Guide by Condition: Ehlers-Danlos Syndromes

Posted by Mike Phipps on December 10, 2018

Understanding and living with Ehlers-Danlos Syndromes

EDS is a group of genetic conditions that can be inherited. Although there is currently no cure, however, treatments, therapy and mobility equipment can help people with EDS to live independent and fulfilling lives.  

What are Ehlers-Danlos syndromes (EDS)?

Ehlers-Danlos syndromes, also abbreviated as EDS, are a group of rare inherited conditions which affect the connective tissue in the body. This tissue helps to provide support in the skin, tendons, ligaments, blood vessels, internal organs and bones, so when it is affected by EDS, it can create symptoms such as joint hypermobility, stretchy skin and fragile skin which breaks or bruises easily.

There are four main types of EDS, including:

• Hypermobile EDS (hEDS) - The most common type of EDS, hEDS can cause symptoms such as joint hypermobility, joints which click and dislocate painfully, digestive problems and dizziness after standing up.

• Classical EDS (cEDS) - Less common than hEDS and more likely to affect the skin, cEDS can cause stretchy skin which is often smooth and velvety and bruises more easily, along with wounds that are slow to heal.

• Vascular EDS (vEDS) - A rare and serious type of EDS, vEDS can cause the blood vessels to split open and cause life-threatening internal bleeding. vEDS can additionally cause organ problems, such as possible tearing of the bowel.

• Kyphoscoliotic EDS (kEDS) - This rare type of EDS can cause a curvature of the spine, loose and easily dislocated joints, soft skin which bruises easily and fragile eyes that have a higher risk of being damaged.

What causes EDS?

EDS is caused by faulty genes, which may have been inherited by one or both parents, depending on the type of EDS. The genes make the connective tissue weaker and cause a variety of symptoms which can affect various parts of the body, such as the skin, bones, tendons, ligaments, blood vessels, internal organs and bones. In some cases, the faulty gene isn’t inherited and instead occurs in the individual for the first time.

Living with EDS

EDS can affect people in different ways, due to the different varieties and symptoms. Due to this, there is no specific treatment for EDS but support and advice can help to manage many of the symptoms. Depending on what type of EDS the individual is living with, they may be advised not to take part in activities like heavy lifting and contact sports, or be required to wear supports to protect the joints. Low-energy activities like swimming and pilates can be suggested to stay healthy in an easier way.

What is the treatment for EDS?

There is currently no specific cure for EDS; however, individuals with the condition may benefit from seeing different healthcare professionals. For example, a physiotherapist can provide exercises which can strengthen the joints and avoid pain, whilst an occupational therapist can offer their advice on any helpful mobility equipment. It’s even possible to visit a gene therapist, who can discuss the cause of the condition and what risk it has of passing down to the next generation.

Helpful products

Depending on the severity and type of EDS, a mobility aid can make a difference in everyday life. If the symptoms are fairly mild, something like a crutch or walking stick can help to provide support and stability, whilst a wheelchair or scooter might be more suitable if the EDS is more severe.

Useful links

• Ehlers-Danlos Support UK - https://www.ehlers-danlos.org
• The Ehlers-Danlos Society - https://www.ehlers-danlos.com