Multidisciplinary treatment of disability in ehlers–danlos syndrome hypermobility type/hypermobility syndrome: A pilot study using a combination of physical and cognitive‐behavioral therapy on 12 women

Abstract

Ehlers-Danlos Syndrome hypermobility type (EDS-HT) and joint hypermobility syndrome (JHS) are two overlapping heritable connective tissue disorders. Patients with these conditions have many and various complaints; limitations in performing daily activities, reduced muscle strength and proprioception, kinesiophobia, and pain. There is a lack of evidence-based treatment approaches; a few studies have shown effect of physiotherapy. Many authors propose multidisciplinary treatment, but this has neither been described nor evaluated for this patient group. The aim of this pilot study was to investigate if a multidisciplinary rehabilitation program combining physical and cognitive-behavioral therapy was feasible, safe and effective for 12 women with EDS-HT/JHS. Intervention was offered as a group program and consisted of three parts: (1) Two and a half week in a rehabilitation unit with testing, physical training, group discussions and lectures. (2) Individual home exercises for three months with weekly guidance by local physiotherapist. (3) Readmission four days for retesting and further training advice. All participants completed the intervention. We found significant changes in perceived performance of daily activities, significant increase of muscle strength and endurance and a significant reduction of kinesiophobia. There were smaller changes in self-perceived pain. The participants also reported increased participation in daily life.

Psychiatric and psychological aspects in the Ehlers–Danlos syndromes

“…However, there are no studies exploring the coping strategies in JHS/hEDS and psychological aspects of pain perception merits more research to develop treatments programs. Some pilot cognitive behavioral therapy (CBT) experiences have been developed and suggested that CBT is valuable in the pain management of JHS/hEDS patients [Bathen et al, ].…”

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

“…This type of presentation should be considered as a centralized pain state belonging to the spectrum of chronic widespread pain, with additional superimposed musculoskeletal components. Some patients who continue to struggle to cope with their pain may need consideration of a multidisciplinary pain management program [Bathen et al, ].…”

“…Psychological counseling should focus on accepting and coping with chronic pain and chronic disease. Cognitive behavioral therapy is particular beneficial, if the patient is willing to actively engage in the process [Bathen et al, ]. Distraction, hypnosis, and judicious use of anti‐depressant medication can also help.…”

Genomic Analysis of 727 Patients with Ehlers-Danlos Syndrome I: Clinical Perspective Relates 23 Genes to a Maternally Influenced Arthritis-Adrenaline Disorder

“…A predominant myopath- ic category would correlate with AAD symptoms in many non-CTD conditions that exhibit hypotonia/poor muscle development [53] and explain why 6 patients with developmental disabilities had DNA variants of relevance to AAD in Table 2. Less muscle support and protection leading to joint-tissue laxity would explain the established benefits of exercise [54] and physical therapy [55] for EDS. Impact on the various tissue elements shown in Figure 3 adds focus on patient-derived stem cells that could repair multiple mesodermal tissues [56] [57], their gene defects characterized by WES and perhaps corrected by emerging technology [58].…”