Wednesday, January 28, 2004

Big Whoop

The last time I had an EMG (where they stick the needles in you and listen for electrical signals), it went OK. This time it was kind of like, pretty painful. They stick them in, which is one thing, and then they do this thing called the "insertion test." That means that they are sticking it in a little deeper, moving the needle around, trying to annoy the muscle and look at the signal. I don't know what the factors were, maybe that I was lying down, or cold in that hospital gown, or that I'd had a workout yesterday, or what, but it was excruciating at times and I was sweating and shaking. They also have this thing, while the needle is still in you, of making you use the muscle they poked. Think of tearing and you'll see my objection. My thumb web muscle is kind of ticked off right now.

After the needles, and all my leg shaking, I was actually limping, and walking very stiffly in the hall. I got off pretty easy though: Apparently they have a test where they put the needles in you and add electric shocks! Let me make one thing clear: I am a wuss. I'm not good with pain, and needles, and like, rectal chainsaws. So what might be excruciating to me might be a walk in the park for someone who would much rather have some wee bitty needles in their flesh than have another chemo treatment.

There are people who go through a LOT of pain and discomfort because of their illness, and ALS just isn't like that, from what I think I know. Basically you slowly wilt and then die. No painful surgery, no being on chemo and trying to decide if you would rather be dead. It's kind of a gentlemanly disease. It gently steals you away.

And yeah, the doctors at the specialty center did confirm that I have it. Big Whoop. We videotaped them giving me the bad news. I have myself on tape right after that saying: "Big Whoop!" Not to them. To the camera, to myself. Big Whoop.

Why? Well, because, despite that I may have it, I intend to die of something else, at an advanced age. Or, given the amazing strides in science that we see every day, never die at all.

The cause of ALS is unknown, and there is no definitive test for diagnosis. But my motor functional tests, and EMG, and MRI, and the negative results for other conditions (such as MS), seem to point to ALS.

There is one approved drug to slow the advance, and they wrote me a prescription today. And there are some clinical trials for other drugs. I'll probably get in one of the trials. Creatine is a good thing to take, and I am already on it. Vitamin E is recommended too. I'll pick that up when I file the prescription.

They have workshops where all the ALS patients get visited by all sorts of specialists, occupational, physical, etc., and various doctors. They have monitoring programs. Basically these people are a full-service ALS center. I am in the right place. I appear to have the right insurance.

There was only one thing they did which seemed odd to me. The unter-doctor told me to always hold on to my MRIs, then later told me he wanted to keep mine for an extra day, so they could be photographed, because the FLAIR signal was abnormally bright. I asked him if this was unusual and he said yes, that in many ALS patients the condition does not even show up in an MRI. Later though, when I asked the main doctor whether the brightness of the FLAIR signal was unusual in my MRIs, he said it wasn't. Maybe he thought I was trying to suggest that the MRI proves I do not have ALS, but I certainly did not say that, and I don't think I conveyed that impression. So I am puzzled why my MRI was so unusual they decided to keep it, and at the same time it was unremarkable.

I asked the head doctor to give me a rosy scenario and a pessimistic scenario. In the pessimistic scenario I could be dead in nine months. He says that's not very likely. In the rosy scenario he says my symptoms could go away and I would be cured. He said that was extremely rare. The second rosy scenario is that my symptoms take a very long time to get worse and I end up living (and here is the magic phrase) "a normal life span." He said he has one patient who was diagnosed at 72 and is now 96. A real feisty old bat, apparently. If I can have "a normal life span," that would mean I get to see my kids graduate from college, and then some. (Grandkids?) And honestly, I have to tell you that if I can make it that far (and I think I can) that somewhere along the way (rather sooner than later), a cure for ALS will de developed. As well as a cure for all cancers, AIDS, and perhaps even aging itself.

He said he has reason to be optimistic about me. At 40 I am young, for someone who has ALS, which apparently gives me a reserve of cells to kill off with relative impunity. (ALS involves what appears to be apoptosis, or programmed cell death). I am chipper, and apparently that matters a lot. I am, uh ... good memory was not one of the factors. I am relatively strong and relatively unimpaired now. Um. Uh. Good thing we got this on videotape. I have good lung capacity. Yes, he says that matters a lot. Or, that is to say, it is a very important marker. Of your decline. There is an expected lung capacity for someone with my profile, and they tested me three times, and each time, I hit 94% of the expected capacity. They say this is good.

(The extra six percent is taken up by the Libyan enriched-Plutonium slugs I am storing, for a fee, in my lungs.)

Well, yeah. So, there you go. Even brain damage can be overcome.

The person I worry about most is not myself, because I at least get to stand in the center and fight back against this thing. And maybe not even so much my kids, unless I start to go downhill very fast, which I doubt will happen. I worry most about the impact on my dear wife, who came with me today, and stayed with me, and was my friend, and who has this condition too but is unable to "take a break" when she feels she needs it. But, she has said it is easier these days, having me around the house. And we got a chance to talk in the car on the way home, and we are going to look for ways to make life flow more smoothly for both of us.

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