Detailed contents
Contributors xvii
Abbreviations xix
SECTION I
Foundations of care
1. History and epidemiology 3
Lorna Fraser, Stephen Connor, and Joan Marston
Introduction 3
Definitions 3
Hospice 4
History 6
Epidemiology 6
Using these data to estimate the need for CPC services 15
Conclusion 16
References 16
2. Communication 17
Jennifer Mack and Bryan Sisk
Introduction 17
Communication as a foundation of palliative care 17
Barriers to effective communication 18
Establishing a relationship and the development of shared knowledge: Learning from the child and family 18
Establishing a relationship and the development of shared knowledge: Transmission of information to the child and family 20
Establishing a relationship and responding to emotions 21
Identifying values and making shared decisions 21
When should conversations about palliative care take place? 22
Communication across cultural and language differences 22
Communicating about prognosis 23
Conclusion 24
References 24
3. ‘Children are not small adults’—the distinctiveness of ethics in children 25
Robert Macauley and Richard Hain
Introduction 25
Decision-making—parental authority to request ‘harmful’ interventions—and ditto to request futile ones 26
Research in children 27
Principle of double effect 32
Euthanasia 33
Summary 34
References 34
4. Decision-making with children, young people, and parents 36
Myra Bluebond-Langner and Richard Langner
Introduction 36
Decision-making 36
The role of parents 38
Participation of CYP in decision-making 39
Clinical implications 41
In summary 42
References 42
5. Culture, spirituality, religion, and ritual 44
Erica Brown, Mary Ann Muckaden, and Nokuzola Mndende
Introduction 44
Definitions 44
Influence of religion, spirituality, and culture on children with life-limiting conditions 44
Worldviews, cycles, and meaning of life 45
The role of spirituality in children’s palliative care 46
The role of religion in children’s palliative care 46
Some faith and cultural traditions 46
Secular beliefs 50
Cultural and religious aspects in perinatal and neonatal palliative care 50
Chaplains and faith leaders 50
Cultural and religious aspects from the literature in
EOL care 50
Effect of religious and spiritual beliefs in treating physicians 51
Cross-cultural support 51
An example of care and spirituality from South Africa—a traditional African perspective 52
Conclusion 52
References 54
SECTION II
Child and family care
6. Assessment of the child and family 59
Nancy Contro and Jane Zimmerman
Introduction 59
Theoretical considerations 59
Practical considerations 60
Evidence-based considerations 63
Summary 65
References 65
7. Children’s views of death 66
Myra Bluebond-Langner and Ignasi Clemente
Introduction 66
Children’s views of death: A multi-dimensional, multi-faceted perspective 66
Views of death in children with life-limiting conditions and lifethreatening illnesses: Expressing their thoughts in words and deeds 67
Well siblings’ views of death: Comparisons with ill siblings 69
Talking with others about death: Conversations between ill children, their parents, siblings, and clinicians 70
Suggestions for discussions with children about death and dying 72
Talking about death with bereaved children: An additional note 73
Conclusion 73
Acknowledgements 73
References 73
8. The psychological impact of life-limiting conditions on the child 75
Jan Aldridge and Barbara M. Sourkes
Introduction 75
Emotional support 76
Clinical supervision 76
Psychological issues 76
Work with parents 77
Brothers and sisters 78
The child’s voice in decision-making 79
Awareness of impending death 79
Psychotherapy—a conceptual framework 81
A case study 82
Conclusion 85
Acknowledgement 86
References 86
9. Adolescents and young adults 87
Chana Korenblum and Finella Craig
Defining adolescence and young adulthood 87
The prevalence of LLC in AYAs 87
Adolescence as a transition phase 87
Normal adolescent development 87
Challenges to adolescent development in the palliative care population 87
Changing relationships with parents 88
Peer group identification 88
Independence 88
Developing a sexual identity 89
Spiritual identity 89
Psychological development 89
Supporting the transition to adulthood 89
Facilitating peer group interaction and independence 89
Support for sexual development 90
Spiritual support 90
Support for decision-making 90
Psychological support 91
Respite care 91
Advance care planning 91
Transition to adult services 92
The needs of professional staff and carers 93
Service development 93
Summary 93
References 94
10. Children expressing themselves 95
Amy Volans and Emma Brown
Introduction 95
Defining and evaluating creative therapies 96
Music therapy 96
Art therapy 96
Play and play therapy 96
Communication and play with infants and children with special communication needs 97
Working clinically with children using creative art therapies 98
Artistic achievements as expressive acts 99
Storytelling and narrative therapy 100
Working with children’s strengths and abilities 101
Nurturing fantasy and pleasurable imagination 101
Supporting the dying child and their family 102
Acknowledgement 102
References 102
11. Education and school 103
Sue Boucher
Introduction 103
Why educate children who have progressive life-limiting illnesses? 103
How illness disrupts education 104
Providing educational opportunities for life-limited children 104
Virtual learning environments (VLE) 104
Principles of good practice to support life-limited children’s access school education 105
Conclusion 110
References 110
12. Impact on the family 111
Veronica Dussel and Barbara Jones with Kevin O’Brien, and Melissa Williams-Platt
Introduction 111
What do we call family? 111
Family systems 111
Family and culture 112
Effects of a child’s LLC on the family 112
Living with the LLC or illness 113
Family adjustments to a child’s LLC 114
How can we help families? 116
The wider context of care 119
A challenge for the future 120
Acknowledgements 124
References 124
13. Bereavement 126
Sara Portnoy and Lori Ives-Baine
Introduction 126
Impact on families 126
Impact on children and young people 127
Manifestations of grieving adults and children 127
Impact on schools 128
Impact on hospitals, community providers, and hospices 128
Helping theories of bereavement make sense 129
Risk and resilience seesaw 130
Time passing 131
Concerned curiosity: A way of talking to bereaved families 131
What helps grieving families? 131
When families may need more help 133
Conclusion 133
Acknowledgements 134
References 134
SECTION III
Symptom care
14. Overview of symptoms and their assessment in life-limiting illness 137
Dilini Rajapakse and Maggie Comac
Introduction 137
The importance of accurate symptom assessment 137
The prevalence of symptoms in children with life-limiting illness 138
The principles of symptom evaluation 140
Symptom measurement in children 141
Challenges to comprehensive symptom evaluation 142
Novel approaches to symptom evaluation 142
Symptom evaluation in paediatric palliative care: Areas for further study 143
References 144
15. Using medication in children’s palliative care 145
Andy Gray, Jane Riddin, and Richard Hain
Introduction 145
Pharmacokinetic considerations in children 145
Distribution 147
Transport 147
Biotransformation and metabolism 147
Distribution 148
Elimination 149
Therapeutic research in children 149
Summary 150
References 151
16. Introduction to pain 153
Antoine Bioy and Chantal Wood
Introduction 153
The history of pain and the actual definition of pain 153
What is pain? 155
The neurophysiology of pain 155
Excitatory mechanisms 156
Different types of pain 157
Pain and the child in palliative care 157
Assessment of the pain 160
The relationship between the carer and the patient who is in pain 161
Conclusion 162
References 163
17. Multimodal analgesia in paediatric palliative care 165
Stefan J. Friedrichsdorf
Introduction 165
Multimodal analgesia 165
Pain in children with SNI 166
Multimodal analgesia 167
Procedural pain and chronic pain treatment 169
Conclusion 170
References 171
18. Opioids and the World Health Organization pain ladder 176
Manuel Rigal, Ricardo Martino, and Richard Hain
Introduction 176
Opioids: Their power and range 176
World Health Organization (WHO) approach to pain management 178
‘By the child’: Taking a pain history 179
‘By the clock’: Regular opioids, breakthrough opioids, and the relationship between them 182
‘By the right route’ 182
Special situations 184
Summary 185
References 186
19. Difficult pain: Adjuvants or co-analgesics 188
Renee McCulloch and Charles Berde
Introduction 188
Combination pharmacotherapy 188
Visceral hyperalgesia 194
Deep tissue pain 196
References 200
20. Integrative approach to pain and other symptoms 202
David M. Steinhorn
Introduction 202
Goals of integrative therapy 202
Why should we include integrative therapies in palliative and hospice care? 203
What are integrative therapies? 203
Use of integrative approaches in paediatric palliative care 204
Summary 211
References 211
21. Gastrointestinal and liver-related symptoms in paediatric palliative care 214
Jo Laddie, Alta Terblanche, and Michelle Meiring
Introduction 214
Common GIT symptoms in children’s palliative care 214
Nausea and vomiting 216
Constipation 218
Diarrhoea 220
Other symptoms 221
Intestinal failure 224
Chronic liver disease 227
Conclusion 229
References 229
22. Feeding, cachexia, and malnutrition in children’s palliative care 231
Sanjay Mahant, Michelle Meiring, and Adam Rapoport
Introduction 231
Definitions 231
FTT and feeding difficulties 231
Cachexia and anorexia 237
Malnutrition 240
Pathophysiology and management 240
Conclusions 242
References 242
23. Neurological and neuromuscular conditions and symptoms 244
Jori F. Bogetz and Julie M. Hauer
Introduction 244
A palliative approach to neurological and neuromuscular conditions in children 244
Specific conditions affecting the nervous system 247
Symptoms of neurological impairment and neuro-specific symptoms 248
Non-pharmacological management 248
Pain, agitation, and irritability 248
Autonomic dysfunction 249
Seizures 250
Spasticity and muscle spasms 251
Dystonia 252
Chorea 252
Myoclonus 253
Medication toxicities 253
Sleep 253
Fatigue 253
Conclusion 254
References 254
24. Depression, anxiety, and delirium 255
Pamela J. Mosher and Anna C. Muriel
Introduction 255
Depression 255
Ketamine 260
Anxiety 260
Diagnostic tools 261
Important details concerning anti-anxiety medications in CYP 261
Cannabinoids 262
Irritability and anger 262
Delirium 262
Mania 264
Conclusion 264
References 266
25. Cardiorespiratory symptoms 267
Emily Harrop and Roxanne Kirsch
Introduction 267
Cough 271
Tachycardia, palpitations, chest pain 273
Fatigue, irritability, feeding intolerance 274
The role of mechanical circulatory support 274
Compassionate discontinuation of ventilator or MCS 276
Case 25.1 277
Case 25.2 277
Case 25.3 278
Conclusion 278
References 278
26. Skin symptoms 280
Carol Hlela, Rene Albertyn, and Michelle Meiring
Introduction 280
Life-threatening primary skin conditions: Congenital 280
Life-threatening primary skin conditions: Acquired 285
Other life-threatening acquired skin disorders that would benefit from a palliative care approach include 288
Psychosocial care and support for patients with life-threatening primary skin conditions 289
Secondary skin complications (dermatological symptoms in children with life-limiting disorders) 291
Fungating wounds and pressures sores 292
Conclusion 293
References 294
27. Haematological symptoms 296
Mei-Yoke Chan and Kevin Weingarten
Introduction 296
Symptoms associated with anaemia 296
Management of symptomatic anaemia in palliative care 297
Thrombosis and its management 301
Ethical issues related to the transfusion of blood products at end of life 301
Summary 302
References 302
28. Palliative care for children with communicable illnesses 304
Michelle Meiring and Tonya Arscott-Mills
Introduction 304
Life-threatening and life-limiting communicable diseases in children 304
ACT I: Potentially curable infections 305
Measles 305
Acute hepatitis 308
Severe bacterial illnesses (SBI) 308
TB 309
Malaria 309
ACT II: Life-limiting but non-progressive (with treatment) 310
ACT III: Progressive and non-curable diseases 312
MDR-TB and XDR-TB 313
ACT IV: Non-progressive but irreversible damage—often associated severe disability 315
Zika virus infection 316
Palliative care in neglected tropical diseases (NTDs) 317
Conclusions 319
References 319
SECTION IV
Delivery of care
29. Perinatal palliative care 325
Áine Ni Laoire, Daniel Nuzum, Maeve O’Reilly, Marie Twomey, Keelin O’Donoghue, and Mary Devins
Background 325
Ethical considerations 325
Diagnosis 326
Types of antenatal diagnosis 326
Palliative care approach to pregnancy 327
Care 329
Investigation and follow-up 330
Summary 331
References 331
30. Intensive care units 332
Brian S. Carter
Introduction 332
The role of PPC in the PICU 333
Who should receive palliative care in the PICU? 333
When children die in the PICU 333
Communication issues 335
Family-centered care in the PICU 336
Providing and respecting family choice with regard to place of care 337
Transferring a child to home or hospice for withdrawal of mechanical ventilation 338
Summary 338
Acknowledgement 339
References 339
31. Planning care 341
Michelle Grunauer and Jenny Hynson
Introduction 341
Benefits of planning in children’s palliative care (CPC) 341
Palliative care planning across the continuum of disease 342
Possible triggers for care planning 342
ACP 343
Barriers to ACP 344
Approaching ACP discussions 345
Patient assessment 345
Clinical circumstances 345
Place of care 347
Special considerations 348
Palliative care in humanitarian disasters 350
Conclusion 350
References 350
32. Care in the final hours and days 352
Dawn Davies and Justin Baker
Introduction 352
Changes in the goals of treatment 352
Requests for hastened death 354
Anticipating likely symptoms 354
Lines of communication 354
Palliative care emergencies 355
Setting for care in the final phase of life 357
References 359
33. Delivering care around the world 361
Julia Downing and Joan Marston
Introduction 361
The history of CPC 361
Identifying the need for CPC globally 363
The status of CPC globally 363
Challenges to the provision of CPC globally 363
CPC in LMICs 365
Developing CPC globally 366
Conclusion 371
References 371
34. Healthcare providers’ responses to the death of a child 373
Danai Papadatou
The myth 373
The reality 373
Aspects of healthcare providers’ suffering 375
A model of healthcare providers’ grieving process 375
Conclusion 380
References 380
35. Teamwork 382
Jan Aldridge and Pat Carragher
Introduction 382
Thinking about teams 382
Limitations of teams? 383
Delivery of paediatric palliative care 384
Key challenges 384
Communication 384
Managing conflict 385
Conflict and caring in paediatric palliative care 386
Leadership 387
Reflective practice 387
A secure base in the face of uncertainty and loss 388
Conclusion 388
References 388
36. Education 390
Fiona Rawlinson and Michelle Meiring
Introduction: Why are education and training important? 390
Who needs education and training? 390
How should education and training be delivered? 392
What should education and training in PC for children include? 397
How do we measure effectiveness of children’s PC education? 398
Summary 399
References 399
37. Quality improvement in paediatric hospice and palliative care 401
Susan Blacker and Rachel Thienprayoon
Introduction 401
Quality in healthcare 401
Quality in hospice and palliative care 401
Embarking on QI: Translating knowledge into practice 402
Implementing change 403
Challenges and opportunities: The future of QI in paediatric palliative care 406
Conclusions 407
QI resources 407
References 408
38. Research in children’s palliative care 410
Harold Siden and Kimberley Widger
Introduction 410
Importance of CPC research and priority areas 410
Challenges to CPC research 410
Strategies to overcome challenges 412
Advice to novice and reluctant researchers 417
Future directions 417
References 417
Appendices
The Association of Paediatric Palliative Medicine Master Formulary, Fifth Edition, 2020 419
Appendix 1: Formulary 421
Appendix 2: Morphine equivalence single dose 481
Appendix 3: SC infusion drug compatibility 482
Appendix 4: Gabapentin to pregabalin switch for neuropathic pain 483
Appendix 5: Benzodiazepines 484
References 486
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
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
A&E accident and emergency
a2-d alpha 2 delta
AAHPM American Academy of Hospice and Palliative Medicine
AAP American Academy of Pediatrics
ACE-I angiotensin-converting enzyme inhibitors
ACh acetylcholine
ACP advanced care plan
ACT acceptance and commitment therapy
ACT activated coagulation time
ACT Association for Children with Life-Threatening or Terminal Conditions
ACT I: potentially curable infections
ACT II: life-limiting but non-progressive (with treatment)
ACT III: progressive and non-curable diseases
ACT IV: non-progressive but irreversible damage— often associated with disability
ADHD attention deficit hyperactivity disorder
AgRP agouti-related peptide
AIDS acquired immunodeficiency syndrome
ALL acute lymphoblastic leukaemia
ALT alanine aminotransferase
AM anthroposophical medicine
AND allow natural death
ANG acute necrotizing gingivitis
ANH artificial, or nutrition, and hydration
Annals ATS Annals of the American Thoracic Society
APCA African Palliative Care Association
APPM Association of Paediatric Palliative Medicine
ARE evidence (research or clinical consensus) with adults
ART antiretroviral therapy
ARV antiretroviral medicines
AST aspartate aminotransferase
ASyMS© Advanced Symptom Management System
AYAs adolescents and young adults
BID/BD twice daily
BNF British National Formulary
BNFC British National Formulary for Children
BSFS Bristol Stool Form Scale
BTX botulinum toxin
BW body weight
CAM complementary and alternative medicine
CAM confusion assessment method
CAPD Cornell Assessment of Pediatric Delirium
CART cocaine and amphetamine-regulated transcript
CBC Child Behavior Checklist
CBD cannabidiol
CBT cognitive behavioural therapy
CC cut into quarters
CD child dose
CDI Child Depression Inventory
CF cystic fibrosis
CFS cutaneous field stimulation
CHI Children’s Hospice International
CHIPRA Children’s Health Insurance Program Reauthorazation Act
ChiSP Children in Scotland Requiring Palliative Care
CINV chemotherapy induced nausea and vomiting
CMV cytomegalovirus
CNPCC Canadian Network of Palliative Care for Children
CNS central nervous system
COMT catechol-O-methyltransferase
CorGA corrected gestational age
COX-1 Cyclooxygenase-1
COX-2 Cyclooxygenase-2
CP cerebral palsy
CPAC Center to Advance Palliative Care
CPAP continuous positive airway pressure
CPC children’s palliative care
CPR cardiopulmonary and pharmacological resuscitation
CQI continuous quality improvement
CRC Convention on the Rights of the Child
CSCI continuous subcutaneous infusion
CSF cerebrospinal fluid
CT computerized tomography
CVL central venous line
CYP children and young people
D2 dopamine
DDEB dominant epidermolysis bullosa
DEBRA International Dystrophic Epidermolysis Bullosa Research Association International
DEXA dexamethasone
DIC disseminated intravascular coagulation
DM delirious mania
DMC decision-making capacity
DMD Duchenne muscular dystrophy
DNAR do not attempt resuscitation/DNR do not resuscitate
DRS delirium rating scale
DR-TB drug resistant TB
DSM-5 Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition)
DVT Deep vein thrombosis
EAPC European Association for Palliative Care
EB epidermolysis bullosa
EBS epidermolysis bullosa simplex
ECG electrocardiogram
ECMO extracorporeal membrane oxygenation
ECUs Ebola care units
EEG electroencephalogram
EKG electrocardiograms
EMLA eutectic mixture of local anaesthetics
EOL end of life
EPEC- Education in Palliative and End-of-life
Pediatrics Care-Pediatrics
EPS extrapyramidal symptoms
ESA erythropoiesis-stimulating agents
ESPGHAN European Society for Pediatric Gastroenterology, Hepatology, and Nutrition
ET endotracheal tube
ETCs Ebola treatment centres
EVD Ebola virus disease/external ventricular drain
FDA US Food and Drug Administration
FFA fatal foetal anomaly
FICA faith beliefs, importance influence community assessment
FLACC face, legs, activity, cry, consolability
fMRI functional magnetic resonance imaging scan
FTT failure to thrive
G/GT gastronomy
GABA γ-aminobutyric acid
GABAA γ-aminobutyric acid type A
GAPRI Global Access to Pain Relief Initiative
GBS group B streptococcal
GCPQA Global Palliative Care Quality Alliance
GCS Glasgow coma scale
GERD gastro-oesophageal reflux
GFR glomerular filtration rate
GGT gamma-glutamyl transferase
GI/GIT gastrointestinal (tract)
GJ gastrojejunostomy
GNP gross national product
GOR gastro-oesophageal reflux
GORD gastro-oesophageal reflux disease
G-protein guanine nucleotide-binding proteins
H histamine
HAART highly active antiretroviral therapy
HBV hepatitis B
HCPs health-care professionals
HFA hydrofluoroalkane
HI harlequin ichthyosis
HIC high-income countries
HICs health information communities
HIV human immunodeficiency virus
HLA human leukocyte antigens
HOOF home oxygen order forms
HOPE H-sources
HPCA Hospice Palliative Care Association of South Africa
HPN Home Parenteral Nutrition
HPNA Hospice and Palliative Nurses Association
HRH reflux-related hospitalization rates
IAHPC International Association of Hospice and Palliative Care
ICPCN International Children’s Palliative Care Network
ICU intensive care unit
IDC-0 International Classification of Diseases (Version 10)
IEM inborn errors of metabolism
IFALD intestinal failure associated liver disease
IFM immunofluorescence antigen mapping
IFN-g Interferon gamma
IHME Institute for Health Metrics and Evaluation Il-6 Interleukin-6
IM integrative medicine/intramuscular
IMCI Integrative Management of Childhood Illnesses programme
IMOC integrated model of care
INF intranasal fentanyl
INR international normalized ratio
IOM Institute of Medicine
IRBs institutional review boards
IRIS immune reconstitution illness syndrome
ISAP International Association for the Study of Pain
ISMAR Innsbruck Sensory Motor Activator and Regulator
IV intravenous
J jejunostomy
JEB junctional epidermolysis bullosa
JMML juvenile myelomonocytic leukaemia
KOR Kappa-receptor opioid
LCSW licenced clinical social worker
LDLT living donation liver transplantation
LETM longitudinally extensive transverse myelitis
LIP lymphocytic interstitial pneumonitis
LLC life-limiting conditions
LMCI low- and middle-income countries
LMWH low-molecular-weight heparin
LSMT life-sustaining medical therapies
LST life-sustaining interventions
LTC life-threatening conditions
LTV long-term ventilation
M3G morphine 3-glucuronide
M3G morphine-3-glucuronide
M6G morphine-6-glucuronide
MAMI management of malnutrition in infants
MAOIs monoamine oxidase inhibitors
MBC measurement-based care
MBO malignant bowel obstruction
MBSR mindfulness-based stress reduction
MCQs multiple choice questions
MCS mechanical circulatory support
MDAS Memorial Delirium Assessment Scale
MDI metered dose inhaler
MDR-TB multi-drug resistant TB
MDR-TB multi-drug-resistant tuberculosis
MDT multidisciplinary teams
MHRA Medical and Health Research Association
MOOCs massive open online courses
MOR micro opioid receptor
MPA medroxyprogesterone acetate
MRC British Medical Research Council
MRI magnetic resonance imaging
MRP multidrug resistance protein
MSAS Memorial Symptom Assessment Scale
MST morphine sulphate tablets/multiple subpial transection
MTB mycobacterium tuberculosis
MTBC mycobacterium tuberculosis complex
MUPS medically unexplained physical symptoms
N&V nausea and vomiting
NA noradrenaline
NaCL sodium chloride
NASPGHAN The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition
NASW National Association of Social Workers
NBM nil by mouth
NCP National Consensus Project
NE norepinephrine
NEC necrotizing enterocolitis
NeuPSIG Special Interest Group on Neuropathic Pain
NF-kB nuclear factor-kB
NG nasal-gastronomy/nasogastric
NGF nerve growth factor
NGOs nongovernmental organizations
NGT nasogastric tube
NHPCO National Hospice and Palliative Care Organization
NICU neonatal intensive care unit
NIPPV noninvasive positive pressure ventilation
NK1 and NK2 neurokinin receptors
NMDA N-methyl-D-aspartate
NNH numbers needed to harm
NNT numbers needed to treat
NO nitrous oxide
NoRE no published evidence but has clinical consensus
NP neuropathic pain
N-PASS the neonatal pain, agitation, and sedation scale
NPCRC National Palliative Care Research Center
NPY neuropeptide Y
NQF National Quality Forum
NRM nucleus raphe magnus
NRU Nutritional Rehabilitation Unit
NSAID non-steroidal anti-inflammatory drug
NTDs neglected tropical diseases
OCT1 ondansetron
ODT oral dissolving tablet
OIH opioid-induced hyperalgesia
OM oral morphine
OME oral morphine equivalence
OR opioid receptors
OROS osmotic-release oral delivery system
ORS oral rehydration solution
PACT Paediatric Advanced Care Team
PACT patient-aligned care team
PAG periaqueductal grey
PaPaS Paediatric Palliative Screening Scale
PAS p-aminosalicylic acid
PC patient care/palliative care
PCA/NCA patient/nurse controlled analgesia
PCQN The Palliative Care Quality Network
PCQN-Peds Palliative Care Quality Network Paediatrics
PCRA patient controlled regional analgesia
PCRN Pediatric Palliative Care Research Network
PCV pneumococcal virus
PDE principle of double effect
PEAT Palliative Education Assessment tool PedPalASCNet
PedsQL Pediatric Quality of Life Inventory
PEG percutaneous endoscopic gastrostomy
PG pyoderma gangrenosum
PHQ Patient Health Questionnaire (modified for adolescents)
PHQ-9 Patient Health Questionnaire
PHRs personal health records
PHS public health strategy
PICC peripherally inserted central catheter
PICU paediatric intensive care unit
PICU psychiatric intensive care unit
PJP pneumocystis jiroveci pneumonia
PKU phenylketonuria
PMNS post-malaria neurological syndrome
PMTCT prevention of mother to child transmission
PNALD parenteral nutrition associated liver disease
PNPC perinatal palliative care
PO by mouth
POM prescription only medication
POMC proopiomelanocortin
PONV post-operative nausea and vomiting
PORTAGE Pediatric Oncology Roundtable to Transform Access to Global Essentials
PPC paediatric palliative care
PPE personal protective equipment
PPI proton-pump inhibitor
PQRST P = precipitating or relieving factors, Q = quality, R = radiation, S = severity, T= timing
PR/F Propofol-Remifantanil
PRN pro re nata: as needed
PROM patient reported outcome measures
PST palliative sedation therapy
PTSD post-traumatic stress disorder
PUVA psoralen ultraviolet A
QA quality assurance
QAM every morning
QHS every night at bedtime
QI quality improvement
QID four times a day
QIDA Quality Improvement Data Aggregator vs4`
QOL quality of life
QoLA Care Quality of Life and Advanced Care
QST quantitative sensory testing
QT qualification test interval seen in an electrocardiogram
RASS Richmond Agitation–Sedation Scale
RCPCH Royal College of Paediatrics and Child Health
RCT randomized controlled trial
RDEB recessive dystrophic epidermolysis bullosa
RHD rheumatic heart disease
RIF resistance to rifampin
RP relative potency
RUTF ready-to-use therapeutic food
Rx medical prescription
SAM severe acute malnutrition
SBI severe bacterial illnesses
SBS short-bowel syndrome/State Behavioral Scale
SC subcutaneous
SCAR spectrum of severe cutaneous reactions
SDGs sustainable development goals
SENCO Special Educational Needs Coordinator
SEPC self-efficacy in palliative care scale
SHS serious health-related suffering
SMA spinal muscular atrophy
SNI severe neurological impairment
SNRI serotonin and noradrenaline reuptake inhibitors
SNRIs serotonin noradrenaline reuptake inhibitors
SPC summary of product characteristics
SPIKES Set up the conversation, assess the patient’s Perceptions, and obtain the patient’s Invitation, all before providing information and Knowledge. As information is transmitted, emotions are addressed with Empathy, and next Steps are planned for future care and conversations
SPPCS specialized paediatric palliative care services
SQ subcutaneous
SRE strong research evidence
SRQ self-reporting questionnaire
SSPE subacute sclerosing panencephalitis
SSRI selective serotonin reuptake inhibitors
STAR*D Sequenced Treatment Alternatives to Relieve Depression
STORCH syphilis, toxoplasmosis, rubella, cytomegalovirus, and herpesvirus
SVCO superior vena cava obstruction syndrome
TAD transient acantholytic dermatosis
TAH total artificial heart
TB tuberculosis
TBAN tuberculosis acute neurology
TBI traumatic brain injury
TBM tuberculosis meningitis
TBSA total body surface area
TCA tricyclic antidepressant
TCH Tygerberg Children’s Hospital
TDS to be taken three times daily
TEN toxic epidermal necrolysis
TENS transcutaneous electrical nerve stimulation
TfFSL Together for Short Lives
THC tetrahydrocannabinol
TIME Toolkit of Instruments to Measure End-of-Life Care
TIPS transjugular intrahepatic portosystemic shunt
TNF tumour necrosis factor
TORCH toxoplasmosis, rubella cytomegalovirus, herpes simplex, and HIV
TPN parenteral feeds
TQS to be taken four times daily
TRPV1 transient potential vanilloid receptor
TTS tarsal tunnel syndrome
UHC universal health coverage
UMN upper motor neuron
UN United Nations
UNICEF United Nations International Children’s Emergency Fund
USBS ultra-short bowel syndrome
UTI urinary tract infection
UVA ultraviolet A
UVB ultraviolet B
VAD ventricular assist devices
VFFS video-fluoroscopic feeding study
VLE virtual learning environment
VP ventriculoperitoneal
VPS ventriculoperitoneal shunt
VR virtual reality
WDR wide dynamic range
WFI (sterile) water for injection
WHA World Health Assembly
WHO World Health Organization
WHPCA Worldwide Palliative Care Alliance
WRE some weak research evidence
XDR-TB extremely drug-resistant tuberculosis
