It’s been a month since my last pandemic update. If there’s anything to say, it’s that not much has changed, which means things are going well. I’d like to take a moment a few things about vascular Ehlers-Danlos Syndrome Awareness month. It has been almost four years since I was in the throes of a series of life-threatening emergencies and a genetic test revealed that I was born with with vEDS. My memory of the day I got my diagnosis is vivid. We’d been waiting a couple of months for results from a genetic test when we found out that the findings were sitting in a file twenty miles away at Tampa General Hospital. They’d been to sent to the doctor who happened to be nearest when they took blood for the test.
It was a Friday and I was scheduled to have surgery on an aneurysm the following Monday, so the contents of that folder were pretty important. They wouldn’t fax them, so Monica had to brave Tampa traffic to get the papers. She must love me.
When she got there the person in the records office informed her that I needed to sign a release form (a requirement they neglected to mention over the phone). Dismayed, Monica explained that I was twenty miles away, still recovering from open-heart surgery. The guy was sympathetic, but “rules are rules.” Then he said, “Are you sure your husband isn’t outside waiting in the car?”
Monica was incredulous. She had just explained rather clearly that I was in St. Pete. But he persisted. “Are you sure your husband isn’t in the lobby where he can sign a medical release form?”
Oh, she thought, I get it.
Monica took the form around the corner where the mythical me signed the form. I’m not exaggerating when I say that this man’s willingness to break a rule saved my life.
The diagnosis was unambiguous: vascular Ehlers-Danlos (formerly known as EDS Type IV). We finally had a name for the thing that had ravaged my vascular system. I’d never heard of vEDS. At first we were excited because the mystery was solved. Surely we’d soon find answers. The excitement soon faded and unfortunately vEDS is not a disease you want to learn about via Google. That weekend, waiting for surgery, was the worst. The diagnosis was confusing and the only silver lining was that we could say, “Now we know.”
Knowing was extremely important.
The following week, my aneurysm surgery failed when my femoral artery ruptured and they had to abort the repair to my right iliac artery. But knowing my diagnosis influenced my surgeons to stop the procedure and let me fight another day. It was several months before I’d speak to a doctor who had other patients with vEDS. But by then I’d read journal articles and other information beyond the Wikipedia page, and prepared myself for that conversation.
After my failed surgery, I was transferred to another hospital by zany pair of EMTs. Monica pored over everything she could find about vEDS. She started calling hospitals and friends who had friends who knew people. By the time my local surgeon walked in the room, he’d gotten a text from a surgeon in Cleveland asking about the vEDS patient in Tampa.
It’s sort of strange to be an adult and learn that you were born with a deadly disease. It was a lot to take and I was also keenly aware that Monica had to process the news from a different point of view. Because I was in the heat of major complications, there wasn’t much time to think about the big picture, but eventually I started to replay my life and little things like bruising and other bodily quirks started to make sense. My mother had passed in the late 1970s when there was almost no way to know she had vEDS. Awareness was nonexistent.
VEDS awareness isn’t just about getting sympathy from the world for our disease or for raising important funding for medical research.
ER Doctors (and perhaps more importantly, front line triage nurses) need to be aware that pain symptoms in vEDS patients are often NOT caused by the most common explanation. It’s probably not “just gas” or appendicitis or a muscle spasm. A vEDS patient experiencing pain doesn’t have time for a waiting room chair. And primary care doctors need to intervene when patients have any level of elevated blood pressure.
On a day-to-day basis, greater understanding of the disease would make the lives of people with vEDS so much easier to manage when it comes to employers and insurance companies. Under even moderate physical strain and mental stress, people with vEDS are at high risk for serious complications. Minor things can quickly become serious.
The term “invisible disability” is often used to describe people with conditions that aren’t evident to the casual observer. I’ve never actually had someone tell me, “You don’t look sick,” but I do know that people with vEDS often struggle through life because their condition is under-recognized or underestimated. Frankly, I feel lucky that my disease is not apparent. It does present awkward moments when people don’t know my physical limitations. But I’ll take an awkward moment with an employee at Home Depot over a visit to the emergency room.
I saw post on social media the other day from a young guy with vEDS lamenting the fact that his wife had to assume lawn mowing duties and I could sympathize. I can’t count how many times my neighbors have watched Monica unload something heavy from our car while I stood and watched, or we’ve had to call over a friend to help move a piece of furniture that I’m able, but forbidden, to move. They’re the sort of frustrations that can turn to anger and self-pity if I lose sight of gratitude.
But gratitude is the prevailing emotion that I’ve felt since my life changed with vEDS. Not once have I woken up in the morning dreading the day because of vEDS. In fact, I’m usually at my happiest when I first wake up to face another day. The paradox is that although I’ve always been a happy person by nature, I’ve been happiest since my diagnosis. Occasionally I have to remind myself of the promise I made to myself after surviving my 6th surgery, to be grateful for every day – don’t sweat the small stuff, and appreciate the little things. The little things are everything.
“Don’t make me turn this van around” is written by Jonathan Kile, and approved by Monica Kile. Jon’s 2014 thriller, The Grandfather Clock, is available free for Kindle on Amazon.com and other eReaders at Smashwords. Reach him at email@example.com. Monica is a leader of a local non-profit, marathoner, baker, tour guide, and prolific bath taker.