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.

Put Your Own Baseball Metaphor Here

I thought the worst thing I would ever hear from a doctor was the time I was lying in the operating room in that blissful twilight state they put you in for a routine angiogram, and, in the middle of the procedure, I heard the surgeon say, “Oops.”

And I thought to myself as everything went black, “This does not bode well.”

That was two years ago. But in May, as I sat on the edge of an examining table ready to make a run for the parking lot after several hours of electric shocks and needle pokes all over my body, a neurologist topped it: “You’ve got Lou Gehrig’s Disease.”

Lou Gehrig’s Disease. Motor Neuron Disease (MND). Also known as ALS — amyotrophic lateral sclerosis: I can’t spell it, can’t pronounce it, and absolutely don’t want it anywhere near my person.

If I hadn’t immediately started bawling my head off, I would have explained to him nicely that I am not the ALS type. It is much too melodramatic and exotic for me. It clearly requires much more character and inner strength than I’m capable of dredging up. I’m more the heart attack type. Or the coming-in-10th-in-a-20-car-pile-up on-the-405 type. Or the leprosy type, for God’s sake, where your fingers and nose fall off and you become so yukky they make you live on a tropical island for the rest of your life.

If I absolutely have to die (and I always figured medicine would solve the death issue by the time it was my turn; in fact, I’ve been counting on it) I figured I’d die of something ordinary, quick, and painless. My overall plan had been to feel great for 70- or 80-some years then abruptly keel over. Or even better, get a great night’s sleep and just keep going from there.

I never anticipated getting something incurable, untreatable, lingering, and paralyzing, which robs you of your ability to move, speak, eat, chew, swallow, and breathe, but which—just for laughs—forces you to be completely aware of everything that is happening to you at all times.

Also, if I hadn’t been bawling so hard at that moment, while counselors patted my shoulders and piled informational pamphlets and inspirational books on my lap, I could have at least come up with the snappy rejoinders I thought of a few weeks later, when the shock started wearing off. Such as: “Lou Gehrig’s Disease? Does this mean I can finally hit a major league fast ball?”

(For those of you under 50, the disease is named for Lou Gehrig, a great NY Yankees baseball player and teammate of Babe Ruth, who contracted ALS at the pinnacle of his career, retired from baseball in 1939 when he began losing the use of his limbs, and died two years later in the state described above.)

(Actually, I’m not quite old enough to remember him either, but I saw the movie.)

But I digress. Every case of ALS is different, and the cause of it is a mystery. So, I’m not sure what all to expect, other than it’s downhill all the way, and ... as I accurately surmised at the “Oops” moment ... this does not bode well.

So, I figured I might as well join the rest of the human race and start blogging to kill a little time, since time is busily returning the favor.

The first thing I did when I got home from the clinic was jump on Google and check out Catholic funerals. My husband is a Catholic and I’m not, and I wasn't sure how it worked. I think he and I should be buried together, as I don’t trust him to make it through the afterlife without me there giving instructions. Turns out, the church does allow non-Catholics to be buried with Catholic family members, which I think is very nice. Whew! One down.

Next, I Googled crematoriums. What a rip-off. Nuff said. Nevertheless, it turns out I can afford to die, although just barely.

Next, I clearly needed to write my obit ASAP. I titled it, “ ‘30 Rock’ Fan Dies.” I thought maybe that might get Tina Fey to my funeral, or at least Jack McBrayer. (Alec Baldwin is clearly beyond my reach.) By the time I had typed about 50 column inches of my life story, however, I realized that publishing my obit was going to cost more than my funeral and maybe I’d better leave out the part about that snot in 6th grade who ruined my life for two weeks. Once I started cutting out the really good parts, it got pretty boring and the obit project withered on the vine.

Much as I am doing.

But with the essentials out of the way, I returned to my alma mater (not to brag, but I have an MD degree from the Google School of Medicine) and Googled “ALS bulbar onset,” and discovered to my horror that my life expectancy is in the range of 18 months, give or take 10 minutes.

And I am already at month 28 of my symptoms, way past my expiration date. This whole thing keeps getting better and better.

Then I realized ... OMG ... I’m going to die before “Lost” ends. I’ll never know how it comes out.

Shit.