A Practical Guide to Palliative Care in Paediatrics

A practical guide to PALLIATIVE CARE in paediatrics

PAEDIATRIC PALLIATIVE CARE IS A CONCEPT OF CARE WHICH PROVIDES COORDINATED MULTIDISCIPLINARY CARE TO CHILDREN WITH A PROGRESSIVE LIFE-LIMITING CONDITION, AND THEIR FAMILIES.

THE CARE IS DELIVERED, WHERE POSSIBLE, IN THE ENVIRONMENT OF THE CHILD AND FAMILY’S CHOICE, AND PROVIDES PHYSICAL, SOCIAL, EMOTIONAL AND SPIRITUAL SUPPORT FOR THE CHILD, THEIR FAMILY, SCHOOL, COMMUNITY AND FRIENDS.

THE CARE INCLUDES GRIEF AND BEREAVEMENT SUPPORT FOR THE CHILD, FAMILY, SCHOOL AND COMMUNITY, DURING THE CHILD’S PALLIATIVE CARE AND FOLLOWING THEIR DEATH.

© Children’s Health Queensland Hospital and Health Service 2014

ISBN 978-1-925341-40-9 (eBook)

Published by Vivid Publishing

P.O. Box 948, Fremantle Western Australia 6959 www.vividpublishing.com.au

eBook conversion and distribution by Fontaine Publishing Group, Australia www.fontaine.com.au

Except as provided by the Copyright Act 1968, no part of this publication may be reproduced, shared or transmitted in any form or by any means without prior permission of the authors.

FOREWORD

The progress in Paediatric Palliative Care in Australia since the first publication of A Practical Guide to Paediatric Oncology Palliative Care in 1999, has been substantial with the continued development of clinical services throughout Australia, and the on-going review of the quality of those services through the National Standards Assessment Program, [NSAP (http://www.palliativecare.org.au/Standards/NSAP.aspx)] which provides a method for each service to benchmark. Further, the development has been towards delivering care at home, or as close to home as possible, as novel communication technologies and education initiatives evolve.

This edition, incorporating care for both children with cancer and non-cancer conditions, is testimony to its value as an education resource, empowering clinicians caring for children at home or as close to home as possible. Coordinated by the Paediatric Palliative Care Service at the Royal Children’s Hospital, Brisbane, it also represents the collaborative efforts of paediatric palliative care clinicians throughout Australia and New Zealand. As such, it is a national resource and a major contribution towards supporting dying children and their families to have the best quality of life.

Dr John Collins AM MB BS, PhD, FRACP

Clinical Associate Professor

Discipline Paediatrics and Child Health

Sydney Medical School

University of Sydney, Australia

&

Head of Department

Pain Medicine and Palliative Care

The Children’s Hospital at Westmead

Sydney, Australia

ACKNOWLEDGEMENTS

Members of the writing team, first edition – cancer, 1999

Dr Helen Irving

Kris Liebke

Dr Liane Lockwood

Michelle Noyes

Delma Pfingst

Tim Rogers

Special thanks to

–Drew Craker

–All who provided valuable comment on the document throughout its development.

–Health professionals who contributed to the research preceding the development of this guide.

–Members of local communities, who by their professional and personal support enable children to die in their local environment.

–Parents and children who have contributed to our recognition of the importance of paediatric palliative care.

–Queensland Health for funding this publication.

Additional contributors to the 2nd edition – cancer, 2009

Project Co-ordinators

Catherine Camden

Lynda Dunstan

Other Contributors

Dr John Collins, NSW

Dr Anthony Herbert, QLD

Peter Barclay, NSW

Elizabeth Heiner, NSW

Carol Quayle, VIC

Sara Fleming, SA

Dr Marianne Philips, WA

Jess Jamieson, NZ

Andrew Thompson, NZ

Dr Peter O’ Regan, QLD

With thanks to

Moggie Panther, NSW

The Australian and New Zealand Paediatric Palliative Care Reference Group

The second edition was supported by funding from the Australian Government, Department of Health and Aging under the Local Palliative Care Grant Round 2 funding.

Additional contributors to this 1st edition for both cancer and non-cancer conditions

Project Co-ordinators

Natalie Bradford

Senior Research Officer

Raelene Boyle Outreach Program

Centre for Online Health

University of Queensland

Brisbane QLD

Dr Anthony Herbert

Clinical Lead and Staff Specialist

Paediatric Palliative Care Service

Royal Children’s Hospital

Brisbane QLD

Lee-anne Pedersen

Nurse Practitioner

Paediatric Palliative Care Service

Royal Children’s Hospital

Brisbane QLD

Dr Helen Irving

Pre-Eminent Staff Specialist

Queensland Children’s Cancer Centre

Royal Children’s Hospital

Brisbane QLD

Other Contributors

Dr John Collins

Head, Department of Pain Medicine & Palliative Care

The Children’s Hospital

Westmead

Sydney NSW

Dr Lucy Cooke

Senior Staff Specialist

Deputy Director Neonatology

Mater Mother’s Hospital

Brisbane QLD

Dr Simon Cohen

Pediatrician and Pain Medicine

Specialist, Sydney Children’s

Hospital, Randwick

Bear Cottage, Manly

Sydney NSW

Leigh Donovan

Bereavement Coordinator

Paediatric Palliative Care Service

Royal Children’s Hospital

Brisbane QLD

Dr Ross Drake

Director and Specialist

Paediatric Palliative Care and Pain Medicine

Starship Children’s Hospital

Auckland NZ

Sara Fleming

Nurse Practitioner

Paediatric Palliative Care

Women’s & Children’s Hospital

Adelaide SA

Jude Frost

Clinical Nurse Consultant Palliative Care

Department of Pain Medicine & Palliative Care

The Children’s Hospital

Westmead

Sydney NSW

Alyson Gundry

Bereavement Coordinator

Paediatric Palliative Care Service

Royal Children’s Hospital

Brisbane QLD

Dr Deidre Hahn,

Consultation Physician in Paediatric Nephrology

Nephrology Service

The Children’s Hospital

at Westmead

Sydney NSW

Rachael Lawson

Acting Oncology Pharmacy Manager

Pharmacy Department

Royal Children’s Hospital

Brisbane QLD

Dr Martha Mherekumombe

Formerly Fellow

Pain Medicine and Palliative Care

The Children’s Hospital

at Westmead

Sydney NSW

Suzanne Momber

Oncology Clinical Nurse

Specialist

Oncology/Haematology/Transplant Unit

Princess Margaret Hospital for Children

Perth WA

Carol O’Ryan

Anglican Chaplaincy Coordinator

Royal Children’s and Royal

Brisbane and Women’s Hospitals

Brisbane QLD

Cindy Paardekooper

PEPA Project Manager

(Program of Experience in the Palliative Approach)

Darwin NT

Andrew Paton

Pharmacist

Pharmacy Department

Royal Children’s Hospital

Brisbane QLD

Dr Marianne Philips

Paediatric and Adolescent

Oncologist and Palliative Care Specialist

Princess Margaret Hospital for Children

Perth WA

Jo Ritchie

Quality Manager

Blood and Marrow Transplant

Service & Paediatric Palliative Care Service

Royal Children’s Hospital

Brisbane QLD

Dr Sharon Ryan

Staff Specialist – Palliative Care

John Hunter Children’s Hospital

Newcastle NSW

Susan Trethewie

Palliative Care Physician

Head of Department

Palliative Medicine Services

Sydney Children’s Hospital

Sydney NSW

Dr Peter Trnka

Paediatric Nephrologist

Queensland Child and Adolescent Renal Service (CARS)

Royal Children’s Hospital

Brisbane QLD

With thanks to

The Sporting Chance Cancer Foundation for their financial support for this edition.

CONTENTS

Foreword

Acknowledgements

Introduction

Quality of life

Place of care

Coordination of care

Psychosocial foundations of palliative care

Respect for the uniqueness of each family

Empowerment

Communication

Community perspectives

Spiritual, Religious and Cultural issues

Aboriginal and Torres Strait Islander considerations

Refugees and Asylum seekers

Schools

Symptom management

Pain

Analgesic agents

Primary analgesics

Breakthrough pain

Side effects and precautions of opioids

Secondary analgesics

Other therapies for pain

Non-pharmacological therapies

Gastrointestinal Symptoms

Oral problems

Nausea and vomiting

Constipation

Diarrhoea

Anorexia and cachexia

Feeding intolerance

Forgoing nutrition and hydration

Respiratory symptoms

Dyspnoea

Cough

Excess secretions

Anaemia and bleeding

Neurological symptoms

Anxiety

Seizures

Muscle spasm and myoclonus

Irritability and agitation

Acute dystonic crisis

Insomnia

Renal

Dermatology

Pruritus

Pressure ulcers

Other skin conditions

Perinatal palliative care

Conditions requiring perinatal palliative care

Steps in creating a birth plan

The dying process

Noisy/rattly breathing

Incontinence

Eye changes

Restlessness and agitation

Continuous subcutaneous infusion

Home care pack

Circulatory and respiratory changes

What to do when a child dies

Death at home

Death in hospital

Organ and tissue donation

Funerals

Bereavement

Grief and anticipatory grief

Bereavement support

Supporting the staff

Quality improvement in paediatric palliative care

Ethics in palliative care

Extent of supportive care

Advanced care plans

Double effect of drugs

Confidentiality, privacy and disclosure

Euthanasia

Resources

Appendices

Appendix 1. Palliative care action plan

Appendix 2. Guidelines for continuous subcutaneous infusions

Appendix 3. Syringe drivers and drug compatibilities

Appendix 4. Contents of home care pack

Appendix 5. Standards for providing quality palliative care for all Australians

Appendix 6. Clinical indicators for paediatric palliative care

Appendix 7. Commonly used drugs and doses

References

Order form

Notes

INTRODUCTION

Children are not expected to die. When faced with the news that a child has a progressive incurable disease it is natural for the child, family and health professionals to have numerous fears, concerns and questions about what will happen. The aim of this book is to highlight and address many of these issues.

DYING CHILDREN AND TEENAGERS

PARENTS AND SIBLINGS

HEALTH PROFESSIONALS AND SCHOOL TEACHERS

Palliative care as a speciality is gaining a reputation and recognition for the positive outcomes that this approach to care can deliver to patients and their families.¹, ² Paediatric palliative care from its outset has shared the same philosophy of care as the adult specialty, (e.g. multi-disciplinary care, maximizing supports at home and collaborative practice) while at the same time developing its own distinctive model.

For example, more than half of the patients a paediatric palliative care service care for will have non-cancer life-limiting conditions which are not seen in adult practice. Paediatric palliative care services will usually remain consultative, including the end of life phase of care, and collaborate closely with other health care teams involved in the child’s care.³ Furthermore, parental desire for resuscitation does not preclude involvement of a paediatric palliative care service in the care of a child with a life-limiting condition.⁴

The types of diseases encountered, the symptoms experienced, and the way children understand and communicate about their illnesses, are all different. These differences need to be acknowledged and inform the care provided to dying children and their families.

It is our experience that many children and families express a wish for their child to die at home in an environment that enhances their sense of security and normality. Dying at home can also increase the opportunity for parents, siblings, friends and family to assist with care, and thereby optimise the child’s quality of life.⁵ However, some children and families will prefer not to die at home and flexible options of care should be available such as, tertiary hospital, hospital closest to home, and if available, hospice.⁶ It is important that children and families receive care appropriate to their needs at the right time in the place of their choice (right care, right place, right time). Such choice in planning for location of care at end of life will reduce regrets that families may experience after their child dies.⁷

The geographical vastness of Australia often means that a child who is dying at home may be hundreds of kilometres from the tertiary paediatric hospital where they received their diagnosis, management and/or treatment. In these situations, health professionals from the child’s local community are required to meet the palliative care needs of the child and family. This can present a challenge to the health professional because of the relatively small number of children who die each year which limits the opportunity for regional and rural health professionals to develop experience in paediatric palliative care.

It is for this reason that A Practical Guide to Palliative Care in Paediatrics has been developed.

This guide addresses the many and varied aspects of caring for dying children and their families. This includes pain and symptom management, practical supports, psychosocial issues, communication and available resources. It is intended to be user-friendly and complementary to the existing knowledge and resources of the health professionals who use it.

Due to the success of the first and second editions of A Practical Guide to Palliative Care in Paediatric Oncology, the current edition has been produced to incorporate aspects of palliative care for both cancer and non-cancer life-limiting and life-threatening conditions. Contributions have been received from all states of Australia and New Zealand. It is hoped that this national approach will benefit children and families, and those caring for them, anywhere in the country – irrespective of diagnosis.

DEFINITIONS

For the purpose of this book, a child has been defined as a young person up to their 19th birthday. The specific upper age range of children managed by a paediatric palliative care service can vary between jurisdictions. There may be some flexibility depending on whether the child is currently being managed by a paediatric team or is still attending school.⁸

Children’s palliative care has been defined as:

Palliative care for children and young people with life-limiting conditions is an active and total approach to care, from the point of diagnosis or recognition, throughout the child’s life, death and beyond. It embraces physical, emotional, social and spiritual elements, and focuses on the enhancement of quality of life for the child/young person and support for the family. It includes management of distressing symptoms, provision of short breaks and care through death and bereavement. (Together for Short Lives, UK).⁸

The World Health Organisation offers a similar definition and makes the following additional points:

•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, psychological, 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 in children’s homes.⁹

A key concept, and one that is not always well understood, is that palliative care can be integrated with on-going efforts to cure or modify disease. The child and their family are supported to live as well as they can for as long as they can, within the limits imposed by the illness. In this context palliative care is about both living and dying.

Categories of life-limiting and life-threatening conditions

Life-limiting conditions are those for which there is no reasonable hope of cure and from which children or young people will die. Some of these conditions cause progressive deterioration over months to years, rendering the child increasingly dependent on parents and carers.⁸, ¹⁰

Life-threatening conditions are those for which curative treatment may be feasible but can fail (e.g. cancer). Children in long-term remission or following successful curative treatment are not included.⁸, ¹⁰

There are four broad groups of life-threatening and life-limiting conditions (see Table 1). Categorisation is not always easy and the examples used are not exhaustive. Some patients can be classified by more than one group. Diagnosis is only part of the process – the spectrum and severity of the disease, subsequent complications, and the needs of, and impact on the child and family need to be taken into account.

CONDITIONS

TABLE 1 Categories of life threatening and life limiting conditions¹⁰