Vascular Ehlers-Danlos syndrome (vEDS) is a rare, life-threatening connective tissue disorder. People with vEDS have often been advised to limit physical activity, yet the safety and feasibility of structured exercise in this group is poorly understood. This study works with people with vEDS, their families and clinicians to co-design a safe, tailored physical activity programme, then tests it in a 12-week randomised feasibility study comparing the programme with usual care. The aim is to find out whether the intervention and the trial procedures are safe, acceptable and practical, in order to inform a future full-scale trial. Outcomes focus on recruitment, retention, adherence, acceptability and safety, alongside exploratory measures of physical function, quality of life and microvascular health.
This is a mixed-methods feasibility study delivered across four phases. Phase 1 uses qualitative interviews with people with vEDS, family members and clinicians to understand experiences of physical activity and decision-making under uncertain clinical guidance. Phase 2 uses co-production focus groups to design the exercise-based lifestyle intervention. Phase 3 is a 12-week randomised feasibility trial in which adults with vEDS are randomised to the co-produced intervention or to usual care, assessing feasibility outcomes (recruitment, retention, adherence, data completeness, acceptability and safety) and exploratory clinical measures. Phase 4 uses post-intervention interviews to explore participant experiences and refine the intervention. The registered trial corresponds to the Phase 3 randomised feasibility component; the qualitative phases provide the development and evaluation context.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
OTHER
Masking
NONE
Enrollment
40
A 12-week individually tailored exercise-based lifestyle programme, co-produced with people with vEDS, delivered remotely with supervision and weekly monitoring. Comprises low-intensity, low-impact aerobic and functional activity progressed to individual capacity, with behavioural support to encourage sustained physical activity.
Sheffield Hallam University
Sheffield, South Yorkshire, United Kingdom
Recruitment rate
Proportion of eligible participants who consent to take part, expressed as a monthly recruitment rate.
Time frame: From study start through completion of recruitment, up to 12 months
Retention rate
Proportion of randomised participants completing the 12-week intervention (progression target ≥80%)
Time frame: 12 weeks
Adherence
Proportion of prescribed exercise sessions completed (target ≥60%), assessed by session completion records and device-based monitoring (step count and heart rate via ActiGraph accelerometer
Time frame: Over the 12-week intervention period
Data completeness
Proportion of participants completing outcome measures at baseline and 12 weeks (target ≥75%)
Time frame: Baseline and 12 weeks
Acceptability
Acceptability assessed via a questionnaire informed by the Theoretical Framework of Acceptability (seven constructs, 5-point Likert scale), supplemented by post-intervention interviews
Time frame: 12 weeks
Adverse events and serious adverse events
Number, type and severity of adverse events and serious adverse events, reviewed by the independent Data Safety Committee.
Time frame: Throughout the 12-week intervention period
Fatigue
FACIT-Fatigue Scale (Functional Assessment of Chronic Illness Therapy - Fatigue); score range 0-52; higher scores indicate less fatigue (better outcome)
Time frame: Baseline and 12 weeks
Anxiety and depression
Hospital Anxiety and Depression Scale (HADS); two subscales (anxiety and depression), each scored 0-21; higher scores indicate greater symptom severity (worse outcome)
Time frame: Baseline and 12 weeks
Health-related quality of life
EQ-5D-5L; index value ranging from below 0 to 1, where 1 = full health and higher = better; includes a 0-100 visual analogue scale (higher = better health)
Time frame: Baseline and 12 weeks
Physical activity
International Physical Activity Questionnaire - Short Form; reported as MET-minutes per week; higher values indicate greater physical activity
Time frame: Baseline and 12 weeks
Lower-limb strength and endurance
30-Second Sit-to-Stand Test; number of full sit-to-stand repetitions completed in 30 seconds; higher counts indicate better lower-limb strength and endurance (better outcome)
Time frame: Baseline and 12 weeks
Sub-maximal aerobic capacity
2-Minute Step Test; total number of steps completed in 2 minutes; higher counts indicate better aerobic capacity (better outcome).
Time frame: Baseline and 12 weeks
Muscle strength
Hand grip strength via Jamar handheld dynamometer, recorded in kilograms (best of three attempts per hand); higher values indicate greater muscle strength (better outcome)
Time frame: Baseline and 12 weeks
Functional mobility
Short Physical Performance Battery (SPPB); composite score 0-12 from balance, 4-metre gait speed, and 5-repetition sit-to-stand; higher scores indicate better lower-extremity function (better outcome)
Time frame: Baseline and 12 weeks
Orthostatic heart rate response
Heart rate measured supine and on standing (1, 3, 5 min) to screen for orthostatic intolerance
Time frame: Baseline and 12 weeks
Orthostatic blood pressure response
Blood pressure measured supine and on standing (1, 3, 5 min) to screen for orthostatic hypotension.
Time frame: Baseline and 12 weeks
Microvascular function
Cutaneous vascular conductance via Laser Speckle Contrast Imaging during local thermal hyperaemia. Expressed as cutaneous vascular conductance (perfusion units/mmHg); higher values indicate greater microvascular reactivity
Time frame: Baseline and 12 weeks
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