Table Of ContentClinics in Developmental Medicine Clinics in Developmental Medicine
The Musculoskeletal System in
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Children with Cerebral Palsy e
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Children with Cerebral Palsy
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A Philosophical Approach to Management s
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l A Philosophical Approach to Management
Martin Gough and Adam Shortland o
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From the Foreword s
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“You will not have read a book like this before. Intellectually fearless,
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it is the product of wide reading and deep thought... Confronted by the n
suffering of a child and family, Gough and Shortland open a space for us to C
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pause and not rush to judgement.” i
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Richard Robinson
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Emeritus Professor of Paediatric Neurology, Guy’s, King’s, and St Thomas’ Hospitals, London, UK e
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Do we need a paradigm shift in our approach to the assessment and management of the
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musculoskeletal system in children with cerebral palsy? This book encourages clinicians to h
reflect on how their thoughts and approaches are shaped by the clinical society. Using new C
perspectives and expertise, this book will inspire clinicians to rethink conventions about the e
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child with cerebral palsy, consider how they communicate these new concepts to their fellow e
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clinicians, and act to bring about positive changes in the management of children with r
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cerebral palsy. l
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• Provides a critical review of the current understanding and management of l
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musculoskeletal deformity in children with cerebral palsy. y
• Transfers current understanding of muscle and bone physiology and function, from
the realms of research into mainstream clinical thinking.
• Advocates for an alternative clinical model of assessment and intervention, focusing G
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on the experience of the child with cerebral palsy and their experience of the world.
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• Proposes the concept of the musculoskeletal system as a linked system of interactive
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processes and subsystems, extending from individual molecules to the child and
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their environment. N
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A critical resource for clinicians and researchers involved in the care of O
children with cerebral palsy including neurologists, physical therapists, R
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orthopaedic surgeons, and neurosurgeons, as well as researchers and A
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clinicians interested in the philosophy of medicine. D
Martin Gough and Adam Shortland
www.mackeith.co.uk
The Musculoskeletal System in
Children With Cerebral Palsy:
A Philosophical Approach
to Management
Clinics in Developmental Medicine
The Musculoskeletal
System in Children
With Cerebral Palsy:
A Philosophical
Approach to
Management
Martin Gough
Consultant Paediatric Orthopaedic Surgeon, Evelina London Children’s Hospital,
Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Adam Shortland
Consultant Clinical Scientist, Evelina London Children’s Hospital,
Guy’s and St Thomas’ NHS Foundation Trust, London, UK
2022
Mac Keith Press
© 2022 Mac Keith Press
Managing Director: Ann-Marie Halligan
Senior Publishing Manager: Sally Wilkinson
Publishing and Marketing Co-ordinator: Paul Grossman
Production Manager: Andy Booth
The views and opinions expressed herein are those of the authors and do not necessarily represent those
of the publisher.
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, electronic, mechanical, photocopying, recording or otherwise,
without the prior written consent of the copyright holder or the publisher.
First published in this edition in 2022 by Mac Keith Press
2nd Floor, Rankin Building, 139–143 Bermondsey Street, London, SE1 3UW
British Library Cataloguing-in-Publication data
A catalogue record for this book is available from the British Library
Cover designer: Marten Sealby
Cover image: The cover image reflects some of the themes of the book: two amorphous figures are either
constructing or deconstructing a pile of bricks against a background of leaves. The book looks at how
we need to form models of complex reality, how these need to be flexible and to change, and why it is
important to avoid replacing one certainty with another. We also liked the colours.
ISBN: 978-1-911612-53-7
Typeset by Riverside Publishing Solutions Ltd
Printed by Jellyfish Solutions Ltd
Contents
Foreword ix
Preface xi
Acknowledgements xiii
1. What We Think and Why We Think It: Our Clinical Model of Cerebral Palsy 1
The Sound of Silence 3
How Do We Know What We Know? 5
Imposing a Structure on Sensory Information: Defining Our Own Reality 6
System 1, Heuristics, and Bias 8
Is All Knowledge the Same? 9
Knowledge Within the Clinical Society 11
The Concept of ‘Spasticity’ 13
Does it Matter if There are Differing Concepts of Spasticity? 15
The Role of Cognitive Dissonance 17
Clinical Evidence and the Scientific Process: Is This Not an
Objective Process? 18
Thomas Kuhn and the Role of the Paradigm 19
Factors Influencing Paradigm Change in a Clinical Society 21
Moving Towards a New Paradigm 23
The Objective Body and the Lived-In Body 26
2. A Made-Up Story About Data, Knowledge, and Clinical Judgement 28
The Randomised Controlled Trial 30
Sample Sizes and Outcome Statistics in Randomised Controlled Trials 30
Bias in Randomised Controlled Trials 37
Short-Term Follow-Up and Long-Term Outcomes 39
v
vi Contents
Clinical Guidelines 40
Summary and Recommendations 44
3. The Musculoskeletal System: Not Just a Structure but a Process 46
What is a System? 46
Linear and Nonlinear Systems 47
Cellos and Muscles 48
The Orchestra as an Example of a System 49
Considering the Human Musculoskeletal System as a Process
Rather Than a Structure 50
Entropy, Information, and the Cell 51
The Cell Membrane: The Difference Between Inside and Outside the Cell 56
Proteins: The Workhorses of the Cell 56
How are Proteins Formed in the Cell? 57
Being a Cell Takes Energy 59
The Mitochondrion: The Cell’s Power Station 59
Cellular Energy Balance 61
Systemic Energy Balance 62
A Cell is a Process as Well as a Structure 63
The Skeleton: More Than Just a Framework 63
Skeletal Muscle: The Prime Mover 65
The Myocyte: The Basic Biological Unit of Skeletal Muscle 65
The Components of Muscle Contraction 66
The Molecular Basis of Muscle Contraction 67
Muscle Architecture and Muscle Function 68
‘Slow’ and ‘Fast’ Muscles 70
Skeletal Muscle is Dynamic and Responsive 70
Muscle Innervation 72
Control of Movement: The Nervous System 72
The Neuron: The Basic Biological Unit of the Nervous System 72
The Basis of Neuronal Signalling: The Action Potential 73
Providing the Energy for Neuronal Signalling 74
The Concept of Upper and Lower Motor Neurons 75
The Central Nervous System Really is a System … 75
… And is Part of a Larger System Which Includes the Skeleton
and Muscles 76
Interactions Within the Musculoskeletal System: Balancing Energy
and Growth 77
Considering Musculoskeletal Growth as a Process 78
Contents vii
4. Musculoskeletal System Development: Typical and Altered Trajectories 79
Concepts in Cell Development: Epigenetics, State Space, and
the Adaptive Possible 79
Considering Musculoskeletal Growth as a Trajectory 80
Entropy and Developmental Trajectories 83
This All Sounds a Bit Complicated … 84
Causal Loop Diagrams 85
Energy Costs of Growth 87
Musculoskeletal Development in the Embryo 88
Muscle Contraction Begins Early 89
The Role of the Corticospinal Tract 89
Movement and Motor Control After Birth 90
Skeletal Muscle Fibre Growth and Development 91
Factors Influencing Muscle Fibre Growth 92
Effect of Skeletal Muscle on Skeletal Development 93
Musculoskeletal Development in Children With Cerebral Palsy 95
Effect of Early Developmental Changes on Subsequent Musculoskeletal
Growth and Development 96
Putting it All Together: Understanding and Exploring the
Musculoskeletal System 99
Where Do We Go From Here? 100
5. Evidence-Based Medicine and Cerebral Palsy 102
Clinical Experience and Evidence-Based Medicine 103
The Problem with Randomised Controlled Trials in Cerebral Palsy 104
Summary of the Weaknesses of Evidence-Based Medicine When
Applied to Cerebral Palsy 117
Building the Evidence for Treatment: Enhancing the Power of
Observational Studies 117
Summary 124
6. What Does All This Mean? 125
Looking Again at How We View Clinical Knowledge 126
The Cynefin Framework and Knowledge Domains 127
Clinical Knowledge and the Cynefin Framework 130
Clinical Knowledge and Uncertainty 131
A Systems Approach to Clinical Knowledge 133
The Role of External Factors in System Development 135
Is This Helpful for Clinical Practice? 136
viii Contents
Critical Systems Heuristics 137
Sartre: Existence Comes Before Essence 141
Merleau-Ponty: We are Ensembles of Lived Meanings 142
The Clinical Relevance of the Lived-In Body 143
The Child With Severe Cognitive Impairment 144
The Lived-In Body as a Focus for Intervention 145
Putting Theory Into Practice 146
Does Life Really Need to be This Complicated? 152
Conclusion: Putting it Into Practice 154
References 156
Index 169
Foreword
Gough and Shortland shine a light on a number of areas of cerebral palsy that have been
largely ignored hitherto. We do not fully understand the neuropathology for increased
tone. Different definitions of spasticity have emerged – with different therapeutic implica-
tions. Are we sure what is primary and what is secondary pathology?
Working with a deficient theoretical base to say nothing of poor outcome data, it is
not surprising that decisions to advise such interventions as physiotherapy, serial casting,
botulinum toxin, and surgery vary widely among practitioners who nevertheless feel driven
by therapeutic imperatives. We do not know how to take into account variability between
children and their different circumstances.
Gough and Shortland confront these dilemmas head on. Given the muddle we are in,
it is appropriate to start in Chapter 1 with how we know what we know, how that knowl-
edge may be erroneous, how experience develops into concepts, how that knowledge can
be shared, and the difficulty of application of knowledge to promote change in clinical
practice.
They recognise that uncertainty is uncomfortable. They are in a fine tradition here.
Ronnie Mac Keith embraced uncertainty directing his endeavours precisely to such areas.
In Chapter 2 they explore the limitations of the randomised controlled trial as currently
practiced, interpreted, and applied in the context of cerebral palsy.
In Chapter 3 we are invited to consider the musculoskeletal system as a process rather
than as a structure. Again we go back to basics with a discussion of entropy and how
this relates to information flows within a network. This is followed by a comprehensive
description of the structure and function of muscles and bones from the molecule upward,
taking in energy generation and distribution along the way. They move to an exposition of
innervation and the integration of muscle, bone, and adipose tissue as an endocrine system.
This is developed further in Chapter 4 with an emphasis on growth and development
and how it may be altered in a child with cerebral palsy. Here we start with the stem cell,
the gene regulatory network, and epigenetic influences. Development is envisaged as a
variety of trajectories with a number of possible outcomes. With time, the number of
possible options diminish – an effect which is discussed within the context of entropy
which here reappears. This forms the basis for the introduction of causal loops, a number
of examples of which illustrate the subsequent ideas. From the fertilised ovum we move
through the migration of myoblasts to myotube development, myoneural innervations, the
role of the corticospinal tract, muscle fibre growth and development, and the influence of
muscles on the growing skeleton.
Cerebral palsy now emerges as the result of altered interaction within a complex adap-
tive system not solely the result of altered innervation but also receiving contributions
ix
