The Hypermobility Spectrum, EDS and Connective Tissue Disorders
Hypermobility can occur in just one joint, or in many. A physical trauma can create a local hypermobility by overstretching the ligaments or passive tissue around a joint, allowing the joint to move excessively. The connective tissue around a joint is non-contractile and has low elasticity, so when it becomes lax, activity of muscles helps to prevent excessive motion. Muscles that are not strong enough for this task can become tired, achy, or even feel “tight,” which often confounds clinicians who observe that the “tight” hypermobile patient presents with excessive mobility. Hypermobility can be symptom-free, so it is important to find an experienced physical therapist who can help determine whether or not hypermobility is contributing to your symptoms and help to create an individualized care plan.
If you inherit hypermobile connective tissue you may have widespread/general hypermobility, and may have been told you are “double-jointed.” Historically, there are many names for the cluster of signs and symptoms that tend to occur with hypermobile connective tissue: “benign joint hypermobility,” “hypermobility syndrome” and Ehlers Danlos Syndrome-III are some of the outdated terms that indicate that your pain, fatigue and symptoms may fit the criteria for what is currently known as a hypermobility spectrum disorder (HSD.) Other inherited connective tissue disorders that may present with hypermobility include rarer types of EDS, Marfan Syndrome, Larson Syndrome or Osteogenesis Imperfecta. The criteria for hypermobility has changed a number of times in recent decades, with the most recent update in 2017 adjusting the criteria for hypermobile EDS (hEDS) to be more stringent in an attempt to narrow this population down to a more homogeneous group in the hopes that the genetic contributions to hEDS may be identified. This is important because currently hEDS is the most common type of EDS and it is the only type with unknown genetic markers. The 2017 criteria made it very clear that any evidence of hypermobility warrants identification and management, regardless of where it lands on the spectrum and whether or not it meet the criteria for EDS. As a PT (who has been through this diagnostic process herself) Dr. Lewan can provide an assessment of hypermobility and make recommendations that may include referral to a medical doctor who can rule in/out a diagnosis of EDS. As a medical diagnosis, EDS is a diagnosis that needs to be made by an MD. However, physicians competent in current EDS criteria are scarce and a wait list of 2 years is not uncommon. There is no need to wait for a diagnosis to begin treatment, as EDS and hypermobility are both treated primarily with self-management tools of education, appropriate exercise, restoration of sleep and nutrition as needed, modification/adaptation to activities and pacing.
Mobility exists on a spectrum– some people have connective tissue that is really stiff and others have tissues that are quite flexible. Most people live between these two extremes and general exercises are usually designed for this majority of the population. Those who are significantly hypermobile may have injuries associated with tissue fragility and a sensitive nervous system that has adapted to chronic stress by amplifying pain in an attempt to protect. While hypermobility and associated symptoms can be assessed by a trained physical therapist, and treatment/self-management of symptoms can begin regardless of where you are in the diagnostic process.
Regardless of where you land on the hypermobility spectrum, it is important to find a care team with experience treating hypermobility and its possible effects on the musculoskeletal system, nervous system, immune system, gastrointestinal system, and cardiovascular system… as well as the psychosocial system. A multidisciplinary and biopsychosocial approach is recommended, especially if your symptoms are chronic and involve multiple body parts or systems.
Individualized conservative (non-invasive) and evidence-based management of hypermobility may include:
- Learning about hypermobility, neuroscience, pacing and the benefits of gradual return to appropriate activity levels.
- Techniques to improve the brain’s ability to inhibit pain and chronic symptoms, including: manual therapy, desensitization, meditation, visualization, physical conditioning, graded exposure to activities, cognitive behavioral strategies, breathing techniques and exercises to improve awareness of danger/safety messages that have been shown to ramp up or down chronic symptoms.
- Various forms of feedback and biofeedback to improve proprioception and dynamic stability.
- Exercise adaptations to promote stability and strength, improve endurance and reduce risk of injury or symptom flares.
- Recommendations for additional educational materials, health care professionals, groups or services that may facilitate a holistic and multidimensional approach to improve your sense of well-being, self-efficacy, and empowered self-management of your symptoms.
Dysfunction of the autonomic nervous system (ANS), often referred to as dysautonomia, involves the bodily functions that are controlled automatically, like heart rate, blood pressure and sweating. Dysautonomia often occurs with hypermobility, Diabetes Mellitus, Parkinson’s Disease and as a response to trauma or hospitalization.
It is estimated that 80% of individuals with hypermobile Ehlers Danlos Syndrome (hEDS) also have Postural Tachycardia Syndrome (POTS,) a form of Dysautonomia where the heart beats faster to drive blood in the lower body up toward the heart and brain. Symptoms occur acutely with changes in position and may lead to chronic fatigue, nausea, dizziness, feeling faint or ill, and pain in the “coat-hanger” region of the head, neck, upper back and shoulders. While the correlation between EDS and POTS is not well understood, both conditions improve with appropriate exercise intervention. However, inappropriate exercise can worsen symptoms in the short-term, which can discourage these individuals from exercising and lead to long-term worsening of symptoms as deconditioning sets in. Evidence has shown that gradual, progressive exercise can lower tachycardia and help to manage symptoms of POTS and dysautonomia.
Conservative (non-invasive, non-pharmalogical) evidence-informed treatment of dysautonomia/POTS may include:
- Gradual, progressive exercise to improve tachycardia and tolerance to activity
- Strengthening to improve muscle tone and support blood vessels
- Breathing strategies to reduce shortness of breath, dizziness, and other symptoms of dysautonomia
- Self-management techniques to improve hydration, electrolyte balance and become aware of triggers
- Pacing and energy conservation, sleep strategies and educational resources for management of fatigue
- Treatment and referral for co-existing pain, functional limitations and/or psychosocial distress due to symptoms
Finding the right approach for you…
If you have hypermobility/EDS and/or dysautonomia/POTS and have found exercise or Physical Therapy to be unhelpful in the past, please don’t be discouraged. You may need to try several different approaches (and perhaps several therapists) before finding a good fit. Whenever possible, try to find a therapist that has experience with these conditions and/or is willing to learn. Even with an expert therapist/therapy team, trial and error is required to find the most effective and efficient approach for you.
Options for working with Dr. Cat Lewan
It is unique to be able to work with a clinician who understands these conditions both professionally and personally. Dr Lewan has hypermobile EDS and POTS and is very empathetic toward those dealing with these issues. There is a high demand for specialized care and a limited number of online consultations are available for those who do not have access to a hypermobility specialist for rehabilitation/management.
If you are seeking in-person physical therapy or telehealth PT in the state of Illinois, you will be directed to Chicago Physical Therapists where Dr. Lewan sees patients. Due to high demand, there are times when she is not able to accommodate new patients and you may be given the option of having her perform an extended evaluation/assessment and making recommendations for your care so that another therapist of your choice can continue with ongoing treatment. She is also available for a limited number of online consults to provide education, resources and self-management tools from a distance.