Palliative Care in Amyotrophic Lateral Sclerosis: From Diagnosis to Bereavement

337 Pages·2014·3.515 MB·English

by  David Oliver, Gian Domenico Borasio, Wendy Johnston

Palliative Care
in Amyotrophic
Lateral Sclerosis
From Diagnosis
to Bereavement
THIRD EDITION
Edited by
David Oliver
Consultant in Palliative Medicine
Wisdom Hospice, Rochester
and Honorary Reader
University of Kent, UK
Gian Domenico Borasio
Chair in Palliative Medicine
Director, Palliative Care Service
Centre Hospitalier Universitaire Vaudois
University of Lausanne, Switzerland
Wendy Johnston
Professor of Neurology
Director, ALS Programme
University of Alberta, Canada
1
1
Great Clarendon Street, Oxford, OX2 6DP,
United Kingdom
Oxford University Press is a department of the University of Oxford.
It furthers the University’s objective of excellence in research, scholarship,
and education by publishing worldwide. Oxford is a registered trade mark of
Oxford University Press in the UK and in certain other countries
© Oxford University Press 2014
The moral rights of the authors have been asserted
Second Edition published in 2006
9780199212934 HB 9780198570486 PB
First Edition published in 2000
9780192631664
Impression: 1
All rights reserved. No part of this publication may be reproduced, stored in
a retrieval system, or transmitted, in any form or by any means, without the
prior permission in writing of Oxford University Press, or as expressly permitted
by law, by licence or under terms agreed with the appropriate reprographics
rights organization. Enquiries concerning reproduction outside the scope of the
above should be sent to the Rights Department, Oxford University Press, at the
address above
You must not circulate this work in any other form
and you must impose this same condition on any acquirer
Published in the United States of America by Oxford University Press
198 Madison Avenue, New York, NY 10016, United States of America
British Library Cataloguing in Publication Data
Data available
Library of Congress Control Number: 2013950545
ISBN 978–0–19–968602–5
Printed and bound by
CPI Group (UK) Ltd, Croydon, CR0 4YY
Oxford University Press makes no representation, express or implied, that the
drug dosages in this book are correct. Readers must therefore always check
the product information and clinical procedures with the most up-to-date
published product information and data sheets provided by the manufacturers
and the most recent codes of conduct and safety regulations. The authors and
the publishers do not accept responsibility or legal liability for any errors in the
text or for the misuse or misapplication of material in this work. Except where
otherwise stated, drug dosages and recommendations are for the non-pregnant
adult who is not breast-feeding
Links to third party websites are provided by Oxford in good faith and
for information only. Oxford disclaims any responsibility for the materials
contained in any third party website referenced in this work.
Preface
Although there is increasing understanding of the genetic basis of amyotrophic
lateral sclerosis (ALS)/motor neuron disease (MND) and the basic science of
the disease, currently available treatments offer only a modest increase in life
expectancy. Therefore, a palliative approach to caring for the patient and their
family is at the centre of good clinical practice in ALS. This book considers the
various aspects of palliative care for patients with ALS, based on the most recent
research in the field. This edition has been updated to include research and
publications from the past 7 years, as well as new chapters on complementary
medicine and family experience.
All contributors are clinicians involved in the care of people with ALS who
provide clear guidelines of the care involved throughout the disease process,
from imparting the diagnosis to bereavement. The international authorship
allows a comparison of the differences across cultures, social circumstances,
healthcare systems, and nations. The palliative approach to the care in ALS is
outlined and the role of the multidisciplinary team emphasized. This book
adopts an evidence-based approach, grounded in the day to day care of patients.
A short case history starts all chapters, focusing the reader on the clinical appli-
cation of the research to be discussed.
The authors aim to provide a reference for all involved in the care of patients
with ALS, including neurologists, primary-care physicians, rehabilitation special-
ists, allied health professionals, and particularly multidisciplinary palliative care
teams. The care of a person with ALS, and their family, is often a challenge, but one
that all should take up so that patients can live their lives as fully as possible.
Throughout the book the term ‘amyotrophic lateral sclerosis’ (ALS) has
been used rather than ‘motor neuron disease’, as the name ALS is recognized
internationally.
We would like to thank all involved in the production of this volume, especially
Caroline Smith from Oxford University Press, Professor Declan Walsh, who was
a co-editor of the first and second editions, and all our colleagues who have
helped us with comments and suggestions throughout the publication process.
DJO, Rochester, UK
GDB, Lausanne, Switzerland
WJ, Edmonton, Canada
June 2013

Contents
List of abbreviations vii
List of contributors ix
1 Amyotrophic lateral sclerosis/motor neuron disease 1
Christopher E. Shaw, Annika Quinn, and Emma Daniel
2 Palliative care 21
David Oliver
3 Communication: breaking the news 33
Richard Sloan and Gian Domenico Borasio
4 Decision-making 49
Wendy Johnston
5 Advance directives 59
Gian Domenico Borasio and Raymond Voltz
6 Respiratory complications 65
Deborah Gelinas
7 Control of symptoms: dysphagia 91
Edith Wagner-Sonntag
8 Control of symptoms: cognitive dysfunction 107
Laura H. Goldstein
9 Pain, psychological distress, and other symptoms 127
David Oliver, Gian Domenico Borasio, and Wendy Johnston
10 Psychosocial care 145
Sue Smith and Maria Wasner
11 Spiritual care 171
Robert Lambert
12 Multidisciplinary care: physiotherapy 187
Ulrike Hammerbeck and Emily Jay
13 Multidisciplinary care: occupational therapy 203
Chris Kingsnorth and Sarah Lavender
14 Multidisciplinary care: speech and language therapy 215
Amanda Scott and Maryanne McPhee
vi CONTENTS
15 Multidisciplinary care: nursing care 233
Dallas A. Forshew
16 Complementary and alternative medicine 245
Gregory T. Carter, Sunil Kumar Aggarwal, Michael Weiss,
and Richard S. Bedlack
17 End-of-life care: ethical issues 257
Leo McCluskey, Lauren Elman, and Wendy Johnston
18 End-of-life care in ALS 277
Nigel Sykes
19 Bereavement 293
Joy Kelly
20 No time to waste: a family’s journey from diagnosis to
bereavement 307
Marika Warren, Michelle Warren, and Douglas Warren
Appendix: List of useful websites for ALS patients 315
Index 317
List of abbreviations
AAC augmentative and alternative FUS fused in sarcoma
communication FVC forced vital capacity
AAN American Academy of GABA gamma-amino-butyric acid
Neurology
GERD gastro-oesophageal reflux
AD advance directive disease
AFO ankle–foot orthosis IGF insulin-like growth factor
ALS amyotrophic lateral sclerosis IPPB Intermittent positive pressure
ALS-bi ALS behavioural impairment breathing
ALSFRS ALS Functional Rating Scale IV intravenous
BDNF brain-derived neurotrophic LMN lower motor neuron
factor LTMV long-term mechanical
BMI body mass index ventilation
bp base pair MBS modified barium swallow
bvFTD behavioural variant of FTD MEP maximum expiratory pressure
CAM complementary and alternative MI-E mechanical insufflator–
medicine exsufflator
C9orf72 chromosome 9 open reading MIP maximum inspiratory pressure
frame 72 MMN multifocal motor neuropathy
CCTR Cochrane Controlled Trials MNDA [UK] Motor Neurone Disease
Register Association
CNS central nervous system MRI magnetic resonance imaging
CNTF ciliary neurotrophic factor MS multiple sclerosis
CSF cerebrospinal fluid NF neurofilament
DPS diaphragmatic pacing NGF nerve growth factor
DVT deep venous thrombosis NICE National Institute for Health
EAAT excitatory amino acid and Care Excellence
transporter NIPPV non-invasive positive-pressure
EAPC European Association for ventilation
Palliative Care NIV non-invasive ventilation
EEG electroencephalogram NSAID non-steroidal anti-
EFNS European Federation of inflammatory drug
Neurological Societies ODWDA Oregon Death with Dignity
EMG electromyography Act
EOL end of life OT occupational therapy
ETRAN eye transfer communication PAD physician-assisted death
board PAS physician-assisted suicide
FEES fibreoptic endoscopic PCF peak cough flow
evaluation of swallowing
PCVGV pressure-controlled volume-
FTD frontotemporal dementia guaranteed ventilation
viii LIST OF ABBREVIATIONS
PE pulmonary embolism SMA spinal muscular atrophy
PEG percutaneous endoscopic SNIP sniff nasal inspiratory pressure
gastrostomy SOD1 superoxide dismutase 1
PLS primary lateral sclerosis SSRI selective serotonin re-uptake
PMA progressive muscular atrophy inhibitor
PRG percutaneous radiological SVC slow vital capacity
gastrostomy TENS transcutaneous electrical nerve
QOL quality of life stimulation
REM rapid eye movement [sleep] TV tracheostomy ventilation
ROM range of movement UMN upper motor neuron
SBMA spinobulbar muscular atrophy VFSS videofluoroscopic swallowing
SDB sleep-disordered breathing study
SLT speech and language therapist WMA World Medical Association
List of contributors
Sunil Kumar Aggarwal Laura H. Goldstein
New York University Rusk Institute Department of Psychology,
of Rehabilitation Medicine, Institute of Psychiatry,
New York, NY, USA London, UK
Richard S. Bedlack Ulrike Hammerbeck
Duke University and Durham Institute of Neurology,
VAMC, Durham, NC, USA Univesity College London,
London, UK
Gian Domenico Borasio
Palliative Care Service, Emily Jay
University of Lausanne, Physiotherapy Department,
Lausanne, Switzerland King’s College Hospital,
London, UK
Gregory T. Carter
St Luke’s Rehabilitation Institute, Wendy Johnston
Spokane, WA, USA Department of Medicine
(Neurology), University of Alberta,
Emma Daniel
Edmonton, Alberta, Canada
Department of Neurology,
Institute of Psychiatry, Joy Kelly
London, UK Counsellor, Kent, UK
Lauren Elman Chris Kingsnorth
Penn Comprehensive Neuroscience Bentley Health Service, Perth,
Center, Perelman School of Medicine Western Australia, Australia
University of Pennsylvania, Robert Lambert
Philadelphia, PA, USA The Pastoral Service,
McGill University Health Centre,
Dallas A. Forshew
Montreal, Canada
Forbes Norris MDA/ALS
Research and Treatment Center, Sarah Lavender
California Pacific Medical Center, Wisdom Hospice,
San Francisco, CA, USA Rochester, UK
Deborah Gelinas Leo McCluskey
Mercy Health Saint Mary’s Penn Comprehensive
Michigan State University, Neuroscience Center,
Grand Rapids, MI, USA Perelman School of Medicine