I worked on a post today trying to finally spit it out and just tell the truth about why I am getting sicker and how impossible our life seems right now. I can't say it.
I have been asked more and more recently about what my connective tissue disorder, Ehlers Danlos Syndrome, is and also, in some comical and sometimes insulting ways, why I don't look that sick at all but have a handicapped tag and can barely get out of bed somedays. I have read so much research on EDS. I have watched as many symposiums as I can. I have searched for a way to simply explain something so complicated to people who care.
Maxim magazine, a pretty filthy men's magazine, recently published a stupid article titled, "Four Diseases We wouldn't Mind Having." Can you imagine? I won't link to them in any way but trust me, it created rage among the EDS community to have our disease not only made fun of but so grossly misrepresented. The backlash of our "rare" zebra community was so strong they agreed to participate in Ehlers Danlos Awareness month and printed the following VERY informative follow up. So, if you have ever wondered what all the hub bub is and why I will forever be a zebra please read, and if you know someone with any of these symptoms, and particularly someone who has been suffering with fibromyalgia for years with no relief from treatment, please share. The statistics of fibro sufferers who are really zebras is fifty percent!
We quickly learned that Ehlers Danlos Syndrome isn’t fun, funny or desirable. But we still needed to find out what the condition actually is, and what makes it the invisible killer. Now we’re very scared...and informed.
Eugene Wilson of the Center For the Ehlers Danlos Syndrome Alliance was kind enough to walk us through the condition and help show us just what complete idiots we were. And sadly, we’re not the only ones in the dark about EDS.
What Is EDS?
EUGENE WILSON: It’s complicated. Put simply, EDS is a collagen deficiency, which is the glue of the body. An EDS patient has difficulty making the collagen. The loose-connective tissue is faulty. But EDS is not just muscular and skeletal. It’s not just in the joints. It’s not just the muscles being hyper-elastic. You can have vision problems. It leads to heart problems. It leads to brain problems. You have some kids that can’t walk. They can’t even move.
How Bad Can it Get?
There are six main types, and people who suffer from the first three types may only have hyper-flexibility and loose skin. But type IV get worse. Type IV is Vascular EDS. People with Type IV know they’re walking a thin line each and every day. They never know if they’re going to wake up the next morning. Kids who are 13, 14, 15 years old are dying from this. Those with Type IV have a life expectancy of 48.
Why Isn’t This Well Known?
There’s a lot of bad information out there. Even some people in the medical field fail to recognize EDS. There’s misinformation and miscommunication. I can take a child with EDS to four different geneticists and all four of them will come up with different answers. Testing for EDS is currently only 30-40% accurate. The medical community needs to step up and realize what’s happening. Right now, it’s been reported that 1 in 1,500 people have EDS. I think that number is closer to 1 in 800. That’s why we need to raise awareness. People need to know.
What Are the Symptoms?
You could start with being double-jointed. If you look at someone and they have a crooked nose and they’ve never been in a fight, that’s a deviated septum, and that’s one sign of EDS. You see someone standing by a counter and their ankles are rolled onto the outside face? That’s another sign they have EDS. If you watch a person write, and their fingers bend backwards, not so much the thumb, but the fingers, that’s another sign. If you’re having unusual pains, but getting a lot of false-negative x-rays and MRIs, you could have EDS. There’s a lot of little clues, some of them almost invisible.
Maxim Is Better Than House (at least)
People heard the voicemail and asked how I kept my composure. But getting mad at you guys would have done more harm than good. At least you called back. There was an episode of House that focused on EDS. It was horrible. It was about a girl who had several miscarriages and some pain and then she became a hoarder. And then House walked in and diagnosed her as EDS. Hoarding doesn’t have anything to do with EDS.
Is This Piano Video Sending the Wrong Message? (A guy who plays the piano backwards because his arms are so hypermobile.)
For me? Any kind of awareness is great. I don’t want people to see that video and say, “Hey, if that person can do that, it’s perfectly fine for me to do the same thing.” If you do that, you’re tearing up your joints faster than you needed to.
There’s a lot of bad, bad information out there. Some don’t like what Stan Lee has done on his show Stan Lee’s Superhumans. There’s a man with EDS on the show who has elastic skin. What people don’t understand is that that man is fortunate enough to not be suffering in the severe pain that other EDS patients suffer.
EDS affects every person differently. I can show you 15 people in the same room, you look at every single one of them, and none of them look sick, but each one has a different severity of pain, and that pain has affected their life in a different way.
Any Hope for a Cure?
There’s a lot of research starting to come through. Most of it has been research in the Vascular EDS, and I can understand that. They’re trying to stop loss of life. You’re looking at nanomedicine. You basically take a pill and little micro-robotic things will fix your body. How does that work? I’m not sure. But there’s the hope. In Texas there’s a research project about new medication for Vascular EDS. Unfortunately our government takes their sweet ol’ time to get any medication on the market. In Europe there’s already some medication available. There is hope. Until we get there, it’ll take much more knowledge in the medical field, and we need to know how many people have EDS and get the right diagnosis on these people.
Why Are Doctors in the Dark?
A lot of it goes back to the med schools. I’ve spoken to doctors who said that in med school, they only had six days or a few weeks of studying all the connective tissue conditions...not just EDS. All of them. If the doctor never sees it again after they come out of school, and then a patient comes to the doctor 15 years later, the odds that the doctor will remember everything about that condition are slim to none.
My son’s doctor had him on the exam table bent up like a pretzel, but never diagnosed him. Finally it took a muscular neurologist to diagnose my son. We need more connective tissue labs and clinics all around the United States. We’d like magazines and media like Maxim to help promote awareness.
Why The Zebra Mascot?
“Zebra” is a medical term for rare or difficult to diagnose diseases. There’s the old saying, “When you hear hoofbeats behind you, you don’t expect to see a zebra.” But we’re flipping it. If you hear hoofbeats, think of Zebras.
For more information on the condition and to find out how you can help, check out The Center for Ehlers Danlos Syndrome Alliance. Be sure to check out the proposed legislation that would help make more people aware of the condition.
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