Chronic Pain: the neglected partner of Ehlers Danlos Syndrome

I don't post about my health here, because I have a private Caringbridge account where I whine about what's happening with my freaky body. But just this once, I am breaking my silence to share something that I live with every single day. 

I was born with a disorder called Ehlers Danlos Syndrome; EDS for short. It is a mutation of the connective tissues. The symptoms and side-effects can be treated, but the disorder itself is literally in every cell of my body. By a freak coincidence, I married someone with EDS. NO we didn't know at the time, nor did we know ALL of the issues it would cause in ourselves or our children - who also both have it, because it's passed genetically. Doctors are still connecting the dots with EDS, but for us much of that knowledge is too late... we have been and will continue to be a few of the “pioneering patients” when it comes to learning about EDS. If you are curious, HERE IS A LINK to an amazing site with information. If you want a shorter read, HERE IS A LINK to a list of comorbidities... or conditions known to be created or exacerbated by EDS. 

This post is NOT about EDS, but about chronic pain - JUST ONE of the symptoms that it causes, and my own experiences with pain. (For those of you who also follow me on Caringbridge, this is an updated copy of last November's post.)

People ask me what EDS FEELS like, because they've never heard of it. Well, neither have most of the doctors I've seen. Imagine walking into a clinic, and watching the doctor GOOGLE your condition right before you, then speaking authoritatively on what you've just watched them look up. Now imagine that this happens NEARLY EVERY TIME you see a new doctor! Yeah, that's my life. This is why I read medical journals and study my own health issues so extensively; because I have, and likely will again, been given treatments that have made me worse. It's not the doctors at fault, the condition and it's myriad connections are simply that unknown.

The most prominent AND overlooked/underrated issue with EDS is chronic pain. And yeah, people with HUNDREDS of ailments live with chronic pain - so I understand why people brush it off. But there's pain... and then there's PAIN! And as with most rare disorders, our pain is blamed on arthritis, and discounted as a minor irritation; nothing to complain about. And after decades of being told that our pain isn't worth mentioning... we become conditioned to ache in silence.

So what does chronic pain mean to someone with EDS? While I can only tell you about myself, I have spoken with over 400 people with EDS and know that while our individual pain levels are higher/lower - the types and triggers tend to be universal. So... from the top down:

Head: EDS patients are prone to migraine. On top of traditional triggers like light, sound, smells, stress, or the weather, ours can be set off by neck-strain, dislocating neck joints, or TMJ. Since starting blood pressure meds that apparently also prevent migraine I personally cope with 6-10 migraines per month, with Tylenol + caffeine taking the sharp edge from the pain, but never taking them away. Six months ago, I was enduring 26-28 migraines per month. Yes, nearly one each day. My migraines come with aura... which means they are often accompanied by visual and olfactory hallucinations, nausea, and vertigo. In the past I have been treated with ergotamines, narcotics, and barbiturates. While these will relieve the migraine, I then have stomach cramps, nausea, and gastrointestinal nastiness because EDS patients are often hypersensitive to medications... which is why I avoid meds for this. I also have TMJ - which means that my jaw randomly dislocates mid-yawn or while chewing. On top of the jaw pain, this triggers an instant migraine. SOME (from talking to others it appears to be roughly 1/3 of us) EDS patients are also nonreactive to local anesthetics... which means that "simple, painless procedures" are never pain-free. All of my baby teeth were pulled at-clinic, with Novocaine doing precious little to ease the pain. Nor did it help with any of the stitches or simple procedures I've had over the years. The two cavities I have had, left me in tears. 

Neck/Spine: EDS patients are prone to dislocating neck joints, scoliosis, crushed/herniated discs, and arthritis of the spine. I was diagnosed with sciatica and crushed discs while pregnant with my daughter 20 years ago, but the therapist I saw then said it would be easier to deal with the pain then to fix it. This has caused constant dull pain, (on the doctor-scale, a 3-4, with flares rising to 7-8) as well as sharp stabbing bursts of pain every time I shift my back while trying to sleep, bend, or walk ever since. Just this year I learned that I have scoliosis, arthritis in my SI joints, and my entire body twists from the hips down. I also have LSTV, which is a "normal abnormality" where my spine fused wrong in-utero, (most common issue? lower back pain) and dislocating neck issues. Turning my head too quickly or too far, (this happens about 10-15 times per day... more often if I'm driving) causes my neck to subluxate. This EITHER causes an instant-migraine, or a sharp stabbing sensation that makes me see stars, and feels as if I've been smacked in the back of the head. The only pain relief I have ever had from this or ANY of my non-migraine related pain issues is codeine-drugs, which will actually bring my pain down to a 2-3, but they make me feel like I have the stomach flu... so I hoard them when they are prescribed, and use them only when the pain is enough to make me cry - which is an 8.5-9 on the doc's scale.

What about non-joint issues? I was born with two hernias. They were both surgically repaired before I turned 9. I have GERD. It hurts to eat, and food literally backs up into my esophagus because my throat muscles have worn out, and I have a "warning spot" in my esophagus that COULD become cancerous over time. My heart has three leaky valves, at least one of which will require open-heart surgery to repair "sometime soon" and is currently causing random pains and twinges, freaky arrhythmia issues, lightheadedness, and high blood pressure. EDS exacerbates eye issues. I have been legally blind without correction since I was 12, and uncorrectable to 20/20 for four years now - and my eyes get worse with every year. This causes eyestrain which can create... yep, more migraines.

Elbows/Wrists: I have "trick" elbows and wrists that dislocate or simply lose the ability to support weight, at-will. Pain here is easy to describe. Imagine trying to lift something as light as a quart of milk, when someone strikes your elbow/wrist with a ball-peen hammer. It's not the temporary pain, because as pain goes this is MILD compared to everything else, so much as your likelihood to drop whatever you're trying to lift that gets you into trouble here. My elbows and wrists can also simply "lock up" and stop working until that same hammer-sensation jolts them into moving again. This happens about 20-30 times per day during the summer, and at LEAST twice as often during the winter. Literally nothing I have tried does anything to stop this from happening, and because the pain is always temporary I have never bothered to try relieving it.

Fingers: Oh, those tiny digits. Weather changes make my hands ache. Typing aches. Writing or coloring and crochet becomes increasingly more painful, meaning I tend to limit myself to 30 minutes or so before needing to take a break. I have not played the piano since I was 25, because of finger pain. On a GOOD day, my fingers dislocate 4-5 times per hour. On a BAD day, they can dislocate that often in a MINUTE. I stopped counting or seeing a doctor for broken fingers and toes when I turned 19. At that time I had broken over 200 fingers and at least as many toes. I have been unable to have digits x-rayed because I exceeded the maximum number of times one can be exposed in a year, several times. (Just one of the reasons I no longer schedule visits for a broken bone... that radiation can NOT be good for me!) So when I say that I know what a broken finger/toe feels like, I KNOW. For the past 15 years or so, I have averaged NO LESS THAN two broken fingers per month. Between the constant breakage, the long-term arthritis, the tendon issue in my left hand, (the tendon in my pinkie literally “falls out of place” causing my fingers to curl up, rendering it useless) and a new issue with my left thumb that goes BEYOND my experiences with digit-trauma, my hands ache constantly at a 3 (with my pinkie at a constant 5) on the doc's pain-scale. Bad days or flares are a 6-7, and yes there are days - especially during the winter, where I cannot do more than use my hands as giant mitts because my fingers have seized to the point of being useless. I use adaptive pens/pencils. When I typed this, it took three days because my fingers became immobile simply because it was November, and cold. Opening a jar of pickles can dislocate my fingers/wrists. Brushing my hair dislocates my fingers at least 6-8 times, and my wrists at least twice. I wear buttonless/zipperless clothing. And there are still days I cannot get out of my jimmy-jams, because of my back/fingers/wrists.

Hips: Yep, our hips dislocate. Both of my children were born when my hips dislocated hard and fast enough to begin labor. With my daughter, I literally SAW them give-way as I stood before a mirror and undid the hip-brace I was ordered to wear for 3 months to prevent premature labor! I'll just leave that one there without the associated pain. On a normal day, my hips sublux. That's when your joint goes PARTWAY out of place and then grinds its way back into place... yeah, it hurts. Yeah, you feel your bones grinding as it happens. They will alternately seize up or sublux literally every time I move from a seated to standing position, or shift positions. They can also give out as I'm walking. And they are ONE of the reasons I am unable to stand in place for more than a few minutes without shifting about... which also causes them to give out. The pain from the hips themselves? Imagine someone kicking you in the hip - with the sharp pain, the 20-30 minutes of sustained dull pain, and sometimes yeah... even the bruising. From getting up in the morning, or daring to get out of a chair.

Knees: This is the most frequent issue for EDS sufferers. It's also the most common in "normal" people, so it's the easiest to describe. Our knees can sublux AND dislocate. A sublux feels like we've been kicked in the knee. It leaves bruises. We fall. We drop things. A dislocated knee? Let's just say that the first time my knee went FULLY out, it cost me an $800 ambulance ride and it took the ER doctors FOUR HOURS to put it back in... they ended up giving me hypnotics and forcefully SHOVING it back into place. It took six weeks with an ankle-to-hip brace and another twelve weeks on crutches, along with three months of 3x weekly physical therapy visits JUST to be able to walk again. Since then I have FORCED my own knee back into place twice, learned to catch and shove my kneecap into place BEFORE it goes fully out, put my son's knee back once, and my daughter's knee back into joint twice. This hurts MORE than unmedicated childbirth. For me, my knees sublux simply by walking. I've honestly never counted, but roughly every 3-5 minutes on a good day, and as many as 3-5 times per MINUTE on a bad day. Hence another reason I use a wheelchair when "out and about." My knees also sublux when I am driving, EVERY SINGLE TIME I move my foot from gas to brake, or back again. I have counted, and on a low-traffic day from here to Target, (a 10-minute drive) this happens about 30 times. One way. In low-traffic. I have a knee brace, simply to allow me to drive. My knees also give out randomly as I'm sitting, lying down, and IN MY SLEEP! I was diagnosed with arthritis in my knees at 11 years old. The pain is a constant 3-4, with flares at a 7-8. Always. I have also had tendonitis 7 times. That's when your knees swell to the size of your upper thighs, and physically cannot support your weight for 3-9 months at a time. Oddly enough while this LOOKS the scariest, it HURTS the LEAST! Something about all that extra swelling actually cushions my bones, and while I'm dealing with tendonitis my knees ONLY hurt at a constant 2-3! Knee pain is something I have lived with since I was 11. It's the reason I was opted out of gym classes in school, the reason I have not owned a bike since I was 21, the reason I can no longer climb stairs, and the reason that so many weight-loss exercises are either insanely painful or impossible for me.

And Ankles/Feet: Have you ever had a sprained ankle? I've had approximately 400. Ever broken your foot? I've broken mine at LEAST 25 times, as WELL as the 3-400 broken toes that I am aware of. As a teen I would go in for a sprained ankle and come home diagnosed with 2-3 newly-noted broken toes, 6-10 times per schoolyear. I literally break my toes so easily and often that I don't register the pain as something to be concerned about. I have in the past few years charted the number of broken toes in a month a few times out of morbid curiousity, and to date my record is 11. Yeah, 11 broken toes in a single month. I began seeing specialists for my feet and "musculoskeletal deformities" at seven months old. From birth, my feet never grew right. Walking hurts. Running, when I COULD, was excruciating. I literally do not remember a time EVER, when my feet did not hurt. UCVs/Orthotics? They do nothing. A specialist once suggested that my bones have been broken so frequently that an orthotic could actually BREAK MY FOOT AGAIN with how... "unique" (his word, not mine) my foot issues are.

When I say that I have chronic pain or that I am ALWAYS in pain, THIS is what I am talking about. THIS is the pain that I live with every single day. THIS is ONE PERSON'S story of what it feels like, to live with the pain from EDS. When people ask how I am and I hesitate, it's because I am assessing how much of my current level of pain is even worth mentioning. When I say I am always in pain and people dismiss this and say, "Well, who isn't?" THIS is what they are dismissing as ordinary. These are JUST my EVERYDAY aches and pains. These are the aches and pains that I don't even bother mentioning.

On bad days, on the days when I actually tell people "I hurt" it's far, far, worse!