This past summer I was diagnosed with Ehlers-Danlos Sydrome (EDS), which is a rare genetic disorder in which a defect in the collagen gene results in faulty connective tissue. Collagen makes up the tiny fibers that give connective tissues (from nerve sheaths to tendons to ligaments to vessels and even organs like the skin) their elasticity. Think of them like rubber bands; not only do they stretch out, they also then snap back. The “rubber bands” holding me together are like the ones that are too old–they stretch way out, but they can’t go back and snap into place. People with EDS describe themselves as “bendy” or flexible.
One of the first questions the geneticist asked was if I had been able to amaze my friends with my flexibility as a child. Check. As a child I had no idea what people meant when they said that they couldn’t reach part of their back. I could reach anywhere on my back. I could lay on my stomach and curl my legs upward until they met the back of my head and then I could go even further until I touched my chin with my toes. Naturally, neither I nor my parents had the slightest idea that this might be a bad thing.
There is no treatment for EDS, but there are co-morbidities that can be treated, like Postural Orthostatic Tachycardia Syndrome (POTS). The geneticist generously granted me that diagnosis as well. More than just a tendency to feel faint when going from sitting to standing, it’s a form of Dysautonia, which is a nervous system disorder. It means that your sympathetic nervous system is sort of always turned on. Something that might make you a little nervous (going to the doctor to renew meds, for example) results in an overblown physical response. I’m really set at “The Tiger Got Me” all the time. It causes anxiety and insomnia and IBS, among other things.
There seems to be no predicting when one’s body will decide to initiate trouble. One of the hallmarks of EDS is chronic, difficult-to-treat pain. This was never really an issue for me until a year and a half ago or so, and now it is part of my daily life. It’s hard to accept the notion that I may never again have an entirely pain free day. My job is hard on my body, and I’ve had to already file for ADA accommodations to protect me from lifting too much or injuring myself at work. Currently, my fine motor skills are still functioning, but should those start to go, I will lose my job.
I wear silver ring splints on my fingers to protect those joints, and they are shiny and sparkly and I am sure someday I’ll turn around to find I’m being followed by curious raccoons, who like shiny things. They are very visible and obvious and I spend a ridiculous amount of time explaining them. I’m not the poster child type, so I don’t really care that much about educating people–I would prefer to be left alone with my reality most of the time. I have other splints that I wear on my thumbs which are not pretty jewelry but hold my CMC joints in the right place–important when I am sewing. I have wrist splints I sleep in at night, and others to wear during the day. I have compression deals for my ankles and inserts for my shoes. I’m considering making a steel boned corset to support my back. I’m supposed to have a knee splint but that is another very long story and the short version is that medical incompetence + resistance to looking stupid = I don’t have one.
All of this weighs on me as I prepare to go back to work this semester. Last semester was unusually physically hard, and I felt like I barely made it through, and needed much of this holiday break to recover from it. This coming semester should not be as demanding, but my job remains a physical job and while I am going to stick it out for as long as I possibly can, I don’t want to end up someone who has to crawl into bed as soon as they get home from work and misses big chunks of their life. At this point we have found nothing that helps with pain beyond hot baths (not easy to do at work) and splinting. Someone told me that wearing any kind of splint takes 10% more energy on your part to function with it. Thus, it can be exhausting just to put on ten rings splints and two thumb splints and two wrist splints and two ankle splints–that’s 40% of my energy being used to deal with wearing them.
Most importantly, to me, is that I love my job and I don’t want to lose the ability to do it. Right now I am in a huge creative swing and I think it’s because I feel like I need to make as much stuff as I can before I lose the ability. Maybe I won’t lose it, but I can’t take the risk. I need to make things. Clothes, art, crafts, projects. I need to produce as much as possible as soon as possible. It’s an odd mindset, but that is where I am at, today, in trying to live with EDS.