For the majority of people with hypermobile Ehlers-Danlos Syndrome (EDS), it will take an average of 10 years to be diagnosed. We can do something about it.
EDS is a group of hereditary connective tissue disorders that which causes a change in the protein, collagen. Collagen gives structure to our connective tissue and maintains the integrity of most organ systems in our body, from our heart to our skin, to our ligaments, tendons, and muscles.
May is EDS awareness month, and awareness is essential to improve treatment for those with this condition. Low awareness and understanding of EDS means that many will be told that their pain is all in their head while others undergo unnecessary medical treatments and surgeries. Education will help, so I wanted to share some important information on this rare condition.
The most prevalent type of EDS, of which there are 13, is hypermobile EDS (hEDS). In clients with abnormal collagen, the ligaments are weak and lack stiffness and tendons are subject to tearing, compromising the stability of the joints. Treatment should be provided and exercise prescribed with this in mind.
The most commonly recognized symptom of hEDS is joint hypermobility with symptoms of musculoskeletal pain and complications of joint instability such as recurrent subluxations or dislocations. The disorder also comes with a host of associated co-morbidities, such as dysautonomia, gastrointestinal dysfunction, and mast cell activation disorder to name just a few. As physical therapists, we focus on the musculoskeletal dysfunction but need to make an effort to learn about each client’s medical history and how it plays a role in the overall care of the person. For instance, with postural orthostatic tachycardia syndrome, changes in body position may significantly impact the cardiovascular system, so the therapist may want to limit the positional changes within the session or reduce standing time.
The other important thing to consider is that symptoms and associated conditions vary widely between patients, and the severity of symptoms might be vastly different from one person to the next. Also, note that the clinical presentation changes with age as the pain from hypermobility and instability is exchanged with stiffness and joint degeneration.
The Beighton Score, shown here, is the most well known criterion for assessment, but it is only one of many factors taken into account such as skin hyperextensibility, atrophic scarring, dental crowding, velvety skin, positive family history, and many more.
If you have any suspicion that you might have a hypermobility syndrome or connective tissue disorder, find a specialist in your area. With hEDS, knowledge truly is power as treatment must be viewed holistically and you can become a better self-advocate for your care. Though there is yet to be a “cure,” physical therapy remains the hallmark of treatment. Here at Zion Physical Therapy, we have taken the time to learn about the unique differences in the provision of care for a client with hEDS. Though many clients with hypermobility have been hurt in physical therapy in the past, you can rest assured that you are in good hands. Contact us today for an appointment.
_ Meg Mizrachi, DPT