Here’s What My Life Is Like with Ehlers Danlos Syndrome

By the author of How to Prep When You’re Broke and The Widow in the Woods

Yesterday, I shared some information about Ehlers Danlos Syndrome, but it was more like a PSA. Today I want to talk about actually living with it.

These articles are being posted because May is Ehlers Danlos Awareness Month.

Early childhood signs

Ever since I was a child, I was “clumsy.” I’ve gotten injured in the strangest ways. I bruised incredibly easily. I dislocated fingers several times playing basketball. I held my pencils in what looked like a death grip, but I was holding it the only way I could with my overly mobile PIP joints. (My finger tips bend backward more than 90 degrees.) I twisted my ankles repeatedly.

I was involved in sports from an early age, and I’d love to say that I was a natural athlete, but it couldn’t be further from the truth. I had to practice more and work harder to maintain my spot on the basketball team. I realize now that a lot of my difficulties came from hypermobile joints. It can be hard to catch a ball when the impact bends your wrist back more than 90 degrees. Running distance always hurt, though I was fine with sprints.

After a conditioning practice, I would come home in incredible pain. I thought at the time it was a sign of me being out of shape, but that wasn’t the case. I was extremely fit from playing competitive sports for up to four hours per day. It was because running caused issues with my hip joints and every joint below that. I know now that my joints weren’t supported the way other people’s joints were and my muscles had to pull extra duty keeping them in place.

Being hypermobile doesn’t necessarily mean that you can do the splits or that you’re a born gymnast.  It means that your joints bend beyond the point that they should. I had “party tricks” such as turning my thumbs around backward, touching my nose with my tongue, and other oddities. (Now, I know that doing these things is harmful, and I don’t do it deliberately.) It means you have less stability and things hurt us that don’t cause other people pain.

Adulthood

I’ve dislocated many, many joints, most of them multiple times. One of the strangest was probably the time I dislocated my hip when I was 22. I got hit by a car while I was cycling and I landed on my knee on that side when out she popped.

I hurt myself in the silliest ways, like waking up to searing pain because I just subluxated something in my sleep, partially dislocating my wrist when painting a wall, and tripping over absolutely nothing and spraining an ankle. The list goes on and on.

I was just particularly clumsy, wasn’t I? Since I’m adopted, I had no medical history that could’ve indicated there was something else going on.

But, no. It’s not about clumsiness. Not exactly. Let me explain. People with Hypermobile Ehlers-Danlos Syndrome often appear unusually clumsy because their joints are unstable and their bodies have impaired proprioception, meaning the brain has a harder time accurately sensing body position and movement in space. I think I know where my feet are, because that’s just natural to know, but sometimes I don’t, and then I wipe out.

Ligaments and connective tissues that are too loose allow joints to move beyond their normal limits, making sprains, dislocations, falls, and muscle strain far more likely during ordinary activities. As a result, seemingly harmless tasks such as getting up from the floor, reaching overhead to change a lightbulb,  or even sitting awkwardly while doing a puzzle can place abnormal stress on joints and soft tissues, leading to injuries that seem bizarre and disproportionate to the activity itself.

I was fit, I did endurance sports that didn’t hurt my joints through my early 20s, like long-distance cycling, and I was always an avid hiker. Because I stayed active, even though I got more injuries than most of my friends, I never realized I had a disease that was affecting me from my unstable neck vertabrae to my wonky little toes. I had adapted so well to it that I didn’t even know it was a problem.

It wasn’t until I met a friend with EDS who said, “Hey, you should get this checked out,” that I began to think there might be something going on. But at that point, I was still very active, I was traveling a lot, and I was in love with life. I still recovered fast from injuries, so I pushed the thought aside.

Finally, things came to a grinding halt. I partially ruptured a tendon in my ankle getting off a mattress on the floor. I wish I had a better story than that, but there it is.  I tried to push through it, and did for about six months, until I visited a bunch of castles on a tour in Romania and walked up and down about a zillion tiny steps that day. I was in so much pain by the end of it that when my daughter called me with a personal problem, I was happy to board a plane and immediately come back home to the US to help her through it.

It just went downhill from there.

This is a disorder you are born with, and for some subtypes, particularly the hypermobile one, diagnosis can take decades.

The first doctor I went to for my ankle injury hemmed and hawed and did cortisone shots in my ankle and foot along the tendon that was injured, telling me I needed to stay off my feet completely (aside from going to the bathroom and stuff) for two weeks. Then he’d do some other little tweak and tell me I needed to stay off my feet some more. No longer could I go for long walks or hit the grocery store with my little cart. By the time I changed doctors, the tendon had completely ruptured, and I could barely walk. My new doctor used the imaging that was already done and said that I needed a complete reconstruction and tendon transfer. He got me in within a month of our first visit, and he was wonderful.

The surgeon broke my heel and realigned my foot, then transferred a tendon from elsewhere in my foot to replace the absolutely destroyed post-tibial tendon. He told my mom and daughter in the waiting room that the tendon was very gummy in texture and was hanging on by the tiniest string. He said he was shocked I’d been able to walk into his office on that foot and he’d never seen anything quite like it.

Everything went well until I began to do physical therapy and to bear weight on that side. Then a different tendon in my foot snapped, and I had to have surgery again. (With the requisite bed rest. I also couldn’t walk after that tendon snapped, so I was in bed both before and after.)

I believe that this was a turning point, due to months of bed rest and time off my feet. Things didn’t heal properly, my active life hit a standstill, and suddenly, I was subluxing joints on almost a weekly basis. It was during this time that I got a diagnosis of hEDS, and things began to make sense.

Living with a disability

It’s been hard to go from being an extremely active person to one who must pace herself carefully and take multiple medications. Sometimes I dream that I’m in Greece or Romania, and the dreams are so real that when I wake up, I’m shocked to discover that I’m at home.

Emotionally, many people with advanced hEDS go through a major psychological shift.  (This is true with many chronic illnesses.) The goal stops being “pushing through” and becomes building a sustainable life inside the body they have now. When I started using mobility aids, I was embarrassed to be seen with a cane or rollator. But soon, I realized that these were tools to reopen the world.

I walk as much as I can without setting myself up for a flare. (This isn’t a lot, but I take 5-10 minute strolls if I don’t have anything else going on that day.) I absolutely do not do exercises like yoga – my joints are stretchy enough without adding to it. If I have appointments, I try to shower the night before and rest as much as possible before and after. My instability has worsened, so things like this – normal, everyday things – take a tremendous toll on my body.

This is worsened by the fact that I developed a major comorbidity: gastroparesis. People with hypermobile Ehlers-Danlos Syndrome may develop severe gastroparesis because the same connective tissue problems that affect joints can also affect the digestive tract and autonomic nervous system. When the stomach’s muscles and nerve signals do not function properly, food moves too slowly through the digestive system, leading to nausea, pain, vomiting, bloating, and difficulty maintaining adequate nutrition.

I had no idea that there was something wrong with my stomach – I just knew that as the months went by, there were fewer and fewer foods I could eat without feeling like there was a rock in my gut for hours and hours. I haven’t gone out to a restaurant in ages because there’s so little that I can tolerate. I mostly eat soft food and carbs at this point.

Flares

The article wouldn’t be complete if I didn’t talk about the flares. I get really bad flares after physical therapy or going out for the day. Any time I do something beyond my new normal, I can expect to be hit with a big whammy.

And I’m not alone. A lot of people with hypermobile Ehlers-Danlos Syndrome experience severe systemic flares after physical overexertion. These episodes can include vomiting, moderately high fever, chills, widespread pain, exhaustion, and flu-like symptoms, even when no infection is present. Researchers believe these flares may involve autonomic nervous system dysfunction and inflammatory responses, causing the body to react as though it is under extreme attack.

And when I’m actually sick? Forget it. The flares are so bad that they are usually what land me in the hospital, where it’s also diagnosed that I have pneumonia or some other infection.

I’ll be blunt. It’s awful.

Sometimes I can’t even figure out why I’m having a flare. Maybe there doesn’t have to be a cause every single time. It’s not something I can just push through to get things done. To do anything other than rest risks stirring up another bout of vomiting and intense pain. A flare results in anywhere from 24-48 hours in bed, feet elevated, head against a big pile of pillows.

It might sound great to spend days in bed – perhaps even pleasant and leisurely. It couldn’t be further from the truth. First, I’m actively sick during at least half of this time, dealing with nausea, chills, severe pain, and an indescribable feeling of exhaustion. Sometimes it feels like I can’t keep my eyes open and I sleep for 20 hours, getting up only for bathroom and Gatorade breaks.  Secondly, I don’t want to be in bed. I want to be hiking or traveling or at the very least cooking in my kitchen and running the vacuum. I always feel as though I don’t pull my share of the chores around here because I spend so much time in bed, sleeping.

Adapting

It’s frustrating, painful, and exhausting. (How many times have I used the word “exhausting” in this article? There seems to be a theme.) I hope that when I have surgery to repair my stomach, it will help since I’ll be able to start eating healthy things like meat and veggies. Maybe with proper fuel, I’ll be able to resume some of my favorite activities again.

I try to focus on what I can do, rather than what I can’t. I’ve written some good articles. I’ve published two novels and I’m working on a third. I still want to leave this world better for having had me in it, and that is what pushes me to write when I’d otherwise be watching YouTube videos about ancient Egypt and doing a diamond-painting craft. I can’t churn out material like I once could, but I want the things I write to mean something.

I really don’t know what I’d do without my daughter, who has kindly taken me in. I don’t have much money to contribute to the household, and I feel like a burden, even though she repeatedly tells me I’m not. It seems surreal that three years ago to the day, I was hopping on a plane in Greece to visit Sofia, Bulgaria, and this morning, my big journey was that I finally made it from my bedroom into the living room after two days of not doing so.

Believe me when I tell you, the identity crisis has been real.

This is where being an adaptable person has really helped me. However, I also have an excellent therapist who has helped me navigate these extreme changes in lifestyle. I don’t think that I’d have handled it nearly as well without him. I can’t recommend enough finding a professional to talk to if you have a limiting diagnosis like this.

I never expected to feel (physically AND mentally) like an old lady at this point in life. (I’m 57.) But here I am, crochet hook in hand and feet on a footstool. While that is a disconcerting thought, it’s certainly put other worries I used to have in perspective.

To quote my granny, I try not to borrow other people’s problems and make them my own. That’s why I no longer really cover current events on this site and try to make the content actionable and life-improving. I spend only about 10 minutes a day reading the news, which is not much at all for this recovering news junkie.

I don’t know what the future will bring. There have been some real advances in the research on the 13 Ehlers-Danlos syndromes, and I hope one day, there will be a treatment that helps give us back some of the mobility and life we lose with it.

I’ll always hope for a future where I can get back on a plane and go explore someplace new. But for now, all I can do is what I can do, here in my small circle of influence. I can do the small tasks that make my daughter’s life easier, like mending clothing, washing the dishes, and doing the laundry (I roll the heavy baskets around on my rollator.) I write when my brain is cooperating and not fielding distress signals from my body, and I try to write stories that people can see themselves in.

I’ve always believed that you don’t have to be a tactical expert to survive bad times. You don’t have to be able to run a marathon to find ways to protect your family. You don’t need a six-figure income to make their lives better in a crisis.

Now, I need to believe that applies to my life too.

Those who adapt are those who survive.

About Daisy