If you’re a fairly involved member of the “spoonie” community, you probably know the term “zebra” and what it means in relation to health. But if you’re not, you’re likely wondering: what in the world is a “zebra” and what does it have to do with health?
A “zebra” is a nickname for a patient with rare or unusual medical diagnoses but has specifically been tagged to Ehlers Danlos Syndrome, a disorder of the body’s connective tissues.
Medical students have long been taught the phrase, “When you hear hoofbeats behind you, don’t expect to see a zebra.” This means that, when faced with a seemingly difficult case, doctors should NOT consider a rare disorder but rather investigate based on what’s most common. Hence, the zebra came to life.
When Ehlers Danlos Syndrome is the answer, we like to say “Sometimes when you hear hoofbeats, it really is a zebra!”
So, What is EDS?
Ehlers Danlos Syndrome, or EDS for short, is a bit of a daunting diagnosis for some. It often takes several years to properly diagnose and can range from mild to severe in terms of disability. There are 13 subtypes of EDS and most forms are genetic, or inherited.
EDS is considered a rare disease as fewer than 200,000 Americans have it. As a group of connective tissue disorders, this means that the condition can impact the entire body in a variety of ways. In most cases, almost all EDS patients have issues with their joints, particularly hypermobility and chronic pain, as well as loose, soft, and easily bruised skin, difficulty healing from injuries, and even problems internally with organ linings, bones, blood vessels, and veins.
Connective tissues are all literally all over the body. When there is a flaw in the connective tissue, namely the collagen, that’s when the trouble comes knocking. Collagen is like the glue of the body, a protein that holds everything together and keeps it supple. Damaged collagen means loose skin, easy bruising, easy injury such as cuts, early signs of aging, joint slipping, and much more.
EDS and Migraine
As is the case with many other health issues, patients with migraine are at a higher risk of developing EDS and vice versa—they are considered comorbid illnesses.
Dr. Vincent Martin of the University of Cincinnati College of Medicine had observed in a study that many of his joint hypermobility patients also suffered from migraine, and he was interested in finding out more about that correlation. Martin believed both joint hypermobility disorders and migraine share roots of being related to collagen.
Dr. Martin hypothesized if collagen is too elastic, it leads to overly flexible joints and stretchy blood vessels. We know today that migraine is not entirely a vascular condition but rather a neurological one, but his theory does have some truth to it because expanding and contracting blood vessels are part of the migraine process. Thus, this leaves those us of with elastic collagen more susceptible to having migraine.
Navigating EDS: Our Stories
Ehlers Danlos Syndrome plays a pretty hefty role for me in regards to my chronic migraine. Several of my major migraine triggers directly correlate to EDS.
For starters, I deal with something called cervical instability. To put it simply, my cervical vertebrae frequently become misaligned resulting in neck pain, and, you guessed it, migraine. Due to my joint instability and hypermobility (thanks EDS), my neck is rarely properly aligned.
Some days are better than others, as how it usually goes with chronic illness, but the constant misalignment of my neck results in a headache that almost always escalates into a migraine.
In addition to my cervical instability, I also deal with TMJD (temporomandibular joint dysfunction). Just like my vertebrae, my jaw joints have a nasty habit of coming unhinged and popping in and out. Almost on cue, as soon as I feel my jaw click out, a migraine comes immediately. There is really nothing I can do in these situations and that is the worst. Thankfully, I have been working on my TMJD issues with a specialist who knows a lot about EDS. The jaw orthotics he made me have really made a difference in my TMJ related migraines.
Due to my joint instability and daily subluxations and dislocations, I am in quite a bit of pain daily. For example, the pain from feeling my elbow snap and dislocate can make me feel so terrible that it ends up giving me a headache. The same thing goes with crying; the pain from my joints being misaligned often results in me in tears. And as many headache and migraine sufferers know, crying only makes the headache or migraine worse.
From my very first symptoms at 9, which we did not know was related, it took until I was 29 years old just for that comorbid digestive disorder to be diagnosed, called colonic dysmotility. The colon had also stretched because of the weakness of the connective tissue and then I had an organ prolapse.
Finally, 10 years after that, after being treated and diagnosed with several more comorbid, systemic, and painful disorders, I was diagnosed by a neurologist and then a specialist with Ehlers Danlos Syndrome. In total it was 30 years for diagnosis and I am still seeking my best treatment plan, which may mean going all the way to Those Island. YES, awareness is needed!
For a long time, since my at least my teen years, I have had joint pain, subluxations of certain joints, hypermobility, easy bruising, and difficulty healing properly.
When I was in 4th grade, I had a playground accident that resulted in a huge gash in my leg under my knee. The cut went straight to the bone and I received 13 large blanket stitches to mend it. The wound never healed properly and constantly opened back up. I now have a gnarly striated scar from the injury, in EDS terms: a “cigarette paper scar” This incident was the first indication of EDS for me as a child, and also played a key role in my diagnosis as an adult.
Then again in my teen years, I had another accident, this time on a scooter. I crashed and landed on asphalt, smashing that same injured knee, and it dislocated immediately. Through extreme pain, I pushed my knee cap back in place, but it has never been the same and now as an adult, this knee subluxes and dislocates quite a bit, giving me trouble anytime the weather turns bad or anytime I walk too much.
Most of these symptoms and issues I simply attributed to my rheumatoid arthritis or fibromyalgia, thinking it was inflammation in my joints, muscles, or nerves. I never had an inkling of a thought that I might have EDS, until I read about it from other spoonie friends and realized how well it mirrored what I experienced.
After about a one year ordeal, going through all the tests, all the grueling appointments, and a few asinine doctors in between, my diagnosis was finally confirmed. So here I am, navigating a whole new life with a rare disease tacked to my back. Rare disease. What a combination of words.
EDS and migraines definitely go hand in hand for me and one can exacerbate the other at times. When I’m having a severe EDS flare up, migraines can be triggered. When I’m having a particularly bad migraine, sometimes I will have an EDS flare up.
But, as with all my other illnesses, I take it a day at a time, moment by moment, and find the blessings (and hoofbeats) in disguise.