title: 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.
authors.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital HF, Nesodden, Norway.
authors.forename: Trine
authors.initials: T
authors.lastname: Bathen
authors.forename: Anett Bjørnødegård
authors.initials: AB
authors.lastname: Hångmann
authors.forename: Marie
authors.initials: M
authors.lastname: Hoff
authors.forename: Liv Øinaes
authors.initials: LØ
authors.lastname: Andersen
authors.forename: Svend
authors.initials: S
authors.lastname: Rand-Hendriksen
mesh_terms: D000328:Adult; D015928:Cognitive Behavioral Therapy; D004535:Ehlers-Danlos Syndrome / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D015444:Exercise; D005260:Female; D006801:Humans; D007593:Joint Instability / Q000628:therapy* / Q000151:congenital; D008875:Middle Aged; D018908:Muscle Weakness / Q000503:physiopathology / Q000628:therapy; D059408:Pain Management; D010865:Pilot Projects
keywords: cognitive therapy; cognitive-behavioral approach; exercise therapy; joint hypermobility; multidisciplinary rehabilitation; pain coping; physical training