Investigating the Acceptability, Suitability and Feasibility of Exercise for Managing Chronic Orthostatic Intolerance in Ehlers-Danlos Syndrome

Hypermobile variants of Ehlers-Danlos Syndrome (EDS) often present with signs and symptoms of Postural Orthostatic Tachycardia Syndrome (POTS) or orthostatic intolerance (OI), affecting more than just the physical aspect of the individual, but often greatly affect the individuals’ psychology and quality of life. POTS and OI are associated with debilitating symptoms (e.g. dizziness, a racing heart, nausea) when standing upright, with symptoms often inadequately acknowledged or understood by clinicians. Physiotherapy is an essential component of the multidisciplinary approach of both EDS and OI, with exercise prescription playing a key role in the non-pharmacological management of orthostatic symptoms. Current research has investigated the impact of land-based exercise on symptoms of OI in a general POTS population, however none have been specific to the EDS population. Water-based exercise may provide many benefits for individuals with EDS and OI, however, there has been no research to date investigating its impact on symptom management.

The co-design of exercise programmes (clinician and participant) for populations with specific and challenging needs such as EDS and OI, have been shown to improve participation. The first step in co-designing exercise programmes for specific populations such as individuals with hypermobile variants of EDS and OI is to understand their wants and needs in relation to exercise. Therefore, this thesis explores the acceptability, suitability and feasibility of land and water-based exercise programmes for individuals with hypermobile variants of Ehlers-Danlos Syndrome (EDS) and chronic orthostatic intolerance (OI), to assist in facilitating effective co-designed exercise programmes for chronic OI management.

An online survey was used to collect quantitative and qualitative data internationally from individuals 16 years and over with hypermobile variants of Ehler-Danlos Syndrome (EDS) and orthostatic intolerance (OI). Data from494 survey responses were analysed, which included demographics, baseline health, preference for types of exercises, frequency and duration of land and water-based exercise sessions and challenges to participation in exercise. The largest percentage of participants preferred a combination of land-based and water-based exercise. Qualitative responses indicate that exercise was acceptable to them if they had i) a positive experience of exercise with a healthcare professional; and/or ii) the programme had led to an improvement or did not exacerbate their symptoms; and/or iii) they had enjoyed an exercise programme. Exercise was considered suitable for them if it accommodated for their capabilities, and feasible if practical barriers such as access to a venue, cost and flexibility for exercise session times were overcome.

Overall, individuals with hypermobile variants of EDS want to participate in land and/or water-based exercise programmes for the treatment of symptoms associated with hypermobile variants of EDS if made available, if programmes are tailored to their individual needs and preferences.