Understanding Ehlers-Danlos Syndrome: Types, Symptoms, and Management

What is EDS

Ehlers-Danlos Syndrome is a group of conditions that affect the connective tissue of the human body by loosening or weakening it. Connective tissue supports the skin, tendons, ligaments, bones, blood vessels, and organs. The condition is generally inherited and is commonly linked with hypermobility, which means joints can move past their normal range of motion. There are 13 types of EDS in total, some of which are very rare. The condition is caused by genetic changes that affect how the body makes and uses collagen, a protein that gives structure and strength to tissues. In some people, it can also happen randomly due to genetic mutations without any family history.

Types and Symptoms

Hypermobile EDS, or hEDS, is the most common type and brings a higher risk of bruising and injury. Symptoms of hEDS include stretchy and fragile skin, loose and clicky joints that dislocate easily, joint pain, extreme fatigue, digestive issues, and poor bladder control. Classical EDS is another type that mostly affects the skin, causing it to stretch easily, split around the elbows, forehead, and knees, and leave obvious scars with slow-healing wounds. Vascular EDS is a rarer and life-threatening form that affects internal organs, resulting in fragile blood vessels that can cause internal bleeding, bowel tearing, womb tearing during pregnancy, and lung collapse. Another rare form is kyphoscoliotic EDS, which affects the spine's curvature and can appear during early childhood alongside joint hypermobility.

Diagnosis and Management

Some of the rarer forms of EDS can be diagnosed through genetic testing. While there is no cure for the condition and symptoms can sometimes be disabling, they can be managed. Physiotherapists can recommend muscle and joint strengthening exercises that offer short- and long-term benefits. Doctors may also suggest pain management and treatment for bowel issues. Depending on how severe the condition is, individuals may need to avoid activities like weight-lifting or contact sports that put pressure on joints, while low-risk activities like swimming and pilates can help keep them fit.