Hypermobile Ehlers-Danlos Syndrome: Caregiver-Child Concordance Across Challenges, Readiness to Change, and Coping

Objectives: Hypermobile Ehlers-Danlos Syndrome (hEDS) is a heritable connective tissue disorder that results in physical symptoms, psychosocial challenges, and functional disability. Children with hEDS and their caregivers face challenges in managing the symptoms and associated impacts of the syndrome. Method: Mixed methods were utilized to allow children with hEDS and their caregivers to share challenges faced, effective coping strategies, and their readiness to engage in a self-management approach to treatment. As caregivers are frequently proxy reporters for their child's experience, concordance between caregiver and child was assessed for each variable. Additionally, child outcomes were assessed in relation to caregiver-child concordance on readiness to engage in self-management. Results: Results suggest moderate concordance across dyads, with many dyads agreeing on the challenges presented by the physical consequences of hEDS but differing on beliefs regarding coping. There were small effect sizes indicating better child psychosocial functioning when dyads were concordant on readiness to engage in self-management. Conclusion: When making treatment recommendations, practitioners should consider differences and similarities between caregivers and children's reported experiences with and beliefs about hEDS. Further, practitioner facilitation of family concordance on beliefs about hEDS may result in better outcomes for the child. Implications for Impact Statement This study suggests that youth with hypermobile Ehlers-Danlos Syndrome (hEDS) and their caregivers tend to agree on the challenges hEDS presents, and their readiness to change their coping approach. However, they differ slightly in their go-to coping strategies. Thus, practitioners are encouraged to recognize where similarities and differences exist across caregiver and child experiences with and beliefs about hEDS when making treatment recommendations and to facilitate family alignment on these beliefs.