“When was the last time you weren’t in pain?”
It was a curious question. It seemed like a trick. I was there for the recent, severe pain I’d begun having the year before.
“Maybe when I was eleven?”
By twelve, most physical activity—tennis and soccer for me—left me needing at least two hours’ recovery nestled among ice packs. But around that time I’d been diagnosed with scoliosis. A few years later, I’d had a major spinal surgery to correct the deformity. Having had two steel bars, bone grafts, and an assortment of screws and hooks installed along my spinal column, it never occurred to me that the pain I felt after, and have continued to feel, concentrated in my back and hips, wasn’t normal.
Until this winter, I was semi-religious about the gym. I’d escape to the elliptical sometime between 5 and 7 AM every day. Then pain and fatigue began creeping in; that routine slowly dissolved. I beat myself up: how lazy. For me, pain without blood or trauma doesn’t merit more than over the counter intervention. Anything else is malingering. So week by week, my energy drained away. Finally, this spring, I realized something was wrong, really wrong.
Walking places had begun to exhaust me, the woman who thinks a 10-mile hike in 105 degrees is refreshing. Yoga had become progressively more painful; I’d begun sitting out poses. Each morning was an exercise in trying to minimize the pain of getting out of bed. I really didn’t feel like myself. There’d been periods like this before, though. My answer to most things, though, is to push through. I tried.
Stepping onto the sidewalk after one last workout, one I’d forced myself to and hated every minute of, my ankles felt wrong. Painful as if they were stiff but floppy as if I might roll them. Wandering mentally down my legs, I recognized my calves’ tightness and knees’ weakness. Something was really wrong. So I went back to my primary care, with little expectation.
There’s no telling how many times I’d gone to an internist about fatigue since adolescence, nor counting the number of ways my report of fatigue was dismissed. Stress. Depression. Diet. Mercury retrograde.
It was in the back of my mind that the standard excuses providers for my fatigue were obsolete: my depression and anxiety are well managed, my narcolepsy’s miraculously in check, and my stress level’s reduced. In short, I was (and am) happy, just feeling like shit. Even so, I expected another brush off.
The sunny nurse practitioner who saw me took me seriously. Her mother had struggled to get a diagnosis with similar symptoms. But we had the same conversation I’ve had with umpteen medical providers. I listed symptoms. She asked questions; I answered. She proposed the same blood tests I’ve had before.
The pain’s every morning, yes; all day, but worse in the morning. No, I don’t scream every morning. No, again, this fatigue isn’t normal. And I know fatigue: I have narcolepsy, it’s under control, thanks.
That was Tuesday three weeks ago. The next day, my girlfriend and I broke up, and I was thinking about everything but the tubes of blood the nurse had scuttled off with.
“Do you have a moment to talk?” Mired in a pile of receipts, navigating months of reimbursements, I’m thinking the RN’s called back to suggest next steps, passing me off to the next doctor.
“There’s a high likelihood you have lupus and a small one that you have Sjogren’s.” It didn’t process at first that she was telling me I had one or the other, not suggesting the possibility. Then I got it.
The next few days were full of feel-good activities. Friends came out in force. Dinner Thursday was sushi and bubbly rosé which, as my friend Jacquelyn promised, pairs well with raw fish. Friday’s lunch was a surprise sent from California via delivery. Dinnertime brought more indulgence: Indian food with a college friend. Sprawled on a Persian rug, backs against the couch, we tore through chicken, spinach, and paneer with chopsticks while watching three of the worst horror movies we could find on Netflix. (Anti-Birth wins.)
Come Sunday, I faced a 6 AM flight and multi-state tour—to talk about the evils of Brett Kavanaugh, a.k.a. the Lord’s work. A friend of a decade, just visiting, came over to be with me before we each flew out. He ordered a gyro, we chatted, then he took upon himself to help me pack and clean, whisking away the bags of recycling and garbage I’d dreaded lifting and carrying down two flights of stairs. He gave me a soul-reviving hug, promised me I’ll be fine, and left me with hope—and a clean apartment.
Then came the action plan.
Two weeks later I walked into Johns Hopkins’ outpatient center. Getting an appointment there has to be one of the greatest strokes of luck I’ve had in a while. The particular doctor I saw, an even greater stroke of luck. His questions were a mix of the familiar and the unexpected. His physical exam began with my hands.
“Are you double-jointed?” He’s squeezing my knuckles one by one.
“Sure.” I pop my thumbs out of joint.
“Have you always known you’re double-jointed?”
“Well, yes.” Where was he going with this?
A thoughtful glance. “You’re part of less than one percent of the population.”
His eyes go back to my hands. He bends my pinkie back, inspects the others, then asks, “Can you touch your thumb to your wrist?”
It’d never occurred to me to try, but damned if both thumbs don’t lie flush against my wrist with little effort.
He moved on to my knees and elbows.
“Does this hurt?” He pokes a chest muscle. “Yes.” I stop. “Not much, though.” “And here?” He jabs at random spots. They all hurt at least a little, now that I think about it—which I don’t notice day to day, because that’s just how my body’s always been. These jabs are nothing compared to what happens when I bend, lean, or walk the wrong way.
He concludes his bizarre exam and sits, poker-faced.
“There’s no question you have some form of autoimmune disease,” he says calmly. “In Europe, we have the term ‘incomplete lupus.’” There’s a 30 percent chance it becomes full-blown lupus within five years, but we can cut that significantly. The treatment is the same as for its nastier successor, anti-malarials. They’re a life sentence, but with a single, unlikely side effect that requires ophthalmological check-ins.
The relief lasts just a few seconds.
“I don’t think that’s what’s causing your pain and fatigue right now,” he says next. My brain melts. I’d thought there was finally an answer. Then he goes on.
“You have what’s called Ehlers Danlos Syndrome, type III, called hypermobility.” I blinked. Connoisseur of bad medical television dramas that I am—I’ve watched Private Practice twice and really tried with Off the Map, Shonda—I recognized the name. It was a struggle to import that information in this setting, though—my body.
“You’ve had it all your life.”
I blinked again, maybe.
“Didn’t you do tricks for other children?”
I resisted replying. I was a human child, not a puppy. I settled on a head shake.
“People like you are usually cheerleaders or dancers.”
People like me? That didn’t deserve a response.
He’d been assessing me on the Beighton Scoring System. “There is a nine-point scale for evaluating the condition,” he informed me. “You have at least eight points.”
It’s not just my thumbs: my pinkies bend back more than 90 degrees; my knees and elbows are at the wrong angles.
“The ninth test is whether you can put your palms to the ground from a standing position.” I look at him. “Yes, I was sure you could do that.”
The body I have is the only one I’ve ever had; how could I have known it’s so different? That the pain I’ve lived with every day is not how other people live? Pain is part of who and how I am.
Do other children sit in circles and tug at their skin to see how far it stretches? Tug at their thumbs and compare angles? Compare pain levels?
Something’s wrong with my collagen. And collagen makes up about 30 percent of the proteins in our bodies. It’s skin, but also ligaments and fascia. I’d had an umbilical hernia in my early 20s; turns out, probably because of my defective connective tissue. Scoliosis, too, is linked. When your ligaments only stretch out and never rebound—they’re supposed to be like rubber bands—it causes all sorts of problems.
Dislocations and subluxations, or partial dislocation, are common. So’s damage to the joints and surrounds. And when ligaments don’t hold muscles and bones together, muscles overcompensate; they’re tight, constantly. I’d noticed this around the same time the exhaustion began intensifying, but I chalked it up to anxiety.
Specific muscles are particularly vulnerable, like those in your neck, doing the full work of holding your head up. Which is why migraine is common among us “zebras,” as Ehlers Danlos patients refer to themselves (ourselves?). Other unpleasant, some NSFW, complications or co-morbidities chip in pain as well. At some point, the brain’s so overloaded with pain signals, it stops regulating properly. In the end, where I was and am, everything hurts.
Contrary to what I’ve told myself for three decades, pain matters. Even if you’re not bleeding; even if there’s not a single bone sticking out anywhere. (Cue deeper inquiry into how women are socialized to discredit their own corporeal experience and their pain is ignored.) The pain I’ve ignored, resented, been ashamed of—it’s not just pain, it’s extraordinary pain. Yet it took an outside authority to give me permission to believe what my body’s been telling me for years.
Extreme pain clouds thinking, makes sleep difficult, and generally wreaks havoc on your body, even affecting hormone production. It even slows healing. But healing’s slow for me anyway because of my stretchy, fragile skin—another gift of Ehlers Danlos.
It’s nice to know why I’m exhausted, why I’m in pain—why I’ve felt so alienated, so agitated at times. That there are things I can do to mitigate this genetic inconvenience. There’s a community out there—here’s my favorite helpful explainer so far. On the other hand, it’d be even nicer if the answer were something that could be fixed or cured.
There are a few immediate next steps. Because the pain in my hip and lower back has been so extreme, and because folks with Ehlers Danlos are prone to various injuries and issues, the doctor sent me for an x-ray. The radiologist’s notes meant nothing to me. Fortunately, my hero translated.
“In adults these x-rays findings are suspicious for tears of the labrum which is a part of the hip joints,” he jotted. “If confirmed, you will need to see an orthopedic surgeon.” Reader, I live in a third-floor walk-up.
Then there are a few less pressing tasks. A geneticist needs to confirm that I don’t have a more serious iteration of Ehlers Danlos. A neuro-psychiatric evaluation will establish why I’ve been so foggy—it’s probably pain, but making sure makes sense.
Life from this point on will involve a lot of caution, a great deal of physical therapy, and maybe, if it’s totally necessary, me getting into a pool and letting a stranger touch me and make me exercise in the water in the middle of the day. Usually you’ll only catch me in the water if it involves a scuba tank or, alternately, the sun, a Kindle, and a cocktail. Usually in combination with a float.
Having a name for what’s wrong, an explanation for how I’ve felt and things I’ve beaten myself up over for more than two decades, has let me forgive myself a bit. It’s not that I’m lazy or weak; perhaps I’ll even find my way to granting myself that bit of grace my therapist urges.
How do I reconcile myself to not one but two life-sentence diagnoses? Lupus isn’t curable. Not yet. Ehlers Danlos never will be, best I can tell.
My coping mechanism is knowledge acquisition. This methodical march through medical journals and forums brings ups and downs; it’s comforting to feel some sense of control, to be engaged in a familiar task, but confronting the worst of what I’m facing can do to the human body is terrifying. Because of my spinal fusion, three vertebrae do most of the work for my back; because of Ehlers Danlos, any normal vertebrae would already have been at greater risk of deterioration.
The NIH warns that I can look forward to degenerative joint disease, if that’s not what’s wrong with my hip already. “Chronic pain, distinct from that associated with acute dislocations,” the site pronounces, “is a serious complication of the condition and can be both physically and psychologically disabling.” Well, yes. Things the doctor didn’t tell me: There’s an explanation for the easy bruising, for a dozen weird bodily quirks I didn’t think to mention that fall under the umbrella of the ominously named dysautonomia.
Rather than feeling like I could reach out to friends during rough stretches, I was ashamed. There were different excuses for missing different obligations when I just really couldn’t make it. Without a name, you can only say “I just don’t feel well” so many times. Then again, invisible disability, named or not, is often treated as invalid: “You looked fine.”
Knowing that what’s wrong isn’t my fault, that there’s a name for it, that it’s serious—it’s let me admit that I want and maybe even deserve support. Do I know what that looks like right now? No. Best I know, it’s a flare-by-flare, appointment-by-appointment sort of thing. Just being willing to come over for dinner when I’m too tired to leave; to order in with me when I’m too tired to cook—little things that relieve isolation are critical. That’s happened already.
Friends’ ingenuity and generosity have awed me; those that know have each been there in their own way. The rest will be there in their own way, and I wouldn’t have it any other way.
Strengthening the muscles around joints will reduce the pain. Until I reach that goal, though, while I’m getting into a rhythm, just moving around can be agony. That’s why I quit going to the gym, of course, as I pointed out to my doctor. “You won’t like me very much for at least a week,” he replied.
Walk by walk, workout by workout, I’m getting better, even if I don’t feel better. What control I have I’ll use to make sure I stay as able-bodied as possible for as long as possible. At least I know my body now.
On the worst days, I can still get semi-comfortable curled up in bed, in a nest of yellow linen. That’s where you’ll find me. Butt down, knees up, back cradled by a pillow quartet, laptop propped on a bolster in front of me, staring out the window. Probably flexing my stiff joints carefully as I can. There’s a lot of stretching—and crackling. Even my toes are double-jointed.