Ehlers-Danlos syndrome: Connective-tissue disorder comes in many forms
When she was 14, she was walking on the porch when her hip dislocated. That time, she was in a wheelchair for six weeks, able to leave it for only very small journeys. Two weeks after that, her other hip dislocated as she was getting up. She was placed in leg braces and a wheelchair for another six weeks.
Then her jaw dislocated, followed by her neck, her fingers, her wrists, her ankles and her knees. She was home-schooled for a while, deemed too fragile to risk public school. Every joint, it appeared, was at risk. Once, another student, jostling past in the hallway, grabbed her arm and the shoulder popped out again.
Kim Hansen was hospitalized for internal bleeding after a blood vessel ripped open when he was jogging. Another time, at work, a pipe hit him in the head and a blood vessel started leaking into his nasal cavity. Doctors had to do brain surgery, including tying off a carotid artery.
One day, playfully wrestling with his two young children, he sustained a little kick to the abdomen and was hospitalized for internal bleeding, which was expected to stop on its own. It didn't. He died from the damage to his organs. He was 36.
There are six major types of EDS, each designated by their symptoms. Chambers' hypermobility is an example of the most common form, which is also the most painful. Fully 95 percent of cases are hypermobile, or vascular, like Hansen's, or "classical," which is associated with scarring, "stretchy" skin, extensive bruising, slower wound healing and various internal-organ issues, according to Terri Keyes of the Ehlers-Danlos National Foundation. Vascular EDS is the least common of the three, but it is the one that can cause premature death.
"All types are woefully underdiagnosed," says Keyes. "Lack of diagnosis increases the health risks for people who have this, especially in surgery and pregnancy, as extra precautions need to be taken to reduce risk and increase the possibility of a successful outcome. Surgeries on undiagnosed vascular type are extremely risky," also true to some degree for all types of the syndrome.
The types do not change within families. A family with joint hypermobility EDS will not produce a child with vascular EDS, for instance. It's estimated that 1 in 5,000 people have EDS, which can afflict children or adults of any race and either gender.
Recent comments
My son is being genetically tested for EDS. When you are searching...
Mom | July 22, 2008 at 12:31 p.m.
im 20 I was diagnoised when i was 15. Doctors thought i was crazy...
sam | April 29, 2008 at 6:46 p.m.
I have EDS and am 55 years old. My knees have dislocated at times...
Debbie | Dec. 9, 2007 at 7:25 a.m.
