Ehlers Danlos Syndrome Awareness Month - Are you Hypermobile?
What is Ehlers-Danlos Syndromes?
The Ehlers-Danlos Syndromes, or commonly known as EDS, are a group of hereditary disorders of connective tissue that are caused by abnormalities in the structure, production and/or processing of collagen. Collagen is a protein that is responsible for healthy joints, connective tissue, cartilage, bones and skin elasticity. Worldwide, EDS appears to occur to 1 in 5,000 individuals with the most common forms affecting as many as 1 in 5,000 to 20,000 people.
EDS patients are sometimes referred to as zebras. Typically, medical students have been told that “when you hear hoofbeats behind you, don’t expect to see a zebra.” Essentially, look towards common diagnoses and not complex, more rare diagnoses. Despite this, those with chronic disease spend large amounts of time trying to understand their diagnosis.
“That’s rare, you definitely don’t have that” or “I’ve never heard of that condition before”.
The zebra became the symbol of Ehlers-Danlos Syndromes as these diagnoses can be the unexpected.
Testing for Hypermobility and EDS
Unsure if you're hypermobile, or want to test your joint flexibility? At Wellness Through Movement, our Exercise Physiologists use the Beighton Scoring System to determine your level of hypermobility.
The Beighton Scoring System looks at 5 areas of the body where hypermobility may be present, fingers, thumbs, elbows, knees and lower back. These 5 manoeuvres use a 9 point scale to determine your range of motion.
Use the image and instructions below to test your hypermobility at home:
1. Pinkie finger on left and right hand reaching 90 degrees or more (2 points if completed successfully on both sides).
2. Thumb on left and right hand, flexing back to touch the wrist (2 points if completed successfully on both sides).
3. Extension in the elbow on the left and right side, greater than 10 degrees, with 180 degrees being normal (2 points if completed successfully on both sides).
4. Extension in the knees on the left and right side, greater than 10 degrees, with 180 degrees being normal (2 points if completed successfully on both sides).
5. Forward flexion through the spine, with the ability to touch the floor with the palms of your hands, with knees being straight (1 point if completed successfully).
You are considered hypermobile if you score a total of 5 or more points out of 9.
Common Characterisations of EDS
EDS is currently classified into thirteen types, with all but the hypermobile subtype having genetic variants identified as the cause of the disorder. EDS is generally characterised by joint hypermobility, instability, dislocations, scoliosis, skin hyperextensibility (stretchier skin than normal), and other structural weaknesses such as hernias or organ prolapses. Because of these symptoms, people with EDS are prone to constant dislocations in joints leading to pain and skin that can bruise easily.
No two zebras have the same stripes. Similarly, no two people with EDS will be identical. Each person may experience different symptoms, be diagnosed with different types of EDS and have different experiences, which highlights the importance of multidisciplinary care in this patient population.
We are working towards a time in which medical practitioners can immediately recognise and diagnose EDS, which can reduce overall diagnosis time and improve care pathways and plans to assist with symptom management.
Beighton Scoring System for hypermobility
Benefits of Exercise for Those with EDS
In healthy individuals, resistance training can improve both skeletal muscle mass and functions while also increasing the stiffness of tendons. For those with EDS, specifically the classical type, it can present with reduced tendon stiffness and increased joint instability. This joint instability can lead to severe pain which can in turn lead to functional impairment. However, research has shown that heavy resistance training can be effective in classic EDS patients by improving tendon and muscle properties.
With EDS being such a complex condition with poor understanding of its underlying pathophysiology, a multidisciplinary health team is most effective in achieving outcomes. In general, the focus of exercise in those with EDS should be on symptom management and limiting the progression of disease. Research shows respiratory training can help with improving lung function. Low impact exercises are also important to help limit disease progression with examples including swimming, cycling and walking. Furthermore, focusing on strength training around joints will help reduce risk of overextension injuries such as dislocations or subluxations. It is important to be aware of fatigue levels and avoid high impact exercises while being aware of the range of motion of joints throughout the activities undertaken.