Building a Broken House: My Ehlers-Danlos Story

I remember the first time my flexibility surprised even me. I was nine-years-old at the park and just to see if I could, I wrapped myself around the monkey bars in a full circle; my hands grasping my feet. Not too crazy, right? Well, I was circled over backwards with my back against the bars.

It wasn’t until I was a teenager that I began to experience pain - first my feet, then my shoulders, then costochondritis. My first foot surgery was in high school, the second in college. By then, I was also receiving cortisone shots in my hips.

I got used to having problems. I broke my foot in my 20’s, but didn’t realize it. My podiatrist caught it on an X-ray years later before my third foot surgery. When you’re used to chronic pain you don’t always notice things like small fractures.

Things got even stranger (and more difficult) in 2004 when I broke out in hives with angioedema. The hives didn’t go away and after every test Mayo Clinic could throw at me, I was diagnosed with Chronic Idiopathic Urticaria. Now, 15 years later, I receive two injections once-a-month and it keeps the hives at bay.

Enough is Enough

A couple years ago I just couldn’t ignore what was happening anymore. As a professional pianist, my hands were starting to hurt enough where regular practice was becoming challenging. It wasn’t until after carrying a bag of groceries into the house and the middle finger on right hand became painfully swollen that I decided I needed real answers.

The doctor I saw for my finger was stumped. My lab tests came back normal. X-rays were normal. He couldn’t deny my swollen finger, but technically “nothing was wrong.”

It wasn’t a good enough answer for me. I poured over all my medical records, reading and re-reading them, looking up words in medical dictionaries. I looked at every x-ray and in every x-ray of my hands the radiologist had noted my thumbs were subluxed (partially dislocated). Why had this never been talked about, or addressed, or even mentioned?

Grasping items can be a challenge when small joints don’t stay in place.

Grasping items can be a challenge when small joints don’t stay in place.

I thought about my family and particularly my oldest son. He was even more flexible than I had been when I was his age. What if this was genetic? I quickly typed in “genetic collagen disorders” into my Google search bar and up popped “Ehlers-Danlos Syndrome.” As I read the symptoms list the tears came pouring down. The symptoms list read like a story of my life.

I brought my findings to my doctor who eventually referred me to Mayo Clinic. A few months later a Mayo Clinic geneticist officially diagnosed me with Ehlers-Danlos Syndrome hypermobile type. It took 21 years from onset of symptoms until I was diagnosed. This is only slightly longer than the average of 10-20 years for most people to receive an EDS diagnosis.

The geneticist explained that a vast majority of people with Ehlers-Danlos also suffer from comorbidities. It turns out my Idiopathic Chronic Urticaria is more-than-likely a result of Mast Cell Activation Syndrome (mast cells release histamine). When I asked the geneticist why these comorbidities are present in people with EDS he replied, “We don’t know why; there’s a gap in our medical knowledge.”

But, what is Ehlers-Danlos, exactly?

Building a Broken House

Imagine a competent and experienced carpenter makes plans to build his house. He gathers the highest quality materials and carefully reads the blueprint. He even has a machine that produces the nails, screws, bolts, flanges, and gusset plates – anything that joins lumber or masonry together.

The foundation is laid and the carpenter begins to build. First, he makes the nails, screws, bolts, flanges, and gusset plates following the metallurgist’s recipe exactly. When he holds the first nail in his had he realizes something isn’t quite right. The nail is pliable, able to be bent by hand, yet strong enough to be driven into lumber. How would this hold his house together?

He tries again and again, but each time the machine produces the same result. Time is running out, he must build his house.

He does his best and soon his house is built. From the outside it looks perfectly normal until a sections begins to sag. Then another. The house would creep and groan; wobble and shake. It would frighten the carpenter to know that at anytime one corner or rafter or joist or doorway could begin to pull apart; the nails, screws, bolts, flanges, and gusset plates bending under the pressure.

Before long, much of the carpenter’s days are spent supporting, bracing, strengthening and fixing his broken house. It takes so much energy simply keeping his house together that he starts to miss work. His employer tells him if something doesn’t change he will lose his job.

Well-meaning friends offer suggestions. Maybe you just need to put better metal alloys into the nail machine? You should bring in an expert! Maybe the carpenter is just lazy and don’t properly take care of his house? What about alternative carpentry? What about prayer?

Other friends try to understand. They sit with the carpenter and listen. When the storms come, they help brace the house. Sometimes they bring coffee. That’s the best.

It’s a Connective Tissue Disorder

Connective tissue is found in virtually every part of the human body from eyes to tendons to ligaments to skin to internal organs and even blood vessels. It’s like the nuts, bolts, screws, flanges, and gusset plates and my “house” creeks and groans, wobbles and shakes. Joints easily slide around causing tendonitis, inflammation or bursitis. Joints can dislocate without much trauma, but, for me, they pop right back. Sometimes, because of injury or when bones and joints slide around too far, surgery is needed to repair tissue or put things back where they belong.  

May is Ehlers-Danlos awareness month. I don’t mind talking about this condition or how it affects me, so feel free to ask questions either below in the comments, via email, or in-person.

I work very hard (just like the carpenter in my story) to keep my broken house together so that I can continue doing the things I love with the people I love. Sometimes I spend so much time keeping myself together that I don’t have as much energy to go around as I would like.

You may see me with KT tape, splints or braces and that’s okay. You can ask me about it, or just know it’s more than likely EDS related. I require no sympathy, but sometimes a listening ear is helpful.

And, coffee.

Coffee is always good

The zebra is the symbol of The Ehlers-Danlos Society. "When you hear hoofbeats, think of horses not zebras,” is a phrase taught to medical students. It encourages physicians to look to common rather than rare causes for illness. EDS is rare and is commonly overlooked resulting in the decades long diagnosis quest for most EDSers.

The zebra is the symbol of The Ehlers-Danlos Society. "When you hear hoofbeats, think of horses not zebras,” is a phrase taught to medical students. It encourages physicians to look to common rather than rare causes for illness. EDS is rare and is commonly overlooked resulting in the decades long diagnosis quest for most EDSers.