Reviewed by Maggie McPherson, SPT

Article:

An Evidence-Based Clinical Commentary for Treating Patients with Hypermobile Ehlers-Danlos Syndrome or a Hypermobility Spectrum Disorder published in Orthopaedic Physical Therapy Practice

Dr. Joseph Signorino, DPT, DSc, Dr. Samuel Bikkers, DPT, and Dr. Kate Divine, DPT put together this clinical commentary to guide physical therapy treatment of individuals with Ehlers-Danlos Syndrome (EDS) or Hypermobility Spectrum Disorder (HSD). They worked to combine evidence-based interventions with their own clinical expertise and clinical reasoning to provide coherent guidelines. The main principles of their commentary included options for monitoring and addressing common symptoms and deficits seen in individuals with EDS and HSD, the importance of education and empathy, the incorporation of the pelvic floor, and some helpful insights into the role of manual therapy for individuals with EDS or HSD.

Some of the common barriers to efficient and pain free movement patterns for individuals with EDS or HSD are identified by the authors as pain, fear of movement, fatigue, and decreased proprioception. Advised interventions to modulate pain perception and decrease fear of movement included education and empathy, graded exposure, progressive resistive exercise (PRE) beginning with gentle isometrics, and aerobic exercise between 40-75% VO2max or at a Borg RPE rating of 13. Aerobic exercise and PRE with isometrics were also recommended as interventions to address fatigue. The authors underlined that since fatigue and pain are common side effects of both resistive and aerobic exercise, clinicians should ensure they are properly dosing exercise interventions, and may need to considerably scale down their resistance depending on how the individual presents. Hence, these authors recommend graded exposure: a gradual progression from isometrics, to multi angle isometrics, to isotonics with gradual progression to resisted movements in multiple planes according to the functional needs of the individual. Additionally, the authors note it is important to be aware of vascular presentations of EDS that need to be seriously considered when dosing any sort of exercise.

Another intervention the authors addressed was manual therapy. While traditionally manual therapy is seen as a means to improve joint mobility and flexibility, which is typically not the goal for the individual with EDS or HSD, the authors identify other potential benefits. They highlight commonly cited effects of manual therapy including changes in pain perception and modulation of the autonomic nervous system which could be beneficial to address pain and kinesiophobia. Regarding proprioception deficits, the authors mention taping, compression garments,  and motor control interventions as potential options for treatment.

Pelvic floor dysfunction is another common symptom that these individuals experience. The authors discussed interventions such as scheduled voiding, urgency strategies, education, and pelvic floor strengthening to mitigate organ prolapse. Here the authors also took time to talk about the importance of education and empathy. They found that individuals with EDS consistently report feeling that their health care providers do not have a good understanding of their condition and do not empathize with their symptoms. Each individual with EDS has a unique presentation, but a solid understanding of the nature of syndrome can help guide clinicians to appropriate treatment strategies.

Clinical Bottom Line:

These authors emphasize a personalized approach, carefully considering graded exposure and pain modulation to address kinesiophobia, pain and fatigability, using a variety of interventions that include gradual progressive resisted exercise starting from isometrics, aerobic exercise, manual therapy, taping, education, and empathy. Additionally, they highlighted the need for pelvic floor interventions as prolapse and incontinence can be concerns for individuals with EDS and HSD.

References:

Signorino JA, Bikkers SJ, Divine K. An Evidenced-Based Clinical Commentary for Treating Patients with Hypermobile Ehlers-Danlos Syndrome or a Hypermobility Spectrum Disorder. Orthopaedic Physical Therapy Practice. 2023;35(3):28-36. Accessed July 16, 2024. https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=164775891&site=eds-live