Thinking Beyond the Joints in Patients with HSD and EDS

Hypermobility spectrum disorders (HSD) and Ehlers-Danlos syndrome (EDS) are not as uncommon as previously thought. While there are many rare types of EDS, hypermobile EDS (hEDS) and HSD are conservatively estimated to be present in 1 to 3 percent of the population in the United States—approximately 10 million people.1 This means that 30 to 55 percent of a rehabilitation therapist’s patients might be within the spectrum of HSD/hEDS2.

Even so, this particular population is commonly under-recognized, misdiagnosed, or mismanaged due to lack of knowledge within the medical community on recognition and treatment strategies. In this article, we’ll take a look at some common misconceptions about HSD/EDS patients and how they can best be treated.

Understanding HSD/EDS Patients

One common misconception about HSD and EDS is that these conditions simply make patients “bendy.” There are actually quite a number of different rare types of EDS in which generalized joint hypermobility is not as prevalent; in fact, joint contractures may be present instead.

While these conditions are much more rare and hypermobility is more common, the fact that this is the case demonstrates that we are dealing with a connective tissue disorder and not a “flexible” condition. In fact, many patients with symptomatic joint hypermobility disorders lose flexibility, developing muscle imbalances as surrounding muscles try to chronically stabilize hypermobile joints.

Patients with HSD/EDS Are Different

When an underlying connective tissue disorder is present, it affects all of the connective tissue in the body, not just joint structures. Recognizing this can expedite the diagnosis process for these patients who seem to be medical mysteries.

Knowing the commonly present conditions that exist with altered connective tissue will improve efficiency in treatment strategies. Conditions more common in HSD/EDS such as altered mast cell activity, dysregulation of the autonomic nervous system, and gastrointestinal disorders can easily change inflammation production and regulation, exacerbating a patient’s symptoms.

Patients with HSD/EDS Need a More Thorough, Coordinated Approach

Patients with HSD/EDS who are dealing with layered, systemic issues typically require more extensive medical teams. With the currently limited knowledge of HSD/EDS within the medical community—along with sometimes complex presentations—many patients do not have a complete team to address all of their personal needs. Quite often, one provider may take on a few different roles to coordinate treatment for the patient.

The key to long-term treatment and management for this population is not only patient education, but provider understanding of how each system is affected by an underlying connective tissue disorder and how each issue can impact other comorbidities or diagnoses.

Listening to the Patient Builds More Than Trust

When patients have a lifelong genetic condition that can take a long time to diagnose (even decades in many cases), signs and symptoms of multiple system issues and comorbidities can pile up over time. Listening to and hearing the patient not only builds trust but, especially with complex, layered presentations, can also paint a clearer picture of how all of their complaints are interrelated. Once the picture is realized, a more efficient treatment program can be created with longer lasting effects.

Finding Order in the Chaos

A person with HSD/EDS may be more commonly dealing with a number of layered issues. An effective medical provider for a patient with HSD/EDS will be able to act as a quarterback or refer to the appropriate providers if and when needed.

Determining an order for which provider the patient should see and when is very helpful for the patient and also for their intended outcomes in therapy. A knowledgeable provider who is familiar with the presentation of the patient’s condition and all it entails will be able to provide a more efficient and thorough treatment plan for the individual.

  1. Tinkle, B., Castori, M., Berglund, B., Cohen, H., Grahame, R., Kazkaz, H., & Levy, H. (2017). Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history. American Journal of Medical Genetics. Part C, Seminars in Medical Genetics, 175(1), 48–69.
  2. Clark, C. J., & Simmonds, J. V. (2011). An exploration of the prevalence of hypermobility and joint hypermobility syndrome in Omani women attending a hospital physiotherapy service. Musculoskeletal Care, 9(1), 1–10.