a delicate equilibrium: living with huntington's disease

296 Pages·2016·4.99 MB·English

by  Christine Middleton

Wilson, Eleanor (2013) A delicate equilibrium: living with
Huntington's disease. PhD thesis, University of
Nottingham.
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A DELICATE EQUILIBRI UM:
LIVING WITH HUNTINGTON’S
DISEASE

Eleanor Wilson

Thesis submitted to the University of Nottingham for the degree of
Doctor of Philosophy

December 2013

1
ABSTRACT
Background: People with Huntington’s disease (HD) can be affected by motor,
cognitive and behavioural symptoms and it is this triad, coupled with its genetic
nature, which makes the disease so complex, requiring multifaceted and
multidisciplinary input. The length of the illness trajectory for HD can result in
patients receiving care at home for an extended period during which the
contribution from family caregivers is invaluable. However, there has been little
research into patient or carer perspectives on needs or how these should be
met, and how these correspond to professional viewpoints.
Objectives: To gain a holistic understanding of living with, caring for and
working in the field of HD to:
 identify the care needs of those diagnosed with HD
 develop understanding of the needs of family caregivers caring for a
person with HD
 improve understanding of the issues encountered by health and social
care professionals in delivering care to people with HD
 gain an understanding of how care needs change over time
 develop understanding of care provision for those living in the family
home and those in residential care settings.
Study design: The study involved people affected by HD across three
geographical localities. The research took a collective case study approach with
the person diagnosed with HD as the central component. Methods of data
collection included the responsive interviewing technique alongside
observation, eco-mapping, and genograms.
Fieldwork: These methods were used to gather data from the person with HD,
their family carer and a nominated health or social care professional to build 15
cases involving 33 individuals and 115 interactions (68 interviews and 47
observations) over three years of study participation.
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Findings: Living with HD requires continued readjustment to maintain balance
between increasing disability, diminishing cognition and living well at home.
This ongoing modification is undertaken by patients, their families and the
health and social care professionals providing services and support. Patients
and carers were challenged to balance: coping with the diagnosis, an impulse to
secrecy and a duty to share that knowledge; autonomy, choice and decision
making throughout the course of the illness; the transformation of homes to
hospitals; and a shift in the burden of care when the patient moved to a
residential care home. Examination of the services provided by the health and
social care professionals in this study has shown how multidisciplinary
working, a key worker approach, disease, person and service specific
knowledge, alongside continuity of staffing contribute to quality care.
Conclusion: This is the first qualitative study of living with HD incorporating
multiple perspectives over time. The study has focused on context and depth to
create multiple detailed case studies. It explored the complexity of living with
HD and the ways in which care can be provided in the community. It has
highlighted the wider implications of the genetic nature of the condition by
exploring families’ decisions to share (or not) genetic information. The study
has identified a number of daily challenges related to decision making and
autonomy for both family and professional carers when changes in capacity
occur slowly over time and are coupled with behavioural changes. Holistic,
multidisciplinary and flexible care, together with a built professional knowledge
and a key point of contact for families have been shown to be essential for those
trying to balance the delicate equilibrium of living with HD.
ii
PUBLICATIONS FROM THIS THESIS

Case study example for Huntington’s disease in: Woodward S and Mestekcy A
(2011) Neuroscience Nursing – evidence-based practice. Oxford: Wiley Blackwell
p452, case example from Elizabeth and James.
Wilson, E, Pollock, K and Aubeeluck, A (2010) Gaining and maintaining consent
when capacity can be an issue – a research study with people with Huntington’s
disease. Clinical Ethics Vol5(3):142-147 Cited on the NIHR website as ‘Valid
informed consent resources’
http://www.crncc.nihr.ac.uk/training/courses/other/vic_resources
Wilson, E (2009) ‘Patients and families’ in Byrne J, McNamara P, Seymour J, and
McClinton, P (eds) Palliative Care in Neurological Disease: A team approach,
Radcliffe, Oxford pp85-98, case example from Sarah and Max
Wilson, E; Elkan, R; Seymour, J and Almack, K (2008) A UK literature review of
progressive long-term neurological conditions. British Journal of Community
Nursing vol13(5):206-212
Aubeeluck A, and Wilson E, (2008) Huntington’s disease. Part 1: essential
background and management. British Journal of Nursing Vol17(3): 146-151

These publications can be found in Appendix A

iii
ACKNOWLEDGEMENTS

I wish to dedicate this thesis to all the families affected by HD who participated
in this study. Without their willingness to devoting their precious time and
energy to helping me understand a little of how they live with this condition this
study would not have been possible. This thesis presents my interpretation of
their stories and I can only hope that I have done them justice.
I also thank all the health and social care professionals who allowed me to
observe them and talk to them about their work and for their continued
enthusiasm for the study over such a long period. Again, without them this
thesis would not have been possible. Special thanks to the CNS and RCA who
have been so central to the study in so many ways.
In addition I thank the Sue Ryder Care Centre for the Study of Supportive,
Palliative and End of Life Care for funding this PhD. I am indebted to my two
academic supervisors, Dr. Kristian Pollock and Dr. Aimee Aubeeluck . They have
supported and encouraged me to broaden my thinking and had the patience to
keep me focused throughout this endeavour. I also thank Professor Jane
Seymour and Dr. Tony Arthur for their encouragement to start the process and
continued support throughout.
Finally I thank my family, friends and my partner Matt who have all contributed
in their own ways, encouraging me to continue this process.
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TABLE OF CONTENTS
ABSTRACT ................................................................................................................................... i
PUBLICATIONS FROM THIS THESIS .......................................................................................... iii
ACKNOWLEDGEMENTS ............................................................................................................ iv
TABLES AND FIGURES ................................................................................................................ x
Tables ........................................................................................................................................ x
Figures ....................................................................................................................................... x
GLOSSARY................................................................................................................................. xi
CHAPTER ONE: INTRODUCTION ................................................................................................ 1
CHAPTER TWO: LITERATURE REVIEW ....................................................................................... 6
Introduction .............................................................................................................................. 6
How HD affects the brain .......................................................................................................... 7
Motor symptoms .................................................................................................................. 8
Cognitive aspects .................................................................................................................. 8
Behavioural aspects .............................................................................................................. 9
Stages of HD ............................................................................................................................10
Genetics ..................................................................................................................................11
Testing .................................................................................................................................12
Impact of testing .................................................................................................................14
Wider implications ..................................................................................................................16
Coping with chronic illness .................................................................................................16
Capacity and autonomy ......................................................................................................17
Epidemiology...........................................................................................................................20
Policy and Guidance ................................................................................................................21
Service Provision .....................................................................................................................24
Multidisciplinary working....................................................................................................25
Specialist nursing input .......................................................................................................26
Family caregivers ................................................................................................................28
Self-management ................................................................................................................29
Palliative care ......................................................................................................................30
Conclusion ...............................................................................................................................32
CHAPTER THREE: METHODOLOGY AND METHODS ................................................................33
Introduction ............................................................................................................................33
Study objectives ......................................................................................................................33
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Study design ............................................................................................................................34
Case studies ........................................................................................................................34
Setting .................................................................................................................................36
Sample.................................................................................................................................37
Methodology: Understanding the approach ..........................................................................38
Interpretive constructionist theory ....................................................................................38
Case study ...........................................................................................................................40
The responsive interview technique ...................................................................................41
Observation .........................................................................................................................43
Eco-maps .............................................................................................................................44
Process of approvals ...............................................................................................................45
Recruitment process ...............................................................................................................46
Identification of participants ..............................................................................................46
Approach .............................................................................................................................47
Recruitment ........................................................................................................................47
Recruiting health and social care professionals ..................................................................48
Methods of data collection .....................................................................................................49
Interviews............................................................................................................................49
Joint interviews .............................................................................................................. 50
Telephone interviews ..................................................................................................... 51
Informal conversations .................................................................................................. 52
Observations .......................................................................................................................53
Eco-maps .............................................................................................................................54
Genograms ..........................................................................................................................55
Case diaries .........................................................................................................................55
Ethical issues ...........................................................................................................................55
Consent ...............................................................................................................................55
Reconsidering informed consent ........................................................................................56
Confidentiality .....................................................................................................................57
Impartiality ..........................................................................................................................57
Respect for emotional boundaries .....................................................................................58
Personal and professional risk ............................................................................................58
Analysis ...................................................................................................................................59
Data transformation and management ..............................................................................59
Coding .................................................................................................................................60
Building cases ......................................................................................................................61
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Building themes ..................................................................................................................62
CHAPTER FOUR: BUILDING CASES ..........................................................................................64
Introduction ............................................................................................................................64
Settings....................................................................................................................................64
Birchtree City ......................................................................................................................64
Oakfield Town .....................................................................................................................65
Larch Tree Care Homes .......................................................................................................65
Case Demographics .................................................................................................................66
Recruitment ........................................................................................................................66
Non-clinic recruitment ................................................................................................... 67
Recruitment period ........................................................................................................ 67
Study duration ....................................................................................................................68
Study participation ..............................................................................................................71
Disease overview ................................................................................................................74
Reconsidering qualitative interviewing ..................................................................................76
Impaired speech ..................................................................................................................77
Using visual cues .................................................................................................................79
Cognitive impairments ........................................................................................................79
Rigour and generalisation .......................................................................................................81
Interpretation .........................................................................................................................82
CHAPTER FIVE: CASE PROFILES ...............................................................................................71
Introduction ............................................................................................................................83
Alison .......................................................................................................................................84
Amy .........................................................................................................................................86
David .......................................................................................................................................88
Elizabeth ..................................................................................................................................90
Erica .........................................................................................................................................92
Harry .......................................................................................................................................94
Helen .......................................................................................................................................96
Kate .........................................................................................................................................98
Martin ...................................................................................................................................100
Mary ......................................................................................................................................102
Rose .......................................................................................................................................104
Sarah .....................................................................................................................................106
Sophie ...................................................................................................................................108
Tom .......................................................................................................................................110
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Vicky ......................................................................................................................................112
QUALITATIVE FINDINGS ........................................................................................................114
CHAPTER SIX: DISCOVERY AND ADJUSTMENT ......................................................................114
Introduction ..........................................................................................................................114
The diagnosis ........................................................................................................................115
Responses to new information .............................................................................................117
Shock and denial ...............................................................................................................117
Practical consequences .....................................................................................................118
Family carer responses .....................................................................................................122
Knowledge Management ......................................................................................................124
Information seeking ..........................................................................................................125
Controlling information ....................................................................................................126
Information blocking .........................................................................................................127
‘Not wanting to know’ ......................................................................................................129
Balancing service input .........................................................................................................132
Building the relationship ...................................................................................................133
Level of input ....................................................................................................................136
The key worker .................................................................................................................141
Balancing needs ................................................................................................................143
Conclusion .............................................................................................................................145
CHAPTER SEVEN: ACCOMMODATION AND MAINTENANCE .................................................146
Introduction ..........................................................................................................................146
Space invaders ......................................................................................................................148
Service input .........................................................................................................................152
Day care ............................................................................................................................152
Home care .........................................................................................................................154
Respite ..............................................................................................................................156
Resisting services ..............................................................................................................158
Tailor made ...........................................................................................................................159
Demands of the disease ....................................................................................................161
Balancing risk ....................................................................................................................162
Funding and bureaucracy .....................................................................................................167
Life beyond ‘caring’ ...............................................................................................................171
Conclusion .............................................................................................................................174
CHAPTER EIGHT: RELOCATION AND RECONSTRUCTION ......................................................176
Introduction ..........................................................................................................................176
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