Wednesday, March 28, 2012

Ehlers-Danlos Syndrome: Why My Heart Might Explode

I mentioned in another post that there can be other conditions that go along with Chiari. Well, now I can welcome myself to the world of Ehlers-Danlos Syndrome.

Ehlers-Danlos is a group of connective tissue disorders that can cause a lot of problems. The symptoms are strange. Here are mine:

- Double jointedness (You should see what I can do with my pinkie.)
- Soft skin (I thought it was the Bath & Body Works all this time!)
- Short stature (I am barely 5 feet tall)
- Joint pain (check!)
- Fine hair (forget trying to brush it-the minute I do it's in tangles again)
- Visible veins (I'm pretty stringy on top and in my legs)
- Difficulty healing from wounds and surgeries (I still have cat scratch scars from the 90's)

The funny thing is, I had NO idea any of these were related.

This syndrome also explains some of the problems I had with my hysterectomy. When the surgeon was in there he said that my tissue was in very poor condition and that it tore and disintegrated whenever he tried to clamp it or move it. He thought I might be a vegetarian. (I'm not.) It turns out, it's part of this nonsense.

So, there are a few problems this can cause. For one, healing after surgery becomes an issue. That's probably why it takes me so long to heal and why my surgery scars remain a deep purple for years afterwards. It also means that for the brain surgery I will probably have to use my own tissue instead of that of a cadaver. Which, of course, is perfectly fine by me.

This might account for my joint pain. Then again, it might not. It probably does account for the fact that every time I move my body cracks and creaks like old floorboards.

The pain can be managed and surgeries can help with the ligament problems. (Essentially, my body is literally falling apart.) However, there is a dark side to this.

Vascular Ehlers-Danlos (or, Type IV) is very serious. It affects the heart and other internal organs. More importantly, it can cause the internal organs to rupture. Now, who wants to think about their intestines exploding? Not me. It could explain why I had a placental abruption. Generally speaking, women with the vascular type are not recommended to get pregnant. Oops.

The bad news is that life expectancy for this is not great. It hovers around 48 years of age with 80% of people having life threatening problems before they are 40. Now, I know that realistically I could step out tomorrow and get hit by a car and die. However, having someone actually tell you that you might not live very long is not a very nice thing to hear. There is no "cure" for this and not even any treatment. If the heart  ruptures then there's very little they can do about it.

I'm kind of pissed off about this.

At any rate, it's now off to the cardiologist. 

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