Ehlers-Danlos Syndrome: A Rare But Painful Disorder

Do you know anyone who is extremely flexible--in the physical sense?  As it turns out, a small number of these individuals actually have a connective tissue disorder.  Known as Ehlers-Danlos syndrome, this inherited disorder of connective tissue results in overly flexible joints and stretchy, fragile skin.  A more severe version of this disorder also affects the walls of the blood vessels.

How does one know this might be a problem? Firstly, the joints are much more flexible than the average person because the connective tissue that holds the joints together is very loose.  Secondly, the skin of affected individuals is very "stretchy...it can be pulled away from the body more than normal, but goes right back into place when released.  The skin is also very fragile, to the point where wounds do not heal well. Those who have the vascular form of this disorder have distinctive facial features( thin nose and upper lip, small earlobes, and prominent eyes), and the heart can be affected as well. They tend to bruise easily and often times their blood vessels are visible through the skin.

There are many possible manifestations or this disorder, such as blood vessels that can easily rupture-but in terms of chronic pain the problems are obvious. Overly flexible joints lend easily to joint dislocations and perhaps arthritis. In addition, the fragility of the skin can lead to problems with wound pain and chronic wound issues.

As mentioned earlier, the cause of Ehlers-Danlos syndrome is genetic.  Genetic counseling may help to reduce the number of afflicted. There is no known cure for the disorder.  Once diagnosed, over the counter pain relievers and avoidance measures are the best hope for maximizing comfort. Blood pressure reducing medications are used in the vascular form to reduce pressure on the vasculature.

This is a lifelong illness- so once diagnosed, it's important to become educated about it as much as possible. Support groups may be of help. It also helps for other people in your life to know and understand your situation so they can accomodate you.