Developing a self-management intervention to manage hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS): an analysis informed by behaviour change theory

Abstract

Purpose Hypermobility Spectrum Disorders (HSD) and Hypermobile Ehlers-Danlos Syndrome (hEDS) are heritable connective tissue disorders associated with joint instability and pain, but with scant guidance for supporting patients. The aim was to determine recommendations for an HSD/hEDS self-management intervention. Materials and methods Barriers to self-management were mapped onto the Theoretical Domains Framework (TDF) and Capability, Opportunity, Motivation-Behaviour (COM-B) model in a behavioural analysis. A modified Nominal Group Technique was used to prioritise behaviour change technique (BCT) interventions (n = 9 women). Results Possible BCTs incorporated: Education Incorporating self-help strategies, education to improve their knowledge of HSD/hEDS, and how to judge information about HSD/hEDS. Training In activity pacing, assertiveness and communication skills, plus what to expect during pregnancy, when symptoms can worsen. Environmental restructuring and enablement Support from occupational therapists to maintain independence at work and home. Modelled behaviour That illustrates how other people with HSD/hEDS have coped with the psychosocial impact. Conclusions This study is the first to apply theoretically-informed approaches to the management of HSD/hEDS. Participants indicated poor access to psychological support, occupational therapy and a lack of knowledge about HSD/hEDS. Future research should evaluate which intervention options would be most acceptable and feasible. Implications for rehabilitation Patients with Hypermobility Spectrum Disorders or Hypermobile Ehlers-Danlos Syndrome can be active partners in the co-design of behaviour change interventions. Behaviour change interventions should target psychological support and patient education, particularly patient information. Additional behaviour change interventions included environmental restructuring and enablement; adaptations to participants’ environment with input from occupational therapy. Participants were keen to suggest opportunities for behavioural modelling; positive fist-person modelling narratives, written by those with HSD/hEDS, which addressed how they coped with the psychosocial impact of their condition.