Ehlers-Danlos Syndrome, a horrible disease

Ehlers-Danlos Syndrome (EDS) is a rare genetic disorder that causes joints to be hyper-flexible.

This is the horrible disease that is affecting Olivia Court, a three years old British kid that had not been able to run, jump, or even walk, until she was provided with a “second skin” suit that enabled her to run and play like other kids her age. Her joints were so flexible that her hips and knees would dislocate when she tried to walk. But with the help of the revolutionary custom-made lycra suit her spine and joints are supported so that they are protected from dislocation. Her doctors hope the suit will allow her muscles to grow strong enough that they will be able to support her joints on their own. EDS affects the body’s ability to build collagen which is essential for building ligaments, arteries, and other body structures. Collagen holds cells together, and the lack of it is causing joist to be extremely mobile and over stretchy, the skin to be very thin, the ligaments to break very easily and might be the cause for ruptures in internal organs and blood vessels.

Lynn Sanders, now aged 52, is the founder of the Ehlers-Danlos Syndrome network C.A.R.E.S from Wisconsin and she suffers from the same form of EDS that Olivia does. She started suffering from EDS when she was a teenager and had joint pain and easy sprains. The doctor kept telling her that she was fine, because the X rays did not show any trouble with her bones. But they could not see the ligaments on the X rays and she suffered from severe joint pain. She started having surgery when she was 18, and she was going to have about 40 surgeries meant to fix her damaged ligaments. The treatment for Sanders’ type of EDS consisted at time on braces, physical therapy to strengthen the muscles around the joints and painkillers. Now the surgeries using ligament grafts became more common, but they are a temporary solution and their effect lasts about five years, because there is no permanent cure for this type of disease. There are six different forms of EDS, manifesting either by stretchy, but fragile skin or by joints bending in both directions. Dr. Gary Gottesman, a medical geneticist at St. Louis University School of Medicine, says that one of her patients even learned how to pop his joints back into place because they used to dislocate several times a day.  The most severe form of EDS is vascular EDS, which causes blood vessels and internal organs to rupture suddenly and patients affected by this form usually do not live more than 40 years. Dr. James Black, an associate professor of surgery at Johns Hopkins, says that he had done surgeries before the rupture actually occurs and implanted grafts, which have proved to be more successful than the classical methods. Lynn Sanders started the Ehlers-Danlos Syndrom Network when there was little knowledge regarding this disease, although it affects one in every 10,000 people. Many patients used to go undiagnosed for decades and others, experiencing hyper mobility, did not know what was behind their pain.