That’s the rate of Ehlers-Danlos Syndrome showing up in people. One in a million.
It makes it very hard to get diagnosed. When you are literally one in a million, odds are your doctor has never seen someone with your condition. To make matters more difficult, Ehlers-Danlos Syndrome has a fairly wide range of possible outcomes. The most recognizable symptoms – a particular set of facial features – are relatively rare. In order to diagnose EDS, a doctor has to look at your whole profile of symptoms. Otherwise, each individual symptom, each individual injury, looks like a singular event rather than a part of a vast web of symptoms.
There are some doctors who will tell you that if you aren’t in a wheelchair by the time you are 25, you don’t really have EDS, you have something like it but not as severe. Some doctors expect you to have the elastic skin that is common but not present in all of us. There is debate about whether hypermobility type EDS and benign joint hypermobility syndrome are the same thing.
Even if you do get your diagnosis, treatment is difficult. To be honest, the greatest difficulty comes again from the fact that there is such a wide range of symptoms. Most of us have fairly bad joint problems, but there are no set joints that are expected to be problematic. Some of us have gastrointestinal problems, bladder problems, central nervous system problems, heart problems, aneurysms, lack of muscle tone…the list is quite extensive. But because EDS is a syndrome – that is, a collection of symptoms that have been determined to be related – there is no guarantee that a person will have any given symptom. We also often present symptoms in atypical fashion. I have atypical trigger points: instead of having the typical point of tension, I tend to have ropes and bars of tension knotted into my muscles. I also have atypical bipolar, atypical skin sensitivity, and I’m sure there are other atypical things that I’m not thinking of off the top of my head.
Harder still is the fact that we are rather unpredictable in how we respond to treatment. A great many of us (myself included) do not respond normally to local anesthetics like lidocaine and novocaine. To have a dentist work on my teeth, I have to be injected with about twice the normal amount of novocaine, and it is a bit unpredictable how long it will take to kick in and how long the effects will last. I know a number of people who the same drugs have no affect whatsoever. My joints sublux, dislocate, and hyperextend doing exercises that are perfectly safe for most people. I’m highly sensitive to psychotropic drugs, but relatively insensitive to allergy medications.
The hard truth of having EDS is that you have to educate yourself. Why? Because we have to educate our doctors. Our doctors, especially general practitioners, rarely know much about EDS at all. It is not uncommon that they have a vauge memory of the involvement of stretchy skin and bad joints and nothing else.
We also have to be able to figure out when we’re getting adequate medical care. It is sadly not uncommon for us to be undermedicated for pain. There is a bizarre perception in some parts of the medical world that our condition does not cause pain. Especially when we are young patients, there is a tendancy to brush off our reports of pain, and as the doctors cannot actually measure pain…well, we suffer sometimes.
We have to know what the standard of care for our condition is, because again, our doctors often don’t. We have to know that we should be having yearly EKGs to check for deformities in our hearts and arteries that could kill us if untreated. We have to know about the kinds of braces used to treat our condition, because it’s rare for a doctor to treat the kind of joint injuries we develop. We need to know that our joints need protection and hyperextention is damaging, because unfortunately it is often written off by medical professionals.
We have to study not only the literature available on EDS, but also our bodies. We have to learn what our normal is, because it’s not the same as everyone else’s. We can’t take for granted what our bodies will and won’t do. We’re better judges of what assistive devices we need, and often we find ourselves in the position of convincing our doctors that the assistive device we choose is more help than harm.
EDS is a difficult condition to live with because of its complexity – it has an ability to reach into just about every part of your life. But that doesn’t mean that life can’t be good with EDS. You just have to be willing to walk, limp, or wheel a different path than most people take. What was it Robert Frost said about the road less travelled?