Today is Rare Disease Day, and since I have Ehlers-Danlos Syndrome, which is a rare disease, I thought it fitting to write a blog post about it.
Most people, including professionals in the medical field, have no idea what Ehlers-Danlos (or EDS) is, so I often have to do a fair bit of explaining.
If you’ve been following my blog, you already know that EDS is a heritable disorder of the connective tissue. Our collagen is faulty, and since collagen is one of the main building blocks of our body, EDS can and does affect the entire body.
We’re not just super bendy.
If only it were that easy, but there’s so much more.
Ehlers-Danlos comes in several different flavours, some worse than others, but all of them pretty nasty. And all of them progressive. Also, there is no cure. And most types are autosomal dominant, which means that with every pregnancy you have a 50% chance of passing the disorder on to your child.
I’ve been diagnosed with the Hypermobility Type, which certainly isn’t the worst, but it is associated with pain. Chronic pain, and a lot of it. I could go into detail about my symptoms and what the Ehlers-Danlos has done to my body over the years, but I have a better idea. I’ll just take you through an average day. Not one of my good days, where I accomplish a lot, and neither one of my bad days, when nothing gets done, but just average.
A day like today.
When I wake up, I’m already in pain. My heels hurt. They burn. They usually do, but sometimes they tingle or – even worse – feel icy cold. Not the kind of cold you get from being cold, but a cold pain. That’s because I probably have some nerve damage to my heels as a result of the fat pad atrophy. And in case you hadn’t guessed it, the fat pads under your heels are partly made up of collagen, which explains why mine have atrophied so horribly.
My back hurts and before I get out of bed, I need to pop my shoulder back in place. It likes to subluxate during my sleep. My elbows hurt. I don’t really know why, but they do. They hurt most mornings.
As I stumble to the bathroom, I bump into a doorframe or two. My house is a bit like Hogwarts: things shift around without my prior consent. I think they do it because they hate me.
As I brush my teeth, my gums bleed profusely. It’s not a matter of bad dental hygiene. It’s the faulty collagen again.
Getting dressed is a noisy business. All my joints pop and crackle like crazy, and if I’m not careful, I may well sublux my shoulder again, but that’s easily fixed, so I don’t really pay too much attention. It’s just one of those things.
Before I go downstairs, I put on my knee braces. Walking down the stairs always hurts, but without the braces I doubt I’d even be able to do it.
Braces. My daughters and I seem to collect them the way others collect dolls or other pretty things.
We love our braces. They help relieve the pain – only a little, but usually just enough – and enable us to do things we’d otherwise be unable to do any longer. Like walking down the stairs and riding our bikes.
Once downstairs, I put the kettle on to make us a nice pot of tea. I unload the dishwasher and get started on my other chores. Best to do them immediately, because I might not be able to do them later on.
I don’t eat breakfast yet. I’m still fighting my morning sickness, and no, I’m not pregnant. It’s just another EDS-thing. Just like the table that got in my way – all by itself – and may well have given me another bruise. I did tell you my house is like Hogwarts, didn’t I?
By the time I’ve got all my chores done, and downed several cups of tea, I might actually be able to eat something. A cracker, or a bowl of yogurt perhaps. Bread is usually a no-no. It makes me gag.
It’s already past noon, and I’ve hardly done a thing yet. My head throbs, my heels hurt worse than before and I’m knackered. I might as well lie down on the couch, or even curled up in a chair, for a nap.
If I’m up to it, I may play the piano for a bit somewhere in the afternoon or early evening. I’ll wear my wrist braces to stop my wrists from hurting overly much.
The little cushion on the floor is to protect my heel when using the pedal. Without the cushion, my heel pain would very quickly score an 11 out of 10. Yes, it’s that bad.
So why don’t I use pain killers?
Let’s see. I could take a paracetamol or two. Not that it helps one bit, but I could do it. It’s just a waste of money, so why bother?
NSAIDs, you say? Let’s go ask my stomach. No. My stomach informs me it won’t have it and will throw it up. Vehemently if I insist.
So where does that leave me? Opiates. I don’t think I’m that desperate. Not yet.
I would tell you more about my average day, but you know what? I’m wasted. I need to go to bed.
If I’m lucky, I might even sleep.