The Impact of Joint Hypermobility Syndrome

Prof Shea Palmer Professor of Musculoskeletal Rehabilitation

Overview

• What is Joint Hypermobility Syndrome (JHS)? • Understanding and measuring the impact of JHS

– Focus groups with patients – Developing a questionnaire

• Other work • Reflections on the influence of patient research

‘partners’ and ‘participants’

What is Joint Hypermobility Syndrome (JHS)?

• Excessive joint range of motion in the presence of pain (Grahame 2003)

• Problems include pain, proprioception, strength, walking, fitness, function, anxiety and QoL

• Symptomatic joint hypermobility in adults: 5% women, 0.6% men (Simpson 2006)

• Brighton criteria (1998) now recommended for diagnosis (Grahame 2000)

Diagnosis - Brighton Criteria

Impact of JHS

• Anecdotally, many issues (Grahame 2003): anxiety, fitness, gait, pain, proprioception, QoL, strength

• Fatoye et al (2009, 2011, 2012) identified gait, pain, proprioception, QoL, ROM, strength in children…

• … but true impact of JHS in adults to be established – informs assessment and management

Outcome Assessment in JHS

• A general health questionnaire (SF-36) and an arthritis questionnaire (AIMS-2) improve following exercise (Sahin et al 2008, Ferrell et al 2004)

• We don’t know anything about other aspects of existing questionnaires in JHS…

• … and those questionnaires don’t ask about many issues raised by JHS patients

• Need for a JHS-specific questionnaire – Asks relevant questions (and not irrelevant ones) – Scores change when JHS changes – Scores stay the same when JHS stays the same

Aims

1. To understand the impact of JHS 2. To develop a JHS-specific questionnaire

• Funded by North Bristol NHS Trust Flexibility

& Sustainability Fund (£16k), Faculty of Health & Life Sciences (£11k), Centre for Health & Clinical Research (£1.1k)

Understanding the impact of JHS

• Participants: – Referred for physiotherapy at NBT – Met Brighton criteria for JHS – ≥18 years

• x2 patient focus groups (n=12) • Individual telephone interviews (n=3) • Total n=15 (n=2 men)

• Walking (stumble/trip, uneven ground, brisk walking, wandering)

• Climbing and descending stairs • Sitting still (>30min, getting up after >30min) • Shoulders (reaching up to high shelves, hair brushing) • Twisting, pushing and bending (squatting, heavy doors) • Holding and gripping (opening jars, peeling/chopping

vegetables, carrying a full saucepan) • Lifting and carrying (heavy bag) • Sleeping (getting comfortable v sleep disturbance) • Spatial awareness (coordination, control of movement)

Functional limitations

• Work/study (e.g. writing ) • Home (e.g. daily activities) • Socialising (e.g. social/family activities) • Exercise/sport (e.g. ability to exercise)

Activities affected by hypermobility

• Fatigue • Anxiety (getting worse, trip or fall) • Planning and management (choice of

footwear/transport, unexpected pain, joints ‘giving way’)

• Strength and weakness (‘tight’, ‘strong’, ‘held together’)

Living with hypermobility

Other Findings

• Reduction in pain/better pain management • Return to ‘normal’ activities (gym or other forms of exercise) • Joints to feel ‘tighter’ and under control • Reduction in dislocations • More management over self • Reduction in flare-ups • To feel strong, ‘sucked back in’, ‘tightened up’, ‘strengthening of

your body’ • ‘Reigniting awareness of what you should or shouldn’t be doing’ • Provide knowledge of how to protect joints in the future

Desirable outcomes from physiotherapy

Questionnaire Development

• Working group (x2 patient research partners and research team) developed a draft questionnaire

• ‘Think aloud’ analysis (n=4 women) • Further refinement by the working group • Final draft had 94 scored questions(!!) • Sent to n=1,504 Hypermobility Syndromes

Association (HMSA) members (with SF-36)

Questionnaire

• n=636 responses x 184 variables = 117,024 data items(!!) – entry and cleaning huge task

• N=624 analysed (n=12 <18 years)

• Characteristics: age = 39 years; 95% women; 98% white

• Site of pain: Back 90%; Knees 86%; Shoulders 84%; Hips 83%; Neck 80%, Hands 79%; Wrists 78%; Feet 73%, Ankles 66%, Elbows 49%

Questionnaire Development

• Criteria for removing questions: – <40% severity (n=37 removed) – Correlation with other Qs (r≥0.7) (n=2 removed)

• Final n=55 scored questions (original n=94) • Strongly related to the SF-36 Physical Component

Score (r=0.722) (Mental Component Score r=0.446)

• Fatigue scores (BRAF-NRS) comparable to Rheumatoid Arthritis (Nicklin et al 2010)

• Re-named the ‘Bristol Impact of Hypermobility’ (BIoH) questionnaire

Other Work

• Further information needed on usefulness of the BIoH questionnaire – funding applications…

• BIoH used in a study of physiotherapy for JHS: – Intervention developed with research partners

following patient focus groups (x4, n=25, 3 men) • Paper on patient ‘lived experience’ • Paper on experiences of physiotherapy

– Physiotherapy intervention piloted (n=8 patients) and amended (n=6 patient interviews)

Other Work

• Systematic review of the impact of JHS • PhD student (Najla Siri) investigating the

impact of JHS (walking, jumping, tissue stiffness, muscle strength, proprioception, pain, general health (SF-12), BIoH questionnaire)

Reflections on Patient Influences

• Patients have been central to: – Developing and refining research questions – Data Monitoring & Ethics and Steering Committees – Study management – Interpretation of findings – Understanding the lived experience of having JHS – Developing BIoH questionnaire – Perspectives on physiotherapy treatment – Development of physiotherapy intervention – Acting as advocates for patients in studies – …………

Acknowledgements

• Patient Research Partners: Alison Cossons, Jennifer Elvin

• Research Assistants: Dr Ginny Gould, Shahid Muhammad

• Co-applicants: Dr Emma Clark, Dr Fiona Cramp, Rachel Lewis, Sarah Nehammer

• PEP-R Group (NBT), Amanda Burston, Genna Lettieri

Acknowledgements

• Funders:

• Main Collaborators:

• Additional Collaborators:

Thank You

Shea.Palmer@uwe.ac.uk