What’s Ehlers Danlos Syndrome?
I could easily spout off the whitewashed definition of “Ehlers-Danlos syndrome is a group of disorders that affect connective tissues supporting the skin, bones, blood vessels, and many other organs and tissues. Defects in connective tissues cause the signs and symptoms of these conditions, which range from mildly loose joints to life-threatening complications.”
But what does that really tell you? Not much. The scope is so wide, there is no way that definition could possibly enlighten the reader of how EDS truly plays out in real life. Unfortunately the number of people who have it and the number of ways it can play out are equal. No two EDSers would be the same. It all boils down to collagen that is structurally different because of a coding error in their genes. Depending on which type of collagen is affected, is what determines where most of the issues lie.
The House That EDS Built
Could you tell if a house was structurally sound or not with a drive by glance? Of course not. It would have to be thoroughly inspected. Is there rebar in the concrete foundation? Are there the right number of 2×4 studs in the walls? What about the right number of floor joists for the size of the house? Are there enough trusses to support the roof? Even if you went into a house, you wouldn’t be able to determine whether all these requirements were met. Either you’d have to start ripping drywall off, or the lack of support would reveal itself over time as the house prematurely fell apart. I distinctly remember watching the movie Money Pit with my Mom as a kid. Tom Hanks and Shelley Long’s characters buy a huge, beautiful house together “for a steal of a deal!” As they begin to renovate the problems with the house snowball out of control, one structural issue always leading to several more. That’s EDS in a nutshell.
Pool Noodle Coding
People with EDS (and please always add the “s”. So many people just call it “ED”… I don’t even have the right body parts for that for Pete’s sake! I also don’t have an Eating Disorder. Digestive disorders yes, eating disorder, no.) Back to our scheduled programming…
People with EDS do in fact have the right number of 2×4’s if we continue with the example of a house. But every couple of 2×4’s, there’s a pool noodle instead. It’s a funny analogy, until not so hilarious issues creep up. The pool noodle won’t help hold up the wall, putting extra strain on the real 2×4’s. Instead of a house lasting the normal amount of time, major problems start happening after only a few years. A regular rainstorm that wouldn’t impact a well built house, could be catastrophic to the pool noodle coded one.
Apparently my pool noodle coding made itself known (but went unrecognized) very early. My parents said when I was little, I was always telling them how much my legs hurt. I honestly don’t remember doing this. I do however remember going to different types of doctors frequently, wearing knee braces, and doing physiotherapy for years on end. The physio didn’t help even a little. My knees would give out for no reason, resulting in the decision to have major knee surgery in hopes to stabilize the joints.
Well that didn’t work!
The results weren’t anywhere near what the doctor was hoping for. I had the surgery in mid-August the summer I turned 16, with the goal of being well enough to crutch my way around school in September (with a full leg cast!)
The surgeon was beyond unimpressed. He was so disappointed in the result he refused to operate on the other knee. He said some bizarre things to me during my recovery:
“I’ve never seen anyone atrophy as fast as you, not even a decrepit old person!”
(Tact and bedside manner were non-existent with this man)
“You’re going to be in a wheelchair by the time your 30.”
(I could not understand why we even did this surgery then!)
And my personal favorite,
“Don’t worry, you’re going to need more surgeries.”
(Well I WASN’T worried, but now…)
All these things pointed to Ehlers Danlos Syndrome, but were never looked into and no connection was made because doctors are taught it’s incredibly rare. It’s not that rare, it’s just rarely recognized and diagnosed. Just to give you an idea, The Ehlers Danlos Society states that it’s about 1 in 2,500 to 1 in 5,000. The ALS Association states that the estimated number of people living with ALS is 5-7 in 100,000. Huntington’s is approximately the same. Everyone has heard of those devastating diseases which statistically are more rare, but no one has heard of EDS. Worse yet, no one really knows how to treat it.
Fulfilling that surgeon’s prophecy of having more surgeries, at 38 I had a procedure to remove a joint from a toe that literally didn’t work anymore. Walking was excruciating. But I would probably choose that pain over what would happen next if I had only known what was really going to transpire.
I had an allergic reaction to the antibiotics that were prescribed as a precaution before the surgery. Little did I know that taking the antibiotics was going to upregulate the effects of EDS in my body. For the next few years, everything became an unsolvable mystery. I couldn’t stay awake, yet I never felt rested. I had trouble answering the simplest questions from my kids because I couldn’t think or comphend from severe brain fog. Eating evoked such pain I resorted to drinking liquids only, which was only slightly better. Every joint hurt, every inch of me became hypersensitive. It felt like my whole body was inflamed. Even wearing clothes was agony.
Finally defining the Enemy
Any and all tests done on me always came back normal. In fact, my doctor told me there were people who would be ecstatic to have blood work as good as mine. Out of complete frustration, my MD sent me to a rheumatologist. I waited 9 months because nothing about my blood suggested I had anything that needed immediate attention. After going through my medical history, she was shaking her head in disbelief that no one had talked to me before about having a genetic connective tissue disorder. The “enemy” had a name.
I went back to my MD. He just silently stared at me for a few minutes. When he broke his silence, he admitted he had no idea what to do. He asked for a few more details of what the appointment with the rheumatologist had been like. Then I hesitatingly said, “I don’t know how to say this, but after she diagnosed me…..well, she got excited. That’s the only way I can describe it. It was weird.” He snorted and said, “well YEAH!” I blinked in disbelief that excitement would be an obvious reaction to an EDS diagnosis. Then he explained.
“Ehlers Danlos was mentioned one time in medical school. And the next sentence after that was, ‘don’t worry about it, you’ll never see it.’”
Our doctors are told to ignore a disorder that is as prevalent as 1 in 2,500 to 1 in 5,000? What garbage. Surely over the course of a medical career, a doctor ends up seeing more than 2,500 people.
So in truth, I had two “enemies”. Ehlers Danlos and the ignorance surrounding it.
Why am I doing this?
It’s been very apparent to me since diagnosis, there are two camps with people who have EDS. One camp is “it’s genetic and there is nothing that can be done.” I understand this camp. I really do. I’ve lived there. The other is “it’s genetic, but let’s see what can be done.” I moved over to this camp when I found some EDSers that had completely changed their lives with different protocols and therapies. EDS isn’t cured, but for some it’s so well managed that they have their lives back. I’m pretty tenacious, so I’m getting my life back. I’m sharing with others what I’ve learned. Not just for EDSers. It’s for anyone who has lost a part of themselves because of health issues. When I dive into research, it’s shocking how one awry mechanism in the body can throw ten more into chaos. I’m here to share all my wellness strategies to help any other tenacious person looking for answers. In short, I’m on the hunt for healthy me!
