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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
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Appendix 1 © 2019 The Association of Paediatric Palliative Medicine (APPM)
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Second Edition published in 2012
Third Edition published in 2021
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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.
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 In dogs there are the same general symptoms with vomiting. The
vomited material is usually remasticated and swallowed. The
swelling in the pharynx can be felt from without, or seen through the
open mouth. The tonsils are usually enlarged. Pressure on the
pharynx or gullet produces instant regurgitation.
Treatment consists in the removal of the tumor when possible.
Malignant growths and multiple tumors are not favorable for
treatment. Actinomycosis can be treated throughout by iodides, or
these may supplement the surgical measures. In the short-faced
animals an ecraseur, or a wire-snare passed through a tube may be
employed. (See pharyngeal polypi).
 ESOPHAGITIS. INFLAMMATION OF THE
GULLET.

Causes: Alimentary and therapeutic; parasitic and accidental traumatisms;

mechanical irritants; acrids; caustics; parasites—gongylonema, coccidia,
spiroptera. Extension inflammations. Lesions: hyperæmia; epithelial degeneration
and desquamation; erosion; petechiæ; suppuration; fibroid contraction;
sacculation; polypi. Symptoms: dysphagia, difficult deglutition; eructation; cough;
upward wave motion in jugular furrow; colicy pains; probang arrested; fever.
Treatment: liquid or semi-liquid food; for caustics, antidotes; cold water; ice;
antiseptics; derivatives; open abscess; potassium iodide.

Causes. This usually arises from injury to the mucous membrane

and in the milder forms remains confined to this structure. In the
more severe, it extends to the muscular coat and even to the
periœsophagean tissues. The causes may be divided into alimentary
and therapeutic irritants; parasitic or accidental traumatisms; and
extension of inflammation from the pharynx or other adjacent part.
Among irritants taken as food, may be named hot mashes, bolted
by a hungry and gluttonous horse, and temporarily arrested in the
gullet by reason of the resulting irritation of the mucous membrane.
In other cases, coarse fibrous fodder is bolted without previous
mastication, and scratches and abrades the œsophagean mucosa
leading to transient or progressive inflammation. In other instances
diseases of the teeth, jaws, temporo-maxillary joint, or salivary
glands prevent the necessary trituration of the food, and it is
swallowed in a rough, fibrous, or even a dry condition. Again the
impaction of a solid body (turnip, apple, potatoe, egg) or of a
quantity of finely divided grain or fodder so as to obstruct the lumen
of the gullet, is an occasional cause. The density of the epithelium
reduces these dangers to the minimum, yet a too rough morsel, or an
undue detention of the less irritating material will determine
hyperæmia and even inflammation and infective invasion. Acrid and
irritant vegetables in the food are less injurious when thoroughly
insalivated, as their contact with the œsophagean walls is then very
slight and transient.
Irritant and caustic chemical agents given for therapeutic
purposes, attack the mouth, pharynx and stomach, more severely
than the gullet through which they are passed with great rapidity. In
some cases, however, the agent will adhere by reason of its powdery,
gummy or balsamic character and will then act as a direct irritant.
Solutions of caustic alkalies (weak lye) given to correct acid gastric
indigestion in the horse, and ammonia to remedy tympany in cattle,
when insufficiently diluted, will dangerously attack the œsophagean
mucosa.
Parasitic irritation is not so common here as in other parts of the
intestinal canal where the contents are longer delayed and are passed
with less friction, yet certain parasites are found in this region and
may even produce considerable irritation. The gongylonema of the
thoracic œsophagean mucosa of ruminants and swine are apparently
harmless. The psorospermia of the œsophagean muscles of the same
animals are alleged to cause œdema of the glottis, asphyxia and
epilepsy. The spiroptera microstoma of the horse has in one instance
known to us caused extensive denudation of the muscular coat
within a foot of the cardiac end of the gullet. Finally we have found
bots hooked on to the œsophagean mucosa close to the cardia,
causing much irritation and spasm. The spiroptera sanguinolenta is
often present in chambers hollowed in the œsophagean mucosa of
the dog.
Traumatic causes appear in the form of contusions and bruises
from without, but much more frequently from foreign bodies, and
probangs operating from within. The use of a whip or of a rope
without a cup-shaped end for the relief of a choked animal. Short of
the occurrence of laceration this often produces contusion and
abrasion which results in local inflammation. Even the too forcible
dislodgment of a solid body by a probang of approved pattern, may
bruise and scratch the gullet when the seat of violent spasm. Pins,
needles, wire, thorns and other sharp bodies are liable to do serious
damage during their passage in an ordinary bolus and when they
transfix the mucosa violent infective inflammation may ensue.
 Extension inflammations from the throat, and from phlegmous,
abscesses, tumors, etc., in the jugular furrow need only be mentioned
in this connection, as the primary disease will be clearly in evidence.
Lesions. These are usually circumscribed when due to a traumatic
injury and extended when caused by caustics or irritants. The
affected section is swollen, and surrounded by some serous effusion.
When the muscular coat is involved it is often paler than normal, and
microscopically shows extensive granular and fatty degeneration.
The mucosa usually sloughs off its epithelial layer, sometimes over
an extensive area (thoracic portion, Renault; whole gullet, Bertheol),
and the exposed raw surface is of a deep red or violet. When the
epithelium is not shed, it is infiltrated, swollen and friable breaking
down under the slightest manipulation. Petechiæ and slight blood
extravasations are abundant, and diffuse suppuration is not
uncommon. In traumatic injuries necrosed areas are found in the
muscular and mucous coats. Strictures, dilatations, and polypoid
growths are liable to follow as sequelæ.
Symptoms. These usually manifest themselves from two to four
days after the operation of the cause. There is much difficulty in
deglutition, the effort to swallow either solids or liquids causing
acute suffering, with extension of the head on the neck and strained
contraction of the facial muscles. If the liquid succeeds in passing the
pharynx, it is arrested at the seat of inflammation and regurgitated
through the nose and mouth, or in solipeds through the nose only.
This takes the appearance of emesis even if nothing actually comes
from the stomach. The animal shakes the head violently, breathes
hurriedly, and has fits of paroxysmal coughing. A wave extending
from below upward along the jugular furrow and followed by nasal
discharge is a marked symptom, as the violence of the inflammation
increases. Uneasy movements of the limbs, pawing and lying down
and rising, indicate the existence of colic, and this is aggravated by
the administration of anodynes or antispasmodics by the mouth. In
cattle, rumination is arrested, froth accumulates around the lips, the
rumen becomes tympanitic, and colicy movements appear.
Oftentimes a swelling extends upward in the jugular furrow, and
even in its absence, pressure with the fingers along the furrow will
often detect an area of tenderness with or without local swelling.
Fever with more or less elevation of temperature, is a general
symptom. There may be wheezing breathing or loud stertor. The
passage of a probang is arrested by the swelling or spasm at the
diseased part and when withdrawn may be covered with pus or fœtid
debris. In the horse a small probang may be passed through the
nose.
Treatment. In a slight congestion at the seat of a recent
obstruction and which tends to renewed obstruction, little more is
necessary than to restrict the feed for a few days to soft mashes so
that irritation of the sensitive surface, spasm and the arrest of the
morsel may be obviated. Plenty of pure water or of well boiled
linseed or other gruel should be allowed.
In cases in which the obstruction is still present in the gullet, its
removal by probang or looped wire is the first consideration, to be
followed by the measures mentioned above.
In case of the swallowing of a caustic agent, no time should be lost
in giving an antidote. For the mineral or caustic organic acids, lime
water, magnesia, or other bland basic agent is demanded. For caustic
alkalies or basic agents, bland acids, such as vinegar, citric acid, or
even a mineral acid very largely diluted will be in order. In both these
cases and in that of caustic salts, albuminous and mucilaginous
agents, eggs, linseed tea, slippery elm, gums, and well boiled gruels
are indicated. To these may be added small doses of laudanum when
the irritation is great. Iced drinking water, iced milk, or iced gruels
are often soothing to the suffering animal, and cold compresses,
snow or ice applied along the jugular furrow is often valuable. To
counteract the septic developments on the affected mucous
membrane, chlorate of potash, boric acid, salol, naphthalin,
naphthol, pyoktannin, or even weak solutions of phenic acid or
creolin may be used. In the slighter forms of inflammation or when
the acute form threatens to persist, an active counter-irritant of
mustard or cantharides may be applied along the jugular furrow.
In case of abscess, as manifested by fluctuation following a hard,
indurated, painful swelling, a free incision should be followed by
frequent injections of antiseptic lotions or by the packing of the
cavity with such bland antiseptics as salol, boric acid, or iodoform on
cotton.
As inflammation subsides, potassium iodide may be given, both as
an antiseptic and a resolvent, to counteract the tendency to fibroid
contraction and stricture of the gullet.
 SPASM OF THE ŒSOPHAGUS.
ŒSOPHAGISMUS.

Causes: nervous disorders or lesions, pharyngeal, œsophagean, or gastric

disease, œsophagean parasites, choking, tumors, ulcers, cold drinks. Symptoms:
extended drooping head, working jaws, frothing, pawing, attempts at swallowing,
alkaline regurgitation, cries, rigid gullet, tenderness. May be paroxysmal with
intervening dullness. Treatment: by sound; by removal of obstruction; by
antispasmodics. Embrocations. Tonics.

Causes. This has been noticed as a concomitant of certain diseases

of the nervous centres, such as rabies, tetanus, or epilepsy, and those
of the pharynx or stomach. Cadeac has seen it in connection with
stricture, and the present writer has observed it as a result of larvæ of
œstri hooked on to the mucosa above the cardia. It is an important
factor in most cases of choking, and may depend on tumors, ulcers,
or even cold beverages. Animals with a specially nervous
organization are particularly subject to it and it may thus be an
hereditary family trait. It has been especially noticed in solipeds and
calves.
Symptoms. A feeding animal suddenly ceases to eat, extends the
head on the neck, drops the nose toward the ground, moves the jaws
constantly, froths at the mouth or lets the saliva drivel to the ground,
moves the fore feet uneasily pushing the litter under the belly, makes
efforts at deglutition during which, waves may be seen to descend
along the jugular furrow, followed by regurgitation and discharge of
the liquid as by emesis. The act is often followed by a slight cry.
Manipulations of the left jugular furrow detects the gullet as a firm,
rigid cord, unless when liquids are passing as above, and
auscultation reveals a rattling or gurgling noise as if in jerks.
Pressure on the gullet is often very painful, increasing the spasm and
rigidity, and causing the animal to cry out. Wheezing breathing may
attend the discharge of saliva through the nose, and violent
paroxysms of coughing may be caused by the entrance of this liquid
into the larynx.
In the majority of cases no food is swallowed and nothing but
saliva is disgorged, which together with the absence of an acid odor
distinguishes this from true vomiting. In an exceptional case of the
author’s, occurring in a colt, the animal continued to masticate and
swallow green food which gradually filled the whole length of the
gullet, practically paralyzing it. In ordinary cases a small sound can
usually be passed into the stomach. In cases of obstruction, however,
by a solid morsel, or by an accumulation of soft solids, the probang
will enable one to detect the condition. The acute symptoms may
occur in paroxysms of a few minutes in length, between which, the
animal remains dull and disspirited until the new attack supervenes.
Recovery is at times as sudden as the onset, though there remains,
for a length of time, liability to a relapse. Cadeac has seen a
succession of such attacks which extended over a year and a half.
Treatment. In many cases the passage of a probang or sound, will,
by the mere distension of the gullet, overcome the local spasm,
though it may be necessary to repeat the operation several times. In
case the sound causes much pain the end of the instrument may be
well smeared with solid extract of belladonna, and after passing this
as far as the obstruction a short time may be allowed, before its
passage is again attempted. In case obstruction by soft solids has
taken place, the passage of the wire loop will serve to break up the
mass and even to draw it up toward the mouth.
The administration of antispasmodics is the next indication.
Chloroform or ether by inhalation or in solution in water, chloral
hydrate as an enema, morphia or atropia hypodermically may be
used according to convenience. Bromide of potassium and other
antispasmodics given by the mouth, too often fail to pass the
obstruction and thus prove useless, except in the intervals of the
spasms.
Fomentations of the lower border of the neck with warm water,
and frictions over the region of the gullet with camphorated spirit,
essential oils, ammonia, or in calves with oil of turpentine, often
contribute to relieve the spasm.
 Finally after the severity of the attack has passed, a course of bitter
tonics and above all of nux vomica will fortify the system against a
relapse.
 PARALYSIS OF THE ŒSOPHAGUS.

Causes: nervous lesions and disorders; arytenectomy; over distension; stricture;

parasites. Symptoms: dysphagia; regurgitation; cough; dyspnœa; hard packed
gullet. Inhalation pneumonia. Lesions. Treatment: remove cause; liquid food;
dilatation; nerve sedatives and stimulants; electricity; counter-irritants.

Causes. This has been noticed in a number of cases in solipeds,

and attributed to central nervous lesions, cerebral concussion
(Straub), encephalitis (Hering, Bornhauser), paralysis of the fore
extremities (Meier), pharyngeal paralysis (Puschmann). Möller has
seen it several times consequent on arytenectomy, while Dieckerhoff
and Graf have seen it occur without any clearly defined cause. In a
case referred to above, the present writer found it connected with the
attachment of larvæ of œstri in the lower end of the gullet. Stricture
and impaction may be a further cause.
Symptoms and lesions. There is more or less interference with
deglutition, culminating in complete inability to swallow, and the
rejection of morsels of masticated food by the nose. Cough may also
occur from the descent of food toward the lungs, with more or less
dyspnœa and oppression of the breathing. Manipulation along the
left jugular furrow, detects the œsophagus as a prominent hard,
rope-like mass which fills up the groove unduly. When death occurs
rapidly the gullet is found gorged with masticated food throughout
its entire length. In certain instances gangrenous pneumonia is
found, the result of the penetration of food into the bronchia. In
other cases there are lesions of the medulla oblongata, or of the
vagus or glossopharyngeal nerves or their œsophagean branches.
Death usually results from obstruction, inanition, or, in case the
paralysis is partial, from pneumonia or exhaustion.
 Treatment. First remove or correct the existing cause of the
disease. Impaction may be broken up by the use of the wire loop, or
pincer probang; parasites may be expelled by passing a cupped
probang; the impactions following arytenectomy can be obviated by
feeding gruels, milk and other liquid foods only, and from a bucket
set on the ground; stricture may be dilated by the use of graduated
sounds; and nervous diseases may be dealt with according to their
specific nature in each several case. When any definite cause of this
kind has been overcome the persistent use of strychnia, subcutem, or
by the mouth, may be effectual in overcoming the paresis of the
gullet. Hypodermic injections are best made along the left jugular
groove, and frictions, stimulating embrocations, and galvanic
currents may be employed with excellent effect.
 ŒSOPHAGEAN TUMORS.
Forms of neoplasm in gullet of horse, ox, sheep, pig, dog. Symptoms: dysphagia;
eructation; vomiting; bloating; cough; dyspnœa; stertor; fœtor; palpitation.
Treatment.
These have been often noticed in the lower animals. In the horse
have been noticed melanoma (Olivier, Röll, Kopp, Besnard,
Pouleau), fibroma (Dandrieu, Dieckerhoff), Carcinoma (Chouard,
Lorenz, Cadeac, Laurent), epithelioma (Blanc, Lorenz), Leiomyoma
(Lucet, Lothes), cystoma (Caillau, Legrand), mucous cysts (Lucet).
In cattle papilloma is especially common, having been noted by
Johne, Mons, Fessler, Schütz, Lusckar, Gratia, Beck, Cadeac and Kitt.
Tubercles, and fibroid masses with cystic purulent centres are
not uncommon. Actinomycosis is also frequent, sometimes hard
and warty and at others soft and vascular.
In the Sheep, Dandrieu found between the muscular and mucous
coats a hard tumor as large as a hen’s egg, the removal of which put a
stop to a persistent choking. In both cattle and sheep, swellings
from coccidiosis are common; in cattle and swine from
gongylonema, and in sheep from filaria (Harms) or spiroptera
(Zurn).
In pigs, fibroma is met with in the walls of the gullet (Raveski)
and in dogs fibroma, papilloma, and the tumors of spiroptera.
Symptoms. The coccidia and spiroptera usually cause few
symptoms or none, but neoplasms usually develop symptoms of
obstruction, dysphagia, eructation, vomiting, and all the indications
of choking according to their seat. These do not come on suddenly
and recover as in simple choking, but even though there may be
periodic obstructions, spasms and paroxysms, there is a slow,
progressive advance as the neoplasms increase. Stertorous or
mucous breathing, cough, dyspnœa and fœtid exhalations are
common, the symptoms may be aggravated when the head is bent,
and the tumor may even be felt on palpation of the throat or left
jugular furrow. In ruminants tympany occurs after feeding.
 Treatment is surgical and consists in the removal of the tumors by
incision and ecraseur or otherwise. Thoracic œsophagean tumors are
usually inoperable.
 IMPACTION OF THE CROP. INGLUVIAL
INDIGESTION.
Gallinaceæ and Palmipeds. Causes; Overfeeding after privation; fermentation;
lack of water; green food in geese and chickens; food containing paralyzing
element. Symptoms; dull; motionless; erect plumes; drooping wings and head;
gapes; ejects liquid from bill; firm cervical swelling. Treatment; manipulation;
incision; surgical precautions. Convalescent feeding.
The cervical dilatation of the œsophagus known as the crop is well
developed in all granivorous birds, (Gallinaceæ, etc.;) and like the
macerating cavities of the ox (first two stomachs) is subject to
overdistension and paralysis. In the palmipeds (ducks, geese) there is
no distinct crop but in its place the cervical portion of the gullet has a
fusiform dilatation, and under given conditions this may be also the
seat of impaction.
Causes. The impaction may result from overfeeding when the bird
has been starved, or when it suddenly gains access to food of a
specially appetizing kind and to which it has been unaccustomed.
The crop like every other hollow viscus is rendered paretic by
overdistension. Then the food undergoes fermentation still further
distending the cavity, affecting the brain by reflex action, and
paralyzing the vagus and its peripheral branches in the lungs, heart,
stomach, liver, intestines, etc. When the food is dry as in the case of
beans, peas, bran, farinas, it may be a simple firm impaction which
the muscular walls of the crop are unable to break up or move
onward. When green food is taken there is often superadded the
additional evil of active fermentation from the great number and
activity of the bacterial ferments contained in it and the soft aqueous
fermentescible nature of the food (See tympany in ruminants).
Dupont states that young geese led out to fresh spring grass may lose
two-thirds of their number in a few hours from such overloading and
that some species of Carex and cynodon dactylon are particularly
injurious. Chickens also gorge the crop with clover, etc. In all such
cases, plants that contain a paralyzing principle like lolium
temulentum, ripening lolium perenne, chick vetch, etc., are to be
specially dreaded. (See Trichosoma Contortum).
 Symptoms. There are first dullness and sluggish movements,
followed by indisposition to move, the bird standing in one place
with ruffled feathers and drooping wings, and at intervals, projecting
the head forward with open beak and in some cases a little liquid is
rejected. If the bird is now caught and examined the crop is found to
be firmly distended, and more or less compressible or indentable
according to the nature of the food impacted. In most cases and
especially if the food has been green or aqueous, there is a certain
resiliency from the presence of gas outside the solid impacted mass.
Treatment. This must be in the line of seconding the physiological
efforts of regurgitation which is a normal and common act in birds.
The duck which has gulped a mouse half-way down the cervical part
of the œsophagus will readily disgorge it when he finds it impossible
to pass it further. The carnivorous birds often reject by vomiting the
indigestible debris such as feathers and bones, after all the more
soluble parts have been disposed of in the stomach. The pigeon even
feeds its young by disgorging into their open bills, the semi-digested
food and milk from its crop. Following these indications we must
break up the contents of the crop by manipulation and force them in
small masses upward into the bill and downward to the
proventriculus. The rejection by the bill may be further stimulated by
introducing the finger into the fauces to rouse the reflex active
emesis. Usually the crop can be quickly and satisfactorily emptied in
this way.
When this proves impossible there remains the operation of direct
incision through the walls of the crop and the evacuation of its
contents. This can be done by a pocket knife or even a pair of
scissors. The crop is punctured in its lower part and the incision is
continued upward as far as may be necessary to allow the escape of
the contents. Usually half an inch will suffice. Then the crop is
squeezed so as to press the contents through this opening and it is
emptied by a process of enucleation. If the contents are fibrous it
may be necessary to employ forceps to dislodge the material. The
empty crop may be washed out with tepid water, any food attached
to the raw edges of the wound must be removed and the skin stitched
accurately together. The wound rarely fails to heal by first intention.
To avoid stretching it, the food for a day or two should be restricted
to milk, gruels, or a little soft mash.
 Lerein notices jaundice as a sequel of impacted crop, and
recommends treatment by sulphate of soda in the water.
 TYMPANITIC INDIGESTION IN THE
RUMEN. BLOATING.
Definition. Susceptible Genera. Causes; gastric paresis, overloading, cold, fear,
exhaustion, poisons, fermentescible food,—new grain, leguminosæ, frosted
vegetables, ruminitis, foreign bodies in rumen, microbian ferments. Symptoms,
abdominal, general. Gases formed under different aliments—carbon dioxide,
marsh gas, hydrogen sulphide, nitrogen, oxygen. Lesions, rupture of rumen or
diaphragm, compression or rupture of liver or spleen, petechiæ, congestion of
lungs and right heart, of cutaneous and cerebral vessels. Prevention, avoid
indigestible and fermentescible aliments, correct adynamic conditions, tonics,
avoid injurious ferments, make alimentary transitions slowly. Treatment, exercise,
bath or douche of cold water, rubbing and kneading, rope round abdomen spirally,
gag in mouth, dragging on tongue, movement of a rope in fauces, probang,
stimulants, antiseptics, alkalies, ammonia, oil of turpentine, oil of peppermint,
alcohol, ether, pepper, ginger, soda, potash, lime, muriatic acid, carbolic acid,
creosote, creoline, sulphites, kerosene, chloride of lime, chlorine, tar, common salt,
hypochlorite of soda, magnesia, eserine, pilocarpin, barium chloride, colchicum,
lard, trochar, Epsom salts, rumenotomy. Treatment of diseased gullet, mediastinal
glands, stomach or intestines.
Definition. The condition is a combination of paresis of the rumen
and gaseous fermentation of its contents. The initial step may be the
paresis or in the more acute forms the fermentation.
Genera susceptible. While all ruminating animals are subject to
this disorder, it is much more frequent in cattle and sheep than in
goats.
Causes. It commences in paresis of the rumen in the weak,
debilitated, convalescent or starved animals which are suddenly put
on rich, and appetizing food. Hence it is common in animals that
break into a cornbin, a store of potatoes, a field of growing corn or
small grain, or that are turned out on green food in early spring.
Cadeac maintains that paresis of the rumen is the essential cause in
all cases, while the nature of the aliments ingested fills a secondary
and comparatively insignificant rôle. According to this view the
torpid stomach can neither relieve itself through regurgitation for
rumination, nor expel through the œsophagus the constantly
evolving gas which therefore distends the viscus to excess. In support
of this view may be adduced the occurrence of tympany through
fatigue, fear, cold, enlarged (tubercular) mediastinal glands pressing
on the gullet and vagus, obstruction of the œsophagus by a solid
body (choking), impaction of a morsel of solid food in the demicanal
of the calf as noticed by Schauber, and the cessation of the normal
vermicular movements of the rumen in connection with
inflammation of its coats, or extensive inflammation elsewhere or
finally of fever. Even in paralysis of the stomach by poisons like lead,
tympany may be a result. Cadeac attributes tympany following the
ingestion of green food wet with a shower, or drenched with dew, of
frosted potatoes or turnips, or of iced water, to the paralyzing action
of the cold on the rumen. This view is manifestly too extreme, as the
bloating occurs often after a warm summer shower, or after the
consumption of potatoes and other roots and tubers which have been
spoiled by frost but which are no longer at a low temperature when
consumed.
Tympany may also start from the ingestion of certain kinds of
food which are in a very fermentescible condition. Green food,
especially if the animal has been unaccustomed to it, is liable to act
in this way. Clover and especially the white and red varieties, lucern
(alfalfa), sainfoin, cowpea and other specially leafy plants, which
harbor an unusual number of microbian ferments, and which
contain in their substance a large amount of nitrogenous material
favorable to the nourishment of such ferments are particularly
dangerous in this respect. All of these are most dangerous when wet
with dew or when drying after a slight shower, partly no doubt at
times by reason of the chilling of the stomach, but mainly because
the ferments have been stimulated into activity by the presence of
abundance of moisture. Drenching and long continued rains are less
dangerous in this respect than the slight showers and heavy dews,
manifestly because the former wash off a large portion of the
microbes, which under a slight wetting multiply more abundantly.
Frosted articles act in a similar way, partly when still cold by the
chilling and paralyzing of the stomach, but cold or warm, by reason
of the special tendency of all frozen vegetables to undergo rapid
fermentation when thawed out. This is true of green food of all kinds
when covered by hoarfrost, of turnips, beets, potatoes, carrots,
apples, cabbage, etc., which have once been frozen, and of frosted
turnips and potato tops, though, in the case of the latter agent, a
narcotic principle is added.
In the case of Indian corn, the smaller cereal grains, and certain
leguminous plants (vetches, tares, peas, beans) which have the seed
fully formed but not yet quite hardened nor ripened, there is the
double action of a paralyzing constituent and an aliment that is
specially susceptible of fermentation.
Inflammation of the rumen, already quoted as a cause, may be
determined by hot as well as cold food, by irritant drugs and poisons,
and by narcotico-irritant and other acrid plants in fodder or pasture.
In the same way the inflammation caused by the introduction of
foreign bodies into the rumen, such as nails, tacks, needles, pins,
wires, knife blades, and masses of hair or wool may at times cause
tympany.
The two main causative factors, of paresis of the rumen on the one
side and of specially fermentescible food and a multiplicity of
microbian ferments on the other, must be recognized as more or less
operative in different cases, and in many instances their combined
action must be admitted. The tympany is the symptom and
culmination of a great variety of morbid causes and conditions, and
its prevention and treatment must correspondingly vary.
Symptoms. The whole left side of the abdomen being occupied by
the rumen, its distension leads to an uniform swelling of that side,
differing from that caused by simple excess of solid ingesta in being
more prominent high up between the last rib and the outer angle of
the ilium, and in giving out in this region a clear tympanitic or
drumlike resonance on percussion. It has also a tense resiliency, like
that of a distended bladder, easily pressed inward by the finger but
starting out to its rotundity the moment the pressure of the finger is
withdrawn. The distension caused by overloading with solids bulges
out lower down, is not resonant but dull or flat when percussed, and
yields like a mass of dough when pressed retaining the indentation of
the finger for some time. The swelling of tympany, when extreme,
rises above the level of the outer angle of the ilium and even of the
lumbar spines on the left side, and if no relief is obtained the right
side may undergo a similar distension.
Auscultation detects an active crepitation over the whole region of
the rumen, finer in some cases and coarser in others, according to
the activity of evolution and the size of the bubbles of gas. The
crepitation is especially coarse and loud in fermentation of green
food, and of spoiled potatoes or other tubers or roots.
In all acute or severe cases, there is anorexia, suspension of
rumination, and the normal movements of the compressed bowels
seem to be largely impaired, though the anus is protruded and a little
semi-liquid fæces or urine may be expelled at intervals. The
breathing is accelerated, short, and labored. The nostrils are dilated,
the nose extended, the face anxious, the eyes bloodshot and the back
arched. Froth may accumulate around the lips, or the mouth may be
held open with the tongue pendent. Sometimes a quantity of gas may
suddenly escape with a loud noise, but without securing permanent
relief. The heart beats are violent and accelerated, the pulse
increasingly small and finally imperceptible, and the visible mucous
membranes are congested and cyanotic. Pregnant females are very
liable to abort.
When the right flank as well as the left rises to the level of the
lumbar spines death is imminent, and this may take place as early as
fifteen or thirty minutes after the apparent onset of the attack. Death
may result from nervous shock, from suffocation, or from the
absorption of deleterious gases, or from all of these combined.
In the less acute cases the animal may live several hours before the
affection terminates in death or recovery. As a rule he stands as long
as he can and finally drops suddenly, the fall often leading to rupture
of the diaphragm or stomach, to protrusion of the rectum, or the
discharge of ingesta by the mouth and nose.
In still slighter cases relief comes through vomiting or more
commonly through frequent and abundant belching of gas, the
swelling of the flanks subsides, rumbling of the bowels may again be
heard, and usually there is a period of diarrhœa.
Gases present. When the rumen is punctured before or after death
so as to give exit to the gas in a fine stream it proves usually more or
less inflammable, the lighted jet burning with a bluish flame. The
usual inflammable ingredients are carbon monoxide, hydrogen
carbide (marsh gas) and hydrogen sulphide, yet the relative
proportion of the gases varies greatly with the nature of the food and
the amount of gas evolved, carbon dioxide being usually largely in
excess. The following table serves to illustrate the variability: