I'm Only Going to Tell You This Once

Cheesh, one crummy blog, and already folks are complaining.

Remember, this is a blog not a doctoral dissertation, so, no, I’m not going to cite my sources. Does Drudge cite his sources? We’re on the Internet. You can look it up.

Any estimate of how long you live with ALS is probably an urban legend. In fact, I have recently learned of one woman with bulbar-onset ALS who is alive nine years later. Which is longer than I’d planned to live even without ALS. And look at Stephen Hawking.

So ... in many places, you’ll read that if you get ALS you’re going to die in ________ (fill in the blank) months/years, but all estimates are basically a bunch of hooey, because some people go fast and some people go slow, it can all speed up or slow down, and no one can predict who’s gonna do what when.

Which doesn’t stop anybody from predicting who’s gonna do what when.

But, just this once, I will make an exception to my no-citation rule, in this case concerning the “18 months to live” statement in my previous blog. In addition to what I learned in “Bulbar Onset 101” in my Google School of Medicine studies, I do have another authoritative source, although this particular source is probably hooey, too. In fact, it may be the hooiest of them all. Neurologist No. 3 (out of seven so far), told me a few months ago that I couldn’t possibly have ALS, because if I did, “You’d be six feet under by now. Heh-Heh.”

That is a direct quote, including the Heh-Heh. He chuckled complacently when he announced that, apparently expecting me to appreciate his wit as much as he did. Leading me to conclude that some neurologists are just sadists who couldn’t get into dental school.

(I tried to work “sleazy trial lawyers” and “crooked accountants” into the last sentence, too, to see how many professions I could insult in one breath, but couldn’t manage it.) (Not that I’m counting sadism as a profession. I think of it as more of a hobby, albeit one that is favored by some professionals more than others.)

Now, Neurologists 5, 6, and 7 have agreed that it is ALS. (I’ll leave out Neurologist No. 4, the nicest doctor I’ve ever met, who thought it might or might not not be ALS, he wasn’t sure . . . he thought maybe something else was going on . . . and admitted right out loud that he wasn’t sure! He goes straight into the Neurological Honesty Hall of Fame.)

Here’s how it works (or in the case of ALS, doesn’t work). Motor neurons in the brain that control muscular movements slowly die. The muscles they control grow weak and eventually atrophy. Most cases of ALS (75% ... you can look it up) start with the limbs. Your hands (or arms, or legs) grow weak, and you gradually lose the ability to use them.

After a while, usually a few years, the process spreads to what is called the “bulbar” region . . . the area of the brain that controls speech, swallowing, and breathing . . . and this is when things really go south. You can’t swallow liquids, you choke a lot, and sometimes when you do swallow, stuff gets into your lungs instead of your stomach, which is Not a Good Thing. You start slurring words, and eventually your speech become unintelligible. (This is where I’m at, and for those who know me, please no comments about how I was never too intelligible to begin with, so what am I complaining about.) Eventually, you lose your voice all together, then maybe you get pneumonia from the gunk in your lungs. Finally, the muscles that control your lungs stop working and you die.

So, the key to living with ALS is to keep breathing. (Actually, that’s pretty much the key to everything in life, isn’t it, when you get right down to it?)

This is why us those of us whose symptoms start in the bulbar region are expected to check out faster than those whose symptoms start in the limbs. We’re already half-way home. However, if you survive long enough with either type, it spreads all over your body, so it makes no nevermind.

Naturally, being medical, it’s a lot more complicated than I have indicated. There are upper motor neurons and lower motor neurons, and telling them apart is a lot like telling Sunni from Shia, which I can do if you give me a few minutes, but I always have to think it through.

The upper motor neurons control muscle atrophy, I know that, and the lower control other things, which I can’t remember offhand, but it doesn’t work the way you’d think it would work. (You’d think upper motor neurons would control the upper part of the body, wouldn’t you? Duh. But, noooooo. That’s much too logical for life on planet Earth.)

There are scads of diseases that mimic ALS, and some of them are treatable, so it’s important to figure out what you’ve got, in case they can do something about it, besides pat you on the head and say, “There, there.” I’m on my fourth diagnosis in 2½ years: first stroke, then Progressive Supranuculear Palsy (don’t ask) (actually, it has something to do with Mad Cow, and I’d like to once again remind you that I’ll make all the smart-ass remarks that are necessary, thank you veddy much), then Myasthenia Gravis, and now ALS.

Diseases that mimic ALS, in addition to the above, are Progressive Bulbar Palsy, Progressive Pseudobulbar Palsy, and on and on and on ... it’s all just one big mess up there in your brain.

Even neurologists can’t tell them apart sometimes. Sometimes, they just wait and see what you die of, then pretend they knew all along.

So, now you know. Run along and play.