Multidisciplinary Approach to Treating Chronic Pain in Patients with Ehlers–Danlos Syndrome: Critically Appraised Topic

The Ehlers–Danlos syndromes (EDS) are a group of 13 related connective tissue disorders with a combined prevalence of 1 in 5000 people, with the most common noted as hypermobile EDS (hEDS). The EDS genetic condition is thought to affect both males and females equally, although most symptomatic patients are female. EDS causes a myriad of symptoms, including skin hyperextensibility and fragility, easy bruising and bleeding, joint hypermobility, subluxation, dislocation, and chronic pain. Pain is one of the most common symptoms of EDS, leading to disability and decreased quality of life. Current guidelines for treating chronic pain in EDS are lacking. Clinicians focus on a conservative multidisciplinary approach in patients with EDS, which avoids surgical interventions and its accompanying risks of morbidity and mortality. The multidisciplinary approach includes physiotherapy, occupational therapy, cognitive behavioral therapy, and pharmacologic interventions to decrease pain. This review identifies literature examining the components of this conservative multidisciplinary approach and their effectiveness across the PubMed, EMBASE, CINAHL, Web of Science, and Trip databases, using the terms “Ehlers-Danlos Syndrome AND Pain Management” that was then subsequently evaluated. The evaluation of this current literature provides weak evidence to support the efficacy of the individual components of the conservative multidisciplinary approach. Lack of alternative approaches leaves medical providers with little choice but to suggest these pain control methods, despite low-grade evidence of weak evidence of their efficacy. More research into the pathophysiology of chronic pain in EDS could help identify additional modes and rationales for therapy.