Living with Hypermobility and Ehlers-Danlos

One of my dear patients was recently diagnosed with Ehlers-Danlos sydrome (EDS). The diagnosis brought a sense of relief for sweet Katie who for years struggled with numerous health problems and was often misunderstood and under cared for by the medical community. Katie was referred to me 2 years ago at 28 for pelvic pain, endometriosis and IC. Upon exam we also discovered a significant elimination disorder and paradoxical elimination. Katie regularly timed her elimination and was spending at times up to 2 hours trying to empty her bowels. As we worked together we uncovered bilateral hip dysplasia, left hip labral tear, ilioinguinal and pudendal neruralgia and POTS (Postural Orthostatic Hypotension Syndrome). Katie already had a history of anxiety and depression but managed well with good family and friend support. When the diagnosis of EDS came, she finally felt like she had an explanation for why her body is like it is. This brought great relief as well as the knowledge that her condition was genetic and her conditions needed to be managed as best as possible to give her the most function, but would likely never be fully resolved.

In her book "A Guide to Living with Ehler's Danlos Syndrome" Isobel Knight does a beautiful job outlining the various genetic subtypes of Ehlers Danlos but also highlighting the fact that EDS hypermobility type (Type III) does not just affect the connective tissue in the musculoskeletal sytem leading to joint instability and hypermoblity, muscle tears, dislocations, subluxations, hip dysplasia and flat feet. EDS can also affect the body's systemic collagen leading to increased risk for endometriosis, POTS, Renauds, bladder problems, fibromyalgia, headaches, restless legs, ashtma, consitpation, bloatedness, prolapse, IBS symptoms, anxiety, depression and learning difficulties. She notes that some people have only a few of these systemic symptoms while others may be more affected. Per Isobel: "it is important that all symptoms are treated seriously and not ridiculed and that the appropriate medical support is given to them when necessary."

It seems that EDS is becoming more widely recognized. As rehabilitation specialists we should be alert to problems stemming from joint hypermobility when we notice how our patients position themselves. Often legs are curled up or double crossed. Upon questioning we might find that the patient has a history of being "double jointed" or was able to do "party tricks" with their bodies. The Bighton scale is a test of joint hypermobility which we should all be familiar with. It is also important to note that a patient may have hypermobility without having EDS, and that EDS is usually associated with pain. A rheumatologist, or in Katie's case a geneticist, can help confirm a suspected EDS diagnosis.

If you have a patient with hypermobliiy or with EDS, know that their ability to know where their body is in space is limited as their joints have much more range of motion than normal. The proprioceptors do not fire well at mid range and the patient will have to be trained to become accustomed to neutral joint positions. This was really painful for Katie and it took a huge mental and physical effort. She is getting stronger now and it is becoming easier to achieve.  Stretching and soft tissue massage can feel really good when your muscles have to work so hard to maintain your joints in healthy positions. Patients should be instructed to not stretch into end range and also not "hang out" on their ligaments. Some patients may have to begin just with isometrics. I used Sara Meeks' program for safe and effective floor exercise with Katie. The floor gave her support while she strengthened her core muscles. Then she was able to progress to seated and seated on a ball as well as standing exercises. She loves the body blade! Yoga, Pilates, exercise in water can be effective for strength, propriception and movement reeducation. Mirrors are helpful for increasing position sense.
It is also helpful to note that even patients with EDS may be hypermobile in some joints and hypomobile in others. Isobel reports that her SI joints were extremely unstable while her thoracic spine was very rigid to the point that her lung capacity was affected. Having her therapist work on the hypomobility and doing breath work was life changing.

As pelvic health therapists and rehabilitation providers we may be the first professional to suspect EDS in a patient. There is a great deal that we and the greater medical and holistic community can do to help patients with EDS lead lives with less pain and dysfunction.