by Jacks Boen
You’ve heard the phrase, I’m sure.
“If you hear hoofbeats, look for horses. Not zebras.”
It appears in medical dramas like ER, New Amsterdam, or Grey’s Anatomy. It originally appeared in an issue of the Arkansas Gazette in 1962, showing a doctor teaching his students about making a diagnosis. The phrase is described as the “heart of proper diagnostic procedure”.
I hate that phrase with everything in me.
If you don’t know, the phrase is used by doctors. It means to look for common causes for issues, not for rare conditions. It’s why the widely accepted symbol for Ehlers – Danlos Syndrome is a zebra. It’s also why the condition is almost always misdiagnosed or shrugged off.
Ehlers – Danlos Syndrome is a group of fifteen or more genetic disorders that affect the joints, skin, cartilage, arteries, and veins. It’s not something that shows up in blood, or urine, or any other typical tests. It can only be diagnosed by listening to your patient, and by genetic testing. It’s something that most doctors would only hear about in a single 20-minute lecture on rare conditions, and then never see again. It also affects millions of people, even if it’s only a small percentage of the overall population.
Doctors, I learned from a young age, don’t like to be questioned. They’ve gone to school, they’ll say, and you shouldn’t confuse your time googling your symptoms with their medical degree. Surprisingly (or maybe unsurprisingly, depending on your experiences with doctors), googling my symptoms is the only reason I got my diagnosis when I did.
2004 – Pediatrician
My first experience with this was when I was seated in a doctor’s office at nine, explaining the pain in my legs and hips when trying to sleep, like someone was shoving red-hot needles into my skin. My mom mentions my clumsiness, and how often I roll my ankles or complain about my wrists aching. My pediatrician patted my hand sympathetically and told me that ‘growing pains were to be expected’ and then diagnosed me with Restless Leg Syndrome. I was just a child, so I nodded along with my exhausted mother and didn’t question.
For a while it becomes a party trick, something I do for the entertainment or convenience of others. I fell and dislocated my shoulder by mistake? Watch me put it back and marvel over how easy it was. It doesn’t even hurt, and my mom is just relieved we don’t have to go to the hospital again. Look how I can bend and twist, I’d be great at dance! I join the dance team, and my double-jointed flexibility becomes an asset.
2017 – Obstetrician
Twelve years later, I’m pregnant for the first time. I’m exhausted, unable to lie down without severe pain and vomit inducing levels of heartburn, eating antacids like candy by the handful. I bring it up to my OB, wondering to her if it was normal to have this much pain, this many problems, in a pregnancy. I questioned whether something else could be wrong, some underlying problem causing the pain to be worse than it should be. My OB pats my hand sympathetically and tells me that that brand of antacids isn’t good for the baby. I should try Tums instead. I spend the next four months sleeping sitting up and barely able to walk, and at 38 weeks I march into Labor and Delivery in tears and tell them they’re breaking my water. It takes a fight, but eventually I have my daughter.
2019 – Urologist
For a while after that, it gets better. I work my way back up to walking every day and even start hiking again. It feels like things are back to normal, for the most part. I struggle with my health still, this time dealing with recurring bladder and kidney infections. They’d develop so quickly it took my breath away; the mild ache that signaled their return devolving into crippling, horrible pain faster each time, like they were trying to outdo the one that came before. The last one nearly requires hospitalization, after less than 48 hours. After several in three months, I’m referred to a urologist. I schedule and wait six months to finally speak with the doctor. I don’t miss the looks I get when I walk into that waiting room. I’m the only person in that waiting room below the age of 60, and it’s clear that I don’t belong there. I wait anyway, fidgeting with the sleeves on my brightly colored anime hoodie and pretending I belong.
I’m relieved to see it’s a female doctor that walks into the pristine little room. In my experience female doctors are better listeners and might be able to help. They tend to be less likely to blame my issues on weight or diet or (my personal favorite) anxiety. She listens as I explain the speed at which the infections happen, developing from the slight twinging pain that lets me know they’ve come back into crippling agony within a few hours. She pats my hand sympathetically and asks me if I’ve tried taking cranberry supplements and if I was sure I was properly cleaning myself.
I did question her then, telling her that I am a full-grown adult who does in fact know how to take care of herself, and this problem was quickly becoming worse. That I didn’t need herbal supplements or a hygiene lesson, I needed help. Doctors, I was reminded, don’t like to be questioned. I leave the appointment in tears, with a prescription for cranberry supplements in one hand and a single sheet of paper explaining proper hygiene practices in the other. I feel like I’m nine years old again instead of an adult. I’d waited over six months for an appointment that lasted less than six minutes and left me with no answers. Each time this happens I lose a little more faith in the medical system.
2020 – Obstetrician
Time moves on, and then I’m pregnant a second time. This one is worse, in every way. The pain is constant, the vomiting and heartburn worse than ever. I start contracting at 23 weeks and spend the remainder of my pregnancy with regular contractions. My OB pats my hand sympathetically, prescribes me medication to prevent me from dilating, and sends me on home. I don’t bother asking her about it this time, I don’t bother listing out the exhaustive, unending symptoms. There’s no point anymore.
I was nearly 34 weeks when a sharp, tearing pain ripped through my stomach as if I’d been cut open. The contractions picked up with a ferocity I couldn’t believe, each one accompanied by a burning, tearing pain that left me breathless, and I was certain something was wrong. I went to the hospital and a smiling nurse told me cheerfully that I was in labor, but it wasn’t time for me to be there yet. I should go home, labor in ‘comfort’ until my contractions are closer together.
I tried to insist that something was wrong, this wasn’t the same as my first labor and I needed help. Her eyes never left my chart as she patted my hand sympathetically and told me earnestly that every labor was different. I did wind up going home and spent four hours sobbing under a scalding hot shower as pain wracked through me. I went back again, determined to stay until someone took my concerns seriously. In the end, it was my water breaking that convinced them. Not because they accepted my word, but because the amount of blood in my broken water was “cause for concern”. I still remember the look of abject horror on my mother’s face as they wheeled me out of the triage room, despite her weak insistence that everything was fine. Everything becomes very blurry, after that.
When my labor finally ends my son is whisked away, with a vague explanation that he “needed some help breathing on the outside”. When my doctor finally returned, she explained that I’d had what was called a placental abruption. The placenta had started to rip away from my uterus, every contraction worsening the tear and causing me to bleed internally. The doctor insists that there was never any real danger, the doctors and nurses handled it exactly right and we were both fine.
I’m not so loopy that I can’t see the case of CYA (Covering Your Ass) in effect, but I’m too exhausted to argue with her. My son and I spend five days in the hospital together before we get to go home.
When I follow up with my OB, two weeks later, she explains that no one knows why this happens, but I should probably avoid another pregnancy. Now that I’ve had one abruption, my likelihood of more increases. She tells me that I’ll be fine now and compliments me on the way I bounce back from the babies. I leave that room with no more answers than when I went in, but with a knowledge that whatever was happening had nearly killed me and my son both.
I don’t bounce back, not this time. I didn’t understand why but walks that would have been cake before I can suddenly barely manage. Where I used to hike mountains and walk miles, now I couldn’t make it through Walmart. My hips started shifting and snapping painfully with each step, what had once been pins and needles became steak knives and ice picks. I start to do research and come across descriptions of Ehlers – Danlos Syndrome. A condition that causes joints and cartilage to be too stretchy and less able to snap back like it should. It doesn’t take a great leap to put it together; pregnancy releases relaxin, which makes your muscles, ligaments, and cartilage stretch to allow for birth. Especially the hips and pelvis. Put that together with a disorder that prevents the same things from snapping back and recovering, and here we are, two pregnancies later and unable to chase my kids or walk to my mailbox and back.
2021 – Endocrinologist
I refuse to schedule with my doctor for a long time, insistent that it will get better, but my husband forces me to see her. She’s my primary care doctor, and my endocrinologist for my HRT. I finally go in to speak with her, explaining the pain and the issues with my joints. Her eyes never leave my chart as I speak, and I feel like a kid in the principal’s office making excuses for late work. I explain it all; the issues with my pregnancies, the way my wrists pop and my hands go numb when I pick up my children, the way my hips roll painfully with each step. I even bring up the condition I’d read about, questioning if it was possible that I had this condition, or something similar, affecting my joints. I can’t help but notice that the doctor looks like she’d rather be anywhere else as I talk.
“I can send you to a physical therapist” she offers it to me reluctantly, like she’s giving me some great boon, “but they’re really busy. I see a lot of kids like you complaining about joint pain.”
I try not to let the irritation show on my face as I nod. I’m not some kid complaining about joint pain, I’m an exhausted and miserable parent who needs help, who needs answers about what’s going on with me. But doctors don’t like to be argued with, so I thank her and leave.
She sends me for bloodwork first, letting me know that I’ll get a call when the referral is put in. The blood work comes back negative for rheumatoid arthritis, for Crohn’s and Lupus, and for other autoimmune conditions she’d checked for. She leaves a note on the bloodwork that states I’m anemic, and I should try adding more iron to my diet, and that will likely help with the pain and symptoms I’m experiencing. The referral for the physical therapist never arrives, and it just adds to the bitterness tinging every pain.
Another year passes and my ability to walk and move deteriorates steadily. The whole family gets COVID and, I know now, the virus accelerated the condition and worsened my symptoms. I still remember sobbing in my bed, feeling like my bones were breaking every time I moved, and wondering to myself if this was how I died. Of course, it wasn’t; nothing was broken, and the world kept turning.
2022 – Emergency Medicine
It’s when I suddenly developed severe pain in my eyes, unable to even open them without sobbing, that I went back to the hospital again. I sat in the waiting room, unable to open my eyes and just listening to the chaos of the ER. I wondered if I’d been blinded, if something had maimed me permanently.
After hours in the hospital the doctor diagnoses it as “Scleritis”, a condition where my immune system, triggered by some illness or virus, attacked the sclera in my eyes as if they were a foreign virus. Temporary, thankfully, just painful. The doctor prescribes me medication and tells me that I should look into auto immune conditions, since that’s the most common cause of Scleritis. My mind turns once again to the condition I’d been learning about, Ehlers – Danlos, and finally schedule a new appointment, with a new doctor. Just one more, I decide, before I give up. This time I’ll be prepared. I’ve done my research by this point, poured over articles and documents and taken notes. As I walk into that office, I’m rehearsing what I’ll say in my head and holding a handful of paper up like some kind of shield between us as I sit in that stuffy, too-bright, eerily quiet office.
2022 – Primary Care Provider
I still remember the sharp smell of antiseptic, offset so jarringly with her flowery perfume when she blew into the room. She was all smiles, blonde curls and cute shoes, and I couldn’t decide if I was relieved it was someone young or wary of yet another “I know exactly what I’m talking about, don’t question me” fresh-out-of-college doctor. I don’t give myself time to question it before launching into my prepared speech like I’m giving a lecture, staring down at my fidgeting hands rather than looking at her.
She listens. She nods quietly as I explain, watches as I swing one leg and show her the way my hip snaps in and out of place. She reads what I hand her and asks questions, not from a place of skepticism but from a place of understanding. When I finally stop my rehearsed spew of word vomit, waiting for the rebuke or the arguments, she takes my hand in hers. She doesn’t pat it sympathetically; she doesn’t look away as she catches my eyes with hers.
“I think you’re right.”
With those four words my whole world crumbles. This should be good, shouldn’t it? She’s confirming what I’ve suspected, she’s agreeing that something’s not right and that I need help. Something I’d been hoping to hear for years, something that’d been 28 years in the making. Shouldn’t that be a relief? Instead of relief, I’m filled with a kind of exhausted dread. I think I knew, even then, that the confirmation I’d received was just the beginning of what life would become from here.
2022 – Physical Therapist
She sends me to a physical therapist, who takes one look at the way I move and meets my gaze with a nod. I haven’t said anything, didn’t want to somehow ruin this run of luck with doctors who believe me. I was almost afraid to say it in case I’d be accused of doctor shopping or attention seeking. I just wait as she looks me over.
“Have you and your doctor considered Ehlers – Danlos?”
I could have kissed her right there. For the first time ever, I didn’t have to present what I thought I had like it was some kind of thesis, I didn’t have to defend it like a dissertation before I could be taken seriously. That was all the confirmation I needed. All the confirmation I was going to get; the only geneticist in the state would not do testing for Ehlers -Danlos Syndrome after all. For a while my doctor and I considered whether we should schedule with an out-of-state geneticist, eventually deciding that it wouldn’t be worth the cost to do the genetic testing. I was showing all of the physical markers of EDS, so it would only narrow the type, which my physical therapist was already pretty certain was hEDS (Hypermobile Ehlers – Danlos Syndrome).
2023 – My New Normal
The next weeks become a whirlwind of exercises and pain management plans; learning how to live with my new normal. I learn the proper way to relocate my joints and exercises I can do to help with my pain. Oh, and tape. Literally, sometimes on bad days I’m held together purely by tape and spite. Kinesiology tape, they call it, and it helps my muscles hold my joints in place.
The diagnosis leads to more doctors and uncovering comorbid conditions that hadn’t been on my radar with the pain of EDS filling my focus. Slowly my quality of life improved, learning to accept my limitations and use the tools I’d been given so I could still live my life. It still takes the better part of an afternoon to do something like the dishes. Realizing that I’d need a wheelchair to navigate large places, such as the zoo, the botanical gardens and the airports was a blow, but I slowly accept it. It was a lot harder to accept that I’d have to stop HRT, but eventually that was accepted as well.
Doctors’ appointments have taken over my life; physical therapy, cardiologists, saline infusions, specialists. Everything I do requires checks with my army of medical professionals. Somehow even worse, is dealing with the public. For some reason, the use of mobility aids as a young person seems to be taken as permission for unsolicited medical advice from the armchair experts. I started hearing the phrase “well have you tried insert random herbal remedy or exercise here” more than I ever have in my life, from every Tom, Karen, and Linda in the grocery store check-out line. Everyone had an opinion, a suggested remedy, or (in one case) a very firm insistence that all of my issues were caused by poor diet and Big Pharma.
It almost feels like starting over. Needing to explain the condition to the doctors and justify the treatments and tests I’ve been referred for, as if to prove that I’m not wasting their time the way mine has been wasted up to now. Having to patiently explain that, no, I’m not too young for the mobility aids I use, I do in fact need the saline infusions and banana bags. Meditation won’t fix it; yoga isn’t the answer. Some days I’m less patient than others. (Here’s a sincere apology to the man in the gas station who was told to take his herbal remedy and shove it somewhere unpleasant. I was having a bad day.)
I thought a few times about looking for a support group, for others with the same condition who’d understand the frustration of suddenly being unable to do everyday tasks. Unfortunately, support for EDS is limited. There’s no in-person support groups, very little I could find online. Surprisingly, it was on TikTok that I found a semblance of a community. Others with EDS, with POTS, with other comorbid conditions that we now share. It’s not communication, but it’s still a relief, knowing that I’m not the only one dealing with this, struggling to do things like shop or clean while managing constant pain. We can compare notes, make jokes no one else would get, complain and cry together, and raise awareness of the condition and its effects and symptoms.
But at least now it has a name, this zebra that walks behind me. It has a face, and I’m learning to co-exist with its echoing hoofbeats in my life. After the fight it took to get them acknowledged, I wear my zebra stripe ribbon with pride. As much as I hate zebra print, I wear those (admittedly gaudy) stripes like a badge of honor. I’m not crazy, I’m not inventing it or faking it.
And I’m no longer afraid to argue with doctors.
Leonardo Magazine 