Wednesday, May 16, 2012

Ehlers-Danlos Syndrome - Living with a Thief

For the most part, I try to keep this blog about pubic history, grad school, living in Canada, stuff like that.  But seeing how May is Ehlers-Danlos Syndrome (EDS) awareness month, I thought I would share something a little more personal.  


I've been thinking a lot recently about what it's like living with EDS, and even though Doug is the one with the genetic disorder, as his wife (and by extension, sometimes caregiver) I too live with EDS.

For those of you that aren't familiar with EDS - and most people aren't - it is a genetic connective tissue disorder.  There are four types of EDS, with type IV (vascular) being the most rare and severe.

Doug has type IV.   

People with type IV have blood vessels that are fragile and prone to tearing which often leads to aneurisms, strokes, and organ failure.  There is no cure and the only treatment is a healthy lifestyle, daily vitamins, and trying to avoid "risky situations." (By risky situations I mean contact sports, roller coasters and sky diving.)

Living with EDS is a bit like living with a thief.

In the beginning, before you have a diagnosis - when you're just the guy who always has shoulders that dislocate easily and seem to be prone to headaches - little things get stolen from you.  At first when these things are small - such as when shoulder surgery at the age of 15 ends your days on the school baseball team and you start to umpire instead - you suspect that perhaps you are just misplacing them and don't think anything of it.  You adapt, go on without them.

But then the thief takes something big.  You're sitting in class and one side of your body goes numb and your eyesight gets blurry.  It's terrifying.  A trip to the hospital and several tests later - after your symptoms start to subside - you're sent home with the diagnosis of a "complex-migraine."  It's akin to your house being broken into, calling the police, but the detective says there are no clues, so he can't catch the guy.  You just kind of live in fear.

Maybe for awhile things are fine.  You've been living with the joint problems, so you've adapted, you can relocate a shoulder like no one's business.  You even chalk the other episode up to college stress.  Things are fine now.

Then one day the thief strikes again, big time now.  Pretty much clears out your house.  Thank goodness this time you're fortunate to have a detective with-it enough to find the very tiny clues left behind.

There's a pain in your leg so severe, at the age of 27 you seriously consider a cane to get from your car to the classroom.  At the two week follow-up appointment for your "pinched nerve" the physicians assistant can't find a strong pulse in your lower leg, so you get sent for a doppler ultrasound.  During the ultrasound the nurses go quiet, and within two hours you are getting a CT scan and being prepped for surgery.  Something is seriously wrong.  But then the cardiovascular surgeon looks at your scans and notices an anomaly.  Rather than operate - which could be life-threatening for someone with vascular EDS - he waits and refers you to the Mayo Clinic.  Thank God this surgeon thought to look for zebras. (The symbol for EDS is the zebra, because as the saying goes, "when you hear hoof-beats you never think to look for a zebra." EDS often goes un- or mis-diagnosed.)

Several trip to the Mayo Clinic and a whole team of fancy doctors later, and your geneticist confirms the diagnosis of vascular ehlers-danlos syndrome.  Your given all the information they have on the disorder (which isn't much, you found all that info researching EDS on the internet), some genetic counseling, and told that you should come back once a year to have your cardiovascular system monitored.  The geneticist is great, but there really isn't anything else he can tell you to do.  You're body will heal itself as best it can (as it did with the stroke that you had three years earlier, that went undiagnosed) and you can be as active as your pain levels will allow you.

So now you know the thief is in the house, but you have educated yourself so you think you can live with it.

Then something else goes missing and you just get really angry.  How do you cope with a disorder that you can't even fight?!  They say the best offense is a good defense.  But how much safer do you really feel with a thief in your house when all you can do about it is constantly take an inventory of your stuff and wait for the next item to go missing?

Will it be something small?  Like recognizing that you can't play soccer or hockey anymore, because even if you keep the contact to a minimum your body aches for days afterwards?  Or will it be something big?  Like getting a call from your husband in the middle of the afternoon because he can't see?  And just when you think you've come to terms with EDS, something else happens and throws you for a loop.

So you sit and wait.  Some days are good.  You enjoy all the things that you can do - ride your bike, play golf, and generally enjoy a high quality of life.  Some days are not so good.  Like when you try to explain to people that even though you look like a perfectly healthy 29 year old, you actually live your life in almost constant pain.

EDS is a thief in your house, and even when everything seems fine and you can't see him, you know in the back of your mind eventually something else will be gone.


To learn more about EDS, visit the Ehlers-Danlos National Foundation website.

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