Exploring the multi-factorial manifestations of joint hypermobility syndrome and the impact on quality of life.

Abstract

Introduction: Performing artistes have entertained audiences for thousands of years. Their repertoires require the integration of a well ‘tuned’ central nervous system and hypermobility. Hypermobility is a common phenomenon that is beneficial for some but not for others. This thesis discusses hypermobility associated with multisystemic symptoms referred to as Joint Hypermobility Syndrome (JHS). It is suggested that the multifactorial manifestations of the condition contribute to deconditioning thus impacting on the physical and mental well being of individuals with JHS. Purpose: To explore the multi-factorial manifestations of JHS including functional difficulties and their impact on quality of life. Methods: A two part study; part one, development of a questionnaire to assess for functional difficulties; part two, a mixed methods approach to explore aspects of JHS. Results: Principal Axis Factoring was employed to explore the structure of the 9-item Functional Difficulties Questionnaire (FDQ-9) to assess functional difficulties reported in childhood and adulthood. Internal consistency was high (0.81), correlations between items were > 0.5 and preliminary findings suggested satisfactory construct validity. Test-retest reliability was good (ICC 0.96 [95% CI 0.92 to 0.98]. Patients with JHS were 3 [95%CI 1.95 – 4.56] times more likely to report functional difficulties both as a child and as an adult than healthy volunteers. Patients with JHS were significantly more likely to report dislocations, autonomic nervous system, gastrointestinal and cardio-respiratory symptoms than healthy volunteers. Chronic widespread pain reported by 86% of patients with JHS was a significant predictor of quality of life using the SF-12. Patients with JHS had significantly lower physical component scores than healthy volunteers (29.2 [SD 10.6] and 54.5 [SD 5.7]) respectively. Conclusions The development of the FDQ-9 contributes to the understanding of the multi-factorial manifestations of JHS and their long term nature. These have important clinical implications as symptoms of JHS appear early in life. Management of this condition requires early recognition and an understanding of the multisystemic nature.