ABOUT EHLERS-DANLOS SYNDROME

Ehlers-Danlos Syndrome (EDS) refers to a group of thirteen (13) heritable genetic disorders that impact the body’s connective tissues. As healthy connective tissue provides structure and support to skin, bones, blood vessels, and virtually all organs and tissues, defects in these connective tissues can lead to problems in multiple systems in the body. Depending on the type of EDS, symptoms can range from mild to severely disabling/life-threatening and can vary greatly between individuals. Symptoms may also vary across an individual’s lifetime, for example, emerging at puberty, or flaring at times of stress or trauma. For these reasons, along with the general lack of awareness of the disorder, EDS often takes many years to accurately diagnose (i.e. over 40 years, in my case) and is believed to be under-diagnosed by the medical community.

Symptoms vary by EDS type, but more commonly include:

Joint Hypermobility – People with EDS often have joints with an excessively large range of motion, which can lead to instability, injuries (subluxation, dislocation, broken bones, ligament tears, etc.) and chronic pain.
Skin Hyperextensibility – People with EDS often have skin that is different. It is often softly textured and more elastic and/or fragile than “normal” skin, leading to excessive bruising, slow-healing wounds, and abnormal scarring.
Tissue Fragility – Other tissues in the body, such as organs and the structures that support them, may also be more fragile or function differently, causing potential issues with the vascular system, abdominal organs, eyes, gums, teeth, and bones.
Chronic Pain and/or Fatigue – Most people with EDS experience varying degrees of chronic pain and fatigue, which can cause significant disruption and impairment.

But wait, there’s more!

There are SO MANY additional symptoms that can be present in EDS – gastrointestinal, vascular, skeletal, neurological, reproductive, mental/emotional – basically anything mediated by connective tissues can be impacted. There are also many “comorbidities” that are often diagnosed alongside EDS, such as Postural Orthostatic Tachycardia Syndrome (POTS), Mast Cell Activation Syndrome (MCAS), immune mediated conditions, and other under-researched conditions, such as Endometriosis.

“If you can’t connect the issues, think connective tissues.”

How is EDS managed?

Good question, we’re all working on that. There is currently no disease-specific cure or treatment for any type of EDS. The best course of action is managing the symptoms that are the most impactful for the individual, which may include a number of components. Some approaches include following:

Exercise/Physical Therapy – Exercise, especially a routine recommended by a knowledgeable doctor or physiotherapist, can help with pain, fatigue, and other symptoms. Exercises can also help address muscle imbalances and joint instability issues for some people with EDS. It is important to find practitioners who know how EDS can affect the body, as some forms of exercise may cause more harm than good if done incorrectly.
Pain Management – Ranging from over-the-counter pain medications (e.g. Tylenol, NSAIDs) to prescription opioids, most people affected by EDS require some type of pain management over their lifetime. Medical cannabis, CBD, and low dose naltrexone are also sometimes used for pain management. Heating pads, ice packs, topical creams, and compression devices (“arthritis” gloves, etc.) may be helpful for localized pain or injuries. There are many other complementary alternative therapies available for pain, and as always, it is best to get clearance from your medical team prior to starting anything new.
Supportive & adaptive devices – Some people with EDS require medical devices to provide additional support for overall mobility or affected body parts. These can vary significantly by individual and may not be needed all the time. Finger ring-splints, wrist braces, KT tape, compression wear, canes, and wheelchairs work for some people with EDS.
Prioritizing rest & Pacing your activity – Fatigue is one of the most debilitating symptoms of EDS. Taking breaks and splitting bigger tasks into smaller pieces can all help lower the impact these activities can have on the body. Rest is incredibly important, but can often be impaired due to pain or nighttime injuries (I wake up with injuries frequently.) Obstructive Sleep Apnea can occur when the tissues supporting airways are affected by EDS, so it may be helpful to rule that out if chronic sleep issues are present.
A Good Medical Team – Having a team of medical professionals to address specific symptoms is incredibly important and often difficult to find. It is also very beneficial for these medical professionals to communicate with each other regularly, as specialists can sometimes operate in medical “silos.” Finding medical professionals who have anything beyond a basic knowledge of EDS can be incredibly difficult as well, but having a doctor who will listen and be willing to learn is the next best thing.

Please see the Ehlers-Danlos Society website for additional information on diagnostic criteria, management suggestions, and so much more.

As always, please read my Disclaimer. This is not medical advice. Please see a qualified medical professional for advice regarding your own health prior to initiating any potential courses of treatment.