figure 9.1:
bilateral upper lobe infiltration due to active tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .141
Figure 9.2:
mediastinal lymphadenopathy and miliary interstitial nodules in the lung fields due to tuberculosis . . . . . . . . .142
CHAPTER 10
SARCOIDOSIS
Figure 10.1: bilateral hilar lymphadenopathy due to sarcoidosis – stage I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .156 Figure 10.2: bilateral hilar lymphadenopathy with mid zone interstitial infiltration – stage II sarcoidosis . . . . . . . . . . .157 Figure 10.3: differential diagnosis of granulomatous disorders within the lung . . . . . . . . . . . . . . . . . . . . . . . . . . .161 CHAPTER 11
COMMON CONDITIONS AFFECTING THE PLEURAL CAVITY
Figure 11.1: medium sized left pleural effusion . . . . . . . . . . . . . . . . . . . .169 Figure 11.2: diagrammatic representation of management of pleural effusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .173 Figure 11.3: left side pneumothorax in a patient with extensive bilateral pulmonary fibrosis following a percutaneous drill biopsy . . . . . . . . . . . . . . . . . . . . . . . . . . .177 CHAPTER 12
PULMONARY FIBROSIS
Figure 12.1: cryptogenic fibrosing alveolitis . . . . . . . . . . . . . . . . . . . . . . . .185 Figure 12.2: some examples of extrinsic allergic alveolitis and their origins . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .190
xxxii
Table of Figures CHAPTER 13
INDUSTRIAL RELATED RESPIRATORY DISEASES
Figure 13.1: pneumoconiosis – progressive massive fibrosis, confluent interstitial shadowing giving rise to mass-like legions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .193 Figure 13.2: calcification of the diaphragmatic pleura together with left pleural plaques due to asbestos exposure . . . . .200 Figure 13.3: left sided mesothelioma of the pleura due to asbestos exposure . . . . . . . . . . . . . . . . . . . . . . . . . . .201 Figure 13.4: mesothelioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .202 CHAPTER 14
RARE RESPIRATORY DISEASES
Figure 14.1: patchy shadow and partial collapse of the right upper lobe due to bronchopulmonary aspergillosis . . . .214 Figure 14.2: the same patient as shown in figure 14.1 – separate episode of right upper lobe collapse/consolidation . . . .215 Figure 14.3: large aspergilloma in an old tuberculous cavity at the left apex . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .216 Figure 14.4: aspergilloma in cavity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .217 Figure 14.5: severe kyphoscoliosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .221 Figure 14.6: cystic fibrosis – hyperinflated lungs with marked upper zone interstitial shadowing and basal tramline shadows due to bronchiectasis . . . . . . . . .223 CHAPTER 15
MISCELLANEOUS CONDITIONS AFFECTING THE LUNGS
Figure 15.1: non-cardiogenic pulmonary oedema in a patient with ARDS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .228 Figure 15.2: sleep apnoea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .231 Figure 15.3: pneumocystis pneumonia in a patient with AIDS – bilateral ground glass appearance in both lower and mid zones . . . . . . . . . . . . . . . . . . . . . . . .236
xxxiii
TABLE OF ABBREVIATIONS ABPA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .allergic bronchopulmonary aspergillosis ACE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .angiotensin converting enzyme AFB . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .acid fast bacilli (tuberculosis) AIDS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .acquired immune deficiency syndrome APTT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .activated partial thromboplastin time ARDS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .adult respiratory distress syndrome AVMA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .Action for Victims of Medical Negligence BCG . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .Bacillus Calmette-Guérin BMJ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .British Medical Journal Br J Hosp Med . . . . . . . . . . . . . . . . . . . . . . . . . . . .British Journal of Hospital Medicine BTS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .British Thoracic Society cANCA . . . . . . . . . . . . . . . . . . . . . . . .cytoplasmic anti-nuclear cytoplasmic antibody CCST . . . . . . . . . . . . . . . . . . . . . . . . . .Certificate of Completion of Specialist Training CFA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .cryptogenic fibrosing alveolitis CME . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .continuing medical eduction CMV . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .cytomegalovirus COAD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .chronic obstructive airways disease COLD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .chronic obstructive lung disease COPD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .chronic obstructive pulmonary disease CPR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .cardio pulmonary resuscitation CPAP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .continuous positive airways pressure CT scan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .computerised tomographic scan CXR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .chest X-ray DDI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .diphenylmethane di-isocyanate DIP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .desquamative interstitial pneumonia DLCO . . . . . . . . . . . . . . . . . . . . . . . . . . .diffusion coefluent for lung carbon monoxide DPG . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .diphosphoglycerate ECG . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .electro-cardiograph EDTA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .ethylenediamine tetra acetic acid FEV1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .forced expired volume in one second FVC . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .forced vital capacity FP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .family practitioner GP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .general practitioner HIV . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .human immuno-deficiency virus HP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .house physician HRT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .hormone replacement therapy xxxv
Respiratory Disorders I125 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .iodine IgE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .immunoglobulin E ILO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .International Labour Office INR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .international normalised ratio ITU . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .intensive therapy unit iv . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .intravenously kPa . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .kilo Pascals KS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .Kaposi’s sarcoma LIP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .lymphocytic interstitial pneumonitis MAST MRI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .magnetic resonance imaging NIPPV . . . . . . . . . . . . . . . . . . . . . . . . . . . . .non-invasive positive pressure ventilation NSAID . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .non-steroidal anti-inflammatory drug PA . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .postero-anterior PaCO2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .partial pressure of carbon dioxide PaO2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .partial pressure of oxygen PCP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .pneumocystis carinii PCR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .polymerase chain reaction PEEP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .positive end expiratory pressure PEFR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .peak expiratory flow rate PO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .per orum (by mouth) RAST SACE . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .serum angiotensin converting enzyme SHO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .senior house officer SPR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .specialist registrar TDI . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .toluene di-isocyanate TLC . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .total lung capacity UIP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .usual interstitial pneumonia V/Q lung scan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .ventilation perfusion lung scan
xxxvi
CHAPTER 1
THE WORK OF THE RESPIRATORY PHYSICIAN
WHAT IS RESPIRATORY MEDICINE? Introduction Respiratory medicine is the speciality concerning diseases of the chest, primarily the lung (excluding diseases of the heart and major blood vessels within the chest). The common problems referred to a respiratory physician include breathlessness, cough with or without the production of sputum, coughing up of blood (haemoptysis), non-cardiac chest pain, abnormalities seen on GP chest X-rays, and wheeze. The types of diagnostic categories therefore seen by a respiratory physician are most commonly asthma, acute bronchitis, chronic bronchitis and emphysema, lung cancer, pneumonia, tuberculosis, sarcoidosis, lung fibrosis, interstitial lung diseases and diseases of the pleura including pleural effusions (fluid), pneumothorax (air in the pleural cavity) and pleural tumours (mesothelioma) and industrial lung diseases. On the basis of claim records, respiratory medicine is rated as a low risk litigation area by the medical defence bodies, although respiratory diseases are extremely common and one of the most common reasons for hospital admission and hospital referral. This low risk pattern might be thought, flatteringly, to be due to the quality of the practitioners in the field of respiratory medicine, but, equally, may have something to do with the fact that many of the diseases seen by the respiratory specialist occur in the lower socio-economic groups who may be unemployed at the time of referral, hence, significant loss of earnings is unlikely to be part of a claim. Secondly, many of the diseases such as lung cancer may be incurable and thus it may be difficult in a court of law to substantiate that a missed or delayed diagnosis affected prognosis and outcome. Similarly, with diseases such as severe chronic bronchitis and emphysema, the patient may be so ill and have such a short expectation of life that medical negligence or accident will have made little difference to the ultimate outcome. At the other end of the spectrum, diseases such as asthma occur in young people and are potentially fatal if treated incorrectly or inappropriately. Overall, however, the number of successful claims against respiratory physicians is low compared to many other medical and surgical specialities.
1
Respiratory Disorders
What is a respiratory physician? The workload of a respiratory physician The majority of respiratory physicians practise both as acute general medical physicians and as respiratory physicians. They will have attained specialist recognition for general internal medicine and respiratory medicine from the Royal College of Physicians, through specialist higher training and accreditation of the Higher Specialist Medicine Training Committee of the Royal Colleges of Physicians of London, Edinburgh or Glasgow. From 1 January 1997, this accreditation changed, with the introduction of the Calman proposals for the specialist registrar grade, when, at the end of successful specialist registrar training, there is the award a Certificate of Completion of Specialist Training (CCST). At the time of writing, all training posts in respiratory medicine are designed to provide dual accreditation in respiratory and general medicine. For the acute general medical part of the respiratory physician’s workload they will be on a rota, usually for about one in four to one in six nights and weekends, where their firm (team) covers all acute medical admissions for the hospital. All medical patients are admitted under the care of the duty physician, whether they suffer from pneumonia or asthma, which are appropriate to the physician’s specialist interest, or whether they have had a stroke, coronary thrombosis (heart attack), acute gastrointestinal bleed, drug overdose or other acute emergency. The physician’s general medical training and that of his team should enable them to manage these patients safely, calling on other specialist physicians when and if needed. It is usual for there to be hospital guidelines or protocols for the management of common emergencies, such as acute myocardial infarct (heart attack), under which patients go to a coronary care unit whose overall management lies in the hands of a cardiologist, and there will normally be shared care, where a cardiologist will be available to provide assistance and advice to the general physician. Similarly, with acute gastrointestinal bleeds there would normally be the assistance of a gastroenterologist who is available for emergency upper gastrointestinal endoscopy to diagnose and deal with the source of the bleeding. In turn, the respiratory physician provides specialist advice to other firms on acute respiratory problems on which other consultants require assistance, such as severe pneumonia or asthma. The volume and demand for acute respiratory medicine (20% of all acute medical admissions) is, however, such that not all acute respiratory admissions can be or are seen by a respiratory specialist. Although there are 2
The Work of the Respiratory Physician standards to be expected from other (non-respiratory) acute medical firms for the basic minimum care of respiratory patients, other general physicians should refer difficult problems to the respiratory specialist. This referral is in the hands of the referring physician.
The firm or team Until recently, the building block of acute medical rotas in hospital was that of the ‘firm’. This usually consisted of a consultant, a middle grade doctor (a registrar or senior house officer) and a pre-registration house physician. With increasing pressure on reducing junior doctors’ hours from 72 hours or more worked per week to 56 or less (there are currently negotiations around the introduction of EEC Directives of a 48 hour maximum working week, from which junior medical practitioners are currently exempt), there has been a change in the medical staffing structure and working of most hospitals. This is gradually leading to a ‘team’ concept, where there is a grouping of six to eight doctors, typically including two or three consultants, one or two middle grade staff (specialist registrars (SPRs) and/or senior house officers (SHOs)) and one or two pre-registration house physicians (HPs). Within this team, there may be shift working or partial shifts at the junior or middle grade level in order to reduce the hours of work. This has the knock-on effect of the possibility of a lack of continuity of patient care. The importance of effective handover and team work is being recognised with the greater involvement of other health professionals leading to an enhancement of multi-disciplinary working, in particular, the development of nurse practitioner roles, doctor’s assistants, clinic care co-ordinators, and phlebotomists. Nonetheless, the overall care for the patient’s medical well being lies with the consultant (or consultants) and, ultimately, the trust for which they work, regardless of how the day to day working of the team is allocated.
Training in respiratory medicine Hitherto, at the end of undergraduate studies, a basic medical qualifying degree of MB BS (Bachelor of Medicine, Bachelor of Surgery) was awarded. There then followed a pre-registration year, consisting of a six month post in medicine and a six month post in surgery. After satisfactory completion of this year, full registration was granted by the General Medical Council and the practitioner was entered on the full medical register. There would then follow a one to two year period as a senior house officer in medicine, during which time the trainee would take the Membership of the Royal College of Physicians examination – Part I being based upon multiple 3
Respiratory Disorders choice questions, Part II examined by written, clinical and oral examination – which was, and still is, a rigorous selection procedure with pass rates of around 35% in Part I and 40% in Part II. After attaining the Membership of the Royal College of Physicians (UK) qualification (MRCP) (which is regarded as an entry criterion to specialist training in medicine), two years were then spent as a registrar, during which time the doctor would usually be the most senior resident doctor in the hospital, being responsible for all admissions (and discharges) and referring to the non-resident consultant as required. Following this general training, there was usually a period of two years in a research laboratory in an area of specialist respiratory medicine, during which time a higher degree, MD (Doctor of Medicine) or PhD (Doctor of Philosophy), would be obtained by thesis. After this, there was a return to clinical medicine in a senior registrar post. This, like the registrar post, usually rotates on an annual basis between a variety of hospitals. At senior registrar level, they would typically rotate between teaching hospitals and postgraduate teaching specialist centres, which, in respiratory medicine, could be a postgraduate chest hospital, such as the Brompton or the London Chest Hospital. After three or four years as a senior registrar, the senior registrar would apply and be appointed as a consultant in general and respiratory medicine to a hospital trust which has advertised a vacant consultant post. From 1 January 1997, the training procedure altered. The training up to and including senior house officer and obtaining MRCP is similar, but, following this, there is a five year SPR appointment (during which time may or may not be taken out for performing research). If the trainee has performed satisfactorily, as judged by formal annual performance reviews during this appointment, in which there is experience in both general internal medicine and respiratory medicine, he or she will be awarded a Certificate of CCST. Appointment to the consultant grade is not allowed unless the appointee holds a CCST, whereas previously, accreditation was not essential for appointment to the consultant grade. SPRs are only allowed to start to apply, and be appointed to a consultant post, within the last three months of their SPR training. They are only allowed a prolongation of their contracts for six months once they have attained their CCST. Previously, there was in effect little pressure on senior registrars at the end of four years to obtain consultant posts. Although not the case in respiratory medicine, there have been timeexpired senior registrars in some specialities who were senior registrars for seven or more years. The overall effects of this new training scheme are unknown at present. There is the suggestion that in many specialities the consultants will be less well trained by having a shorter period of training than previously. There may
4
The Work of the Respiratory Physician well be the creation of a junior consultant post of, say, five years in order to give trainees more experience before they become senior consultants, perhaps at another hospital – which will, in effect, be what is currently understood to be a consultant post. Trainees in respiratory medicine, once accredited, not only fill general and respiratory medicine posts, but also, as part of their training, receive training in intensive care medicine and thus may become intensivists. They should also obtain training in HIV/AIDS-related illnesses and thus may become specialists in this field. They may take an interest in industrial medicine and become specialists in that field. As noted earlier, such is the volume of respiratory illness that not all respiratory medicine is managed by trainees or consultants who have been trained in this area. In a few trusts, respiratory medicine is offered by consultants (sometimes, specialists in elderly care) who have been registrars, but never senior registrars in respiratory medicine. They are practising respiratory medicine because there is no appointed respiratory specialist physician within the trust. In future, this tendency will cease because of the CCST, but, clearly, there are many years before this will come out of the system through retirements.
Typical working week for the respiratory physician The respiratory physician is normally responsible for two or three out-patient clinics per week, which are increasingly of a specialist nature, with GP referrals for problems such as breathlessness, cough, chest pain or abnormal GP chest X-rays. There is an increasing tendency to set up specialist clinics for asthma, chronic obstructive airways disease and lung cancer where there may be assistance from other groups of staff. In addition, there is usually a tuberculosis clinic and a tuberculosis contact tracing clinic, depending upon the prevalence of tuberculosis locally. This is high in London and other urban centres and low in more rural communities. The recommendation is that all cases of tuberculosis diagnosed in hospital (whichever organ is affected) should be managed by the respiratory physician. In addition to these clinics, the consultant will do two ward rounds a week on their own patients, accompanied by the team of doctors, who are supervised in the diagnosis and treatment of the patients. The consultant will be responsible for any respiratory referrals which have been made from other medical and surgical firms in the hospital. Practice varies from hospital to hospital regarding whether physicians who specialise in adult respiratory disorders will look after paediatric cases. Only a minority of respiratory physicians have paediatric training and experience other than in asthma and tuberculosis.
5
Respiratory Disorders The sixth or seventh fixed session of the week (there being 11 sessions in the week) is usually a fibreoptic bronchoscopy list (internal examination of the bronchial tree performed by the respiratory consultant and his trainees, in particular, to look for lung cancer).
Support in out-patients Staffing The support provided to the consultant for in-patients was described earlier – the support for out-patients is usually provided by the same team. A typical clinic will consist of the consultant, specialist registrar and, in addition, there may be a clinical assistant or hospital practitioner grade physician, often a local GP who has a long term commitment to and interest in respiratory medicine. There may be an associate specialist; a career grade doctor of lower rank than consultant, but nonetheless of considerable experience within respiratory medicine who is responsible ultimately to the consultant. There may also, although less satisfactorily, be a senior house officer undergoing professional training present in the clinic. There is a general trend to try and remove senior house officers or house physicians from the clinic, because they are relatively junior members of the team who realistically have less experience than the referring GP. Whereas their presence in the clinic may be useful, and indeed essential to their education in becoming an SPR and a consultant, their work should be closely supervised. They should be referring to the consultant for their decision making on further investigation or discharge. Clearly, in order for there to be a continuous service there are a large number of weeks of the year in which a consultant is in fact not present in his or her clinic. Indeed, a consultant could usually be thought to be present on only around 44 weeks of the year. The consultant is entitled to six weeks holiday. He will often be absent for two further weeks for study leave and postgraduate education and, usually, another two weeks for a variety of other professional commitments, such as appointment committees, giving lectures or examining. It is not usual practice for clinics to be totally cancelled when the consultant is away. The clinic continues in the consultant’s name with the SPR and clinical assistant present. (Similarly, the consultant does not have the support of the clinical assistant and SPR all the time: they also have leave.) However, it is accepted practice that problems which arise in the clinic when the SPR and clinical assistant are unsupervised are referred later to the consultant by the SPR or clinical assistant holding back the notes and X-rays to discuss with the consultant on his return.
6
The Work of the Respiratory Physician Other personnel increasingly available within out-patient clinics are respiratory nurse specialists who have a particular interest in managing patients with asthma and chronic obstructive airways disease and who deal with their nebulisers and manage home oxygen or other ventilators. There may well be palliative care (Macmillan) nurses caring for patients with lung cancer. Tuberculosis health visitors are responsible jointly to the consultant and the local consultant in communicable diseases for the treatment of and the contact tracing of patients with tuberculosis.
Investigational support for out-patients The minimum support expected for a chest clinic is the availability of chest radiology (immediate access) and lung function testing (at least simple spirometry and measurement of peak flow). More complex tests, such as measurement of gas transfer and lung volumes, may not be immediately available on site. Routine haematology and biochemistry investigations should be available, as should bacteriology for culture of sputum and cytology for examination of sputum for malignant cells. Bronchoscopy is available for internal examination of the bronchial tree and scanning using computerised tomography (with needle biopsy facilities) is also expected in most hospitals, although clearly the latter would not have been available until relatively recently and many hospitals have had to refer patients, particularly for computerised tomography (CT) scanning, elsewhere. Nuclear medicine lung scanning, to check for pulmonary emboli, may often only be available in larger centres to which patients need to be transferred for the test.
Follow-up in out-patients? There is an increasing trend for the hospital out-patients to be regarded as a one stop clinic, where a patient, once referred from a GP, will see the consultant who makes a diagnosis and provides advice at a single visit and refers back to the GP. The consultant may suggest or perform further investigation and inform the patient and the GP how the results of these can be obtained from the hospital rather than expecting the patient to come back up to the hospital to obtain the result of an investigation in person. The hospital consultant may start therapy or may make therapeutic suggestions for the GP to start. In any case, the maximum therapy usually prescribable from a hospital is now two weeks. There are important exceptions, however, to this rule of seeing once or twice and referring straight back to the GP (and not taking on longer term treatment and management).
7
Respiratory Disorders Examples of diseases for which the chest consultant would expect to take on chronic management include: (a) tuberculosis: patients and their contacts should be seen and the complete course of treatment given under the supervision of the hospital physician; (b) severe asthma: particularly in those patients on oral steroids (very few) or high dose inhaled steroids, and patients who have had severe life-threatening attacks or who have required intensive care in the past, or those in whom it is difficult to control symptoms; (c) lung cancer: care is often shared between GP and the hospital (oncologists and chest physicians); (d) sarcoidosis: lung function and regular X-ray follow-up are required; (e) lung fibrosis: lung function and regular X-ray follow-up are required; (f) industrial lung disease: complex follow-up is also required.
Referral to other specialists As far as diseases of the lung are concerned, there are common referral patterns to other specialists, particularly in the field of lung cancer. With the introduction of the Calman-Hine cancer proposals of local cancer units (usually in district general hospitals), which are allied to more specialist cancer centres (usually teaching or postgraduate hospitals), there is a nominated lead lung cancer specialist who will work with the other respiratory physicians and oncologists. There should be a policy for referral and treatment. Particularly, there is referral to a lung (thoracic) surgeon (usually also a cardiac surgeon), if the lung cancer is felt to be operable on certain accepted criteria, or referral to a cancer specialist (radiotherapist or oncologist), if the cancer is felt to be inoperable. For terminal lung cancer, referral is to the local palliative care team so that there can be support for the patient and their relatives at home. The patient may choose to die at home, or in a hospice or in hospital, during and following which the palliative care team will provide ongoing support for the patient and later the bereaved relatives. Typically, lung surgery and radiotherapy are not available other than in the Calman-Hine centre, but the Calman unit usually has visiting radiotherapists and/or oncologists who do clinics within the hospital and
8
The Work of the Respiratory Physician provide local referral and advice. Cancer chemotherapy is often performed under the guidance of the local lead lung cancer specialist or visiting oncologist within the local cancer unit, so that the patient does not have to travel to a cancer centre. There is also normally a combined meeting of the chest and oncology teams to discuss mutual patients and their care and to audit management on at least a monthly basis.
Professional societies and standards Within the UK, the professional society to which respiratory consultant physicians would be expected to belong is the British Thoracic Society (BTS). This holds professional educational meetings twice a year, one of three days, the other of two days, at least one of which the majority of consultants will try to attend. The British Thoracic Society has a Standards of Care Subcommittee from which guidelines are developed for the management of the most common respiratory medical conditions. In addition, many of the members of the British Thoracic Society will be members of the American Thoracic Society (which holds a very large annual meeting in the USA) and the European Respiratory Society (which also holds a large annual meeting). Each society publishes a journal containing original research and review papers: Thorax, the American Review of Respiratory Disease and the European Respiratory Journal, respectively.
Postgraduate continuing medical education for the trained physician The Royal Colleges of Physicians of London, Edinburgh and Glasgow have provided each consultant with a personal diary to collect information on their continuing medical education (CME). In the initial years of CME, there have been targets set of a total of 50 hours a year, this being an initial target which may subsequently be altered. The target has been set at 25 hours of CME within and 25 hours outside the trust. Activities which count towards internal CME include grand rounds, clinical and inter-disciplinary meetings, audit meetings, and hospital postgraduate meetings. External CME includes college-based educational events, specialist society or association meetings (such as the British Thoracic Society), international overseas meetings (such as the American Thoracic Society and the European Respiratory Society), national audit meetings, professional development courses, teaching and presentations at approved meetings, examining postgraduates or other CME-approved meetings or activities. A confidential 9
Respiratory Disorders annual return to the college has now started and targets will be adjusted accordingly. Many respiratory physicians will be increasingly personally responsible for the supervision and training of the specialist registrar grade (SPR) which will involve them in more education and training of their middle grade staff. This is likely to take them around half a day a week. Many respiratory physicians are involved in the teaching of undergraduates and postgraduates as well.
Management involvement An informal observation is that there is a greater input from respiratory consultant physicians into management posts (whether as clinical director or medical director) within their trust than than other specialities. Similarly, a higher percentage of consultant respiratory physicians hold merit awards and discretionary points than would be expected from the size of speciality.
Medical complaints procedure Each trust has a complaints procedure. There is an emphasis on complaints, wherever possible, being dealt with verbally and on ‘the shop floor’, but once they have been put in writing, there is an informal investigation and a reply from the chief executive. A formal procedure follows, if the complainant is still unhappy with this, with an internal inquiry where a trust board member leads the hearing. There are separate clinical complaints procedures outside the trust. None of these procedures, however, provide financial compensation for the complainant or their family.
Legal financial settlements All consultants working within an NHS trust are covered by their trust, which will conduct the defence of any clinical claim against a consultant. The consultant is, however, well advised to become a member of a defence organisation, such as the Medical Defence Union or the Medical Protection Society. This is to protect his own professional reputation. It may well be that the legal advisers to the trust wish to settle a matter cheaply to get rid of it, even though the consultant feels strongly that there has been no medical accident or negligence. Additionally, any consultant involved in private practice will have to depend upon his defence organisation and would be extremely ill advised not to be a member of such an organisation.
10
The Work of the Respiratory Physician Most trusts have insurance to lay off the local risks of claims and such policies now have rebates if the claims are lower than expected.
Source of respiratory experts for medical reports Instructing a respiratory physician – when? Most respiratory physicians will currently be practising both general and respiratory medicine. Thus, it may be appropriate to instruct them for either type of case.
General medicine Many potential medico-legal actions are taken against hospital physicians working as general physicians who are practising outside their immediate sphere of expertise. They have been acting as acute ‘on-take’ physicians, covering all medical emergencies. Examples include a patient with a heart attack being admitted under a gastroenterologist, or an epileptic or stroke patient being admitted or treated by a cardiologist. In any of these examples, it might have been a respiratory physician instead. Thus, it may be appropriate to instruct the respiratory physician to give a medical report in such cases when trying to obtain the views of a consultant physician on what is reasonable practice in acute non-selected ‘on-take’ medicine.
Respiratory medicine As far as specialist respiratory medicine is concerned, there are three areas where one may wish to instruct the expert respiratory physician: (a) respiratory medicine treated by the specialist respiratory consultant; (b) respiratory medicine treated by the non-respiratory specialist consultant physician, for example, an acute asthmatic treated in an Accident and Emergency department, or admitted under a general physician with a different specialist interest, such as gastroenterology; (c) respiratory medicine treated by the GP. Care should be taken, however, in the latter two categories – particularly in the last category: the hospital-based consultant commenting in areas of which he has little knowledge, that is, general practice. In all cases where a GP is being criticised for his standard of respiratory care of the patient, it is essential to get a GP specialist report as well. This may also necessarily be the case for respiratory medicine arising during acute unselected general medicine. It may
11
Respiratory Disorders be necessary, initially, to have a report from a respiratory physician expert, saying whether there were or were not shortcomings in management. If there were no shortcomings, then, clearly, there is no need for a further report. If there were shortcomings, however, it would be up to the non-specialist respiratory physician or GP, depending upon the complaint, as to whether these shortcomings were unacceptable at the level of the non-specialist practitioner.
What type of respiratory specialist? Within respiratory medicine there are ‘super specialists’ – those working within a very narrow specialised area of respiratory medicine who practice their specialist area of respiratory medicine to the exclusion of all other kinds of respiratory medicine and, certainly, practise no general medicine. Such specialists are likely to be working within postgraduate institutions, possibly holding academic posts. The specialist will give the ideal theoretical treatment and management of a specific condition and may be very useful for specific areas of expertise and prognosis, but may be too specialised if, for instance, you are looking for a report on the standard of asthma care being delivered by a gastroenterologist in a busy district general hospital. It is always appropriate to try to get a report from a specialist who has the working knowledge of the situation being criticised. It is useful to look for a specialist who has experience (preferably current) of teaching undergraduates and postgraduates and who is active in professional societies and who examines undergraduates and postgraduates, all of which are good signs of being up to date with current practice in the profession. Ideally, they should be of sufficient standing to have been elected to the Fellowship of the Royal College of Physicians. They should have experience in either the writing of textbooks or peer reviewed articles in journals. Previous experience in writing medical reports and attending conferences with counsel is essential. Previous appearances in court would be helpful; however, with the increasing number of cases that are settled out of court, a large number of experts do not have significant court experience. Membership of the Expert Witness Institute or being on the Law Society’s list of experts gives helpful guidance.
Practising or retired respiratory physicians? Providing expert medical reports for medico-legal purposes, attending conferences with counsel and court appearances are all time consuming for the increasingly busy hospital consultant.
12
The Work of the Respiratory Physician There is the temptation from the experienced, recently retired physician to enter the market and give medico-legal reports. Regrettably for this group of retired physicians, the practice of hospital medicine within the NHS moves on and changes with alarming speed and, whilst one can understand the appeal of such a hobby in retirement, such reports, although based on a wealth of previous professional experience, may not necessarily reflect current practice. With increasing age, there is a disinclination to be flexible in approach and, in particular, to accept that there are several views on a point. This, in turn, may mislead a solicitor and counsel (and raise false hopes in clients) and lead to a large waste of unnecessary time and expenditure. It is this author’s view that solicitors should instruct practising doctors, and cease to instruct, in the majority of cases, two to three years after cessation of active medical practice (after all, with many cases, it may be another few years before the case comes to court). This approach was also recommended by a legal speaker at an address given to the British Thoracic Society at a recent annual winter meeting.
Standard reference books The physician providing a report and instructing solicitors should have access to standard texts. In general medicine, examples of such are as follows.
Textbooks of general medicine Souhami, RL and Moxham, J (eds), Textbook of Medicine, 3rd edn, 1997, Edinburgh: Churchill Livingstone Kumar, P and Clark, M (eds), Clinical Medicine: A Textbook for Medical Students and Doctors, 3rd edn, 1994, London: Bailliere Tindall Weatherall, DJ, Ledingham, JGG and Warrell, DA (eds), Oxford Textbook of Medicine, 3rd edn, 1996, Oxford: OUP (in three volumes) Fauci, AS et al (eds), Harrison’s Principles of Internal Medicine, 14th edn, 1998, New York: McGraw-Hill, Health Professions Division
Standard reference for prescribing British Medical Association and Royal Pharmaceutical Society of Great Britain, The British National Formulary, twice yearly, London: British Medical Association and Royal Pharmaceutical Society of Great Britain For each drug, reference should be made to the manufacturer’s data sheet
13
Respiratory Disorders
Standard specialist reference books Seaton, A (ed), Crofton and Douglas’s Respiratory Diseases, 5th edn, 1997, Oxford: Blackwell Scientific Brewis, RAL et al (eds), Respiratory Medicine, 2nd edn, 1995, London: WB Saunders
Specialist journals on thoracic medicine Thorax Respiratory Medicine American Journal of Respiratory Diseases European Journal of Respiratory Diseases
General journals and sources The British Medical Journal The Lancet New England Journal of Medicine Medline Experts providing reports should have ready access to all of these sources and, in addition, an expert providing many reports would be assisted greatly by having access via the Internet to the BMA library to obtain Medline searches directly on line at home.
Guidelines and protocols The British Thoracic Society has developed and published, in the British Medical Journal, Thorax and the British Journal of Hospital Medicine, guidelines for the management of the common respiratory conditions which are available to all practising physicians. These cover asthma, chronic obstructive pulmonary disease, community-acquired pneumonia, tuberculosis and pulmonary embolism: these will be referred to later and references are included in the ‘Useful References’ section, pp 245–47. Many hospitals have developed their own protocols for the management of common medical conditions, usually based on the national guidelines. Whereas guidelines and protocols are useful in the management of patients who are sick with respiratory conditions, each patient should be regarded as an individual and there may be good reasons why there is deviation from such protocols or guidelines, a simple example being that the patient refuses the treatment!
14
The Work of the Respiratory Physician Whereas deviation from a guideline or protocol does not therefore mean that the patient’s doctor has been negligent, strict adherence to such a protocol or guideline would provide the practitioner with an extremely strong defence. One example of where guidelines can be useful in advising solicitors and their clients is in the area of asthma and treatment with steroids in particular. If it can be demonstrated that the patient’s doctor has adhered to the published guidelines, both solicitor and client can be satisfied that there can be no grounds for legal action.
Common types of problem over which clients wish to take advice as to whether to sue their doctor In general, a minority of cases in which a patient complains have a basis of true medical negligence, but there are a variety of reasons for which a patient may be dissatisfied with their doctor or the hospital. If they have not obtained satisfaction from the trust, they seek legal advice instead: (a) failure of communication: commonly, doctors have consultations with patients, and what the doctor thinks he says is not what the patient hears, partly due to the patient’s or relative’s expectations, partly due to their anxieties and the ability to be able to listen and absorb a large number of new facts at once. Common examples are with relatives of dying patients. The extremely poor outlook is often clearly documented in the notes and discussions about this and resuscitation status have been clearly noted. The patient dies and then the bereaved relatives complain that they were never told that the patient was so ill and dying and therefore there must have been a mistake in management; (b) early discharge: increasingly, there is pressure on acute hospital beds with patients being discharged home or elsewhere into the community earlier. If not handled sensitively, this can lead to anger amongst relatives and breakdown in communications between the hospital and the GP. Many such patients are elderly with a short expectation of life in any case and inevitably a certain number will be re-admitted or die shortly after being returned home. In these cases, there is always the feeling that the hospital has been negligent. This, of course, may or may not be the case, but the truth of the matter is that it will almost certainly be impossible to convince the relatives that the hospital was not negligent, whatever the outcome of any medical report, if death occurs soon after discharge;
15
Respiratory Disorders (c) non-admission: the patient is referred to a hospital and evaluated by the physicians, but not thought ill enough to be admitted. The patient returns home, deteriorates and dies; (d) straightforward errors: examples of these include: • misplacement of intercostal tubes, which, if placed too low, on the left may enter and perforate the spleen requiring a laparotomy and splenectomy, and on the right may be inserted into the liver, again causing a large amount of bleeding, necessitating transfusion and laparotomy; • biopsy of intercostal nerve instead of pleura; • injection of pelican ink – 1–2 ml of pelican ink instead of a single spot to mark the site of a Kveim test, leading to an extensive 1 x 2 cm tattoo; (e) unexpected deaths: the patient’s death is unexpected both to the physicians and the relatives, thus, there is the feeling that there must have been an error; (f) failure to diagnose and treat: such examples would be failure to diagnose and treat a lung cancer, observed on a routine chest X-ray done in another department for another reason, or failure to diagnose a deep vein thrombosis leading to a pulmonary embolus (blood clot on the lungs); (g) dissatisfaction with hospital treatment and attitude: a variety of factors – arguments with or attitude of ward staff, disagreements with ward sisters and nurses, failure to be fed, failure to be bathed – give an impression of less than ideal background or even negligent medical treatment and, thus, a variety of rather non-specific complaints are made. There is then a ‘fishing expedition’ as to whether there has been negligent treatment or not; (h) the ‘ghastly’ disease: the patient develops a very unpleasant disease, often at a young age, is incapacitated and unable to work, leading to either long term disability or death. The patient (or relatives) fails to ‘accept’ the disease, is angry – somebody has to be blamed for the disease (many of which have unknown causes) and, therefore, in a number of cases the medical profession is expected to be responsible. Thus, legal action is taken even though there is no substantial evidence for medical negligence. This group, of course, need especially sympathetic handling by the medical experts and the instructing solicitors.
16
The Work of the Respiratory Physician
Sources of experts Lists of experts may be obtained from the Action for Victims of Medical Accidents (AVMA), amongst other solicitors, especially those specialising in medical negligence, amongst barristers, other experts in the field, the British Academy of Experts, the Law Society Directory of Experts and Helpline, or from the Expert Witness Institute.
Availability of experts and instructions First, a letter should be written, checking on availability and willingness to provide an independent report, and asking for a quote for the client or, for legal aid purposes, the hourly rate and likely overall time such a report might take, giving an outline of the volume of material you expect the expert to have to read, the number of X-rays, and also giving an expected time course as to when a conference might be and when a court appearance (and its length) would be necessary, so that the expert can make time available at an early stage. In such an instructing letter, it is helpful to name any of the doctors against whom litigation may be taken so that the expert can advise whether these are personally known to him and, hence, whether he is able to provide a truly independent report. Once instructions have been accepted, experts will require the following material: (a) proof of evidence or witness statement; (b) other corroborative material; (c) supporting diaries and photographs; (d) full medical records: • GP including nurse records and co-operation cards (for example, asthma cards); • NHS hospital records; • private doctor and hospital records; • computer held records; • X-rays (all chest X-rays essential and scans, particularly ventilation perfusion lung scans and CT scans; • ECGs; • community records – district nurse, health visitors; • patient held records; • any records of complaints;
17
Respiratory Disorders • work records and occupational health records; • other expert witness reports; • full occupational history.
Suggested obligations of instructing solicitors In general, the instructing solicitor will help his expert if he: (a) supplies a clear letter of instruction; (b) provides paginated notes, in temporal order, separating GP from hospital records. A list of events and sequence made up by a nurse or other company on behalf of the solicitor is helpful, but needs to be checked; (c) ensures photocopies of all pages are legible ; (d) provides all chest X-rays, CT scans, ventilation perfusion scans and all notes at the beginning of proceedings. If these arrive some months later, the process may have to be started all over again, wasting time unnecessarily; (e) ideally, reads and comments and asks questions upon the physician’s report as soon as it arrives, so that the physician can remember all the details of the case. If these questions arrive three or four months later, the physician may have to start all over again, get notes out of store, look at the X-rays again, etc; (f) pays promptly; (g) keeps the physician informed of progress of case; (h) sends other experts’ reports and asks for comment; (i) sets conferences with counsel later in the day (5–7 pm), thus causing minimum disruption of clinical duties. Sending a map of counsel’s chambers and the name and the address of counsel is also helpful; (j) books the physician for court early; (k) cancels court appearances early. Clinical duties can rarely be reinstated at a day’s notice; (l) books or recommends a local hotel if the expert is a stranger to the town or city where the court hearing is to be held. Send a map of directions to the court and the exact time that any pre-meeting is required with counsel. A subpoena, whilst perhaps a blow to the professional pride of a witness, can in some cases be a useful tool which the expert can use with his or her trust in order to be released from clinical duties; (m)notifies the expert of the outcome, including: • the amount of the settlement; • instructions as to what should be done with the notes and X-rays;
18
The Work of the Respiratory Physician • if proceedings are discontinued, this should be notified and, similarly, instructions as to what should be done with notes and X-rays should be provided; (n) finally, most experts appreciate overall feedback on the quality of their report, the relative expense, etc. Such forms are often returned to AVMA and feedback to the expert is commonly appreciated.
Obligation of respiratory physician to instructing solicitors Respiratory physicians instructed by solicitors should: (a) read the letter of instruction carefully; (b) consider whether they are of comparable expertise to the physician being complained of; (c) consider whether they know any of those doctors involved well enough to put their independence in doubt. Physicians should not be tempted by the instruction letter which seems to outline a superficial complaint which can easily be rejected, because once notes are received a much more serious problem of negligence may be discovered. (d) be independent, regard themselves as having a duty to the court and capable of giving an identical report whether acting for the plaintiff or the defence; (e) read everything. Often, the act of omission/negligence is completely different from the one being complained about and, frequently, the key evidence is hidden away in one page of the pathology reports. Physicians should not assume they have been supplied with all pathology reports or X-rays; (f) explain the strengths and weaknesses of each case; (g) explain these to the client and instructing solicitor. It may be unfair to hide behind the instructing solicitor to have to explain what the strengths and weaknesses of the case are when these are medical matters which the physician is better qualified to discuss and explain to the patient than the instructing solicitor; (h) always see the patient involved (unless they are dead or brain injured and incapable of giving a history and being examined). Physicians should not rely solely upon the reports and examinations of other experts; (i) if current therapy is not ideal and improvement can be obtained or the physician is worried about the current medical condition or treatment, he should say so and recommend what he considers more appropriate. He should recommend that the solicitor suggests the client goes to see their GP, if necessary, with the part of physician’s report which contains
19
Respiratory Disorders suggestions, or with a separate side letter which deals with any active medical problems which you consider the GP needs to act upon. Physicians should not interfere directly with treatment; (j) not hesitate to recommend other suitable experts if their own expertise does not cover the entire complexity of the case – frequently, it will not. Examples include the need for a consultant radiologist to report on X-rays and scans. Physicians may also need other experts, such as a haematologist, if blood clotting disorders are present. Intensivists may be needed to cover intensive therapy unit (ITU) aspects of care. (k) be willing to be flexible if new evidence, more notes and more X-rays or other experts’ reports become available; they should be willing to consider their position and alter it if it genuinely needs to be altered. Physicians should resist undue pressure, should it be applied, from well meaning solicitors or barristers, to alter their reports, especially if their independence may be jeopardised.
20
CHAPTER 2
STRUCTURE AND FUNCTION OF THE LUNGS AND LUNG FUNCTION TESTING
Figure 2.1: surface anatomy of the lungs
ANATOMY
21
Respiratory Disorders
The lungs There are two lungs, one on each side. The left one has two lobes, the lower lobe and the upper lobe, part of the upper lobe being known as the lingula (Latin – tongue), a tongue-like part of the lung which comes in front of the heart. On the right hand side there are three lobes, the upper, middle and lower lobes, each supplied with their own bronchus (tube leading into the lung). The right lung constitutes approximately 55% of the total lung volume, the left lung 45%. It is smaller because the heart is almost invariably situated in the left side of the chest and occupies the rest of the space that the lung would otherwise occupy; the exception being with dextrocardia, when the side of the organs is reversed – a very rare situation.
The mediastinum The lungs are separated from one another anatomically by a compartment known as the mediastinum. This central compartment contains the heart, the great vessels (the aorta, the superior and inferior venae cavae), the trachea (windpipe), lymph nodes, oesophagus (gullet), phrenic nerve, vagus nerve and the thymus gland. Each lung is covered by a smooth film-like layer of tissue known as the pleura.
The pleura There are two layers of pleura, one on the inner surface of the chest wall, the other on the outer surface of the lung, the visceral and parietal pleura respectively. These two surfaces provide low friction surfaces to enable the lungs to expand and contract easily with as little friction as possible. In health, there is a microscopic layer of fluid between the two layers of pleura, this fluid constantly being secreted and re-absorbed. If this mechanism of re-absorption should become disturbed in any way, such as blockage of the lymphatic drainage system, then fluid will accumulate within the pleural space (sometimes many litres on a side) causing what is known as a pleural effusion. The two layers of pleura on each side are actually connected to one another in the midline so that one can visualise the pleura being a balloon into which a fist has been pressed – the fists being the lungs. Thus, the pleural space in health represents a potential rather than an actual space. The possibility of fluid collecting in this space has already been mentioned. There is also the potential for air to escape from the lung and collect in this space, causing what is termed a pneumothorax. If blood collects in this space, for a variety of pathological reasons, this is known as a haemothorax, and if pus collects in the pleural space, the term used is empyema. Rarely, lymph can collect (chylothorax).
22
Structure and Function of the Lungs and Lung Function Testing Figure 2.2: bronchial tree
Bronchial tree Air enters the lungs through either the mouth or nose (which join posteriorly in the pharynx). Air then passes through the larynx (voice box), down the windpipe (trachea) in the midline and then enters the right and left lungs respectively at the carina, the point at which the windpipe branches into the right and left main bronchi. These then each subdivide into an ever-increasing number of branches of smaller bronchi, each branch being a smaller diameter, until the air is distributed into a small sac, the alveolus (at the end of the respiratory bronchioles), where gas exchange occurs. The cut surface of each lung looks like a sponge because of these alveoli. If the total surface area of the lung were to be laid out, each lung would cover approximately half a tennis court – 23
Respiratory Disorders which gives some idea of the way in which the lung acts as an amplification system to increase the surface area over which gas exchange can occur.
Chest wall The chest wall is rigid by virtue of the presence of the ribs (12 on each side) which are connected posteriorly to the vertebral column and anteriorly to costal cartilage (lower ribs) or to the sternum (breast bone) – upper ribs. The ribs slant downwards from posterior to anterior and are capable of moving upwards a small amount by virtue of the movement caused by the contraction of the intercostal muscles, which by bringing the ribs into a more horizontal position will have the effect of increasing the chest diameter and hence circumference during inspiration and decreasing it during expiration (relaxation of muscles). Positioned under the lungs and attached to the lower ribs and vertebrae is the main muscle of respiration, the diaphragm, which moves upwards and downwards with each breath. A downward movement causes the abdomen to push out and the chest to expand and lungs to inflate. Whilst men primarily use diaphragmatic breathing during quiet respiration, women tend to use chest wall movement.
Nerve supply The nerve supply to each half of the diaphragm comes from the phrenic nerve on that side. These leave the spinal cord in the middle of the neck at the third, fourth and fifth cervical nerve roots, whereas the intercostal nerves supplying the intercostal muscles leave the spinal cord on both sides through the intervertebral foramina (holes) at the same level as the intercostal muscle supplied (T1–T12). The nerves run in a groove underneath the ribs together with the intercostal vein and artery, the vein being uppermost, then the artery, and below that the nerve. This groove is underneath and towards the inside of the chest and thus a direct injury with a needle through an intercostal space is unlikely to penetrate any of these structures in usual medical practice. The phrenic nerve has a long course through the neck down through the midline structures of the chest (the mediastinum) and then fans out onto the diaphragm. This long course means that pathology within the neck or mediastinum may affect the nerve function and cause paralysis of one of the phrenic nerves. The most common example would be a lung cancer involving the mediastinal lymph nodes by metastatic spread, causing pressure on or invasion of the phrenic nerve on one side and causing diaphragmatic paralysis on that side. The clinical relevance of the phrenic nerve origin being in the middle of the neck is that with spinal cord injuries (such as a car crash) at any level 24
Structure and Function of the Lungs and Lung Function Testing below C5 the phrenic nerves will be intact and, therefore, spontaneous respiration will continue. With fractures above the level of the origin of the phrenic nerve (C3) spontaneous respiration is impaired and assisted ventilation is required.
Purpose of respiration All living cells in the body require a supply of oxygen in order to function properly; without this cell death occurs. The by-product of the process of respiration is carbon dioxide. Oxygen (O2) is delivered to the body’s cells by being attached to haemoglobin within the circulating red blood cells. As oxygen is relatively insoluble, very little is freely dissolved. Carbon dioxide (CO2) is largely dissolved in the plasma and transported away from the cells by the blood stream. The lung is the organ of the body where oxygen is absorbed from the air and carbon dioxide is excreted into the air. Room air contains 20% oxygen and very little CO2. The blood supply to the lungs comes from the right side of the heart via the pulmonary artery; it is low in oxygen because the oxygen has been extracted for use by the rest of the body and is blue in colour. During its passage through the lungs, oxygen is attracted to the red blood cells and the red oxygenated blood passes through the pulmonary veins into the left side of the heart through the left atrium and left ventricle, to be pumped out through the aorta and distributed throughout the body. Oxygen is then absorbed by the cells of the body and the blood becomes deoxygenated. This blood (blue) passes via the venous system back into the right side of the heart, either via the inferior or superior vena cavas, and passes through the right atrium, right ventricle, and out into the lungs again; hence one complete circulation of the blood has occurred – usually within about a minute. Carbon dioxide, the waste product of tissue respiration, is produced in the tissues and then dissolves readily in the blood plasma. Thus, the deoxygenated blood from the tissues going back to the right side of the heart is richer in carbon dioxide. This carbon dioxide diffuses across the alveolar wall from the respiratory capillaries into the alveoli and is exhaled.
Tests of respiratory function Introduction A large number of respiratory function tests have been developed and used over many years. These have ranged from the entirely clinical and non-numerical: the simplest of these are observing whether the patient can speak full sentences 25
Respiratory Disorders without having to stop to inhale, or the distance from the mouth at which a lighted match can be blown out. Other useful simple observational tests which are still used are the distance that can be walked on the flat – this can be expressed as an absolute distance over a defined period of time, such as a six minute or a 12 minute walking test on the flat, or the number of stairs which can be climbed before the patient has to stop.
Peak expiratory flow rate The simplest objective measurement of respiratory function is the peak expiratory flow rate (PEFR), commonly referred to as peak flow. The instrument used, a peak flow meter, measures the maximum flow rate at the beginning of a forced expiration. This measurement depends upon (a) effort by the patient, and (b) the diameter of the large airways. The normal or reference value depends upon the age, sex, height and race of the patient. The normal value for a 6 ft, 25 year old Caucasian male is of the order of 650 litres/minute. For a 5 ft 6 in, Caucasian female in her 30s, should be around 450–500 litres/minute. Figure 2.3: normal valves for peak expiratory flow rates according to age, sex and height
26
Structure and Function of the Lungs and Lung Function Testing The peak flow rate is reduced in conditions affecting the diameter (and hence resistance) of the large air passages into the lungs. Typical examples include asthma, where the air passages constrict in attacks and dilate between them. Thus the peak flow rate may vary considerably from hour to hour or day to day. The peak flow is also reduced in diseases such as chronic bronchitis (chronic obstructive airways disease), where there is progressive narrowing of the air passages with increasing years of cigarette smoking; here, there is little (if any) variability. These measurements can be done very easily and not only should all GPs own a peak flow meter, they should prescribe them for suitable patients. Asthmatics can be prescribed mini peak flow meters that cost around £7.50 on an FP10 prescription to help them monitor their asthma at home. It is expected normal good practice that any asthmatic or patient with chronic obstructive airways disease, who is being followed by their GP or hospital chest clinic, has their peak flow recorded at each assessment of their chest.
Spirometry Ideally, all GPs should have a spirometer in order to measure the forced expiratory volume in the first second – FEV1 – and forced vital capacity – FVC. The FEV1 is the volume of air that can be expired in the first second of a forced expiration following a full inhalation of air. The FVC is the total volume of air that can be exhaled from a full inspiration to a forced expiration. Both these values depend upon age, sex, height and race of the patients. The FEV1 should in health be more than 80% of the FVC. Anything less than this implies that there is a degree of airways narrowing, such as is found in asthma or chronic bronchitis.
27
Respiratory Disorders
Patterns of disorder Figure 2.4: spirogram tracings obtained from a vitograph
28
Structure and Function of the Lungs and Lung Function Testing Figure 2.5: typical patterns of abnormal lung function tests
There are two broad patterns of disorder of respiratory function. They concern the diseases which affect the calibre of airways and cause narrowing of the air passages (obstructive airways disease): first, where the narrowing is variable (reversible) – asthma – and secondly where it is progressive and irreversible – chronic obstructive airways disease (chronic bronchitis). In both conditions the FEV 1 is reduced and the FEV 1 as a percentage of FVC is reduced. However, the absolute FVC may be normal or reduced.
29
Respiratory Disorders Other lung conditions which primarily affect the overall size (volume) of the lungs cause what are known as restrictive defects. In these, the problem is one of shrinkage of the lung and loss of lung volume rather than narrowing of the airways. Hence, the FVC is decreased, and because the airways are not affected, the FEV1, although reduced in absolute terms, is not reduced in proportion to the vital capacity. Hence, the FEV1/FVC ratio is normal or may in fact be increased. Examples of restrictive diseases are pulmonary fibrosis from causes such as cryptogenic fibrosing alveolitis or asbestosis. Conditions such as when there is an accumulation of fluid around the lung (pleural effusions) also cause such a restrictive pattern. One expects the availability of spirometry in all chest clinics and would hope that it is available in most GPs’ surgeries, particularly with the advent of smaller, often hand held microspirometers. However, the reality of the situation is that spirometers are often only found in hospital chest clinics and wards.
Gas transfer – diffusing capacity (DLCO) Gas transfer is a measure of the efficiency by which the lungs can absorb oxygen from air into the blood stream. It is measured by using carbon monoxide as a tracer and substitute for oxygen. It is calculated as the ability of the lungs to absorb an amount of carbon monoxide inhaled into the lungs over a fixed period of time (a 10 second breath hold), and is measured by means of a respirometer. The amount of carbon monoxide absorbed depends upon: (a) the concentration of haemoglobin in the blood; (b) the surface area of the lung available for the transfer; (c) the thickness of alveolar barrier between the blood steam and the inhaled gas; (d) the overall lung volume. Gas transfer can only be measured in a hospital respiratory function laboratory using complex machinery and is not available in any GP’s surgery. Conditions where the gas transfer is decreased include anaemia, pulmonary fibrosis (from any cause), and emphysema. The gas transfer is increased when the level of haemoglobin is increased in a rare blood condition known as polycythaemia.
30
Structure and Function of the Lungs and Lung Function Testing
Overall lung size – total lung capacity (TLC) Lung volumes may be measured in two ways. First, by helium dilution, where a known volume (V1) and concentration (C1) of helium is inhaled into the lungs. When equilibrium is achieved by dilution of the original helium throughout the lungs, the new concentration of exhaled helium is measured (C2). By the formula V1 x C1 = V2 x C2, because C1 and C2 are measured and because V1, the original volume of helium is known, V2 can be calculated. Thus, the lung volumes can be calculated. V2 being the original volume of inhaled gas (V1) plus the volume of the lungs which helium has reached. Hence, V2 - V1 = lung volume (TLC). The second method of measuring lung volumes is in a closed chamber, a body plethysmograph (body box). By employing the principles of Boyle’s law (P1V1=P2V2), where three of the factors in the equation are known (or can be measured), the volume of the patient’s lungs can be calculated. The lung volumes are reduced in patients with lung fibrosis, surgical removal of a lobe or lung or pleural effusions (fluid around the lung). Helium dilution methods of lung volumes are available in most chest clinic respiratory laboratories, but body plethysmography is more rarely available, usually, only in teaching hospitals.
Histamine challenge test Histamine is a potent constrictor of air passages and, if a subject inhales a dilute solution of histamine under carefully controlled conditions, they will bronchoconstrict. Depending upon the concentration at which the bronchoconstriction occurs, a spectrum of responses can be observed from those who bronchoconstrict with a very dilute concentration – asthmatics – to those who only bronchoconstrict with a relatively more concentrated solution – normal. The standard response is taken as the concentration to cause a fall of 20% in the FEV1. In between, there is a group of patients who are said to have irritable or hypersensitive airways. This test is not routinely available and should only be done in centres with specialist expertise and the ability to treat the bronchoconstriction should it occur. It should not be carried out on patients who have proven airways obstruction or known asthmatics, because of the risks of precipitating severe airways obstruction. Methacholine may be used in place of histamine.
31
Respiratory Disorders
Allergy skin tests In order to determine the substances to which an asthmatic is allergic – such as house dust mites, grass pollen, tree pollen or animal fur – a dilute solution of the particular allergen is placed as a droplet on the forearm of the individual and then a single prick with a needle is made through the droplet. After 20 minutes, the area of redness and central raised white area, the ‘wheal and flare response’, is measured and noted. Figure 2.6: forearm showing positive allergy skin prick tests
If the individual is not allergic, no response will be seen. In a very allergic individual, a large central wheal will be seen with an even bigger flare. There is no precise correlation between skin tests and bronchial responsiveness, but they are taken as indirect guides and are used by the physician in advising the asthmatic patient as to what alterations to their lifestyle may be necessary. 32
Structure and Function of the Lungs and Lung Function Testing
Other respiratory tests Blood gases The normal blood tests performed in a GP’s surgery or hospital to test haematological and biochemical values are taken from a patient’s veins. Blood gas measurement is made from a sample taken from the patient’s artery in a heparinised syringe (to prevent the blood clotting). The taking of such a sample is painful because of the nerves running in the arterial wall and, thus, is not unduly popular with the patient. The samples are normally taken from the radial artery at the wrist. However, obtaining blood gas samples in order to measure both the partial pressure of oxygen (PaO2) and the partial pressure of carbon dioxide (PaCO2), together with the calculations of the oxygen saturation measurement and the acidity of the blood (pH, bicarbonate and base excess), give good estimations of the adequacy of respiration and tissue perfusion and acid base balance in the body. They are essential in the management of the critically ill patient. Figure 2.7: typical patterns of blood gas abnormalities
33
Respiratory Disorders The PaO2 depends primarily upon the ventilation and perfusion matching within the lungs. The P aCO 2 depends primarily upon the adequacy of ventilation and only to a lesser extent on ventilation perfusion matching. Figure 2.8: oxyhaemoglobin dissociation curve
The oxygen saturation of the blood depends upon the PaO2 but there is not a linear relationship because of the shape of the haemoglobin dissociation curve, which is sigmoid in shape. Thus, below a certain level of PaO2, a small drop results in a very large fall in oxygen saturation, whereas, up to this point, quite a large drop in PaO2 will not cause a very significant drop in oxygen saturation (and, hence, oxygen delivery to the body’s tissues).
34
Figure 2.9: report form for blood gas analysis showing normal ranges and shifts with acid base disturbances Structure and Function of the Lungs and Lung Function Testing
35
Respiratory Disorders Acid base balance (pH) depends not only upon respiration and the elimination of carbon dioxide, but also upon renal function. In renal failure, there is also acidosis. Low pH (acidosis) can be caused by other conditions, such as diabetes; pH is also affected by poor tissue perfusion and severe infection. In normal health, the body’s pH is kept between very tightly regulated limits. Arterial blood gases are not available in GPs’ surgeries. However, they are obtainable in all hospitals and, ideally, should be assessed in all patients with severe respiratory illness who require hospitalisation. They are monitored regularly when patients are in intensive care units (often by an arterial catheter being inserted to allow for repetitive sampling).
Oxygen saturation Oxygen saturation may be measured swiftly and simply using oximetry, in which a photoelectrocell ‘clip’ is placed onto a patient’s finger. This clip contains a light which shines through the finger tip and an estimation of oxygen saturation is made. This is a simple method, used increasingly as a non-invasive way of measuring patient’s oxygenation indirectly as an alternative (but second best) to blood gas (PaO2) estimation. The main limitations to this technique are: first, that because of the shape of the oxygen dissociation curve, oxygen saturation is maintained relatively well until there has been quite a large drop in PaO2, and then, beyond this, there is a sudden precipitous drop in oxygen saturation, with a relatively small drop in PaO2. Secondly, it gives no idea of the adequacy of ventilation, because there can be an increase in PaCO2 which is not picked up at all, as only oxygen saturation is measured. Oximetry is also limited in situations where there is poor tissue perfusion, such as shock, or any other conditions with low blood pressure. Nonetheless, it is a very useful way of monitoring oxygenation non-invasively such as an asthmatic during an attack.
Medico-legal points Failure to perform or act on the appropriate test To all intents and purposes it is the peak flow rate which is the everyday measurement carried out in clinical practice on patients with the common respiratory diseases, such as asthma and chronic bronchitis. From a medicolegal point of view, the situations where GPs and hospitals can be criticised are, usually, where there has been a failure to perform any or sufficient objective measurements of respiratory function in asthmatics and, from this, the following criticisms might be made:
36
Structure and Function of the Lungs and Lung Function Testing (a) failure to assess the severity of an attack; (b) failure of monitoring, so that there is no objective evidence of improvement or deterioration in respiratory function in response to therapy; or (c) alternatively, if a measurement has been performed, there may be failure to act upon the reading or failure to appreciate the severity of an asthmatic attack, occasionally, regrettably, with fatal consequences. The national guidelines for the management of asthma use the peak flow rate as the criterion for management and reference values.
Failure to perform the correct respiratory function test In rare neuromuscular diseases, such as myasthenia gravis, there is variable and, occasionally, increasing weakness of the respiratory muscles. It is essential (and standard respiratory practice) to gauge the adequacy of respiration by the ability to perform the forced vital capacity manoeuvre by spirometry (FEV1, FVC). Vital capacity should be measured at least daily to check that there is no respiratory embarrassment. Failure to do this may lead to unrecognised respiratory failure, hypoventilation and death. The purpose of measuring respiratory function is to recognise these difficulties and artificially ventilate the patient if necessary.
Failure to measure oxygen status An assessment of oxygen status is essential in all patients being admitted to hospital with severe respiratory diseases such as pneumonia, severe asthma attacks, or exacerbations of chronic obstructive airways disease. This should be either by oximetry or by arterial blood gases. Failure to do either test means failure to assess the severity of the patient’s condition adequately. Regrettably, this error may prove fatal.
37
CHAPTER 3
COMMON CHEST SYMPTOMS AND THEIR INVESTIGATION
CLINICAL APPROACH Chest diseases present with a limited number of symptoms. It is, therefore, essential that the chest or general physician is vigilant in knowing the potential significance of these symptoms; their cause may range from minor everyday illness to the much more sinister or rare condition. It is self-evident that it is unreasonable to expect physicians to be able to arrive at rare diagnoses immediately upon the presentation of very common symptoms, since, in reality, some of these rare diagnoses may be a ‘once in a lifetime’ experience for the physician. Nevertheless, it is vital that there is a well laid out, methodical approach to investigation of symptoms, so that, by a process of elimination, the correct diagnosis is achieved. The most common symptoms on presentation to the chest clinic are: • cough – with or without sputum; • coughing of blood (haemoptysis); • shortness of breath (dyspnoea) – with or without wheezing; • chest pains; • abnormal chest X-ray, taken either for routine purposes or by the GP because of symptoms such as those above.
INVESTIGATION OF SYMPTOMS AND GENERAL POINTS It is necessary to go through the time honoured routine of: • clinical history; • physical examination; • further investigations. Although, in undergraduate medical textbooks, the importance of taking a full history and doing a full examination is stressed, routine clinical practice in a busy chest clinic rarely gives time for this complete procedure (which is likely to take around 45 minutes per patient). The time allotted to a new patient in these circumstances may well be only 15 minutes. There are, therefore, inevitably shortcuts which the experienced consultant physician takes. These are clearly acceptable when the diagnosis is made correctly and the patient given a good standard of treatment. They become more questionable and less justifiable if a diagnosis is not made. It is with experience that the physician 39
Respiratory Disorders should be able to select those patients who can be ‘fast tracked’ and those in whom a full history needs to be taken. It is usual, however, in a chest clinic, to concentrate more upon questions related to the chest and respiratory system and less (or not at all) on questions of the abdominal and urinogenital systems, for example. Similarly, the examination is concentrated on the chest (lungs and heart) and it would be unusual for a full neurological examination to be performed unless there were specific neurological symptoms. Internal examinations, such as rectal examination, would not be routinely performed. Nonetheless, it is extremely important that a careful history is obtained from the patient because often the diagnosis can be made from this alone. Not only is the history important to make the diagnosis, but also to discern how incapacitated the patient is by their symptoms and the extent to which the quality of their life is affected. Physical examination confirms or refutes the possibilities suggested by the clinical history, and simple investigations, particularly a chest X-ray and pulmonary function tests, support the history and examination. Figure 3.1: normal chest X-ray (postero-anterior)
40
Common Chest Symptoms and their Investigation Figure 3.2: diagram of a postero-anterior chest X-ray
a = trachea
e = left atrial appendage
b = aortic arch
f = right atrium
c = superior vena cava
g = left ventricle
d = pulmonary artery
h = diaphragms – right 2 cm higher than left
i = hilar shadows consisting of pulmonary arteries and veins and lymph nodes j = lung fields k = costophrenic angles
In some cases, it is essential to perform more complex investigations, such as CT scanning, bronchoscopy or needle biopsy of lung tumours seen on X-ray or scanning. A peculiarity of hospital-based chest medicine is that often the patient is referred to the out-patient clinic because of an abnormal chest X-ray performed by the GP and from this X-ray alone the diagnosis may be selfevident. In these cases, although the presenting history may be taken very briefly, there will be careful questioning as to how the patient’s disease is affecting their life and that of their family. In particular, with malignant disease, there will be questioning concerning the support the patient has at home. 41
Respiratory Disorders Before considering specific presenting features, there are general points which cover the diagnosis of lung disease. These of course are not absolute guides, but are useful.
Age In younger patients (up to 30 years) Common diseases include: • atopic (allergic) asthma; • pneumothorax; • sarcoidosis. Rarer diseases include: • cystic fibrosis; • congenital heart disease.
In older patients (over 50 years of age) Common diseases include: • intrinsic asthma; • chronic bronchitis and emphysema; • ischaemic heart disease (angina, heart attacks); • lung cancer. Rarer diseases include: • cryptogenic fibrosing alveolitis; • pneumoconiosis.
Occupation A full occupational history for past and present is essential; in particular, exposure to dusts or chemicals should be considered. Occupations specifically at risk are: • coal miners; • farmers; • asbestos workers (including anyone in the building industry); • workers with epoxy resins or isocyanates.
Racial origin Immigrants, especially those from Asia or Central Africa, are particularly at risk from tuberculosis, especially during the first two or three years after 42
Common Chest Symptoms and their Investigation entering the UK. In addition, HIV infection is particularly prevalent in Central Africa.
Drug addiction Intravenous drug abusers and patients who have had blood transfusions are at higher risk from HIV infection than the rest of the population.
Sexual orientation It is important to ask patients of their risk of HIV infection. Although more common in the gay community, the heterosexual population is also at risk, particularly if coming from Central Africa.
Smoking history Lung cancer, chronic obstructive pulmonary disease and ischaemic heart disease are all more common in patients who smoke.
TAKING THE PATIENT’S HISTORY In addition to the specific complaints, other information is essential.
Past medical history Details of the following are very important: • previous illnesses and operations to include all hospitalisation, or cancer therapy; • previous chest X-rays. The practitioner should discover why X-rays were made of the chest, when and where. A previous chest X-ray from several years earlier may save a lot of unnecessary investigation; • previous abnormalities found on medical examinations; • history of hay fever, asthma and other allergies; • previous tuberculosis or contact with tuberculosis; • any history of venous thromboses.
Family history The practitioner should find out whether there is a history of any other chest disease in the family, or any other problems related to respiratory disorders, such as: 43
Respiratory Disorders • • • • •
tuberculosis; asthma; cystic fibrosis; emphysema at a young age – alpha 1 antitrypsin deficiency; clotting disorders.
Social history The practitioner needs to get information about the patient’s lifestyle, such as: • smoking; • alcohol consumption; • country of origin (length of time in the UK); • living accommodation – house or flat, number of stairs; • number of children; • presence of pets – cats and dogs may aggravate asthma. Pigeons and budgerigars may cause pulmonary fibrosis. Parrots may cause pneumonia; • the patient’s hobbies; • recent overseas travel or contact with infectious diseases.
Current medications and any recent changes Particular drugs may be risk factors for respiratory diseases: • the oral contraceptive pill and HRT – venous thrombosis and pulmonary embolism; • beta blockers (including eye drops) – airways obstruction and asthma; • ACE inhibitors – chronic cough; • steroid therapy – osteoporosis and vertebral collapse.
Allergies All known allergies should be recorded, not only because of asthma, but also because of drug reactions.
PRESENTING SYMPTOMS With all common presenting symptoms, it is first important to get the full history of the presenting illness to ascertain when the patient was last completely well and whether any similar illness has occurred in the past. The 44
Common Chest Symptoms and their Investigation main triad of respiratory symptoms – shortness of breath, chest pain and cough– should be asked for specifically.
COUGH The following points should receive attention when the history is being taken: • how long the cough has been present? Extensive investigation is not warranted in the first two weeks of a cough in an otherwise well patient – only if it persists; • when it occurs, day or night, or both? Most coughs are worse at night or first thing in the morning; • is the cough persistent or intermittent? • is it painful? • is there any possibility of foreign body inhalation? • is there sputum? If so what colour and volume? • is there blood in the sputum? • is there associated breathlessness or chest pain?
Common causes of cough Acute cough – less than two weeks A wide range of causes need to be considered, such as: • tracheitis – viral or bacterial; • acute bronchitis – viral or bacterial; • bronchiolitis – viral or bacterial; • pneumonia – viral or bacterial; • asthma; • pulmonary oedema; • pulmonary embolism; • whooping cough; • foreign body inhalation; • onset of chronic cough.
Chronic cough – more than two weeks Again, a wide range of causes need to be considered: • chronic obstructive airways disease (chronic bronchitis and emphysema); 45
Respiratory Disorders • • • • • • • • • •
asthma; lung cancer; tuberculosis; interstitial lung diseases; bronchiectasis; foreign body inhalation; benign tumours; whooping cough; mediastinal lesions, lymph nodes, aortic aneurysm; habit cough.
Minimum acceptable standard for investigation of a chronic cough Proper investigation of any patient complaining of a cough should cover the following points: • a full history; • examination of the chest; • PA chest X-ray; • measurement of lung function – spirometry ideal, peak flow acceptable (but less informative); • if there is sputum production – culture and sensitivity. In high risk areas for study for tuberculosis – if there is any suspicion of possibility of bronchial carcinoma, the sputum should be sent for examination for neoplastic cells.
HAEMOPTYSIS (COUGHING OF BLOOD) Although most patients who cough up blood and consult their doctor are worried about the possibility of lung cancer, 3% or less with this symptom have cancer. Nonetheless, it is a very important symptom and needs to be specifically asked for from the patient, and when detected needs careful investigation. The following points can be made: (a) it is important to check that the blood is actually coming from the chest and is being coughed up. It is frequent for patients to confuse vomiting of blood with coughing of blood and also to confuse blood coming from the nose or postnasal space with coughing of blood; (b) despite careful investigation, haemoptysis will remain unexplained in up to 50% of patients.
46
Figure 3.3: clinical features of common causes of haemoptysis
Common Chest Symptoms and their Investigation
47
Respiratory Disorders Common causes of haemoptysis which should be considered are: • acute tracheobronchitis; • pneumonia; • lung cancer; • tuberculosis; • pulmonary infarction (pulmonary emboli); • bronchiectasis. Rarer causes of haemoptysis include: • lung abscess; • aspergilloma; • apical fibrosis; • foreign body inhalation; • benign tumours; • rare vascular disorders such as hereditary haemorrhagic telangiectasia; • Wegener’s granulomatosis; • Goodpasture’s syndrome. Non-pulmonary causes of haemoptysis include: • left ventricular failure; • mitral valve disease; • blood disorders – leukaemia.
Minimum acceptable investigation for patients with haemoptysis Proper investigation of any patient complaining of haemoptysis should cover the following points: • history and examination; • PA chest X-ray to exclude more sinister pathology; • sputum (if present) – culture and sensitivity and tuberculosis studies; • in middle aged smokers, it is advisable to send the sputum for neoplastic cytology. If the chest X-ray is abnormal, referral to the chest clinic is mandatory and investigation of the abnormality carried out until there is a definitive diagnosis. This may well involve: (a) bronchoscopy; (b) CT scanning; (c) percutaneous needle biopsy of any mass lesion.
48
Common Chest Symptoms and their Investigation There is usually little problem with investigation and diagnosis if the chest Xray is abnormal. There is usually a larger area of concern from the physician’s point of view (and possibly the widest areas of clinical variation) in the investigation of a patient with haemoptysis with a normal chest X-ray. In the younger patient (that is, under 40) who is a non-smoker and in whom the haemoptysis is a single event, with a normal chest X-ray when the haemoptysis has occurred at the time of an acute respiratory infection, not referring the patient to the chest clinic could be justified. However, if the symptoms recur, a further chest X-ray and referral would be mandatory. In the majority of patients however referral to the chest clinic is warranted. There is always the anxiety that a single episode of haemoptysis may herald a malignant lesion that is too small to see on a chest X-ray. There is a limit to the sensitivity of a chest X-ray and thus it is usual practice (unless a specific cause has been found) to perform a CT scan if there is any suspicion of a neoplasm. On the CT scan the following may be found to explain haemoptysis: • a small area of bronchiectasis; • rarely, small tumours which are too small to be seen on the X-ray. In some very cautious departments (but not all), bronchoscopy would also be carried out to inspect the central airways. In addition, sputum (if it is present) should be sent for culture and sensitivity, tuberculosis examination and neoplastic cell cytology. At bronchoscopy, bronchial suckings or lavage can be sent for similar investigation. Despite all these investigations, many cases remain unexplained and then the question is whether the physician is going to be bold and discharge the patient or be more cautious and want to exclude the possibility of there being a lesion that is too small to show by imaging. It is usually considered good practice to arrange for a further chest X-ray in three or four months, either by the GP or in the clinic. If the film is still normal, the patient is discharged.
SHORTNESS OF BREATH Breathlessness is one of the most common respiratory symptoms; it is a subjective rather than an objective feeling. Figure 3.4: causes and features of breathlessness
49
Figure 3.5: associated features of breathlessness
Respiratory Disorders
50
Common respiratory (lung) causes of breathlessness
Common Chest Symptoms and their Investigation
51
Respiratory Disorders Common respiratory (lung) causes of breathlessness which may need consideration include: • asthma attack – usually intermittent, frequently with nocturnal attacks; • chronic bronchitis and emphysema – there is usually a chronic insidious onset with recurrent infective episodes; • pneumonia – acute onset with the patient having fever and feeling acutely unwell; • tuberculosis – a gradual onset over weeks often with cough, fever, malaise and weight loss. Sometimes there is haemoptysis; • interstitial lung disease including pneumoconiosis. Chronic insidious onset with history of industrial exposure (if occupation related); • pleural effusion – onset of gradually increasing breathlessness over days and weeks; • pneumothorax – acute onset – associated with unilateral chest pain; • lung cancer – onset over days or weeks, often associated with malaise and weight loss; • allergic alveolitis – acute onset after exposure. Recurrent episodes are frequent and industrial exposure is the rule; • pulmonary embolism – acute or subacute onset. May be recurrent episodes. Often associated with chest pain and haemoptysis; • bronchiectasis – chronic breathlessness with copious sputum.
Cardiac causes of breathlessness These are frequent and include: • left ventricular failure (the onset is acute and often nocturnal) caused by: (a) ischaemic heart disease; (b) valvular heart disease; (c) hypertension; (d) cardiomyopathies; (e) over-transfusion. Rarer causes of breathlessness include: •
neuromuscular diseases affecting the diaphragm or muscles of the chest wall, including: (a) severe kyphoscoliosis (curvature of the spine);
52
Common Chest Symptoms and their Investigation (b) ankylosing spondylitis; (c) Guillain Barré syndrome; (d) myasthenia gravis; (e) poliomyelitis; • renal disease – with kidney failure a patient may become breathless either: (a) because of a failure of the ability to excrete fluid through the kidneys and hence go into pulmonary oedema (heart failure); or (b) because they become acidotic and hyperventilate to try and excrete extra carbon dioxide; • diabetes mellitus – in severe diabetic ketoacidosis (uncontrolled diabetes) air hunger occurs due to central simulation because of the acidosis; • anaemia or altitude – breathlessness occurs because of reduced oxygen carrying capacity of the blood (anaemia) or reduced ambient PaO2 (high altitude).
The minimum expectation for investigating breathlessness Any patient complaining of breathlessness should be considered for the following protocol: (a) initial screen: • a history; • physical examination of the heart and lungs to include pulse rate and blood pressure and observation of the ankles; • measurement of respiratory function, ideally spirometry (FEV1/FVC) but a peak expiratory flow rate would be acceptable; (b) additional tests: if the breathlessness still remains unexplained, referral to a hospital physician at the chest clinic or for a chest X-ray. In patients where the diagnosis still remains unclear, more complex respiratory function tests should be performed including: • gas transfer; • oximetry; • blood gases to measure the oxygen status at rest and on exertion; • cardiological investigations may also be required if there are no obvious respiratory causes. Such investigations would include an ECG and echocardiogram.
53
Respiratory Disorders Figure 3.6: physical signs in chest disease
54
Common Chest Symptoms and their Investigation Figure 3.7: common causes of abnormal physical signs in the chest
55
Respiratory Disorders Figure 3.7: common causes of abnormal physical signs in the chest (continued)
In patients where breathlessness still remains obscure: • CT scan of the lungs to exclude minor degrees of fibrosis; • ventilation perfusion lung scans to consider the possibility of pulmonary vascular disease, should be carried out. Clearly, there is a time sequence for all of these investigations, not all will be necessary on everyone and it would take a number of visits to go through these tests and arrive at a diagnosis in more complex cases.
Wheeze When breathlessness is associated with wheezing (a musical sound caused by turbulent airflow through narrowed air passages), the breathlessness is most likely to be due to airways obstruction caused by:
56
Common Chest Symptoms and their Investigation • • • •
asthma; chronic bronchitis and emphysema; bronchiolitis (children); left ventricular failure (cardiac asthma) – rarer.
Stridor Whereas wheezing is a noise that is more pronounced during expiration when the bronchioles are shortened and narrowed, stridor is a term applied to noisy respiration that is always worse on inspiration. It results from obstruction to central large airways. The causes of this rarer situation are: • carcinoma of the larynx, trachea or major bronchus; • tracheal stenosis, for example, post-tracheostomy; • extrinsic compression, for example, goitre, laryngeal oedema (angiodema, anaphylaxis).
CHEST PAIN Chest pain is a very common cause of presentation to the doctor. It is important for the doctor to try and differentiate between the different kinds and nature of chest pain in order to arrive at a clinical diagnosis, and to select those patients who require further investigation from those who do not. The site and character of the chest pain often lead to a clinical suspicion of the cause.
Angina pectoris Pain coming from the heart caused by ischaemic heart disease (narrowing of the coronary blood vessels). This is characteristically worse on exertion and relieved by resting. It is a squeezing type of pain around the lower chest. The patient describes a pain which is dull and vice-like rather than sharp. It often radiates up into the neck and down the left arm. If a pain like this continues and is associated with nausea, vomiting, sweating and is not relieved by rest, there has to be a high index of suspicion that the patient is having a heart attack.
Pleurisy Caused by inflammation of the outer (pleural) surfaces of the lung. This is a sharp pain aggravated by respiration. It is unilateral, often focused in a small area of the chest. It is often severe and knife-like. A ‘pleural rub’ may be 57
Respiratory Disorders present and audible through the stethoscope. Involvement of the diaphragmatic pleura may cause pain referred to the shoulder tip. • • • • •
Common causes of pleural pain include: pneumonia; pulmonary embolism (infarction); pneumothorax; lung cancer; rib fractures.
Retrosternal pain – discomfort localised behind the breast bone Important points to bear in mind when investigating this symptom are: • that it is often associated with acute tracheitis and coughing; • oesphagitis or hiatus hernia are aggravated by eating food and related to posture – worse when lying down, better when standing up; • dull pain or discomfort may be associated with mediastinal swellings such as lymphadenopathy from cancer or lymphoma.
Other causes of chest wall pain (intercostal nerve distribution) Other conditions which may cause diagnostic difficulty are: • shingles – herpes zoster – often associated with vesicular (blistering) rash; • thoracic vertebral collapse – local tenderness over the particular vertebra; • Coxsackie B infection (Bornholm disease) – tender intercostal muscles; • costochondritis – pain over one or more costochondral junctions; • pericarditis – (inflammation of the outer surface of the heart) causes a central poorly localised tightness related to postural movement. There may be associated fever and audible pericardial rub (through stethoscope).
Chest pain: minimum investigation A careful history is essential, in particular to try and sort out whether one is dealing with cardiac causes (ischaemic heart disease) which may imply sinister and perhaps serious coronary artery disease as a precursor of a heart attack, or whether the chest pain has a more benign cause. The minimal expectations for examination of a patient with chest pain will be:
58
Common Chest Symptoms and their Investigation • physical examination of the heart and lungs, taking blood pressure and pulse and observing the ankles; • a resting electrocardiogram in any patient in whom the history is suggestive of ischaemic heart disease. If this is negative, but the pain is persisting and is exertional, an exercise ECG may be needed; • chest X-ray, particularly with pleuritic chest pain associated with the patient feeling unwell and febrile, or coughing purulent sputum or haemoptysis; • if the chest X-ray is normal and there is a suspicion that the patient may have pulmonary emboli, an imaging procedure to exclude pulmonary emboli such as ventilation perfusion lung scanning or a spiral CT lung scan (or even a pulmonary angiogram) is essential.
Features associated with chest pain In any patient presenting with breathlessness, cough or chest pain, it is essential to search for other symptoms, such as a fever.
Significance of fever in respiratory illness Commonly associated with: • acute infections: (a) bronchitis; (b) pneumonia; • chronic infections: (a) tuberculosis; (b) lung cancer or lymphoma.
Poor appetite and weight loss Appetite loss may occur with acute self-limiting illnesses of an infective nature. However, appetite loss and weight loss over a protracted time are serious features which require investigation. Protracted weight loss may be found with: •
emphysema;
•
infections – tuberculosis/HIV infection;
•
lung cancer.
59
Respiratory Disorders
Ankle swelling Although a small amount of ankle swelling may be found in health, more profound and persistent swelling is an important sign which needs investigation. Causes of persistent ankle swelling: • cardiac failure; • renal failure; • liver failure. These are usually bilateral, symmetrical and painless, unlike deep vein thrombosis which is normally unilateral and painful at onset.
60
CHAPTER 4
ASTHMA
INTRODUCTION Asthma is a very common respiratory disease, affecting up to 5% of the adult population at some stage in their lives. Its onset can be at any time from birth until well over retirement age. The incidence of asthma is increasing for unknown reasons. From a medico-legal point of view, asthma is one of the most important chest conditions for the following reasons: • asthma can be caused by occupational exposure, thus leading to potential litigation against the employer; • asthma is a fatal condition in a small number of patients. About 2,000 patients a year die in the UK because of asthma. Some of these are young adults and there are often allegations that the deaths may have been avoidable. There are also tragedies where a severe asthma attack has led to a respiratory arrest and successful resuscitation has occurred, but there has been brain damage because of prolonged cerebral hypoxia. In these circumstances, subsequent problems, such as long term care of the patient, arise. Fortunately, such cases are rare, but clearly they are of great legal importance because the cost of providing lifetime long term care for, say, a 20 year old, is enormous.
CLINICAL FEATURES Asthma is a condition characterised by reversible airways obstruction. The air passages leading into the lungs, the bronchi and respiratory bronchi become inflamed. The smooth muscle in their walls is in spasm and there is excess mucus production, leading to constriction and narrowing of these air passages. This inflammation is reversible spontaneously or with treatment with no particular time limit to the reversibility. It may be within a few minutes, or may take a few weeks or more to reverse completely. Asthma is conventionally defined as variation of 20% or more of the measurements of respiratory function such as peak flow rate or forced expiratory volume (FEV1). Lesser amounts of reversibility can be observed in patients with other causes of airways obstruction – such as chronic bronchitis.
61
Respiratory Disorders The airways obstruction in asthma is due to a mixture of bronchial small smooth muscle spasm, bronchial wall oedema and inflammation and increased mucus production, all of which are potentially reversible.
USUAL SYMPTOMS Symptoms occur spontaneously or are related to the season of the year or specific exposure to allergens (substances to which the individual is allergic) or exercise. The usual symptoms are wheezing, cough, sputum production and episodic breathlessness. Accompanied with this are the usual signs which are more obvious in severe asthma – visible breathlessness and increased rate of breathing, rapid pulse rate, hyperinflated chest, diminished air entry on both sides and wheezing – usually expiratory. Signs of an extremely severe attack of asthma include cyanosis (a blue appearance of the tongue and lips due to lack of oxygen), a silent chest because there is inadequate airflow in and out to generate wheezes, and a drop in blood pressure (hypotension).
ASSESSMENT OF THE SEVERITY OF AN ATTACK In an asthma attack, the GP (or hospital physician) is expected to make measurements which give a guide to the severity of airways obstruction: • peak flow rate or FEV1; Figure 4.1: peak flow chart showing recovery from an acute asthma attack
❍
– – – ❍ = before salbutamol inhalation
•–––• = after salbutamol inhalation
62
Asthma • the pulse rate; • estimation of blood oxygen concentration, either by blood gases or oximetry (in hospital, as unavailable to most GPs). Figure 4.2: changes in the arterial PaCO2 with increasing severity of an asthma attack
Initially, there is a low PaCO2, due to hyperventilation. As the airways obstruction worsens, so there is alveolar hypoventilation and a rise in the PaCO2. A normal or raised PaCO2 in an asthma attack is a serious sign.
The importance of these is that they are objective measurements. Subjective variables such as the patient’s feeling of breathlessness and degree of wheeziness play little part in managing acute asthma attacks because it is notorious how poor patients are at subjectively monitoring the severity of their asthma attacks. Doctors, equally, can be misled unless these essential objective measurements are made repeatedly and recorded.
INVESTIGATIONS OF PATIENTS WITH ASTHMA Respiratory function tests In adults, the simplest test is the peak flow expiratory rate (PEFR) measurement made with a domiciliary peak flow meter, which can be prescribed by the GP on an FP10 prescription form. In young children, a low 63
Respiratory Disorders range or mini Wright peak flow meter should be used instead. Spirometry to obtain the forced expiratory volume in one second (FEV 1 ), using a Vitalograph or Microspirometer, is more complex and few GPs have either of these, although this is often used in addition to peak flow as the mainstay of measurement in a chest clinic. On hospital wards or in Accident and Emergency departments the peak flow is routinely used. Tests should be done before or after giving a bronchodilator such as Salbutamol. The respiratory function tests are necessary: • to confirm a diagnosis of asthma in the first place; • to monitor response to therapy. Full lung function tests typically show reversibility in the airways obstruction with large hyperinflated lungs and normal gas transfer. Thus, the peak flow rate and FEV1 are diminished, but the forced vital capacity is relatively well maintained. After bronchodilator therapy (either immediately or after perhaps a few weeks of oral steroid therapy), the peak flow rate and FEV1 will have improved by more than 20%.
Variability of lung function Asthma is episodic and between attacks patients’ lung function can be completely normal.
64
Asthma Figure 4.3: simple exercise test to diagnose exercise-induced asthma
Baseline: the patient ran, following which, her peak flow was measured. This dropped from 460 litres/minute to 240 litres/minute at five minutes, thus confirming the diagnosis. The following day, the patient underwent the same test, having been tested with sodium cromoglycate 20 minutes before the test. The broncho constriction was completely prevented.
Exercise test If patients complain of asthma induced by exercise, the peak flow should be measured prior to, and during the 30 minutes following, five minutes of vigorous exercise to highlight those with exercise-induced asthma. A deterioration of peak flow by 20% is diagnostic. The tests can be repeated after pre-treatment with either Salbutamol or an inhaled steroid to see if these successfully abolish the exercise-induced asthma.
65
Respiratory Disorders
Domiciliary measurement One way to obtain a picture of how the patient is at more than a spot time in the surgery is to give them a peak flow meter and ask them to monitor their peak flow at home twice daily (first thing in the morning and last thing at night) for two or three weeks to observe the variability. A variability of 20% is diagnostic of asthma.
Workplace measurement Measuring peak flow at home and in the workplace may be particularly useful where one suspects the patient is suffering from occupational asthma. The peak flows will be better in the morning than in the evening. They will be worse in the workplace during the week than at home at the weekend. They will be worse during periods of work than periods of holiday.
Allergy tests Allergy testing is performed either by skin tests or blood tests.
Skin tests Asthmatics should have skin prick tests performed to detect allergies to common allergens such as house dust mites, dog and cat fur, feathers, eggs, milk, moulds, tree and flower pollens. These tests require a very small amount of solution of allergen being placed onto the forearm, a single prick being made with a needle and then the allergic reaction (if any) being observed at 20 minutes. The central swelling (wheal), rather than the area of redness (flare), is taken as the response. There is not 100% concordance between skin tests and allergens causing asthma.
Blood tests Specific antibodies to common inhaled and ingested antigens can be measured by blood test, RAST, radioallergosorbent test or MAST. Elevated total serum immunoglobulin E (IgE) levels give an indication of an allergic basis. However, many patients, particularly older ones, do not have any specific allergens discovered.
Allergic bronchopulmonary aspergillosis (ABPA) In the rare disease caused by allergy to a fungus, aspergillus, an allergic reaction in the skin is found four to six hours after a skin test for aspergillus. There are also aspergillus precipitins found in the blood.
66
Asthma
Chest X-rays Usually the chest X-ray in asthma is normal apart from rather large hyperinflated lungs, with flat diaphragms. Nevertheless, chest X-rays are essential in severe asthma attacks resulting in admission to hospital for the following reasons: • to exclude pneumothorax; • to exclude co-existing pneumonia; • to diagnose mediastinal emphysema.
Sputum Sputum examination is rarely helpful unless there is bacterial infection (most asthma attacks are caused by viral infections). It is good practice to send purulent sputum (if present) for culture. Figure 4.4: oxyhaemoglobin dissociation curve
Note shifts in the curve due to changes in pH, temperature and 2,3 DPG. Note that the oxygencarrying capacity of blood does not fall much until there has been a considerable reduction in the PaO2. T = tissues
L = lungs
V = venous
67
A = arterial
Respiratory Disorders
Blood oxygen Oxygen can be measured most simply by use of a pulse oximeter. A finger clip is applied to the finger and the oxygen saturation in the blood is measured (as a percentage). It cannot measure carbon dioxide. Below 92% a very small decrease in the oxygen tension in the blood causes a very rapid fall in the oxygen saturation (see above, figure 4.4). Thus, below 92% arterial blood gases should be measured to have a more precise measurement of oxygen (PaO2) and carbon dioxide (PaCO2) in the arterial blood. The problem with blood gas estimation is that because the blood is taken from an artery rather than a vein, it is more painful because of the nerve endings in the arterial wall. This acts as a disincentive for patients to attend hospital if they have previously had this done frequently. Patients are also reluctant to have them done repetitively on a single visit to hospital. Because measurement of blood gases requires an expensive analyser, blood gases are not available in GPs’ surgeries. Some good GP practices, however, have a pulse oximeter, some of which are now very small and portable. In asthma attacks (because of the mismatch of ventilation and perfusion in the lungs), the oxygen in the arterial blood (PaO2) falls progressively with the increasing severity of an attack. Because the patient is over-breathing, the carbon dioxide level (PaCO2) initially falls. As the severity of the attack increases, the airways narrow further so ventilation is gradually impaired, causing carbon dioxide first to climb into the normal range and then later to become elevated. Thus, in an asthma attack with a reduced PaO2, the doctor has to be wary of a patient with a normal or elevated carbon dioxide. These are both signs of a very severe attack. After an attack the arterial blood gases often take 10–14 days longer to return to normal than the peak flow or FEV1 because the mucus plugging in the airways causes ventilation perfusion mismatch.
RECOGNITION OF CAUSATIVE FACTORS A full history is essential – particularly occupational history (see below, Chapter 13). The patient will have noted the onset of their symptoms, whether they are worse at work than at home, whether they are worse on a Monday than during the weekend, whether they progress with the working week, whether they are worse at work than during the holidays. Are there particular things they have noted that have made the asthma worse? Is it worse if they go into a room where an animal has been? Is it worse using hairsprays or drinking wine? Is it related to exertion? Is it seasonal? Is it perennial?
68
Asthma
PREVENTION OF ASTHMA If at all possible, the first mainstay of treatment is prevention, first, with causative antigen avoidance. It should be remembered that skin tests identify possible, but not definite, causes of asthma. Specific advice should be given about the avoidance of allergens, at least for a trial period: the most common is the house dust mite (but it can be difficult to avoid contact with this altogether). With house dust allergy, the patient should vacuum their home with a low dust machine as much as possible to eliminate dust, including mattresses and bedding. They should use synthetic bedding and pillows. Wrapping the mattress in a plastic sheet is an effective way of reducing antigen exposure, but is rather uncomfortable. Replacing mattresses is rather useless, as it only takes six months for house dust mites to get up to full numbers in a new mattress. Patients with occupational asthma need advice about changing their working environment or obtaining breathing apparatus for personal protection.
DESENSITISATION Whereas there was a vogue for desensitisation some years ago where repeated injections were given to try to raise blocking antibodies, even in the most enthusiastic hands there was limited success and the products were withdrawn after recorded fatalities due to anaphylaxis and asthma precipitation were found to be greater than the cure rate. Desensitisation that is carried out currently is limited to wasp and bee sting allergy (stings which may be fatal) and these should be carried out in experienced hands in hospital. The patient needs to be observed for at least 30 minutes after each injection. Treatment for anaphylaxis – adrenaline and hydrocortisone injections – need to be drawn up by the patient’s bedside ready for use in emergency. The availability for full resuscitation is also required.
69
Respiratory Disorders
DRUG AVOIDANCE There are certain groups of drugs which are known to cause airways narrowing in everyone. These should be avoided in all asthmatics. In addition, there are some which may cause asthma attacks only in certain allergic individuals. Examples of the first group of drugs (which include propranolol) are non-selective beta blockers, which may be prescribed for conditions such as hypertension, angina, anxiety and occasionally migraine (beta blocker eyedrops are also contraindicated). Even selective beta blockers, such as atenolol and metoprolol, should only be used in asthmatics with great caution and are best avoided altogether. Examples of the second group of drugs (to which some asthmatic patients may be allergic) include aspirin and other non-steroidal anti-inflammatories (such as indomethacin or diclofenac). These are known to precipitate asthma in some sensitive individuals.
INDUSTRIAL AND OCCUPATIONAL ASTHMA There are a large number of known sensitisers which cause asthma. The following list should be regarded as incomplete, because new causes of occupational asthma are constantly being described and the list being enlarged, but it gives a guide. A literature search should be done on behalf of any patient who comes to you complaining that their asthma might be due to their occupation or surroundings. Even exposure to American cockroaches can cause asthma. Reports on the English variety are awaited!
70
Asthma Figure 4.5: some causes of occupational asthma Grains, flour and plants Wheat Rye Buckwheat Hops Green coffee beans Castor beans Tea Tobacco Gum Sunflower pollen Soya flour Karaya gum (hairdressers) Dental adhesive Latex rubber gloves
Drugs Aminophylline Alginates Cimetidine Salbutamol Piperazine Penicillin Cephalosporins Tetracyclines Isoniazid Hepatitis B vaccine
Laboratory and other animals Rats Mice Guinea-pigs Rabbits Pigs Deer Bat guano
Crustaceans King crabs Prawns
Metals Platinum salts Nickel sulphate Chromates Cobalt Vanadium
Isocyanates Toluene-di-isocyanate (TDI) Diphenylmethane di-isocyanate (DDI)
Solder fluxes Colophony
Dyes Brilliant scarlet Brilliant blue Brilliant yellow
Arthropods and insects Locusts Silk worms Beetles Cockroaches Housefly maggot Grain weevil Fruit flies Blow flies
Enzymes Bacillis subtilis Trypsin
Gases Freons
Chemicals Tannic acid Chloramine T Formaldehyde Glutaraldehyde Ethylene diamine
Woods Red cedar Ramin Oak Mahogany Californian redwood Sawmill workers
Fungi Cladosporium Mushrooms Aspergillus niger Candida tropicalis Oil mists Contaminated humidifiers
71
Respiratory Disorders
TREATMENT OF ASTHMA It should be remembered that around 2,000 asthmatics die in the UK from their disease each year. Often, they are young. The aims and object of treatment are to allow asthmatics to lead a normal life with the help of good adequate treatment. By doing this and allowing patients to refer themselves directly to hospital, some of these deaths might be avoided. Early discharge from casualty departments after a single nebulisation of bronchodilator or intravenous injection of bronchodilator should be discouraged, as, in fact, most of these patients require proper control of their asthma and hospitalisation for a few days. Good indicators as to whether the patient’s asthma is out of control are: • waking in the early hours of the morning due to breathlessness; • increasing morning dips in peak flow rate; • reduction in peak flow (if the peak flow has been monitored on a daily basis). Large swings and dips in peak flow also occur in patients in hospital recovering from acute asthma attacks – they should not be discharged until the dips have largely reduced or disappeared.
BRITISH GUIDELINES ON ASTHMA MANAGEMENT The original 1990 British Thoracic Society Guidelines on Asthma Management were revised in 1993 and have again been revised in 1997 as a result of extensive collaborative work by a committee comprising experts from both primary and secondary care (see Thorax (1997) 52 Suppl 1 S1–S21). They are consultative rather than completely evidence-based and take into account recognised areas of uncertainties, new research findings and availability of new treatment. The main recommendations are to gain rapid control, and facilitate self-management. These suggestions for management of chronic asthma in adults and schoolchildren, and the recommendations for the management of acute severe asthma in adults, are essential references and should be obtained from a reference library to be read in conjunction with this book which, inevitably, can only briefly summarise the points (see ‘Useful References’, pp 245–47). It should be remembered that these are guidelines and not set protocols, and, thus, although a doctor could quote them as an absolute defence of his treatment, variations are permitted so long as there is a good and justifiable reason for departure from them. They provide a very useful outline for the basis of therapy.
72
Asthma
ASTHMA THERAPY The groups of drugs which are the mainstay of asthma therapy include the following.
Beta agonist bronchodilators These are adrenaline-like drugs which act on the smooth muscle of the bronchial wall to cause relaxation and enhance bronchodilation. Examples of such drugs include salbutamol (Ventolin) or terbutaline (Bricanyl). Longer acting drugs, such as salmeterol (Serevent), have a similar mode of action and a longer duration of action, but have a slower onset of action. These drugs come in a variety of delivery devices: • standard metered dose inhaler; • autohaler; • powdered delivery devices such as rotacaps, rotahaler and turbohalers; • nebuliser solutions. Salbutamol may, in very severe attacks, be used intravenously by slow infusion.
Anticholinergic drugs These are anti muscarinic agents which act on the bronchi to cause bronchodilation by a different mode of action than beta agonists. Examples of this group include ipratropium bromide (Atrovent) and oxytropium (Oxivent).
Antiallergic compounds When these compounds are inhaled into the bronchial tree, they act on and stabilise the MAST cells in the bronchial wall and prevent release of histamine which would otherwise have been released in the allergic (asthmatic) reaction, which occurs if an allergic asthmatic inhales an antigen to which they are sensitive. Examples of these drugs are cromoglygate (Intal) and nedocromil (Tilade).
Corticosteroids These are powerful anti-inflammatory agents which reduce airway inflammation. They may be given either by inhaler – examples include beclamethasone (Becotide, Becloforte), budesonide (Pulmicort); by mouth – prednisone and prednisolone; or by injection – hydrocortisone. They are slower in their onset than other bronchodilators such as salbutamol. 73
Respiratory Disorders
MANAGEMENT OF CHRONIC ASTHMA Essential supplements to drug therapy include: • avoidance of provoking factors where possible; • patient involvement in education; • observation of inhaler technique (obtaining best inhaler technique); • prescription of peak flow meter to monitor response of treatment; • treatment ‘stepped up’ as necessary to achieve good control; • treatment ‘stepped down’ if control of asthma is good.
Steps Treatment is ‘stepped up’ or down according to the level of control of symptoms with the general principle of giving as little treatment as is necessary to control asthma as well as possible: step 1: occasional need of bronchodilators such as salbutamol; step 2: regular inhaled anti-inflammatory agent such as inhaled beclamethasone or budesonide (steroids) or cromoglycate; step 3: high dose inhaled steroids (for example, Becloforte) or low dose inhaled steroids (for example, Becotide) plus long acting inhaled beta agonist bronchodilators (for example, Serevent); step 4: high dose inhaled steroids and regular bronchodilators; step 5: addition of regular steroid tablets. Treatment should be reviewed every three to six months in order to step down the treatment if the patient’s asthma is ideally controlled.
TREATMENT OF ACUTE SEVERE ASTHMA
Immediate Immediate treatment involves the provision of: • oxygen 40–60%; • salbutamol or terbutaline via oxygen driven nebuliser; • oral prednisolone 30–60 mg or intravenous hydrocortisone; • no sedatives of any kind; • chest radiograph to exclude pneumothorax. If life-threatening features are present, it is advisable to: 74
Asthma • add ipratropium to the nebuliser; • give aminophylline or salbutamol intravenously.
Subsequent management If the patient is improving: • continue oxygen; • continue steroids • repeat nebuliser four hourly. If the patient is not improving: • continue oxygen and steroids; • repeat nebulised salbutamol every 15–30 minutes; • add ipratropium to nebuliser, repeat six hourly. If the patient is still not still not improving after this treatment: • give aminophylline infusion; • salbutamol or terbutaline infusion; • monitor treatment with peak flow rate every 15–30 minutes, and oximetry to keep a saturation above 90%; • repeat blood gases within two hours if initial PaO2 less than 8 kPa unless subsequent saturation greater than 92%, or, if PaCO2 is normal or raised, to check whether patient is deteriorating; • chart peak flow before and after giving nebuliser and inhaled beta agonists four times daily throughout hospital stay.
Indications for transfer to intensive care unit These include: • deteriorating peak flow, worsening or persisting hypoxia or elevated PaCO2; • exhaustion, feeble respiration, confusion or drowsiness; • coma or respiratory arrest.
TREATMENT OF ACUTE SEVERE ASTHMA IN GENERAL PRACTICE This is along very similar principles. It is important to recognise that many deaths from asthma are preventable and delay can be fatal. Factors which contribute to fatalities include:
75
Respiratory Disorders • patients or relatives failing to appreciate severity of attack; • under-use of corticosteroids; • doctors failing to assess the severity by objective measurements. Assessment should include: • symptoms in response to self-treatment; • heart and respiratory rates; • peak expiratory flow rates. It is important to realise that patients with severe or life-threatening attacks may not look distressed and may not have all these abnormalities. The presence of any of these abnormalities should alert the doctor to the need for early hospital admission.
MEDICO-LEGAL ASPECTS OF ASTHMA MANAGEMENT The three main areas in which there is medico-legal interest in asthma management are: (a) death/near death; (b) oral corticosteroid therapy; (c) occupational causes.
Death/near death If a young person dies or becomes brain dead (or severely mentally impaired) because of a severe asthma attack (or prolonged, relatively unsuccessful, resuscitation) the question is, whether this was avoidable. The factors which may be involved include the following.
Poor long term supervision and control Examples include: • never seeing the patient, merely giving repeat prescriptions; • not monitoring respiratory function – peak flows never being measured; • patient being controlled remotely without visiting the surgery for any assessment.
Inadequate assessment and recognition of severity of attack Examples include: • no peak flow measurement;
76
Asthma • • • •
no measurement of oxygen saturation; no measurement of pulse rate; no physical examination; no chest X-ray in hospital.
Inadequate treatment In particular, failure to give appropriate high dose oral steroid therapy has been shown to be a major factor in causing death.
Delay in referral to hospital Examples include: • delay in visiting following telephone calls; • delay in referral to hospital once the GP has arrived and has made an assessment; • inappropriate waiting for clinical improvement at home.
Delay in arrival of the emergency services All of these may add up to a situation where a respiratory arrest occurs either at home or in the ambulance on the way to the hospital. These types of respiratory arrest – occurring where no anaesthetist is present to intubate and ventilate the patient – have less chance of success (and a higher chance of brain death because of the brain being starved of oxygenated blood) than resuscitations occurring in hospital.
Delay of therapy or failure of recognition of severity in hospital Often, because of inadequate assessment – peak flow or oxygen saturation not being measured or monitored regularly.
Inadequate treatment in hospital Examples include: • delay in calling for resuscitation; • failure to recognise complications such as a pneumothorax or pneumonia; • inadequacies or availability of a resuscitation team and facilities. Despite all these potential inadequacies, it is important to realise that not all asthma deaths are avoidable. However, in a minority of fatal cases, there is medical negligence.
77
Respiratory Disorders
Case studies: general Case 1: asthma – sudden severe attack and death caused by hairspray A 28 year old hairdresser was asymptomatic when she arrived at work. She regularly used aerosol hair conditioners at work and had previously noted that they induced a little breathlessness. On the morning in question, immediately after use she developed acute very severe asthma. The ambulance was called immediately and delivered her to hospital within 10 minutes of the call. She had a respiratory arrest on the way to hospital. By the time she arrived in Accident and Emergency she had stopped breathing for five minutes. Resuscitation was successful in that circulation was restored after she had been intubated and ventilated with oxygen. She had severe brain damage because of this arrest and the prolonged hypoxia. She was ventilated for 10 days in intensive care, but never regained consciousness and died. Comment There was no question of fault, that is, negligence, on the part of anyone. This was an example of a sudden severe fatal attack in which, despite the most rapid treatment, practically nothing could be done fast enough. Case 2: asthma – sudden death from pneumothoraces A 20 year old student went home to visit her parents in Cornwall for a weekend. She experienced a sudden severe attack of asthma with bilateral chest pain and died suddenly at home before the GP could arrive (20 minutes later). At post mortem, it was discovered that she had developed spontaneous bilateral pneumothoraces with her asthma, and thus had died within a matter of minutes, before either the ambulance or GP could arrive. Comment Again, no one was negligent.
Case studies: primary care Case 3: asthma death – inadequate appreciation of severity of attack – delay in referral to hospital A GP was called to a known 35 year old asthmatic at 10.00 pm. The patient’s peak flow rate was under 100 litres/minute (exceedingly low – the normal peak flow for this young asthmatic was known to be 350 litres/minute). A nebulisation of a bronchodilator was given, but the peak flow had not significantly improved. The GP left the asthmatic in the hands of his wife and went out on his further visits, saying he would return in half an hour. He asked the wife to call him if things got worse. Regrettably, the patient died
78
Asthma before the GP could return and the wife and GP were both, understandably, distraught. Appropriate treatment should have been immediate referral to hospital following nebulisation and waiting with the patient until the ambulance arrived. Comment The British Thoracic Society National Guidelines on Asthma Management make it clear that this patient required emergency admission. Case 4: asthma – respiratory arrest – brain damage – delay in GP visit A GP received a call at 2.00 am on a Sunday morning from a known asthmatic saying he was having an asthma attack. Although the patient had a peak flow meter, the GP did not ask him his peak flow. It was mutually agreed that a visit at 10.00 am would be adequate. The visit was carried out. Unfortunately, at this stage, the GP found a patient who was moribund and who required immediate hospitalisation. Despite appropriate treatment from this point, the patient had a respiratory arrest in the ambulance and suffered long term brain damage. Comment Arguably, there were eight vital hours lost. If the GP had asked for the peak flow measurement she would have realised the severity of the attack and not delayed eight hours in visiting. Alternatively, she could have suggested the asthmatic went immediately to an Accident and Emergency department for appropriate treatment, without waiting the agreed eight hours for the GP to visit. Either course could be argued to have potentially prevented the respiratory arrest and the consequent long term brain damage. Case 5: asthma death – inadequate therapy A GP was regularly monitoring a patient’s asthma. The normal peak flow rate was in the order of 250–300 litres/minute when adequately controlled. The patient came to see the GP because of increasing breathlessness. The GP noted that the peak flow was 120 litres/minute but failed to alter therapy. Regrettably, three days later, the patient died at home because of worsening asthma. Comment The appropriate course of action would have been an immediate prescription for high oral steroids, a nebulisation of bronchodilator and hospitalisation. This is an example of how poor a patient’s assessment of the severity of their own condition can be. It also displays how a GP can underestimate the severity of an attack. Historically, there have been many cases where patients visited their GP complaining of asthma, no assessment was made and the patient died. It is fortunate that, with increasing awareness, these types of incidents are now 79
Respiratory Disorders diminishing in frequency. Regrettably, in this case, the assessment of severity was made and recorded, but inadequate action taken. Case 6: asthma – respiratory arrest – brain damage – delay in ambulance service A 34 year old man was taken ill with a sudden severe asthma attack. The GP was called, arrived at the house within half an hour, immediately called for an ambulance and gave a nebulisation of bronchodilators appropriately. Due to confusion in the ambulance service, the ambulance did not arrive for almost an hour, despite being in a northern city centre. When the ambulance eventually arrived and took the patient to hospital (with the doctor in attendance), the patient had a respiratory arrest on the journey. There was resuscitation of the collapsed, pulseless and not breathing patient in the Accident and Emergency department of the receiving hospital. The man survived, but had severe brain damage. Comment It can be argued that the delay by the emergency services was largely responsible for harm caused in this case. A shortened ambulance time might well have resulted in a better outcome. Case 7: asthma death – inappropriate prescription A GP was tried for manslaughter. She had inadvertently prescribed a beta blocker to a known asthmatic for anxiety attacks, but the patient died soon after taking the first tablet. Subsequently, she altered the patient’s records to try and delete the references to asthma. She was found ‘not guilty’ on the charge of manslaughter, but was found ‘guilty’ on the lesser charge of attempting to pervert the course of justice, because she altered the patient’s records to remove all mention of the word asthma. For this, she received a suspended jail sentence. Comment To err is human: she made a mistake in a busy clinic; however, alteration of patients’ records cannot be condoned.
Case studies: secondary hospital care Fortunately, in hospital, the number of problems arising around asthma management in hospital are fewer, largely because of the availability of immediate resuscitation with an anaesthetist and resuscitation team who can paralyse, intubate and ventilate patients and transfer them to ITU.
80
Asthma Case 8: asthma death – allergy to a non-steroid anti-inflammatory drug A 35 year old male asthmatic was referred to hospital because of musculoskeletal chest pain. She had no problems with her asthma, indeed on arrival, her peak flow was normal and was recorded at 600 litres/minute. For her chest wall pain, she was given indomethacin (Indocid), a non-steroidal anti-inflammatory drug. Immediately, she became breathless and had a respiratory arrest. Resuscitation was difficult, during which she suffered permanent hypoxic brain damage. She survived in ITU for 18 months before dying. Comment The doctor prescribing Indocid had failed to recognise that the patient was known to be allergic to it. It had been recorded in the hospital notes on her previous admission that she was allergic to indomethacin. (Indomethacin is the generic drug name for which Indocid is the trade name.) It was clearly shown (on a previous admission) that indomethacin was precipitating her asthma. This case was, not surprisingly, settled prior to coming to court.
Steroid therapy Increasingly, patients who receive oral steroids for their asthma are seeking legal advice as to whether they should be on oral steroids and if so whether they are receiving too high a dose. Oral steroid therapy has many possible side effects, including increased blood pressure, increased weight, osteoporosis, cataracts, aggravating diabetes, easy bruising, skin thinning and immunosuppression. In these situations, although oral steroids may be indicated in step 5 treatment of chronic asthma, and are indicated in acute severe attacks, there has to be evidence: (a) that there has been correct monitoring of the patient’s asthma; (b) that there have been attempts to keep the patient on the minimum dose of steroid therapy possible to control their asthma adequately, that is: • to give the least possible dose needed of relieving bronchodilators; • to ensure the minimum possible limitation of activity; • to ensure the least possible variation in peak expiratory flow; • to maintain a balance between the best expiratory peak flow rate and the least adverse effects from the medicine. It is, therefore, important to show that the GP or the hospital are monitoring the patient’s condition regularly and not just giving repeat prescriptions by post or by telephone. There is a recommendation that any patient on oral
81
Respiratory Disorders steroids (tablets) for their asthma should be managed in a hospital chest clinic rather than by the GP, where they may only have been seen by the practice nurse. It is important to show that: • the peak flow rate or other measurement of lung function has been measured on a regular basis; • all types of delivery device have been tried to make sure that inhaled steroids can be used as effectively as possible; • attempts to reduce oral steroid dose have been made.
Possible pitfalls In these situations of possible oral steroid abuse, it is important to recognise that there are occasions when patients may be their own worst enemy. Patients may like to take control of their own steroid therapy and feel that they know better than the doctor because oral steroids act as a tonic and euphoriant. Frequently, the allegations made by patients about being prescribed and receiving too much oral steroids do not correlate with the GP’s records.
Symptoms The side effect about which the patient is complaining to a lawyer, such as osteoporosis, may not actually have been documented or diagnosed. Oral steroids may not necessarily be the cause of the patient’s symptoms.
Therapy It is essential, in all cases, to review critically the GP’s notes, and to tabulate the quantity and monitoring of oral and inhaled steroids and other bronchodilators prescribed: • to see whether and, if so, when the peak flow rate has been measured over the years; • to assess the number of attendances and visits; • to assess the number and details of prescriptions; • to quantify the total dose of steroid pills (that is, prednisone or prednisolone) prescribed; • to gauge whether these were in short courses of high dose steroids (which are perfectly acceptable for asthma attacks) or whether there had been prolonged permanent treatment (which is less acceptable unless all else has failed).
82
Asthma Comment Frequently, patients have complained to lawyers about prolonged high dose oral steroids whereas in fact they have only ever been given short sharp courses of high dose oral prednisone which is perfectly acceptable. Alternatively, the total dose of prednisone or other steroid medications actually recorded as prescribed is nowhere near what the plaintiff says they have received even though the records would appear to be complete in all other respects. Unfortunately, a patient’s memory five to 10 years back is less reliable than the GP records. Going through these notes is very slow and time consuming, but is the only way to arrive at the evidence.
Inadequate steroids On the other side of the coin, with steroid therapy, there are instances where too little has been prescribed. Steroids have been refused either in the form of inhaled or oral steroids in the mistaken belief that they will do harm. One example is pregnancy and the idea that they will harm the baby more than severe asthma. There are situations where inhaled steroid therapy has been discontinued during pregnancy with tragic results. Similarly, there have been episodes where patients with severe asthma attacks have not been given oral steroid courses with similarly disastrous effects. Additionally, there is a small group of asthmatics with very severe disease who require long term oral steroids – they are bound to develop side effects.
Occupational asthma: medico-legal aspects A full occupational history is essential. Peak flow rates twice daily during the week and at weekends or on holidays are ideal; exposure to known precipitants and the number of other workers similarly affected in that occupation. The occupational health record, with the number of visits and asthma attacks before and after starting the occupation, is clearly essential. Detailed sickness records – it is essential, when inspecting the GP’s or hospital notes when there is an accusation that asthma only started when exposed to a certain chemical in an occupation, to check that this is the case by checking dates of onset. Frequently, this is true, but on many occasions it may be clearly documented in the GP’s notes that asthma had started a year or more before the occupation was commenced. Similarly, there are asthmatics in whom it is alleged that the asthma has become worse after a particular exposure but on reviewing the GP’s notes, the number of visits, the number of prescriptions and total use of inhalers or steroids has, in fact, not fluctuated after the
83
Respiratory Disorders occupational event. Occupational health records are essential, as are details of respiratory protection and precautions provided in the workplace. (On medico-legal aspects of occupational asthma, see, further, below, Chapter 13.) Occupational asthma is a compensatable disease with benefit payable.
84
CHAPTER 5
PNEUMONIA
INTRODUCTION Pneumonia is an infection of the lung tissue itself, whereas bronchitis is an infection of the air passages leading into the lung. A large number of different micro-organisms can cause pneumonia. These include bacteria, viruses and fungi. Because of the variety and number of micro-organisms and their differing sensitivities to antibacterial drugs (antibiotics), it is important to try to identify the cause so that the appropriate antimicrobial agent can be given. However, because pneumonia is a serious condition and identification of the causative organism takes time, it is essential that therapy is started on a ‘best guess’ basis, depending upon where the infection was acquired, the clinical presentation and the chest X-ray appearance, before results of cultures are available. The British Thoracic Society has published guidelines on the management of community-acquired pneumonia in adults admitted to hospital (Br J Hosp Med (1993) 49 346–50) and most hospital trusts in the UK have their own ‘inhouse’ modifications of these guidelines as to how a patient presenting with pneumonia should be treated. Historically, pneumonias were divided into those which affected an entire lobe, called lobar pneumonia, and those where the process was restricted to alveoli adjoining bronchi, where it was called bronchopneumonia. However, confluent bronchopneumonia is often indistinguishable from lobar pneumonia. With some types of pneumonia, notably staphylococcal pneumonia, cavities may occur within the lung.
85
Respiratory Disorders Figure 5.1: right lower lobe consolidation (some consolidation on left, also)
COMMON CAUSES OF PNEUMONIA Clinically, it is useful to divide patients into groups according to whether they acquired their pneumonia within the community or within hospital (or nursing home).
Causes of community-acquired pneumonia Common infecting organisms in community-acquired pneumonia include: • streptococcus pneumoniae; • haemophilus influenzae; • legionella pneumophila; • mycoplasma pneumoniae; • chlamydia. 86
Pneumonia Streptococcus pneumoniae accounts for about 80% of cases of communityacquired pneumonia. Pneumonia may also be caused by viruses, such as the influenza virus.
Causes of hospital-acquired pneumonia Although the above causative organisms may be responsible, more than 50% of cases of hospital-acquired pneumonia are due to aerobic gram-negative bacilli and pseudomonas aeruginosa and staphylococcus aureus (10%). With pneumonia acquired in nursing homes, the frequency of causes lies between the distribution of causative organisms found in the community and hospital-acquired pneumonia. In communities with a high incidence of HIV infection, pneumocystis carinii pneumonia and mycobacterium tuberculosis are more common causes of community-acquired pneumonia. In certain geographical areas, fungi such as histoplasmosis, blastomycosis and coccidioidomycosis are important causes of pneumonia. In bird handlers, chlamydia psittaci is an important cause.
CLINICAL FEATURES The common symptoms of pneumonia are: • malaise; • muscle aches and pains; • cough; • fever; • chest pain, particularly pleuritic in nature; • increasing breathlessness; • sputum production; • occasionally, haemoptysis (coughing of blood). There is often a rapid deterioration in the patient’s state of health which can be accompanied by any of the following clinical signs: • fever; • increased respiratory rate; • rapid pulse rate; • on examination of the chest, there is decreased movement and decreased percussion note with the presence of crackles or bronchial breathing on listening over the affected area; • there may also be the presence of pleural fluid; • in severe pneumonia, the blood pressure falls. 87
Respiratory Disorders
ASSESSMENT Routine assessment of all patients with pneumonia includes observation of the following points: • temperature; • pulse rate; • blood pressure; • oxygen status, either using oximetry or measuring blood gases (preferable); • measurement of respiratory function with peak flow or spirometry; • blood count to include white cell count, urea and electrolytes; • blood cultures; • sputum culture to include tuberculosis; • plain PA chest X-ray – usually shows area of infection – some causes of pneumonia show cavities (see figures 5.2 and 5.3); • antibodies for mycoplasma and legionella; • viral studies may also be performed. In 50% of cases, no cause is ever identified. Figure 5.2: active pulmonary tuberculosis
Note the cavity in the left side mid-zone. 88
Pneumonia Figure 5.3: tomogram of figure 5.2 confirming cavitation and showing area of calcification
CAUSES OF CAVITATING PNEUMONIA Infectious causes Organisms causing cavitating pneumonia include: • bacteria: ❍ staphylococcus aureus; ❍ mycobacterium tuberculosis; ❍ oral anaerobes (bacteroides species); ❍ nocardia species; 89
Respiratory Disorders • fungi: ❍ histoplasmosis; ❍ coccidioidomycosis; ❍ blastomycosis; ❍ Aspergillus.
Non-infectious causes Non-infectious causes of cavitating pneumonia include: • neoplasms; • Wegener’s granumolatosis; • infarction; • infected bullae and cysts.
PRINCIPLES OF PNEUMONIA MANAGEMENT The majority of patients with community-acquired pneumonia are treated in their homes by their GP but about 30% of patients require hospital admission. Criteria for hospitalisation of patients with pneumonia include the following points: (a) elderly (> 85 years of age); (b) significant other disease (for example, kidney, heart, or lung disease; diabetes mellitus; neoplasm; immunosuppression); (c) leukopaenia (< 5,000 white blood cells/10 9 /litre or leukocytosis > 20,000/109/litre); (d) staphylococcus aureus, gram-negative bacilli or anaerobes as the suspected cause of pneumonia; (e) complications (for example, empyema, arthritis, meningitis, endocarditis); (f) failure of out-patient management; (g) inability to take oral medication; (h) tachypnoea (> 30/min); tachycardia (> 140/min); hypotension (< 90 mm Hg systolic); hypoxaemia (arterial PO2 < 60 mm Hg); acute alteration of mental status; elevated blood urea; (i) inadequate social support.
90
Pneumonia
Hospital admission Urgent hospital admission is required if the patient cannot be managed at home. Figure 5.4: antibiotic therapy for home management of community-acquired pneumonia PATHOGEN
AUGMENTIN DOXYCYCLINE
CLARITHROMYCIN
CIPROFLOXACIN
Streptococcus pneumoniae
+a
+
+
+
Haemophilus influenzae
+
+
+
+
Mycoplasma pneumoniae
–
+
+
+
Chlamydia pneumoniae
–
+
+
+
Legionella pneumophila
–
+
+
+
Anaerobes
+
–
–
–
+ effective – ineffective + sometimes effective a Up to 10% resistant Usual initial recommended choice:
amoxycillin or augmentin or clarithromycin
91
}
orally
+
– – –
Anaerobic gram-positive cocci
Anaerobic gram-negative bacilli
Chlamydia
Legionella pneumophila
–
Mycoplasma pneumoniae
–
–
Staphylococcus aureus
Haemophilus influenzae
+
PENICILLIN G
Streptococcus pneumoniae
PATHOGEN
92
+
+
–
+
+
+
–
+
CLARITHROMYCIN
–
–
–
+
+
-
+
+
THIRD GENERATION CEPHALOSPORIN
–
–
+
+
–
–
–
–
METRONIDAZOLE
–
–
+
+
+
–
+
+
AMOXYCILLIN
Figure 5.5: antibiotic therapy for in-patient management of community-acquired pneumonia
Respiratory Disorders
Pneumonia + effective
–
Usual initial choice:
ineffective augmentin or amoxycillin plus clarithromycin
}
intravenously (iv)
Figure 5.6: antibiotic therapy for hospital-acquired pneumonia PRESUMPTIVE AETIOLOGY
ANTIBIOTIC
Staphylococcus aureus
Flucloxacillin or vancomycin iv
Enteric aerobic gram-negative bacilli or pseudomonas aeruginosa
(a) ceftazadime iv (b) imipenem +/- aminoglycoside iv (gentamicin) (c) aminoglycoside or beta lactam iv
Mixed flora
(a) ceftazadime, metronidazole +/– aminoglycoside (b) imipenem +/– aminoglycoside iv
Antibiotics Intravenous if the patient is severely ill, oral otherwise. The choice of antibiotics initially is selected on a ‘best guess’ basis until blood cultures and sputum results become available (see above, figures 5.4, 5.5 and 5.6). For most community-acquired pneumonias the choice would be a combination of clarithromycin and amoxycillin or equivalent drugs. For hospital-acquired infection, the choice of antibiotics needs to include cover of gram-negative organisms, particularly pseudomonas (ciprofloxacin would be such an antibiotic), and cover should include staphylococci in hospital-acquired pneumonia or following influenza (flucloxacillin). The antibiotics need to be given as soon as the diagnosis is made. Antibiotics should be changed if there is failure to respond. Causes of failure to respond to antibiotics include: • incorrect microbiological diagnosis; • inappropriate antibiotics; • drug hypersensitivity; • empyema, superinfection; • atelectasis, pleural effusion, phlebitis; 93
Respiratory Disorders • poor host defences; • endobronchial obstruction; • life-threatening other diseases.
Oxygen therapy All patients need to have their oxygen saturation measured by oximetry. However it is preferable to have blood gases so that one can measure the acid base status and the degree of ventilation (PaCO2). Oxygen needs to be given continuously in patients who are hypoxic, in order to try and return the oxygen levels to normal.
Bronchodilators Such as nebulised salbutamol and ipratropium need to be given to all patients in whom there is significant co-existing airways obstruction. If this exists, oral steroids are also appropriate.
Intravenous fluids If the patient is dehydrated or if their blood pressure is low, they require intravenous fluids in order to rectify this.
Physiotherapy Useful in order to loosen secretions and aid expectoration and clear airways.
Assisted ventilation If all else fails, consideration needs to be given to assisted (artificial) ventilation, at an early stage, prior to there being a cardio respiratory arrest. In patients in whom the pneumonia complicates chronic obstructive airways disease, there is severe hypoventilation. A respiratory stimulant such as doxapram may be a useful step prior to assisted ventilation.
PROGNOSIS IN PNEUMONIA The prognosis of an episode of pneumonia depends not only upon the causative organism but also on a variety of other factors. Those which, over the years, have been found to correlate with a poor prognosis include: (a) increasing age; 94
Pneumonia (b) co-morbidity – the co-existence of other severe diseases such as heart failure, renal failure, cancer; (c) multilobe involvement on chest X-ray; (d) diastolic blood pressure below 60 mm Hg; (e) raised blood urea; (f) very high (>20 x 10 109/litre) or very low (<5 x 10 109/litre) white blood cell count; (g) atrial fibrillation; (h) immunosuppression with HIV infection.
MEDICO-LEGAL ASPECTS OF PNEUMONIA As with any severe medical condition, regrettably deaths occur, even with the best medical therapy – and sometimes the patients are young people. The diagnosis and treatment of pneumonia is an area where there are commonly grounds for anger, resentment and blame towards the doctor if the patient dies. Equally well, it is an area in which there can be many potential shortcomings in the treatment and management by the GP or hospital physician. These problems may be divided into: • failure of diagnosis; • failure to assess severity; • failure of therapy: ❍ failure to give oxygen; ❍ failure to choose appropriate antibiotics or delay in giving them; ❍ failure to rehydrate adequately with intravenous fluids; ❍ failure to give bronchodilators; • failure to institute artificial ventilation; • a difference of opinion over the appropriateness of active therapy, particularly in the very elderly.
Failure of diagnosis Case 9: pneumonia – failure to diagnose leading to death A 55 year old man went camping for his summer holiday. Whilst away on holiday, he became unwell with marked prostration, diarrhoea, abdominal pains and breathlessness. He called the local general practice and one of the partners came to visit him on the campsite as an emergency. He was seen at 10.00 pm. There was a record of a full examination, including listening to the 95
Respiratory Disorders chest and feeling the abdomen. A diagnosis of gastroenteritis was made and appropriate therapy was given. The following day, he was no better and again, that evening, the GP was called out. This time, a different doctor arrived and he once again recorded a full examination, including examining the chest. He also felt that the condition was one of gastroenteritis and did not feel that he was ill enough for hospital admission. During the night, he deteriorated further and was tragically found dead by his wife the following morning. Legal proceedings were started against the GPs after the coroner’s post mortem found that he had died from an extensive upper lobe pneumonia. It was pointed out by the respiratory medical expert that, if he had arrived at hospital, a chest X-ray would have confirmed the diagnosis, after which he would have been started on appropriate intravenous antibiotics, given oxygen and intravenous fluids and, almost certainly, would have survived. The GP expert, however, felt that there had been no negligence, in that the GPs had attended when asked to, and had performed full examinations in difficult circumstances (in a tent, on a campsite, in the dark). They had obviously examined his chest and, despite this, neither of them had found abnormal physical signs. It was, therefore, felt reasonable that many other GPs in these circumstances would have confirmed a diagnosis of gastroenteritis and would not have referred him to hospital. They would have given the same treatment of oral rehydration and would not have given antibiotics. Thus, the overall standard of care (although resulting in the patient’s death) was not felt to be below an acceptable standard. Case 10: pneumonia death – complicating asthma and pneumothorax – whose responsibility? A 45 year old asthmatic with chronic alcohol abuse had a love/hate relationship with her local GP and would only attend the surgery on rare occasions. She was, however, attending a psychiatrist on a regular basis in order to have treatment for her alcohol problem. There was regular, good communication between her family, herself and the psychiatrist. As far as her asthma was concerned, she appeared on a ‘repeat prescription’ basis with her GP and, despite being asked, on numerous occasions, to come to the surgery for assessment, she would always refuse and, when appointments were made, her mother would attend on a proxy basis. When she developed pleurisy and increasing breathlessness, she refused, despite her mother’s encouragement, to visit her GP, but she did agree to see the psychiatrist. The psychiatrist saw her, did not examine her, but advised her to go to see her GP. The psychiatrist gave no treatment for her increasing
96
Pneumonia breathlessness and pleurisy. Things got worse. She became more debilitated, increasingly breathless and generally more prostrate. Her husband contacted the psychiatrist, who saw her again, did not examine her but, again, tried to persuade her to see her GP. Things continued to deteriorate over another four or five days, until her mother eventually called an ambulance to take her to hospital, but she died en route. The coroner’s post mortem found that, in addition to her chronic asthma, she had experienced a pneumothorax on one side and an extensive pneumonia in the other lung. Her widower wished to take legal action against the physicians looking after her. The advice from the respiratory expert was that this was an avoidable death, in that her asthma, pneumothorax and pneumonia could (and should) all have been treated successfully. However, it was quite clear that no blame could be laid at the door of the GP, because he had, on numerous occasions, tried to get the patient to come to see him, but she had always refused. Indeed, it appeared she was using the psychiatrist as her GP. Although the respiratory physician and the GP expert felt that the psychiatrist could and should have done better by examining the patient, arriving at a diagnosis and treating her or referring her to the hospital, the psychiatric expert was of the view that the remit of the psychiatrist was only to treat her alcohol abuse and that he had no responsibility for the organic disease occurring in her chest. The case was therefore discontinued. Case 11: pneumonia death because of inadequate assessment A 35 year old man was admitted to hospital on Good Friday. He was noted by the SHO, on his admission chest X-ray, to have a bilateral pneumonia. The initial blood gases showed him to be in respiratory failure with a very low oxygen and very low peak flow, a rapid pulse rate, but a normal blood pressure. He was febrile. He was seen by the consultant on the post-take ward round at 8.00 am, and the findings and diagnosis were confirmed. Subsequently, he appeared to have no further observations until 5.00 pm, when the SHO was called to see him, at which time, he had a cardio respiratory arrest, from which, although there was, initially, successful resuscitation, he suffered prolonged cerebral hypoxia and irreversible brain injury. He died in intensive care. The respiratory expert was of the opinion that, in this case, there was a failure to have continued adequate clinical assessment of him once he had been found to be low in oxygen (hypoxic). In particular, oxygen saturation was not monitored. He had no further observations made of his temperature, pulse rate, respiratory rate or blood pressure which should all have been measured at least four hourly (preferably, two hourly). He had not been
97
Respiratory Disorders treated with oxygen and his blood gases had not been repeated after admission. Whilst the defence found difficulty in denying liability and a failure of care, their argument was that he had such an overwhelming pneumonia on admission that regardless of any other intervention, he was likely to die anyway. Case 12: pneumonia – failure to recognise severity of illness and inadequate therapy A middle aged man was admitted to hospital via Accident and Emergency, on a Saturday, with typical pneumonia. The chest X-ray showed bilateral changes. The diagnosis was made within 20 minutes of arriving in Accident and Emergency. Although the diagnosis was made at noon and an appropriate combination of antibiotics was written up by the house physician at this time, the first dose of antibiotics was not administered until 5.30 pm. His oxygen status was not measured (either by oximetry or blood gases) on admission and he was not administered oxygen or intravenous fluid. He continued to deteriorate during the course of the evening and his relatives were very worried about him, but were eventually sent home by the ward staff and told to stop worrying. At 3.00 am, the duty SHO was called to the ward. The patient’s blood pressure was unrecordable and he looked blue. The blood gasses were measured, which showed him to be severely hypoxic and acidotic. The SHO started intravenous fluids and oxygen. His blood pressure did not respond to the flow rate of IV fluids chosen and the response to oxygen was not recorded. At 9.00 am, he was reviewed and the record ‘no improvement’ was made in the notes. Blood pressure had still not recovered to normal. He was still blue and still the oxygen level was not measured. At 5.00 pm, he (predictably) had a cardio respiratory arrest, he was resuscitated and has survived with extensive brain damage. In this tragic case, the general physician expert pointed out that not only was there an inadequate assessment in the beginning, with failure to measure blood oxygen levels or oximetry, there was also a delay in giving antibiotics (even though they were the appropriate choice). The treatment at 3.00 am, although along the right lines, gave an inadequate volume of intravenous fluids to restore blood pressure, combat dehydration and the circulatory failure. The amount of oxygen given was inadequate. There had been failure to involve a more senior doctor or to consider transfer to the intensive care unit for more aggressive treatment – including artificial ventilation. By 9.00 am, the situation was worse and yet still no consideration of intensive therapy was given. The fact that he recovered after the cardio respiratory arrest was good evidence that, if the cardio respiratory arrest had been averted by more aggressive earlier therapy, the situation could have been remedied. The case
98
Pneumonia was indefensible and, once again, exhibited the dangers of sick patients being put on understaffed wards, managed by inexperienced physicians at weekends!
Note Case 12 is a typical example of failure to institute artificial ventilation at an early stage. It is always advisable to ventilate electively rather than to wait for a cardio respiratory arrest. The indications for artificial ventilation are worsening respiratory failure and exhaustion. These require early recognition of the situation and rapid action. The case described is typical of its type which are all characterised by an under-assessment or a failure to recognise the severity of the patient’s condition, leading to a delay in artificial ventilation. The second area in which there can be a medico-legal interest in the failure to ventilate is over the decision on clinical grounds as to whether artificial ventilation is appropriate or not. Artificial ventilation is appropriate for respiratory failure for conditions which are reversible, such as pneumonia. However, it is accepted that is inappropriate if there is severe co-existing disease, such as a bronchial carcinoma or end-stage chronic obstructive pulmonary disease, where the quality of life prior to the acute event has been unacceptably poor. The vast majority of physicians involve the patient and their relatives in this decision making process, and it is usual for the discussion and decision not to ventilate to be recorded in the notes. On occasion, even though this has been the case, there has been an inquiry about potential legal action because (in hindsight) the relatives felt their loved one should have been ventilated. It is self-evident that on many occasions the relatives and doctors may have been talking at cross purposes but, nonetheless, the doctors had tried to involve the patients and obtain their consent in each case. In the elderly, pneumonia is frequently regarded as the ‘old man’s friend’, being a terminal event at the end of a natural life span. Problems may come, however, in the young chronically disabled, of which Case 13 is an example. Case 13: pneumonia – decision not to ventilate A 47 year old woman with multiple physical and mental handicaps was cared for very attentively at home by her mother. She developed a severe pneumonia, was admitted to hospital and, despite excellent assessment and clinical management, continued to deteriorate and went into worsening respiratory failure. There was a very full record in the clinical notes about a discussion with her mother concerning the fact that she was going to die if not ventilated, and a record of the pros and cons of artificial ventilation was made. The fact that the doctors felt it inappropriate to ventilate in view of her long standing physical and mental disabilities and poor quality of life was 99
Respiratory Disorders recorded. Furthermore, it was recorded in the medical and nursing notes that her mother had agreed with this decision. She continued to deteriorate and died from the pneumonia. Within a few months of the death, her widowed mother was seeking a medico-legal opinion, with a view to suing the hospital trust because of their failure to ventilate her daughter, in particular, she alleged that she had never been consulted in the decision process as to whether to ventilate. During the investigation, it became clear from the medical records that she had been kept informed and consulted by the physicians and nurses throughout. It was recorded in several places in the notes that the medical staff had felt that they had her agreement with the policy. The independent opinion felt that many other physicians would also not have offered artificial ventilation in these circumstances. The trust was applauded for their excellent practice in involving the mother and their good record keeping. It was pointed out that, frequently, relatives or patients are extremely worried and do not take on board discussions. Thus, they often have no recollection of them. It is also the case that the death of a handicapped child or dependant comes as an extremely severe blow to long term carers, who have often given up their entire lives to care for the handicapped person. The shock of the death and resultant emptiness experienced in their lives leads to a grief reaction characterised by a need to find people to blame, even though this may be inappropriate.
100
CHAPTER 6
CHRONIC BRONCHITIS AND EMPHYSEMA
INTRODUCTION Chronic bronchitis is defined as the presence of a cough productive of sputum (phlegm) on most days for three successive months in two consecutive years. It should be differentiated from emphysema, which is a pathological condition involving dilation of the terminal air spaces and destruction of alveolar walls. This turns the lungs from what is, in health, a very fine sponge into a very coarse sponge with loss of surface area over which gas exchange and absorption of oxygen (into the bloodstream) can occur. Both conditions may co-exist to a greater or lesser extent, with one or other predominating. Increasingly, the pattern of patients seen is one without sputum production but with the other features of chronic bronchitis, that of narrowing of the air passages leading into the lung, resulting in wheeze and breathlessness. Hence, the terms chronic obstructive airways disease (COAD), chronic obstructive pulmonary disease (COPD) or chronic obstructive lung disease (COLD) are used to describe patients with this group of conditions. Indeed the terms are often used interchangeably in clinical practice. The British Thoracic Society has published guidelines in the management of chronic obstructive pulmonary disease (see Thorax (1997) 52 Suppl 5 S1–S28), which should be read in conjunction with this chapter.
Causes The most common causes of bronchitis and emphysema are: • tobacco smoking; • atmospheric pollution (including industrial).
Symptoms The symptoms of bronchitis and emphysema are: • coughing (beginning as a smoker’s cough); • sputum – clear (but may be discoloured – yellow or green, with attacks of infection); • wheezing – breathing is noisy, especially in expiration; • shortness of breath – gradual reduction in exercise capability, leading eventually to breathlessness at rest. 101
Respiratory Disorders
Clinical features Early in the disease there are none, but gradually one or more of the following occur: • hyperinflation of the chest; • bilateral expiratory wheezing; • reduced peak flow rate (and FEV1).
Complications Acute exacerbations (attacks) Usually caused by bacterial infection. These are typified by increased episodes when there is increasing breathlessness and increasing (discoloured – yellow or green) sputum production.
Pneumonia With more severe infection, the alveoli may also be infected, resulting in a pneumonia rather than there being infection only in the airways (bronchitis). In this case the patient will be sicker.
Right heart failure (cor pulmonale) In chronic bronchitis, the resistance of the blood vessels going through the lungs rises over the years. This leads to strain on the right side of the heart. The main clinical symptoms the patient notices are of ankle swelling and increasing breathlessness. The physician will note the added clinical signs of elevation of the jugular venous pressure and hepatomegaly, as well as the peripheral swelling (oedema).
Respiratory failure In acute infective attacks of COPD (or in end-stage disease) due to the airways narrowing, there is a reduction in the level of oxygen being carried in the blood (PaO2 – hypoxia), together with carbon dioxide retention (elevation of the PaCO2). This type of respiratory failure is known as Type 2 respiratory failure, and this group of patients is sometimes referred to as ‘blue bloaters’, due to their appearance – often overweight, blue and somnolent. Type 2 respiratory failure may be a chronic situation in some patients, but when presenting as an acute new problem, it is a medical emergency and requires urgent action (Type 1 respiratory failure is a low oxygen with normal PaCO2, as seen in acute pneumonia).
102
Chronic Bronchitis and Emphysema
Secondary polycythaemia Chronic lack of oxygen causes increased reproduction of the hormone erythropoietin from the kidneys, which stimulates the bone marrow to produce red blood cells. Thus, in some patients with COPD, there may be an elevation of the haemoglobin concentration where there has been prolonged hypoxia for months or years.
Lung cancer The incidence of lung cancer in male chronic bronchitics is more than double the expected figure, as the diseases have a common cause, that is, smoking.
Pneumothorax A bulla may rupture if there is co-existent emphysema: this leads to a ‘punctured lung’ with sudden increase in breathlessness, together with chest pain on the affected side.
Investigations The investigations performed in patients with chronic bronchitis when referred to hospital will depend upon whether it is a routine out-patient referral or an Accident and Emergency attendance.
Routine out-patient referral One would expect a history and examination of the patient’s chest to be made with a routine PA chest X-ray and lung function tests (spirometry). Usually, the FEV1 and FVC and the peak flow rate are all measured. Serum alpha 1 antitrypsin level should be measured in any patient with basal emphysema under the age of 50, particularly if they are a non-smoker and have a family history of lung disease. Additional investigations which might be made would include: • blood count; • urea and electrolytes; • liver function tests; • estimation of blood gases – PaO2 and PaCO2; • oxygen concentration – oximetry; • ECG. During infective exacerbations one would expect:
103
Respiratory Disorders • sputum to be sent off to culture for pathogens, such as haemophilus influenzae and streptococcus pneumoniae. If there is shadowing on the X-ray, which failed to clear with routine antibiotics, then sputum should also be examined for tuberculosis (acid-fast bacilli); • if there is any suspicion of a mass lesion on chest X-ray, further investigations (as described in Chapter 8) should be performed to exclude a co-existent lung cancer (sputum for cytology, fibreoptic bronchoscopy, CT scan of chest and percutaneous needle biopsy).
Therapy Routine out-patient visits • Smoking – cigarette smoking should be discouraged in order to stop further deterioration in lung function. If smoking continues, deterioration inevitably occurs. • If there are specific atmospheric causes at work then these should also be avoided or special breathing apparatus provided. • Bronchodilators – by definition, there is little reversibility in the airways obstruction in chronic bronchitis. Nevertheless, inhaled betagonists (salbutamol and terbutaline) are useful and anticholinergics (such as inhaled ipratropium) may also be tried. These can also be used in meter dose inhalers (or by nebulisers for severe disease). • Corticosteroids – as yet, there is little evidence that either oral or inhaled steroids play an important part in the routine maintenance and therapy of chronic bronchitis. Current research is being performed into this area. However, when there is doubt as to whether a patient might be a chronic asthmatic with reversible airways obstruction, rather than a chronic bronchitic with a irreversible airways obstruction, a two week trial of oral prednisone, 30 mg daily, should be tried to check the maximum degree of reversibility and hence exclude asthma. All new patients referred to hospital clinics with what appears to be COPD should have a trial of steroids to check their maximum reversibility and exclude asthma. It is convention for high dose oral steroids to be given for acute exacerbations of chronic airways obstruction, although there is little definite evidence to support their efficacy. However, the dangers of missing an asthmatic outweigh the dangers of giving a short course of steroids to chronic bronchitics with irreversible disease. • Mucolytic drugs – there are a number of mucolytic drugs whose role is to thin the sputum, none with convincing benefit.
104
Chronic Bronchitis and Emphysema • Antibiotics for acute infective exacerbations – broad spectrum antibiotics, such as amoxycillin, ampicillin, oxytetracycline and augmentin are used to cover haemophilus influenzae and streptococcus pneumoniae. These should be started immediately an acute attack starts and prior to the sputum culture results being available. • Diuretics – once cor pulmonale has set in, diuretics, such as frusemide, together with amiloride are indicated. • Oxygen – there is evidence that chronic oxygen therapy given at two litres/minute for 16 hours a day, by an oxygen concentrator at home, delays the onset of pulmonary hypertension and cor pulmonale and prolongs life in the chronically hypoxic patients.
Drugs to be avoided Opiates, such as diamorphine and morphine, or sedatives, such as sleeping pills, should be avoided because they depress respiratory drive and might precipitate respiratory failure. Beta blocking drugs may precipitate worsening of bronchospasm.
The acutely ill patient warranting hospital assessment/admission At this stage, the investigations which were optional in out-patients become mandatory: • blood count; • urea and electrolytes; • liver function tests; • estimation of blood gases, PaO2 and PaCO2; • oxygen concentration (oximetry); • ECG; • chest X-ray; • sputum and blood cultures.
Therapy of acute exacerbation • Oxygen – continuous oxygen is normally given at 24% initially, increasing according to blood gas results. It is important to check the PaCO2 to make sure this is not rising. This would indicate that, paradoxically, the patient was breathing less, rather than more. This occurs if there is already significant depression of the respiratory centre and it is running entirely on a low oxygen to stimulate it. If this is the case and too much oxygen is given, the patient may breathe less and less and stop breathing altogether. Hence, it is essential that the oxygen concentration is controlled carefully 105
Respiratory Disorders
• • •
• • • •
•
to avoid depressing respiration. These patients are insensitive to rises in the PaCO2, which is the the drive for respiration in normal people. Broad spectrum antibiotics – such as amoxycillin or augmentin initially given intravenously if the patient is febrile and very sick. Chest physiotherapy – to aid expectoration of the sputum. Bronchodilators – usually given via a nebuliser – usually salbutamol (or terbutaline) together with ipratropium. Oral or intravenous aminophylline may be used in addition. Diuretics – if there is a co-existent cor pulmonale. Digoxin – if there is co-existing atrial fibrillation. If the patient is in respiratory failure and failing to respond – an infusion of doxapram may be tried. Respiratory support – artificial respiratory support techniques, such as NIPPV (non-invasive positive pressure ventilation), may be tried in the ward, but eventually paralysis and intubation using an endotracheal tube and full artificial ventilation in ITU might be needed. Decisions to institute assisted ventilation depend upon: (a) whether the underlying problem is a new one which can be reversed, such as a pneumonia or an overdose of a sedative; (b) the patient’s pre-existing exercise tolerance and quality of life. Intercostal drain and underwater seal – only necessary if there is a pneumothorax. Even a small pneumothorax may be life-threatening in COPD because of the poor respiratory reserve – it will need urgent treatment with an intercostal tube to re-inflate the lung.
Alpha 1 antitrypsin deficiency Alpha 1 antitrypsin is an inhibitor of enzymes such as elastase which are released during pulmonary inflammation caused by infection or inhalation of pollutants such as cigarette smoke. These enzymes cause destruction of the alveolar wall leading to panlobular emphysema. Alpha 1 antitrypsin deficiency is a genetic disorder resulting in progressive breathlessness and basal emphysema, particularly in younger patients (under 50), who may even be non-smokers. As well as lung disease, liver cirrhosis may also occur. It is particularly important to advise patients with possible alpha 1 antitrypsin deficiency to avoid working in polluted atmospheres. Their relatives should also be checked for alpha 1 antitrypsin deficiency, so that the relatives can be advised appropriately about avoiding smoking and occupational exposure. Ideally, this should be done in childhood after their parents have initially been diagnosed.
106
Chronic Bronchitis and Emphysema Figure 6.1: large bilateral basal bullae due to emphysema caused by alpha 1 antitrypsin deficiency
MEDICO-LEGAL ASPECTS The most frequently encountered medico-legal aspects of COPD and its management are: (a) problems with the management of acute exacerbations; (b) co-existing diseases; (c) ability to work; (d) life expectancy.
107
Respiratory Disorders
Management problems Inadequate assessment There may be failure to recognise the severity of an exacerbation of chronic bronchitis. In particular, respiratory failure may not be recognised, leading to inadequate or inappropriate treatment and, regrettably, death. Fortunately, there are relatively few incidents falling into this category and one has to recognise that this is a serious illness, occurring usually in elderly patients, with a relatively high mortality whatever the therapy, and in any case a limited life expectancy. However, there have been numerous cases around the more contentious area of whether the patient was fit for discharge. Typically, the patient is discharged after an acute exacerbation of COPD and treatment in hospital – often they have been discharged at a relatively early stage because of the everpresent ‘pressure on beds’, at a point where the relatives did not feel totally happy with taking their relative home. If the patient develops a further infection and dies at home, either from a further infection or from co-existing diseases such as coronary thrombosis (also linked to the same causative factor – cigarette smoking), there is resentment and anger in the relatives and a visit to their lawyer. Rarely has there been negligence. Indeed, infections are more common in hospital and, in fact, the longer a chronic bronchitic is kept in hospital, the more likely they are to pick up a further (secondary) infection and return to ‘square one’. Thus, good practice should be to encourage early discharge in the patient’s interest once the acute episode is over.
Inappropriate use of drugs There are several groups of drugs, which are respiratory depressants, such as opiates (for example, morphine) or benzodiazepines (such as night sedatives – diazepam, etc). Both of these will reduce respiratory drive and in patients with acute attacks of chronic obstructive airways disease they may cause a patient to stop breathing altogether and die. It is inappropriate to use such drugs unless the relative risks have been worked out. It is good medical practice to avoid them. However, there comes a stage, as part of good palliative (terminal) care, when a patient with chronic bronchitis and emphysema is distressed by severe breathlessness to such an extent that these groups of drugs may be used to reduce the anxiety and feeling of breathlessness. This is legitimate so long as they are used in doses which are intended to alleviate symptoms, rather than immediately cause cessation of breathing and death. Their use in small doses in these situations can be very helpful. The patient should be
108
Chronic Bronchitis and Emphysema personally assessed by the prescriber before they are given and the restrictions around the prescribing of these drugs should be adhered to.
Artificial ventilation Frequently, patients with long standing severe chronic obstructive airways disease have a very poor quality of life. Although it is theoretically possible to prolong their life (albeit for a day or two) by offering artificial ventilation in ITU if they get a co-existing severe pneumonia and are very sick, often a medical decision is made that this is ‘inappropriate’. There are a number of cases where this has been a bone of contention between the executors of the patient and the hospital trust concerned. Usually, the problem is that the relatives did not realise how ill the patient had been and how poor the prognosis was. In some cases, the doctors had had conversations with the relatives, fully recorded in the hospital notes, about how poor the prognosis and outlook were, and yet the relatives had not taken this on board. Similarly, the doctors had not realised that the relatives had failed to understand. Improved communications could have avoided the departure into expensive and unsuccessful legal action.
Co-existing diseases Lung cancer Lung cancer is more frequent in patients with chronic bronchitis and therefore it is inevitable that patients who are being ‘routinely’ followed by their general practitioner or the hospital out-patients for their chronic bronchitis will develop lung cancer and die. There is little or no evidence to suggest that the regular screening of patients with chest X-rays to make an early diagnosis of lung cancer is helpful. It is not the routine practice of chest clinics or general practitioners to do routine chest X-rays whilst following up this group of patients. Thus, it would be totally unreasonable for a patient to try and make a claim that they were being followed up for chronic bronchitis and that lung cancer was missed because an X-ray was not taken. However, if an X-ray had been taken and a co-existing lung cancer was missed on the X-ray, this could be a different matter. Nevertheless, often the severity of the obstructive airways disease means that the failure to diagnose has not altered prognosis. This is because curative surgery and hence the ability to argue that the patient might live for five years with treatment is not possible because of inadequate respiratory function for anaesthetic and operation. 109
Respiratory Disorders Situations have occurred where patients with COPD have been admitted to hospital with pneumonia and the fact that the pneumonia was caused by a lung cancer narrowing the airways has been missed. A month or two later, the lung cancer is then diagnosed, much to the anger of the patient, who usually proceeds to instruct a solicitor. Nonetheless, it is not indicated in every patient with COPD who develops a pneumonia to undertake more extensive investigations (such as CT or bronchoscopy) at the time to exclude a lung cancer. However, further investigation is essential if there is suspicion of a mass on CXR and the pneumonia on CXR should be followed to resolution (clearing of CXR). In any case, the severity of the COPD would often preclude any curative surgery.
Heart attacks Coronary thrombosis, lung cancer and chronic bronchitis share a common aetiology – smoking. It is, therefore, not surprising that a patient with an attack of chronic bronchitis may have a heart attack simultaneously and die. This is a cause of unexpected death during hospitalisation or shortly after discharge from hospital. This is upsetting to the attending physicians and to the relatives, but is neither’s fault and cannot be avoided. Better communication and education of relatives could avoid some of the possible legal claims in these areas.
Ability to work A person’s ability to perform a regular job depends upon how active the job is and hence the amount of energy and oxygen that are required to perform that job. There is clearly a large difference between driving from the front door of one’s house to an office building, walking inside and sitting at a desk all day, as opposed to walking two miles to work every day over hilly terrain and then doing an active manual job, say, as a bricklayer, going up and down a ladder carrying bricks. Chronic bronchitis because of the airways narrowing and loss of respiratory function leads gradually to increasing breathlessness, as well as the additional acute infective exacerbations. Initially, work will be lost during infective exacerbations with relative normality in between, but eventually when respiratory function falls beneath a certain level, active work becomes impossible. The level below which work becomes impossible varies a little between individuals, but is more dependent to occupation (and oxygen requirement). The quantum of a disability claim therefore depends upon the natural rate of decline in respiratory function and:
110
Chronic Bronchitis and Emphysema (a) whether the claimant was able to work at the time of the accident and for how many years he would have been able to continue after it; (b) whether without the incident the claimant would have been able to continue his normal jobs; (c) whether or not the claimant would have continued to work until normal retirement age. The ability to work depends on many factors, including: (a) the patient’s determination to work; (b) the attitude of the patient’s employer; (c) respiratory function and exercise capacity – this can be measured by the physician in the clinic and by a six or 12 minute walk, or the number of stairs that can be climbed; (d) the nature of the job and any modifications that can be made to it. At the end of the day, clinical judgment may well vary between one experienced clinician and another. It is not an exact science.
Life expectancy Overall, life expectancy is reduced, particularly with severe chronic obstructive airways disease, as is the length of time that a patient can continue working in full time employment. Again, there is little guidance in the published literature and therefore an inspired guess based on clinical judgment and past experience is the best that can be done. Often, what appears to happen is that the lawyers representing the two sides obtain estimates from their physicians and then take the average.
111
CHAPTER 7
PULMONARY EMBOLISM AND DEEP VEIN THROMBOSIS
INTRODUCTION The diagnosis of pulmonary embolism (blood clots in the lung) is fraught with difficulties for the clinician. It is one of the most frequently under-diagnosed conditions and also on clinical grounds one of the most frequently incorrect diagnoses. The cause of pulmonary emboli is blood clots (or sections of them) breaking off from within the deep veins either in the legs or the pelvis. These are then carried up in the venous system through the right side of the heart and then into the lungs where they lodge in the pulmonary arteries. Depending upon the size of these blood clots and the size of pulmonary artery blocked, so the symptoms will vary. The degree of overall circulatory disturbance depends upon the number, size and distribution of the emboli. Small pulmonary emboli may be asymptomatic, large ones can be (and often are) fatal. Pulmonary emboli are found in up to 25% of all patients at post mortem. The British Thoracic Society has published guidelines in the management of pulmonary embolism which should be read in conjunction with this chapter (Thorax (1997) 52 Suppl 4 S1–S24).
CLINICAL FEATURES
Massive pulmonary emboli These are relatively easy to diagnose in that they present with a lifethreatening illness characterised by: • sudden, severe chest discomfort; • acute severe breathlessness; • cyanosis (blue discolouration); • fast pulse; • elevated jugular venous pressure; • collapse and possibly death.
113
Respiratory Disorders
Smaller pulmonary emboli These may present with some of the following features: • pleuritic type chest pain; • coughing of blood; • breathlessness; • fever; • there may be a pleural friction rub on listening to the chest. Massive or smaller pulmonary emboli may be associated with signs of peripheral deep vein thrombosis, but this is no means universal.
Predisposing factors These may provide helpful clues in the history and the following points should be considered: • recent surgery – especially abdominal, pelvic or large orthopaedic operations; • prolonged immobility; • long intercontinental air flights; • dehydration; • pregnancy; • oral contraceptive pill; • hormone replacement therapy; • rare familial disorders, such as antithrombin III deficiency, protein-S deficiency or lupus anticoagulant syndrome; • malignancy (particularly lung and pancreatic cancer).
DIAGNOSIS AND INVESTIGATIONS First, a careful history and examination are necessary. Then there needs to be examination of the chest and heart and of the limbs to examine for a deep vein thrombosis (swollen painful leg). On physical examination, it is usually impossible to confirm or refute the diagnosis of pulmonary embolism with confidence, hence further tests are needed.
114
Pulmonary Embolism and Deep Vein Thrombosis
Essential investigations Chest X-ray PA This is usually normal in acute episodes, but there may be reduced vascular markings, pulmonary infiltrates or linear atelectasis. Later, wedge-shaped shadows may be evident with pulmonary infarction. It is often very difficult to differentiate between the X-ray of pulmonary emboli and infection (pneumonia). Figure 7.1: multiple pulmonary infarcts
Note the wedge shaped shadows at the right base.
115
Respiratory Disorders Figure 7.2: ECG showing acute pulmonary embolism
ECG: in a minority (about 15%) of patients there is a classical abnormal appearance of S1, Q3, T3 pattern.
Blood gas estimation or oximetry This shows hypoxia due to ventilation perfusion mismatch and reduced PaCO2 due to hyperventilation.
Ventilation perfusion lung scan A radioisotope lung scan showing multiple defects in perfusion which are not matched by defects in ventilation are diagnostically helpful because in infection the defects are matched with similar defects in both ventilation and perfusion. To be confident of the diagnosis of pulmonary embolism on a lung scan, the mismatched defects should be multiple.
116
Pulmonary Embolism and Deep Vein Thrombosis Figure 7.3: perfusion radioisotope lung scan showing multiple perfusion defects compatible with pulmonary emboli
117
Respiratory Disorders Figure 7.4: normal ventilation scan of the same patient confirming the perfusion defects to be due to emboli
Spiral CT scanning of lungs This is useful in diagnosing large proximal blood clots but they may be less useful and miss subsegmental defects; the clinical importance of this is unclear as yet. Currently, the spiral CT is taking over as the first line investigation (done together with venous Döppler studies of the legs).
Pulmonary angiogram The ‘gold standard’ is a pulmonary angiogram, but it is invasive and not without hazard. However, it may need to be performed in certain circumstances particularly, for example, if a decision is being made whether to go onto lifetime anticoagulation or not. 118
Pulmonary Embolism and Deep Vein Thrombosis Figure 7.5: multiple pulmonary emboli
Note the loss of vessel markings, particularly in the right mid-zone.
119
Respiratory Disorders Figure 7.6: pulmonary angiogram of the same patient confirming the occlusion of many major pulmonary vessels due to emboli
Investigations of limb veins Investigations of lower limb veins should be carried out to exclude sources of pulmonary emboli. Venography is the best way to demonstrate clots in deep veins in the legs and pelvis but it is uncomfortable and not rapidly obtainable. Döppler ultrasound gives a large number of false negatives both in the calves and pelvic veins and is very operator dependent. I125 fibrinogen scanning is of limited use because clots within the abdomen are not demonstrated.
120
Pulmonary Embolism and Deep Vein Thrombosis Figure 7.7: venogram showing extensive venous thrombosis in the left femoral vein
Note the thrombus outlined by contrast running circumferentially.
The conventional method of investigation to exclude pulmonary emboli in most hospitals in the UK is Döppler ultrasound of veins together with ventilation perfusion (V/Q) lung scan or spiral CT scanning.
121
Respiratory Disorders
PREVENTION Risk factors of pulmonary embolism should be avoided wherever possible. In certain cases, prophylactic anticoagulation with subcutaneous heparin should be considered, such as prolonged bed rest in hospital, with hemiplegia, congestive cardiac failure, myocardial infarction, prolonged infection, surgery such as hip or knee replacement and other trauma. Prophylactic heparin subcutaneously should also be given routinely pre and post-operatively and in the coronary care unit. Low molecular heparins are also effective. Foot pumps and stockings are also used in prophylaxis in surgical operations. Other avoidable/preventable risk factors include high oestrogen contraceptive pills, HRT, smoking, obesity, dehydration and prolonged air flights.
TREATMENT
Massive life-threatening pulmonary embolism Emergency resuscitation is necessary and involves the following procedures: • external cardiac massage; • intubation with endotracheal tube; • artificial ventilation with high oxygen concentration; • analgesia including morphine or diamorphine; • anticoagulation with heparin intravenously followed (if the patient survives) by oral warfarin; • streptokinase as a thrombolytic is given via a central catheter into the pulmonary artery to aid dissolving the clots. Contraindication to this streptokinase therapy include bleeding disorders, peptic ulceration, hypertension, recent surgery, or previous cerebrovascular haemorrhage (stroke); • in severe cases, surgical removal of the blood clot under cardiopulmonary bypass may be considered. This is reserved for the critically ill patient and can only be carried out in centres where there is cardiovascular surgery available on site, and is necessary in patients where the pulmonary circulation is impaired by 75% or more.
122
Pulmonary Embolism and Deep Vein Thrombosis
Non-life-threatening pulmonary embolism This requires: • 60% oxygen via a mask; • analgesia, morphine or diamorphine; • intravenous heparin initially, converted to oral warfarin later, which should be continued for at least three months; • if there is fresh clot in the deep veins in the pelvis which is likely to reembolise, surgical procedures such as insertion of Kimray-Greenfield filters in the inferior vena cava may be considered. These filters may also be inserted into patients who have recurring pulmonary emboli despite full anticoagulation. Clinically, it is often difficult to differentiate between pneumonia and pulmonary embolism. Indeed pulmonary infarcts often become infected, hence broad spectrum antibiotics are often given, but these are not necessary for routine treatment of pulmonary embolism if the initial diagnosis has been confidently (and correctly) made.
Recurrent thromboembolism There are a small group of patients with recurrent (asymptomatic) pulmonary emboli who present with increasing breathlessness and fatigue and, later, right heart failure. At first, there is lack of oxygen only on exertion. These patients require lifetime anticoagulation with warfarin which needs to be stopped during pregnancy, because of the teratogenic effects of warfarin when the patient is converted to heparin with the warfarin being restarted after delivery.
Anticoagulant control It is conventional to commence treatment with intravenous heparin and then convert to oral warfarin. When the oral warfarin is acting, the heparin infusion is stopped and then the patient is discharged and followed regularly in an anticoagulant clinic to control the warfarin dose. During the period of anticoagulation, there needs to be adequate observation of the effect and control of the dose of heparin and warfarin. Heparin is controlled by measuring the activated partial thromboplastin time (APTT) to ensure that this ratio of the patient’s clotting to a control sample is between two and three times normal. It is frequent for the dose to
123
Respiratory Disorders need adjusting and for action to be required either to increase or decrease the dose depending upon the results. The test to control warfarin dose is the international normalised ratio (INR). The aim is to keep the patient’s INR at two and a half to three and a half times the control. Initially, it may take a few days to get the INR correct and too little or too much warfarin may be given, but action should be taken to adjust the dose. What is unacceptable in both circumstances is, first, not to monitor at all; secondly, not to take action upon abnormal results; and, thirdly, to monitor the relevant drug with the wrong test.
MEDICO-LEGAL ASPECTS Regrettably, patients may experience a very frightening and uncomfortable time with a pulmonary embolus or recurrent pulmonary emboli; even worse, they may die. Because of this, legal action over pulmonary emboli may be considered by patients or their relatives for these three reasons: (a) failure of prevention; (b) failure of diagnosis and hence treatment; (c) failure to supervise therapy.
Prevention As far as the avoidance of pulmonary emboli and deep vein thrombosis are concerned, there are certain circumstances in which routine prophylactic anticoagulation should be given. The main situation is pre-operatively for a large surgical operation, whether it be intra-abdominal or orthopaedic (such as a large joint replacement) where it has been shown that there is a reduction in the rate of pulmonary embolis if pre-operative anticoagulation is given routinely. It is important to realise that the use of anticoagulation prophylactically means a reduction and not a total abolition of the risks of pulmonary embolism. If a patient in a high risk group has not been anticoagulated prophylactically by the surgeon, there needs to be good reason – such as previous bleeding disorders or co-existing known active peptic ulceration, which would have made it more dangerous to anticoagulate than not to do so. The patient’s age may also come into this decision in that with the very elderly there is an increased risk of bleeding into the brain (stroke).
124
Pulmonary Embolism and Deep Vein Thrombosis
Failure to treat The second group of claims is where there is a failure to treat deep vein thrombosis adequately. Whereas there is a historical precedent for not treating patients with clinical deep vein thromboses which occur below the knee, it is increasingly conventional practice to consider deep vein thrombosis below the knee as a precursor of a deep vein thrombosis above the knee, and hence a possible precursor of pulmonary emboli. Hence, it is now recommended that all patients with proven deep vein thromboses should be adequately anticoagulated. However, there are still patients who are not anticoagulated for below the knee deep vein thromboses who subsequently develop pulmonary emboli and then consult their legal advisors.
Failure to warn patients of the effects of therapy Lawyers may be consulted by clients who have been prescribed oral contraceptive pills who develop pulmonary emboli and who wish to make complaints against their GPs or the manufacturer of the contraceptive pills. However, with the lay press being full of the embolic risks of oral contraception, it is difficult for this type of claim to be successful. There is also the argument that the risks of pulmonary embolism and deep vein thrombosis in pregnancy are greater than those with the contraceptive pill. Nonetheless, pulmonary embolism and death from the oral contraceptive pill are a tragedy whenever they occur. It is possible that legal action might succeed if the oral contraceptive was prescribed for another reason, such as acne, and the patient was not warned of the risks.
Failure to make the correct diagnosis The diagnosis of pulmonary embolism is notoriously difficult to make correctly. At one end of the spectrum there are the easy cases – those who have had major surgery 10 days before, have been doing well, then have developed a swollen leg and suddenly become short of breath and blue. No one has difficulty with this diagnosis. At the other end of the spectrum and the more common scenario, however, is the 18 year old on the pill who develops some pleuritic chest pain with normal legs. The question is whether this is infective, musculoskeletal or pulmonary embolic. In these cases it is important to have a routine whereby pulmonary embolism can be confirmed or excluded. This includes taking a history, performing a physical examination, a chest X-ray, ECG and a
125
Respiratory Disorders scanning procedure of the legs to exclude deep vein thrombosis coupled usually with a ventilation perfusion lung scan or spiral CT scan. It is not conventional to perform a pulmonary angiogram on many patients. Even with this battery of tests, diagnosis is often uncertain and one often has to accept a balance of probabilities rather than an absolute answer. It is conventional, and safest, if the diagnosis of pulmonary embolism is considered to be at all likely, to anticoagulate the patient whilst investigating and to stop the anticoagulation when the investigations are negative. This is the safest course of action to avoid further, possibly more severe pulmonary embolism. There are a large number of legal cases where a GP or hospital physician has failed to make the diagnosis initially, but the diagnosis has been made four or five days later. Fortunately, the ultimate outcome for the patient is often not altered, because, once treated, pulmonary emboli tend to resolve and there is complete resolution without permanent loss of lung function. However, the patient may have had a very unpleasant time during the intervening two or three days that it has taken to recognise that the patient has indeed had a pulmonary embolus. There is a more serious problem, of course, in circumstances where the diagnosis was missed in the initial stage of the illness, treatment was not instituted and the patient then experiences a second catastrophic embolus and dies. It has to be shown, in these circumstances, first, that the diagnosis of pulmonary embolism should have been considered and, secondly, that investigations would have been performed to confirm the diagnosis. Case 14: pulmonary embolism – poor anticoagulant control A 19 year old girl with suspected pulmonary embolism was anticoagulated, over a Bank Holiday, with heparin, the control of which was being performed by a pre-registration house physician who was using the INR (which is not affected by heparin) to adjust the dose of the heparin infusion. As the INR was normal, he kept on increasing the dose of heparin. The patient developed massive spontaneous internal intestinal bleeding, became shocked and needed to be rapidly transfused five units of blood. Fortunately, she survived, but had a very unpleasant time due to this elementary error. However, the case was not contested and was settled out of court to her entire satisfaction.
126
CHAPTER 8
LUNG CANCER AND OTHER TUMOURS
LUNG TUMOURS Figure 8.1: carcinoma of the left upper lobe bronchus, appearing as a left hilar mass and causing left upper lobe collapse
Note the overexpanded left lower lobe and herniation of the right upper lobe across the midline.
Well over 95% of lung tumours are malignant (lung cancers). There are, however, a very small percentage of tumours of intermediate malignancy (carcinoid tumours) or benign tumours (hamartoma). Lung cancers are the most common malignant tumours in males in the UK and although their frequency is no longer rising in men, they are still increasing in women, in whom they are the second most common cancer after breast cancer.
127
Respiratory Disorders Lung cancers are thought to be primarily related to cigarette smoking, but other known causative factors include exposure to inhaled particles, such as asbestos and other carcinogenic pollutants. The risk of lung cancer in a smoker is 12 times that of a non-smoker. After stopping smoking, it takes 10 years for the risk of lung cancer to fall back to that of the non-smoking population.
The natural history of lung cancer Lung cancers are aggressive tumours, which cause problems both by local effects and more distant spread (metastases). They can cause local effects by blocking off the air passage (bronchus) in which they arise, resulting in local infection (pneumonia). They may also spread locally to the outer surface of the lung and cause the fluid to accumulate around the lung in the pleural cavity (pleural effusions). This fluid tends to accumulate and cause breathlessness. They can also invade through the pleura into the chest wall and cause local pain. More distant spread is first to the draining lymph nodes within the mediastinum. These enlarged lymph nodes can be asymptomatic, or they then press on or cause invasion of local structures in the mediastinum, causing nerve paralysis, particularly of the left recurrent laryngeal nerve, causing hoarseness and voice alteration or phrenic nerve paralysis, resulting in paralysis of one side of the diaphragm and, hence, breathlessness. If pressure and obstruction of the superior vena cava occurs, this results in swelling of the head and arms through obstruction of venous drainage. With enlargement of the mediastinal lymph nodes, there may also be pressure on and narrowing of the oesophagus, causing difficulty with swallowing. There may be more distant spread via the blood stream to the bones anywhere in the body, particularly long bones, vertebrae or ribs, causing pain or local fractures. Ultimately, spread to the liver is common, causing weight loss, loss of appetite, but very rarely jaundice. Spread to the brain, causing fits or paralysis, occurs in around 5% of patients. A secondary tumour from lung cancer is, in fact, the most common form of tumour occurring in the brain.
Clinical presentation Lung cancers may be picked up on a routine chest X-ray in asymptomatic individuals; unfortunately, this is rarely the case. It is more frequent for them to present at a rather later stage when either they are causing local problems, such as a pneumonia, or often presenting later still through symptoms caused by the spread of the disease, for example, bone pain or weight loss.
128
Lung Cancer and Other Tumours
Diagnosis of lung cancer and staging of the disease A full clinical history and examination is performed followed by further tests, first, to confirm the diagnosis histologically; secondly, to identify important co-existing diseases, such as diabetes or heart disease, chronic obstructive lung disease, or other pulmonary disease; and, thirdly, to stage the extent of the lung cancer. The staging of lung cancer is based upon a TNM classification: T for tumour size; N for lymph node involvement; and M for metastases.
Classification (shortened) T0 – T1 – T2 – T3 –
no evidence of primary tumour tumour 3 cm or less tumour greater than 3 cm a tumour of any size with direct extension into an adjacent structure, such as pleura or chest wall or a tumour bronchoscopically close to (less than 2 cm) the carina
N0 – N1 – N2 –
no lymph node metastases lymph node in the peri bronchial or ipsilateral hilar region metastases to the lymph nodes in the mediastinum
M0 – M1 –
no distant metastases distant metastases to a structure such as the scalene, cervical or contra lateral hilar lymph nodes, brain, bones, liver or contra lateral lung It is essential to check that the patient has adequate respiratory reserve if surgery is contemplated and to check for symptoms that would make one suspect spread outside the lung, such as persistent bone pain; voice change; abdominal swelling; enlarged lymph nodes; enlarged liver; neurological features; or superior vena caval obstruction.
Tests The measurement of lung function with a spirometer, to obtain the forced expiratory volume in one second and forced vital capacity, is essential and is a very useful starting point in the clinic. Often, this may be so severely impaired that curative surgery is out of the question. Blood tests should be performed to check on the haemoglobin, white cell count, the kidney function and liver function.
129
Respiratory Disorders
Chest X-ray The patient will usually present with an abnormal chest X-ray (this may be why the GP has referred the patient up to the clinic). On the basis of the X-ray abnormality, further investigations will be performed to arrive at a definite diagnosis. These will often include a day case bronchoscopy in which a fibreoptic bronchoscope is inserted through the nose into the bronchial tree. This is examined for a tumour which, if present, can be biopsied to confirm the diagnosis. Brushings and bronchoalveolar lavage are often performed to obtain cells for cytology. Figure 8.2: view of normal bronchi through a bronchoscope
130
Lung Cancer and Other Tumours Figure 8.3: carcinoma of the bronchus
A computerised tomographic (CT) scan is also routinely performed, not only to check on the presence and size of the primary tumour, but to look for spread to the mediastinal lymph nodes, the liver or adrenal glands.
131
Respiratory Disorders Figure 8.4: carcinoma of the bronchus – chest wall (rib) and vertebral invasion
Figure 8.5: carcinoma of the bronchus – liver metastases
132
Lung Cancer and Other Tumours If bronchoscopy has been unsuccessful in making a diagnosis, but a mass is seen on the CT scan, a needle can be inserted under local anaesthetic through the chest wall under CT scanning control into the mass seen and a sample aspirated and sent for cytology and histology. Although it is possible to be comprehensive by excluding the spread to the brain by performing a CT or MRI scan of the brain and a radio isotope bone scan, it is not conventional, in the majority of departments, to do these routinely on all patients unless, in the first instance, there are neurological symptoms and signs and, in the second, there is bone pain. Otherwise, the yield in both of these cases is very low (around 3%). Figure 8.6: CT scan of the brain showing a large intracerebral tumor, a secondary from a bronchial carcinoma
Cell types Biopsies, histological and cytological confirmation of the diagnosis should be obtained wherever possible. There are three main cell types of lung cancer, squamous cell carcinoma, adenocarcinoma and small cell (oat cell) carcinoma. Squamous cell carcinoma is the most common. Adenocarcinoma is the second most common and may be difficult to differentiate from a secondary cancer from a primary site elsewhere in the body. The least common is small cell carcinoma, which tends to be the most aggressive type of cancer, which usually presents at the stage when there is already widespread dissemination throughout the body.
133
Respiratory Disorders
Therapies and prognosis The mean expectation of life from diagnosis to death in lung cancer is of the order of six to eight months and depends on the cell type, degree of spread and co-existent disease. The only form of treatment which offers a more hopeful outlook is that of surgical removal of the tumour by lobectomy (a lobe of the lung) or pneumonectomy (the entire lung). However, the screening of patients to check that they are fit for surgery reveals that only around 20–25% of patients have a tumour which is amenable to surgical resection, once one has excluded those patients who are unfit for reasons such as having: (a) other serious diseases; (b) limited lung function (FEV 1 < 1.5 litre); (c) evidence of mediastinal lymph node spread; or (d) metastatic spread to the other lung or to distant sites such as the brain, bones or liver. Even if one looks at the total five year survival rate for patients who come to surgery, this is only around 25%. Thus, the overall five year survival rate in lung cancer is of the order of 5%. There is, however, a small subgroup of patients with small peripheral tumours who will do well, and in this selected subgroup there is a greater than 50% five year survival rate after surgical excision. Prognosis depends upon TNM staging (see figure 8.7). Figure 8.7: cumulative percentage surviving five years by clinical and surgical (including pathological examination of specimen) TNM subsets Non-small cell lung cancer Clinical staging TNM subset
Surgical staging
Median survival (months)
Surviving (%)
Surviving (%)
Median survival (months)
T1
N0
M0
62
60+
69
60+
T2
N0
M0
36
26
60
60+
T1
N1
M0
34
20
54
60+
T2
N1
M0
23
17
40
29
T3
N0
M0
8
8
44
26
T3
N1
M0
8
8
18
16
Any
N2
M0
5
11
29
22
Any
M1
2
6 none operated none operated
Total
If the patient has a squamous or adenocarcinoma and is unfit for surgery then he may be considered for either local radiotherapy, chemotherapy or initially 134
Lung Cancer and Other Tumours no treatment, holding radiotherapy and chemotherapy in reserve for specific complications at a later stage. Often, the patient is beyond any form of active intervention and requires palliative therapy (terminal care) either at home, in hospital or in a hospice. Realistically, none of these treatments offers a prospect of long term survival, although there are a few patients in whom this is reported. As far as small cell carcinomas are concerned, they are more responsive to aggressive chemotherapy, and there is a small percentage (about 5%) who survive up to five years with chemotherapy – its main effect being to increase survival by anything from three or four months to around 10 months.
Practicalities of lung cancer care At present most district general hospitals and chest clinics do not have lung surgery or radiotherapy on site. Although the diagnosis is made in the district general hospital, the patients are usually referred for radiotherapy and lung surgery to a bigger centre such as the Calman cancer centre. However, chemotherapy may be performed locally. There are proposals for Calman cancer centres to enable patients with cancers to be treated in centres that have expertise. These centres have a number of units based in district general hospitals attached to them. As far as lung cancer is concerned, the proposal that there should be joint clinics between chest physicians, oncologists and the palliative care team is unlikely to make a radical difference to the long term survival of patients with this very unpleasant disease. Efforts should be concentrated on discouraging smoking and primary prevention.
MEDICO-LEGAL ASPECTS Potential medico-legal claims around the diagnosis and treatment of lung cancer are unfortunately frequent. They may be because the diagnosis has been missed, the wrong diagnosis has been made or the diagnosis has been made too slowly. Cancer is an emotive area and it is difficult to convince patients and relatives that an earlier diagnosis may not have made any difference. There is always a ‘gut feeling’ that earlier diagnosis would have lead to a different (better) outcome. Regrettably, the limiting factor in many claims is rarely the liability issue, but causation and outcome. In brief, unless it can be shown that a patient had a small peripheral carcinoma without any spread by the time of presentation, and that the patient was otherwise fit and had normal respiratory function, it is very difficult to show that an earlier diagnosis would have made a difference to the outcome, as far as five year survival is concerned.
135
Respiratory Disorders Case 15: lung cancer – wrong diagnosis A 45 year old woman had sputum samples sent to look for malignant cells when she was in hospital. These were reported by the SHO in the histopathology department as showing definite malignant (squamous) cells. The patient was therefore referred to the chest physician who carried out extensive investigations, such as a bronchoscopy and CT scans. In order to obtain informed consent for these investigations, the chest physician had to tell the patient why she had been referred, that is, because malignant cells were reported as being present in her sputum. On the basis of this information, the woman took early retirement from her job. Repetitive investigations with further bronchoscopies and CT scans over the next six months failed to show any evidence of tumour. In fact, it was not until after the third bronchoscopy that the patient was given the all clear. At no stage could it, in fact, be shown that the chest physician had given advice for her to retire and, indeed, her retirement did not occur until after the second bronchoscopy gave her the all clear. However, the advice that she had obtained from the occupational physician was clearly erroneous. When the original cytology slides, as a result of which all the further investigations had (quite rightly) been performed, were reviewed by an experienced consultant cytopathologist, no malignant cells could be seen. The original cytology was, therefore, erroneous and of an unacceptable standard, in that the report should have been checked by a more senior pathologist than an SHO. In this case, it was quite clear that, on the issue of liability, both the cytopathologist who reported the original film of the sputum and the occupational physician who recommended early retirement were at fault. However, it became clear to the solicitors, on further detailed investigations of the quantum of the claim, that the plaintiff was, financially, considerably better off having accepted early retirement than if she had continued in her employment! Hence, the case did not proceed. This case illustrates several principles. First, a positive cytology result needs further investigation and, if no obvious tumour is found, the original cytology result needs double checking by other senior pathologists. It is inevitable that any positive cytology result will lead to considerable anxiety over a matter of months for the patient concerned. It must be shown that the work of junior trainee pathologists has been checked by a more senior pathologist. It also needs to be shown that a suspicious sputum cytology result has been followed through clinically and further checks carried out to exclude the possibility of a carcinoma. If no tumour can be found, it is important to have the cytology double checked again by the pathologist or his or her colleagues. Repetitive investigations following positive cytology need to be carried out over a matter of at least three to six months, to exclude the possibility of a small tumour having been missed initially and becoming apparent later. Such investigations would include repeat chest X-rays, CT scans or bronchoscopies. 136
Lung Cancer and Other Tumours Another example of where an incorrect diagnosis can lead to anxiety is where the diagnosis has been made on entirely clinical grounds, even though biopsy (histology and cytology) could have been obtained. It is not acceptable practice to tell patients or their relatives that there is definitely a cancer present unless this has been proven histologically. However, there are exceptional circumstances, either when attempts have been made to obtain biopsies, but for one reason or another these have been technically impossible to obtain, or when obtaining them may put the patient’s life at risk. In these instances, one would have to accept that a ‘clinical diagnosis’ would have to be made – this affects only a very small group of patients. To get a wrong diagnosis in these circumstances can understandably cause a large amount of anxiety. The physician in this situation needs to have shown that he is constantly keeping an open mind as to whether the clinical diagnosis is in fact correct. The patient and relatives need to be fully informed of the situation that there is lack of proof of the diagnosis.
Missed diagnosis Broadly, this falls into two groups. First, a chest X-ray has been taken and the radiologist or other physician has looked at the film and missed the lesion which then becomes apparent some months later. In turn this may fall into two categories: (a) the lesion on the film which was missed was of such a size and was so obvious that any competent radiologist or chest physician would have seen it. This is an extremely rare occurrence; (b) more commonly, the patient has presented with a tumour, an old film is found and with the benefit of hindsight, it is possible to see the lesion present on the earlier film. All medicine is easy in hindsight! The importance of a missed diagnosis is that where there is a very small lesion (cancer) it may be possible to show that earlier diagnosis could have led to a marked difference in overall prognosis. This is because a very small, solitary, peripheral lesion would have more than a 50% five year survival rate with surgery (should the patient be operable on all other grounds). Regrettably, it is all too easy to start with an instruction letter saying that a patient has a bronchial carcinoma and be supplied with a packet of X-rays and to be wise after the event because one knows that there is a cancer in retrospect. Although the medical expert has a personal view as to whether the lesions should have been missed or not, in these circumstances it is sensible to take the routine precaution of making sure that the X-rays are presented to their clinical and radiological colleagues ‘blind’ (with their having no clinical history) to see whether they notice and report the lesion. In any event, in these types of case, it is essential for the instructing solicitors to have a full radiological review of the X-rays, not only by a case physician, but also by a consultant radiologist with experience in medico-legal work. 137
Respiratory Disorders The second group of errors of missed diagnosis of lung cancer are those where a chest X-ray has been taken in hospital, reported as showing a lesion suspicious of a cancer and the report filed, either in the X-ray folder or in the patient’s notes, without the doctor taking action upon it (or the requesting physician looking at the X-rays themselves). Regrettably, this is a relatively frequent occurrence and most commonly seen when a patient who has been in hospital for an entirely different reason, such as an operation, has developed a chest infection after the operation and has been sent for a chest X-ray which none of the clinicians has looked at. The patient recovers and goes home. The X-ray is then sent for reporting, and the consultant radiologist sees a tumour, but no one has acted on the report, other than to file it neatly in the notes. The patient later presents with more advanced (and, by then, incurable) cancer some months later. Although this is the most common error, it is relatively rare but, nonetheless, inexcusable and indefensible. Case 16: lung cancer – missed diagnosis – incurable mediastinal spread A 48 year old man, who was in hospital having varicose veins operated upon, developed a chest infection post-operatively and a chest X-ray was requested by the houseman. He was prescribed antibiotics and recovered. The houseman looked at the X-ray but noted nothing abnormal. After the patient was sent home, the X-ray was reported and filed as showing a possible cancer of his right upper lobe. This was a large 4 cm tumour and the mediastinum looked rather wide on the X-ray. A few months later he was diagnosed as having lung cancer and rapidly died while on holiday in Portugal. His relatives instructed solicitors. Although it was undeniable that the lung cancer could (and should) have been diagnosed when he was in hospital having his varicose veins operation, as he had not had any staging procedures, such as a CT scan, and the mediastinum was wide on the X-ray, it was impossible to say whether or not he had mediastinal lymph node spread at the time which would have made him incurable. On the balance of probabilities, both the medical expert and the consultant radiologist felt that he probably already had mediastinal lymph node spread and was therefore inoperable by the time the original diagnosis should have been made. Nonetheless, this was unacceptable medical practice, but it was argued successfully that, if he knew he had cancer, he would have wanted to make a last visit for a holiday in Portugal anyway; hence, no financial loss had occurred. Case 17: lung cancer – missed diagnosis – inadequate lung function A 64 year old man had a 1 cm right mid zone lesion missed on a chest X-ray which was done as part of a routine follow-up at a chest clinic. He was seen six months later, by which time the tumour had enlarged to 4 cm and spread to the mediastinum. He also had liver metastases and died relatively soon afterwards. 138
Lung Cancer and Other Tumours Although liability here was not in doubt, the hospital case records meticulously recorded the level of respiratory function and his exercise tolerance. Over the previous two years, there were notes such as ‘breathless on minimal exertion’, ‘very breathless on walking into the consulting room’. The FEV1 was recorded at 0.5 litres, hence, on the grounds of respiratory reserve, even if this small tumour had been diagnosed in the beginning, he was inoperable at the time. The alternative therapy – radiotherapy – might well have made him far more breathless. Chemotherapy would not have offered a cure. Thus, it was difficult to show that earlier diagnosis would have affected the outcome.
Slow diagnosis and treatment There are occasionally accusations made that because the process of obtaining referral from a GP to a chest clinic and then having a series of investigations such as bronchoscopy and CT scan to confirm diagnosis are slow, and may take, say, two months from beginning to end, that this has affected the outcome. Whilst most chest clinics like to keep a very short waiting list for possible cancers, usually less than two weeks, and can also have referrals direct from the X-ray department (for GP films), it is difficult to argue that, within the National Health Service, a process of diagnosis of two months differs greatly from the overall norm or that in lung cancer, realistically, that it would have made a difference to overall survival. There is also a group of patients who are brought up to Accident and Emergency departments because of lung pain who are sent home and then return because of the pain. Eventually they are sent to the chest clinic and a diagnosis of carcinoma of the lung is made. Regrettably, in these cases the usual cause of the initial presentation is spread of the lung cancer to bone, and hence the disease was incurable at the time of the original presentation. Even though the diagnosis has been missed initially (often by the doctor in Accident and Emergency being a rather inexperienced one), on an emotional level, it is difficult for relatives to understand that it has not made a difference to the outcome because of the disease having spread too far. Nonetheless, because they have had a poor and unacceptable level of medical investigation and diagnosis, the hospital’s reputation suffers, even though a legal claim is unlikely to be worth pursuing.
OTHER LUNG TUMOURS Alveolar cell carcinoma This is a rare (less than 1%) malignant lung tumour which occurs in the same age group as other lung cancers. It is unusual in that it is often multifocal in 139
Respiratory Disorders origin, growing throughout the air spaces. It responds poorly to radiotherapy or chemotherapy. Limited localised disease may be treated by surgery.
Clinical features Typical features of this condition include: • haemoptysis; • breathlessness; • copious watery sputum; • hypoxic respiratory failure.
Investigations The range of tests required is similar to that of other lung tumours.
Bronchial adenoma These are rare tumours, representing about 1% of all lung tumours. The two main types are carcinoid and cylindroma. Often, they are referred to as ‘benign’, but, in fact, are of low grade malignant potential and may metastasise.
Clinical features These are similar to lung cancer but occur in younger (non-smoking) age groups. Very rarely, the carcinoid syndrome may occur with metastatic tumours: • flushes; • wheezing; • breathlessness; • diarrhoea. In these situations a chemical, 5-hydroxyindoleacetic acid, is excreted in increased amounts in the urine. Surgical excision is the treatment of choice. Prognosis is good for totally excised tumours.
Hamartoma Very uncommon benign tumour often lobulated with specks of calcification on X-ray or CT. Usually asymptomatic – important as a benign alternative to lung cancer for masses seen on chest X-ray. However, diagnosis is usually made after the lung has been resected. 140
CHAPTER 9
TUBERCULOSIS
Introduction Tuberculosis is an infectious disease caused by the bacterium mycobacterium tuberculosis. It is spread by droplet, from man to man, by an infected person (the source case) exhaling (usually by coughing) tubercle bacilli into the environment. If these are inhaled by an uninfected person, tuberculosis normally develops in the following few months. Figure 9.1: bilateral upper lobe infiltration due to active tuberculosis
141
Respiratory Disorders Figure 9.2: mediastinal lymphadenopathy and miliary interstitial nodules in the lung fields due to tuberculosis
Types of disease Tuberculous infection has been historically described as primary or postprimary. In primary tuberculosis, the primary infective complex is a focus in the mid zone of the lungs (known as a Ghon focus). This usually occurs in children, and is associated with enlargement of the hilar lymph glands. This primary complex is usually asymptomatic, but may be detected on a routine chest X-ray, for example, on contact tracing. It may, however, progress to other clinical forms of symptomatic tuberculosis such as: (a) bronchial spread – either causing obstruction of a bronchus or endobronchial rupture causing local pneumonic disease; (b) pleural effusion – where fluid accumulates in the pleural space between the chest wall and the lung, causing breathlessness; (c) spread via the blood stream – causing miliary tuberculosis or meningitis; (d) spread to the bones, joints, or genito-urinary system; (e) post-primary tuberculosis – caused by the breakdown of the primary lesion. 142
Tuberculosis Whereas (a), (b) and (c) normally manifest themselves within a year of infection, (d) may not occur until 10–15 years after primary infection. Post-primary tuberculosis – (e) – is the most common type of adult disease, usually occurring within the posterior segment of the upper lobe or apical segment of the lower lobe. Initially, there is an infective infiltrate on the chest X-ray, which may progress to cavitation or healing by fibrosis with calcification.
POPULATION AT RISK Tuberculosis is relatively common in inner cities and the physician must be constantly aware of the possibility of tuberculosis because of its many and diverse presentations. Tuberculosis is more common amongst particular groups, including: • recent immigrants from Central and East Africa, Somalia, Ethiopia and Asia, who usually develop the disease within a few years of arriving in the UK; • down and outs: poor housing conditions, overcrowding and malnutrition contribute to making this group vulnerable; • alcoholics; • institution dwellers living in, for example, working men’s hostels, prisons and mental hospitals; • doctors and nurses; teachers; medical laboratory workers; • the elderly; very young children, particularly if their mother is affected by the disease; • the immunosuppressed, for example, those suffering from HIV infection or undergoing chemotherapy.
Common symptoms and signs By far the most common site for tuberculosis is the lungs (70%), with other sites accounting for only 30% of cases. The symptoms of pulmonary disease are: • general malaise; • fever; • weight loss; • cough with sputum production; • occasionally, coughing of blood (haemoptysis); • chest pain (rare).
143
Respiratory Disorders A chest X-ray usually shows upper lobe infiltrate with or without cavitation. Other lobes of the lung, however, may be involved.
Glandular tuberculosis Lymph nodes may enlarge either within the chest – the hilar or mediastinal lymph nodes. These are often discovered on routine X-ray. Patients may be asymptomatic or they may have malaise, weight loss or fever. Other glands frequently affected are lymph nodes in the neck, axillae (armpits) or groin – the patient notices painless swelling in the affected area. When the glands become very large, they may be fluctuant to the feel and they may discharge pus. Very rarely, they are hot and inflamed. Pleural effusions may occur after initial pleurisy. Shortness of breath occurs due to formation of fluid in the pleural space.
Rarer symptoms and signs Tuberculosis may, occasionally, lead to the following developments: • pneumonia in lobes other than the upper lobes, in which case there is cough, sputum, fever and prostration; • meningitis with headache, fever, neck stiffness, vomiting, confusion and eventually fitting and loss of consciousness; • bone and joint tuberculosis presenting with local pain: if it occurs within the spine this may lead to neurological symptoms and complications due to spinal cord compression; • the skin may be affected with erythema nodosum, a painful raised lumpy skin rash. A rare form of skin tuberculosis known as lupus vulgaris affects the nose and face; • miliary tuberculosis may occur when there is blood borne spread throughout the body to the lungs, spleen, liver, meninges, eyes and bone marrow. The patient is often febrile and may be moribund; • renal and genital tuberculosis may lead to pus in the urine, fever, sterility and testicular swelling; • intestinal tuberculosis may lead to abdominal pain and diarrhoea and it may also lead to fluid within the peritoneal cavity (ascites); • tuberculosis may present as a tuberculoma, a mass-like lesion occurring because of the local response to tuberculosis. This may occur as coin-like lesions on chest X-rays, or within the brain, causing symptoms and signs similar to a brain tumour.
144
Tuberculosis
Diagnosis and investigation Definite diagnosis depends upon the identification of mycobacterium tuberculosis (acid fast bacilli (AFB)). This is made on specimens by using auramine or Ziehl-Neelsen stains. The mycobacterium tuberculosis is cultured using Lowenstein-Jensen medium, but takes between two and four weeks to grow. Depending on the site of the body, affected specimens can be obtained and diagnosis made from: • sputum; • bronchial aspirates via bronchoalveolar lavage (bronchoscopy); • induced sputum; • gastric washings and laryngeal swabs (of historical interest only); • lymph node aspirate; • lymph node biopsies; • bone marrow aspirate; • liver biopsy; • pleural biopsy or pleural fluid; • cerebrospinal fluid; • histological examination and culture of other excised specimens. The typical appearance of tuberculosis is that of caseating granulomata containing epithelioid giant cells. There are other causes of granulomata, but caseation and identification and staining of acid fast bacilli on the sample is confirmatory of tuberculosis. The tuberculin skin test (Mantoux, Heaf or Tine) is usually positive in very dilute strengths in active tuberculosis. In the extremely ill, tuberculin sensitivity may be lost and the test may be negative.
Therapy The current recommendation for treatment of tuberculosis in the UK is for a six month regimen, initially using three drugs (rifampicin, isoniazid and pyrazinamide). When drug sensitivity of the organism is known, one drug is stopped after two months and two others to which the organism is sensitive are continued. Usually, the recommendation is to stop the pyrazinamide after two months and continue with rifampicin and isoniazid for the rest of the six month course. All drugs are taken once daily before breakfast. On this regimen, with a six month course of therapy, the relapse rate is around 3%, whereas if treatment is incomplete or shortened, the relapse rate is
145
Respiratory Disorders far higher: for example, if it is reduced to four months, the relapse rate is unacceptable, at 12%. If the organism is resistant to one or more drugs, the drug regimen has to be adjusted according to the sensitivities. In addition, vitamin B6 (pyridoxine) is given to patients to prevent the risks of an isoniazid-induced peripheral neuropathy. Because of the potential side effects of the drugs, the following precautions should be taken before therapy is started: • base line liver function tests should be carried out, as rifampicin, pyrazinamide and isoniazid are all hepatotoxic; • the patient must be warned that his urine and stools will become orange/red with the dye in rifampicin; • if other drugs such as ethambutol are used, the visual acuity will need to be checked. Up to 2% of patients on ethambutol will have visual disturbance caused by this drug; • rifampicin induces liver enzymes and will, therefore, render the oral contraceptive pill inactive. Thus, patients put on to rifampicin therapy need to be warned to use other methods of contraception; • patients should be warned that the dye of rifampicin comes out in their tears and thus contact lenses will be ruined; • it is the physician’s obligation to notify a patient with tuberculosis to the local consultant in communicable disease control via whose office the local chest clinic and tuberculosis health visitors will be informed who will arrange for contact tracing of all the close household or close work contacts of the patient. Tuberculosis is not a very infectious disease and, therefore, contact tracing is kept to close contacts as opposed to casual contacts. Anti-tuberculous drugs, such as rifampicin and isoniazid, are thought to be safe in pregnancy. In situations where the patient is extremely unwell, such as miliary tuberculosis or meningitis, a fourth anti-tuberculous drug may well be used. Conventionally, this would be intra-muscular streptomycin. Similarly, with multiple drug resistance, four drugs may initially be required, together with isolation of the patient. Additionally, oral steroids may be considered in patients who have tuberculosis and are severely unwell, with the intention of trying to reduce residual fibrosis. Steroids may be added to a patient’s treatment when they are found to be suffering from: • pleural effusion; or • peritonitis; or • pericarditis; or • meningitis. In the severely malnourished, steroids may also be used as an appetite stimulant for weight gain or, in the desperately sick patient, to reduce toxicity
146
Tuberculosis of the disease. Steroids are normally only used in these circumstances for four to six weeks.
Routine follow-up The patient rarely requires hospital admission for diagnosis. The investigations can usually be done on an out-patient basis. After the diagnosis has been made and they have been started on therapy, they will be seen regularly through their six months of treatment. Conventionally, this would be after two weeks, three months and at the end of therapy, when they would either be discharged or seen once at a follow-up three months following the end of the treatment. At each visit, they will be questioned and examined clinically and chest Xrays taken to monitor the course of therapy (provided it was pulmonary disease).
Tuberculosis contacts People who have been in close contact with tuberculosis will be contacted by the tuberculosis health visitor and checked for active tuberculosis by means of a chest X-ray and a Mantoux (Heaf) test. They will also be given a health questionnaire to check for any symptoms of active tuberculosis. They will be asked whether they have had tuberculosis before or whether they have had immunisation (previous BCG vaccination against tuberculosis, given routinely to schoolchildren at the age of 12 or 13, gives 85% protection against tuberculosis over a 10 year period). BCG vaccination changes (converts) the Mantoux (Heaf) test from negative (the natural state) to positive. Those tuberculosis contacts who might have active tuberculosis and show, for example, abnormal chest X-ray, or a very positive Mantoux (Heaf) test, will be referred to the chest physician for examination and investigation followed by treatment with anti-tuberculous drugs to prevent further development of the disease. Those patients who are negative on the screening process will be reviewed once more, usually, at six months, and the chest X-ray and health screen questionnaire repeated. If they are well, they will be discharged. The length of follow-up varies from clinic to clinic according to local protocols. In some, there is a six month follow-up; in others, two years. There have been several published guidelines on the management of Tuberculosis – the most recent published in Thorax (1998) 53 S36–48. Details of previous protocols and British Thoracic Society recommendations on management and chemotherapy can be found in Thorax (1990) 45 403–08; Thorax (1994) 49 1193–200; BMJ (1990) 300 995–99; and BMJ (1992) 304 1231–33. These are all essential references which should be obtained. 147
Respiratory Disorders
MEDICO-LEGAL ASPECTS Case 18: tuberculosis – delay in diagnosis – ignored X-ray A 40 year old man with a known history of alcohol abuse was referred by his GP to the rheumatology department of a hospital. Investigations were carried out, including a chest X-ray. Despite extensive investigations, no cause for his malaise and weight loss could be discovered and he was discharged to his GP. He continued to feel unwell for six months and lost further weight. Eventually, he felt extremely unwell and was referred as an emergency admission to the same hospital. On admission, it was discovered on his chest X-ray that he had cavitation in his upper lobes. A speedy diagnosis of tuberculosis was made from his sputum and appropriate treatment instituted and a complete cure achieved. Regrettably, however, in hindsight, the chest X-ray ordered in the gastroenterology department was obviously abnormal on his first visit, but this chest X-ray had neither been reported nor had it been seen by the rheumatologist. There was, therefore, an indefensible case when he sued the hospital for his prolonged suffering which had resulted the loss of his job. The case was settled out of court, and provides a salutary lesson that, if a physician orders an X-ray, it is their obligation to see the X-ray or check the report. Case 19: tuberculosis – delayed diagnosis – ignored X-ray report A 72 year old man had an accident on his bike which had been caused by the negligence of the council in not maintaining the cycle path adjacent to a pavement. As part of his claim, he was seen by a consultant orthopaedic surgeon because of the damage to his shoulder. As part of this examination, he took X-rays of the shoulder which were reported as showing cavitation in the lung apex. Regrettably, in the body of his report, he did not draw the instructing solicitor’s attention to the significance of the appended X-ray report. The consultant radiologist who reported the film mentioned cavitation at the apex which should have been clear in meaning to the orthopaedic surgeon, but, of course, would not be clear to an instructing solicitor. The man’s general health declined over the next few months until he went to his own doctor because of persistent cough, and at this stage, following a chest X-ray, a diagnosis of tuberculosis was made. His initial claim against the council, that the tuberculosis was caused by the bicycle accident, could not be sustained. However, it was very clear that he had active tuberculosis at the time he was seen by the orthopaedic surgeon, who had a duty, even though this had been an orthopaedic examination, to point out to the instructing solicitor that the shoulder X-ray showed the possibility of active pulmonary 148
Tuberculosis tuberculosis, that he should have a full chest X-ray, be seen by his GP and be referred to a chest clinic. If this had been done, he would have had several months’ less suffering and debility and would have maintained his part time employment. Case 20: tuberculosis – delay in diagnosis – no chest X-ray A 35 year old successful businesswoman had suffered from chronic laryngitis and persistent coughing. Her GP diagnosed chronic laryngitis and continued to treat her laryngitis non-specifically and at no point during the first six months took a chest X-ray (despite the fact that the patient had a cough and frequently travelled to countries where tuberculosis is common). Eventually, when the patient began to lose weight, felt generally unwell, had fevers and night sweats, a chest X-ray was taken which showed very extensive pulmonary tuberculosis. In fact, the original diagnosis was that of tuberculous laryngitis secondary to pulmonary tuberculosis. The diagnosis of pulmonary tuberculosis had been considerably delayed – because of this she had more extensive lung damage than she would have had otherwise. Case 21: tuberculosis – delayed diagnosis – the value of audit? Many hospital chest clinics run a tuberculosis audit whereby all patients who have tuberculosis diagnosed by tuberculosis organisms grown in the pathology lab, or on histology, or are started on anti-tuberculous therapy, routinely have their results sent to the chest clinic, so that tuberculosis visitors and chest physicians can check that all these patients are aware of their diagnosis and that they are being treated. As part of one such audit, a 45 year old man was found to have had a neck node removed and a diagnosis of tuberculosis made by the pathologist two months earlier, but the surgeon had not informed the man of the diagnosis, nor had he treated him. When recalled by the chest clinic, he was informed of the diagnosis and anti-tuberculous therapy was started and completed. He then sued the surgeon and trust for a variety of reasons. Most, if not all, of his claimed symptoms and effects were unrelatable to cervical node tuberculosis, but a great deal of time and money (in lawyers’ fees) was expended in defending the claim, which was eventually settled for a small sum in an out of court settlement, in recognition of the anxiety that the delay in the treatment had caused him. Clearly, the delay in diagnosis could have led to more extreme problems. Case 22: tuberculosis – delay in diagnosing meningitis A 28 year old man was correctly diagnosed by his GP as having a mycoplasma pneumonia, with typical abnormalities showing on his chest X-ray. However, as this had virtually resolved, he became non-specifically unwell with
149
Respiratory Disorders headache and confusion. He was admitted to hospital and, because he had been recently unwell, this was put down to a sequela of his pneumonia. Regrettably, he was not investigated adequately. In particular, a lumbar puncture was not performed until after he had fitted and had permanent brain damage from his tuberculous meningitis. This would have been avoidable had a more timely investigation been carried out and diagnosis made. Claims may arise because patients have unnecessary operations in order to diagnose tuberculosis. However, there are occasions when all reasonable steps have been made to make the diagnosis of tuberculosis, but there is still no definitive diagnosis and, therefore, operation on the chest under general anaesthetic is required. Case 23: tuberculosis – unnecessary lung resection? A 50 year old woman who worked in a pub was known to have problems with alcohol abuse. She had shadowing on her chest X-ray due to old tuberculosis which had suddenly deteriorated. It was felt that this was either reactivation of tuberculosis or a carcinoma. Extensive investigation, including bronchoscopy, bronchoalveolar lavage, CT scanning of the chest, all failed to produce a diagnosis. She was, therefore, submitted to operation and had a left upper lobectomy (removal of the upper lobe of the left lung), on section of which, tuberculosis was proven. Fortunately, there was no evidence of cancer. She then wished to sue the physician and surgeon involved. The case was defended most vigorously, in that the physicians and surgeons had pursued a dogged, logical and determined approach to the diagnosis of her possible cancer or tuberculosis, but the tests had all been negative. The claim failed, because the patient had not recognised that she had had an abnormal chest Xray for 10 years, which had suddenly deteriorated, showing that her physicians were not investigating a new lesion, but a deteriorating old one. The plaintiff also failed to realise that, even in the best of hands, no medical test or examination is 100% guaranteed, as far as providing a diagnosis is concerned, and that, so long as the physician and surgeon concerned had exercised all due care, had performed the correct investigations in a logical sequence and still did not arrive at a diagnosis, then it was likely that this was one of the well recognised, but rare, cases, where diagnosis of tuberculosis could only be made at operation. The patient claimed that, because of the lobectomy, she had experienced more pain and suffering than she would otherwise have had to. Furthermore, because of the stress of being told that she might have a cancer, this had precipitated alcoholism and dependence upon benzodiazepines.
150
Tuberculosis There was, however, a long history of alcohol and benzodiazepine abuse in her GP records, pre-dating the first visit to the physician and surgeon for her chest X-ray. The case was defended strongly and the plaintiff dropped her case after her legal aid certificate was withdrawn. Case 24: tuberculosis – unnecessary surgery – ignored sputum result A patient with chronic obstructive pulmonary disease (chronic bronchitis) underwent a right upper lobectomy for suspected co-existing carcinoma. He had coughed up blood, lost weight and his chest X-ray showed a mass-like shadow at the right apex. The thoracic surgeon had sent off sputum examination for tuberculosis and cytology for malignant cells and had booked the patient for an urgent bronchoscopy and right upper lobectomy as the mass seemed operable. When the patient was anaesthetised and on the operating table, the surgeon performed the bronchoscopy, which looked to him as if there was a mass occluding the right upper lobe. The bacteriology laboratory phoned the operating theatre urgently and gave a message which was passed to the surgeon saying there were copious acid fast bacilli (tuberculosis) in the patient’s sputum, confirming a diagnosis of tuberculosis. The surgeon’s response was that he would do an upper lobectomy anyway, because this looked to him like cancer and, thus, he believed there were two diagnoses. Regrettably, when the lobe was removed, there was only tuberculosis and, with the loss of the lobe, the patient was left more breathless than he would have been had he had anti-tuberculous therapy and lung preserved rather than a lobectomy. He then sued the surgeon and trust arguing that: (a) he would not have been less breathless had he not had the lobectomy; (b) in view of the positive sputum for tuberculosis, he should have been given anti-tuberculous therapy and not a lobectomy as he was unlikely to have two diagnoses simultaneously (and there was no evidence of cancer); (c) there was no need for lobectomy. At the bronchoscopy, biopsies could have been taken and could have been examined for malignancy. The case was not considered defensible, as the plaintiff’s case was undeniable. Settlement was made out of court. Fortunately, the surgeon involved retired shortly afterwards! Case 25: tuberculosis – all that cavitates is not tuberculosis! A 19 year old student, who was taking A levels prior to applying to university, became unwell with a cough with repetitive coughing of blood (haemoptysis), persistent fever and cavitation on chest X-ray with a very high sedimentation rate and anaemia.
151
Respiratory Disorders He was admitted to hospital for investigation. Throughout a five week stay in hospital, no definitive diagnosis was made and, when he was ultimately discharged, he was still anaemic. His fever was persisting, even though he had been put on empirical anti-tuberculous therapy (it is often excusable to do this if other investigations fail) and told to watch for a response. There was no such response. Regrettably, during his stay in hospital, inadequate investigations of his pulmonary condition were made; in particular, he did not have a bronchoscopy or bronchoalveolar lavage. Neither was a transbronchial biopsy performed, nor did the physicians persist and go on to an open lung biopsy. They also ignored the fact that his renal function was deteriorating and that, with the passage of time, he was developing swelling of the ankles because of impaired renal function. The physician in charge also refused the mother’s request for a second opinion. In fairness to the junior medical staff, there was clear evidence throughout that they were pressing the consultant to investigate the young man more thoroughly. However, at each consultant ward round, the suggestion of further investigations was vetoed by the consultant. Regrettably, three days after discharge from hospital, the young man was re-admitted as an emergency, having started to convulse at home. He was found to have an extremely high blood pressure. His chest X-ray was worse and he was found to be in severe renal (kidney) failure. It was then decided to transfer him urgently to the nearest renal dialysis unit, where the correct diagnosis, which had eluded the original hospital for nigh on six weeks, was made within three hours of admission (that of Wegener’s granulomatosis). In the meantime, however, the young man had been left hemiplegic (by a stroke) and was left with some mental impairment. His impaired renal function required permanent dialysis prior to kidney transplantation. It was certainly arguable that, had the diagnosis been made at an earlier stage, his kidney function would have been preserved and he would not have ended up hemiplegic, or with mental deterioration. He therefore sued the first trust for failure to diagnose his condition. As part of his claim, he required a lifetime of support at home and a lifetime loss of earnings. Although, initially, the trust contested the action, it was settled prior to coming to court because it was clear that a diagnosis of tuberculosis had never been made, despite many tests; that more extensive investigations to try to prove the diagnosis had additionally not been made; and that other investigations to consider the alternative differential diagnoses had not been made either. There had also been a total neglect of his deteriorating renal function. Hence, the case was settled in his favour.
152
Tuberculosis Case 26: tuberculosis – failure of contact tracing A one year old child, whose father had active pulmonary tuberculosis, was contact traced together with her mother and three year old brother. Both she and her brother were found to be tuberculin test positive. Normally, the children would tuberculin test negative unless they had been exposed to tuberculosis, for example, through having had had active tuberculosis, or a BCG vaccination (which this child had not). In the chest clinic records, it was clearly noted that there was a positive Mantoux test for both her and her brother, but it would appear that no action was taken on this. The conventional action where a young child who was a household contact is diagnosed as tuberculin test positive is for them to be given antituberculous chemoprophylaxis, in the form of treatment with antituberculosis drugs, to prevent active tuberculosis (in particular, meningitis). This was not instituted and, regrettably, the child developed tuberculous meningitis and her brother, who also failed to receive anti-tuberculous chemoprophylaxis, developed lymph node tuberculosis but came to no harm. Unfortunately, the one year old child developed lasting brain damage following the tuberculous meningitis. The argument was that the contact tracing procedure and protocols for chemoprophylaxis in the chest clinic had not been followed. This was demonstrably the case and, if the tuberculosis chemoprophylaxis had been instituted, tuberculous meningitis would have been avoided. The large trials of tuberculosis chemoprophylaxis demonstrate that there is a reduction in the instance of tuberculosis in those patients who have received chemoprophylaxis and, in particular, the trials show that tuberculous meningitis is abolished altogether. This case was settled in favour of the plaintiff. Case 27: tuberculosis – side effects of anti-tuberculous drugs – visual disturbance All drugs have side effects. Some of these side effects are predictable and others less so. Any doctor who starts anti-tuberculous therapy must warn the patient of the side effects and record this in the notes. A patient was started on ethambutol treatment as part of anti-tuberculous therapy. He was not warned that the ethambutol might affect his vision. After six weeks, the patient noted deterioration in his vision, particularly his colour vision. After stopping the anti-tuberculous therapy, a month later, his vision did not rectify itself. There was no record in the notes that the patient had been warned of possible disturbance of vision and he was not given instructions to cease therapy should there be any change in vision. Vision had not been checked prior to the start of therapy.
153
Respiratory Disorders The case was not defended and an out of court settlement was made by the hospital concerned. Good practice is to record, in the notes, when warnings have been given. Case 28: tuberculosis – side effects of therapy – hepatitis and death Anti-tuberculous drugs (in particular, isoniazid and rifampicin) affect the liver, causing a minor rise in liver enzymes. This is usually of minor consequence and requires no action. Rarely, more severe hepatitis may occur, which will rectify itself when the drugs are stopped. Very occasionally, there is more severe hepatitis, leading to hepatic necrosis, liver failure and death. All patients started on anti-tuberculous therapy are tested for liver function abnormality and are warned of possible liver inflammation. A 25 year old man with acute pulmonary tuberculosis was started on routine therapy. He developed acute hepatic necrosis after three weeks of anti-tuberculous therapy – all warnings and precautions had been taken. Regrettably, he died. Unfortunately, rare cases of liver failure are unavoidable and legal action is unlikely to succeed if the patient’s liver function has been found to be normal at the beginning and the correct warnings given.
154
CHAPTER 10
SARCOIDOSIS
INTRODUCTION Sarcoidosis is important from a medico-legal point of view for four reasons. (a) in its chronic form there may be accusations of mismanagement (and therefore increased disability); (b) it may co-exist in patients who have had other acute episodes/accidents such as chest trauma. Therefore, the dilemma in these latter cases is how much of the current disability is due to the new acute event for which there is recourse to law and compensation and how much is due to their pre-existing chronic sarcoidosis; (c) it may also come to the attention of the lawyer as part of a claim for misdiagnosis – it being on occasions difficult to differentiate between sarcoidosis and tuberculosis; (d) there may be a claim because of possible negligence in the performance of diagnostic techniques such as a Kveim test, bronchoscopy and transbronchial biopsy and mediastinoscopy.
BACKGROUND Sarcoidosis is a disease of unknown origin which affects many parts of the body. Usually, it occurs initially within the third or fourth decades. The histological appearance of tissue affected by sarcoidosis is a nodular collection of large, closely packed inflammatory cells – ‘epithelioid’ histiocytes – amongst which there are a few multinucleate giant cells and some lymphocytes at the periphery of this ‘granuloma’. Necrosis (caseating) does not occur. This is a similar type of appearance to tuberculosis, except that, in tuberculosis, the granulomata normally caseate; additionally, in tuberculosis, mycobacterium tuberculosis can often be identified by special stains. Sarcoidosis may be an infectious disease, but, as yet, the infective organism has not been isolated. There are theories concerning its similarity in appearance to tuberculosis and the possibility of a mycobacterial cause exists, but is as yet unproven.
155
Respiratory Disorders
CLINICAL FEATURES The lung is the most common part of the body to be affected by sarcoidosis and there are two main types of presentation: acute self-limiting sarcoidosis and chronic forms of the disease. Figure 10.1: bilateral hilar lymphadenopathy due to sarcoidosis – stage I
156
Sarcoidosis Figure 10.2: bilateral hilar lymphadenopathy with mid zone interstitial infiltration – stage II sarcoidosis
Acute self-limiting sarcoidosis Common features are: • vague, flu-like illness; • arthralgia (joint pains); • painful skin rash on lower legs – erythema nodosum; • enlargement of hilar lymph nodes on chest X-ray. This pattern of disease usually resolves rapidly, with the arthralgia and skin rash resolving in four to six weeks with the help of non-steroidal antiinflammatories and the chest X-ray returning to normal within a year to 18 months. Recurrence is unusual.
Chronic forms of the disease Pulmonary sarcoidosis The bilateral hilar adenopathy is associated with (or progresses to) lung infiltration, or rarer still with lung infiltration or fibrosis alone. These patterns 157
Respiratory Disorders of disease are often referred to as Stage I (hilar adenopathy alone), Stage II (adenopathy and infiltration), Stage III (infiltration) and Stage IV (fibrosis), respectively. Parenchymal sarcoidosis usually presents with: • cough; • breathlessness; • physical examination of the chest is usually unremarkable although there may be some fine inspiratory crackles at both bases; • the diagnosis is suspected by the chest X-ray changes – lung infiltration with or without lymph node enlargement.
Sarcoidosis of the skin • Erythema nodosum (see above) is common and is associated with fever, lymph adenopathy and arthralgia. Other causes of erythema nodosum include the oral contraceptive pill, tuberculosis, sulphonamide therapy and streptococcal infection. • Lupus pernio – a blue/red rash over face and nose. • Localised skin infiltration (plaques or nodules).
Sarcoidosis of the eyes • Lachrymal gland enlargement usually with salivary (parotid) gland enlargement (Heerfordt’s syndrome). • Acute iritis is relatively common, presenting with painful red eyes and impairment of vision.
Reticulo endothelial sarcoidosis • Lymphadenopathy – anywhere in the body but most commonly in the neck as well as the mediastinum. • Hepatosplenomegaly – the liver and spleen enlarge; with liver involvement the patient often feels unwell and runs a low grade fever with weight loss. Jaundice is rare.
Skeletal and neuromuscular sarcoidosis • Arthralgia. • Bone cysts. • Lower motor neurone seventh cranial nerve palsy (paralysis of one side of face). • Peripheral neuropathy. • Intracerebral sarcoid acting like space occupying lesions (tumours) may present with convulsions. 158
Sarcoidosis
Other metabolic and endocrine problems • Hypercalcaemia and hypercalciuria (high blood and urine calcium levels). Usually asymptomatic, but may lead to deposition of calcium in the kidney (nephrocalcinosis) and renal failure. • Diabetes insipidus due to sarcoid deposits in the posterior pituitary gland.
Cardiac sarcoidosis • Cardiomyopathy due to deposition of sarcoidosis in the cardiac muscle presents as either (a) cardiac arrythmias, for example, heart block, or (b) congestive cardiac failure, or even (c) sudden death. • Cor pulmonale (right heart failure) secondary to pulmonary fibrosis (rare).
INVESTIGATION AND DIAGNOSIS OF SARCOIDOSIS Plain chest X-ray This frequently shows bilateral hilar and mediastinal lymphadenopathy with or without mid zone parenchymal infiltration or fibrosis.
Lung function tests These should be performed including gas transfer. They will initially show diminution in gas transfer and then, at a later stage, there will be decrease in lung volumes if fibrosis intervenes. There is frequently co-existing airways obstruction of an asthmatic variety.
Haematology frequently, there is a lymphopenia and the sedimentation rate is nonspecifically elevated.
Biochemistry The liver function tests show an elevated alkaline phosphatase if there are liver granulomata. Serum calcium and 24 hour urinary calcium are measured to check for high calcium and hypercalciuria. Urea and electrolytes and creatinine clearance are performed to check on renal function. Serum angiotensin converting enzyme (SACE) is elevated according to the activity of sarcoidosis. It is a non-specific test, also being elevated in other granulomatous diseases. However its elevation can be used to monitor disease activity. 159
Respiratory Disorders
Bacteriology Sputum when present needs to be examined for culture and sensitivity and to exclude tuberculosis. Any biopsy specimen should also be cultured.
Gallium scans These radioisotope scans are performed in some centres. Once again, these are non-specific tests, and although they are positive in sarcoidosis and can be used repetitively for monitoring disease activity, they involve a considerable amount of radioactivity and, thus, are of limited value. They should only be repeated if absolutely necessary.
Kveim skin test This skin test involves injection of a homogenate of sarcoid spleen into the forearm of an individual with suspected sarcoidosis, and has largely fallen into disuse. Even in acute sarcoidosis, it only had a 70% positivity rate. Its main disadvantages are that it takes four to six weeks to develop and involves the injection of human material into an individual (and in the USA is banned for this reason). The test is read by the skin being biopsied and examined under the microscope.
Histology None of the previous tests is diagnostic of sarcoidosis. Hence, it is advisable to obtain a tissue sample (biopsy) wherever possible to confirm the diagnosis, although there may be exceptions in clear cut cases, such as young women presenting with a flu-like illness, arthralgia, erythema nodosum and bilateral hilar adenopathy on chest X-ray. The site of biopsy will depend upon the clinical presentation. Common biopsy sites include: • lung – bronchial or transbronchial biopsy are positive in up to 80% of patients; • lymph nodes – cervical, scalene or mediastinal lymph nodes (from mediastinoscopy); • liver biopsy; • skin biopsy; • lachrymal glands. If granulomata are found in the biopsy, careful search must be made for mycobacterium to exclude tuberculosis and, ideally, the biopsies should also be cultured to exclude this. There is a wide differential diagnosis to
160
Sarcoidosis granulomatous changes found on tissue biopsies (see figure 10.3). Thus, the biopsies and clinical features have to be interpreted in conjunction with one another. It is important to be aware of the widely differential diagnostic possibilities and particularly the difficulties in differentiating between tuberculosis and sarcoidosis on histology alone. This may well lead to trials of empirical anti-tuberculous therapy in patients who, in fact, have sarcoidosis. Figure 10.3: differential diagnosis of granulomatous disorders within the lung Infections Bacteria Mycobacteria
Brucella Mycobacterium tuberculosis Mycobacterium kansasii Mycobacterium avium Bacillus Calmette-Guérin vaccine Histoplasmosis Blastomycosis Aspergillus Coccidioidomycosis Cryptococcus
Fungi
Extrinsic allergic alveolitis Farmer’s lung Bird fancier’s lung Chemicals Beryllium Silica Starch Neoplasia Carcinoma Seminoma Reticulosis Idiopathic Sarcoidosis Wegener’s granulomatosis Churg-Strauss allergic granulomatosis Giant cell arteritis Systemic lupus erythematosus Hypogammaglobulinaemia Histiocytosis X Miscellaneous Radiotherapy
161
Respiratory Disorders
THERAPY IN SARCOIDOSIS The majority of patients with sarcoidosis need careful observation until their disease resolves spontaneously. At each clinic visit, the patient will be interviewed, examined and have a chest X-ray and/or pulmonary function tests. In patients known to be hypercalcaemic, the serum calcium level will be checked regularly and in addition the angiotensin-converting enzyme may be checked on a regular basis. In patients whose disease is not self-limiting, active therapy is indicated in the form of oral steroids (prednisolone). The accepted indications for oral steroid therapy include: • pulmonary disease which is either (a) symptomatic (progressive breathlessness); or (b) failing to clear spontaneously after up to six months’ observation based on the chest X-ray or pulmonary function. The object of therapy is to prevent permanent damage (fibrosis) of the lung; • eye involvement – always requires therapy but, usually, with steroid eye drops; • persistent hypercalcaemia or hypercalciuria; • seventh cranial nerve palsy; • intracranial sarcoidosis – often requires very high doses; • skin disease – for cosmetic appearance; • parotid or lachrymal enlargement – for cosmetic reasons; • myocardial disease – to prevent arrythmias and cardiac failure. The dose of steroids used is usually of the order of 30 mg prednisolone daily, reducing after a few months to a maintenance dose of around 10 mg daily, being controlled according to the individual’s response. Because, in chronic sarcoidosis, steroid therapy may be lifelong, it is important that an attempt is made to reduce the dose of oral steroids to the lowest possible that an individual needs. This will be done by monitoring the patient’s respiratory function and chest X-ray appearance on a regular basis. Attempts are made to reduce the dose gradually, to the point at which there is deterioration in either pulmonary function or the chest X-ray. At this point, the dose of steroids is increased slightly once more. In some patients, regrettably, steroid treatment may be lifelong and, therefore, it is inevitable that there will be side effects of steroid therapy, such as: • cataract formation • osteoporosis; • skin thinning; • easy bruising;
162
Sarcoidosis • aggravation of diabetic tendency; • increased blood pressure. There is, therefore, an obligation on the physician first, to try and minimise the dose and secondly, to offer as much protection against these side effects as possible. In the example of osteoporosis, bone densitometry should be performed to check whether there is any evidence of osteoporosis, and prophylactic treatment should be given against the formation of osteoporosis with etidranate or other drugs.
PROGNOSIS IN SARCOIDOSIS In the vast majority of patients, sarcoidosis is a relatively trivial, self-limiting disease. However, in a very few cases, particularly those with myocardial or intracranial disease, it may be rapidly fatal. Bilateral hilar lymphadenopathy takes an average of eight months to resolve with there being a spontaneous resolution rate of 80% at a year and 90% at two years. With parenchymal shadowing (Stage II), 50% of patients clear spontaneously, and a further 30% clear with steroid therapy. In Stage III disease, the outlook is worse, with only 40% of patients remitting even with steroid therapy. Regrettably, if pulmonary sarcoidosis fails to respond it leads to pulmonary fibrosis and right heart failure due to pulmonary hypertension, the clinical features of which are increasing breathlessness and ultimately death because of respiratory failure. It is important to realise that this occurs even in the hands of the most careful and experienced respiratory physician.
MEDICO-LEGAL ASPECTS OF SARCOIDOSIS Co-existing sarcoidosis – its effect upon personal injury claims It is relatively frequent for patients who have either suffered from or who are currently suffering with sarcoidosis to be involved in claims for personal injury unrelated to their sarcoidosis. In these situations it is clearly essential for the physician to be supplied by the instructing solicitors with all previous hospital and GP records, including all previous chest X-rays and respiratory function tests. It is necessary to see and examine the plaintiff, and to perform an up to date chest X-ray and measure respiratory function, so that these can be compared with the previous tests. The following two cases are demonstrative.
163
Respiratory Disorders Case 29: sarcoidosis and occupational asthma A man of 35 developed asthma because of his occupation as a laboratory animal technician (he became sensitised to male rat urine) and was complaining of breathlessness. In his past medical history, it had been noted that he had had pulmonary sarcoidosis in his late 20s. The argument was raised that part of his breathlessness was due to his sarcoidosis, rather than the industrial exposure. However, from his medical records and X-rays it was quite clear that in his mid-20s he had had a self-limiting form of sarcoidosis with bilateral hilar lymphadenopathy, with normal lung function tests. His chest X-ray had returned to normal by the age of 30 and there was no evidence of any recurrence since. He had made no further visits to his GP or the hospital because of breathlessness until developing asthma. At this time, his lung function showed airways obstruction only (typical of asthma) and chest X-ray showed no evidence of either lymphadenopathy or interstitial lung disease. There was thus no possibility of his sarcoidosis contributing to his breathlessness, which was all due to industrial related asthma. Case 30: sarcoidosis – end-stage disease or industrial accident? The plaintiff experienced an accident in which she was exposed to smoke and unspecified fumes when in a fire. She was adamant that, prior to this event, her respiratory function had been normal, but, afterwards, she had been unable to return to work and had been restricted to minimal exertion because of breathlessness. Interestingly, she was aware that she had had previous lung investigations, including two lung biopsies, and had been told that she had sarcoidosis, but she said that she had no confidence in the doctors in the two different hospitals which had made the diagnoses and that she had never believed it. The GP and hospital records and X-rays were obtained. These showed that there was little doubt that she had long standing extensive pulmonary sarcoidosis and there had been long standing problems with her complying with attendance and therapy. The ongoing decrease in respiratory function was well documented over a 10 year period prior to her accident, with many comments in the medical records of her refusal, first, to accept the diagnosis, despite biopsy proof and, secondly, to agree to take any treatment. She had lung biopsies in both hospitals. During her legal action against the construction company, for the sake of completeness, both lung biopsies were obtained and reviewed by an independent pathologist who confirmed sarcoidosis. Her respiratory function was documented throughout a 10 year period and these were plotted showing 164
Sarcoidosis a continuous decline – the rate of which had not been affected by her industrial accident. Her industrial accident occurred at very much the time that it would have been expected for her to be unable to work because of the deterioration in pulmonary function from her sarcoidosis. Although her legal advisors told her that she was unlikely to win the case (indeed, her legal aid certificate was withdrawn), she decided to pay the same amount of attention to her lawyers as she had to her doctors! She had her ‘day in court’, where she represented herself and, not only managed to lose the case, but made herself bankrupt in the process. Case 31: sarcoidosis – the wrong diagnosis A 35 year old Caucasian man felt unwell with a cough and fever and presented to his GP, who referred him for a chest X-ray which showed enlargement of the mediastinal glands, compatible with sarcoidosis. He was reassured that this was a self-limiting disease and needed no further investigation. Regrettably, three weeks later, he developed headaches, photophobia and neck stiffness. He was admitted to hospital where, after a lumbar puncture, the diagnosis of tuberculous meningitis was made. Fortunately, he was successfully treated with anti-tuberculous therapy, but in the process of the misdiagnosis and initial inadequate investigation, he needed four months off work and suffered reduced salary as a self-employed person. The medical opinions on both sides were very similar in stating that, although tuberculosis is rare in the ‘shire counties’, amongst the Caucasian population, born in this country, who have had BCG, it is an unsafe assumption to make the diagnosis of sarcoidosis where there is a differential diagnosis of possible tuberculosis on the chest X-ray, without there being further investigations to obtain histology and culture. In this instance, a transbronchial biopsy or mediastinoscopy should have been performed to exclude the possibility of tuberculosis or, alternatively, ‘blind’ antituberculous therapy should have been given. Although the defendants had not been responsible for his tuberculosis, it was pointed out that he would have had a maximum of two weeks off work if his tuberculosis been diagnosed at the time of presentation with the original abnormal chest X-ray. With earlier diagnosis, there would have been much less pain, suffering and loss of work. Case 32: sarcoidosis – negligent Kveim testing A consultant physician decided to perform a Kveim test in order to confirm the diagnosis of sarcoidosis on a young man with bilateral hilar adenopathy who was otherwise well. He asked his SHO to perform the test. The SHO obtained the Kveim material from the pathology laboratory. He had never
165
Respiratory Disorders performed (or even seen) a Kveim test before (because these are very infrequently performed). He thought that the Kveim suspension material and the black pelican ink marker should be mixed together in a syringe and injected subcutaneously, whereas, in fact, the Kveim suspension should be injected first, followed by a very small pinprick of pelican ink to mark the site, so that the biopsy site can be identified four to six weeks later. The net effect of this episode was that the patient’s sarcoidosis resolved spontaneously (as expected) but he was left with a 7.5 cm x 5 cm Indian ink tattoo on his forearm. This meant that he felt he could not wear short sleeve shirts or go swimming, and the only way to remove the tattoo involved extensive plastic surgery. The case was clearly indefensible and was settled in the form of a sum for pain and suffering and a further amount for plastic surgery to remove the tattoo. Case 33: sarcoidosis – delayed investigation? A patient with breathlessness and interstitial shadowing on a chest X-ray was thought to have sarcoidosis. The consultant judged that, as the patient was not unduly breathless, she could be booked for a routine transbronchial biopsy, which was not performed in his hospital, but in a neighbouring centre. She was therefore put onto a routine waiting list for the bronchoscopy and transbronchial biopsy. She was given no treatment whilst awaiting the result of the test. Two weeks later, the patient felt a little more breathless when going into the second hospital as a day case for transbronchial biopsy. The second hospital appeared to her to be a smarter and better equipped hospital than the first. She had the biopsy uneventfully, but at midnight, two days later, received a telephone call from the enthusiastic medical registrar who had performed the biopsy to inform her that she had a disease called sarcoidosis and needed immediate steroid therapy. The registrar asked her to attend the hospital the following morning for a prescription. Regrettably, despite the steroid therapy, the patient’s sarcoidosis continued to deteriorate for a while and she then referred herself to a third centre. Her sarcoidosis was brought under control, but with some residual fibrosis. She then investigated the possibility of legal action against the first physician on the grounds that, clearly, if the second hospital felt it so urgent that she needed to be rung at midnight, it was inexcusable that there should have been a delay of two weeks, with her (a) having no treatment; and (b) waiting for an investigation. Review of the notes and X-rays, however, showed that there had been: (a) no change in her chest X-ray over a period of the untreated two weeks; (b) no change in her lung function tests. 166
Sarcoidosis It was felt that two weeks was a reasonable time to wait for the investigation, in any case. Additionally, it is standard practice to wait several months to find out whether the patient requires steroids or not. Clearly, in this situation, the patient wished to find some one to blame for her misfortune in having developed sarcoidosis and, in these situations, it is all too common for treating physicians to find themselves in the ‘hot seat’. The situation had been aggravated considerably by the enthusiastic registrar ‘upping the stakes’ by waking a somewhat nervous patient from her slumbers to tell her of his diagnosis and making everything seem more urgent than it actually was. Understandably, the plaintiff was unhappy with the independent medical opinion, and disliked the advice given by her lawyers. Case 34: sarcoidosis – pneumothorax post-transbronchial biopsy A physician believed that a 37 year old man with interstitial shadowing on his chest X-ray and breathlessness had sarcoidosis. Thus, a transbronchial biopsy was requested and performed as a day case. This went uneventfully and the patient was sent home three hours after the procedure. An hour after returning home, the patient became acutely breathless and developed chest pain on the side of the biopsy. He was put in an ambulance and brought back to casualty, where a pneumothorax was identified on the side of the biopsy. An intercostal tube (chest drain) was inserted and the lung re-inflated. He stayed in hospital for three days and was then discharged. He sought legal advice and an independent medical opinion upon his situation, which he found unacceptable as he had not been warned that his lung might be punctured as part of the procedure. He was so incensed by this that two independent medical reports were obtained, both of which gave the same advice – that a pneumothorax is a standard complication to be expected in a limited number of patients who have a transbronchial biopsy and that, although the majority of these are immediate, a few of them may be delayed until after the patient has been discharged. There was, therefore, discussion as to whether he had or had not given fully informed consent. It was accepted that there would be argument as to whether he had been informed of the risk of pneumothorax as a potential complication. Furthermore, there would be discussion as to whether, if he had registered that he had been told that pneumothorax was a complication, this would in fact have prevented his giving his permission. At this stage, he decided to take the case no further. Case 35: sarcoidosis – the wrong patient? A 35 year old woman who had a mild flu-like illness was referred by her GP to hospital for a chest X-ray. This showed bilateral hilar lymphadenopathy ,with interstitial shadowing. She was referred to the chest clinic, where little more was obtained in history or examination. She had routine investigations and was advised on the basis of the X-ray to have a transbronchial biopsy for 167
Respiratory Disorders diagnostic purposes to confirm sarcoidosis. At the follow-up film (to exclude an immediate pneumothorax), there was no pneumothorax. Neither was there any interstitial shadowing or any lymphadenopathy! It was therefore felt that the original chest X-ray could not have been hers. On going back through the out-patient GP X-ray records, there was only one other lady who had been present at the same time as the first lady and her X-ray had been reported as normal. The second lady was recalled and a further chest X-ray taken, which, not surprisingly, showed that it was indeed she who had bilateral hilar adenopathy and interstitial shadowing. When she was referred to the chest clinic for a transbronchial biopsy, this confirmed the diagnosis of sarcoidosis. Later, when the first lady was seen at the chest clinic, she received a full explanation and apology on behalf of the radiology department and the mistake within the department was admitted. Although she was well aware of the fact that she could sue the hospital for the time and inconvenience of having an unnecessary invasive procedure, she decided to accept the apologies and take the matter no further.
168
CHAPTER 11
COMMON CONDITIONS AFFECTING THE PLEURAL CAVITY
PLEURAL EFFUSION Introduction Pleural effusion is the term used to describe a collection of fluid between the lung and the inside of the chest wall. Figure 11.1: medium sized left pleural effusion
169
Respiratory Disorders The fluid collects in the pleural space, the outer border of which (the chest wall) is lined by a membrane comprising a single layer of cells (the parietal pleura). The inner lining covering the outer layer of the lung is termed the visceral pleura. In health, this ‘space’ has zero volume, but is moistened by pleural fluid, which is secreted by the lungs and re-absorbed through the veins and lymphatics. Following inflammation, infection, invasion by cancer or blockage of local lymphatics, there is an exudation of fluid with a high protein content – protein > 30 g/litre. If fluid accumulates in the pleural space faster than the normal rate of re-absorption, a pleural effusion develops. In these circumstances, the volume of fluid can be very large. In order for fluid to be seen at all on a chest X-ray, there needs to be around 500 ml. In extreme cases, there can be a complete obscuration of one lung on the chest X-ray (a ‘white out’). In these extreme cases, 3 litres of fluid or more may have collected. By contrast, in conditions where the primary pathology is elevation of the venous pressure, fluid retention or a low serum albumin, there is transudation of fluid with a low protein content (protein < 30 g/litre) into the pleural cavity.
Causes of transudation (protein < 30 g/litre) Common causes include: • left ventricular failure (heart failure); • fluid overload – excess iv fluids; • renal failure. Rarer causes include: • hypoproteinaemia – cirrhosis of the liver; • failure of albumin synthesis; • kidney disease – excess albumin excretion; • constrictive pericarditis.
Causes of exudation (protein > 30 g/litre) Common causes include: • post-infective (pneumonia) – usually, small volume; • pulmonary infarction (pulmonary embolism) – usually, small volume and blood stained; • malignancy (usually, over 50) either direct spread or metastases from lung cancer or spread from cancer of other sites such as breast, ovary or lymphoma – often, large volume and blood stained;
170
Common Conditions Affecting the Pleural Cavity • tuberculosis – large volume, clear. Rarer causes include: • connective tissue disorders, for example, rheumatoid arthritis or systemic lupus erythematosis; • drugs; • mesothelioma – primary malignancy of the pleura associated with asbestos exposure.
Haemothorax – the collection of blood in the pleural cavity Common causes of this condition are: • trauma – blunt or penetrating injuries; • malignancy; • pulmonary infarction. In the latter two, the fluid is usually blood stained rather than pure blood.
Empyema – the collection of pus in the pleural cavity Common causes may be listed as: • bacterial pneumonia; • tuberculosis; • iatrogenic, chest aspiration or surgery; • subphrenic abscess; • ruptured oesophagus.
Chylothorax Chylothorax is a rare condition with leakage of lymph into the pleural space from an interruption, blockage or rupture of the thoracic duct which drains lymph back into the superior vena cava. Common causes include: • traumatic; • neoplastic.
171
Respiratory Disorders
Clinical features of pleural effusions Symptoms The commonest symptoms of fluid in the pleural cavity are: • increasing breathlessness – the severity of which depends upon the volume of fluid and the reserve of the rest of the lung (that is, co-existing asthma, chronic bronchitis); • coughing; • pleuritic pain particularly with infection (pneumonic), infarction, pulmonary embolism or mesothelioma (malignancy).
Physical signs On examination of the chest, the classic abnormalities observed on the affected side are: • reduction in movement; • dullness to percussion; • decreased breath sounds. These features occur over the area affected by pleural fluid.
Imaging The following procedures are useful: • PA chest X-ray – in the upright position shows opacification on the side. The height of the fluid level depends upon the volume of fluid. The position of the fluid level depends upon gravity; • decubitus chest X-ray – if the patient lays on their side the fluid level will be at right angles to that on the upright PA film; • ultrasound – in particular can be helpful for guiding aspiration and drainage; • CT – good for differentiating fluid from solid masses.
Management and investigation of pleural effusions If the patient is extremely breathless, relief of the breathlessness by drainage is the first priority. It is important to exclude hypoproteinaemia as a cause, as removal of the pleural fluid will merely aggravate the situation. Correction of the hypoproteinaemia is needed instead. With constrictive pericarditis, urgent surgical relief by drainage of the fluid causing the constriction is required. Diagnosis of the cause of pleural effusion is essential. A flow chart for an efficient management plan can be given as follows. 172
Common Conditions Affecting the Pleural Cavity Figure 11.2: diagrammatic representation of management of pleural effusions Evidence of hypoproteinaemia?
No
Yes
Treat appropriately
Evidence of constrictive pericarditis?
No
Yes
Treat surgically
Evidence of left heart failure?
No
Yes
Diuretic therapy
Clears
Pleural biopsy and aspiration
Histology culture (including AFB)
Analysis protein/sugar/amylase/microscopy including malignant cells/culture including acid fast bacilli (AFB)/rheumatoid factor/complement Diagnosis
If no diagnosis, repeat biopsy and aspiration If still no diagnosis Thoracoscopy and biopsy under anaesthesia 173
Respiratory Disorders By following the protocol and laboratory analysis, it is almost always possible to ascertain the cause of an effusion but, occasionally, the cause remains elusive. Because litigation can arise in relation to pleural aspiration and biopsy, it is worthwhile describing the techniques briefly.
Pleural aspiration and biopsy • The technique is explained to the patient. Although some may obtain written consent, it is more usual to have verbal consent for this bedside technique done under local anaesthetic. • The patient sits comfortably over the side of the bed, leaning onto a bedside table, having received a premedication of pethidine (an analgesic) and atropine (to prevent pleural shock). • The chest X-ray is checked so that the operation is on the correct side. • Under sterile conditions, the patient’s back is cleaned and the area of maximum dullness chosen by percussion, not lower than the seventh intercostal space. • The chest wall is infiltrated down to the pleura using 10 ml 1% plain Lignocaine as local anaesthetic. The approach is made over the top of a rib in order to avoid the neurovascular bundle (intercostal nerve, artery and vein) which run in a groove underneath each rib. Using this needle and syringe it is possible to check that the correct site has been chosen by aspirating 5 ml of pleural fluid into the syringe. This ensures that the approach has neither been above the level of the fluid nor below the level of the diaphragm – in which latter case there could be damage to the subdiaphragmatic organs, such as the liver or spleen. • After waiting three to four minutes for the local anaesthetic to work, a small skin incision is made with a scalpel. • An Abram’s needle is advanced and two or three biopsies of pleura are taken using this needle, avoiding taking a biopsy in the headwards direction, that is, avoiding injuring the neurovascular bundle running above the needle (under the ribs). The needle is like a crochet hook with sharp jaws which are opened and closed in order to trap a piece of tissue for histological analysis. Typically, biopsies are taken at 3 o’clock, 6 o’clock and 9 o’clock, and not at 12 o’clock. • Pleural fluid is then aspirated using a needle, syringe and three way tap and is collected into a bag.
174
Common Conditions Affecting the Pleural Cavity • The most important factor in obtaining the sample successfully is to ensure the patient and doctor are both comfortable. If the patient becomes uncomfortable for any reason, such as increasing pain, shortness of breath or coughing, the procedure should be discontinued. • The fluid may be drained more rapidly by the insertion of a larger intercostal tube such as is used for drainage of air from the pleural space – the fluid being drained by an underwater seal by the bedside. The disadvantage of this is that it is a large tube, therefore the introduction is more hazardous and it tends to be left in the patient for a longer period of time, therefore restricting their mobility. However, with large volumes of fluid the advantage of an intercostal tube is that when all the fluid has been drained (never possible with simple needle aspiration), drugs or other irritants can be inserted into the pleural space in order to obtain a pleurodesis, that is, to cause a chemical irritation which will stick the two layers of pleura together – thereby abolishing the pleural space and preventing possible fluid re-accumulation. This procedure – commonly used for malignant pleural effusions – has about 60% chance of success and is worthwhile. A variety of irritants can be used, tetracycline, bleomycin and talc being three used commonly. • Chest X-ray following aspiration to exclude hydropneumothorax and to check if any fluid is left.
Pleural fluid – analysis Once aspirated, the pleural fluid is sent to the laboratory and the following points should receive attention: • protein estimation – exudate > 30 g/l – transudate < 30 g/l; • glucose estimation – low in tuberculosis, rheumatoid arthritis, empyema or malignant effusions; • differential cell count – increased lymphocytes imply long standing effusion, tuberculosis or malignancy. Polymorph leukocytes are increased in infection. Eosinophils – increased in hypersensitivity (allergic reaction) or malignancy; • cytology for malignant cells; • bacteriology – culture and sensitivity and tuberculosis studies. Direct smears for tuberculosis are rarely positive, but culture is more commonly positive. Polymerase chain reaction (PCR) studies are becoming more widely available.
175
Respiratory Disorders
Complications of pleural effusions and drainage A number of difficulties can arise, of which perhaps the most important are: • hydropneumothorax – caused by the penetration (puncture) of the underlying lung by the sharp needle during the aspiration of pleural fluid. This is rarely large enough to require separate drainage. A post-aspiration chest X-ray is always recommended; • empyema – either before, or as a consequence of aspiration. In all cases, empyema need rapid and adequate drainage through a large intercostal tube with administration of appropriate systemic antibiotics; • pleural shock – this should be preventable by premedication with atropine, but, if it occurs during aspiration, lay the patient flat and administer further atropine intravenously immediately; • air embolism and pulmonary oedema – both extremely rare complications. The patient requires full resuscitation. Neither of these two rare complications is avoidable. If simple aspiration and Abram’s needle biopsy do not provide the diagnosis, the patient is referred to the thoracic surgeon for thoracoscopy – an operation under general anaesthetic where a telescopic instrument is inserted through the chest wall into the pleural cavity so that the pleural surfaces can be directly inspected and biopsies taken.
176
Common Conditions Affecting the Pleural Cavity
PNEUMOTHORAX Introduction Pneumothorax is the term given to the situation where the lung has ‘burst’ or been ‘holed’ and has collapsed with air escaping into the pleural space. These are termed ‘spontaneous’ when there is spontaneous escape of air from the lung as a result of abnormality of the visceral pleura – usually a ruptured bulla or bleb at the apex of a lung – or ‘traumatic’ when resulting from chest wall injury with rib fractures leading to damage and puncture of the underlying lung. Figure 11.3: left side pneumothorax in a patient with extensive bilateral pulmonary fibrosis following a percutaneous drill biopsy
177
Respiratory Disorders
Common causes of pneumothorax There are numerous causes of this condition and any or all of the following may need consideration: • ruptured pleural bleb or bulla – common in young male adults, especially those who are tall and thin (the condition may be recurrent and bilateral and there may be a family history); • trauma – surgery, stabbing, fractured rib (for example, during cardio pulmonary resuscitation (CPR) or car crash); • ruptured emphysematous bulla in chronic obstructive pulmonary disease • lung biopsies either percutaneous needle or transbronchial biopsies; • pleural aspiration; • insertion of subclavian and other central venous catheters; • rupture of cavities into the pleural space. Staphylococcal abscesses, cavitating tuberculosis or carcinoma; • rarely resuscitation and artificial ventilation particular in the new born; • external cardiac massage and resuscitation in adults; • artificial ventilation; • asthma; • connective tissue disorders; • cystic fibrosis; • Marfan’s syndrome.
Clinical features of pneumothorax Symptoms The features of pneumothorax are usually: • acute chest pain on the affected side; • breathlessness – the severity depending upon the size of the pneumothorax and the lung function.
Physical signs Clinical examination is likely to show: • diminished movement on affected side; • hyper-resonance on affected side; • diminished breath sounds on affected side.
178
Common Conditions Affecting the Pleural Cavity As there may often be difficulty in interpreting the signs, a chest X-ray is essential if the diagnosis is considered.
Investigations A chest X-ray is diagnostic. An expiratory film shows the pneumothorax as being larger than on the inspiratory film because the positive intrathoracic pressure in expiration compresses the lung.
Tension pneumothorax This is the term applied to the situation which develops when air continues to accumulate in the pleural space faster than it can be re-absorbed. This may occur if a ruptured bleb acts as a one way flap valve. A tension pneumothorax constitutes a potentially life-threatening emergency which may complicate either a traumatic or spontaneous pneumothorax and needs urgent attention. This is recognised by: • increasing shortness of breath; • shift of the mediastinum to the opposite side, that is, trachea and apex beat of the heart are displaced; • increasing tachycardia.
Management of pneumothorax The British Thoracic Society has published guidelines for the management of spontaneous pneumothorax which also contains guidance, for example, on the management of intercostal drains (BMJ (1993) 307 114–16); these are currently being reviewed and evaluated. All pneumothoraces should be managed with pain control – adequate analgesia. The next steps are variable: • small pneumothoraces will re-absorb spontaneously • in slightly larger pneumothoraces, the air should be aspirated; • for very large pneumothoraces, an intercostal tube should be inserted; • any pneumothorax which is large enough to cause distressing shortness of breath, sweating or blue discolouration must be drained immediately.
Drainage techniques used There are several techniques for the aspiration of air.
179
Respiratory Disorders
Simple air aspiration This is relatively atraumatic for the patient and the operator proceeds as follows: • a needle covered by a plastic sheath is inserted into the second anterior intercostal space after infiltration with local anaesthetic (lignocaine); • the needle is withdrawn and a three way tap and 60 ml syringe are connected to the plastic sheath; • the air is aspirated into the syringe with the patient reclining at 30%; • the process is repeated to allow the lung to re-expand slowly; • when no more air may be withdrawn despite repositioning of the patient, the plastic cannula is withdrawn; • occasionally, the lung deflates again but, usually, this therapy is adequate.
Intercostal tube drainage If it is decided that an intercostal tube is needed, this is done in a similar fashion to that described for pleural fluid drainage. All of the following steps are important: • the drain must be performed under full sterile conditions with the patient being given a premedication; • infiltration of skin and intercostal space with 1% lignocaine; • the site of insertion should be over the upper edge of the rib in the fourth or fifth intercostal space just behind the anterior axillary line; • the alternative of the second intercostal space anteriorly (although a well described site) may lead to problematical scars, particularly in young women; • a scalpel is used to cut a hole through the chest wall skin large enough to insert the intercostal tube using blunt dissection with scissors or Spencer Wells forceps down to the pleura; • a hole big enough for the insertion of the tube is made; • the tube is then placed gently through this preformed hole and then stitched in place with a purse string suture; • the tube is connected to a bottle containing 500 ml of 0.9% saline forming an underwater seal; • clamps are left by the bedside so the tube can be clamped off in an emergency; • the patient is then asked to cough to re-inflate the lung; • the tube is left in place for 24 hours after it has stopped bubbling and then removed;
180
Common Conditions Affecting the Pleural Cavity • the wound is sealed by tying the purse string suture; • if the lung fails to re-inflate and the tube keeps bubbling, this signifies a continuing air leak through the pleura (a bronchopleural fistula). Gentle suction using a pump is then applied and after several days or even weeks, the bubbling usually ceases; • very rarely, thoracotomy is needed to seal an emphysematous bulla or divide pleural adhesions.
Air aspiration versus tube drainage In general, air aspiration of pneumothoraces is recommended, in that there is less trauma and a shorter stay in hospital. However, it is not suitable for very large pneumothoraces or bronchopleural fistula or life-threatening situations where rapid air evacuation is essential.
Tension pneumothorax This is an acute medical emergency and a chest drain should be inserted immediately. If this is not available, the largest available needle should be inserted into the side of the pneumothorax relieving the tension; even though this will not inflate the rest of the lung, it will prevent the pneumothorax from increasing in size.
Complications of pneumothorax There are a number of important points here and they include: • surgical emphysema, skin crepitus due to air leaking along the drainage tube into subcutaneous tissue. It requires no therapy except in the very rare cases where the air may track up into and around the larynx causing airway obstruction. In these cases, skin incisions in the neck and in extreme cases, tracheostomy may be life-saving; • tension pneumothorax (see above); • haemopneumothorax requires drainage through a large basal tube. This is usually caused by damage to the intercostal vein or artery at the original injury, or by the insertion of the drainage tube itself. In rare cases, urgent referral to a thoracic surgeon for ligation of the bleeding vessel is necessary; • re-expansion pulmonary oedema. If the lung has been collapsed for a long time and re-inflates rapidly, there is the chance of fluid accumulating within the lung (pulmonary oedema). The patient becomes very breathless and has difficulty with gas exchange. There is little that can be done to avoid this, other than to stop the procedure if vigorous coughing occurs. If pulmonary oedema occurs, artificial ventilation may be required. 181
Respiratory Disorders
Prognosis and indications for further surgery A quarter to a third of patients with spontaneous pneumothoraces have a recurrence. After the second recurrence, consideration should be given to permanent elimination of the pleural space by referral to a thoracic surgeon, who may perform a pleuradhesis, where the pleural surfaces are roughened either medically with drugs or surgically by abrasion. Alternatively, pleurectomy is performed under a general anaesthetic – where the layers of pleura on the inside of the chest wall are removed by stripping.
Prevention of pneumothorax There are certain situations where pneumothorax is more likely to occur, although not always totally preventable. Special care needs to be taken with: • resuscitation of the new born; • positive pressure ventilation, especially in emphysematous patients; • insertion of subclavian or other central venous pressure lines (it is particularly important to avoid bilateral approaches); • percutaneous or transbronchial lung biopsies; • chest aspiration; • external cardiac massage.
MEDICO-LEGAL ASPECTS Regrettably, there are relatively frequent problems with pleural biopsies and drainage. The following cases are illustrative.
Pleural biopsy Case 36: pleural effusion – intercostal nerve damage with pleural biopsy? A 55 year old woman presented with a pleural effusion. Her pleural fluid was aspirated and a pleural biopsy performed. A diagnosis of tuberculosis was made on the basis of the biopsy. She was given the correct anti-tuberculous treatment and recovered. However, following the pleural biopsy, she complained of an area of complete loss of sensation over her chest wall in the area supplied by the intercostal nerve in the intercostal space in which she had the pleural biopsy. Her allegation was that the pleural biopsy had been done without due care and attention and that the biopsy had damaged her intercostal nerve and that, with good technique, this damage could have been avoided. 182
Common Conditions Affecting the Pleural Cavity Although theoretically possible to damage the nerve by biopsying at 12 o’clock, that is, above the needle, there was much discussion on both sides. The weakness in the plaintiff’s case was that there was no section of nerve that had been removed in the biopsy sent to the laboratory. Nonetheless, it was argued that there could be blunt injury to the nerve without it actually having been biopsied. The main problem arose in that the plaintiff’s story appeared to vary as to the distribution of the pain and area of loss of sensation – not helped by her poor command of English. To some experts, she said that there was a band of sensory loss that went at an angle around her body (which is the correct anatomical distribution), and to others (the defence!), it was a band horizontally around her body, which does not fit with a correct anatomical distribution of the nerve and, hence, is medically impossible. This variation of distribution was well demonstrated in the witness box. Whereas the plaintiff’s experts felt that she was a bona fide case, the defendant’s neurologist described her as a ‘frank malingerer’. After the plaintiff’s display in the witness box, the judge found on behalf of the defendants.
Placing of intercostal tubes The recurring themes with errors in placement of intercostal tubes are threefold: • inexperienced operator (the most junior member of the team – often one who has not inserted one before); • incorrect placement (wrong intercostal space – usually too low); • incorrect technique (most often failure to dissect a hole large enough for the intercostal tube to be merely placed into the pleural space, resulting in it being pushed in a sudden uncontrollable ‘lunge’ when the chest wall is penetrated). Case 37: pneumothorax – death from intercostal drain cardiac puncture A 30 year old woman presented to accident and emergency with a left sided pneumothorax. The senior house officer was then instructed by his registrar to insert an intercostal drain with underwater seal in order to re-inflate the lung. The senior house officer had performed this procedure on one previous occasion and thus felt competent to perform it again. Regrettably, by using an imperfect technique, which must have involved ‘boring’ his way through the chest wall with the intercostal tube and introducer, there was a sudden lunge when he reached the pleural cavity. This carried the tube and introducer rapidly through one side of the left ventricle and out through the other, thus, causing a ‘kebab’-like effect, with the heart skewered on the intercostal tube and introducer. Death resulted from exsanguination into the pleural space. The coroner was rightly critical of the 183
Respiratory Disorders surgical technique employed. This death could have been avoided by employing the correct technique of making a hole large enough for the intercostal tube by blunt dissection. Whilst death is a rare and extreme outcome, more common errors occur by tube insertion lower than the recommended fourth or fifth intercostal space. By going further down the chest, it is possible to injure or penetrate the diaphragm, causing bleeding or injury to the abdominal organs, the liver on the right side or the spleen on the left. Case 38: pneumothorax – intercostal drain causing splenic rupture A 35 year old woman fell off her bike, fractured a rib and developed a large left pneumothorax secondary to this. She presented to the local accident and emergency department. It was decided to re-inflate her lung by the use of an intercostal tube. This was inserted into the left side of her chest posteriorly in what later transpired to be the 10th intercostal space. Instead of air coming out of the tube, there was blood, accompanied by the development of sudden abdominal pain. A consultant surgeon was called. He felt that, in view of the likelihood of splenic penetration or rupture by the intercostal tube, the patient needed an emergency laparotomy and splenectomy (removal of the spleen). This was performed and the splenic rupture confirmed. At operation, a tube was inserted into her chest in the correct position and her pneumothorax resolved. Following discharge from hospital, the patient sued the trust concerned on the basis that her laparotomy was unnecessary and that she had experienced unnecessary pain and suffering. In addition, as her spleen had been removed, she was put at lifelong increased risk of infection, particularly from pneumococcal infection, which is known to occur in patients who have no spleen. Initially, the defendants tried to contest the action, on the basis that this was a possible complication of any correctly performed insertion of an intercostal tube. They also argued that the intercostal tube had indeed been inserted in the correct position. Regrettably for them, however, an intercostal tube leaves a scar on the chest wall on which a paper clip or other skin marker can be placed and an Xray taken. This was done and proved that the tube had been inserted into the 10th intercostal space. As there is no published literature supporting an approach as low as this, the case settled out of court in favour of the plaintiff.
184
CHAPTER 12
PULMONARY FIBROSIS
INTRODUCTION The lung responds to injury in a limited number of ways. One common end result from lung injury is pulmonary fibrosis (shrinking and scarring). Patients with lung fibrosis present to their doctors with a dry cough and progressive shortness of breath – initially on exertion and then later at rest. Some of the well established causes of pulmonary fibrosis are discussed in the other chapters and so, after a brief reference here, most of this chapter will be devoted to cryptogenic fibrosing alveolitis (CFA), a disease of unknown cause; and extrinsic allergic alveolitis, a disease caused by reaction to inhaled allergens. Figure 12.1: cryptogenic fibrosing alveolitis
185
Respiratory Disorders
CAUSES OF PULMONARY FIBROSIS These are legion and can conveniently be classified under the following headings.
Industrial exposure Examples of pulmonary fibrosis caused by exposure to agents in industry include: • coal miner’s pneumoconiosis; • silicosis; • asbestosis; • berylliosis; Fibrosis may occur as the end result of extrinsic allergic alveolitis: • bird fancier’s lung; • farmer’s lung.
Connective tissue disorders Pulmonary fibrosis may be associated with the following connective tissue disorders: • rheumatoid arthritis; • systemic lupus erythematosus; • scleroderma.
Miscellaneous Other causes may include: • sarcoidosis; • radiotherapy; • drugs – cyclophosphamide, bleomycin, nitrofurantoin, sulphasalazine, busulphan, gold, suxamethonium; • severe organised pneumonias; • histiocytosis X; • chronic pulmonary oedema; • long standing mitral stenosis; • lympangitis carcinomatosa (frequently, comes into differential diagnosis, but the changes in this circumstance are not due to fibrosis); • CFA (if all the above causes can be excluded). 186
Pulmonary Fibrosis
CRYPTOGENIC FIBROSING ALVEOLITIS
Clinical features of cryptogenic fibrosing alveolitis The cause of CFA is unknown, but it is characterised by the following points: • the patient is often in their 60s or 70s; • the patient suffers from increasing dry cough; • the patient suffers from increasing breathlessness; • widespread radiographic shadows in the lung fields comprising bilateral reticular nodular shadowing, mainly in the lower zones, pleural disease being rare; • widespread fine crepitations, usually at the end of inspiration; • pulmonary fibrosis shows on biopsy (if available); • subpleural fibrosis shows on CT scanning; • finger clubbing may also be present; • restricted ventilatory defect is apparent on lung function tests.
Investigations of cryptogenic fibrosing alveolitis Important diagnostic criteria include: • chest X-ray shows loss of lung volume and mainly basal reticular nodular shadowing; • CT scan shows interstitial changes with fibrosis and distortion, there is characteristic subpleural fibrosis; • lung function tests show a restrictive defect with relative preservation of peak flow rates. Thus, there is a low FEV 1 and FVC with normal or increased FEV1/FVC percentage. The total lung capacity and gas transfer are reduced – the lungs are small and stiff; • blood gases show hypoxia – initially only on exertion, but later in the disease, even at rest; • lung biopsy – it is usually advisable to have histological confirmation. Transbronchial biopsy is the safest and least invasive technique, but specimens are small and therefore may be non-diagnostic. Open lung biopsy will give a larger specimen and is to be preferred if the patient is fit enough; • histological abnormalities are twofold: (a) usual interstitial pneumonia (UIP) which is the more common; and (b) desquamative interstitial pneumonia (DIP).
187
Respiratory Disorders
Complications of cryptogenic fibrosing alveolitis The patient is at risk of two significant complications: • gradual, progressive onset of Type I (hypoxic) respiratory failure; • increased risk of lung cancer.
Therapy for cryptogenic fibrosing alveolitis Oral corticosteroids are tried in virtually all cases. However, they are successful in only about 40% of patients. Initially, 60 mg prednisone is given daily for four weeks and then, gradually, tailed down to 20 mg daily if there is a response. Alternatively, cyclophosphamide may be given daily with prednisone on alternate days. Steroids are most likely to work in the following instances: • young patients who are not breathless; • active alveolitis on bronchoalveolar lavage, gallium scan or EDTA clearance; • desquamative interstitial pneumonia (DIP) histology 60% response; • usual interstitial pneumonia (UIP) histology only 12% response; • little fibrosis present. To assess the response to therapy, the patient is seen regularly in the clinic with repeat lung function tests and chest X-ray. EDTA clearance (or gallium scanning) may be repeated, as may fine cut CT scanning. Options for treatment include: • lung transplantation for younger, fitter patients; • oxygen for the chronically hypoxic.
Prognosis for cryptogenic fibrosing alveolitis The length of time between diagnosis and death varies between one and 30 years, with a mean of six years. In general, the longer the history at diagnosis, the better the prognosis.
188
Pulmonary Fibrosis
Medico-legal aspects of cryptogenic fibrosing alveolitis By definition, CFA does not have a known identifiable cause and this excludes a large number of potential types of legal action. As CFA occurs mainly in patients in their 60s, it is also rare for it to be a co-existing factor in personal injury litigation in contradistinction to sarcoidosis, which occurs most frequently two or three decades earlier. However, if CFA does co-exist in a patient who is taking legal action for other reasons, it is essential to obtain all the old hospital notes and records, in particular previous lung function tests and previous chest X-rays to compare with up to date function.
EXTRINSIC ALLERGIC ALVEOLITIS In certain sensitised individuals, the inhalation and alveolar deposition of small organic particles leads to a local immune response within the lung which, with continued exposure, leads to inflammation in the form of granuloma formation and obliterative bronchiolitis.
Clinical features of extrinsic allergic alveolitis Acute episodes which occur four to six hours after antigen exposure are characterised by: • fevers; • myalgia; • rigors; • breathlessness without wheeze; • dry cough; • ubiquitous crepitations. Chronic exposure leads to complications – such as pulmonary fibrosis and, eventually, pulmonary hypertension – which cause: • increasing breathlessness; • weight loss; • respiratory failure Type I hypoxia; • cor pulmonale (right sided heart failure, caused by circulatory obstruction in the lungs).
189
Respiratory Disorders Figure 12.2: some examples of extrinsic allergic alveolitis and their origins DISEASE
ANTIGEN
SOURCE
Farmer’s lung
Micropolyspora faeni Thermoactinomyces vulgaris
Mouldy hay
Bird fancier’s lung Pigeon fancier’s lung
Serum protein in bird droppings
Bird droppings – budgerigar or pigeon
Maltworker’s lung
Aspergillus clavatus
Mouldy barley
Humidifier fever
Thermophilic actinomyces
Air conditioners Humidifiers
Mushroom worker’s lung
Micropolyspora faeni Thermoactinomyces vulgaris
Mushroom compost
Bagassosis
Thermoactinomyces sacchari
Mouldy bagasse (sugar cane)
}
Investigations of extrinsic allergic alveolitis In the acute phase, the chest X-ray shows mottled consolidation in mid zones. With repeat attacks there is fibrosis, particularly in the upper lobe, eventually leading to ‘honeycomb lung’.
Pulmonary function tests These show a restrictive defect with low gas transfer, small lung volumes, low FEV1, low FVC, but normal FEV1/FVC ratio, peak flow is normal. Initially, the changes are reversible by removal from antigen, but, with repeated exposure to the antigen, the lung function abnormalities persist.
Blood gases There is hypoxia and a low PaCO2 in attacks.
Skin tests Positive delayed skin test four to six hours after antigen test.
190
Pulmonary Fibrosis
Blood tests Blood tests are often performed to try to identify the cause: • serum precipitins should be sought to specific antigens (see figure 12.2). The presence of these precipitins is not diagnostic in that they may occur in some asymptomatic individuals who have merely been exposed; • blood eosinophilia – does not occur.
Treatment of extrinsic allergic alveolitis Acute episodes Treatment for acute episodes should include: • removing the sufferer from exposure; • giving oxygen in high concentration; • administering intravenous steroids, followed by oral steroids.
Chronic disease Chronic disease is treated by: • avoiding exposure – this may be difficult, in that it may involve change of job or giving up a hobby; • wearing effective mask or positive pressure powered respiratory helmets.
Compensation Farmer’s lung or malt worker’s lung are recognised occupational diseases. If identifiable cases are found related to employment exposure, legal claims may be pursued.
Prognosis The outlook for acute attacks is very good. However, after persistent prolonged exposure, pulmonary fibrosis and respiratory failure may occur, hence the importance of antigen avoidance if possible.
191
CHAPTER 13
INDUSTRIAL RELATED RESPIRATORY DISEASES
PNEUMOCONIOSIS Figure 13.1: pneumoconiosis – progressive massive fibrosis, confluent interstitial shadowing giving rise to mass-like legions
Pneumoconiosis is the term that covers the group of fibrosing lung conditions caused by dust inhalation, the most common of which are: • coal miners’ pneumoconiosis; • asbestosis – defined for industrial injury purposes as fibrosis of the lungs due to silica dust, asbestos or other dust; • silicosis.
193
Respiratory Disorders The term ‘pneumoconiosis’ also includes the lung condition ‘dust reticulation’. Pneumoconiosis is caused by inhalation of a variety of dusts. However, it is important to recognise that dust accumulation alone does not constitute pneumoconiosis. The pathological changes of fibrosis must be present within the lung to constitute pneumoconiosis.
Diagnosis Diagnosis depends upon: • chest X-ray; • clinical examination; • respiratory function tests; • complete occupational history, which should include: ❍ all jobs, with dates; ❍ exposure to dust; ❍ precautions taken for avoidance. Larger particles deposit in the larger, more central airways. These are removed from the lung by mucociliary clearance. The smaller particles (less than five microns in diameter) deposit peripherally in the respiratory bronchioles, or in the alveoli, and cause fibrosis.
COALMINERS’ PNEUMOCONIOSIS This is the most common dust disease in the United Kingdom. There are several hundred new cases each year. Because coal dust is not particularly fibrogenic, considerable exposure over 15–20 years is usually needed to cause the disease, whereas silica is far more fibrogenic and likely to cause the disease in a shorter length of time.
Clinical features Simple pneumoconiosis is caused by the lungs gradually filling with coal dust, but with little fibrosis. The clinical features are: • asymptomatic; • chest X-ray shows multiple opacities, mainly in the upper zones. The opacities vary in size from punctiform (up to 1.5 mm), miliary (1.5–3 mm) or nodular (3–10 mm); • associated chronic bronchitis is common. Complicated pneumoconiosis is due to progressive massive fibrosis (PMF). In complicated pneumoconiosis, there is: 194
Industrial Related Respiratory Diseases • • • •
breathlessness; fibrotic masses, several centimetres in diameter, in the upper lobes; bronchiectasis; cor pulmonale (right heart failure) may develop, because of the fibrosis, leading to increase in the pulmonary vascular resistance.
Therapy Therapeutic remedies include: • removal from exposure to coal dust; • treatment of existing chronic bronchitis with bronchodilators and antibiotics; • a claim for compensation and industrial related benefit.
Assessment of damage and disability The chest X-ray is the mainstay of the assessment made by the medical boards. They use the short version of the International Labour Office classification of X-rays of pneumoconiosis. These are classified according to opacity size .
Small opacities These are classified 1, 2 or 3 (to indicate the density of small rounded opacities): 1 = a few small rounded opacities; 2 = numerous small rounded opacities, normal lung markings still visible; 3 = very numerous small rounded opacities with partial or total obscuration of lung markings. The letters, p, q and r, refer to the size of the opacities: p = round opacities up to 1.5 mm in diameter; q = rounded opacities exceeding 1.5 mm and up to 3 mm; r = opacities from 3 mm to 10 mm in diameter. Standardised X-rays are used for reference.
Large opacities Category A: opacity having greatest diameter between 1 cm and 5 cm, or several opacities each greater than 1 cm. The sum of the greatest diameters does not exceed 5 cm. 195
Respiratory Disorders Category B:
Category C:
one or more opacities, the largest one more numerous than in category A, whose combined area does not exceed the equivalent of the right upper zone. one or more opacities whose combined areas exceed the equivalent of the right upper zone.
Clinical features of pneumoconiosis • Simple pneumoconiosis is asymptomic. If symptoms of chest disease such as coughing, wheezing and breathlessness, are present they are due to other lung disease, most frequently, chronic bronchitis and emphysema due to smoking. Category A lesions usually cause no symptoms, but occasionally, with larger masses, there may be severe disability. There are no typical abnormal physical signs. • Lung function test abnormalities. Again, there is no typical abnormal pattern. In simple pneumoconiosis lung function is normal unless there is co-existing other lung disease, such as chronic bronchitis. Some patients with p and q abnormalities have a small reduction in gas transfer. In progressive massive fibrosis (categories B and C), the observed lung function test abnormalities vary widely and correlate poorly with X-ray changes, the abnormalities will vary according to the position and size of masses and the presence of associated scarring or chronic bronchitis.
Disability The different types of pneumoconiosis may disable the sufferer in the following ways: • simple coalminers’ pneumoconiosis: none and no reduction in life expectancy; • category A – disability and premature death; • categories B and C – progressive massive fibrosis – severe disability, early death.
RHEUMATOID PNEUMOCONIOSIS (CAPLAN’S SYNDROME) Caplan’s syndrome is the descriptive term applied to patients with: • rheumatoid arthritis; • pneumoconiosis;
196
Industrial Related Respiratory Diseases • peripheral round pulmonary nodules 0.5–5 cm which may cavitate and surprisingly slight disturbance of respiratory function.
Differential diagnosis of pneumoconiosis and other disorders Simple pneumoconiosis must be distinguished from all other causes of multiple lung field opacities on a chest X-ray. Progressive massive fibrosis must not be confused with carcinoma or tuberculosis (simple coal miners’ pneumoconiosis does not predispose to lung cancer or pulmonary tuberculosis).
Bronchitis and emphysema Bronchitis and emphysema in coalminers is usually felt to be due to coexisting smoking. However, there is need for further investigation, particularly in dust exposed workers who are non-smokers, and any potential claim from a litigant should be the subject of a literature search, so that the up to date information is available. This is a growing area of research. At present, the medical boards, in assessing disablement, make allowance for the effects of coexisting chronic bronchitis and emphysema, in aggravating disablement, attributable to prescribed pulmonary disease.
ASBESTOS RELATED DISEASES A number of respiratory diseases can result from exposure to asbestos: • benign pleural plaques; • fibrosis (asbestosis); • bilateral diffuse pleural thickening; • diffuse malignant mesothelioma; • asbestos related lung cancer.
Asbestosis Asbestos is a general term covering a number of fibrous silicates whose main uses are as fire retardants and thermal and acoustic insulators. Although there are six varieties, only three have been in general use: chrysotile (white asbestos); amosite (brown asbestos); and crocidolite (blue asbestos). Chrysotile represents 90% of the world production of asbestos. Crocidolite, the main type associated with malignant mesothelioma, is no longer imported into the UK (since 1970).
197
Respiratory Disorders Occupational activities which expose workers to risk include: • loading and unloading asbestos sacks from boats (dockworkers); • mixing asbestos for industrial purposes, particularly spraying; • removing old lagging material in confined spaces (builders); • working with lagging and textile material (building site workers); • cutting and sawing asbestos cement products. Others may be exposed to the risk by living close to factories. Small straight fibres less than three microns in diameter are those most likely to cause harm, as these are able to penetrate furthest into the lungs.
Asbestos bodies Asbestos bodies are coated asbestos fibres which can be found in sputum or bronchoalveolar lavage. Whilst these are an indication of past exposure, they do not indicate current disease. However, their presence in a lung biopsy can help differentiate between asbestosis and other causes of fibrosis, such as cryptogenic fibrosing alveolitis.
Clinical features of asbestosis The following features are characteristic: • increasing breathlessness over a number of years; • cough; • occasional chest tightness; • finger clubbing may be present; • basal crackles, usually bilateral and unaffected by coughing.
Chest X-ray appearances Initially, on chest X-rays, there are irregular linear opacities in the costophrenic angles and, later, most of the lower zone. These become coarser with progressive disease. These opacities are categorised according to the ILO classification scale, based on increasing thickness.
198
Industrial Related Respiratory Diseases
Lung function test Lung function tests show a restrictive defect, with reduced lung capacities and gas transfer. There is hypoxia on exertion.
Diagnosis of asbestosis Diagnosis of asbestosis depends upon: • history of substantial exposure to asbestos; • persistent basal crackles; • radiological diffuse fibrosis; • impaired lung function ; Note that lung biopsy for the purpose of compensation or litigation alone is not justifiable.
Asbestosis or not? Features which indicate a cause other than asbestosis for diffuse fibrosis include the following: • rapid development and progression of disease; • little or no contact with asbestos; • crackles all over the chest or predominantly the upper zones; • coarse crackles in expiration as well as inspiration; • radiological changes in the upper zones as well as lower zones; • more impaired lung function than would be expected for the degree of Xray appearance.
199
Respiratory Disorders Figure 13.2: calcification of the diagphragmatic pleura together with left pleural plaques due to asbestos exposure
Pleural plaques from asbestos exposure are not compensatable, as they do not cause respiratory disablement. They may, however, cause anxiety. Diffuse pleural fibrosis can be caused by asbestos exposure and can occur in the absence of lung fibrosis. It can lead to a restrictive lung function impairment and disablement. In asbestos workers, it is usually bilateral. Chest X-ray shows widespread pleural thickening which classically obliterates the costophrenic angles on PA chest X-ray and thins towards the apex of the lung. Diffuse thickening over the upper zone should suggest
200
Industrial Related Respiratory Diseases causes other than asbestos. Thickening of more than 5 mm affecting more than a quarter of the height of the chest wall is significant enough to cause symptoms. There may be difficulty in differentiating between benign pleural thickening and mesothelioma or, indeed, subpleural fat. CT scanning, followed by a pleural biopsy, may be needed to be completely confident that the process is not malignant. Lung function tests in benign pleural thickening cause a restrictive defect (reduced forced vital capacity) with a normal gas transfer.
MALIGNANT MESOTHELIOMA Figure 13.3: left sided mesothelioma of the pleura due to asbestos exposure
201
Respiratory Disorders Figure 13.4: mesothelioma
Malignant mesothelioma is a primary neoplasm of the pleura, the pericardium or the peritoneum. In 85–90% of individuals, there is significant previous asbestos exposure. Crocidolite is the main form of asbestos implicated, but not the only one. There is an average of a 20 year delay between exposure and development of tumours. There is usually substantial exposure, but sometimes it is only limited.
Presentation of mesothelioma Presentation of mesothelioma includes: • persistent chest wall pain due to local invasion by tumour – local metastasis is common. Distant metastases are rare, but may occur; • pleural effusion may cause increasing breathlessness. On chest X-ray (or CT scan), a pleural mass is seen, as well as fluid; • definitive diagnosis (biopsy), usually needs to be made from either thoracotomy or thoracoscopy. Histology is not necessary for medical boards to award compensation.
202
Industrial Related Respiratory Diseases
Prognosis of mesothelioma Mean expectation of life from diagnosis is between one year and 18 months. Surgery may be attempted for small early tumours, with removal of part of the chest wall, but this is only in the very small minority of cases. They tend to be resistant to radiotherapy and chemotherapy.
Asbestos related lung cancer Lung cancer is a prescribed asbestos related disease, if occurring at the same this as asbestosis or bilateral diffuse pleural thickening. It is unknown whether asbestos exposure leads to lung cancer without asbestosis. Other occupational causes of lung cancer include: • exposure to arsenic (used in copper smelting and pesticides); • exposure to chromates (in the nickel refinery industry); • radon gas exposure (in the tin mining industry).
SILICOSIS Silicosis is pulmonary fibrosis caused by inhalation of silica dust. Occupational activities which expose the worker to risk, include: • mining of: ❍ tin; ❍ haematite; ❍ graphite; ❍ sandstone; ❍ granite; ❍ copper; ❍ gold; • working in: ❍ iron and steel foundries; ❍ sandblasting; ❍ pottery industry;. ❍ manufacture of abrasive soap; ❍ some coal mines; ❍ tunnelling; ❍ road way construction; ❍ silica manufacture;
203
Respiratory Disorders flint crushing; ❍ metal grinding; ❍ boiler scaling. Silica is much more fibrogenic than coal dust and may be complicated by tuberculosis. The chest X-ray appearance in silicosis is more abnormal than it is in simple pneumoconiosis. In particular, there are larger opacities in the upper lobes. In addition, egg shell calcification of hilar glands may occur. In the later stages, emphysema and cor pulmonale may develop. ❍
Symptoms and signs of silicosis In early disease, there are no symptoms. Gradually, a cough develops and increasing breathlessness occurs, but the sufferer feels systemically well, unless tuberculosis or heart failure supervene. In the early stages, lung function is normal. In more advanced disease, there is a restrictive defect with the total lung capacity and vital capacity being decreased with some reduction in gas transfer. In the later stages, gas transfer is markedly impaired, and in some cases there is progression to severe respiratory disability and death from respiratory failure.
TALC AND KAOLIN Pneumoconiosis due to talc or kaolin is regarded as a variant of silicosis. However, the chest X-ray appearance can be more varied. The main industries concerned with talc are: • paint industries; • cosmetic industries; • pharmaceutical manufacturing. Note that the use of talc in the rubber industry has greatly decreased. The main industries concerned with kaolin are: • china clay industry and china manufacturing; • plastics industries; • rubber industries; • paper industries.
204
Industrial Related Respiratory Diseases
BYSSINOSIS Cotton and flax dust can cause this disease in exposed workers. Workers in cotton chambers or blowing and carding rooms are most at risk, but it can occur with other processes, such as spinning, winding and weaving.
Clinical features of byssinosis Symptoms usually occur after several years of employment, and include the following: • chest tightness: this typically occurs on return to work on Monday or the first shift of the week, or following a holiday or longer break. The following day the patient feels normal and usually does so for the rest of the week. There may be no further progression of symptoms for years. After many years, they may notice that the chest tightness persists further into the week, although symptoms are usually worst on Mondays; • chronic cough and breathlessness may develop. Initially, symptoms are reversible. Later, they are not. The following examinations will usually reveal abnormalities: • chest examination: will appear normal in early cases; wheezing may become apparent in later examinations; • lung function tests: usually portray an obstructive picture (fall in FEV1 and peak flow) during the working day or week. Initially reversible, but this becomes permanent with time; • chest X-ray: appears normal.
OCCUPATIONAL ASTHMA Occupational asthma (reversible airways obstruction) is caused by exposure to sensitising agents at work. Initially, there is a period of exposure to agents before the symptoms of sensitisation appear. Usually, when a worker is removed from exposure to the sensitising agent, their asthma remits, but symptoms may sometimes persist. Re-exposure precipitates further asthma attacks and some subjects may experience asthma attacks when exposed to non-specific irritants, such as laughing, coughing, exercising, cigarette smoke, or change in ambient temperature. The pattern of asthma observed may be ‘immediate’ (occurring a short time after exposure), ‘late’ (occurring several hours after exposure), or ‘intermediate’ (between the two). There may be an associated rhinitis or hay fever-like reaction.
205
Respiratory Disorders
Differential diagnosis of occupational asthma The most frequent problem is differentiating between those asthmatics in whom asthma is unrelated to their occupation and those who have been sensitised by a sensitising agent. The most important features in making a diagnosis of occupational asthma include: • a full and accurate clinical and occupational history; • checking carefully through all the general practitioner and hospital notes for the pre-existence of asthma prior to occupational exposure. Preexistence may take the form of asthma diagnosed per se or prescriptions of bronchodilators, such as salbutamol, or previous peak flow rates being recorded by the GP. The diagnostic points that should be sought are: • presence of a sensitising agent at work; • exposure to the agent for a period from days to months or years before asthma develops; • improvement of symptoms when sufferer is away from work: weekends, holidays; • on return to work, increased sensitivity is apparent when exposed to much smaller amounts of the agent than before asthma developed. Aids to diagnosis of occupational asthma include: • skin prick tests; • blood tests; • lung function tests; • bronchial provocation tests.
Skin tests Skin tests are of use in sensitivity to: • platinum salts; • proteolytic enzymes; • laboratory animals (proteins).
Blood tests Blood tests may be difficult to interpret and are of little help.
Lung function tests Lung function tests will confirm the diagnosis of asthma, but a single set of readings will not prove that it is occupational. Lung function tests are useful 206
Industrial Related Respiratory Diseases to assess the degree of disability. A series of peak flow recordings at home and at work is particularly useful.
Bronchial provocation tests Bronchial provocation tests should only be carried out by specialist units, because of the risks involved (induction of severe bronchospasm). In inexperienced hands, they may be dangerous. They should not be carried out routinely, nor solely for the purpose of litigation.
Clinical features Clinical features of occupational asthma include: • coughing; • wheeziness; • breathlessness; • chest tightness. Patients with pre-existing asthma are not excluded from claiming for subsequent occupational asthma due to sensitisation to a prescribed agent, if it has aggravated their asthmatic symptoms and worsened their disability. However, if their asthma is made worse in a non-specific way, by a prescribed agent, without evidence of sensitisation, they would not normally be diagnosed as having occupational asthma. Sensitising agents encountered at work include a large and ever-increasing number of substances and an up to date Medline search is always indicated on behalf of a plaintiff. Some well described causes include: • isocyanates used in the manufacture of plastic foam, inks, paints and adhesives; • acid anhydride and amine hardening agents, including epoxy resins: found in adhesives, plastics and surface coatings; • soldering rosin (colophony), solder fluxes; • platinum salts; • flour and grain dust, to which workers involved in milling, baking, harvesting, drying, transporting and storing grain (barley, oats, rye, wheat and maize) may be exposed; • laboratory animals and insects: rats, mice, guinea pigs, rabbits, cockroaches; • proteolytic enzymes: workers involved in the manufacture of washing detergents, brewing, baking, workers in the fish, silk and leather industries may be exposed;
207
Respiratory Disorders • pharmaceuticals: penicillin, cephalosporin and cimetidine manufacture may put the worker at risk; • plant material, wood dust: red cedar, mahogany, ispaghula powder used in bulk laxatives, castor bean used in ipecacuanha manufacture; • chemicals: azodicarbonamide, expanded foam for walls and floor insulation.
Prevention and treatment Occupational asthma may be relieved or prevented in the following ways: • substitution of sensitising material by one which is less hazardous; • total enclosure of the hazardous process; • improved exhaust ventilation; • personal respiratory protection and vented clothing; • surveillance of workers who are exposed.
EXTRINSIC ALLERGIC ALVEOLITIS This term covers a group of diseases caused by the inhalation of organic material, leading to the development of granulomatous inflammation in the lungs, associated with the presence of antibodies (precipitins) in the blood. The condition has been described in more detail in Chapter 12. Examples include: • farmer’s lung: caused by inhalation of mouldy hay; • bird fancier’s lung; • mushroom workers’ lung; • maltworker’s lung: inhalation of mouldy barley or malt; • bagassosis: inhalation of mouldy cane bagasse;
Farmer’s lung This is caused by the inhalation of mouldy hay, which is usually found to occur between January and March. The main antigens are thermoactinomyces vulgaris (micropolyspora faeni). In the acute phase of the disease, serum precipitins are usually positive, but they are also found to be positive in around 20% of unaffected farmers. The absence of precipitins does not exclude the diagnosis.
208
Industrial Related Respiratory Diseases
Clinical features Some hours after exposure, the patient may experience: • acute coughing; • severe breathlessness; • scanty sputum which may be blood stained; • fever; • sweating.
Examination and investigations Examination will usually reveal: • crackles present over the lungs; • chest X-ray shows miliary mottling in the middle and lower zones, or patchy, ill defined shadowing; • lung biopsy shows granuloma; • lung function tests show a restrictive ventilatory defect, with decreased FVC and reduced gas transfer; • hypoxia. The attack normally wears off three to four hours after removal from exposure. With repeated attacks, resolution is slower. With chronic disease, there are non-specific irregularities and honeycomb lung with fibrosis. The differential diagnosis of farmer’s lung includes the following conditions: • influenza; • asthma; • allergic bronchopulmonary aspergillosis; • pneumonia; • left ventricular failure; • acute bronchitis; • chronic bronchitis and emphysema; • cryptogenic fibrosing alveolitis; • sarcoidosis.
CONDITIONS WHICH ARE CAUSED BY INHALATION OF OXIDES OF NITROGEN The following gases are important: • nitrogen dioxide;
209
Respiratory Disorders • nitric oxide; • nitrous oxide; • dinitrogen tetroxide; • dinitrogen trioxide; • dinitrogen pentoxide; Together, these are termed the oxides of nitrogen. When liberated into the atmosphere, nitric oxide reacts to form ozone which is harmful to both plants and animals.
Clinical features Mild exposure is irritating, and causes coughing. High concentrations may produce laryngospasm. Usually, the initial symptoms are minor, but can be followed after two to 30 hours by increasing respiratory distress which may be very severe. Sometimes, profound hypoxia can develop, and may even result in death. This is caused by pulmonary oedema. The clinical features may be difficult to differentiate from pneumonia. Two to six weeks after this (in the survivors), another acute respiratory illness – bronchiolitis – can occur, even without further exposure, this usually takes two or three weeks to resolve, but, again, this may prove fatal.
CADMIUM Cadmium is used in the manufacture of electrodes in alkaline batteries, jewellery, nuclear reactors, and fluorescent lamps.
Features of acute cadmium poisoning Features of acute Cadmium poisoning include: • flu like symptoms; • breathlessness; • chills; • sweating; • chest tightness; • chest pain; • chest crepitations; • gradual improvement usually occurs, but death may result.
210
Industrial Related Respiratory Diseases
Features of chronic cadmium poisoning Features of chronic cadmium poisoning can include: • emphysema; • nephrotoxicity.
BERYLLIOSIS Beryllium was previously frequently used in phosphorescent light bulbs and neon signs. It can cause pneumonitis, often associated with skin rashes, rhinitis and conjunctivitis.
HARD METAL DISEASE Hard metal is a synthetic alloy of tungsten carbide and cobalt. It is used in high speed cutting tools, drills, bullets and armour plating. It can cause either fibrosis or asthma.
211
CHAPTER 14
RARE RESPIRATORY DISEASES
ASPERGILLUS IN THE LUNG Aspergillus comprises a group of fungi which may cause a number of distinct pulmonary diseases. The most common fungus causing lung disease is Aspergillus fumigatus.
DISEASES CAUSED BY ASPERGILLUS FUMIGATUS A number of diseases may be caused by the ubiquitous fungus Aspergillus fumigatus. These include the following.
Simple extrinsic asthma In cases of simple extrinsic asthma, the patient presents with breathlessness and wheezing on exposure to Aspergillus spores. Immediate skin test to Aspergillus is positive.
Allergic bronchopulmonary aspergillosis Allergic bronchopulmonary aspergillosis consists of asthma with eosinophilia in the blood or sputum associated with transient segmental areas of collapse and consolidation on chest X-ray (most often in the upper lobes) beyond mucus plugs which consist of Aspergillus hyphae. This may result in proximal bronchiectasis (chronic infection) and requires oral steroid treatment which is occasionally lifelong. Aspergillus skin test is positive, as are serum precipitins.
213
Respiratory Disorders Figure 14.1: patchy shadow and partial collapse of the right upper lobe due to bronchopulmonary aspergillosis
214
Rare Respiratory Diseases Figure 14.2: the same patient as shown in figure 14.1 – separate episode of right upper lobe collapse/consolidation
215
Respiratory Disorders
Aspergilloma A fungal ball develops in a cavity usually formed from previous tuberculosis or sarcoidosis. Figure 14.3: large aspergilloma in an old tuberculous cavity at the left apex
Note the crescent of air outlining the upper margin of the intracavity fungal ball.
216
Rare Respiratory Diseases Figure 14.4: aspergilloma in cavity
Common clinical features The following are common clinical features present in diseases caused by Aspergillus fumigatus: • haemoptysis – which may be copious and even life-threatening; • lethargy; • weight loss.
Investigations Investigations will show: • characteristic chest X-ray and CT scan appearances of an opacity within a cavity; • sputum grows Aspergillus; • positive serum precipitins to Aspergillus.
217
Respiratory Disorders
Therapy Therapy includes: • in an asymptomatic patient – nil; • surgical removal is the treatment of choice for solitary symptomatic lesions.
Invasive aspergillosis In patients who are immunosuppressed because of cancer chemotherapy or HIV infection, Aspergillus may cause an invasive pneumonia, particularly after broad spectrum antibiotic cover.
Investigations • • • •
Sputum grows fungal hyphae. Positive Aspergillus serum precipitins. Chest X-ray shows consolidation sometimes with abscess formation. Lung biopsy shows fungal hyphae.
Therapy • Intravenous amphotericin or other anti-fungal agent. • Oxygen. Despite treatment, the prognosis is often poor.
ASPERGILLUS CLAVATUS Exposure to Aspergillus clavatus can cause extrinsic allergic alveolitis (eg, malt worker’s lung). Diagnosis is made from a history of exposure and the presence of symptoms suggestive of allergic alveolitis. If untreated, pulmonary fibrosis may eventually occur. Compensation is payable under the Industrial Injuries Act.
PULMONARY EOSINOPHILIA A rare group of conditions characterised by: • fluffy, transient shadows on chest X-ray; • blood eosonophilia; • sputum eosinophilia. They are subdivided into those patients with or without asthma: 218
Rare Respiratory Diseases
With asthma The usual causes of eosinophilia associated with asthma are: • allergic aspergillosis – 60%; • other – 40% cause rarely ever found.
Without asthma Eosinophilia without asthma may be caused by: • simple eosinophilia (Loeffler’s syndrome). A short illness with chest X-ray changes. Causes include fungi (Aspergillus), worms – ascaris, ancyclostoma, taenia, saginatum, trichuris, toxocariosus – and drugs – sulphonamides, nitrofurantoin, anti-tuberculosis treatment, gold, penicillin, aspirin; • tropical eosinophilia. Filariasis is the most common cause.
Clinical features of pulmonary eosinophilia The clinical features of pulmonary eosinophilia are: • coughing; • shortness of breath; • fever; • chest pain.
CONNECTIVE TISSUE DISORDERS AND THE LUNG Rheumatoid arthritis Rheumatoid arthritis is a painful disfiguring condition usually affecting the hands and feet as well as larger joints such as the knees. Lung complications are relatively unusual but may include: • pleurisy and pleural effusion; • bronchiolitis obliterans; • bronchiectasis; • pulmonary fibrosis; • rheumatoid nodules (which may be multiple). These may provide a difficulty in diagnosis from lung cancer; • Caplan’s syndrome in coal workers with pneumoconiosis. Large nodules which may cavitate.
219
Respiratory Disorders
Systemic lupus erythematosus This is a rare disorder which often presents as a ‘butterfly rash’ on the face with arthralgia, lymphadenopathy and fever, most commonly in young women. Lung complications may include: • pleurisy and pleural effusions – both are common. Effusions are small; • lupus pneumonia. Bilateral patchy pneumonia with fever and hypoxia; • ‘shrinking lung’ – restrictive ventilatory defect. The diminished lung volume and size is thought to be due more to respiratory muscle weakness than lung fibrosis.
Therapy Therapy is with high dose steroids and other immunosuppressants.
Polyarteritis nodosum Polyarteritis nodosum is a rare inflammatory disease of unknown cause in which medium sized blood vessels in body organs are inflamed. It is associated with hypertension, neuritis, mononeuritis multiplex and a rheumatoid-like arthropathy. Pulmonary features may include: • asthma; • eosinophilia; • pneumonia.
Therapy Treatment is with steroids and immunosuppressants.
Systemic sclerosis A rare condition where the skin and connective tissues in the body become thickened. The facial features become taut, the fingers become tight. As part of this process the lung may become involved causing: • pulmonary fibrosis; • aspiration pneumonia with associated oesophageal motility problems.
Ankylosing spondylitis This condition is primarily a fusion of the spine which restricts movement and may result in a motionless ‘bamboo’ spine. 220
Rare Respiratory Diseases
Clinical features affecting the lung Clinical features affecting the lungs include: • restriction of chest wall movement; • fusion of the costovertebral joints; • leads to increasing breathlessness; • increased number of respiratory infections including pneumonia; • apical fibrosis which may cavitate simulating tuberculosis; • aspergilloma within these cavities; • respiratory failure, particularly after upper abdominal surgery when diaphragmatic movement may become difficult due to pain (chest wall movement greatly reduced). Figure 14.5: severe kyphoscoliosis
Vertebrae arrowed. 221
Respiratory Disorders
Kyphoscoliosis Kyphoscoliosis is a double curvature of the spine. Kyphosis is antero-posterior curvature (concave forwards) and scoliosis is from side to side in an ‘S’ shape. This arises from one of the following: • congenital abnormality; • previous thoracic surgery; • idiopathic; • neuromuscular skeletal diseases – tuberculosis, ankylosing spondylitis, osteoporosis, polio. Scoliosis causes more problems than kyphosis. Severe scoliosis can lead to progressive shortness of breath with restrictive lung volumes, hypoxia with ventilation perfusion mismatch and pulmonary hypertension leading to cor pulmonale and respiratory failure, often in the fifth or sixth decades of life.
Therapy The usual treatment for kyphoscoliosis is as follows: • surgical correction in the young; • antibiotics for secondary infection; • diuretics for secondary heart failure; • oxygen is given both for acute problems and also long term domiciliary therapy with chronic hypoxia; • assisted ventilation, continuous positive airways pressure when in established respiratory failure.
Prognosis Unfortunately, the prognosis for severe scoliosis is often poor once serious complications such as respiratory failure have appeared. Long term oxygen therapy may prolong life.
222
Rare Respiratory Diseases Figure 14.6: cystic fibrosis – hyperinflated lungs with marked upper zone interstitial shadowing and basal tramline shadows due to bronchiectasis
Cystic fibrosis Cystic fibrosis is a rare genetically linked disease which occurs in one in 2,000 live births. It affects the body’s mucus secreting glands. The clinical features of the disease relate mainly to the increased viscosity of this mucus. There is defective chloride transport across the respiratory and sweat duct epithelial cells with increased sodium transport.
223
Respiratory Disorders
Clinical features Usually, the disease presents in childhood but, in milder cases, may present in teenagers or even adults. Common presentations include: • recurrent upper and lower respiratory infections leading to bronchiectasis causing cough, purulent sputum, wheeze and progressive shortness of breath; • intestinal obstruction in neonates; • recurrent abdominal pain in older children; • faecal impaction and rectal prolapse; • malabsorption and growth retardation due to pancreatic insufficiency; • liver cirrhosis; • nasal polyps (adults); • recurrent bronchiectasis; • pneumothoraces; • respiratory failure and cor pulmonale; • pancreatic insufficiency and diabetes (10–20%); • recurrent bowel obstruction.
Investigations The following points may help to confirm the diagnosis: • chest X-rays show hyperinflation with scattered area of infection and gradual destruction of lung architecture; • lung function tests show progressive airways obstruction with hyperinflation; • sweat tests (which are more reliable in children than adults) show increased concentration of sodium chloride. Neonatal screening includes the Guthrie test.
Therapy Out-patient treatment Out-patient treatment includes: • regular physiotherapy and postural drainage; • oral and nebulised antibiotics; • bronchodilators; • pancreatic replacement therapy for malabsorption;
224
Rare Respiratory Diseases • high protein and high calorie diet; • extra fat soluble vitamins (A, D, E and K); • extra salt in hot weather; • oral or nebulised mucolytics. In-patient treatment Acute infective exacerbations and pneumothoraces may lead to hospital admission. The main infecting organisms are pseudomonas, staphylococcus aureus and haemophilus. In-patient treatment includes: • intravenous antibiotics; • physiotherapy with postural drainage; • oxygen; • salbutamol nebulisation; • diuretics.
Prognosis With vigorous physiotherapy and antibiotics, prognosis is greatly improved, with 80% of children living to adult life. With most severe disease, lung transplantation may be considered.
Goodpasture’s syndrome This is an immune mediated disease of unknown cause in which immune complexes form; these cross-react between kidney glomeruli and the lung alveolar basement membrane.
Clinical features Clinical features are: • intrapulmonary haemorrhage associated with haemoptysis, hypoxia and diffuse pulmonary infiltrates; • associated renal failure due to glomerulonephritis.
Therapy Early treatment is essential and includes: • high dose steroids; • immunosuppression with drugs such as cyclophosphamide or azathioprine; • diagnosis is made by high index of suspicion and the presence of antiglomerular basement membrane antibodies. 225
Respiratory Disorders Goodpasture’s syndrome is a rare disease with highly specialised therapy which is best undertaken by referral to a centre with expertise in the management of this condition.
Wegener’s granulomatosis This is a rare disease of unknown cause in which there is granulomatous inflammation, occurring mostly in midline structures. Clinical features include: • nose bleeds; • coughing of blood from inflammation of pulmonary blood vessels (pulmonary vasculitis); • glomerulo-nephritis leading to renal failure.
Diagnosis Diagnosis is made by: • high index of clinical suspicion; • chest X-ray showing either diffuse or nodular shadowing, often bilateral; • haematuria and proteinurea; • hypertension; • deteriorating renal function; • diagnosis may be made on histology but with the typical clinical appearances a positive cANCA (cytoplasmic anti-nuclear cytoplasmic antibody) blood test is adequate.
Therapy Wegener’s granulomatosis is treated with high dose steroids and other immunosuppressants, such as cyclophosphamide or azathioprine, and careful observation of renal function.
226
CHAPTER 15
MISCELLANEOUS CONDITIONS AFFECTING THE LUNGS
SICKLE CELL DISEASE Sickle cell disease is a blood disorder which affects the non-white population, often of Afro-Caribbean origin. There are around 5,000 patients at risk in the UK. It is caused by substitution of a single amino acid in a beta chain of haemoglobin S. Such patients are at risk from a syndrome called sickle cell crisis and a severe chronic anaemia caused by the red cells breaking down more easily and blocking small blood vessels. As the disease can also affect the chest, it needs to be mentioned here.
Clinical features of sickle cell disease The sickle cell crisis may take one of three forms: (a) occlusion of blood vessels leading to severe pain from tissue death (necrosis) in the region of the blood supply such as bones, abdominal viscera, or chest; (b) bone marrow aplasia (failure), often induced by viruses; (c) sequestration (blood pooling) in the spleen or liver leading to circulatory collapse. When the disease affects the chest, the most frequent characteristics are: • chest pain; • fever; • raised white cell count; • basal shadows on chest X-ray.
Investigations The following investigations should be performed: • culture from throat; • sputum and blood culture; • blood count and electrolytes; • viral and mycoplasma screen; • pneumococcal antigen; • blood gases or oximetry (oxygen status); • chest X-ray. 227
Respiratory Disorders
Therapy for sickle cell disease Therapy needs to include: • pain relief (for example, pethidine) – checking that respiration is not depressed; • rehydration – intravenously or nasogastrically; • oxygen to restore oxygen saturation; • antibiotics – intravenously initially; • in very severe cases, artificial ventilation where there is respiratory failure. Figure 15.1: non-cardiogenic pulmonary oedema in a patient with ARDS
228
Miscellaneous Conditions Affecting the Lungs
ADULT RESPIRATORY DISTRESS SYNDROME – SHOCK LUNG Shock lung (ARDS) is the term used to cover a variety of non-cardiac causes of pulmonary oedema (fluid in the lungs) which lead to lung injury typified by increased pulmonary capillary permeability. This is usually induced by chemical mediators released from cell damage (cytokines). It is most frequently seen in the intensive care unit either as a reason for admission to the unit or developing whilst a critically ill patient is being treated there.
Causes of adult respiratory distress syndrome There are numerous causes of adult respiratory distress syndrome (ARDS) and any of the following may be responsible: • inhalation of smoke and fumes; • viral pneumonia; • gram-negative sepsis; • cardiopulmonary bypass; • re-expansion of lung after pneumothorax; • aspiration of gastric acid; • heroin overdose; • near drowning; • hypothermia; • altitude sickness; • neurogenic – following head injuries; • major trauma; • acute pancreatitis.
Clinical features of adult respiratory distress syndrome The condition is characterised by the following points: • the patient is very sick; • tachycardia (rapid pulse rate); • tachypnoea (rapid respiratory rate); • hypoxia (PaO2 less than 10 kPa in spite of greater than 50% inspirated oxygen concentration); • bilateral alveolar shadowing on chest X-ray with characteristic sparing of costophrenic angles;
229
Respiratory Disorders • • • •
normal heart size; Type I respiratory failure; need for artificial ventilation to obtain adequate oxygenation; pulmonary artery wedge pressure less than 18 mm Hg (that is, normal).
Therapy in adult respiratory distress syndrome Appropriate treatment for ARDS includes: • maximising tissue oxygenation – by giving high inspired oxygen (60%); • ventilation with positive end expiratory pressure (PEEP) if necessary; • maintaining cardiac output; • correcting hypovolaemia; • inotropes – dopamine, dobutamine; • correcting phosphate deficiency to help improve oxygen carriage; • avoiding the use of steroids, as these do not improve survival; • treating co-existing disease (such as infection) aggressively.
230
Miscellaneous Conditions Affecting the Lungs Figure 15.2: sleep apnoea
SLEEP APNOEA Sleep apnoea is defined as the occurrence of 12 apnoeic episodes (cessation of breathing) of at least 10 seconds’ duration per hour. The apnoeic episodes occur during REM (rapid eye movement) sleep. It is 10 times more frequent in men than women. Two main types are recognised: both may occur simultaneously. 231
Respiratory Disorders
Obstructive sleep apnoea This is where there is no airflow at the nose or mouth but normal or increased chest wall movement. This implies obstruction in the upper airway either in the oro or nasopharynx.
Central apnoea There is neither chest wall movement nor airflow at the nose or mouth and implies defective respiratory drive within the nervous system.
Causes Causes of obstructive sleep apnoea include: • obesity; • acromegaly; • myxoedema; • superior vena caval obstruction; • large tonsils and adenoids; • idiopathic (no identifiable cause) – the largest group.
Clinical features Clinical features of obstructive sleep apnoea include: • snoring; • drowsiness in the day; • morning headache (due to lack of oxygen); • intellectual deterioration; • personality change; • mental problems; • impotence; • enuresis; • abnormal movements during sleep; • sleep walking; • cardiac dysrhythmias; • polycythaemia; • pulmonary hypertension; • cor pulmonale (right heart failure).
232
Miscellaneous Conditions Affecting the Lungs
Investigation Investigation of sleep apnoea includes sleep studies during which sleep and breathing are observed. The breathing pattern and continuous monitoring of oxygen saturation during sleep, together with chest wall movement, are monitored.
Therapy Treatment of sleep apnoea mainly concerns the treatment of associated diseases and involves: • weight loss; • pharyngeal wall surgery; • tonsillectomy and adenoidectomy if these are responsible for obstruction; • continuous positive airways pressure (CPAP).
THERMAL INJURY (SMOKE INHALATION) Most deaths in fires are caused by asphyxiation. However, anyone who has been exposed to toxic fumes or smoke needs to be observed in hospital for 24 hours in case they develop complications such as: • acute airways obstruction associated with wheeziness, coughing, blackened sputum due to mucosal swelling and spasm of the air passages; • pulmonary oedema due to damage of the lung tissue. This reaction is often delayed up to 24 hours after exposure. It is associated with acute shortness of breath with wheezing. Blackening of the nasal and oral passages gives a clue to those who are most severely affected.
Treatment of smoke inhalation The following points are important: • remove patient from source of smoke or fire; • administer oxygen (humidified); • administer intravenous steroids; • avoid overhydration; • provide artificial ventilation if required; • administer antibiotics for superadded infection.
233
Respiratory Disorders
Diagnosis and quantification of damage where smoke has been inhaled The vast majority of people who have been exposed to smoke have only minor exposure and have no consequences. However, a small number have had severe exposure which leads to permanent lung damage; this is likely to be only part of their overall claim for personal injury as they are likely to have extensive burns elsewhere. However, assessment of their personal injury claim will need to include assessment of pulmonary function, lung function tests and a chest X-ray.
CARBON MONOXIDE POISONING There are about 1,000 deaths in the UK per annum from carbon monoxide poisoning (about a third of all deaths from poisoning). Although poisoning may be as a result of attempted suicide from car exhaust fumes, a common cause is faulty gas appliances, in particular boilers, where there is a build-up of carbon monoxide as an incomplete product of combustion within a house or flat. Carbon monoxide has a high affinity for haemoglobin (200 times that of oxygen) and combines to form carboxyhaemoglobin. This carboxyhaemoglobin prevents the uptake of oxygen onto the haemoglobin and reduces oxygen delivery throughout the body. The half life of carboxyhaemoglobin is five hours, thus, the effect is quite long lasting.
Clinical features of carbon monoxide poisoning Symptoms vary according to extent and length of exposure to carbon monoxide.
Acute heavy exposure Acute heavy exposure is characterised by: • coma; • convulsions; • death; • pallor, which is more common than the cherry pink colour classically described; • skin necrosis at contact points.
Chronic exposure Chronic exposure will cause: • fatigue; 234
Miscellaneous Conditions Affecting the Lungs • headache; • confusion; • nausea and vomiting; • diarrhoea and abdominal pain. Late consequences in up to 40% of patients include: • headaches and irritability; • personality change; • confusion; • memory loss
Investigations Investigations for carbon monoxide poisoning should include examination of the following: • carboxyhaemoglobin level – although this does not correlate well with the clinical picture; • ECG to check for myocardial infarction; • blood gases – check for acidosis.
Therapy Management of carbon monoxide poisoning should include: • removal of the patient from source; • mouth to mouth resuscitation; • oxygen in high concentration; • if unconscious, the patient may need artificial ventilation; • hyperbaric oxygen can increase the elimination of carbon monoxide but there are few centres in the UK that are able to provide this form of treatment.
HIV INFECTION AND THE LUNG Respiratory problems are very common in patients with HIV infection, particularly in the later stages with the development of the acquired immune deficiency syndrome (AIDS). However, relatively early in the development, patients with HIV are more prone to infection with pulmonary tuberculosis and thus HIV infection should be considered in patients presenting with tuberculosis, particularly if the patient has come from an area of high prevalence of HIV infection, such as Central Africa, or belongs to a high risk group. 235
Respiratory Disorders Figure 15.3: pneumocystis pneumonia in patient with AIDS – bilateral ground glass appearance in both lower and mid zones
Respiratory diseases in HIV infection The lung is a very commonly affected organ in patients with HIV infection. The symptoms may be minor or major.
Minor symptoms Minor respiratory infections in HIV patients include: • common cold; • influenza.
236
Miscellaneous Conditions Affecting the Lungs
Major symptoms More serious symptoms include: • pneumonia: ❍ pneumocystis carinii (PCP); PCP affects 85% of patients with AIDS at some time; 35% of episodes are recurrent; PCP is sole pathogen (70% of cases); or with other pathogens simultaneously (15%). Other pathogens (15% of cases) affecting the lung include: • cytomegalovirus (CMV); • mycobacterium avium intracellulare; • mycobacterium tuberculosis; • pneumococcus; • haemophilus influenzae; • legionella; • klebsiella; • Kaposi’s sarcoma (KS): 15% of patients have pulmonary involvement; • lymphoid interstitial pneumonitis (LIP).
Clinical features of respiratory involvement in HIV infection – pneumocystis pneumonia These include: • persistent dry cough for two to three weeks; • fever; • malaise, for several months; • increasing breathlessness (often for two to three weeks); • feeling of inability to expand chest fully; • progressive generalised lymphadenopathy; • progressive weight loss; • history of high risk group (homosexual, bisexual, intravenous drug abuser, previous blood transfusion); • oropharyngeal candidiasis; • hypoxia – first only on exertion, later at rest leading if untreated to Type I respiratory failure; • chest examination normal or a few bilateral crackles; • Kaposi’s sarcoma (in some patients).
237
Respiratory Disorders
Investigation of patients with HIV who may have developed lung involvement Investigations will reveal: • HIV test positive (test done after counselling and with the patient’s permission); • chest X-ray shows bilateral diffuse perihilar ground glass alveolar appearances. Classical ‘bat’s wings’ distribution. Many other patterns are possible – as is a normal chest X-ray initially. Focal segmental shadows suggest other bacterial causes; • lung function tests show reduced gas transfer; • blood gases show decreased PaO2 with normal or low PaCO2; • sputum or induced sputum should be sent for Gram stain, culture and sensitivity for ordinary bacteria, plus for pneumocystis and tuberculosis; • bronchoalveolar lavage sent for pneumocystis, tuberculosis and CMV. Many centres reserve bronchoalveolar lavage for those patients who are not responding to first line antibiotic treatment.
Therapy The following treatment is normally given for pneumocystis pneumonia: • oxygen in high enough concentration to treat hypoxia; • high dose intravenous cotrimoxazole; • methylprednisolone (steroids) intravenously; • prophylaxis against recurrent pneumocystis should begin with either cotrimoxazole or Fansidar. Otherwise, there will be a recurrence rate of around 25%; • antiviral treatment against the HIV virus (a retrovirus) with three retroviral drugs is required once the initial pneumonic illness has been treated.
Complications Possible complications of this condition include: • Type I (hypoxic) respiratory failure. Most hospitals now have a policy that they will ventilate patients for their first episode of pneumocystis pneumonia as the prognosis is relatively good, but not for second or subsequent episodes, unless there are specific indications to the contrary.
238
CHAPTER 16
SURGICAL PROCEDURES IN RESPIRATORY MEDICINE
Because medico-legal claims may arise from various surgical procedures performed on patients being investigated or treated for lung diseases, these are now briefly described.
BRONCHOSCOPY Bronchoscopy is the internal inspection of the tubes leading into the lung from the larynx – the trachea, the main and the subsegmental bronchi. This procedure can either be done with a fibreoptic instrument, most commonly using a local anaesthetic and sedatives such as midazolam as a day case procedure, or less commonly with a rigid instrument under general anaesthetic.
Indications The most common indications for bronchoscopy are: • coughing of blood (haemoptysis); • abnormalities seen on chest X-ray such as suspected cancer.
Samples At bronchoscopy, samples are taken in some of all or the following ways: • biopsies with forceps for histology from any abnormality seen; • brushings for cytology; • suckings from the bronchial tree; • broncho alveolar lavage can be performed where fluid (150 ml or more) is instilled into the bronchial tree and then sucked back through the bronchoscope. Samples are taken for the following analyses: • culture and sensitivity; • tuberculosis studies; • neoplastic cytology. Not all these procedures are conducted at every bronchoscopy. It depends on the clinical indications and the judgment of the operator. Rigid bronchoscopy
239
Respiratory Disorders under a general anaesthetic is a better way to remove foreign bodies and take larger biopsies. The flexible bronchoscope can also be used through the rigid bronchoscope.
THORACOSCOPY A thoracoscope is a rigid telescope-like fibreoptic instrument whereby through a small hole in the chest wall under general or local anaesthetic the pleural surfaces can be inspected and biopsies taken under direct vision. This is a very useful procedure following unsuccessful pleural biopsies using an Abram’s needle.
MEDIASTINOSCOPY A rigid telescope-like instrument is inserted behind the breast bone for close inspection and biopsy of midline structures, such as lymph nodes or other masses. This is used to investigate causes of mediastinal enlargement on chest X-ray. These causes vary according to the site.
Causes in the superior mediastinum In the superior mediastinum, the causes of an abnormal mass are likely to be: • enlarged lymph nodes; • tuberculosis; • sarcoidosis; • lymphomas; • secondaries from lung cancer; • retrosternal thyroid; • thymic tumours; • aortic aneurysms; • mediastinal abscess.
Causes in the anterior mediastinum In the anterior mediastinum, the causes of a mass are likely to be: • thymic tumours; • lymphomas; • thyroid teratoma.
240
Surgical Procedures in Respiratory Medicine
Causes in the middle mediastinum In the middle mediastinum, the causes of a mass are likely to be: • aortic aneurysm; • lymph nodes, for example, tuberculosis; • sarcoidosis; • lymphoma; • bronchial carcinoma; • lipoma or bronchogenic cyst.
Causes in the posterior mediastinum In the posterior mediastinum, the causes of a mass are likely to be: • aortic aneurysms; • neurogenic tumours; • oesophageal lesions; • paravertebral abscess; • diaphragmatic hernia.
Common presentations of mediastinal masses Mediastinal masses can come to the doctor’s attention in a variety of ways: • routine X-ray in an asymptomatic patient; • through pressure on the trachea; • cough and dyspnoea; • dysphagia due to pressure on the oesophagus; • hoarseness with recurrent laryngeal nerve palsy; • superior vena caval obstruction through local compression; • shortness of breath if there is a phrenic nerve palsy.
Investigations A mediastinal lesion should be investigated by means of: • chest X-ray; • CT chest scan; • bronchoscopy; • mediastinoscopy.
241
Respiratory Disorders
OPEN LUNG BIOPSY A small (10 cm) incision is made in the lateral chest wall under a general anaesthetic through which a segment of lung is removed. The lung is then stapled and sealed and the biopsies taken for histological examination. This is most commonly used in interstitial lung diseases such as a fibrosing or infiltrative or infective process. Disease such as carcinoma, sarcoidosis or cryptogenic fibrosing alveolitis can be diagnosed this way. There is a low mortality involved and as with any surgical procedure informed written consent is necessary.
LOBECTOMY AND PNEUMONECTOMY These are operations at which the thoracic surgeon removes either a part (lobectomy) or the whole (pneumonectomy) of one lung under general anaesthetic. The right lung has three lobes, upper, middle and lower; the left lung two, upper and lower. The mortality of a pneumonectomy is considerably higher than that of a lobectomy. In either case, it is essential for the physician and surgeon to have assessed the patient adequately prior to the operation. This will require obtaining written informed consent, and assessing respiratory function to make sure there is adequate respiratory reserve following operation. Ideally, there should be a definitive diagnosis prior to such a procedure or frozen section available during the procedure, so that if the condition is benign, the lung is not resected. It is also essential to perform scanning procedures to show that that there is no evidence of spread of cancer beyond the lung. If there is, the procedure of resection would be futile.
COMPLICATIONS OF SURGICAL PROCEDURES ON THE LUNG Unfortunately, litigation surrounds surgical procedures if they go wrong or if these procedures have not been undertaken and recorded carefully. The most common problems arising after surgery are: • breathlessness; • infection of the pleural space; • bronchopleural fistula – a communication between the air passageways and the pleural space; • death.
242
Surgical Procedures in Respiratory Medicine
MEDICO-LEGAL ASPECTS Case 39: unnecessary pneumonectomy complicated by breathlessness and chronic chest sepsis A 45 year old woman was known to have sarcoidosis based on a skin lesion on her knee. She had an abnormal chest X-ray which showed an enlarged hilum. The enlarged hilum had been present for some 12 years, but had recently increased in size. CT scanning suggested that this was not a malignancy, but a mediastinal cyst – a benign condition. However, the surgeon was not happy with this and felt that an operation was required to make the diagnosis. At operation, a mass was discovered, and a biopsy was taken. The frozen biopsy done before the procedure went any further showed the benign histological changes of sarcoidosis. No investigations such as needling and aspiration were done to see if this was a cyst. Despite the benign histology, the surgeon went on to remove the patient’s lung containing the bronchogenic cyst. The end result was that the patient remained too breathless ever to return to work. She also developed infection in the pleural space and a bronchopleural fistula, and described her life as being ‘ruined’. Regrettably, this was only one of a series of cases in which this particular surgeon was criticised. The trust concerned has ceased to employ him. The case was felt to be indefensible and settled out of court.
243
USEFUL REFERENCES PNEUMONIA British Thoracic Society, ‘Guidelines on the management of community acquired pneumonia in adults admitted to hospital’ Br J Hosp Med (1993) 49 346–50
CHRONIC OBSTRUCTIVE PULMONARY DISEASE American Thoracic Society, ‘Standards for the diagnosis and care of patients with chronic obstructive pulmonary disease’ American Journal Respiratory Critical Care Med (1995) 152s 77–122 British Thoracic Society, ‘Guidelines for the management of chronic obstructive pulmonary disease’ (1997) 52 Thorax Suppl 5 S1–28 Medical Research Council Working Group, ‘Long-term domiciliary oxygen therapy and chronic hypoxic cor pulmonale complicating chronic bronchitis, emphysema’ Lancet (1981) I 681–86 Burrows, B, ‘The cause and prognosis of different types of chronic airflow limited in a general population sample from Arizona: comparison with the Chicago COPD series’ American Review of Respiratory Disease (1989) 140s 92–94 Costello, R and McNicholas, WT, ‘Five year follow up of respiratory failure in COPD’ Thorax (1993) 48 419p Department of Health, Domiciliary Oxygen Therapy Service: Drug Tariff, Pt X, 1990, London: HMSO Dunne, WF, Nelson, SB and Hubmayr, RD, ‘Oxygen induced hypercarbia in obstructive pulmonary disease’ American Review of Respiratory Disease (1991) 144 526–30 Intermittent Positive Pressure Breathing Trial Group, ‘The intermittent positive pressure breathing therapy for chronic obstructive pulmonary disease’ Annals of Internal Medicine (1983) 99 612–20 Jeffrey, AA, Warren, PM and Fennelly, DC, ‘Acute hypercapnic respiratory failure in patients with chronic obstructive lung disease, risk factors and use of guidelines for management’ Thorax (1992) 47 34–40 Rimmington, LD, Spence, DPS, Nisar, M, Earis, JE, Calverley, PMA and Pearson, MG, ‘Predictors of five year mortality in COPD’ American Review of Respiratory Disease (1993) 147a 323
245
Respiratory Disorders Schwartz, JS, Bencovitz, HZ and Mosa, KM, ‘Air travel hypoxaemia with chronic obstructive pulmonary disease’ Annals of Internal Medicine (1984) 10 473–77 Siafakas, NM, Vermeir, EP, Pride, NB, Paoletti, P, Gibson, GJ, Howard, P et al, ‘Optimal assessment and management of chronic obstructive pulmonary disease’ European Respiratory Journal (1995) 8 1398–420
TUBERCULOSIS The British Thoracic Society has published recommended guidelines on management, which have been regularly updated. See: BMJ (1990) 300 995–99 Thorax (1990) 45 403–08 BMJ (1992) 304 1231–33 Thorax (1994) 49 1193–200 Thorax (1998) 53 536–48
ASTHMA There are British Guidelines produced by the British Thoracic Society which have been published and regularly updated. See: BMJ (1990) 301 651–53 Thorax (1990) 301 797–800 BMJ (1993) 306 776–82 Thorax (1993) 48 S1–24 Thorax (1997) 52 Suppl 1 S1–21
PULMONARY EMBOLISM Thromboembolic Risk Factors Consensus Group, ‘Risk and prophylaxis of venous thromboembolism in hospital patients’ BMJ (1992) 305 567–73 British Thoracic Society, ‘Suspected pulmonary embolism: a practical approach’ Thorax (1997) 52 Suppl 4 S1–24
246
Useful References
PNEUMOTHORAX British Thoracic Society, ‘Guidelines for the management of spontaneous pneumothorax’ BMJ (1993) 307 114–16
LUNG CANCER British Thoracic Society, ‘BTS recommendations to respiratory physicians for organising the care of patients with lung cancer’ Thorax (1998) 53 Suppl 1 S1–8
GENERAL American Thoracic Society, ‘Evaluation of impairment/disability secondary to respiratory disorders’ American Review of Respiratory Disease (1986) 133 1205–09
247
INDEX acid base balance (pH) test,..............................36
anaphylaxis,........................................................69
acquired immune deficiency syndrome see HIV and AIDS
angina pectoris, ..................................................57 angiogram, ........................................................118 ankle swelling, chest pain and, .......................60
Action for Victims of Medical Accidents (AVMA), ....................17
ankylosing spondylitis, ............................220–21 antiallergic drugs, ..............................................73
activated partial thromboplastin time (APTT) test,.......................................123
antibiotic treatment chronic obstructive airways disease, ........................105, 106 pneumonia, ...........................................91–94
adenocarcioma, ........................................133, 134 adenoma, bronchial,........................................140 adult respiratory distress syndrome (ARDS),...........................229, 230 causes,.........................................................229 clinical features, ..................................229–30 treatment,...................................................230
anticholinergic drugs, .......................................73
age, diagnosis of lung disease and, .......................................................42, 143
appetite loss, chest pain and, ...........................59
anticoagulant treatment, ..................122, 123–24 antitrypsin,..........................................103, 106–07 apnoea see sleep apnoea APTT (activated partial thromboplastin time) test,.......................123
AIDS see HIV and AIDS
arsenic related lung cancer,............................203
air
artificial ventilation, ..........................................94 chronic obstructive airways disease, ........................106, 109 decision not to ventilate, ...........99–100, 109
composition,................................................25 embolism, ..................................................176 pneumothorax,............................................22 see, also, respiration
asbestos related diseases, .......................197–203 asbestosis, ....................................30, 197–201 lung cancer, ...............................................203 malignant mesothelioma, .................201–03
allergies allergic bronchopulmonary aspergillosis (ABPA), ...........66, 213–15 allergy tests,...........................................32, 66 chest symptoms history taking, ...................................................44 desensitisation,............................................69 extrinsic allergic alveolitis,.....................186, 189–91, 218 see, also, asthma
aspergillus fungus, ..........................................213 allergic bronchopulmonary aspergillosis (ABPA), ...........66, 213–15 aspergilloma,.......................................216–18 asthma, .......................................................213 extrinsic allergic alveolitis,......................218
alpha 1 antitrypsin, ...........................103, 106–07
aspirin, .................................................................70
altitude, as cause of breathlessness, ............................................53
asthma,.............................................................1, 61 aspergillus reaction, .................................213 assessment of severity of attacks, ............................................62–63 causative factors, ........................................68 clinical features, ...................................61–62, 207–08 clinics, .............................................................5 eosinophilia, ........................................218–19 investigations, .............................................83 allergy tests, ...................................32, 66 blood oxygen tests, .............................68 respiratory function tests,..........................................27, 29, 31, 36, 63–66
alveolar cell carcinoma, ............................139–40 alveoli,..................................................................23 alveolitis see cryptogenic fibrosing alveolitis; extrinsic allergic alveolitis American Review of Respiratory Disease (journal), ....................9 American Thoracic Society, ...............................9 anaemia as cause of breathlessness, ........................53 investigations, .............................................30
249
Respiratory Disorders sputum examination, .........................67 X-rays, ...................................................67 occupational see occupational diseases, asthma prevention desensitisation, ....................................69 drug avoidance,...................................70 occupational asthma, ...........70–71, 208 symptoms, ...................................................62 treatment and management, ........8, 72, 208 acute severe asthma,.....................74–76 case studies, ...................................78–81 chronic asthma, ...................................74 drugs, ........................................73, 81–83 in general practice,........................75–76 guidelines and protocols, ............15, 72 legal issues, ..............................61, 76–84
sarcoidosis, .........................................159 see, also, haemoptysis (coughing of blood) blue bloaters,.....................................................102 body plethysmography, overall lung size – total lung capacity (TLC) test, ........................31 bone sarcoidosis, ................................................158 tuberculosis, ..............................................144 see, also, spine Bornholm disease, .............................................58 brain, secondary tumours in,.................128, 133 breathing see respiration British Academy of Experts, ............................17
bacteriology testing, ............................................7
British Thoracic Society (BTS), .............9, 14, 72, 85, 113, 147, 179
bagassosis,.................................................190, 208
bronchial adenoma,.........................................140
bee sting allergy, ................................................69
bronchial tree, anatomy,.............................23–24
berylliosis, .........................................................211
bronchitis, chronic see chronic obstructive airways disease
beta agonist bronchodilators, ..........................73
bronchoconstriction, histamine challenge test,..............................................31
beta blockers, ..............................................70, 105 biopsy open lung biopsy,.....................................242 pleural effusion,.................................174–75, 182–83 pulmonary fibrosis,..................................187 sarcoidosis, ..........................................160–61
bronchodilators,..........................................73, 94, 104, 106 bronchopleural fistula,....................................181 bronchopneumonia, ..........................................85 bronchoscopy, ....................................6, 7, 239–40
bird fancier’s lung,...................................190, 208
byssinosis, .........................................................205
blood clots see pulmonary embolism haemopneumothorax, .............................181 oxygenation,................................................25 in pleural effusion (haemothorax), ............................22, 171 sickle cell disease,...............................227–28 testing, ............................................................7 asthma investigation, ...................66, 68 blood gases,..............................33–36, 37 chronic obstructive airways disease investigation, .....................................103 gas transfer diffusing capacity (DLCO) test, ......................................30 in lung cancer, ...................................129 pulmonary fibrosis, ..........187, 190, 191
cadmium poisoning, .................................210–11 Caplan’s syndrome, ..........................196–97, 219 carbon dioxide, respiration and, .....................25 carbon monoxide gas transfer – diffusing capacity (DLCO) test, .........................30 poisoning, ..................................................234 clinical features,...........................234–35 investigations,....................................235 treatment, ...........................................235 carcinoid tumours, ..................................127, 140 carcinoma see lung cancer cardiopulmonary bypass,...............................122 carina,...................................................................23
250
Index oxygen therapy,...........................105–06 problems,......................................108–09
case studies asthma treatment and management, .................................78–81 pleural effusion,..................................182–83 pneumonia, .........................................95–100 pneumothorax, ...................................183–84 pulmonary embolism, .............................126 sarcoidosis, ..........................................164–68 surgery, ......................................................243 tuberculosis, ........................................148–54
chylothorax, ................................................22, 171 clinics see out-patient clinics coalminers’ pneumoconiosis, ..................194–96 assessment of damage and disability, ......................................195–96 clinical features,..........................194–95, 196 treatment,...................................................195 communication, failure of,...............15, 100, 109
cavitating pneumonia, ......................................85 causes, ....................................................89–90
community-acquired pneumonia,..................................................85 causes, ....................................................86–87
central apnoea, ...........................................232–33 Certificate of Completion of Specialist Medicine (CCST), ...................................................2, 4–5
complaints procedure,..............................................10–11 types of problems,................................15–16
chemotherapy, .....................................9, 134, 135
computerised tomography (CT), ......................7 chronic obstructive airways disease,................................................104 haemoptysis, ...............................................49 lung cancer, .......................................131, 133 pleural effusion,........................................172 pulmonary embolism, .............................118 pulmonary fibrosis,..................................187
chest symptoms clinical approach,........................................39 general points,.......................................42–43 history taking,...........................39–40, 41–44 investigations,................................39–41, 46, 48–49, 58–59 physical signs of chest disease,............................................54–56 presenting symptoms, .........................44–45 chest pain,.......................................57–60 cough,..............................................45–46 haemoptysis (coughing of blood), ........................................46–49 shortness of breath,.......................49–57
connective tissue disorders ankylosing spondylitis, .....................220–21 cystic fibrosis,......................................223–25 Goodpasture’s syndrome, ................225–26 kyphoscoliosis,....................................221–22 polyarteritis nodosum, ............................220 rheumatoid arthritis,................................219 systemic lupus erythematosus, ..................................220 systemic sclerosis,.....................................220 Wegener’s granulomatosis, ............152, 226
chest wall, anatomy,..........................................24 chromate related lung cancer, .......................203 chronic obstructive airways disease, .......................................................101 ability to work and,............................110–11 causes,.........................................................101 clinical features, ........................................102 clinics, .............................................................5 co-existing diseases,...................109–10, 197 complications, .....................................102–03 investigations, ......................................27, 29, 103–04 life expectancy with, ............................1, 111 symptoms, .................................................101 treatment and management alpha 1 antitrypsin treatment, .....................................106–07 hospital admission,...........................105 legal issues, ..................................107–11 out-patient,...................................104–05
contact tracing, tuberculosis, ................5, 7, 146, 147, 153 contraceptive pill, pulmonary embolism and,...........................................125 cor pulmonale, .........................................102, 159 corticosteroids see steroids costochondritis, ..................................................58 cotton dust byssinosis, ....................................205 cough causes of,................................................45–46 investigation,...............................................46 see, also, haemoptysis (coughing of blood) Coxsackie B infection, .......................................58
251
Respiratory Disorders cryptogenic fibrosing alveolitis, ......................30 clinical features, ........................................187 complications, ...........................................188 investigation,.............................................187 legal issues,................................................189 prognosis, ..................................................188 treatment,...................................................188
emphysema,...............................................30, 101, 106–07, 197 surgical, ......................................................181 see, also, chronic obstructive airways disease employment chronic obstructive airways disease and,..................................110–11 diagnosis of lung disease and,..........................................42 see, also, occupational diseases
CT scanning see computerised tomography (CT) cylindroma,.......................................................140 cystic fibrosis, ...................................................223 clinical features, ........................................224 investigations, ...........................................224 prognosis, ..................................................225 treatment,.............................................224–25
empyema, ...........................................22, 171, 176 eosinophilia, ...............................................218–19 erythema nodosum, ........................144, 157, 158 European Respiratory Journal,..........................9
cytology, ........................................................7, 136
European Respiratory Society, ..........................9 exercise test for asthma,....................................65
death of patients asthma, ...................................................76–81 complaints about, .................................15, 16 pneumonia, ...........................................95–98 tuberculosis treatment,............................154
Expert Witness Institute, ............................12, 17 expert witnesses and reports, ....................11–13 availability of experts, ...............................17 general medicine, .......................................11 instructions to experts, ..................17, 18–19 obligation of experts, ...........................19–20 retired physicians, ......................................13 sources of experts, ......................................17
deep vein thrombosis,............................113, 114, 120, 125 desensitisation, asthma prevention,...................................................69
extrinsic allergic alveolitis, .............................189 causes, ........................................186, 190, 218 clinical features, ........................................189 compensation,...........................................191 investigations, .....................................190–91 prognosis, ..................................................191 treatment,...................................................191
dextrocardia,.......................................................22 diabetes blood gases test,..........................................36 as cause of breathlessness, ........................53 diabetes insipidus,....................................159 diagnosis failure of, ......................................................16 lung cancer,..................................135–39 pneumonia, ....................................95–99 pulmonary embolism,................125–26 sarcoidosis,...........................155, 165–68 tuberculosis,.................................148–50
eyes, sarcoidosis,..............................................158 family history, chest symptoms, ...............43–44 farmer’s lung, ..........................................190, 191, 208–09
diaphragm,..........................................................24
fever, chest pain and, ........................................59
diffusing capacity (DLCO) test,.......................30
fibrosis see pulmonary fibrosis
digoxin treatment, ...........................................106
financial settlements,...................................10–11
discharge from hospital, complaints about, .......................................15
fire, toxic smoke inhalation,.....................233–34 flax dust byssinosis,.........................................205
disease, failure to accept,..................................16 diuretics,....................................................105, 106
gas transfer – diffusing capacity (DLCO) test,.................................30
drug addiction, diagnosis of lung disease and, ........................................43
General Medical Council,...................................3
252
Index general medicine medical reports, ..........................................11 reference books/journals,...................13, 14 respiratory medicine and,.......................2–3 training in, .............................................2, 3–4
human immunodeficiency virus see HIV and AIDS humidifier fever, ..............................................190 hydropneumothorax,......................................176 hypercalcaemia and hypercalciuria, ..........................................159
general practitioners, asthma case studies,...........................................78–80
hypoproteinaemia, ..................................171, 172
genital tuberculosis, ........................................144 glandular tuberculosis, ...................................144 Goodpasture’s syndrome,........................225–26
industrial diseases see occupational diseases
haemoglobin,................................................25, 30
INR (international normalised ratio) test, ...........................................124, 126
haemopneumothorax,.....................................181
intercostal artery, ...............................................24
haemoptysis (coughing of blood) causes,.....................................................47, 48 investigation,.........................................48–49
intercostal muscles,............................................24 intercostal nerves,.................................16, 24, 58, 182–83
haemothorax,..............................................22, 171 hard metal disease,..........................................211
intercostal tubes, .....................................106, 167, 180–81 misplacement of, ..........................16, 183–84
health visitors, ......................................................7
intercostal vein, ..................................................24
heart cardiac causes of breathlessness, .....................................52 chest pain, ..............................................57, 58 chronic obstructive airways disease and,................................102, 110 misplacement of intercostal tube and puncture of,..................................183–84 position of, ...................................................22 respiration and,...........................................25 sarcoidosis, ................................................159
international normalised ratio (INR) test, .................................124, 126
hamartoma,...............................................127, 140
intestinal tuberculosis, ....................................144 intravenous fluids, pneumonia, ......................94 joint tuberculosis,.............................................144 journals, ...........................................................9, 14 kaolin silicosis,..................................................204 kidneys nephrocalcinosis, ......................................159 renal causes of breathlessness, .................53 renal function tests, ....................................36 tuberculosis, ..............................................144 Wegener’s granulomatosis, ....................152
Heerfordt’s syndrome,....................................158 helium dilution, overall lung size – total lung capacity (TLC) test, .............................................31 histamine challenge test, ..................................31
Kveim test, .................................................16, 160, 165–66
histology see biopsy history taking chest symptoms,.......................39–40, 41–44 general points,.......................................42–43
kyphoscoliosis,...........................................221–22 larynx, ..................................................................23
HIV and AIDS, ...............................................5, 43 lung involvement, ..............................235–38 pneumocystis pneumonia, .......236, 237–38
Law Society, Directory of Experts, ...........12, 17 legal action, ...........................................................1 expert witnesses see expert witnesses and reports financial settlements, ...........................10–11
honeycomb lung, .............................................190 hospital-acquired pneumonia, ........................87
253
Respiratory Disorders legal issues in asthma treatment and management,...............................................61 death/near death of patient, ............................................76–81 occupational asthma, .............61, 83–84 steroid treatment,..........................81–83 common types of problems for which patients seek legal advice, ...................................15–16 failure to perform or act on respiratory function tests,............36–37 lung cancer, .........................................135–39 misplacement of intercostal tubes, .......................................16, 183–84 pleural biopsy, ....................................182–83 pneumonia, .........................................95–100 pulmonary embolism, .......................124–26 pulmonary fibrosis,..........................189, 191 sarcoidosis, ..................................155, 163–68 surgery, ......................................................243 tuberculosis, ........................................148–54 see, also, legal action
cell types, ...................................................133 chronic obstructive airways disease and,..........................103, 109–10 clinical presentation,........................128, 140 clinics, .....................................................5, 135 diagnosis and staging,.............................129 hamartoma, .......................................127, 140 investigations, ..........................................109, 129–33, 140 legal issues,..........................................135–39 mesothelioma,.....................................201–03 natural history,..........................................128 nerves and, ..................................................24 prognosis, ........................................1, 134–35 referral to other specialists,.....................8–9 treatment and management, ........8, 134–35 lung function tests see respiratory function testing lupus, systemic lupus erythematosus,..........................................220 lupus pernio,.....................................................158 lupus vulgaris,..................................................144
life expectancy, with chronic obstructive airways disease,...............1, 111
lymph fluid, chylothorax, ........................22, 171 lymph nodes glandular tuberculosis,............................144 lung cancer and, .......................................128 sarcoidosis, ................................................158
lingula, .................................................................22 litigation see legal action liver cirrhosis,.....................................................106 misplacement of intercostal tube and puncture of,.......................184 sarcoidosis, ................................................158 secondary tumours in,.....................128, 132 tuberculosis treatment side effects, .........................................146, 154
Macmillan nurses,................................................7 malignant mesothelioma,.........................201–03 maltworker’s lung, .................................190, 191, 208, 218
lobar pneumonia,...............................................85
management posts, respiratory medicine and,..............................................10
lobectomy,.........................................................242
mediastinoscopy,.......................................240–41
lung(s) anatomy, ................................................21–25 bronchial tree,................................23–24 chest wall,.............................................24 mediastinum,.......................................22 nerve supply, .................................24–25 pleura, ...................................................22 respiration, ...........................................25
mediastinum, anatomy,....................................22 medical complaints see complaints Medical Defence Union, ...................................10 medical negligence, common types of problems for which patients seek advice on,.......................15–16 Medical Protection Society,..............................10
lung cancer,.................................................127–28 alveolar cell carcinoma,.....................139–40 asbestos related,........................................203 bronchial adenoma, .................................140 case studies,........................................135–36, 138–39
medical records, alteration,..............................80 medical reports general medicine, .......................................11 respiratory medicine,...........................11–13 standard reference books for,.............13–15
254
Index medico-legal issues see legal issues
miliary tuberculosis,........................................144
hard metal disease,...................................211 nitrogen oxides inhalation,...............209–10 pneumoconioses see pneumoconiosis pulmonary fibrosis,.........................190, 191, 203–04, 208–09 silicosis, ................................................203–04
mucolytic drug treatment, .............................104
oncology departments, ...................................8–9
mushroom worker’s lung, .....................190, 208
opiates,...............................................................105
myasthenia gravis,.............................................37
out-patient clinics, .........................................5, 41 chronic obstructive airways disease investigations,..............................103–04 treatment, .....................................104–05 cystic fibrosis treatment, ...................224–25 follow-up, ..........................................7–8, 147 investigational support, ..............................7 referral to other specialists,.....................8–9 staffing,.......................................................6–7 tuberculosis follow-up,............................147
meningitis, ..........................................144, 149–50 mesothelioma, ............................................201–03 methacholine challenge test,............................31
negligence see medical negligence nephrocalcinosis, .............................................159 nerves lung nerve supply, ...............................24–25 sarcoidosis, ................................................158 nitrogen oxides inhalation, ......................209–10 non-admission to hospital, complaints about, .......................................16
overall lung size – total lung capacity (TLC) test, ....................................31
non-steroidal anti-inflammatories,.............................70, 81
oximetry, .........................................36, 37, 68, 116 oxygen oxygen saturation tests,................34, 36, 37, 68, 116 oxygen therapy chronic obstructive airways disease, ..........................105–06 pneumonia, ..........................................94 respiration and,...........................................25
nuclear medicine,.................................................7 nurses palliative care, ...............................................7 respiratory specialists, .................................7 oat cell carcinoma, ...........................................133 obstructive airways disease see chronic obstructive airways disease
paediatric medicine, ............................................5 pain chest pain,..............................................57–60 associated features,.......................59–60 causes, .............................................57–58 investigation, .................................58–59
obstructive sleep apnoea, ...............................232 occupational diseases,.........................................8 asbestos related diseases,................197–203 asbestosis,.............................30, 197–201 lung cancer, ........................................203 malignant mesothelioma, .............................201–03 asthma, .......................................................205 clinical features,...........................207–08 differential diagnosis,.................206–07 investigation, .................................66, 83 legal issues, ..............................61, 83–84 prevention,.............................70–71, 208 sarcoidosis and, .................................164 treatment, ...........................................208 berylliosis,..................................................211 byssinosis,..................................................205 cadmium poisoning,..........................210–11
palliative care, ................................8, 108–09, 134 nurses,.............................................................7 parietal pleura, ...................................................22 peak expiratory flow rate (PEFR), .......................................26–27, 63–66 pelican ink injection, .................................16, 166 pericarditis,.........................................58, 171, 172 pH test,.................................................................36 pharynx, ..............................................................23 phrenic nerves, ...................................................24
255
Respiratory Disorders physiotherapy chronic obstructive airways disease,................................................106 pneumonia,..................................................94
HIV/AIDS and, ..........................236, 237–38 legal issues,..........................................95–100 management, ..................................85, 90–94 prognosis, ..............................................94–95 tuberculosis and, ................................88, 144
pigeon fancier’s lung,......................................190
pneumothorax,..........................................22, 103, 167, 177 case studies,.........................................183–84 causes,.........................................................178 clinical features, ..................................178–79 complications, ...........................................181 investigations, ...........................................179 prevention,.................................................182 prognosis, ..................................................182 tension pneumothorax, ...................179, 181 treatment and management, ............179–81
plethysmography, overall lung size – total lung capacity (TLC) test, ....................................31 pleura anatomy, ......................................................22 mesothelioma,.....................................201–03 pleural effusion, ........................................22, 128, 144, 169–70 case study, ...........................................182–83 causes, ..................................................170–71 clinical features, ........................................172 complications, ...........................................176 investigations,..............................30, 31, 172, 174–75 treatment and management, ............172–74 types,...........................................................171
polyarteritis nodosum, ...................................220 polycythaemia,...........................................30, 103 pregnancy anticogulant treatment and, ...................123 steroid treatment and, ...............................83
pleural shock, ...................................................176
prescribing, reference books, ...........................13
pleurisy, chest pain,.....................................57–58
pulmonary angiogram,...................................118
pneumoconiosis, ........................................193–94 asbestosis, ....................................30, 197–201 assessment of damage and disability, ..............................195–96 byssinosis,..................................................205 clinical features,.........................194–95, 196, 198–99, 204, 205 coalminers’ pneumoconiosis, .........................194–96 co-existing conditions,.............................197 diagnosis,...........................................194, 199 differential diagnosis,..............197, 199–201 rheumatoid pneumoconiosis, .................196–97, 219 silicosis, ................................................203–04 treatment,...................................................195
pulmonary artery,..............................................25 see, also, pulmonary embolism pulmonary embolism,.....................................113 case study, .................................................126 cause, ..........................................................113 clinical features, ..................................113–14 diagnosis, ...................................................114 investigations, .....................................115–21 legal issues,..........................................124–26 predisposing factors,................................114 prevention, ........................................122, 124 treatment,.....................................122–24, 126 pulmonary eosinophilia, ..........................218–19 pulmonary fibrosis, .........................................185 causes, ................................................186, 190 clinical features, ................................187, 189 complications, ...........................................188 cryptogenic fibrosing alveolitis, ................................30, 187–88 extrinsic allergic alveolitis,.....................186, 189–91, 218 investigations, ......................................30, 31, 187, 190–91 legal issues,........................................189, 191 occupational, ....................................190, 191, 203–04, 208–09 pneumoconioses,................................193–97
pneumocystis pneumonia,...............236, 237–38 pneumonectomy,.............................................242 pneumonia, .........................................................85 assessment, ..................................................88 case studies,.........................................95–100 causes, .............................................85, 86–87, 89–90 chronic obstructive airways disease and,........................................102 clinical features, ..........................................87
256
Index prognosis, ..........................................188, 191 treatment and management, .........................8, 188, 191
cystic fibrosis,............................................224 gas transfer – diffusing capacity (DLCO) test, .........................30 histamine challenge test, ...........................31 legal issues,............................................36–37 in lung cancer,...........................................129 overall lung size – total lung capacity (TLC) test,....................31 oxygen saturation,...............................34, 36, 37, 68, 116 peak expiratory flow rate (PEFR),................................26–27, 63–66 in pulmonary fibrosis, .....................187, 190 sarcoidosis, ................................................159 spirometry, .....................................27–30, 37, 64, 103, 129
pulmonary oedema,................................176, 181 pulmonary sarcoidosis, ............................157–58 pulmonary veins,...............................................25 pus, empyema,...................................22, 171, 176 racial origin diagnosis of lung disease and,............................42–43, 143 sickle cell disease, .....................................227 radioisotope scans, ..........................116, 117, 160 radiology see X-rays
respiratory medicine complaints against see complaints expert reports see expert witnesses and reports ‘firm’ concept, ...............................................3 litigation see legal action management posts and, ............................10 nature of,..................................................1–20 non-specialist practitioners and, ..........................2–3, 5 out-patients see out-patient clinics professional societies and standards, .......................................9 specialisms within,.....................................12 team concept in,............................................3 training in, .............................................2, 3–4 continuing medical education, .........................................9–10 workload of respiratory physician, ...................................2–3, 5–6
radon gas related lung cancer, ......................203 reference books general medicine, .................................13, 14 guidelines and protocols,....................14–15 list,.........................................................245–47 prescribing,..................................................13 respiratory medicine,...........................14–15 respiration assisted ventilation see artificial ventilation chest wall and, ............................................24 nerve damage and,.....................................25 purpose of,...................................................25 shortness of breath (breathlessness) associated features,.................51, 56–57 causes, .......................................50, 52–53 chest symptom, .............................49–57 investigation, .................................53, 56 respiratory distress syndrome see adult respiratory distress syndrome (ARDS)
respiratory symptoms see chest symptoms
respiratory failure Type 1,................................................102, 188 Type 2, ........................................................102
reticulo endothelial sarcoidosis,....................158 retired physicians, expert reports from,................................................13
respiratory function testing, ..................7, 25–26 allergy tests,.................................................32 in asbestosis,..............................................199 in asthma, .......................................27, 29, 31, 36, 63–66, 206–07 blood gases, ..........................................33–36, 37, 68, 116 in chronic obstructive airways disease, ....................27, 29, 103
retrosternal pain,................................................58 rheumatoid arthritis, .......................................219 rheumatoid pneumoconiosis,..........196–97, 219 ribs,.......................................................................24 Royal College of Physicians,..............................2 continuing medical education (CME),...............................................9–10
257
Respiratory Disorders Fellowship (FRCP), ....................................12 Membership (MRCP),..............................3–4
socio-economic groups, respiratory disorders and, ..................1, 143 solicitors, instructions to experts, ........................................17, 18–19
sarcoidosis,........................................................155 case studies,.........................................164–68 clinical features, ..................................156–59 differential diagnosis, ........................160–61 investigation and diagnosis,......................................159–61 legal issues,..................................155, 163–68 prognosis, ..................................................163 treatment and management, .............8, 155, 162–63
spinal cord injuries, respiration and,.....................................24–25 spine ankylosing spondylitis, .....................220–21 kyphoscoliosis,....................................221–22 vertebral collapse,.......................................58 spirometry,.............................................27–30, 37, 64, 103, 129 spleen misplacement of intercostal tube and puncture of,........................................184 sarcoidosis, ................................................158
scoliosis,.......................................................221–22 sexual orientation, diagnosis of lung disease and, ...................................43 shingles,...............................................................58 shock lung see adult respiratory distress syndrome (ARDS)
sputum examination asthma, .........................................................67 chronic obstructive airways disease,................................................104 lung cancer, .........................................135–36 sarcoidosis, ................................................160
sickle cell disease, ............................................227 clinical features, ........................................227 investigations, ...........................................227 treatment,...................................................228
squamous cell carcinoma, ......................133, 134
silicosis,........................................................203–04
steroids asthma treatment, ..........................73, 81–83 chronic obstructive airways disease treatment, ...........................................104 pulmonary fibrosis,..................................188 sarcoidosis treatment,........................162–63 tuberculosis treatment,......................146–47
skin allergy tests,...........................................32, 66 Kveim test,..........................................16, 160, 165–66 pulmonary fibrosis test, ..........................190 sarcoidosis, ........................................158, 160 tuberculin test, ..........................................145
streptokinase treatment, .................................122
sleep apnoea, ....................................................231 causes,.........................................................232 central,..................................................232–33 clinical features, ........................................232 investigation,.............................................233 obstructive, ................................................232 treatment,...................................................233
stridor, shortness of breath (breathlessness) and,..................................57 surgery bronchoscopy,.............................6, 7, 239–40 cardiopulmonary bypass, .......................122 case studies,...............................................243 complications, ...........................................242 intercostal tubes,..............................106, 167, 180–81 misplacement of,...................16, 183–84 legal issues,................................................243 lobectomy, .................................................242 lung cancer, ...........................................8, 134 mediastinoscopy,................................240–41 open lung biopsy,.....................................242 pleural fluid drainage and biopsy, ....................174–75, 182–83
small cell carcinoma, .......................................133 smoke inhalation, ......................................233–34 smoking chronic obstructive airways disease and,........................101, 104, 110 diagnosis of lung disease and,..........................................43 lung cancer and, .......................................128 social history, chest symptoms,.......................44
258
Index pneumonectomy,......................................242 pneumothorax, ...........................180–81, 182 pulmonary embolism, .....................122, 123 surgical emphysema,...............................181 thoracoscopy, ............................................240 unnecessary,................................150–51, 243
veins, deep vein thrombosis,.......................................113, 114, 120, 125 venography,................................................120–21 ventilation see artificial ventilation
sweat tests, ........................................................224
vertebral collapse,..............................................58
systemic lupus erythematosus, .....................220
visceral pleura, ...................................................22
systemic sclerosis,............................................220
voice box,.............................................................23
talc silicosis, ......................................................204
wasp sting allergy,.............................................69
tension pneumothorax,...........................179, 181
Wegener’s granulomatosis, ...................152, 226
textbooks see reference books
weight loss, chest pain and, .............................59
thermal injury,............................................233–34 thoracoscopy,....................................................240
wheezing, shortness of breath (breathlessness) and,............................56–57
Thorax (journal), ..................................................9
windpipe, ............................................................23
total lung capacity (TLC) test, .........................31
working hours for junior doctors,.....................3
toxic smoke inhalation,.............................233–34 trachea,.................................................................23
X-rays, ....................................................................7 diagram of postero-anterior chest X-ray,...........................................41 investigation asbestosis, ..................................................198 asthma, .........................................................67 chest symptoms, ..................................40, 41, 48–49 chronic obstructive airways disease,........................................103, 104 cystic fibrosis,............................................224 lung cancer, ......................................109, 130, 137–38 pleural effusion,........................................172 pneumoconioses,................................195–96 pneumothorax, .........................................179 pulmonary embolism, .....................115, 116 pulmonary fibrosis,..................................187 sarcoidosis, ................................................159 tuberculosis, ..............................................147 normal chest X-ray, ....................................40 haemoptysis with, ......................................49 radiotherapy, lung cancer,..................8, 134
tuberculoma,.....................................................144 tuberculosis,......................................................141 case studies,.........................................148–54 clinics, .............................................................5 contact tracing,.................................5, 7, 146, 147, 153 diagnosis, ...................................................145 differential diagnosis, ........................160–61 investigation,.....................................145, 147 legal issues,..........................................148–54 pneumonia and,..................................88, 144 population at risk, ........................42–43, 143 symptoms and signs,.........................143–44 treatment and management, ..........................8, 145–47, 153–54 side effects,...........................146, 153–54 types of,................................................142–43 tumours see lung cancer ultrasound,..........................................120–21, 172
259
