Hypermobile Ehlers-Danlos Syndrome

Clinical Decision Support
1

OT Clinical Decision Support for hEDS

Occupational therapy is always tailored to the needs of the individual client. But, this page is intended to give therapists a possible starting point of ideas to consider for people with Hypermobile Ehlers-Danlos Syndrome (hEDS), based on the latest research and expert input. This list is not intended to replace clinical decision making.

You can find OT professionals who have Ehlers-Danlos Syndrome tagged a focus area here.

Possible assessments:

`Ehlers-Danlos Syndrome Assessments

Possible goal areas:

  • Joint protection, positioning, ADL modification
  • Fatigue and energy management
  • Pain education and management
  • Sleep education and management
  • Strengthening and stability
  • Proprioception and body awareness
  • Balance, postural sway
  • Postural alignment
  • Interoception
  • Sensory processing
  • Ergonomic positioning and work setup
  • Work and school exploration, accommodations, and support
  • Leisure and play pursuits
  • Social participation
  • Splinting/ orthotic management
  • Mental health support and coping skills
  • Health management
  • Self-advocacy skills
  • Education on condition, exacerbating and relieving factors, management, and co-occurring conditions as appropriate.
  • Home program

Possible treatment interventions:

  • Strengthening (stability, low and slow) 2,7
  • Joint protection 8
    • Positioning
    • ADL modification
    • Splinting/ orthotic management 9
  • Fatigue and energy management 3,10
    • Pacing
    • Energy conservation
    • Work simplification
  • Fine motor skills and hand function 11
    • Handwriting
    • Dressing, buttoning
  • Pain education and management 3,10,12,13
    • Pain neuroscience education
    • Coping skills and flare kit
    • Thought management
    • Distraction
    • Mindfulness
  • Sleep education and management 3
    • Sleep hygiene
    • Routines
  • Proprioception and body awareness 14,15,16
  • Balance, postural sway 4
  • Postural alignment 8
  • Interoception 17,18,19
  • Sensory processing 20
  • Ergonomic positioning and work setup
  • Work and school exploration, accommodations, and support 2, 22
  • Leisure and play pursuits
  • Social participation 23
  • Mental health support and coping skills 10
    • Validation 24
    • Working toward acceptance
  • Health management
    • Increasing self-awareness of symptoms 24
    • Pregnancy and post-partum considerations 10
  • Self-advocacy skills 10, 25
  • Education 10, 26
    • Condition pathophysiology
    • exacerbating and relieving factors
    • management
    • co-occurring conditions as appropriate
    • How to find and evaluate information related to HSD/hEDS
  • Home program 25
    • Increasing motivation, adapting routines, seeing benefit, and partnering with family/others helps increase adherence to exercise 27

Discharge suggestions:

  • Home exercise program
  • Community supports
  • Self-management and empowerment

Red flags:

Caution:

  • Skin integrity
  • Easy bruising
  • Dislocations and subluxations
  • Upper cervical instability
  • Orthostatic intolerance (dehydration, prolonged static standing, positional changes, extremes of temperatures)
  • Mast cell activation (sensitivities to odors, perfumes, extreme temperatures, vibration, exertion, and more)
  • Vascular Ehlers-Danlos is a very different life-limiting condition

Education Resources:

Clinician Education

Patient Education

Handouts:

References:

  1. Lyon S, Rich E. OT and Ehlers-Danlos Syndrome. OT Potential Podcast | Occupational Therapy CEUs. 2024.

  2. Hakimi A, Bergoin C, Mucci P. Immediate and 6-week after effects of a rehabilitation program for Ehlers–Danlos syndrome hypermobile type patients: A retrospective study. American Journal of Medical Genetics Part A. 2020;182(10):2263-2271.

  3. Hakimi A, Bergoin C, Mucci P. What are the most important symptoms to assess in hypermobile Ehlers-Danlos syndrome? A questionnaire study based on the Delphi technique. Disability and Rehabilitation. 2022;44(26):8325-8331.

  4. Iatridou K, Mandalidis D, Chronopoulos E, Vagenas G, Athanasopoulos S. Static and dynamic body balance following provocation of the visual and vestibular systems in females with and without joint hypermobility syndrome. Journal of Bodywork and Movement Therapies. 2014;18(2):159-164.

  5. Ewer ER, De Pauw R, Kazkazk H, et al. The Spider: a visual, multisystemic symptom impact questionnaire for people with hypermobility-related disorders—validation in adults. Clin Rheumatol. 2024;43(9):3005-3017.

  6. Nicholson LL, Chan C. The Upper Limb Hypermobility Assessment Tool: A novel validated measure of adult joint mobility. Musculoskeletal Science and Practice. 2018;35:38-45.

  7. Scheper M, Rombaut L, de Vries J, et al. The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers–Danlos syndrome: the impact of proprioception. Disability and Rehabilitation. 2017;39(14):1391-1397.

  8. Keer R, Simmonds J. Joint protection and physical rehabilitation of the adult with hypermobility syndrome. Current Opinion in Rheumatology. 2011;23(2):131.

  9. Jensen AM, Andersen JQ, Quisth L, Ramstrand N. Finger orthoses for management of joint hypermobility disorders: Relative effects on hand function and cognitive load. Prosthetics and Orthotics International. 2021;45(1):36.

  10. Bennett SE, Walsh N, Moss T, Palmer S. Developing a self-management intervention to manage hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos syndrome (hEDS): an analysis informed by behaviour change theory. Disability and Rehabilitation. 2022;44(18).

  11. Wesley A, Bray P, Pacey V, Chan C, Nicholson LL. Hand Impairment and Function in Children and Adolescents With Heritable Disorders of Connective Tissue. The American Journal of Occupational Therapy. 2022;76(6):7606205030.

  12. Jones JT, Black WR, Cogan W, Callen E. Resource utilization and multidisciplinary care needs for patients with Ehlers–Danlos syndrome. Mol Genet Genomic Med. 2022;10(11):e2057.

  13. Chew MT, Ilhan E, Nicholson LL, Kobayashi S, Chan C. An online pain management program for people with hypermobile Ehlers-Danlos Syndrome or hypermobility spectrum disorder: a three-staged development process. Disability and Rehabilitation. 0(0):1-11.

  14. Clayton HA, Jones SAH, Henriques DYP. Proprioceptive precision is impaired in Ehlers-Danlos syndrome. Springerplus. 2015;4:323.

  15. Ferrell WR, Tennant N, Sturrock RD, et al. Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Arthritis Rheumatology. 2004;50(10):3323-3328.

  16. Smith TO, Jerman E, Easton V, et al. Do people with benign joint hypermobility syndrome (BJHS) have reduced joint proprioception? A systematic review and meta-analysis. Rheumatol Int. 2013;33(11):2709-2716.

  17. Di Lernia D, Serino S, Riva G. Pain in the body. Altered interoception in chronic pain conditions: A systematic review. Neuroscience & Biobehavioral Reviews. 2016;71:328-341.

  18. Mallorquí-Bagué N, Garfinkel SN, Engels M, et al. Neuroimaging and psychophysiological investigation of the link between anxiety, enhanced affective reactivity and interoception in people with joint hypermobility. Front Psychol. 2014;5.

  19. Niermeyer M, Ball D, Green M, et al. Interoceptive attention regulation in Ehlers–Danlos syndromes: associations between pain and psychiatric symptom severity. Transl Behav Med. 2021;11(10):1923-1930.

  20. Baeza-Velasco C, Bourdon C, Montalescot L, et al. Low- and high-anxious hypermobile Ehlers-Danlos syndrome patients: comparison of psychosocial and health variables. Rheumatol Int. 2018;38(5):871-878.

  21. De Baets S, Calders P, Verhoost L, et al. Patient perspectives on employment participation in the “hypermobile Ehlers–Danlos syndrome.” Disability and Rehabilitation. 2021;43(5):668-677.

  22. Hill JC. Zebras in the workplace: Vocational rehabilitation considerations for individuals with Ehlers-Danlos Syndrome. Journal of Vocational Rehabilitation. 2017;47(2):197-206.

  23. Baeza-Velasco C, Bourdon C, Montalescot L, et al. Low- and high-anxious hypermobile Ehlers-Danlos syndrome patients: comparison of psychosocial and health variables. Rheumatol Int. 2018;38(5):871-878.

  24. Scheierman H. How Illness Hurts: The Impact of Ehlers Danlos Syndrome on Well-Being and Self-Esteem. Doctoral Dissertation. Alliant International University; 2024.

  25. Bovet C, Carlson M, Taylor M. Quality of life, unmet needs, and iatrogenic injuries in rehabilitation of patients with Ehlers–Danlos Syndrome hypermobility type/Joint Hypermobility Syndrome. American Journal of Medical Genetics Part A. 2016;170(8):2044-2051.

  26. Feldman ECH, Homan KJ, Williams SE, Ting TV, Goldschneider KR, Kashikar-Zuck S. A narrative review of the literature on illness uncertainty in hypermobile ehlers-danlos syndrome: implications for research and clinical practice. Pediatric Rheumatology. 2023;21(1):121.

  27. Birt L, Pfeil M, MacGregor A, Armon K, Poland F. Adherence to Home Physiotherapy Treatment in Children and Young People with Joint Hypermobility: A Qualitative Report of Family Perspectives on Acceptability and Efficacy. Musculoskeletal Care. 2014;12(1):56-61.

Contributors:

Emily Rich, PhD., OTR/L
Sarah Lyon, OTR/L

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