Oxford Textbook of Palliative Care for

Children 3rd Edition Richard Hain

Visit to download the full and correct content document:
https://ebookmass.com/product/oxford-textbook-of-palliative-care-for-children-3rd-edit
ion-richard-hain/
Oxford Textbook of

Palliative Care
for Children
Oxford Textbook of

Palliative Care
for Children
THIRD EDITION

EDITED BY

Richard Hain
Clinical Lead, Welsh Paediatric Palliative Medicine Managed Clinical Network; Visiting Professor,
University of Glamorgan; and Honorary Senior Lecturer, Bangor University, Wales, UK

Ann Goldman
Paediatrician and Palliative Care Specialist, London, UK

WITH
Adam Rapoport
Medical Director, Paediatric Advanced Care Team (PACT), The Hospital for Sick Children (SickKids);
University of Toronto; and Emily’s House Children’s Hospice, Toronto, ON, Canada

Michelle Meiring
Paedspal and the Department of Paediatrics and Child Health, University of Cape Town,
South Africa

1
3
Great Clarendon Street, Oxford, OX2 6DP,
United Kingdom
Oxford University Press is a department of the University of Oxford.
It furthers the University’s objective of excellence in research, scholarship,
and education by publishing worldwide. Oxford is a registered trade mark of
Oxford University Press in the UK and in certain other countries
© Oxford University Press 2021
Appendix 1 © 2019 The Association of Paediatric Palliative Medicine (APPM)
The moral rights of the authors have been asserted
First Edition published in 2005
Second Edition published in 2012
Third Edition published in 2021
Impression: 1
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, without the
prior permission in writing of Oxford University Press, or as expressly permitted
by law, by licence or under terms agreed with the appropriate reprographics
rights organization. Enquiries concerning reproduction outside the scope of the
above should be sent to the Rights Department, Oxford University Press, at the
address above
You must not circulate this work in any other form
and you must impose this same condition on any acquirer
Published in the United States of America by Oxford University Press
198 Madison Avenue, New York, NY 10016, United States of America
British Library Cataloguing in Publication Data
Data available
Library of Congress Control Number: 2020944532
ISBN 978–​0–​19–​882131–​1
DOI: 10.1093/​med/​9780198821311.001.0001
Printed in Great Britain by
Bell & Bain Ltd., Glasgow
Oxford University Press makes no representation, express or implied, that the
drug dosages in this book are correct. Readers must therefore always check
the product information and clinical procedures with the most up-​to-​date
published product information and data sheets provided by the manufacturers
and the most recent codes of conduct and safety regulations. The authors and
the publishers do not accept responsibility or legal liability for any errors in the
text or for the misuse or misapplication of material in this work. Except where
otherwise stated, drug dosages and recommendations are for the non-​pregnant
adult who is not breast-​feeding
Links to third party websites are provided by Oxford in good faith and
for information only. Oxford disclaims any responsibility for the materials
contained in any third party website referenced in this work.
Preface
Bringing together this third edition of the Oxford Textbook of
Palliative Care in Children has, as always, been both a delight
and a challenge. It has been a delight to work with authors from
every continent, drawn from almost every profession and discip-
line (including parents) that contribute to the care of children with
life limiting conditions. To draw on the experience and knowledge
of such a diverse range of contributors is essential in a book that
claims, as this one does, to address the psychosocial and spiritual
needs of children as well as the physical. It is impossible for any one
profession on its own, let alone any one discipline within a profes-
sion, to offer the range of support that families need. It has been a
privilege to work alongside so many who have chosen to apply their
own professional and personal ‘life’ skills to accompanying families
through the worst time of their lives.
It has also, of course, also been an enormous challenge to pro-
duce a book that claims (again as this one does) both to be globally
relevant, and at the same time to be informed by the most reliable
and up-to-date knowledge and evidence available. The underlying
philosophies of palliative care do not change with geography. The
aim of palliative care is to ensure as far as possible the comfort and
wellbeing of a child and family even though the child has a condi-
tion that cannot be cured and will ultimately result in a premature
end to her life. Its aim is neither to hasten nor to delay death, but to
improve the quality of the child and family’s lived existence while
dying occurs. The principles of child- and family-centred care that
is flexible and individualised, of teamwork, and of evidence-based
and reflective practice are similarly universal.
The ways in which those general principles have to be worked out
in practice, however, certainly do depend on geography. Our edi-
torial team now includes colleagues from Europe, North America,
and Africa and, in this edition, we have tried to acknowledge the
very different cultural, political and especially resource contexts in
which paediatric palliative care must be delivered in different coun-
tries. To that end, we have recruited authors from all over the world
and have tried to preserve authors’ original voices where we could.
While we have encouraged authors to use terms that will be under-
stood in the same way in every country, where possible we have
also allowed local variations to stand. So, for example, the North
American phrase ‘critical care unit’ is used alongside the more usual
‘neonatal’ or ‘paediatric intensive care unit’ to describe critical care
environments.
One term in particular deserves special mention because it is cen-
tral to the theme of this book. The two phrases ‘paediatric palliative
care (PPC)’ and ‘children’s palliative care (CPC)’ mean exactly the
same thing, but we discovered that some contributors nevertheless
had a strong preference for one over the other. The reasons were not
always clear; some felt that ‘children’s palliative care’ avoids an over-
emphasis on physical interventions using medication prescribed by
doctors, while others felt that ‘paediatric palliative care’ is a useful
reminder of the distinct nature of children, and of palliative care in
children. For the purposes of this book, however, the editors con-
sider the meanings of the two phrases to be identical. There may
well be specific situations in which individual readers prefer one
term over the other, but as descriptors of the work that this book is
designed to support, they are interchangeable and the editors have
left them as the authors wrote them.
The editors would like to record our enormous thanks and grati-
tude to Caroline Smith at Oxford University Press, who has pa-
tiently guided us from our first meeting in a garden in Wales one
summer to the launch of the third edition of the Oxford Textbook
of Palliative Care in Children. Our thanks also to previous editor
Stephen Liben and to all the authors of earlier editions of the text-
book, on whose foundations this latest edition is built. We hope the
book will continue to be of value to all those caring for children and
young people with life-threatening illnesses and their families; clin-
icians, volunteers, and families. We hope it will contribute to their
skill and confidence and so help them ensure the children in their
care can enjoy life even as they approach death, and can die peace-
fully and with dignity wherever they are.
Richard Hain
Ann Goldman
Adam Rapoport
Michelle Meiring
Contents
Detailed contents ix
Contributors xvii
Abbreviations xix
SECTION I
Foundations of care
1. History and epidemiology 3
Lorna Fraser, Stephen Connor, and Joan Marston
2. Communication 17
Jennifer Mack and Bryan Sisk
3. ‘Children are not small adults’—​the distinctiveness
of ethics in children 25
Robert Macauley and Richard Hain
4. Decision-​making with children, young people,
and parents 36
Myra Bluebond-​Langner and Richard Langner
5. Culture, spirituality, religion, and ritual 44
Erica Brown, Mary Ann Muckaden, and Nokuzola Mndende
SECTION II
Child and family care
6. Assessment of the child and family 59
Nancy Contro and Jane Zimmerman
7. Children’s views of death 66
Myra Bluebond-​Langner and Ignasi Clemente
8. The psychological impact of life-​limiting
conditions on the child 75
Jan Aldridge and Barbara M. Sourkes
9. Adolescents and young adults 87
Chana Korenblum and Finella Craig
10. Children expressing themselves 95
Amy Volans and Emma Brown
11. Education and school 103
Sue Boucher
12. Impact on the family 111
Veronica Dussel, Barbara Jones with
Kevin O’Brien, and Melissa Williams-Platt
13. Bereavement 126
Sara Portnoy and Lori Ives-Baine
SECTION III
Symptom care
14. Overview of symptoms and their assessment
in life-​limiting illness 137
Dilini Rajapakse and Maggie Comac
15. Using medication in children’s palliative care 145
Andy Gray, Jane Riddin, and Richard Hain
16. Introduction to pain 153
Antoine Bioy and Chantal Wood
17. Multimodal analgesia in paediatric palliative
care 165
Stefan J. Friedrichsdorf
18. Opioids and the World Health Organization
pain ladder 176
Manuel Rigal, Ricardo Martino, and Richard Hain
19. Difficult pain: Adjuvants or co-​analgesics 188
Renee McCulloch and Charles Berde
20. Integrative approach to pain and other
symptoms 202
David M. Steinhorn
viii Contents

21. Gastrointestinal and liver-​related symptoms in 32. Care in the final hours and days 352
paediatric palliative care 214 Dawn Davies and Justin Baker
Jo Laddie, Alta Terblanche, and Michelle Meiring
33. Delivering care around the world 361
22. Feeding, cachexia, and malnutrition in children’s Julia Downing and Joan Marston
palliative care 231
34. Healthcare providers’ responses to the death
Sanjay Mahant, Michelle Meiring, and Adam Rapoport
of a child 373
23. Neurological and neuromuscular conditions and Danai Papadatou
symptoms 244
35. Teamwork 382
Jori F. Bogetz and Julie M. Hauer
Jan Aldridge and Pat Carragher
24. Depression, anxiety, and delirium 255
36. Education 390
Pamela J. Mosher and Anna C. Muriel
Fiona Rawlinson and Michelle Meiring
25. Cardiorespiratory symptoms 267
37. Quality improvement in paediatric hospice
Emily Harrop and Roxanne Kirsch
and palliative care 401
26. Skin symptoms 280 Susan Blacker and Rachel Thienprayoon
Carol Hlela, Rene Albertyn, and Michelle Meiring
38. Research in children’s palliative care 410
27. Haematological symptoms 296 Harold Siden and Kimberley Widger
Mei-​Yoke Chan and Kevin Weingarten

28. Palliative care for children with communicable Appendices

illnesses 304
Michelle Meiring and Tonya Arscott-​Mills
The Association of Paediatric Palliative Medicine Master
Formulary, Fifth Edition, 2020 419

SECTION IV Index 501

Delivery of care
29. Perinatal palliative care 325
Áine Ni Laoire, Daniel Nuzum, Maeve O’Reilly, Marie Twomey,
Keelin O’Donoghue, and Mary Devins

30. Intensive care units 332

Brian S. Carter

31. Planning care 341

Michelle Grunauer and Jenny Hynson
Detailed contents

Contributors xvii 3. ‘Children are not small adults’—​the distinctiveness

Abbreviations xix of ethics in children 25
Robert Macauley and Richard Hain
Introduction 25
Decision-​making—​parental authority to request ‘harmful’
SECTION I interventions—​and ditto to request futile ones 26
Foundations of care Research in children 27
Principle of double effect 32
1. History and epidemiology 3 Euthanasia 33
Lorna Fraser, Stephen Connor, and Joan Marston Summary 34
Introduction 3 References 34
Definitions 3
Hospice 4 4. Decision-​making with children, young people,
History 6 and parents 36
Myra Bluebond-​Langner and Richard Langner
Epidemiology 6
Using these data to estimate the need for CPC services 15 Introduction 36
Conclusion 16 Decision-​making 36
References 16 The role of parents 38
Participation of CYP in decision-​making 39
2. Communication 17 Clinical implications 41
Jennifer Mack and Bryan Sisk In summary 42
Introduction 17 References 42
Communication as a foundation of palliative care 17
Barriers to effective communication 18 5. Culture, spirituality, religion, and ritual 44
Erica Brown, Mary Ann Muckaden, and Nokuzola Mndende
Establishing a relationship and the development of shared
knowledge: Learning from the child and family 18 Introduction 44
Establishing a relationship and the development of shared Definitions 44
knowledge: Transmission of information to the child and Influence of religion, spirituality, and culture on children with
family 20 life-​limiting conditions 44
Establishing a relationship and responding to emotions 21 Worldviews, cycles, and meaning of life 45
Identifying values and making shared decisions 21 The role of spirituality in children’s palliative care 46
When should conversations about palliative care take place? 22 The role of religion in children’s palliative care 46
Communication across cultural and language differences 22 Some faith and cultural traditions 46
Communicating about prognosis 23 Secular beliefs 50
Conclusion 24 Cultural and religious aspects in perinatal and neonatal palliative
References 24 care 50
Chaplains and faith leaders 50
x Detailed contents

Cultural and religious aspects from the literature in The child’s voice in decision-​making 79
EOL care 50 Awareness of impending death 79
Effect of religious and spiritual beliefs in treating physicians 51 Psychotherapy—​a conceptual framework 81
Cross-​cultural support 51 A case study 82
An example of care and spirituality from South Africa—​a Conclusion 85
traditional African perspective 52
Acknowledgement 86
Conclusion 52
References 86
References 54
9. Adolescents and young adults 87
Chana Korenblum and Finella Craig

SECTION II Defining adolescence and young adulthood 87

The prevalence of LLC in AYAs 87
Child and family care Adolescence as a transition phase 87
Normal adolescent development 87
6. Assessment of the child and family 59
Nancy Contro and Jane Zimmerman Challenges to adolescent development in the palliative care
population 87
Introduction 59
Changing relationships with parents 88
Theoretical considerations 59
Peer group identification 88
Practical considerations 60
Independence 88
Evidence-​based considerations 63
Developing a sexual identity 89
Summary 65
Spiritual identity 89
References 65
Psychological development 89
7. Children’s views of death 66 Supporting the transition to adulthood 89
Myra Bluebond-​Langner and Ignasi Clemente Facilitating peer group interaction and independence 89
Introduction 66 Support for sexual development 90
Children’s views of death: A multi-​dimensional, Spiritual support 90
multi-​faceted perspective 66 Support for decision-​making 90
Views of death in children with life-​limiting conditions and life-​ Psychological support 91
threatening illnesses: Expressing their thoughts in words and Respite care 91
deeds 67
Advance care planning 91
Well siblings’ views of death: Comparisons with ill siblings 69
Transition to adult services 92
Talking with others about death: Conversations between
The needs of professional staff and carers 93
ill children, their parents, siblings, and clinicians 70
Service development 93
Suggestions for discussions with children about death
and dying 72 Summary 93
Talking about death with bereaved children: References 94
An additional note 73
Conclusion 73 10. Children expressing themselves 95
Amy Volans and Emma Brown
Acknowledgements 73
References 73 Introduction 95
Defining and evaluating creative therapies 96
8. The psychological impact of life-​limiting Music therapy 96
conditions on the child 75 Art therapy 96
Jan Aldridge and Barbara M. Sourkes Play and play therapy 96
Introduction 75 Communication and play with infants and children with
Emotional support 76 special communication needs 97
Clinical supervision 76 Working clinically with children using creative art therapies 98
Psychological issues 76 Artistic achievements as expressive acts 99
Work with parents 77 Storytelling and narrative therapy 100
Brothers and sisters 78 Working with children’s strengths and abilities 101
 Detailed contents xi

Nurturing fantasy and pleasurable imagination 101 Acknowledgements 134

Supporting the dying child and their family 102 References 134
Acknowledgement 102
References 102

11. Education and school 103 SECTION III

Sue Boucher Symptom care
Introduction 103
Why educate children who have progressive life-​limiting 14. Overview of symptoms and their assessment
illnesses? 103 in life-​limiting illness 137
How illness disrupts education 104 Dilini Rajapakse and Maggie Comac
Providing educational opportunities for life-​limited Introduction 137
children 104 The importance of accurate symptom assessment 137
Virtual learning environments (VLE) 104 The prevalence of symptoms in children with
Principles of good practice to support life-limited children’s life-​limiting illness 138
access school education 105 The principles of symptom evaluation 140
Conclusion 110 Symptom measurement in children 141
References 110 Challenges to comprehensive symptom evaluation 142
Novel approaches to symptom evaluation 142
12. Impact on the family 111
Symptom evaluation in paediatric palliative care: Areas for
Veronica Dussel and Barbara Jones with
further study 143
Kevin O’Brien, and Melissa Williams-Platt
References 144
Introduction 111
What do we call family? 111 15. Using medication in children’s palliative care 145
Family systems 111 Andy Gray, Jane Riddin, and Richard Hain
Family and culture 112 Introduction 145
Effects of a child’s LLC on the family 112 Pharmacokinetic considerations in children 145
Living with the LLC or illness 113 Distribution 147
Family adjustments to a child’s LLC 114 Transport 147
How can we help families? 116 Biotransformation and metabolism 147
The wider context of care 119 Distribution 148
A challenge for the future 120 Elimination 149
Acknowledgements 124 Therapeutic research in children 149
References 124 Summary 150
References 151
13. Bereavement 126
Sara Portnoy and Lori Ives-Baine 16. Introduction to pain 153
Introduction 126 Antoine Bioy and Chantal Wood
Impact on families 126 Introduction 153
Impact on children and young people 127 The history of pain and the actual definition of pain 153
Manifestations of grieving adults and children 127 What is pain? 155
Impact on schools 128 The neurophysiology of pain 155
Impact on hospitals, community providers, and hospices 128 Excitatory mechanisms 156
Helping theories of bereavement make sense 129 Different types of pain 157
Risk and resilience seesaw 130 Pain and the child in palliative care 157
Time passing 131 Assessment of the pain 160
Concerned curiosity: A way of talking to bereaved The relationship between the carer and the patient who is in
families 131 pain 161
What helps grieving families? 131 Conclusion 162
When families may need more help 133 References 163
Conclusion 133
xii Detailed contents

17. Multimodal analgesia in paediatric palliative Common GIT symptoms in children’s palliative
care 165 care 214
Stefan J. Friedrichsdorf Nausea and vomiting 216

Introduction 165 Constipation 218

Multimodal analgesia 165 Diarrhoea 220

Pain in children with SNI 166 Other symptoms 221

Multimodal analgesia 167 Intestinal failure 224

Procedural pain and chronic pain treatment 169 Chronic liver disease 227

Conclusion 170 Conclusion 229

References 171 References 229

18. Opioids and the World Health Organization 22. Feeding, cachexia, and malnutrition in children’s
pain ladder 176 palliative care 231
Manuel Rigal, Ricardo Martino, and Richard Hain Sanjay Mahant, Michelle Meiring, and Adam Rapoport

Introduction 176 Introduction 231

Opioids: Their power and range 176 Definitions 231

World Health Organization (WHO) approach to pain FTT and feeding difficulties 231
management 178 Cachexia and anorexia 237
‘By the child’: Taking a pain history 179 Malnutrition 240
‘By the clock’: Regular opioids, breakthrough opioids, and the Pathophysiology and management 240
relationship between them 182 Conclusions 242
‘By the right route’ 182 References 242
Special situations 184
Summary 185 23. Neurological and neuromuscular conditions
References 186 and symptoms 244
Jori F. Bogetz and Julie M. Hauer
19. Difficult pain: Adjuvants or co-​analgesics 188 Introduction 244
Renee McCulloch and Charles Berde A palliative approach to neurological and neuromuscular
Introduction 188 conditions in children 244
Combination pharmacotherapy 188 Specific conditions affecting the nervous system 247
Visceral hyperalgesia 194 Symptoms of neurological impairment and neuro-​specific
symptoms 248
Deep tissue pain 196
Non-​pharmacological management 248
References 200
Pain, agitation, and irritability 248
20. Integrative approach to pain and other Autonomic dysfunction 249
symptoms 202 Seizures 250
David M. Steinhorn Spasticity and muscle spasms 251
Introduction 202 Dystonia 252
Goals of integrative therapy 202 Chorea 252
Why should we include integrative therapies in palliative and Myoclonus 253
hospice care? 203 Medication toxicities 253
What are integrative therapies? 203 Sleep 253
Use of integrative approaches in paediatric palliative care 204 Fatigue 253
Summary 211 Conclusion 254
References 211 References 254

21. Gastrointestinal and liver-​related symptoms in 24. Depression, anxiety, and delirium 255
paediatric palliative care 214 Pamela J. Mosher and Anna C. Muriel
Jo Laddie, Alta Terblanche, and Michelle Meiring
Introduction 255
Introduction 214 Depression 255
 Detailed contents xiii

Ketamine 260 Summary 302

Anxiety 260 References 302
Diagnostic tools 261
28. Palliative care for children with communicable
Important details concerning anti-​anxiety medications
in CYP 261 illnesses 304
Cannabinoids 262 Michelle Meiring and Tonya Arscott-​Mills
Irritability and anger 262 Introduction 304
Delirium 262 Life-​threatening and life-​limiting communicable diseases in
children 304
Mania 264
ACT I: Potentially curable infections 305
Conclusion 264
Measles 305
References 266
Acute hepatitis 308
25. Cardiorespiratory symptoms 267 Severe bacterial illnesses (SBI) 308
Emily Harrop and Roxanne Kirsch TB 309
Introduction 267 Malaria 309
Cough 271 ACT II: Life-limiting but non-progressive (with treatment) 310
Tachycardia, palpitations, chest pain 273 ACT III: Progressive and non-curable diseases 312
Fatigue, irritability, feeding intolerance 274 MDR-TB and XDR-TB 313
The role of mechanical circulatory support 274 ACT IV: Non-progressive but irreversible damage—often
Compassionate discontinuation of ventilator or MCS 276 associated severe disability 315
Case 25.1 277 Zika virus infection 316
Case 25.2 277 Palliative care in neglected tropical diseases (NTDs) 317
Case 25.3 278 Conclusions 319
Conclusion 278 References 319
References 278

26. Skin symptoms 280

Carol Hlela, Rene Albertyn, and Michelle Meiring
SECTION IV
Introduction 280 Delivery of care
Life-​threatening primary skin conditions: Congenital 280
29. Perinatal palliative care 325
Life-threatening primary skin conditions: Acquired 285
Áine Ni Laoire, Daniel Nuzum, Maeve O’Reilly, Marie Twomey,
Other life-​threatening acquired skin disorders that would Keelin O’Donoghue, and Mary Devins
benefit from a palliative care approach include 288
Background 325
Psychosocial care and support for patients with life-​threatening
primary skin conditions 289 Ethical considerations 325
Secondary skin complications (dermatological symptoms in Diagnosis 326
children with life-​limiting disorders) 291 Types of antenatal diagnosis 326
Fungating wounds and pressures sores 292 Palliative care approach to pregnancy 327
Conclusion 293 Care 329
References 294 Investigation and follow-​up 330
Summary 331
27. Haematological symptoms 296
References 331
Mei-​Yoke Chan and Kevin Weingarten
Introduction 296 30. Intensive care units 332
Symptoms associated with anaemia 296 Brian S. Carter
Management of symptomatic anaemia in palliative Introduction 332
care 297 The role of PPC in the PICU 333
Thrombosis and its management 301 Who should receive palliative care in the PICU? 333
Ethical issues related to the transfusion of blood products When children die in the PICU 333
at end of life 301
Communication issues 335
xiv Detailed contents

Family-​centered care in the PICU 336 34. Healthcare providers’ responses to the death
Providing and respecting family choice with regard to place of of a child 373
care 337 Danai Papadatou
Transferring a child to home or hospice for withdrawal of The myth 373
mechanical ventilation 338
The reality 373
Summary 338
Aspects of healthcare providers’ suffering 375
Acknowledgement 339
A model of healthcare providers’ grieving process 375
References 339
Conclusion 380
31. Planning care 341 References 380
Michelle Grunauer and Jenny Hynson
35. Teamwork 382
Introduction 341
Jan Aldridge and Pat Carragher
Benefits of planning in children’s palliative care (CPC) 341
Introduction 382
Palliative care planning across the continuum of disease 342
Thinking about teams 382
Possible triggers for care planning 342
Limitations of teams? 383
ACP 343
Delivery of paediatric palliative care 384
Barriers to ACP 344
Key challenges 384
Approaching ACP discussions 345
Communication 384
Patient assessment 345
Managing conflict 385
Clinical circumstances 345
Conflict and caring in paediatric palliative care 386
Place of care 347
Leadership 387
Special considerations 348
Reflective practice 387
Palliative care in humanitarian disasters 350
A secure base in the face of uncertainty and loss 388
Conclusion 350
Conclusion 388
References 350
References 388
32. Care in the final hours and days 352
36. Education 390
Dawn Davies and Justin Baker
Fiona Rawlinson and Michelle Meiring
Introduction 352
Introduction: Why are education and training
Changes in the goals of treatment 352 important? 390
Requests for hastened death 354 Who needs education and training? 390
Anticipating likely symptoms 354 How should education and training be delivered? 392
Lines of communication 354 What should education and training in PC for children
Palliative care emergencies 355 include? 397
Setting for care in the final phase of life 357 How do we measure effectiveness of children’s PC
References 359 education? 398
Summary 399
33. Delivering care around the world 361 References 399
Julia Downing and Joan Marston
Introduction 361 37. Quality improvement in paediatric hospice
The history of CPC 361 and palliative care 401
Susan Blacker and Rachel Thienprayoon
Identifying the need for CPC globally 363
The status of CPC globally 363 Introduction 401
Challenges to the provision of CPC globally 363 Quality in healthcare 401
CPC in LMICs 365 Quality in hospice and palliative care 401
Developing CPC globally 366 Embarking on QI: Translating knowledge into practice 402
Conclusion 371 Implementing change 403
References 371 Challenges and opportunities: The future of QI in paediatric
palliative care 406
 Detailed contents xv

Conclusions 407 Appendices

QI resources 407 The Association of Paediatric Palliative Medicine Master
References 408 Formulary, Fifth Edition, 2020 419
38. Research in children’s palliative care 410 Appendix 1: Formulary 421
Harold Siden and Kimberley Widger Appendix 2: Morphine equivalence single dose 481
Appendix 3: SC infusion drug compatibility 482
Introduction 410
Appendix 4: Gabapentin to pregabalin switch for
Importance of CPC research and priority areas 410
neuropathic pain 483
Challenges to CPC research 410
Appendix 5: Benzodiazepines 484
Strategies to overcome challenges 412
References 486
Advice to novice and reluctant researchers 417
Future directions 417
References 417
Index 501
Contributors
Rene Albertyn Senior Researcher and Lecturer,
Department of Paediatric Surgery, Red Cross
War Memorial Children’s Hospital; University of
Cape Town, Rondebosch, South Africa
Jan Aldridge Consultant Clinical Psychologist,
Leeds Children`s Hospital, UK; Professor
(Research), Department of Social Policy and
Social Work, University of York, York, UK
Mary Ann Muckaden Tata Memorial Centre,
Homi Bhabha National University,
Mumbai, India
Tonya Arscott-​Mills Clinical Assistant Professor
of Pediatrics, Perelman School of Medicine at
the University of Pennsylvania, Philadelphia,
PA, USA
Justin Baker Chief, Division of Quality of Life and
Palliative Care,Director, Pediatric Hematology/
Oncology Fellowship Program,Attending
Physician, Quality of Life for All (QoLA)
Team,St Jude Children’s Research Hospital
Memphis, TN, USA
Charles Berde Sara Page Mayo Chair in Pediatric
Pain Medicine, Department of Anesthesiology,
Critical Care and Pain Medicine, Boston
Children’s Hospital; Professor of Anaesthesia,
Harvard Medical School, Boston, MA, USA
Antoine Bioy Full Professer of Psychology,
University of Paris 8 and Ipnosia Center, Paris,
France
Susan Blacker Sinai Health System, Toronto,
ON, Canada
Myra Bluebond-​Langner Professor and True
Colours Chair in Palliative Care for Children
and Young People, Louis Dundas Centre for
Children’s Palliative Care, UCL-​Institute of
Child Health, London, UK; Board of Governors’
Professor of Anthropology, Rutgers University,
Camden, NJ, USA
Jori F. Bogetz Division of Bioethics and Palliative
Care, Department of Pediatrics, University
of Washington School of Medicine, Seattle
Children’s Hospital, Seattle, WA, USA
Sue Boucher Palliative Treatment for Children
South Africa (PatchSA), South Africa
Emma Brown Health Play Specialist, Diana
Children’s Community Palliative Care Team,
Newham Children’s Community Nursing
Service, East London NHS Foundation Trust,
London, UK
Erica Brown Senior Research Fellow, University of
Worcester, Worcester, UK
Pat Carragher Medical Director to Children’s
Hospices Across Scotland (CHAS), Edinburgh,
Scotland, UK
Brian S. Carter Sirridge Professor of Medical
Humanities and Bioethics, University of
Missouri-​Kansas City, School of Medicine; and
Professor of Pediatrics (Neonatology and Fetal
Health), Children’s Mercy Hospital, Kansas City,
MO, USA
Mei-​Yoke Chan Senior Consultant, Paediatric
Haematology/​Oncology, Department of
Paediatric Subspecialties, KK Women’s and
Children’s Hospital, Singapore
Ignasi Clemente Department of Anthropology,
Hunter College CUNY, New York, NY, USA; and
Louis Dundas Centre for Children’s Palliative
Care, UCL-Institute of Child Health, London,
UK
Maggie Comac Advanced Nurse Practitioner,
Great Ormond Street Hospital Oncology
Outreach and Palliative Care Team, Louis
Dundas Centre, London, UK
Stephen Connor Executive Director, Worldwide
Hospice Palliative Care Alliance, Fairfax Station,
Virginia, VA, USA
Nancy Contro Executive Director, National
Center for Equine Facilitated Therapy (NCEFT),
Woodside, CA, USA
Finella Craig Consultant in Paediatric Palliative
Medicine, the Louis Dundas Centre for
Children’s Palliative Care, Great Ormond Street
Hospital NHS Trust, London, UK
Dawn Davies Medical Director, Pediatric Palliative
Care Program, Stollery Children’s Hospital;
Associate Professor, Department of Pediatrics,
University of Alberta, Edmonton, AB, Canada
Mary Devins Consultant Paediatrician with a
Special Interest in Paediatric Palliative Medicine,
Children’s Health Ireland, Crumlin; and The
Coombe Women and Infants Maternity Hospital,
Dublin, Ireland
Julia Downing Chief Executive, International
Children’s Palliative Care Network (ICPCN),
Professor Makerere University, Uganda, UK
Veronica Dussel Director, Center for Research
and Implementation in Palliative Care (CII-CP)
Institute for Clinical Effectiveness and Health
Policy (IECS), Buenos Aires, Argentina, and,
Associate Research Scientist, Pediatric Palliative
Care, Dana-Faber Boston Children’s Cancer and
Blood Disorders Center, Boston, MA, USA
Lorna Fraser Professor of Epidemiology and
Director of the Martin House Research Centre,
Department of Health Science, University of
York, York, UK
Stefan J. Friedrichsdorf Medical Director, Center
of Pediatric Pain Medicine, Palliative Care
and Integrative Medicine, Benioff Children’s
Hospitals in Oakland and San Francisco; and
Professor, Department of Pediatrics, University
of California at San Francisco (UCSF),
San Francisco, CA, USA
Ann Goldman Paediatrician and Palliative Care
Specialist, London, UK
Andy Gray Division of Pharmacology, Discipline
of Pharmaceutical Sciences, University of
KwaZulu-​Natal, Durban, South Africa
Michelle Grunauer Dean of the School of
Medicine, Colegio de Ciencias de la Salud,
Universidad San Francisco dr Quito; Consultant
and Academic Director of the Pediatric Intensive
Care Unit, Hospital de los Valles, Quito, Ecuador
Richard Hain Consultant and Clinical Lead,
All-Wales Managed Clinical Network in
Paediatric Palliative Medicine and Honorary
Professor in Clinical Ethics, University of
Swansea, Wales, UK
Emily Harrop Medical Director & Consultant in
Paediatric Palliative Care, Helen & Douglas
House; and Honorary Consultant Oxford
University Hospitals NHS Trust, Oxford, UK
Julie M. Hauer Medical Director, Seven
Hills Pediatric Center, Division of General
Pediatrics, Children’s Hospital Boston, Harvard
Medical School, Boston, MA, USA
Carol Hlela Head of Unit, Paediatric Dermatology,
Department of Paediatrics and Child Health,
Red Cross Children’s Hospital University of
Cape Town, Rondebosch, South Africa
Jenny Hynson Clinical Associate Professor,
University of Melbourne and Medical Director,
Victorian Paediatric Palliative Care Program
Melbourne, Australia
Lori Ives-​Baine Grief Support Coordinator,
Paediatric Advanced Care Team, The Hospital
for Sick Children (SickKids), University of
Toronto, Toronto, ON, Canada
Barbara Jones University Distinguished Teaching
Professor, Associate Dean for Health Affairs,
and Co-​Director for Institute for Collaborative
Health Research and Practice, The University
of Texas, Austin, TX, USA
xviii Abbreviations
Roxanne Kirsch Cardiac Critical Care
Medicine (CCCU), Clinical Bioethics Associate, The
Hospital for Sick Children, Toronto, ON, Canada
Chana Korenblum Department of Supportive Care,
Princess Margaret Cancer Centre, and Division of
Adolescent Medicine, Department of Pediatrics,
The Hospital for Sick Children (SickKids),
University of Toronto, Toronto, ON, Canada
Jo Laddie Consultant in Paediatric Palliative Care,
Evelina London Children’s Hospital, Guys and
St Thomas’s NHS Foundation Trust, London, UK
Richard Langner Palliative Care for Children
and Young People, Louis Dundas Centre for
Children’s Palliative Care, UCL-​Institute of
Child Health, London, UK
Robert Macauley Cambia Health Foundation
Endowed Chair in Pediatric Palliative Care,
Oregon Health and Science University, Portland,
OR, USA
Jennifer Mack Pediatrics, Harvard Medical School;
Pediatric Oncology, Dana-​Farber Cancer
Institute, Boston, MA, USA
Sanjay Mahant Associate Professor, Department
of Paediatrics, The Hospital for Sick Children
(SickKids), University of Toronto, Toronto,
ON, Canada
Joan Marston Executive Coordinator for Palliative
Care in Humanitarian Aid Situations and
Emergencies PallCHASE; Paediatric Palliative
Care Nurse Consultant, Sunflower Children’s
Hospice, South Africa
Ricardo Martino Lead Consultant in Paediatric
Palliative Medicine, Pediatric Palliative
Care Service, Niño Jesús University Children’s
Hospital, Madrid, Spain; Course Director
Master’s Degree in Paediatric Palliative Care, La
Rioja International University (UNIR), Spain
Renee McCulloch Lead Consultant in Paediatric
Pain and Palliative Medicine, NBK hospital,
Ministry of Health, Kuwait, Honorary Senior
Lecturer, Great Ormond Street Hospital,
Institute of Child Health, London, UK
Michelle Meiring Paedspal and the Department
of Paediatrics and Child Health, University of
Cape Town, South Africa
Nokuzola Mndende Research Associate, University
of Free State, Bloemfontein, South Africa
Pamela J. Mosher Department of Supportive
Care, Division of Psychosocial Oncology,
Princess Margaret Cancer Centre; Department
of Psychiatry, University of Toronto; Consultant,
Pediatric Advanced Care Team (PACT), The
Hospital for Sick Children, ON, Canada
Anna C. Muriel Associate Professor of Psychiatry,
Department of Psychosocial Oncology and
Palliative Care, Dana-​Farber Cancer Institute;
Department of Psychiatry, Harvard Medical,
Boston Children’s Hospital, Boston, MA, USA
Áine Ni Laoire Consultant in Palliative Medicine,
South East Palliative Care Centre, University
Hospital Waterford, Waterford, Ireland
Daniel Nuzum Healthcare Chaplain and Clinical
Pastoral Education Supervisor, Cork University
Hospital; Department of Obstetrics and
Gynaecology, College of Medicine and Health,
University College Cork, Ireland
Kevin O’Brien Bereaved Father (Catie’s dad),
Mechanicsburg, Pennsylvania, PA, USA
Keelin O’Donoghue Consultant Obstetrician,
Cork University Maternity Hospital; and
Senior Lecturer, Department of Obstetrics and
Gynaecology, University College Cork, Ireland
Maeve O’Reilly Consultant in Palliative Medicine,
St. Luke’s Hospital, Dublin; and Children’s
Health Ireland, Crumlin, Ireland
Danai Papadatou Professor of Clinical
Psychology Work Setting: Faculty of Nursing,
National and Kapodistrian, University
of Athens, Greece
Sara Portnoy Consultant Clinical Psychologist,
University College Hospital; Life Force
(Community Paediatric Palliative Care and
Bereavement Team) in Camden, Islington and
Haringey, London, UK
Dilini Rajapakse The Louis Dundas Centre for
Children’s Palliative Care, Great Ormond Street
Hospital for Children NHS Foundation Trust,
London, UK
Adam Rapoport Medical Director, Paediatric
Advanced Care Team (PACT), The Hospital for
Sick Children (SickKids); University of Toronto
and Emily’s House Children’s Hospice, Toronto,
ON, Canada
Fiona Rawlinson Consultant in Palliative Medicine
and Postgraduate Course Director, School of
Medicine, Cardiff University, UK
Jane Riddin Affordable Medicines Directorate,
National Department of Health, Pretoria,
South Africa
Manuel Rigal Consultant in Paediatric Palliative
Medicine, Pediatric Palliative Care Service, Niño
Jesús University Children’s Hospital, Madrid, Spain
Harold Siden Medical Director, Canuck Place
Children’s Hospice, Child and Family Research
Institute, BC Children’s Hospital, University of
British Columbia, Vancouver, BC, Canada
Bryan Sisk Division of Hematology and Oncology,
Department of Pediatrics, Washington
University, St. Louis, MO, USA
Barbara M. Sourkes Professor of Pediatrics,
Stanford University School of Medicine,
Kriewall-​Haehl Director; Pediatric Palliative
Care Program, Lucile Packard Children’s
Hospital Stanford, Palo Alto, CA, USA
David M. Steinhorn Director, Pediatric
and Perinatal Program Development,
The Elizabeth Hospice; Adjunct Clinical
Professor of Pediatrics Children’s Hospital of
Los Angeles Keck School of Medicine - USC,
California, CA, USA
Alta Terblanche University of Pretoria,
South Africa
Rachel Thienprayoon Medical Director, StarShine
Hospice and Palliative Care, Associate Professor
of Anesthesia (Palliative Care) University of
Cincinnati College of Medicine, Cincinnati
Children’s Hospital Medical Center, Cincinnati,
OH, USA
Marie Twomey Consultants in Palliative Medicine,
St. Luke’s Hospital, Dublin; and Children’s
Health Ireland, Crumlin, Ireland
Amy Volans Clinical Psychologist and Family
Therapist, Diana Children’s Community
Palliative Care Team, Newham Children’s
Community Nursing Service, East London NHS
Foundation Trust, London, UK
Kevin Weingarten Paediatric Advanced Care
Team (PACT); The Hospital for Sick Children
(SickKids), University of Toronto, Toronto,
ON, Canada
Kimberley Widger Associate Professor and Tier 2
Canada Research Chair-Pediatric Palliative Care,
Lawrence S. Bloomberg Faculty of Nursing,
University of Toronto, Nursing Research
Associate, Paediatric Advanced Care Team
(PACT), The Hospital for Sick Children; Adjunct
Scientist, Lifespan Program, ICES, Toronto, ON,
Canada
Melissa Williams-Platt Integral and Palliative
Coach, Footprints 4 Sam Trust, Patch SA
and Kotula Management Services (Pty) Ltd,
Johannesburg, South Africa
Chantal Wood Department of Spine,
Neurostimulation and Rehabilitation, University
Hospital, Poitiers, France
Jane Zimmerman Licensed Clinical Social
Worker, Stanford Children’s Health, Palo Alto,
CA, USA
Abbreviations

HT1-​5 1–​5-​hydroxytryptamine CAM complementary and alternative medicine

A&E accident and emergency CAM confusion assessment method
a2-​d alpha 2 delta CAPD Cornell Assessment of Pediatric Delirium
AAHPM American Academy of Hospice and Palliative CART cocaine and amphetamine-​regulated transcript
Medicine CBC Child Behavior Checklist
AAP American Academy of Pediatrics CBD cannabidiol
ACE-​I angiotensin-​converting enzyme inhibitors CBT cognitive behavioural therapy
ACh acetylcholine CC cut into quarters
ACP advanced care plan CD child dose
ACT acceptance and commitment therapy CDI Child Depression Inventory
ACT activated coagulation time CF cystic fibrosis
ACT Association for Children with Life-​Threatening CFS cutaneous field stimulation
or Terminal Conditions CHI Children’s Hospice International
ACT I: potentially curable infections CHIPRA Children’s Health Insurance Program
ACT II: life-​limiting but non-​progressive (with Reauthorazation Act
treatment) ChiSP Children in Scotland Requiring Palliative Care
ACT III: progressive and non-​curable diseases CINV chemotherapy induced nausea and vomiting
ACT IV: non-​progressive but irreversible damage—​ CMV cytomegalovirus
often associated with disability CNPCC Canadian Network of Palliative Care for
ADHD attention deficit hyperactivity disorder Children
AgRP agouti-​related peptide CNS central nervous system
AIDS acquired immunodeficiency syndrome COMT catechol-​O-​methyltransferase
ALL acute lymphoblastic leukaemia CorGA corrected gestational age
ALT alanine aminotransferase COX-​1 Cyclooxygenase-​1
AM anthroposophical medicine COX-​2 Cyclooxygenase-​2
AND allow natural death CP cerebral palsy
ANG acute necrotizing gingivitis CPAC Center to Advance Palliative Care
ANH artificial, or nutrition, and hydration CPAP continuous positive airway pressure
Annals ATS Annals of the American Thoracic CPC children’s palliative care
Society CPR cardiopulmonary and pharmacological
APCA African Palliative Care Association resuscitation
APPM Association of Paediatric Palliative Medicine CQI continuous quality improvement
ARE evidence (research or clinical consensus) CRC Convention on the Rights of the Child
with adults CSCI continuous subcutaneous infusion
ART antiretroviral therapy CSF cerebrospinal fluid
ARV antiretroviral medicines CT computerized tomography
AST aspartate aminotransferase CVL central venous line
ASyMS© Advanced Symptom Management System CYP children and young people
AYAs adolescents and young adults D2 dopamine
BID/​BD twice daily DDEB dominant epidermolysis bullosa
BNF British National Formulary DEBRA International Dystrophic Epidermolysis Bullosa
BNFC British National Formulary for Children Research Association International
BSFS Bristol Stool Form Scale DEXA dexamethasone
BTX botulinum toxin DIC disseminated intravascular coagulation
BW body weight DM delirious mania
xx Abbreviations

DMC decision-​making capacity HICs health information communities

DMD Duchenne muscular dystrophy HIV human immunodeficiency virus
DNAR do not attempt resuscitation/​DNR do not HLA human leukocyte antigens
resuscitate HOOF home oxygen order forms
DRS delirium rating scale HOPE H-​sources
DR-​TB drug resistant TB HPCA Hospice Palliative Care Association of
DSM-​5 Diagnostic and Statistical Manual of Mental South Africa
Disorders (Fifth Edition) HPN Home Parenteral Nutrition
DVT Deep vein thrombosis HPNA Hospice and Palliative Nurses Association
EAPC European Association for Palliative Care HRH reflux-​related hospitalization rates
EB epidermolysis bullosa IAHPC International Association of Hospice and
EBS epidermolysis bullosa simplex Palliative Care
ECG electrocardiogram ICPCN International Children’s Palliative Care Network
ECMO extracorporeal membrane oxygenation ICU intensive care unit
ECUs Ebola care units IDC-​0 International Classification of Diseases
EEG electroencephalogram (Version 10)
EKG electrocardiograms IEM inborn errors of metabolism
EMLA eutectic mixture of local anaesthetics IFALD intestinal failure associated liver disease
EOL end of life IFM immunofluorescence antigen mapping
EPEC-​ Education in Palliative and End-​of-​life IFN-​g Interferon gamma
Pediatrics Care-​Pediatrics IHME Institute for Health Metrics and Evaluation
EPS extrapyramidal symptoms Il-​6 Interleukin-​6
ESA erythropoiesis-​stimulating agents IM integrative medicine/​intramuscular
ESPGHAN European Society for Pediatric IMCI Integrative Management of Childhood Illnesses
Gastroenterology, Hepatology, and Nutrition programme
ET endotracheal tube IMOC integrated model of care
ETCs Ebola treatment centres INF intranasal fentanyl
EVD Ebola virus disease/​external ventricular drain INR international normalized ratio
FDA US Food and Drug Administration IOM Institute of Medicine
FFA fatal foetal anomaly IRBs institutional review boards
FICA faith beliefs, importance influence community IRIS immune reconstitution illness syndrome
assessment ISAP International Association for the Study of Pain
FLACC face, legs, activity, cry, consolability ISMAR Innsbruck Sensory Motor Activator and
fMRI functional magnetic resonance imaging scan Regulator
FTT failure to thrive IV intravenous
G/​GT gastronomy J jejunostomy
GABA γ-​aminobutyric acid JEB junctional epidermolysis bullosa
GABAA γ-​aminobutyric acid type A JMML juvenile myelomonocytic leukaemia
GAPRI Global Access to Pain Relief Initiative KOR Kappa-​receptor opioid
GBS group B streptococcal LCSW licenced clinical social worker
GCPQA Global Palliative Care Quality Alliance LDLT living donation liver transplantation
GCS Glasgow coma scale LETM longitudinally extensive transverse myelitis
GERD gastro-​oesophageal reflux LIP lymphocytic interstitial pneumonitis
GFR glomerular filtration rate LLC life-​limiting conditions
GGT gamma-​glutamyl transferase LMCI low-​and middle-​income countries
GI/​GIT gastrointestinal (tract) LMWH low-​molecular-​weight heparin
GJ gastrojejunostomy LSMT life-​sustaining medical therapies
GNP gross national product LST life-​sustaining interventions
GOR gastro-​oesophageal reflux LTC life-​threatening conditions
GORD gastro-​oesophageal reflux disease LTV long-​term ventilation
G-​protein guanine nucleotide-​binding proteins M3G morphine 3-​glucuronide
H histamine M3G morphine-​3-​glucuronide
HAART highly active antiretroviral therapy M6G morphine-​6-​glucuronide
HBV hepatitis B MAMI management of malnutrition in infants
HCPs health-​care professionals MAOIs monoamine oxidase inhibitors
HFA hydrofluoroalkane MBC measurement-​based care
HI harlequin ichthyosis MBO malignant bowel obstruction
HIC high-​income countries MBSR mindfulness-​based stress reduction
 Abbreviations xxi

MCQs multiple choice questions OIH opioid-​induced hyperalgesia

MCS mechanical circulatory support OM oral morphine
MDAS Memorial Delirium Assessment Scale OME oral morphine equivalence
MDI metered dose inhaler OR opioid receptors
MDR-​TB multi-​drug resistant TB OROS® osmotic-​release oral delivery system
MDR-​TB multi-​drug-​resistant tuberculosis ORS oral rehydration solution
MDT multidisciplinary teams PACT Paediatric Advanced Care Team
MHRA Medical and Health Research Association PACT patient-​aligned care team
MOOCs massive open online courses PAG periaqueductal grey
MOR micro opioid receptor PaPaS Paediatric Palliative Screening Scale
MPA medroxyprogesterone acetate PAS p-​aminosalicylic acid
MRC British Medical Research Council PC patient care/​palliative care
MRI magnetic resonance imaging PCA/​NCA patient/​nurse controlled analgesia
MRP multidrug resistance protein PCQN The Palliative Care Quality Network
MSAS Memorial Symptom Assessment Scale PCQN-​Peds Palliative Care Quality Network Paediatrics
MST morphine sulphate tablets/​multiple subpial PCRA patient controlled regional analgesia
transection PCRN Pediatric Palliative Care Research Network
MTB mycobacterium tuberculosis PCV pneumococcal virus
MTBC mycobacterium tuberculosis complex PDE principle of double effect
MUPS medically unexplained physical symptoms PEAT Palliative Education Assessment tool
N&V nausea and vomiting PedPalASCNet
NA noradrenaline PedsQL Pediatric Quality of Life Inventory
NaCL sodium chloride PEG percutaneous endoscopic gastrostomy
NASPGHAN The North American Society for Pediatric PG pyoderma gangrenosum
Gastroenterology, Hepatology, and Nutrition PHQ Patient Health Questionnaire (modified for
NASW National Association of Social Workers adolescents)
NBM nil by mouth PHQ-​9 Patient Health Questionnaire
NCP National Consensus Project PHRs personal health records
NE norepinephrine PHS public health strategy
NEC necrotizing enterocolitis PICC peripherally inserted central catheter
NeuPSIG Special Interest Group on Neuropathic Pain PICU paediatric intensive care unit
NF-​kB nuclear factor-​kB PICU psychiatric intensive care unit
NG nasal-​gastronomy/​nasogastric PJP pneumocystis jiroveci pneumonia
NGF nerve growth factor PKU phenylketonuria
NGOs nongovernmental organizations PMNS post-​malaria neurological syndrome
NGT nasogastric tube PMTCT prevention of mother to child transmission
NHPCO National Hospice and Palliative Care PNALD parenteral nutrition associated liver disease
Organization PNPC perinatal palliative care
NICU neonatal intensive care unit PO by mouth
NIPPV noninvasive positive pressure ventilation POM prescription only medication
NK1 and NK2 neurokinin receptors POMC proopiomelanocortin
NMDA N-​methyl-​D-​aspartate PONV post-​operative nausea and vomiting
NNH numbers needed to harm PORTAGE Pediatric Oncology Roundtable to Transform
NNT numbers needed to treat Access to Global Essentials
NO nitrous oxide PPC paediatric palliative care
NoRE no published evidence but has clinical PPE personal protective equipment
consensus PPI proton-​pump inhibitor
NP neuropathic pain PQRST P = precipitating or relieving factors,
N-​PASS the neonatal pain, agitation, and sedation scale Q = quality, R = radiation, S = severity,
NPCRC National Palliative Care Research Center T= timing
NPY neuropeptide Y PR/​F Propofol-​Remifantanil
NQF National Quality Forum PRN pro re nata: as needed
NRM nucleus raphe magnus PROM patient reported outcome measures
NRU Nutritional Rehabilitation Unit PST palliative sedation therapy
NSAID non-​steroidal anti-​inflammatory drug PTSD post-​traumatic stress disorder
NTDs neglected tropical diseases PUVA psoralen ultraviolet A
OCT1 ondansetron QA quality assurance
ODT oral dissolving tablet QAM every morning
xxii Abbreviations

QHS every night at bedtime STORCH syphilis, toxoplasmosis, rubella,

QI quality improvement cytomegalovirus, and herpesvirus
QID four times a day SVCO superior vena cava obstruction syndrome
QIDA Quality Improvement Data Aggregator vs4` TAD transient acantholytic dermatosis
QOL quality of life TAH total artificial heart
QoLA Care Quality of Life and Advanced Care TB tuberculosis
QST quantitative sensory testing TBAN tuberculosis acute neurology
QT qualification test interval seen in an TBI traumatic brain injury
electrocardiogram TBM tuberculosis meningitis
RASS Richmond Agitation–​Sedation Scale TBSA total body surface area
RCPCH Royal College of Paediatrics and Child Health TCA tricyclic antidepressant
RCT randomized controlled trial TCH Tygerberg Children’s Hospital
RDEB recessive dystrophic epidermolysis bullosa TDS to be taken three times daily
RHD rheumatic heart disease TEN toxic epidermal necrolysis
RIF resistance to rifampin TENS transcutaneous electrical nerve stimulation
RP relative potency TfFSL Together for Short Lives
RUTF ready-​to-​use therapeutic food THC tetrahydrocannabinol
Rx medical prescription TIME Toolkit of Instruments to Measure
SAM severe acute malnutrition End-​of-​Life Care
SBI severe bacterial illnesses TIPS transjugular intrahepatic portosystemic shunt
SBS short-​bowel syndrome/​State Behavioral Scale TNF tumour necrosis factor
SC subcutaneous TORCH toxoplasmosis, rubella cytomegalovirus, herpes
SCAR spectrum of severe cutaneous reactions simplex, and HIV
SDGs sustainable development goals TPN parenteral feeds
SENCO Special Educational Needs Coordinator TQS to be taken four times daily
SEPC self-​efficacy in palliative care scale TRPV1 transient potential vanilloid receptor
SHS serious health-​related suffering TTS tarsal tunnel syndrome
SMA spinal muscular atrophy UHC universal health coverage
SNI severe neurological impairment UMN upper motor neuron
SNRI serotonin and noradrenaline reuptake UN United Nations
inhibitors UNICEF United Nations International Children’s
SNRIs serotonin noradrenaline reuptake inhibitors Emergency Fund
SPC summary of product characteristics USBS ultra-​short bowel syndrome
SPIKES Set up the conversation, assess the patient’s UTI urinary tract infection
Perceptions, and obtain the patient’s Invitation, UVA ultraviolet A
all before providing information and UVB ultraviolet B
Knowledge. As information is transmitted, VAD ventricular assist devices
emotions are addressed with Empathy, and VFFS video-​fluoroscopic feeding study
next Steps are planned for future care and VLE virtual learning environment
conversations VP ventriculoperitoneal
SPPCS specialized paediatric palliative care VPS ventriculoperitoneal shunt
services VR virtual reality
SQ subcutaneous WDR wide dynamic range
SRE strong research evidence WFI (sterile) water for injection
SRQ self-​reporting questionnaire WHA World Health Assembly
SSPE subacute sclerosing panencephalitis WHO World Health Organization
SSRI selective serotonin reuptake inhibitors WHPCA Worldwide Palliative Care Alliance
STAR*D Sequenced Treatment Alternatives to Relieve WRE some weak research evidence
Depression XDR-​TB extremely drug-​resistant tuberculosis
SECTION I
Foundations of care

1. History and epidemiology 3 4. Decision-​making with children, young people,

Lorna Fraser, Stephen Connor, and Joan Marston and parents 36
Myra Bluebond-​Langner and Richard Langner
2. Communication 17
Jennifer Mack and Bryan Sisk 5. Culture, spirituality, religion, and ritual 44
Erica Brown, Mary Ann Muckaden, and Nokuzola Mndende
3. ‘Children are not small adults’—​the distinctiveness
of ethics in children 25
Robert Macauley and Richard Hain
1
History and epidemiology
Lorna Fraser, Stephen Connor, and Joan Marston
Introduction
Children’s palliative care (CPC) has been developing at varying
rates worldwide over the last three to four decades. The effects of
both the epidemiological transition and the impact of major public
health intervention programmes such as the millennium develop-
ment goals, and the sustainable development goals have resulted in
a different picture of childhood morbidity and morbidity than was
evident 30 years ago.
In this chapter, we will introduce some of the key definitions in
CPC and describe the epidemiological and health services research
to date, highlighting the importance of population-​based data to
help plan and develop services. We use examples from both the
highly developed countries, where data is more readily available,
and the low-​and middle-​income countries (LMIC) where pos-
sible. This chapter concludes with some examples of methods to use
available data to estimate the number of children with life-​limiting
(LLC) or life-​threatening conditions (LTC) in your city, region, or
country in order to plan, develop, and deliver palliative care to these
children.
Definitions
The key definitions for this chapter are listed here.
Child
The UN Convention on the Rights of the Child defines a child
as a person under the age of 18 years. From an epidemiological
perspective, many data sets count children up until the age of
19. The ‘paediatric cut off ’ at which stage children are transi-
tioned to adult services varies greatly between countries and
programmes.
Neonate
Those in the first 28 days of life.
Infant
Those less than 1 year of age.
Adolescent
Those children aged 10–​19 years. Further divided into three stages:
• Early adolescence (age 10–​14 years)
• Middle adolescence (age 15–​17 years)
• Late adolescence (age 18–​19 years)
The World Health Organization (WHO) definition
of CPC
Palliative care for children represents a special, albeit closely related
field to adult palliative care. WHO’s definition of palliative care ap-
propriate for children and their families is as follows; the principles
apply to other paediatric chronic disorders (1):
• ‘Palliative care for children is the active total care of the child’s
body, mind, and spirit, and also involves giving support to the
family.
• It begins when illness is diagnosed, and continues regardless of
whether or not a child receives treatment directed at the disease.
• Health providers must evaluate and alleviate a child’s physical, psy-
chological, and social distress.
• Effective palliative care requires a broad multidisciplinary approach
that includes the family and makes use of available community
resources; it can be successfully implemented even if resources are
limited.
• It can be provided in tertiary care facilities, in community health
centres, and even in children’s own homes’ (1).
The International Association of Hospice and Palliative Care
(IAHPC) has recently revised the WHO definition of palliative care
through a global consensus process involving members across the
world and has proposed a new combined adult and paediatric def-
inition as follows.
New IAHPC palliative care definition (2018)
Palliative care is the active holistic care of individuals across all ages
with serious health-​related suffering due to severe illness, and espe-
cially of those near the end of life. It aims to improve the quality of
life of patients, their families, and their caregivers (2).
4 SECTION I Foundations of care
Palliative care
• ‘Palliative care includes, prevention, early identification, compre-
hensive assessment, and management of physical issues, including
pain and other distressing symptoms, psychological distress, spir-
itual distress, and social needs. Whenever possible, these interven-
tions must be evidence based.
• Provides support to help patients live as fully as possible until death
by facilitating effective communication, helping them and their
families determine goals of care.
• Is applicable throughout the course of an illness, according to the
patient’s needs.
• Is provided in conjunction with disease modifying therapies when-
ever needed.
• May positively influence the course of illness.
• Intends neither to hasten nor postpone death, affirms life, and
recognizes dying as a natural process.
• Provides support to the family and the caregivers during the pa-
tients’ illness, and in their own bereavement.
• Is delivered recognizing and respecting the cultural values and be-
liefs of the patient and the family.
• Is applicable throughout all health-​care settings (place of residence
and institutions) and in all levels (primary to tertiary).
• Can be provided by professionals with basic palliative care training.
• Requires specialist palliative care with a multi-​professional team for
referral of complex cases’ (2, pp. 15–16).
This definition introduces two new terms into the palliative care
arena, namely health-​related suffering and serious illness that are
defined in the next section.
Health-​related suffering
Suffering is health-​related when it is associated with illness or in-
jury of any kind. Health-​related suffering is serious when it cannot
be relieved without medical intervention and when it compromises
physical, social, and/​or emotional functioning.
Serious illness
Any acute or chronic illness and/​or condition that causes significant
impairment, and may lead to long-​term impairment, disability, and/​
or death.
End-​of-​life care
An important component of palliative care is the care in the final
weeks, days, and hours of life. Usually started from that point at
which it is recognized that the child’s condition is in a state of pro-
gressive decline.
Terminal care
Care provided when the patient/​child is actively dying usually de-
fined as the last 24–​48 hours of life.
Bereavement care
Bereavement care or grief counselling can be provided either be-
fore the child has died (addressing the experience of loss due to the
diagnosis or anticipatory grief) or after the child’s death. The com-
ponents, quality, and duration of bereavement care services appear
to vary substantially across different care settings.
Hospice
Derived from the Latin word, Hospitium, meaning guesthouse,
the term can be traced back to medieval times when it referred
to a place of shelter and rest for weary or ill travellers on a long
journey. The name was first applied to specialized care for dying
patients in 1967 by Dame Cicely Saunders on the founding of the
first modern hospice in London. In the USA, the definition of hos-
pice care was driven by medical insurance providers and restricted
to care provided in the last 6 months of life. The word hospice is
now more generally used and often synonymously with the term
palliative care and no longer limited to a specific place of care but
rather to a philosophy of care. The association of the word hospice
with dying however has been a major obstacle to acceptance by
patients and families to the broader tenants of palliative care.
Life-​limiting condition
LLCs are those for which there is no reasonable hope of cure and
from which children or young people will die.
Life-​threatening condition
LTCs are those for which curative treatment may be feasible but can
fail, such as cancer.
Categories of LLC and LTC
There are nearly 400 individual diagnoses that would be classified
as life-​limiting or life-​threatening in children. The Association for
Children with life-​threatening or Terminal Conditions (ACT) since
renamed Together for Short Lives (TfSL) came up with a categor-
ization system grouping these conditions into four categories based
largely on disease trajectories and outcomes (see Table 1.1) (3).
A disease trajectory refers to the change in health status over time.
Many factors determine the trajectory of a disease including the
pathophysiology of the disease, the patient’s general state of health
beforehand, treatment received and resources available, complica-
tions experienced, and also factors beyond anyone’s control. Whilst
disease trajectories give an idea as to the child’s and families’ journeys
ahead, prognostication is difficult and needs to be individualized.
Examples of disease trajectories in the ACT
(TfSL) categories
Category 1: LTCs for which curative treatment may be
feasible but can fail
In this example (see Figure 1.1) a previously well child suddenly
takes ill but is diagnosed timeously with acute lymphoblastic leu-
kaemia (ALL) and started on treatment. The child’s state of health
oscillates between well and unwell during the course of chemo-
therapy and its complications, but induction therapy is successful.
Going forward there are three possible scenarios:
A. The child continues to respond well to treatment and goes into
lifelong remission (best case scenario).
B. The child’s (after achieving remission, relapses) treatment is
reinitiated but no bone marrow donor is found, and the child
dies from his disease.
C. Sudden death occurs on treatment from neutropaenic sepsis or
if treatment is abandoned (resource poor settings).
 CHAPTER 1 History and epidemiology 5

Table 1.1 Four groups of children with life-​limiting and life-​threatening conditions

Category 1 LTC for which curative treatment may be feasible but can fail, where access to palliative care services may be necessary when treatment fails,
irrespective of the duration of that threat to life. On reaching long-​term remission or following successful curative treatment there is no longer a
need for palliative care services.
Examples Cancer, organ failures of heart, liver, kidney, transplant and children on long-​term ventilation.
Category 2 Conditions when premature death is inevitable; these may involve long periods of intensive disease-​directed treatment aimed at prolonging life
and allowing participation in normal activities. Children and young people in this category may be significantly disabled but have long periods of
relatively good health.
Examples Cystic fibrosis, Duchenne muscular dystrophy and SMA Type 1 on ventilation. *HIV on HAART (author’s addition).
Category 3 Progressive conditions without curative treatment options, where treatment is exclusively palliative and may commonly extend over many years.
Examples Batten disease, mucopolysaccharidoses and other severe metabolic conditions, SMA type 1 without ventilation, HIV no HAART.
Category 4 Irreversible but non-​progressive conditions causing severe disability leading to susceptibility to health complications and likelihood of premature
death. Palliative care may be required at any stage and there may be unpredictable and periodic episodes of care.
Examples Severe cerebral palsy, complex disabilities such as following brain or spinal cord injury. Congenital infections (STORCH and Zika
virus infection) spina bifida and TB spine, (author’s addition).

Adapted with permission from A Guide to Children’s Palliative Care: Supporting Babies, Children and Young People with Life-​Limiting and Life-​Threatening Conditions
and their Families, Fourth edition (2018). England, UK: Together for Short Lives. https://​www.togetherforshortlives.org.uk/​wp-​content/​uploads/​2018/​03/​
TfSL-​A-​Guide-​to-​Children%E2%80%99s-​Palliative-​Care-​Fourth-​Edition-​5.pdf.

A palliative care approach would generally be provided in an inte-
grated manner by the palliative care trained treating oncologists,
with assistance from palliative care teams, for pain management
and psycho-​social support where needed, for most patients fol-
lowing trajectory A. The child would be discharged from palliative
care services on remission but may be re-​referred if he/she relapsed
(trajectory B). Given that the chances of dying would be more likely
with a relapse especially if there were no bone marrow donors or
second line treatment failed, referral to a specialist palliative care
team might be warranted and planned for. With sudden death from
a complication (trajectory C), there may not be time for the child to
be referred to palliative care or the palliative care team may meet the
child (in multi-​organ failure from septic shock) in an intensive care
setting. In resource poor settings the family who communicates
their need to go home and forgo treatment may also be referred to
palliative care (if available) at this point.
Other examples of the Association for Children with Life
threatening or Terminal Conditions (ACT I) conditions include
organ failures where transplant may or may not be available. It may
be argued that given the complexity of post-​transplant care that
many of these children would not necessarily be discharged from
palliative care and could be seen as ACT Class II patients.
Category 2: Conditions where premature death is inevitable
These may involve long periods of intensive disease-​directed treat-
ment aimed at prolonging life and allowing participation in normal
activities. Children and young people in this category may be sig-
nificantly disabled but have long periods of relatively good health.
Others in this group may need palliative care from an early stage.
In this example, a child presents with a severe life-​threatening op-
portunistic disease, during which human immunodeficiency virus
(HIV) is diagnosed and treatment instituted. The child recovers well
and has a number of years of good quality of life on highly active
antiretroviral therapy (HAART). Treatment compliance becomes
problematic when guardianship is changed, and the child starts to
experience treatment failure as resistance develops. A number of
severe opportunistic infections including multi-​drug resistant tu-
berculosis (TB) occur from which the child ultimately succumbs.
Other examples include cystic fibrosis, Duchenne muscular dys-
trophy, and spinal muscular atrophy (SMA) Type 1 on ventilation.
In resource-​poor settings where treatment is not available, the dis-
ease trajectory becomes more like that of an ACT 3 category disease
(Figure 1.2).

Complications
Healthy

Remission
Healthy A Treatment
Relapse Diagnosis

Diagnosis
Treatment C Sudden B
death

Death Death
Time Time

Figure 1.1 Act Category 1 disease trajectory for acute lymphoblastic Figure 1.2 ACT Category II disease: Child with HIV on HAART who
leukaemia (ALL) develops treatment resistance
6 SECTION I Foundations of care
Healthy
Diagnosis
Death
Time
Figure 1.3 Act Category 3: Child with inborn error of metabolism for
which there is no available therapy
Category 3: Progressive conditions without curative treatment
options, where treatment is exclusively palliative and may
commonly extend over many years
In this example, a child presents in the first month of life with failure
to thrive and seizures (Figure 1.3). A diagnosis of a mitochondrial
disorder is made for which there are no treatment options. The child
continues to progress over time and receives palliative care from the
time of diagnosis until death a few months later.
Other examples include Batten disease, mucopolysaccharidoses
and other severe metabolic conditions, SMA type 1 without ventila-
tion, and HIV with no HAART available.
Category 4: Irreversible but non-​progressive conditions
causing severe disability leading to susceptibility to health
complications and likelihood of premature death
In this example, a child with severe cerebral palsy (CP) starts to de-
teriorate during adolescence (Figure 1.4). She experiences recur-
rent chest infections that are aggravated by an increasing windswept
chest deformity and scoliosis that progresses as she experiences a
pubertal growth spurt.
Other examples include complex disabilities such as following brain
or spinal cord injury, congenital infections ((syphilis, toxoplasmosis,
Healthy
Diagnosis
Complications
Demands of adolescence
Death
Time
Figure 1.4 Act class 4: Child with cerebral palsy
rubella, cytomegalovirus, and herpesvirus (STORCH)) and Zika
virus infection), spina bifida, and TB of the spine.
History
The history of children’s hospice and palliative care is a rich tapestry
of visionary individuals, pioneering organizations, and social trends
that have influenced the development of a field that aims to relieve
the suffering of neonates, children, and adolescents with LLCs.
Within this tapestry are patients and families, health and other pro-
fessionals, volunteers, policy-​makers, advocates, researchers, edu-
cators, donors, and many others.
The work and writings of Dame Cicely Saunders, the ‘Founder’
of the modern hospice movement and St Christopher’s Hospice in
1967, influenced many CPC pioneers. In addition, the influential
book On Death and Dying written by Dr Elisabeth Kübler-​Ross
in 1969 was described by Dr Allan Kellehear in his ‘Foreword’ to
the fortieth anniversary edition as ‘. . . one of the most important
humanitarian works on the care of the dying written in the Western
world . . .’ (4).
This short timeline (Figure 1.5) is simply representative of im-
portant milestones, seeks to honour everyone’s contribution, but
for the sake of space only includes those key events, organizations,
and sentinel works whose influence has led to international or re-
gional development. To be more inclusive would need a textbook
of its own. A more comprehensive history may be found on the
website of the International Children’s Palliative Care Network
www.icpcn.org.
Epidemiology
The need for palliative care in a given population is often esti-
mated from mortality and prevalence data of particular diseases.
Whilst mortality data is often easier to find, it is more a measure
of the need for end-​of-​life or terminal care than prevalence data
that helps to determine the burden of a particular disease that may
benefit from a palliative care approach from the time of diagnosis
(Figure 1.6).
Definitions
Counting the absolute number of children with a specific condition
or group of conditions may give the information that is required
to develop services or plan budgets. However, in order to make
any comparisons, between countries or age groups for example, a
measure of disease frequency that takes account of the underlying
population at risk with or without a time component is necessary.
Incidence, incidence rate, or prevalence are the most common
measures of frequency used.
Incidence
Incidence is a measure of new cases that occurred in a specific time
period. There are several measures of incidence that can be calcu-
lated, i.e. cumulative incidence/​risk or incidence rate.
 CHAPTER 1 History and epidemiology 7

KEY EVENTS: DEVELOPED WORLD KEY EVENTS: DEVELOPING WORLD

1975
Home care for the dying child (Minnesota, USA) 1976

EDMARC Hospice for Children (Virginia, USA) 1978

The Private Worlds of Dying Children; Book (USA)
CLIC(Cancer and Leukaemia in children) UK
1980
Neonatal Hospice, Denver Children’s Hospital USA
Helen House Children’s Hospice opens (Oxford, UK) 1982
Children’s Hospice International established and first 1983
CHI Conference (San Francisco, USA), Hospital based PPC service in St Mary’s hospital, New York
College course on Children and Dying (USA) 1984
Very Special Kids (Malvern, Australia) 1985 1985 Prager-Bild Foundation PPC Programme, Buenos Aires, Argentina

Rainbows Trust (UK) 1986

Listen-my child has a lot of living to do-book (UK) 1987
Great Ormond Street Hospital Programme (London, UK)
ACT-The Association for Children with Terminal Illnesses (UK) 1988
UN Convention on the Rights of the Child, UN (Geneva, Switzerland) 1989

1990 Hospital of San Jose Paediatric Pain and PPC Clinic, Costa Rica

IWK Health Centre PPC Programme Canada 1994 1994 Warsaw Hospice for Children and Belarusian Children’s Hospice open

OSI: Project on Death in America (USA) 1995 1995 Merimna Foundation, Athens, Greece
IWK Health Centre PPC Program, Halifax, Canada
Canuck Place Children’s Hospice opened (Vancouver, Canada)

Diana Princess of Wales Memorial Fund (DPOWMF) 1997 1997 Cotlands Children’s Hospice (HIV/AIDS), Johannesburg,
WHO Definition of Palliative Care for Children and Guidelines developed for Cancer pain + PPC 1998 1998 St Nicholas ( now Sunflower) Children’s Hospice opens (Bloemfontein, SA)
CHiPPs Conclave
Bear Cottage opened in Sydney, Australia
Fondazione Maruzza Rome, Italy 1999
Salzburg PPC Seminars (Austria)

International Palliative Care Initiative (Open Society institute) 2000

Postgrade Diploma in PPC (Cardiff University) 2001 2001 Tata Memorial Centre PPC Programme, Mumbai, India
First Cardiff Conference in PPC and ELNEC course starts 2002 2002 Umodzi, PPC hospital team at QECH, (Blantyre) Malawi
First Chair in Paediatric Palliative Medicine (Germany)

George Mark Children’s House, California, USA 2004

ICPCN first Statement on PC for Children 2005 2005 Wishing Well foundation, Bangkok, Thailand
Bundesverband Kinderhospiz, Germany ICPCN established
1st Edition Oxford Textbook of Palliative Care for Children 2006 2006 Hospice Casa Sperantei PPC Team, Brasov, Romania
Rachel house Children’s Hospice, Jakarta, Indonesia
Butterfly House, China
1st South African hospital PPC service: Bigshoes-Baragwanath
hospital (Soweto)
2007 PPC Fellowship, Hyderabad, India
2008 Children’s Palliative Care in Africa Textbook
2009 Beacon Centre Project: SA, Tanzania and Uganda (DPOWMF)

EPEC Paediatric course begins (USA)

True Colours Chair for Palliative Care for Children and 2010 5 year project Developing PPC in In Malawi and Maharashtra, India
Young People, London, UK

ICPCN e-learning modules start
WHO Guidelines on Persisting Pain in Children with Medical Illnesses
Bayt Abdullah Children’s Hospice, Kuwait
2011 2011 1st PPC unit opens in Garissa, Kenya
2012 2012 Ukraine’s first PPC Programme in Ivano-Frankivsk
2013 PPC Diploma for Africa, Mildmay, Uganda
Three country PPC needs estimate (Uganda, Kenya and SA):
ICPCN + UNICEF and the Rocking Horse Project Swaziland

WHA 67.19 resolution on Palliative Care (Geneva, Switzerland) 2014 2014 ICPCN Hats on for CPC Global Awareness Day
1st ICPCN conference in Mumbai, India Declaration of Mumbai
on Euthanasia and PAD for children

Kiran Stordalen and Horst Rechelbacher Pediatric Pain, Palliative and 2015
Integrative Medicine Clinic: Children’s Minnesota
Religions of the World Charter for Children’s Palliative Care (Vatican City, Italy)

2016 Commitment of Buenos Aires (2nd ICPCN conference)

Lancet Commission report on Palliative Care and UHC 2017 2017 Firefly World Children’s Hospice, Tbilisi, Georgia
WHO Guide to Integrating Palliative Care into Paediatrics 2018 2018 Association for Children’s Palliative for Eastern Europe and Central Asia

2020

Figure 1.5 The history of children’s palliative care—​a timeline of key events
8 SECTION I Foundations of care

For example, in 2014 there were 5,553 children aged 0–​19 years
in Scotland with an LLC. The total population of children aged
MORTALITY TERMINAL CARE NEED
0–​19 years in Scotland in 2014 was estimated to be 1,167,599.
Therefore, the prevalence is calculated as shown here:

5553
prevalence = = 47.6 per 10000
1167599
MORBIDITY PALLIATIVE CARE NEED
Prevalence From the time of diagnosis Data quality
Data
Sometimes good quality data is available to calculate incidence rate,
e.g. many developed countries have high-​quality cancer registration
systems that allow them to monitor the incidence rate and survival
Figure 1.6 The relationship between mortality and prevalence in of cancer in their population over time. However often that data is
determining the palliative care need not available therefore it is important to understand the relationship
between incidence and prevalence if estimating methods are to be
used (Figure 1.7).
Cumulative incidence or risk is calculated in the following An example of the relationship between incidence and preva-
examples. lence is further illustrated in the following diagram that describes
changes over time in the HIV/​ AIDS epidemic following the
number of new cases with the disease in rollout of HAART and prevention of mother to child transmission
a specified time period (PMTCT). The incidence of paediatric HIV has decreased thanks
cumulative incidence =
total population during this timee period to PMTCT, whilst prevalence has been influenced by an increased
survival of children on HAART, fewer deaths, and the transitioning
Incidence rate of survivors on to adult services (Figure 1.8).
Data to calculate incidence rates or prevalence figures accurately
number of new cases with the disease in is not always available especially in LMIC. The section on preva-
specified time period lence later in this chapter will outline some suggested methodolo-
incidence rate =
me period
total person time at risk during this tim gies, which may assist depending upon the availability of data and
the quality of those data. It should also be noted that even in the
For example, there were 562 new cases of central nervous system developed world, data can be of variable quality.
tumours in children and young people in the Yorkshire region of A recent study in the UK, where data quality should be high,
the UK aged 0–​29 years between 1990–​2005. The total time at risk showed that using death certificate data alone may underestimate
for the population was 21.53 million years. Therefore, the incidence the number of children who died with an LLC or LTC by 20% (5).
rate is calculated in the following:
Medical conditions which may require palliative care
562 The LLCs and LTCs that can occur in infants, children, adolescents,
incidence rate = = 26.1 per 1 000 000 person years and young adults are numerous and diverse. For some diagnoses,
21.53 million
these may or may not be life-​limiting depending on the availability
Prevalence
Incidence
Prevalence is the proportion of persons in a population who have
a particular disease at a specified point in time or over a specified
period of time. Prevalence differs from incidence in that prevalence
includes all cases, both new and pre-​existing, in the population at
the specified time, whereas incidence is limited to new cases only.
Prevalence is not a rate as there is no measure of time included in
the calculation.
Point prevalence refers to the prevalence measured at a particular
point in time. It is the proportion of persons with a particular dis- Prevalence
ease or attribute on a particular date.

number of people with a disease at

a specific point in timee
prevalence = Deaths
population at risk at the same point in time Cures
Transitions
Period prevalence refers to prevalence measured over an interval Figure 1.7 Relationship between incidence and prevalence
of time. It is the proportion of persons with a particular disease or Adapted with permission from Celentano D, and Szklo M. (2018). Gordis Epidemiology,
attribute at any time during the interval. Sixth Edition, p.53. Philadelphia, USA: Elsevier. Copyright © 2019 by Elsevier Inc.
 CHAPTER 1 History and epidemiology 9

New HIV infections

among children
Children living with
98,000
HIV age 15
2010 280,000 2,200,000
200,000
AIDS related deaths

Children living with

110,000
HIV age 15
2015 190,000 1,900,000
120,000 AIDS related deaths

Children living with

120,000
HIV age 15
2018 160,000 1,700,000
100,000 AIDS related deaths

Children living with

133,000
2020 HIV age 15
120,000 1,600,000
projected 76,000 AIDS related deaths

Figure 1.8 Changing incidence and prevalence of paediatric HIV/​AIDS over time
Reproduced with permission from Mahy M. (2017). Latest Estimates for Pediatric and Adolescent HIV Prevalence and Coverage. Geneva, Switzerland: UNAIDS. http://regist2.
virology-education.com/2017/9HIVped/01_Mahy.pdf.

of healthcare resources (such as cardiac surgery and intensive care)
and the use of medical technologies.
In the UK, an analysis of a list of diagnoses from children who
were under the care of five children’s hospices and one hospital-​
based specialist paediatric palliative care service identified 376
different diagnoses that were considered to be life-​limiting or life-​
threatening (6). Due to the source data for this study coming only
from the UK, this list does not contain some of the diagnoses, which
are commoner in LMIC such as communicable diseases, extensive
burns, and sequelae of severe accidents.
Patients who are enrolled in paediatric palliative care programmes
have a wide range of conditions. In a case series of patients from
eight paediatric palliative care programmes in Canada, patients had
diseases of the nervous system (39.1%), congenital conditions or
illnesses originating during the perinatal period (22.1%), malignan-
cies (22.1%), and other illnesses (16.7%) (7).
A single centre study in the UK showed that over a 20-​year
period there was an increasing proportion of referrals to a
children’s hospice for children in ACT category 4. In the period
2004–​08, 13.1% referrals were for children in ACT category 1
(mostly cancer), 12.8% category 2, 38.7% category 3 (mostly
neuromuscular conditions), and 38.5% category 4 (majority com-
plex cerebral palsy) (8).
In comparison to the Paedspal programme in Cape Town, South
Africa had nearly half its patients in ACT category 3, 24% in ACT
category 2 (including HIV), 20% in ACT category 4, and only 7% in
ACT category 1 (including cancer) (Figure 1.9) (9).
It is important to realize that factors (other than prevalence
of disease) such as referral patterns, that may be influenced by
the primary providers concept of palliative care, also affect the
proportionate percentage of children seen in the different ACT
categories.

UK distribution of conditions RSA distribution of conditions

ACT I:
ACT I: 7%
ACT IV:
13%
20%
ACT IV: ACT II: ACT II:
37% 12% 24%

ACT III: ACT III:

38% 49%

Figure 1.9 A comparison between ACT Category distribution in the UK and South Africa
Source: data from Meiring M. (2016). Paedspal First Annual Review 2006. Cape Town, South Africa.
10 SECTION I Foundations of care

Major diagnostic groups These data highlight the variation and therefore the importance
A joint publication of the WHO and the Worldwide Hospice Palliative of understanding the epidemiology of LLC and LTC of children
Care Alliance identified ten major diagnostic groups that needed CPC in the region or country that clinicians or service providers are
(10) and estimated minimum need for palliative care (Figure 1.10). working.
More recently, a Lancet Commission Report also identified sixteen Data on deaths in children
conditions where children need end-​of-​life care palliative care and
estimated the terminal care need at 2.4 million, mainly in LMIC (11). End-​of-​life care is a small but very important component of CPC.
These conditions included: All children who die should receive high-​quality end-​of-​life care ir-
respective of their underlying diagnosis but longer-​term palliative
1. HIV disease care should be available for those infants and children who have
2. Lung disease been diagnosed with an LLC or LTC who may survive for some
3. Degenerative central nervous system (CNS) disease time. In the UK it is estimated that approximately 50% of deaths in
4. Renal failure children (12) are for children with an LLC or LTC.
5. Malignant neoplasm
Trends in mortality rates
6. Tuberculosis
7. Congenital malformations
Through the millennium development goals and more recently the
sustainable development goals there has been a continuing reduc-
8. Malnutrition
tion in mortality rates in children worldwide with some countries
9. Liver disease
and regions improving at a faster rate than others (Figures 1.11
10. Musculoskeletal disorder
and 1.12) (13). However, despite this marked improvement there
11. Low birth weight—​prematurity—​birth trauma were still 6.6 million deaths in children (0–​14 years) worldwide in
12. Inflammatory CNS disease 2016. The vast majority of those deaths (5.6 million) occurred in
13. Leukaemia children aged under 5 years with 75% of those deaths (4.2 million)
14. Injury occurring in the first year of life (13).
15. Non-​ischaemic heart disease The mortality rates and absolute numbers of child deaths vary
16. Haemorrhagic fevers between countries and regions of the world. The under-​5 mortality

Neurologicial conditions* Kidney diseases

2.31% 2.25%

Cancer Cirrhosis of the liver1.06%

5.69%
Congenital anomalies*
Endocrine, blood, immune disorders 25.06%
5.85%

Cardiovascular disease
6.18%

HIV/AIDS 10.23%

Meningitis 12.62%

Protein energy malnutrition Neonatal conditions*

14.12% 14.64%

* see original publication for list of excluded conditions

N=1,170,011

Figure 1.10 Distribution of children in need of palliative care at the end of life by disease groups
Adapted with permission from Connor S, and Bermedo MCS. (Eds.) (2014). Global atlas of palliative care at the end-​of-​life. Worldwide Hospice Palliative Care Alliance and
World Health Organization. http://​www.thewhpca.org/​resources/​global-​atlas-​on-​end-​of-​life-​care.
 CHAPTER 1 History and epidemiology 11

Under five mortality rate by Sustainable Development Goal region

200
180

Deaths per 1000 live births

160
140
120
100
80
60
40
20
0
1990 1995 2000 2005 2010 2015 2016
Northern America and Europe Latin America and the Caribbean Central Asia and Southern Asia
Eastern Asia and South-Eastern Asia Western Asia and Northern Africa SubSaharan africa
Oceania

Figure 1.11 Under-​5 mortality rates 1990–​2016

Source: data from United Nations Inter-​agency Group for Child Mortality Estimation (UN IGME) (2018). Levels and Trends in Child Mortality: Report 2018, Estimates developed by
the UN Inter-​agency Group for Child Mortality Estimation. New York: United Nations Children’s Fund.

in 2016 ranged from a low of 2 per 1,000 (Slovenia) to 133 per 1,000
(Somalia) (13). It is important to highlight that more than 98% of
all child deaths in 2016 occurred in less developed countries of
the world. Data is unfortunately not available for the whole of the
worldwide population and many of these data are estimates.
Causes of death in children
The leading causes of deaths in children vary both between LMIC
and High Income Countries (HICs) but also between countries
within these broad income groups. The leading causes of death
worldwide in children <5 years are prematurity, pneumonia, diar-
rhoea, and congenital anomalies, where in the 5–​14 years age
groups about 50% of deaths are from perinatal, nutritional, and in-
fectious diseases, 25% of deaths are from injuries (drowning and
road deaths the most common), and approximately 25% from non-​
communicable diseases (13).
Figure 1.13 highlights that although the percentage of deaths from
congenital anomalies in children under 5 is rising worldwide there
is large variation between regions in the percentage of deaths that
they are responsible for. More than 20% of deaths in this age group in
Europe and the Americas are caused by congenital anomalies, but less
than 6% of deaths in the African region in this age group.
Age is an important differentiating factor in causes of death, for
example in England the commonest cause of death in infants is pre-
maturity and other perinatal causes (22.8%) and congenital mal-
formations (22.0%). In the 1–​4-​year age group, cancer (14.5%) and
diseases of the nervous system (13.8%) are the commonest causes. In
children aged 5–​9 years cancer (29.6%) and nervous system disorders

Mortality rates in children aged 5–14 years by Sustainable Development Goal regions
45
40
35
30
25
20
15
10
5
0
1990 2000 2010 2015 2016

Northern America and Europe Latin America and the Caribbean Central Asia and Southern Asia
Eastern Asia and South-Eastern Asia Western Asia and Northern Africa SubSaharan africa
Oceania

Figure 1.12 Mortality rates for children aged 5–​14 years 1990–​2016
Source: data from United Nations Inter-​agency Group for Child Mortality Estimation (UN IGME) (2018). Levels and Trends in Child Mortality: Report 2018, Estimates developed by
the UN Inter-​agency Group for Child Mortality Estimation. New York: United Nations Children’s Fund.
12 SECTION I Foundations of care
25
20
Percentage of deaths
15
10
0
2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015
Figure 1.13 Deaths in children <5 years from congenital abnormalities
Source: data from Connor S, and Bermedo, MCS. (Eds.) (2014). Global atlas of palliative care at the end-of-life. Worldwide Hospice Palliative Care Alliance and World Health
Organization. https://​www.who.int/​nmh/​Global_​Atlas_​of_​Palliative_​Care.pdf.
(12.4%) remain the largest causes of death, but by age 10–​14 years
cancer (24.2%) and external causes dominate (19.0%) (14).
In the USA most infant deaths are due to premature birth and
other perinatal, congenital, or chromosomal conditions, whereas
children and to an even greater extent adolescents and young adults
are likely to die from traumatic injury or violence or other external
causes. Although cancer is perhaps most closely linked to the his-
torical development of paediatric palliative and hospice care, it
never accounts for more than 18.3% of deaths in any age group (15).
In comparison, in South Africa in 2015, the commonest causes
of death in infants was respiratory and cardiovascular disease in
perinatal period (15.6%), followed by intestinal infectious disease
(9.5%), and respiratory infections (8.4%). In the 1–​14-​years-​olds
the infectious diseases predominate with 8.9% intestinal infections,
and 6.8% influenza and pneumonia, followed by 5% of children in
this age group dying of malnutrition (16).
Place of death
Place of death has been used in policy documentation as a measure
of quality of palliative or end-​of-​life care in developed countries
such as the UK. The assumption that everyone wants to die at home
has been contested in both children’s and adult’s palliative care in
recent years (17, 18). However, at a population level, if we are truly
able to offer choice then we should see a distribution between the
possible places of death; hospital, home, and hospice.
A recent national study showed that in the 20 years from 1993–​
2014 amongst children who died from cancer in England, those
dying at home remained static at approximately 40%; hospital
deaths decreased slightly from >50 to 45% and hospice deaths in-
creased from 6 to 13% (19).
There is variation between developed countries with some evi-
dence from the USA that more children dying with complex chronic
Africa Region
Region of the Americas
South-East Asia Region
European
Eastern Mediterranean Region
Western Pacific Region
conditions are dying at home (10.1% in 1989 rising to 18.2% in
2003) (20). Age has been shown in several studies to be associated
with dying at home with the infants less likely than older children to
die outside hospital (20, 21).
Palliative care input has been associated with more children dying
outside the hospital. A national study from England and Wales of
7,709 children who died after being discharged from paediatric inten-
sive care units showed that children who had palliative care recorded
at the time of discharge were eight times more likely to die in the com-
munity than children who were not referred to palliative care (22).
There is little published data available on place of death in chil-
dren in LMIC where resources are more limited.
Prevalence data
As noted, prevalence data can be a more accurate way to determine
the number of children needing palliative care throughout their dis-
ease trajectory. Mortality data tends to underestimate the need, es-
pecially in children who are more unpredictable when ill compared
to adults. However, prevalence data is more difficult to obtain and to
estimate in a population than mortality. The main source of preva-
lence data on health conditions comes from the Global Burden of
Disease Study coordinated through the Institute for Health Metrics
and Evaluation at the University of Washington.
Prevalence data for a given diagnosis is usually broken down into
a large number of sequelae that differentiate the manifestations of
the illness or condition. Many of the sequela for conditions needing
palliative care are not life limiting or threatening and thus should
not be counted in the need for palliative care. For instance, con-
genital anomalies manifesting with cleft lip or palate or hearing loss
would not require palliative care (23) (see Table 1.2).
In addition, staging of the diagnosis should be considered along
with severity of symptoms or disability. For example, ordinarily
Table 1.2 Modifications and adjustments to data used in CPC conditions

Condition Adjustments and assumptions

All cancers • All cancers included except non-​melanoma skin
• Symptom factor for childhood cancers is 80%
• Prevalence data from IHME
HIV/​AIDS • All categories except HIV pre-​AIDS asymptomatic
• Symptom factor 60%
• Prevalence data from UNAIDS
Progressive non-​malignant diseases • Symptom factor for all PNMD’s is 67%
Cardiovascular diseases • Prevalence data from IHME
• Sudden deaths removed (33%*) before application of the 67% symptom prevalence rule
• Rheumatic heart disease (RHD)
— Valvular disease due to RHD—​Use only 1.5%
• Other cardiovascular and circulatory diseases—​Use only 10%
• Excludes
— Acute myocarditis
— Atrial fibrillation and flutter
— Endocarditis
Cirrhosis of liver • Prevalence data from IHME
• Includes all causes/​sequel
Congenital anomalies • Prevalence data from IHME
• Includes
— Neural tube defect
— Congenital heart anomalies (50%#)
— Heart failure due to congenital heart anomalies
— Chromosomal unbalanced rearrangements
— Other congenital anomalies
• Excludes
— Cleft lip and palate
— Down’s syndrome
— Turner’s syndrome
— Klinefelter syndrome
— Hearing loss due to congenital anomalies
Endocrine, blood, and immune • Prevalence data from IHME
disorders • Excludes most anaemias except
— Sickle cell, Thalassemia, Fanconi’s, Aplastic
Meningitis • Based on WHO mortality data not prevalence
Kidney diseases • Prevalence data from IHME
• All causes
• Chronic kidney disease unspecified
— Anaemia due to unspecified or stage III CKD—​Use only 10%
Protein energy malnutrition • Based on WHO mortality data not prevalence
Neurological conditions • Based on WHO mortality data (higher than prevalence data)
• Includes
— Alzheimer’s and other dementias
— Parkinson’s and multiple sclerosis
— Other neurological conditions (Guillain-​Barré Syndrome)
• Excludes
— All epilepsy
— All migraine and headache
• All other neurological disorders
Neonatal conditions • Prevalence data from IHME
• Includes
— Most preterm birth complications for 0–​9-​year-​olds
— Impairment due to neonatal encephalopathy for 0–​9-​year-​olds—​birth asphyxia and birth trauma (75% of these are
removed due to WHO position that these can be resuscitated)
• Excludes
— 25% out of the 35% of pre-​term babies that die on the day of birth
— Retinopathy of prematurity due to preterm birth complications
— All sepsis and other infections of new born babies
• Other neonatal disorders
Tuberculosis (1.417% DR-​TB) • Based on WHO Stop TB mortality data, not prevalence
• Only includes children with MDR or XDR-​TB
— Calculation of 1.417% of mortality is based on the global proportion of TB prevalence that is drug resistant (170,000
drug resistant out of 12 million prevalence)

*The 33% figure is used to be consistent with the Atlas of Palliative Care. Sudden death from CVD is estimated to be one third of cases based on Centres for Disease Control reports.
#Many children with congenital heart anomalies die suddenly without symptom burden. The 50% figure is used to be consistent with assumptions used in the Atlas of Palliative Care.
£ WHO reports that for 35% of children that die, death occurs on the day of birth and do not consider most to need palliative care. We removed 25% of these cases but 10% were
retained to be consistent with the Global Atlas of Palliative Care Assumptions to account for children that we know will die shortly after birth.
Adapted with permission from Connor SR, Downing J, and Marston J. (2017). Estimating the global need for palliative care for children: A cross-​sectional analysis. J Pain Sympt Mant.
53(2):171–​7. https://​doi.org/​10.1016/​j.jpainsymman.2016.08.020.
14 SECTION I Foundations of care
stage one cancers would not require palliative intervention. In the
Global Atlas (21) pain was used as a surrogate for need for pallia-
tive care and pain incidence was estimated from the literature. The
Lancet Commission Report (19) also was able to estimate suffering
for each condition using a more extensive number of symptoms
including: pain, shortness of breath, fatigue, weakness, nausea and
vomiting, diarrhoea, dry mouth, itching, bleeding, constipation,
wounds, depressed mood, anxiety, confusion, and dementia.
Total number of children needing palliative care
The total number of children needing palliative care is unknown.
However, a global estimate has been published (23) that finds the
overall need at over 21 million children. This estimate was derived
from period prevalence data for 2010 from the Institute for Health
Metrics and Evaluation (IHME)’s Global Burden of Disease Study
for the eleven diagnostic groups in the Global Atlas (10).
This estimate was further broken down between those needing
a palliative care approach and those that could need specialized or
ongoing palliative care services. The specialized need was estimated
at 8 million children between 0–​19 years.
The methods used in this study were developed in a pilot project
for the United Nations Children’s Fund (UNICEF) that examined
the need for palliative care in three African countries (Kenya, South
Africa, and Zimbabwe). A ratio of number of children needing
specialized palliative care per 10,000 children in the population was
derived along with an estimate of the capability in the country to
deliver CPC. This allowed an ability to measure the gap in unmet
need for CPC. An article reporting the results for South Africa (24)
found that the need was over 800,000 including a specialized need
of over 304,000 or 151.92 per 10,000 children. Less than 5% of this
need was able to be met in the country.
Generally, need for CPC is higher in LMIC than in HICs. In HICs
data may be more readily available, e.g. a study done in the UK esti-
mated a prevalence of LLCs or LTCs in children aged 0–​19 years of
thirty-​two per 10,000 (25). This study used a variety of data sources
including hospitalization data and did not differentiate between gen-
eral and specialized need but is consistent with previous findings.
Low-​ vs high-​income need
The need for CPC is predominately in LMIC. Both the Global Atlas
(10) and the Lancet Commission Report (11) indicate that 98% of
the need for CPC is in LMIC. Rates of need by WHO region are
highest in the African region at 49% of the total CPC need due to
HIV disease, perinatal conditions, malnutrition, and general lack
of access to modern health facilities (Figures 1.11, 1.12, and 1.14).
High-​risk groups of the population
There are a number of controversial questions about who needs
CPC that are somewhat unresolved. These include questions about
CP, neonatal cases, non-​HIV infectious diseases, injury, and neuro-
logical conditions.
Many palliative care services in LMIC include care for children
with severe CP. It is difficult to find and understand prevalence data
for CP in LMIC as it is considered by statisticians as an imprecise
code. This term is meant to mean a neurological disorder caused by
a non-​progressive brain injury but many different conditions use
this code so it may lack precision. In addition, when a child dies
from CP it is usually coded to another cause such as heart failure,
sepsis, or some other condition.
Upper middle income
14.4%
High income
2.1%
Lower middle income
48.5%
Low income
35%
N = 1,170,011
Figure 1.14 Distribution of children in need of palliative care at the
end of life by World Bank country income groups
Adapted with permission from Connor S, and Bermedo MCS. (Eds.) (2014).
Global atlas of palliative care at the end-of-life. Worldwide Hospice Palliative Care
Alliance and World Health Organization. http://www.thewhpca.org/resources/
global-atlas-on-end-of-life-care.
The exclusion of children that die in the neonatal period can raise
ethical issues. In cases of birth trauma or asphyxia, many children
would have survived if the birth was attended by someone with
medical training that could administer resuscitation. However,
many of these children do survive but are significantly impaired and
would benefit from palliative care.
Traditionally palliative care has not been considered for non-​
HIV infectious disease however many of these illnesses are life
threatening and cause significant suffering such as with menin-
gitis and haemorrhagic fevers. Similarly, children that suffer major
trauma or burns need palliative care to relieve suffering whether
they manage to survive or die following their injuries. A variety of
neurological conditions, in some cases from injury, cause children
to have significant disabilities that may lead to early demise and
should also have access to palliative care.
Trends over time
Figures 1.11 and 1.12 have shown the reduction in child mor-
tality rates worldwide since 1990. This could be interpreted as a
decreasing need for end-​of-​life care in children but the prevalence
data from the UK has shown an increase in the numbers of chil-
dren who would benefit from palliative care services. This increase
in prevalence is thought to mainly be from an increase in survival
due to more aggressive management of complications such as in-
fections and the use of medical technologies such as enteral feeding
and home ventilation.
Data on referral to palliative care
There is a paucity of population-​based data on referrals to palliative
care services. A couple of studies from the UK show that for chil-
dren with cancer, children with central nervous system tumours are
more likely to be referred to a specialized paediatric palliative care
services (SPPCS) than children with haematological malignancies
(26). The distance from paediatric intensive care unit (PICU) and

the local hospice was significant for whether children were referred
to palliative care from PICUs in the UK (27). National data on re-
ferrals from the National Hospice and Palliative Care Organization
indicate that hospitals make the most referrals (45%) followed by
physicians (23%), nursing facilities (15%), self or family (10%), and
other (7%). Overall, an average of 76% of referrals are converted to
admissions for adults and children.
Stage of condition data
There is an acknowledgement that simply counting the number of
children, particularly using administrative or health datasets, may
not give enough detail on the severity of the condition or the child’s
needs. This is important if resources are restricted and services are
to be targeted at those with most need. The Children in Scotland
Requiring Palliative Care (ChiSP) study (21) used the patterns of
healthcare usage to categorize children with LLCs or LTCs as stable,
unstable, deteriorating, or dying (Figure 1.15).
Overall 80% of children were stable each year but this varied
greatly by age, with the infants less likely to be stable and more likely
to be unstable, deteriorating or dying. The 16–​25-​year-​olds were
also more likely to be unstable or dying than the 1–​15-​year-​olds.
Benefits of palliative care
A recent systematic review of the evidence on the benefits of SPPCS
concluded that, despite low-​quality studies, there was some evi-
dence that SPPCS enable improved quality of life for children young
people and their parents, improved symptom control, has an impact
on the place of care, and place of death (28). A UK study published
Dying
28 days
before
death
Deteriorating
Any emergency Discharge
ICU admissions from ICU
to hospital
Discharge to
Unstable normal place of
residence (and
no death within
Emergency inpatient 28 days)
admission lasting two
or more days
Stable
Figure 1.15 Four Stages of condition for children with a life-​limiting or
life-​threatening condition. Red arrows indicate worsening condition and
green arrows indicate improving condition
Reproduced with permission from Fraser L. et al. (2015). Children in Scotland requiring
Palliative Care: Identifying numbers and needs (The ChiSP Study), p. 14. Copyright ©
2015 University of York.
CHAPTER 1 History and epidemiology 15
since this review showed that children who had been discharged
from paediatric intensive care units and subsequently died, were
eight times more likely to die at home or hospice than in the hos-
pital if they had palliative care input at the point of discharge (22).
There is a need for high-​quality studies to assess the impact of pal-
liative care on children and their families using appropriate and val-
idated outcome measures.
Using these data to estimate the need
for CPC services
To estimate the need for CPC depends on the level you are working
on; national, regional, or local. It is best to obtain both mortality
and prevalence data if available. National cause of death data for
all countries is available from WHO’s Global Health Estimates at:
http://​www.who.int/​healthinfo/​global_​burden_​disease/​estimates/​
en/​index1.html
For most LMIC these data are estimates based on the Global
Burden of Disease (GBD) studies but adjusted by WHO using add-
itional population models. It is also advisable to obtain data from
the country’s ministry of health, if available. It is not uncommon for
the WHO and local data to differ, but it is important to be able to
explain any differences from local data.
If you are trying to estimate CPC need for your local area, you
should also try to obtain any local data from local hospitals, health
departments, or other authorities. Local data may not capture the
whole need since it is limited to those cases admitted or reported to
local authorities.
Prevalence data is more difficult to obtain than mortality data.
If it is not available from local or national authorities, you may not
be able to use prevalence. Scientists from the Institute for Health
Metrics and Evaluation have national data for each country from
the GBD study, but access may be restricted unless requested by the
government. Some prevalence data may be available for specific dis-
eases such as HIV, cancer, or TB.
National mortality data can be used locally if you use population
to adjust the totals. For instance, if you know that there are 8,000
cancer deaths for a population of 5 million people in your country
and you want to estimate for a local population of 1 million, you
can roughly say that 20% of 8,000 is 1,600 cancer deaths. You may
also use death rates per 100,000 population if available, taking into
account any local peculiarities such as higher death rates due to
local environmental conditions.
In the Global Atlas (10) we simply doubled the end-​of-​life need
to account for those needing palliative care prior to their last year
of life. The rationale for this was that a typical palliative care pa-
tient will begin to have symptom problems around 2 years prior
to their death. The Lancet Commission Report (11) used a more
sensitive approach by calculating days of suffering from all the
most common symptoms. In that study the end-​of-​life need for
palliative care was 25.6 million decedents and 35.5 million non-​
decedents with a total of 61.1 million with palliative care needs.
Thus, the end-​of-​life need more than doubled to almost 1.4 times
the end-​of-​life need.
These numbers included both adults and children, however we
know that children are more resilient than adults and so this ratio
is likely much higher. A child may need palliative care for 1 year
16 SECTION I Foundations of care
or 20 years. In the report on global need for palliative care (23) the
population need for CPC was over 2.6 times the specialized need.
Conclusion
Our understanding of the field of CPC has grown considerably in the
past few decades. However, there is much more to do than has been
done to date. Children are still less likely to access palliative care than
adults. Ninety-​eight per cent of the need for CPC is in LMIC and
progress to date has been mainly achieved in HICs. Research on CPC
lags behind APC, which itself lags behind other fields of medicine. In
this chapter we have attempted to update some of the important ques-
tions about the field of CPC including who needs palliative care, the
benefits of CPC, estimates of how many need CPC, how this is meas-
ured and so forth. The epidemiology of CPC continues to evolve, as
does the definition. Hopefully we are moving toward a time when all
children who need CPC will receive the care they deserve.
ACKNOWLEDGEMENTS
Text extracts from Radbruch L. et al. (2020). Redefining
palliative care—​
a new consensus-​ based definition. J Pain
Sympt Man. S0885–​3924(20)30247–​55. DOI: 10.1016/​
j.jpainsymman.2020.04.027. Reproduced with permission.
REFERENCES
1. WHO Definition of Palliative Care for Children 2013. Available
from: http://​www.who.int/​cancer/​palliative/​definition/​en/​.
2. Radbruch L, Liliana DL, Felicia K, Roberto W, Zipporah A, Sushma
B, et al. Redefining palliative care—​a new consensus-​based
definition. J Pain Sympt Man. 2020;6 May;S0885–​3924(20)30247–​5.
Doi: 10.1016/​j.jpainsymman.2020.04.027. Online ahead of print.
3. Together for Short Lives. A Guide to Children’s Palliative Care.
Bristol: Together for Short Lives; 2018.
4. A. K. On Death and Dying. 40th Anniversary Edition ed., 1999.
5. Jarvis S, Fraser LK. Comparing routine inpatient data and death
records as a means of identifying children and young people with
life-​limiting conditions. Palliat Med. 2018;32(2):543–​53.
6. Hain R, Devins M, Hastings R, Noyes J. Paediatric palliative
care: Development and pilot study of a ‘Directory’ of life-​limiting
conditions. BMC Palliat Care. 2013;12.
7. Feudtner C, Kang TI, Hexem KR, Friedrichsdorf SJ, Osenga K,
Siden H, et al. Pediatric palliative care patients: A prospective
multicenter cohort study. Pediatrics. 2011;127(6):1094–​101.
8. Taylor LK, Miller M, Joffe T, Parslow RC, Aldridge J, Bailey CC,
et al. Palliative care in Yorkshire, UK 1987–​2008: Survival and
mortality in a hospice. Arch Dis Child. 2010;95(2):89–​93.
9. Meiring M. Paedspal First Annual Review 2006. Cape
Town: Paedspal, 2016.
10. Connor SR, Sepulveda C. (Eds.) Global Atlas of Palliative Care at
the End-​of-​Life. London, UK, Geneva CH: Worldwide Hospice
Palliative Care Alliance and World Health Organization; 2014.
11. Knaul FM, Farmer PE, Krakauer EL, De Lima L, Bhadelia
A, Jiang Kwete X, et al. Alleviating the access abyss in
palliative care and pain relief—​an imperative of universal
health coverage: The Lancet Commission Report. Lancet.
2018;391(10128):1391–​454.
12. Sidebotham P, Fraser J, Fleming P, Ward-​Platt M, Hain R. Child
death in high-​income countries: 2 Patterns of child death in
England and Wales. Lancet. 2014;384(9946):904–​14.
13. United Nations Inter-​agency Group for Child Mortality
Estimation (UN IGME). Levels and Trends in Child
Mortality: Report 2017, Estimates Developed by the UN Inter-​
agency Group for Child Mortality Estimation. New York: United
Nations; 2017.
14. Office for National Statistics. Child Mortality in England and
Wales. London, UK: Office for National Statistics; 2017.
15. Feudtner C, Silveira MJ, Christakis DA. Where do children with
complex chronic conditions die? Patterns in Washington State,
1980–​1998. Pediatrics. 2002;109(4):656–​60.
16. Statistics South Africa. Mortality and Causes of Death in South
Africa, 2015: Findings from Death Notification, 2017.
17. Bluebond-​Langner M, Beecham E, Candy B, Langner R, Jones
L. Preferred place of death for children and young people with
life-​limiting and life-​threatening conditions: A systematic review
of the literature and recommendations for future inquiry and
policy. Palliat Med. 2013;27(8):705–​13.
18. Pollock K. Is home always the best and preferred place of death?
BMJ. 2015;351.
19. Gao W, Verne J, Peacock J, Stiller C, Wells C, Greenough A, et al.
Place of death in children and young people with cancer and
implications for end of life care: A population-​based study in
England, 1993–​2014. BMC Cancer. 2016;16.
20. Feudtner C, Feinstein JA, Satchell M, Zhao H, Kang TI. Shifting
place of death among children with complex chronic conditions
in the United States, 1989–​2003. JAMA. 2007;297(24):2725–​32.
21. Jarvis S, Parslow RC, Carragher P, Beresford B, Fraser LK. How
many children and young people with life-​limiting conditions
are clinically unstable? A national data linkage study. Arch Dis
Child. 2017;102(2):131–​8.
22. Fraser LK, Fleming S, Parslow R. Changing place of death in
children who died after discharge from paediatric intensive
care units: A national, data linkage study. Palliat Med.
2018;32(2):337–​46.
23. Connor SR, Downing J, Marston J. Estimating the global need
for palliative care for children: A cross-​sectional analysis. J Pain
Sympt Man. 2017;53(2):171–​7.
24. Connor S, Sisimayi C, Downing J, King E, Lim Ah, Ken P, Yates
R, et al. Assessment of the need for palliative care for children in
South Africa. Int J Palliat Nurs. 2014;20(3):130–​4.
25. Fraser LK, Miller M, Hain R, Norman P, Aldridge J, McKinney
PA, et al. Rising national prevalence of life-​limiting conditions in
children in England. Pediatrics. 2012;129.
26. Fraser LK, Miller M, McKinney PA, Parslow RC, Feltbower
RG. Referral to a specialist paediatric palliative care service in
oncology patients. Pediatr Blood Cancer. 2011;56(4):677–​80.
27. Fraser LK, Fleming T, Miller M, Draper ES, McKinney
PA, Parslow RC, et al. Palliative care discharge from
paediatric intensive care units in Great Britain. Palliat Med.
2010;24(6):608–​15.
28. Mitchell S, Morris A, Bennett K, Sajid L, Dale J. Specialist
paediatric palliative care services: What are the benefits? Arch
Dis Child. 2017;102(10):923–​9.
2
Communication
Jennifer Mack and Bryan Sisk
Introduction
Communication, the two-​way interchange of ideas and feelings, is
the goal of much of the work in paediatric palliative care, and clin-
ical conversations are the ‘method’ that is used to attain this goal.
For the clinician working in paediatric palliative care, effective com-
munication is an essential and learnable skill that forms the bedrock
of the practice of palliative care. In this chapter, we outline prin-
ciples and offer suggestions that support effective communication.
However, communication is more than merely a ‘clinical tool’ in
palliative care; meaningful and real two-​way communication is the
primary method by which clinicians forge relationships of mutual
trust, respect, and caring with the children and families under their
care, and it also gives back to clinicians a sense of meaning and pur-
pose in their work. Good communication is good care for children
and their families.
Effective communication in paediatric palliative care can be
understood as a process that allows for:
• The development of shared knowledge between the child, family,
and medical team, both by allowing the clinician to know the
child and family, and by providing the child and family with the
information they need to best make decisions for the child’s life
and medical care.
• Establishes relationships of mutual respect, caring, and trust be-
tween the child, family, and medical team, and provides a safe en-
vironment for the expression of emotion by acknowledging and
exploring feelings, and providing appropriate support.
• Allows supportive, shared, decision-​making between the child,
family, and medical team, while at the same time establishing a
process for ongoing support, transmission of information, and
decision-​making.
• Enables and encourages parents to fulfil their perceived duties
and roles as good and loving parents.
In practice these are intimately linked and evolve together.
Communication as a foundation of palliative care
Communication is one of the core interventions utilized by pallia-
tive care clinicians to help fulfil the goals of palliative care, including
‘the active total care of the child’s body, mind and spirit, and . . . sup-
port to the family,’ as defined by the World Health Organization (1).
Communication allows clinicians to recognize and attend to areas
of suffering and impaired quality of life. In addition, communica-
tion is the primary tool for helping patients and their family mem-
bers to acknowledge and accept the limitations posed by the child’s
disease in order to develop and carry out appropriate plans for care,
treatment, and their daily lives.
Communication in palliative care benefits from an open model.
Rather than being purely focused on the words used by the clinician
and the information exchanged, open communication allows for the
bidirectional exchange of thoughts and ideas, providing a window
to the values and beliefs of children and their families in their own
words. For some clinicians and parents, using open-​ended ques-
tioning is a fundamental shift from the more typical closed-​ended
questioning about symptoms, and such open questioning can re-
affirm to parents and children that their words and ideas are im-
portant. Using open-​ended questions along with careful listening
also provides clinicians with essential information about the child
and family, which might not be otherwise discovered.
Several formal models for communication have been developed
and can serve as useful guides. For example, the SPIKES model de-
veloped by Walter Baile encourages clinicians to Set up the con-
versation, assess the patient’s Perceptions, and obtain the patient’s
Invitation, all before providing information and Knowledge. As
information is transmitted, emotions are addressed with Empathy,
and next Steps are planned for future care and conversations (2).
This model proposes that the patient’s perceptions and wishes
would serve as the foundation for the entire communication en-
counter. While clinicians may enter a conversation with a particular
plan or agenda for communication, the needs of the patient take
precedence over that agenda. Other models have been developed,
perhaps the simplest of which is the recommendation that, in all
encounters, clinicians remember to ‘ask, tell, ask’—​ask the patient
what he or she understands and wishes to know, offer information,
and then ask the patient to reflect on the information, emotionally
and cognitively. Communication requires constant adaptation to
the needs of each patient and family. These general strategies put
the individualized approach at the forefront of every conversation.
Communication holds a central role in palliative care throughout
the course of the child’s illness. Parents consider preparation for
18 SECTION I Foundations of care
what to expect in the child’s future to be one of the essential elements
of high-​quality palliative care. We know from studies of cancer pa-
tients that early discussions about prognosis alter the care received
at the end of life, leading to earlier use of palliative care and less
reliance on life-​prolonging measures. This central role of communi-
cation is equally important in other prolonged chronic illnesses. For
example, parents of children with severe neurologic disability may
have difficulty understanding the child’s expected future course of
illness without early conversations about the signposts of change.
Early preparation for possible future outcomes can be revisited
over time, as the child’s illness changes and as the parents process
information.
Barriers to effective communication
Communicating effectively is a learnable skill that should be as inte-
gral to fulfilling the role of a clinician as prescribing medications or
performing procedures, but dedicated training or instruction in the
practice of communication is still uncommon. Clinicians may feel
that their ability to communicate is hampered by a lack of know-
ledge about the right words to say or when to say them. Consciously
or unconsciously, clinicians often avoid difficult conversations.
Over-​reliance on giving information, offering premature reassur-
ance, and ignoring the cues offered by the child and family are all
common tactics we can observe both in our colleagues and our-
selves. Anxiety when facing difficult situations and conversations
stems from our fears of causing distress, our ability to cope with
the child’s and families’ powerful emotions, and ultimately acknow-
ledging our own emotions and vulnerability in the face of death. If
this lack of comfort leads clinicians to avoid challenging conver-
sations it can rob children and families of the benefits of under-
standing the illness and incorporating that knowledge into their
plans for the child’s care and remaining life.
Even with knowledge and experience, the complexities of com-
munication still pose challenges. There are no ‘right words’ that will
work for every family in every situation. Communication cannot
be taught via a rigid instruction manual; rather, it is a process of
genuine human interaction, lifelong learning, and adaptation,
with new challenges and opportunities presenting with each new
encounter. Ideally, clinicians will have the opportunity to receive
mentorship over time, with observation of difficult conversations
by an experienced and effective mentor followed by reflection on
encounters and their outcomes. When dedicated mentorship is not
available, reflection within a diverse clinical team can be an effective
way to learn and sustain this work.
Despite best intentions, even dedicated clinicians who strive to
communicate will face the challenge of limited time. Conversations
often move at their own pace and can seldom be rushed. Few
practitioners have the luxury of unlimited time, but clinicians in
resource-​limited countries in particular may have far more patients
to attend to than time can allow. However, good communication
is a fundamental aspect of palliative care, not an option to be put
aside when time is limited. An early investment of time and effort in
effective communications may avoid later investment of time in in-
tensive treatments that can increase suffering at the cost of quality of
life. In addition, while communication does require time, conversa-
tions need not always be long—​sometimes a few minutes is enough
to move understanding forward, especially if there is an established
relationship and commitment to return to conversations over time.
Establishing a relationship and the development
of shared knowledge: Learning from the child
and family
Although clinicians often focus on the information that they pro-
vide, eliciting information from the child and family is just as im-
portant. This process enables the clinician to ascertain the child’s
and family’s present understanding of the situation, guiding the clin-
ician to an appropriate starting point for information transmission.
In addition, eliciting information allows the clinician to understand
more about the child and family, as well as demonstrate his or her
care, concern, and respect for the family. Providing care requires the
building of a human relationship that emerges over time. By better
understanding the child and family members as unique individuals,
the clinician can serve as a guide for important decisions and also be
present with them through the difficult journey that involves many
transitions.
When meeting for the first time, the clinician may start with gen-
eral questions about how the child would describe him or herself
as a person, what he or she cares about most, and what parts of
life are most important or meaningful. This conversation affirms
the clinician’s caring for the child as an individual and begins to
create a relationship in which the child can feel known. In the case
of non-​verbal children, it is particularly important to seek the par-
ental perspective on how they see their child as unique and special.
Questions that may be helpful include the following:
• What are some of your favourite things about your child?
• What do they most enjoy doing?
• What do they most dislike doing?
• What is their day like from the time they wake up in the morning?
This early communication also provides the clinician with valuable
information about personal values and goals, as well as what makes
the child uniquely him- or herself. With time, this information may
help to foster individualized decision-​making. Palliative care practi-
tioners can also provide these important insights to others in order
to create a shared understanding of the family’s goals and values
among all clinical providers.
Over time, the clinician may wish to deepen this conversation
by learning about religious and spiritual beliefs, life goals, and the
meaning of illness in the life of the child and their family. Many
parents and children struggle to accept the presence of illness and
death in the child’s life, and this conflict may be at the core of de-
cisions about care. A wise clinician will know when to serve as
witness by listening (being), rather than fixing (doing), as families
navigate this process. The opportunity to be part of a child’s and
family’s search for meaning can be transformative and meaningful
for the clinician as well as for the child and family and is one of the
great privileges of this work.
Questions may help clinicians to explore these important topics,
which find meaning and dignity, so long as they are used in the
context of a genuine interaction and are not formulaic. Some
examples are:

• What gives your life meaning (worth, or purpose)?
• Where do you find strength and support?
• What inspires you?
• Where or with whom do you feel most connected?
• How do you like to be thought of?
• What are you most proud of?
• Are there particular things you’d like your family to know about
you? Are there particular things you’d like them to remember
about you?
• When was the last time you laughed really hard?
• Are you frightened by all of this? What exactly are you
frightened of?
• I know you have pain, but are there things that are even worse
than the pain?
• What is the worst thing about all of this?
• What is the meaning of this (illness) experience to you? Do you
ever think about why this happened to you?
• What are your hopes and dreams for the people you care about?
• How does your faith fit into this experience? (For children and
parents who speak about their faith.)
In addition to gaining understanding of the child and family mem-
bers as individuals, listening allows the clinician to understand their
perceptions of the medical situation and the child’s future. The clin-
ician should take care to assess the perceptions of both the child
and the parents as a foundation for future information sharing.
Assessing perceptions at the start of the conversation enables the
clinician to know where to begin the conversation and evaluates
their level of understanding of the issues at hand so the information
given can be tailored appropriately and any misconceptions can be
corrected.
Importantly, communication occurs over time rather than
during a single conversation and must be revisited and processed
throughout the course of the illness. Information that is conveyed
at only one point in time may not be fully retained or understood,
especially when it is received under stressful circumstances. Most
parents report a preference for revisiting information longitudin-
ally with their clinical team, even if the information is emotionally
difficult to address (4), so the clinician should have a low threshold
for returning to important conversations over time and regularly
checking for facts that have been conveyed previously.
Some useful questions include the following:
• Can you start by telling me what your understanding of your
illness is so far?
• A lot has happened over the past few days that I would like to re-
view with you, but first I’d like to start by hearing from you about
how you see your illness at this moment.
• I know that we have discussed information about your illness.
I want to make sure that I remembered to tell you all of the most
important parts. Can you review with me what your under-
standing is at this moment?
These and similar questions can be addressed to both children and
their parents as a way of beginning the conversation and noting
issues to expand upon and explore as the conversation continues.
It can also be helpful to destigmatize the parents’ or child’s lack of
understanding. For conversations where difficult information has
CHAPTER 2 Communication 19
been exchanged, ending with summaries of most important points
can also be helpful.
Once the clinician knows what the child and family understand
about the medical situation, they may want to seek permission to
move on to new topics. Although many patients want complete in-
formation about their illness, some do not, while others want full in-
formation but also want to be able to manage the extent and timing
of disclosure. In addition, parents may worry about disclosure of
information to their children, and a request for permission to dis-
cuss specific topics offers the opportunity to share these concerns.
A clinician’s request for permission to have a conversation may be a
simple question about the content of the discussion—​for example,
‘Earlier, you described worrying about your future. I wonder if it
would be helpful to talk more about what the future may look like
for you?’
Alternatively, there may need to be a more active negotiation
about how information is conveyed. For example, when discussing
prognostic information, it may be helpful to ask the parents how
they would like to hear such information. One might say:
People like to hear this information in different ways. Some parents
want to hear general information about the chance that a child will be
cured, such as whether we think treatment offers a good or not-​so-​good
chance of cure. Others prefer to receive the most specific information
possible, such as the percentage chance of cure. What would be most
helpful for you?
For situations where a disease process is known to be incurable,
some parents may want to know all available information about
progression of disease, limitations, quality of life, and life expect-
ancy. However, other parents may not want this information until
later in the disease course. Also, some clinical scenarios are com-
plicated by persistent uncertainty about the cause of acute illness
or likelihood of recovery. For example, patients with chronic, life-​
limiting illnesses often become acutely ill repeatedly, sometimes
without a clear understanding of underlying aetiology. Such uncer-
tainty complicates prognostication and decision-​making for parents
and clinicians.
In addition to responding to invitations offered by clinicians,
children may indirectly invite important conversations. As clin-
icians, we often expect families to discuss issues on our schedule,
but the child may have his or her own timetable of readiness, and
an astute clinician will follow the child’s lead. The child’s invitation
to discuss important topics may be subtle, especially when they are
young, and they may bring up topics obliquely to observe how they
are received. For example, they might say ‘I heard people can die
from cystic fibrosis,’ and then watch for the response of the listener. It
is important to be attentive to such overtures, because an invitation
that is missed may not be repeated. In addition, the clinician should
notice the words that are used by the child. Mirroring this language
back to them helps them feel respected and heard and avoids con-
cern that a topic is being addressed too directly for comfort.
Another helpful process may be to explicitly request permission
to ask questions. Whenever the clinician asks for the child’s and
parent’s permission to begin a conversation, he or she must be pre-
pared for the possibility that the parent or child is not ready for such
a discussion and be willing to respect that preference. Importantly,
parents and children who do not feel ready to discuss specific
issues may not be able to move forward with decisions relating to
20 SECTION I Foundations of care
these issues, even when the clinical team considers such decisions
to be urgent (for example: decisions about resuscitation). If there
are topics that the clinician feels absolutely must be discussed at a
particular moment in time, regardless of patient and family pref-
erences, he or she should be honest and open about this intention.
Ideally such circumstances are relatively rare, because the patient
and family members may find themselves feeling unsupported
when their needs and wishes are not allowed to guide the discus-
sion. This may undermine a central goal of communication, which
is the building of a trusting relationship in which the patient and
parents feel listened to, understood, and respected.
Establishing a relationship and the development
of shared knowledge: Transmission
of information to the child and family
Although the receiving of information and the transmission of in-
formation are discussed as separate topics, it is important to em-
phasize that the two processes are intimately linked. A conversation
should be tailored to the needs of the child and parents, with their
understanding and emotional response being assessed along the
way. The simple rule of ‘first ask, then tell, then ask again’ incorp-
orates the principles of assessing knowledge, providing additional
information, and then assessing understanding again. This rule is
appropriate for both parents and children, however, communica-
tion should not be rigid. Information exchange can be viewed as
a fluid back and forth process that alternates between recognition
and guidance (5). One need not wait until the end of a conversa-
tion to check for understanding or respond to emotional gestures.
Emotional signals can be directly addressed in the midst of a con-
versation, for example, by naming emotions that are observed: ‘That
last topic seemed to upset you quite a bit.’
Transmission of information is often the aspect of communica-
tion with which clinicians are most comfortable. Clinicians who
are ‘giving’ information should do so in language that parents can
understand, without excessive jargon or euphemisms. Information
exchange can be supported with visual aids, even if this is a simple
picture drawn on a piece of paper. Information should be discussed
in a sensitive yet truthful manner. Clinicians may feel uncomfort-
able disclosing difficult information, but a multitude of studies
have shown that parents and children prefer honest yet empathic
disclosure (6). In adult studies, when difficult information is ex-
changed, patients are more likely to report that their physicians are
less compassionate, but utilizing negative terms is similarly associ-
ated with better prognostic understanding (7). Therefore, clinicians
must pay extra attention to their words and mannerisms as they
engage in these important conversations.
Clinicians, however, are not the only source of medical infor-
mation for patients and their families. One study of the parents
of paediatric cancer patients found that physicians and nurses are
the predominant sources of prognostic information, but 41% of
parents also found the internet to be extremely informative (3). In
searching the internet, parents may utilize disease specific websites,
web-​based support groups, social media, or they may simply utilize
a search engine and click on the first webpages available. Some of
these internet resources are reliable, though many are not, and even
reliable resources will contain information that does not apply to
every child. Physicians sometimes discourage parents from seeking
information or support on the internet, but this well-​intentioned
approach is unrealistic and may make parents feel embarrassed
or unable to discuss articles they find. Another approach is to ac-
knowledge openly that many parents seek information from the
internet in an effort to make the best decisions for their children.
The clinician can offer to work with them, facilitating and checking
facts, rather than discounting the parents’ findings. Navigating this
common scenario well can help to bolster the clinician–​family rela-
tionship, which is the foundation of effective communication.
Communication with children and young adults poses unique
communication challenges. Depending on the age of the child, his or
her developmental level, and the parents’ preferences and concerns
about transmission of information, the decision to include the child
requires exploration. For older children and adolescents, exclusion
from important conversations may heighten anxiety and raise con-
cerns that the team is not being completely honest. Understanding
their illness and situation can help adolescents to fully participate
in their care and decision-​making and may increase their adher-
ence to recommended therapy. As a guiding principle, if a child is
old enough to understand the information, he or she should be in-
cluded in this conversation. Additionally, the child may also need
his or her own space with the clinician, apart from the parents.
Some parents do not want the child to participate, but with-
holding information may not be fully supportive of young people,
especially those for whom information will allow them to manage
anxiety, rather than exacerbating their fears. Although including
children and young people in these conversations meets the ethical
expectations and cultural norms for disclosure and assent, especially
in many well-​resourced countries, it may not be the pattern of com-
munication for some families and some cultures. When parents are
resistant to including children in medical conversations, the clin-
ician can respond first by exploring parental concerns and beliefs:
• Tell me more about your concerns.
• When you think about including your child in this conversation,
what are you most worried about?
• How do you think he or she would react to this information?
• How have you handled communication in your family in the past?
• How has your child received difficult news in the past?
• What do you think his or her understanding of the situation is at
the moment?
• What kind of information would you like him or her to know?
• What kind of information are you hoping will not be offered
to him or her?
The clinician’s role should not be to convince the parents to include
the child, but rather to negotiate a communication strategy that
works best for the child and the family. Understanding the parents’
perspective allows the clinician to provide additional information
for the parents to help to inform their decisions. The clinician may
acknowledge the parents’ understandable effort to protect their
child, while also expressing that most children will indirectly pick
up information about diagnosis, illness, and prognosis by observing
distress in their parents, noticing the situation of other children in
the clinic or on the hospital ward, or hearing parts of conversations
of others, including medical providers and family members (8). An

incomplete understanding may heighten anxiety. The clinician may
be able to help the parents feel more comfortable by describing the
information that will be given to the child and offering examples
of specific words that will be used. Using language that has been
agreed upon, rather than words that might be perceived as trouble-
some by the parents, may help parents to accept direct clinician–​
child conversations. Clinicians may also offer the support of other
professionals, such as psychologists and child life specialists if these
resources are available.
Some families will not feel comfortable about including the child
in direct communication or in more significant family meetings. In
this case, with the parents’ permission, the clinician should offer the
child a separate opportunity to receive information and ask ques-
tions. Although some parents may wish to convey news themselves,
it is often helpful for a medical team member to deliver the news
to the child directly. If the parents are not comfortable with having
the medical staff lead the communication with the child, a clinician
should at least offer to be present as a witness to the conversation
so the team will understand the nature and content of the com-
munication that takes place. With the development of a trusting
relationship, some families will be able to accept more direct com-
munication with the child over time.
Finally, some parents will be unable to accept any communica-
tion with the child about certain subjects. Although such situations
are relatively rare, they can be very distressing to clinicians. In these
circumstances, respect for the way things work in the child’s family
should remain a guiding principle. However, the clinician should
identify ethical boundaries in a non-​confrontational way:
One thing I want to let you know is that I always promise patients and
families that I will never lie to them. If your child asks me a direct ques-
tion, I will not intentionally mislead him. However, I will also do my best
to respect your concerns about discussions of these difficult topics.
Establishing a relationship and responding
to emotions
Relationships between the child, family, and clinician evolve through
the encounters and conversations experienced whilst developing
a shared knowledge. Powerful emotions are often evoked, and all
clinicians have a role and a responsibility in responding to these
emotions and supporting families and patients with life-​threatening
illnesses. The core of empathy in an encounter is a willingness
to listen intently and seek to understand the others in the room,
and the recognition of emotion should extend from that desire.
Opportunities for the expression of empathy and acknowledgement
of emotion are often missed by clinicians. Also, different care pro-
viders may have differing views regarding their roles in responding
to emotions. For example, nurses often feel a responsibility to re-
spond to outward emotional expressions, whereas many physicians
view their role as more biomedical (9). Additionally, because com-
munication is often centred around difficult topics, clinicians them-
selves commonly experience anxiety, personal pain, and distress. As
a result of their own emotions, they may avoid difficult topics, even
when patients and families would like such discussion.
Relatively simple techniques can allow the clinician to openly
address emotion in the conversation. At times, simply naming the
CHAPTER 2 Communication 21
emotion can be useful, e.g. ‘Am I correct in thinking that you are
feeling sad?’ or ‘It would be understandable if you were angry—​is
that the case right now?’. Acknowledging powerful emotions pre-
sent in the moment is often helpful in diffusing difficult situations.
Alternatively, when emotions are not clear, it may be appropriate to
ask the child or parent to describe how they are feeling. The clin-
ician may then want to explore the emotion by asking more about
the source of those feelings. When the parent or child has had the
opportunity to express their feelings, the clinician may wish to re-
spond by acknowledging and naming. For example, they might
simply say ‘I’m sorry that this is so hard.’
Although it can be helpful in certain situations to use phrases that
have been carefully thought through such phrases should not be
used to turn a natural, empathic human encounter into something
that is forced. It is better to engage in authentic human dialogue
than in a mechanical step-​by-​step process. If things are said that the
clinician later, or even immediately, regrets or that were misinter-
preted, the power of a genuine apology cannot be understated. ‘I am
sorry for what I said yesterday about . . .’. Such phrases, when said
honestly and with no defensiveness, can be effective interventions
for healing and reinforcing the relationship.
Importantly, parents may not always express their emotions, and
external expressions of calm are not necessarily indicative of in-
ternal calm (10). In addition to empathic words, the clinician may
wish to respond with a listening silence, an attentive expression,
a helpful act (e.g. offering a glass of water), or a physical gesture
such as a touch on the arm. There are many ways other than spoken
words for clinicians to help children and families to feel known and
understood.
Identifying values and making shared decisions
Throughout a child’s illness parents feel a duty to fulfil their own
perception of the role of a ‘good parent’, and this drive can largely
impact their decision-​making process (11). In addition, they use
their understanding of the situation and medical information to
reach their decisions about care for their child.
When the child’s illness is life-​threatening, imminent decisions
can be highly significant, such as whether care will be directed pri-
marily at relieving symptoms or prolonging life, and where care will
be delivered. In these situations, the goal of the decision-​making
process is not to reach a single right decision, but rather to reach
the decision that best aligns with the goals and values of the child
and their family at that moment in time. Goal-​oriented decision-​
making is best instituted early in the illness, to allow the child and
parents to become accustomed and comfortable with this kind of
decision-​making. Although goals may change over time, the frame-
work can stay in place throughout the illness. In addition, children
and parents whose goals are known and understood by the medical
team may themselves feel better understood.
Importantly, children and parents may not have fully developed
their preferences or goals for treatment, or they may not have fully
explored their deeply held values and beliefs. Part of the work of
palliative care is helping families to explore their values and con-
struct their preferences and goals of care, a process that takes time
and attentive effort. In this process, it is crucial to ensure that the
clinician avoids imposing his or her values on the family.
22 SECTION I Foundations of care
Some questions that help elucidate or develop the goals of chil-
dren and parents include:
• As you think about your future, what is most important to you?
• Are there particular things that you would like to have happen in
the future?
• What are you most worried about?
• What are you hoping for?
Although open questions tend to be most useful, some families
need more concrete examples of the kinds of goals that might be
possible for them. In this situation the clinician might, for example,
use the following approach:
Some families might say that it is most important to them for the child
to live as long as possible, even if that means spending more time in the
hospital or having more medical interventions. Other families might
say that they want the child to have the best quality of life possible,
even if it means not living quite as long. Still other families might have
very different goals, like wanting to make sure the child can make it to
a special event or occasion. As you think about the future, what is most
important to you?
The clinician may wish to ask additional questions that explore the
meaning of the responses. For example, if a child is worried about
their parents’ well-​being, the clinician may ask the child, ‘What in
particular are you worried might happen to your parents?’. Similarly,
children or parents who are worried about concrete, physical aspects
of death might be asked to explain their worries in more detail. The
clinician may then ask them if it would be helpful to hear more in-
formation about how things may be as death draws closer. Although
these can be very difficult topics, they may also be some of the most
distressing issues for children and parents. The opportunity to talk
openly about them may offer the clinician a chance to alleviate the
child’s or parents’ fears and suffering as they contemplate the future.
Although some children and parents may want the opportunity
to address concerns directly, not all parents and children feel able
to talk about a difficult future in such concrete terms. Some parents
find it difficult to express any hopes for the future other than the
child’s good health and well-​being. Others have described hope
and reality as two aspects of a parent’s difficult present (12). It is
important to note that some parents will express hopes even while
they hold quite different expectations for their child’s illness (13).
Therefore, the expression of hopes should not necessarily be under-
stood as a lack of understanding of a difficult prognosis.
Other parents may find that a poor prognosis is difficult for them
to accept. For such parents, ‘awareness cannot be forced it can only
be supported’ (14). One communication strategy that is sometimes
helpful is modelled by the phrase ‘Hope for the best, prepare for the
worst’ (15). Thus, there is acknowledgement of hopes for the fu-
ture, while at the same time the child or parents are asked to con-
sider what care they would want, should the desired good outcome
not be possible. By allowing room for the parent’s or child’s hopes,
parents can feel supported in their decision-​making process while
also understanding the clinical reality.
The identification of goals for care is rarely simple and straight-
forward. Rather, it requires reflection, including contemplation of
deep values. Goals for care in serious illness touch on spiritual and
religious beliefs about death, the afterlife, and the moral basis for
care near the end of life. In addition, parents and children often
consider the impact of their decisions on others—​especially on one
another, on siblings, and on extended family members, but also on
children in the future who may benefit from their child’s participa-
tion in a clinical trial.
When should conversations about palliative care
take place?
Clinicians need not be certain that a patient is dying in order to
have conversations about values, preferences, and goals. Having
these conversations may be helpful to all children with life-​
threatening illnesses and their families. These discussions play
important roles in addressing fears about the future, identifying
important values and goals for care, and allowing the patient, the
family, and the medical team to build a relationship. Rather than
being distressing, such conversations may reassure children and
families that the clinical team knows them as people and will do
everything possible to uphold their wishes. Clinicians can return to
the values and wishes identified in these discussions over time, as
the medical situation changes or as children and families are faced
with new decisions.
Certain clinical scenarios necessitate a more urgent initiation of
such conversations (for example severe medical deterioration or
immanent death). Clinicians may also need to broach these topics
when patients are experiencing undue suffering in the setting of
advanced illness. Clinicians might recognize their own discomfort
with such care as a cue to begin or continue discussions about the
patient’s goals and values. Patients and families themselves may im-
plicitly or explicitly express a wish to have these discussions. For
example, patients may ask directly about issues related to pallia-
tive care or death. As well as providing the requested information,
clinicians may take this opportunity to explore patient and family
concerns about what lies ahead. Such questions may have a sub-
text of fears about the future or concerns about suffering and dying.
Sometimes children or their parents may question the future indir-
ectly or express fear, anxiety, or distress, which cannot be allayed
by addressing more practical concerns. This may indicate a need
to explore distress as the manifestation of emotional, existential, or
spiritual suffering.
Communication across cultural and
language differences
Cultural and spiritual beliefs form the bedrock upon which many
lives are built. To be an effective clinician and communicator, one
must strive to recognize and respect the beliefs of children and fam-
ilies under our care. In addition, to more obvious differences of
religion or ethnic background, clinicians often come to care from
the perspective of a medical model, where illness has an origin and
pathophysiology that can be medically understood. However, fam-
ilies may have very different understandings and explanations for
illness, and different ideas about the best ways to manage it. Rather
than seeking to overturn core beliefs in favour of the medical model,
clinicians who explore the belief systems of patients and families can
learn fundamental issues about their approaches to care. Ultimately,
Another random document with
no related content on Scribd:
back
back
back
back
back
back
back
back
back
back
back
back
back
back
back
back
back
back
back
back