Pain management in the Ehlers–Danlos syndromes

Pain management in the Ehlers–Danlos syndromes – American Journal of Medical Genetics Part C: Seminars in Medical Genetics – February 2017 – free full-text article

All the authors are internationally recognized EDS specialists: Brad Tinkle, Claude Hamonet, Isabelle Brock, Anne Gompel, Antonio Bulbena, Clair Francomano

Chronic pain is one of the major symptoms presented by patients with hEDS [Sacheti et al., 1997; Voermans et al., 2010].

It often presents as diffuse body pain affecting almost every part of the body.

It is common and may be severe [Voermans et al., 2009]. 

In one study, the prevalence of chronic pain was 90% in patients with various types of EDS, with the highest scores on severity of pain found in hEDS [Voermans et al., 2009].

Pain and fatigue have a high prevalence in EDS, frequently manifesting as the predominant symptoms and as the most disabling features [Rombaut et al., 2011].

Clinical examination, pain questionnaires, quantitative sensory testing, and neurophysiological responses disclosed no somatosensory nervous system damage.

In a study of 206 female patients with EDS, the impact of pain and functional impairment was similar to fibromyalgia but worse than that of rheumatoid arthritis [Rombaut et al., 2011].

Any form of pain be it nociceptive or neuropathic may be a secondary or even tertiary effect of underlying causes


Chronic pain in the Ehlers–Danlos syndromes (EDS) is common and may be severe. According to one study, nearly 90% of patients report some form of chronic pain.

Pain, which is often one of the first symptoms to occur, may be widespread or localized to one region such as an arm or a leg.

Studies on treatment modalities are few and insufficient to guide management.

The following is a discussion of the evidence regarding the underlying mechanisms of pain in EDS.

The causes of pain in this condition are multifactorial and include

  • joint subluxations and dislocations,
  • previous surgery,
  • muscle weakness,
  • proprioceptive disorders, and
  • vertebral instability.
  • Affected persons may also present with
  • generalized body pain,
  • fatigue,
  • headaches,
  • gastrointestinal pain,
  • temporomandibular joint pain,
  • dysmenorrhea, and
  • vulvodynia.

Pain management strategies may be focused around treating the cause of the pain (e.g., dislocation of a joint, proprioceptive disorder) and minimizing the sensation of pain.

Management strategies for chronic pain in EDS includes physical therapy, medications, as well as durable medical equipment such as cushions, compressive garments, and braces. The different modalities are discussed in this paper.


Pain is common in Ehlers–Danlos syndrome (EDS) and may correlate with

  • hypermobility,
  • frequency of subluxations and dislocations,
  • soft tissue injury,
  • history of previous surgery,
  • myalgias, and
  • may become chronic.

Pain may be musculoskeletal and/or widespread.

It may be acute and/or chronic.

The pain may interfere with socialization and activities of daily living.

Table I. Review of Literature of Types of Pain in hEDS

Manifestations Number of patients studied Incidence (%) References
Generalized body pain >800 (cumulative) 90 Jerosch and Prymka [1996]; Camerota et al. [2011]; Hamonet et al. [2012, 2014]; Hamonet and Brock [2015]; Scheper et al. [2015]; Voermans and Knoop, 2011
Soft-tissue pain >800 (cumulative) 90 Hudson et al. [1998]; Hamonet et al. [2012, 2014]; Scheper et al. [2015]
Dislocations >800 (cumulative) 78 Voermans et al. [2010]; Hamonet et al. [2012, 2014]
Joint pain 28* Elbow (43)* Moore et al. [1985]; Aktas et al. [1989]*; Sacheti et al. [1997]; Tubiana [2000]; Berglund et al. [2005]; McCulloch and Redmond [2010]; Hamonet et al. [2012]^; Hamonet et al. [2014]; Hamonet and Brock, 2015#]; Christopherson and Adams [2014]; Scheper et al. [2015]
232# Shoulders (80)*
644^ Hands (75)*
Knees (71)*
Spine (67)*
Fatigue 644 [cumulative] 95 Gulbahar et al. [2006]; Voermans et al. [2009, 2010]; *Celletti et al. [2012]; Hamonet et al. [2012]
11 [cumulative] 6 (55)*
Bone loss 23 16 (70) Gulbahar et al. [2006]
Neuropathic pain 29* 68* DeGraaf [1973]; Kass and Kayed [1979]; Stoler and Oaklander [2006]*; Camerota et al. [2011]; Voermans et al. [2011]
Loss of proprioception 18*, 32#, 22^ Significant P-value Helliwell [1994]*; Ferrell et al. [2004]; Fatoye et al. [2009]; #Rombaut et al. [2010]; Zarate et al. [2010]; Celletti et al. [2011]; Galli et al. [2011]; ^Clayton et al. [2013]; Smith et al. [2013]; Deparcy [2016]
Headaches 28* 75* Sansur et al. [2003]; Schievink et al. [2004]; DeCoster et al. [2005]; Henderson et al. [2005a]; Gulbahar et al. [2006]; Milhorat et al. [2007]*; Bendik et al. [2011]; Rozen [2014]; Hamonet and Brock [2015]
Gastrointestinal pain 21* 85.7* Douglas and Douglas [1973]; Petros and Swash [2008]; Castori et al. [2010]*; Zarate et al. [2010]; Dordoni et al. [2015]; Hamonet and Brock [2015]; Mohammed et al., 2010
Temporomandibular joint pain 42* 71.4* *DeCoster et al. [2004, 2005]; Hagberg et al. [2004]
Menorrhagia 387 77.57 Gompel [2016]
Dysmenorrhea 73.1
Vulvodynia/dyspareunia 387 42 Gompel [2016]


The article goes on to delve into a detailed description of the kinds of pain resulting from EDS: Pain management in the Ehlers–Danlos syndromes


2 thoughts on “Pain management in the Ehlers–Danlos syndromes

  1. BirdLoverInMichigan

    We suffer from overwhelming and constant pain and fatigue with Ehlers Danlos Syndrome.

    I have the hyper mobile form. It’s a big drag. There’s no glory in being an imploding Gumby at odds with the earth’s gravitational pull.

    Why is the world 🌎 still in the dark on this?

    We need a high profile spokesperson to give our disorder an identity. Jim Carrey, IF you have it like I suspect you do, are you listening?

    Liked by 2 people

    1. Zyp Czyk Post author

      I don’t expect docs to know about every possible disease and disorder and syndrome people can have because there are just too many.

      Instead, they should do a little research in PubMed and educate themselves instead of working with their limited and probably outdated knowledge. Just like even pro software engineers Google search the latest programming tools & tips, doctors should access updated and relevant information stored in a database like PubMed whenever they encounter something they don’t have recent knowledge or experience with.

      To me, looking things up is a sign of intelligence and humility, a simple acknowledgement that there could be new info available because science is always adapting to the latest discoveries (at least when it’s working right).

      If my doc could only treat me for things she happens to have learned before and never dealt with since, she’d risk big mistakes and oversights. Docs have to keep learning just like software engineers or they become obsolete and dangerous to others.

      I had a doc, an experienced and expert neurologist of all things, who would break out the Physician’s Desk Reference (first, the huge book, then an electronic file he kept on his cell phone, and finally an online database) before writing me a new prescription so we could discuss the benefits, harms, and dosages together. What a guy!!

      Liked by 1 person


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