Juvenile Idiopathic Arthritis and Young People in Ireland Author(s)

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Quality of Life Issues: Juvenile Idiopathic Arthritis and Young
Title
People in Ireland
Author(s) O'Hara, Mary
Publication
2013-05-28
Date
Item record http://hdl.handle.net/10379/3553
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Quality of Life Issues: Juvenile Idiopathic Arthritis and Young
People in Ireland

Submitted in part fulfillment of the academic requirements for
the degree of Doctor of Philosophy (PhD)

Candidate: Mary O’Hara
Supervisor: Dr. Margaret Hodgins
Submitted to: Discipline of Health Promotion
College of Medicine, Nursing and Health Sciences,
National University of Ireland, Galway
Submitted: December 2012
Table of Contents
Abstract .................................................................................................... 1
Declaration .................................................................................................... 2
Acknowledgements .................................................................................................... 3

1. INTRODUCTION ..................................................................................... 4
1.1 Background ................................................................................................ 6
1.2 Aims and Objectives of the Study ............................................................. 7
1.2.1 Objectives: .......................................................................................... 7
1.3 Rationale for the Study .............................................................................. 8
1.4 Summary of Chapters ................................................................................ 9

2. LITERATURE REVIEW ........................................................................ 13
2.1 Introduction ............................................................................................. 13
2.1.1 Literature Search .............................................................................. 13
2.2 The Concept of Quality of Life ............................................................... 14
2.3 The Medical Approach/Health-Related Quality of Life Approach ......... 16
2.3.1 Health-Related Quality of Life (HRQOL) in Young People ............ 23
2.3.2 Health-Related Quality of Life – Measurement Issues .................... 26
2.4 Disease-Specific Questionnaires for Juvenile Arthritis ........................... 30
2.4.1 The Childhood Health Assessment Questionnaire (CHAQ) ............ 30
2.4.2 Juvenile Arthritis Functional Assessment Scale and Report ............ 31
2.4.3 Juvenile Arthritis Self-Report Index ................................................ 31
2.4.4 Juvenile Arthritis Quality of Life Questionnaire .............................. 31
2.4.5 Childhood Arthritis Health Profile ................................................... 32
2.4.6 Paediatric Rheumatology International Trials Organization Clinical
Assessment ....................................................................................... 32
2.4.8 The DISABKIDS Health-Related Quality of Life Measure ............. 33
2.5 Summary .................................................................................................. 36
2.6 What is Juvenile Idiopathic Arthritis? ..................................................... 38
2.6.1 Types of JIA ..................................................................................... 42
2.6.2 Oligoarticular JIA ............................................................................. 42
2.6.3 Polyarticular JIA ............................................................................... 44
2.6.3.1 Polyarthritis (rheumatoid factor negative) ............................. 44
2.6.3.2 Polyarthritis (rheumatoid factor positive) .............................. 45
2.6.4 Systemic Arthritis ............................................................................. 45
2.6.5 Psoriatic Arthritis.............................................................................. 46
2.6.6 Enthesitis-Related Arthritis .............................................................. 46
2.7 Principles of Management of the Disease ............................................... 48
2.8 Pain Assessment and Management ......................................................... 51
2.9 Psychosocial Impact of Juvenile Idiopathic Arthritis .............................. 56
2.10 Healthcare Strategies for Young People with Chronic Diseases ............. 65
2.11 Communications ...................................................................................... 66
2.12 Social Support ......................................................................................... 71
2.13 Transition to Adult Services .................................................................... 75
3. ETHICAL CONSIDERATIONS ............................................................ 79
3.1 Children as Participants in Research ....................................................... 81
3.2 Informed Consent .................................................................................... 83
3.3 Anonymity and Confidentiality ............................................................... 84
3.4 Gaining Ethical Approval for the Study .................................................. 84
3.5 Summary .................................................................................................. 86

4. DESIGN APPROACHES METHODOLOGY ....................................... 88
4.1 Introduction ............................................................................................. 88
4.2 Historical Perspective .............................................................................. 88
4.3 Rationale for the Utilization of Mixed Methods Methodology ............... 89
4.4 Methods That ‘Fit’ the Research Question .............................................. 90
4.5 The Appropriateness of Pragmatism ....................................................... 91
4.6 The Rationale for Purposeful Sampling .................................................. 93
4.7 Summary of Theoretical Framework ....................................................... 94
4.8 PHASE 1: Consultant Survey .................................................................. 95
4.8.1 Method for the Consultant Survey ................................................... 95
4.9 PHASE 2: Methods for Interviews with Young People with JIA to
Develop Questions for a Health Services Questionnaire .......... 97
4.9.1 Methods for Interviews with Young People with JIA ...................... 97
4.9.2 Interviews ......................................................................................... 98
4.9.3 Choosing Semi-Structured Interviews .............................................. 98
4.9.4 Purposive Sampling .......................................................................... 99
4.9.5 The Interview Process ...................................................................... 99
4.9.6 The Interviewees ............................................................................ 100
4.10 PHASE 3: Methods for Questionnaire to Young People ...................... 102
4.10.1 Questionnaire to Young People ...................................................... 102
4.10.2 Principal Investigators at Research Sites ........................................ 102
4.10.3 Peer Group for Comparison............................................................ 103
4.10.4 DISABKIDS Condition Specific Module ...................................... 104
4.10.5 Descriptive Statistical Tests ........................................................... 107
4.11 PHASE 4: Methods for Interviews with Parents of Young People
with JIA ................................................................................... 108
4.11.1 Interview with Parents .................................................................... 109
4.11.2 Telephone Interviews Versus Face-to-Face Interviews ................. 110
4.11.3 The Interviewing Process ............................................................... 111
4.12 PHASE 5: Methods for Interviews with Clinicians of Young People
with JIA ................................................................................... 114
4.12.1 Methods for Interviews with Clinicians ......................................... 114
5. RESULTS .............................................................................................. 117
5.1 Results: Postal survey of Consultant Paediatricians and Consultant
Rheumatologists ...................................................................... 117
5.1.1 Numbers of Patients with Juvenile Idiopathic Arthritis? ............... 117
5.1.2 Ages of Patients with Juvenile Idiopathic Arthritis ........................ 117
5.2 Results of Interviews with Young People ............................................. 119
5.3 Results of the Questionnaire to the Young People (DISABKIDS
Questionnaire) ......................................................................... 122
5.3.1 Physical Well-being and Health ..................................................... 122
5.4 Results of Interviews with Parents ........................................................ 140
5.4.1 Quality of Life: Physical Well-being and the Impact of the
Disease ............................................................................................ 141
5.4.2 Pre-diagnosis: Prior to a Confirmed Diagnosis of Juvenile
Idiopathic Arthritis ......................................................................... 141
5.4.3 Post-diagnosis: After a Confirmed Diagnosis of Juvenile
Idiopathic Arthritis ......................................................................... 142
5.4.4 Quality of Life: Psychological Well-being and the Impact of the
Disease ............................................................................................ 144
5.4.5 Quality of Life: the Impact of the Diagnosis Process, Physically
and Psychologically ........................................................................ 146
5.4.6 Quality of Life: Parents as Advocates for their Children ............... 147
5.4.7 Quality of Life: Referral to a Regional Rheumatology Centre ...... 150
5.4.8 Quality of Life: the Global Well-being of the Young Person ....... 152
5.4.9 Quality of Life: Diagnosis and the Impact on the Family .............. 153
5.4.10 Quality of life: The Impact of Illness on Family Life .................... 155
5.4.11 Quality of Life: Medication ............................................................ 160
5.4.12 Quality of Life: Social Support for the Family .............................. 162
5.4.13 Family Support ............................................................................... 162
5.4.14 Quality of Life: Knowledge and Information ................................. 163
5.4.15 Quality of Life: Financial Resources .............................................. 165
5.4.16 Quality of Life: Social Acceptance, Normalcy and Moving On
in Life ............................................................................................. 166
5.4.17 Opportunity to Talk with a Researcher .......................................... 168
5.4.18 Summary of Results of Interviews with Parents ............................ 169
5.5 Results of Interviews with Consultant Rheumatologists and
Rheumatology Specialist Nurses ............................................. 169
5.5.1 Diagnosis ........................................................................................ 170
5.5.2 Young People ................................................................................. 174
5.5.3 Medication/Treatment .................................................................... 176
5.5.4 Communication .............................................................................. 177
5.5.5 Culture ............................................................................................ 180
5.5.6 Young Adults and Transition from Juvenile Services to Adult
Services........................................................................................... 180
5.5.7 The Healthcare Service for Young People with Juvenile Idiopathic
Arthritis........................................................................................... 182
5.5.8 The Role of the Team ..................................................................... 184
5.5.9 Summary......................................................................................... 185

6 DISCUSSION ........................................................................................ 188
6.1 Introduction ........................................................................................... 188
6.2 Section One ........................................................................................... 190
6.2.1 The Impact of JIA on the Bio-psychosocial Well-being and
Quality of Life on a Young Person with Juvenile Idiopathic
Arthritis (JIA) ............................................................................. 190
6.2.2 The Impact of JIA on Family Quality of Life ................................ 196
6.2.3 The Impact of Social Support on Quality of Life ........................... 201
6.2.4 The Organization of Care ............................................................... 204
6.2.5 Transition to Adult Services ........................................................... 210
6.3 Section Two ........................................................................................... 213
6.3.1. Health Promotion Theoretical Framework Underpinning the
Study ............................................................................................... 213
6.4 Section Three ......................................................................................... 215
6.4.1 Research Methodological Issues .................................................... 215
6.4.1.2 Sample of Parents ................................................................ 217
6.4.1.2 Reliability of Results ........................................................... 219
6.4.1.3 Ethical Issues Relating to Children and Research ............... 220
6.5 Section Three ......................................................................................... 221
6.5.1 The Future – a Proposed Pathway of Care ..................................... 221
6.6 Summary ................................................................................................ 224
6.7 Conclusions ........................................................................................... 225
6.8 Limitations of the Study ........................................................................ 228
6.8.1 Methodological Issues .................................................................... 228
6.8.2 Other Issues .................................................................................... 229
6.9 Recommendations ................................................................................. 229

REFERENCES ................................................................................................ 232
APPENDICES ................................................................................................ 295
Glossary ................................................................................................ 345

Appendices
APPENDIX 1: Tannahill’s Model of Health Promotion
APPENDIX 2: Differential Diagnosis of Joint Pain In Children
APPENDIX 3: Potential Complications of Juvenile Idiopathic Arthritis
APPENDIX 4: Cytokine Modulators Currently In Use or Under Assessment for
the Treatment of Severe JIA
APPENDIX 5: Garda Clearance
APPENDIX 6: Ethical Approval from Hospitals
APPENDIX 7: Interview Schedule with Young People
APPENDIX 8: Information for Participants with Arthritis (a)
APPENDIX 9: Interview Schedule for Parents
APPENDIX 10: Topics for Discussion with Clinicians
APPENDIX 11: Consultant Survey
APPENDIX 12: Data from Three Perspectives
APPENDIX 13: An Example of Physical and Personal
APPENDIX 14: An Example of Coping Resources/Skills Needed By a Young
Person with JIA
APPENDIX 15: Analogy of the Pit Stop Crew
Tables
Table 2.1: Characteristics of JIA .............................................................................. 43
Table 4.1: Phases of Thematic Analysis ................................................................. 116
Table 5.1: Health and Activity ............................................................................... 124
Table 5.2: Psychological Well-being ...................................................................... 125
Table 5.3: Moods and Emotions ............................................................................. 127
Table 5.4: Leisure Time .......................................................................................... 128
Table 5.5: Time with Parents .................................................................................. 129
Table 5.6: Finance ................................................................................................ 129
Table 5.7: Friends ................................................................................................ 131
Table 5.8: School ................................................................................................ 132
Table 5.9: The Impact of Arthritis .......................................................................... 133
Table 5.10: Quality of Life: Medical Treatment .................................................... 135
Table 5.11: Questionnaire: Quality of Life About Symptoms of the Disease ........ 136
Table 5.12: Questionnaire: Part E About Your Healthcare Services ..................... 138

Figures
Figure 1.1: An Overview of the Research Study Process ......................................... 12
Figure 4.1: Phase One ............................................................................................... 95
Figure 4.2: Phase Two .............................................................................................. 97
Figure 4.3: Phase Three .......................................................................................... 102
Figure 4.4: Adapted from the DISABKIDs Group (2001) 1 .................................. 106
Figure 4.5: Adapted from the DISABKIDs Group (2001) 2 .................................. 107
Figure 4.6: Phase Four ............................................................................................ 108
Figure 4.7: Phase Five ............................................................................................ 114
Figure 5.1: Key Issues to Emerge from the Study .................................................. 186
Figure 5.2: Demonstrating Convergence and Non-Convergence of Findings ....... 187
Figure A: An Example of a ‘Micro-context’ Model of Care for Young People with
JIA .......................................................................................... 348
Abstract
The aim of this study was to explore quality of life issues of young people in
Ireland with juvenile idiopathic arthritis (JIA). JIA is an autoimmune
inflammatory disease diagnosed during childhood before the age of 16 with an
incidence of 10-20/100,000. It is characterized by persistent synovial
inflammation which can cause functional impairment, pain, and activity
limitation; all of which may affect quality of life. Treatment modalities are not
curative and aim to control the inflammatory process. This was a mixed
methods study. To obtain multiple perspectives, young people with JIA were
surveyed and parents and clinicians were interviewed.

Findings
The young people perceived that they had a good quality of life. Social support
was found to be a protective factor in buffering the impact of juvenile arthritis.
Parents had a strong role in buffering their children from adverse circumstances
and promoting their children’s quality of life.

Parental recognition of the practical difficulties that children were encountering
and pride in their efforts to adapt to difficult situations was a major theme.
Education, school services and future prospects were frequent issues.
Socialization was considered very important. However, services and service
organization for young people with JIA were frequently encountered as
problematic. Safety considerations relating to the long-term effects of the
newer biologic medications, and whether they had made the right choices for
their children, were significant concerns for parents.

Conclusions
Person-centred care from an early age is needed to meet the needs of young
people with JIA across their lifespan, to optimize their life opportunities and
quality of life. Service provision and service organization needs to be more
collaborative and co-ordinated. A dedicated liaison person is needed to co-
ordinate and support young people with JIA and their families to manage and
adapt to the illness and improve their quality of life. Finally, families need more
knowledge and information relating to the newer biologic medications.
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Declaration
National University of Ireland, Galway

Title: Quality of Life Issues:
Juvenile Idiopathic Arthritis and Young
People in Ireland

Name: Mary O’Hara

Student Identity Number: 98976893

Date Submitted:

I hereby declare this work is entirely my own and that I have acknowledged the
writings, ideas and work of others.

Furthermore I have not knowingly allowed another to copy my work.

Signature: ___________________________________ Date: ______________

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Acknowledgements

First and foremost: Thank you to all of the young people and their
families.
You demonstrate such fortitude and courage in your quiet understated
way.
You are truly magnificent.

Grateful thanks and acknowledgement to:
Dr.Margaret Hodgins,
Professor B.G. Loftus, Dr. Robert Coughlan, Professor Owen Hensey,
Professor Mick Malloy all of whom went above and beyond the call of
duty to be helpful.

Professor Margaret Barry and staff of the Discipline of Health Promotion,
National University of Ireland, Galway.
Clinical and administrative staff in all the participating hospitals.
Friends and colleagues, in the School of Nursing & Midwifery, National
University of Ireland, Galway.
The Library Staff at the National University of Ireland, Galway.
The Villanova Scholarship Committee.
An Bord Altranais Scholarship Committee.

Special acknowledgment: Professor Barry Breshnihan
Grateful appreciation to him for his interest, invaluable assistance and
kindness.

A special word of thanks to Mrs Joan Kavanagh, Michele and Dave, Ciarán,
Anna, John, Anthony and Catherine for their generous support.

Paddy and Eily, family and friends – ‘the support team’.

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