Quality of life, unmet needs, and iatrogenic injuries in rehabilitation of patients with Ehlers–Danlos Syndrome hypermobility type/Joint Hypermobility Syndrome

Abstract

Ehlers-Danlos Syndrome, hypermobility type (EDS-HT) and the joint hypermobility syndrome (JHS) are connective tissue disorders that form an overlapping clinical syndrome and are associated with frequent medical visits and substantial morbidity. EDS-HT/JHS-associated pain correlates with poor quality of life. While physical therapy is the recommended treatment for EDS-HT/JHS, little is known about therapy-related patient experiences and iatrogenic injuries. We studied 38 adult EDS-HT/JHS patients, eliciting health-related quality of life (HRQoL) from 28 patients through the RAND SF-36 questionnaire. We also explored physical therapy experiences through focus groups with 13 patients. Our patients displayed poor HRQoL, with 71% reporting worse health over the past year. SF-36 scores were significantly lower than the scores of the average American population (P < 0.001 for 8 of 10 categories assessed), but were comparable to EDS-HT/JHS populations in Belgium, the Netherlands, Sweden, and Italy. Focus groups identified factors associated with: negative past physical therapy experiences, iatrogenic joint injuries, positive treatment experiences, and unmet rehabilitation needs. This group of EDS-HT/JHS patients has significant decrements in HRQoL and many unmet treatment needs, as well as a risk for iatrogenic injuries. We identify several approaches to help meet patients' needs and improve joint rehabilitation in patients with EDS-HT/JHS. © 2016 Wiley Periodicals, Inc.

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history

“…Children with hEDS and fatigue experienced poor health‐related QoL [Pacey et al, ]. In 38 hEDS patients, baseline QoL was significantly reduced and worsened with experiences in physical therapy and iatrogenic injury [Bovet et al, ]. A recent meta‐analysis revealed significant disability related to pain, fatigue, and psychological distress in hEDS [Scheper et al, ].…”

“…Exercise regimens are often initiated with formal physical therapy, but once learned by the patient, can and should be continued indefinitely, independent of formal instruction or supervision. It is important to understand that physical therapy should be done by experienced and learned professionals as many patients commonly report increased pain and decreased QoL with improper exercise regimens [Bovet et al, ]. It often takes several months of routine toning exercise to halt progressive deterioration in pain, and it may be several years before substantial reduction in pain is recognized.…”

Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review

“…EDS is associated with significant morbidity, including life threatening vessel and organ rupture, debilitating joint instability and pain, and prolonged, complicated wound healing . Significant disability and reduced quality of life frequently occur due to musculoskeletal problems, severe chronic pain, and persistent fatigue . Overall, EDS remains understudied and there is a lack of data on prevalence, presentations, and natural history …”

Psychological well‐being in patients with aneurysms‐osteoarthritis syndrome

“…This assumption is supported by data from patients with Ehlers–Danlos syndrome, a connective tissue disorder which presents with extreme musculoskeletal symptoms including hypermobility (De Paepe & Malfait, ). Ehlers–Danlos patients report even lower physical function score (39.6) and general health score (26.8) compared to AOS patients (Berglund et al, ; Bovet, Carlson, & Taylor, ). We did not find a difference between patients with and without orthopaedic symptoms.…”