Physical therapy treatment of hypermobile Ehlers–Danlos syndrome: A systematic review

Abstract

Physiotherapy techniques are regularly prescribed in the hypermobile type Ehlers-Danlos syndrome (hEDS) and they are appreciated by the patients. The objective of this systematic review was to investigate the effect of the different physiotherapy techniques related to the children and adult patients with hEDS. PubMed, SPORTDiscus, Cochrane Library, PEDro, Scopus, and Embase databases were analyzed from inception to April 2020. Characteristics of the studies (authors), patients (sample size, sex, age, Beighton score), and nonpharmacological treatment (length of the program, number of session, duration of the session, and type of intervention), and the results with the dropout rate were extracted. From the 1045 retrieved references, 6 randomized controlled trial with a sample size ranging from 20 to 57 patients were included in the systematic review. There was a huge heterogeneity in the interventions. The durations of the program were from 4 to 8 weeks. Pain or proprioception demonstrated significant improvements in the intervention group regardless of the type of intervention. A benefit of the inspiratory muscle training was observed on functional exercise capacity. The quality of life was systematically improved. Physiotherapy benefits on proprioception and pain in patients with hEDS even if robust randomized control studies are missing.

The value of dynamic elastomeric fabric orthoses in the management of a complex hypermobile Ehlers‐Danlos syndrome patient: A case report

“…Long‐term physical inactivity leads to physical deconditioning, 54 including decreased muscle strength and cardio‐respiratory fitness. Despite studies identifying kinesiophobia as a possible barrier to exercise, 53 the mainstay of treatment for HSD/hEDs is exercise and pain management 41 . Literature conveys that these patients already have higher levels of anxiety, 55 which may also have a dysautonomia element with symptoms such as hyperventilation, nausea, and light‐headedness, which are significantly more common in HSD/hEDS patients 56 .…”

“… 37 The patient's decreased awareness of her body posture meant she was unable to maintain the position, which decreased her pain; therefore, we wanted to assess to see whether a DEFO, in the form of leggings, which also encompassed the hip and pelvic region, could assist in reducing her sway back posture. Core control, hip, lower limb, breathing, and proprioceptive exercises 41 were tailored to her needs so that they were manageable; paced to accommodate her pain, chronic fatigue, and dizziness; and were relevant to helping her function postoperatively. In a systematic review of exercise programs for hEDS patients, a period of 4–8 weeks was commonly reported in studies.…”

The value of Dynamic Movement Orthoses in the management of a complex Hypermobile Ehlers-Danlos Syndrome patient: a case report

“…Long-term physical inactivity leads to physical deconditioning [54], including decreased muscle strength and cardio-respiratory fitness. Despite studies identifying kinesiophobia as a possible barrier to exercise [53], the mainstay of treatment for HSD/hEDs is exercise and pain management [41]. Literature conveys that these patients already have higher levels of anxiety [55], which may also have a dysautonomia element with symptoms such as hyperventiliation, nausea and light-headedness which are significantly more common in HSD/hEDS patients [56].…”

“…The patient's decreased awareness of her body posture meant she was unable to maintain the position which decreased her pain, therefore we wanted to assess to see if a DMO(r), in the form of leggings which also encompassed the hip and pelvic region, could assist in reducing her sway back posture. Core control, hip, lower limb, breathing and proprioceptive exercises [41] were tailored to her needs so that they were manageable; paced to accommodate her pain, chronic fatigue and dizziness; and were relevant to helping her function post-operatively. In a systematic review of exercise programmes for hEDS patients, a period of 4-8 weeks was commonly reported in studies [41].…”

“…Core control, hip, lower limb, breathing and proprioceptive exercises [41] were tailored to her needs so that they were manageable; paced to accommodate her pain, chronic fatigue and dizziness; and were relevant to helping her function post-operatively. In a systematic review of exercise programmes for hEDS patients, a period of 4-8 weeks was commonly reported in studies [41]. Our exercise programme had a longer time span (16 weeks) as it was dictated by the planned surgery date.…”