Ehlers Danlos Syndrome and Hypermobility Spectrum Disorder Awareness Month

May is International Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD) Awareness Month. Awareness of these conditions in our Pilates Teacher community is imperative. Movement Therapies play a pivotal role in managing EDS and HSD. An EDS and HSD-aware Pilates Teacher can be an invaluable member of the health support team for people living with these disorders. On the flip side, a lack of awareness could lead to a less-than-optimal movement experience and a deepening of already present movement hesitancy. Pilates is being recommended and sought out by people with these conditions as an effective and safe way to strengthen their bodies. I hope the following information helps the Pilates community to be able to be more aware of EDS and HSD.

There are 13 types of EDS and joint hypermobility is present in all of them. All types, except the Hypermobile EDS (hEDS), are rare and are diagnosed via genetic testing. hEDS is diagnosed via an hEDS diagnostic checklist. The diagnosis is made by an EDS-aware Rheumatologist or suitably qualified Physiotherapist or Occupational Therapist.

Hypermobile EDS has been considered a rare disorder. However, there is growing awareness that it is more common than previously thought. It is often missed or misdiagnosed as something else e.g. fibromyalgia. In fact, the prevalence of hEDS and HSD is high. HSD is the most common heritable connective tissue disorder. hEDS makes up 85- 90% of all EDS types.

HSD is diagnosed when joint hypermobility is present and has become symptomatic, but the criteria for an hEDS diagnosis are not met. Both EDS and HSD are heritable disorders of connective tissue that are passed from parent to child. hEDS and HSD can be difficult to distinguish from each other. Having an HSD diagnosis does not necessarily mean that the symptoms are less severe. HSD can be equally as debilitating. The main difference is that in hEDS the skin is a lot more fragile and heals poorly.

Symptoms

hEDS and HSD present differently in each person. Along with musculoskeletal issues, there can be many co-morbidities present. All systems of the body have connective tissue, therefore many of the body’s systems are affected and may be symptomatic. Here are some examples of symptoms in the various body systems that can occur.

The musculoskeletal system: joint hypermobility and instability, sprains, muscle spasms, muscle pain, joint pain, jaw pain, headaches, osteoarthritis, fibromyalgia, myofascial pain, osteopenia, osteoporosis, joint hypermobility, subluxations/ dislocations (ribs, shoulders, hips).

The nervous system: anxiety disorders, heightened sympathetic nervous system, dysautonomia, POTS, elevated heart rate, feinting, low blood pressure, poor temperature control, difficulty sleeping, chronic fatigue.

The cardiovascular system: elevated heart rate, fainting, low blood pressure.

The gastro-intestinal system: Abdominal pain, diarrhoea, constipation, gastroparesis.

The integumentary system (skin): easy bruising, stretch marks, indented scarring, poor wound healing, frequent hernias, varicose veins.

The immune system: allergic reactions, sensitivity to medications, mast cell activation system.

The urogenital system: heavy, painful periods, prolapses, chronic bladder inflammation, UTI’s.

 

Research

The research into Pilates as a modality to help people with hSD and hEDS is positive. A 2016 study by J. Simmonds et al found that Pilates, walking and swimming were the three most beneficial types of exercise, as reported by individuals with HEDS/ HSD. In 2017 a study of an 8-week stabilisation program significantly improved pain, balance and spinal muscle endurance (Toprak Celenay, S. and Ozer Kaya, D).

During the COVID-19 pandemic, Polestar Pilates Instructor, Jeannie Di Bon (based in the UK) ran an online Pilates program. She found that 88% would recommend the program.

 

Recommendations for Management

The Ehlers Danlos Society has recommendations for therapeutic exercise programs for those with hEDS or HSD. A tailored Studio Pilates session can be designed in a way that adheres to these guidelines.

There are key considerations when working with this population. They have specific needs in an exercise program due to their different connective tissue structure. The passive stability in joints is compromised due to the less stiff ligaments and tendons. As a result the neuromuscular system has to step up and work harder. This is challenging and fatiguing.

Also, a person hEDS and HSD is often deconditioned. Sadly, previous attempts to participate in exercise and strengthening programs often lead to increased pain and or injury. Moving less ensues. These clients therefore are starting their Pilates at a lower baseline, in terms of strength, than the connective tissue typical person. Another key consideration is that this population has muscle imbalance between the deep and superficial muscular systems. They tend to over-recruit the larger, more superficial muscles and struggle with activation of the deep stabilisers. Supportive positions, breathing and relaxation techniques can assist in down training hypertonic muscles.

When working with EDS or HSD it is recommended that the Pilates Teacher:

• Choose closed chain exercises for the poorly controlled joints
• Choose supportive positions and implement breathing and relaxation techniques at the start of a Pilates session to assist in down training hypertonic muscles.
• Start with low load and build resistance gradually.
• Work in in well controlled ranges (mid and inner) before moving into outer ranges and into open chain.
• Work for quality verses quantity (can fatigue more easily than CT typical)
• Start with gravity eliminated and supported positions before more upright unsupported positions
• Include balance training exercises- NB wobble board (easier than balance pads)
• Teach postural awareness
• Facilitate proprioception
• Consider shorter sessions of 20 minutes to begin with to minimise fatigue
• Seek feedback on response to Pilates program for the 48 hours following the session

If you are working with a hypermobile client that you are finding challenging to progress or that is experiencing pain after their session, refer them for an assessment and treatment with an EDS and HSD aware Therapist.

You’ll find a global directory here

For more information and excellent resources head to Ehlers-Danlos.com or Ehlers-Danlos.org

 

written by

Lisa Anthony

Physiotherapist, Pilates Instructor, PAA member

Lisa has participated in EDS Society’s Allied Health Professionals ECHO program and has taught several workshops on becoming a EDS and HDS aware Pilates Instructor

Website: PhysioXtra