Sunday, February 27, 2005

D+20: Slightly worse

Left and right grip strengths are 48 and 95 pounds respectively. Inhale volume is 4500 mL.

For a change of pace, here's my chart for one-legged balance duration. Keep in mind that I don't record any duration over 30 seconds. The "improvement" you see is all due to practice, I think. I may be stronger, but I still feel as clumsy. Maybe I'd feel much more so if we'd never done the ceftriaxone. Who knows? I do know that my speech continues to deterioriate, to the point where almost every time I talk to someone outside my family, I think they may notice the lazy tongue. I staggered into a lady on the street on my most recent date with my lovely wife, and apologized. She and her friends laughed a bit, perhaps thinking I was drunk.

The good news is my family came back last night.

The bad news is that its started raining hard, and the streets were gushing, and the ceiling in the dining room started dripping, and I had to put a big plastic storage bin under it to catch the water.

"Daddy, why is it raining inside the house?" asked my son when he got home.

The roofers came today, and they think they have found and fixed the hole. I looked at the hole when, despite my clumsiness and fear of heights, I climbed the ladder. I told them I was afraid of heights. And in a little back-patting manuever, let me say that I think it's manly to admit when you are afraid, and childlike not to. There was a little clonus on the way down, but otherwise I did fine.

The new dishwasher will come Wednesday.

All this happens just when I thought I was catching up on the house projects.

Tomorrow I am posting a naked picture of myself on the internet. Full-frontal nudity, stark and complete.

D+19: Harsh buzzer sound

Left grip is 47 (47, 47, 47), right is 95 (90, 89, 95) and inhale is 4590 mL.

My whole concept on Feb. 7 about calibrating the hand dynamometers was wrong, and it was only by coincidence that both the one the doctor owns, and the different type that I own, happened to read 44 on that day. However, my strength increase, as measured on his equipment, was real.

His method is to have you squeeze his dynamometer three times. But he has told me that's totally arbitrary ... he just does that to obtain a crude averaging. On mine, you squeeze once only. So the one-squeeze reading on his, when I got 44 as a sum of three tries, would be somewhere around 14.6. And no, his is not reading in kilograms, because that would come out to about 32, not 44. The units on his dynamometer are arbitrary (but highly reproducible).

I just happened to get a score that day of 44 after squeezing three times, and I was getting a score of 44 on mine, too. But the scales are different, and there doesn't appear to be any useful correlation.

I appreciate lessons like this, the ones that teach me to be humble, and cautious in my thinking.

...I started missing the family on Saturday morning. I have not gotten as much done on the house as I thought I would. But I have made some garish holes. Today I will tape plastic over the holes near the ceiling, put up the painted shelves, and return the furniture to its former locations, to make everything as normal as possible for the kids when they get back. Monday the roofer comes. We're going to have an addition built onto the house and it will join through that exact rotting wall. So maybe we can have them make that part of their mission.

Oh by the way, the dishwasher absolutely and completely died. It stopped working. There are no control panel lights, even when plugged in, and that trick of jamming a screwdriver into the door mechanism no longer works either. This one is a lemon. It's a Maytag. I'm going to go to the mall and order a new dishwasher this morning after breakfast.

Saturday, February 26, 2005

D+18: Bolus

Does this look like a problem to you? Does this look like a problem? If the lathe behind the West wall is rotted and black, still wet? Is THAT A PROBLEM?

No, not when you know as much about house repair as I do. But it does mean a lot more work than I had planned (this was a painting project, recall).

Later in the day I will blog up my meeting with the neurologist, detailing the objectives, timetables, and jumping-off points of Operation Bolus.

Today's numbers are 51 pounds left grip (48, 51, 51), 96 pounds right grip (91, 96, 94) and inhale volume of 4580 mL. I seem to be slowly working out of the lung problems caused by the recent cold and cough. The cold symptoms are gone but the cough lingers, as mine always have since Prague in 1996.

Remember the 100-yard dash metric? That one didn't prove so useful. I tried it about at one week, and again one month, after my diagnosis, and I wound up giving myself a cramp that hurt by leg muscles. So I stopped. But now that I am even more disabled (yes, I have used that term a couple of times to describe myself), I had this hunch that my 'running' would be so impaired that I was not likely to pull any muscles. Fortunately, I was right.

I walked out to the track and marked my end points. On the first 'sprint' I crossed the line in 34.14 seconds. It is my impression that my gate has improved, or let's just say adjusted, since when I last wrote about not being able to run properly. That time, my left leg was so stiff that my body compensated by swinging it out to the side in a wide arc (so as not to let the toe catch on anything). I didn't plan this, the body has its own logic. This morning the left leg sure was stiff, but it stayed in line, Granted, there were a couple of strides that were just skipping on the right leg, and there were several times towards the end that the toe scraped on the dirt track a bit. But the leg stayed in line. Having accomplished that one, I now thought I could do better. I had learned that the trick was to give up on bending the left knee, and just jerk the left leg up as quickly as possible by using the hip muscles. This time there was no skipping, only a couple of toe drags towards the end, and I crossed in 30.02 seconds.

Both times, I pointed skywards with both index fingers, Like Joe Montana used to do after a nice touchdown pass. Only, my left index finger doesn't straighten, so it was kind of a hook and point gesture. There were high school boys in the next field, warming up for baseball. They're hale, fit, healthy, and destined for lives of alcohol dependancy, taxation, television, and surly children.

Mission accomplished.

I walked home, past a teenager with bandana low over his eyebrows. He was shooting baskets, and this is the thing about basketball, no matter what barriers of race, age, or social category separate you, on the court, any basketball player will accept any other. Except if you're a woman, in which case you gotta carry a little bit more ganas. But I know several women, (my lovely wife among them), who have no problem taking to the court and baffling teenage boys. I have video to prove it.

So as I walked past, I asked him if I could shoot one. He passed me the ball, which I fumbled, and did that skip-run trying to retrieve before it hit a car. I fumbled it some more after I recovered it. "I used to play all the time," I said, putting it in terms I thought he would understand, "until I messed up my leg." I put in a close backboard shot and was grateful it went in, as I have a notable tendency to miss from any distance.

On the way home I crossed with a blind woman using a seeing-eye dog. That's something I didn't even consider putting on the table during the Amputation Derby. I think neither she nor I would want to trade. However, if I were progressing faster, as some of my fellows PWALS are, I'd probably leap at the chance. And I don't want to sound smug, talking about my 'sprint.' It's just that I have been given to pilot this one body, and this one life. So I have to treat it on its own terms, and often that means its frame of reference becomes the only frame of reference.

My technical copy of Mitsumoto's "Amyotrophic Lateral Sclerosis" arrived yesterday. Good.

Operation Bolus

I went to see the local neurologist yesterday. I really like this guy. We planned Operation Bolus. This operation is based on the intelligence that penicillin, the father Abraham of beta-Lactam antibiotics, was examined in the Rothstein study, along with ceftriaxone, and was found to be effective in increasing expression of GLT-1, which clears out the used glutamate which is thought to kill the motor neurons. But, as Rothstein notes, penicillin has trouble crossing the blood-brain barrier.

And, as you know from following Dee Chiplock's story, ceftriaxone is expensive. And as you can intuit from the press release there is a pretty penny to be made if the human trials show that ceftriaxone is effective against ALS. The patent claims have already been staked:

The newfound ability of ceftriaxone, other beta-lactam antibiotics, and other classes of drugs to activate glutamate transporters and to protect nerves, and the drugs' potential therapeutic use in a wide range of neurological and psychiatric conditions, are covered by patent applications held by The Johns Hopkins University and licensed to Ruxton Pharmaceuticals, Inc.

Yet, as my doctor noted, penicillin, if given in large enough doses, does cross the blood-brain barrier.

The case in point apparently is the disease syphilis which attacks the brain, and in which (forgive me if I misunderstood), even advanced cases can be treated by large doses of penicillin. This means that an effective dose does reach the brain.

And, as I noted, it's passed out of patent now. Nobody owns it. My doctor says it is "as cheap as water."

I doubt that the patent, if granted, would preclude doctors from treating their patients with penicillin for ALS. If it did, 30,000 people would get acne over night. Penicillin can be used to treat pimples. I have a prescription right here to prove it. He gave "folliculitis" as the reason. And in fact, I do have a big pimple on my cheek. Indeed, I have noticed more than one in the past few weeks. It's a severe problem that worries me to death, and so I'm glad my neurologist is on top of things!

I have no objection to a brilliant researcher like Rothstein making money from his excellent discoveries. The American pharmaceutical and health delivery systems are deeply unjust, and need to be changed, but a way must be found to reward someone like Rothstein.

That said, I wouldn't mind a cheaper way to excite my expression of GLT-1.

So in Operation Bolus, I will start taking large doses of penicillin, one gram twice a day. The objective is to maintain my inhale volume, grip strengths, and one-legged balance durations at current levels. The timetable is to begin some time in early March, and continue the operational tempo indefinitely -- 20 days or more. The jumping off point -- and here is the best part -- is my mouth. Yes, I'm going to take oral penicillin. No needles!

"Bolus" means, in this case, a big pill. I'm sure the pill will be reasonably sized, so I guess it's meant to convey the concept of the dose being hefty.

I think it is time to analyze the results of the Ceftriaxone. It's now 18 days after the final dose, and I would have expected to see a downward trend by this point. The study mentions positive effects that "persisted for at least seven days in vitro." That language is ambiguous enough to allow for indefinite benefit, but I take it to mean that seven days is about all you can confidently expect. And while there is plenty of bouncing around in my metrics data, I am surprised that left and right grips are both at record highs. Touch wood, we seem to be in a plateau right now. I credit my slow-mover history as the reason I have not yet seen a decline in the post-ceftriaxone metrics. Maybe I have somewhat good GLT-1 expression, for an ALS patient, and my system is almost keeping up, at the moment. Maybe, maybe, maybe. Who knows? I mentioned to the doctor that as a systems debugger, I have half a mind to deprive the subject of treatment until we can see the data change. He shook his head and told me that you don't want to mess around when your life is at risk. I agree. Who cares if I don't get good data, as long as I'm around to complain about it!

We're holding the ceftriaxone in reserve and will commit the reserve if opposition forces threaten a breakout. There are rumors they'll land at Normandy, but we are convinced that their real objective is Le Havre or ports to the East.

I have mentioned before that I often think of my struggle against ALS in terms of a war (when I'm not thinking of it in terms of computer programming), and I am a student of World War Two history, and that, unfortunately, means being the Germans, because I am on the defensive. I don't like being the Germans, I'd rather be the Americans. And I hope the day will come when we go on the offensive against ALS. And then I'll switch sides, and be the Americans.

Friday, February 25, 2005

D+17: Bee

Left grip is 51 pounds again, right is up to 95 pounds, and inhale has improved to 4550 mL. I saw the neurologist today, and I'll write it up for tomorrow's post.

Yesterday after dropping off my adorable family at the train station, I picked up all the fallen fruit from under the tree (as instructed by my lovely wife), ordered a copy of our utility bill so that we can prove we live where we say we do and get my son into kindergarten, sent my metrics to my neurologist (who I'll see today), cleared all the furniture and kid stuff out of the dining room, removed the unpainted shelves from the wall in there, drove the antique medicine cabinet mirror a long way to the place that will re-silver it, painted the shelves I took down, scraped some peeling paint from the wall (not the moisture-stained area), and painted the peeled area. And I took a walk and ate food, put out the trash to the curb, and bought two Whitney Houston songs via iTunes.

I also had a couple of minor water gag moments. I have decided to quantify them as minor, medium, and major. But I will only log one choke, the largest choke, for the day.

But there is a problem. While scraping paint off the wall where the stain is, I poked right through the wall and exposed this little beauty. It's a hole in the interior porton of the wall, caused by water damage, probably years ago before they decided to sell the house. There is a new, good roof on there now, but I get the impression that they ignored house maintenance for many years.

Thursday, February 24, 2005

D+16: Our Moolah

One of the funny things about George W. Bush talking about not letting the Iranian "Moolahs" get a "newkular" weapon is that his Texas bumpkin style is feigned. He's a Mainer who went to not one, but two, elite East Coast colleges. He's about as Texas as Barbra Streisand.

Left grip 40 pounds (40, 38, 25), right 82 pounds (82, 75, 80), inhale 4300 mL. Everybody has an off day now and then.

OK, I'm going to violate the testing protocol, which only allows three tries, and post numbers replacing the ones I posted at first today. I "cheated" by testing myself again later in the morning. Left grip was 51 (51, 49) and right grip was 85 (85, 85). Inhale was 4500 mL. Give me a break, it's not like I faked evidence about Iraqi weapons of mass destruction.

I think the reason for my low scores was that my lovely wife is taking the kids out of town for a few days, and the change in the morning routine threw me off. I have lots of painting touchup to do while they are gone. I look forward to it.


Wednesday, February 23, 2005

D+15: Corner

It's official. Yesterday I turned the corner on the cold, felt good energy all day, no afternoon crash. I expect the episodic coughing to go bye-bye soon.

Left grip is 48 pounds (48, 45, 45), right grip is 90 pounds (82, 89, 90), and inhale is 4490 mL.

The average left grip from 2/9 onward is 47.46. The average right grip in the same period is 88. Average inhale in the same period is 4521.33.

And just so that y'all can't accuse me of focusing only on the data that I like, while ignoring the data that does not fit, I hereby present you with the right grip strength from 1/10/05 onward. The period in which the data vanishes is during the infusion week when the needle was in my right arm and I did not test it.

If we use the average of 88 pounds since the end of infusion, we see that the score is down almost seven percent since 2/2/05, and down 12 percent since 1/10/05.

Oh well. That's data, and I'm not going to duck it.

I like it that the breathing is the same as before the drug, and at one point spiked to 4750, and remains at 4500 despite my respiratory infection.

I just feel better and more durable than I did before. And I'm the only customer.

Tuesday, February 22, 2005

D+14: Dandelions

It would be hubris to declare victory or even supremacy in the war against dandelions, but I must say that after the massive campaign in the back lawn a few years ago, they rarely show in that yard. And the policy of digging when they flag themselves with a flower seems to be working in the front as well. Some weeks none show themselves at all, and usually it is only two or three week during the wet season. I dug up three a couple of days ago. None have showed since. Back pat. If you had seen the miserable condition of the lawns about a year after we moved in, you would think that today's balance was a good one. I think maybe there is some parallel to maintaining your health. Do what you can, don't go overboard, appreciate what you have. Of course, my case of ALS is lucky -- very lucky. For most people it's not a question of containing the dandelions in the front lawn, it's an issue of how soon the sink hole will consume the house.

On the other hand (ha!), my left thumb no longer does the "thumbs up!" sign. That appears to have happened after the ceftriaxone, but not, I think, as a result of it. I assume that the disorder has several pathways to mess you up, and glutamate is just one of them.

I think I've turned the corner on the cold. I had phlegm this morning, and I'm not all better, but after my shower I was not so exhausted that I just had to lie down for half an hour, like I was yesterday and the day before. Left grip is back to 49 (43, 49, 40), right grip is at 89 (89, 84, 85), and inhale is 4490.

I've developed a new metric, to measure duration of one-legged balancing. I recall thinking about this at the time I was diagnosed, but at that time I felt that it was too tedious: I was able to balance for such a long time on both legs, that waiting for failure would be too much. It did not occur to me to set an upper bound, a point at which the duration of balance would be judged "good enough." Well, now I have settled on a method of taking this metric: Using any footwear you like, balance on one leg until the other foot (or your hand or the back of our skull or what have you) comes in contact with something else. No balance poles or other balance aids are allowed. The maximum time to record is 30 seconds. Record this as the duration if you exceed it. You only get two tries for each leg, not three. I wish I had been doing this all along, because I'd like to have data to compare to now, the post-ceftriaxone period. The reason is that I have perceived that my ability to balance on my left leg has improved. It's currently 5.5 seconds on the left and 30 (or more) on the right.

The chiropractor says my scoliosis has not changed since last time. She did notice tension on the right near the hip. I notice that too, when I push my son to preschool in the jogger stroller. I think my right side is doing more work. So I have tried to make the left side do more.

Monday, February 21, 2005

D+13: Water choke

Left grip is down to 44 (43, 42, 44), right grip is 86 (86, 85, 80) and inhale is 4500 mL. The cold is worse but still seems semi-contained.

I'm under the impression that I do a lot less choking on water than I used to. My lovely wife agrees. No, I'm sorry, I have no been keeping track of how often this occurs, so I can't say whether it changed after the ceftriaxone. But I feel that it did. I lay it to a positive effect of the drug. I propose that from here on out, I will keep track of days in which I choke or gag, no matter how mildly. I used to wind up choking and coughing at least once a day, sometimes at every meal. Now I notice that choking is the exception. I wish I had the data going back into the past. But I'm going to assume that it was every day. Let me be clear about my standard going forward: Even the slightest gag or spit-back will be marked as choking, even if I don't wind up hacking. I'll treat it as a bulbar malfunction and mark it as a choke day. Yesterday, for example, I went the whole day without any kind of gag or splurt -- despite the rasping cough I have from the cold germs that are attacking me. That's pretty remarkable.

But John, who has ALS, cautions:
My initial symptoms were slurring and stumbling while talking but they developed into progressively more severe choking episodes both while eating and drinking any fluid, but water was the worst. At one point, I really thought that I would succumb at some meal when I lost my concentration for a moment while swallowing. Then, erratically, the choking episodes have abated over the past couple of years to the point that now I rarely choke and even less frequently choke severely. Lest you think I am improving overall, I'm not. Limb weakness has been steady and relentless. The point I am trying to make is that choking/swallowing problems are unpredictable and if you do find yourself improving in that area, not to give too much credit to the ceftriaxone.


Sunday, February 20, 2005

D+12: Practical life skills

We are weighing whether to send my son to a supremely excellent montessori early years program when he is ready for kindergarten, or to the local, excellent, public schools. I like both options and am very impressed by the private school, and I mean no disrespect by the following, but I do get a smirk from thinking about telling the Montessori: "I want my son to be proficient in belt fighting, as well as some day to be able to support his family by cooking up methamphetamine in a bathtub. And I doubt your school will offer him these curricula."

Cold is getting close to miserable, some phlegm going on. Yuck. She may be the most charming two-year-old in history but she still woke us up at 5:00 AM. Ow.

Left grip 48 (45, 48, 47). Right grip 87 (84, 81, 87). Inhale 4500 mL.

Saturday, February 19, 2005

D+11: Harbinger

Here's one of those ALS harbingers, which has occurred to me now because my throat is being attacked by germs and my voice is raspy, or brassy, or whatever. I recall that several times in the year before I was diagnosed, my voice became similarly altered. It was deeper, and stayed that way for a long time each time I got sick. Even after I got well. I noticed it, but I didn't think anything of it, and I didn't think of it as new.

But it was new. That didn't used to happen when I got sick: It didn't used to stay that way for so long after I got well. That was also the period of time in which I was constantly choking on the water I drank, when my buddy made a crack about it.

My theory is that my throat muscles where changing, and I didn't know it, and the persistent brassy tone was a harbinger.

Left grip slipped down to 45 (45, 45, 42) and right grip down to 88 (88, 84, 84). Inhale volume is 4500 mL. The cold is making progress but is still contained.

Friday, February 18, 2005

D+10: Raw

When our daughter woke my wife up (again) at 6:00 AM, my throat was raw and it hurt to swallow. My lovely wife went to absorb the blasts from a distraught two-year-old, with a perky and pesky five-year-old adding energy to the dawn mix. She let me slorp in bed for another hour, until, now when I get up, the throat is merely froggy and not hurting.

If I had to face what she faces every morning I know I would be sneezing and hacking and running at the nose right now. This is probably the most important thing anyone does for my health.

Last night was kindergarten prep night for parents. My boy is moving up to the big league.

Left grip is down to 46 (40, 41, 46) and right grip down to 89 (80, 86, 89). Inhale maintains at 4400 mL. I blame the cold germs that are attacking me.

The battery on my iPod appears to be whack. I've been charging it for two days and it stays at about one-third full.

Thursday, February 17, 2005

D+9: Duro

I feel more durable and vital than during the post-Thanksgiving period of my decline. There's plenty of reason not to: my deteriorating speech, and the two brief and slightly painful spasm cramps I've gotten in my left forearm and forefinger this past week ... even my current cold, which is still not out of control but which is making its way. Plenty of reason not to feel durable and strong, but I do.

The left grip hit a new record at 51 (42, 51, 50.5). The right grip remains at 91 (91, 91, 86). Inhale volume is 4400 mL and the cold germs are making progress. But if I handle it properly, I may avoid being miserable

Wednesday, February 16, 2005

D+8: Pop!

The knuckle cracking I adopted under the Change Is Good doctrine has not cured me of ALS. And it does annoy my lovely wife, so I have decided to try to quit. When I quit in 1997, I used mental imagery that I was being fined a penny every time I did it, and I tried to imagine that the finger in question was now hurting, ow! hurting so badly! But now I am unwilling to use negative thoughts that involve me making believe my body is being hurt. So I have adopted the approach that if I happen to do it out of habit or while not thinking, that's OK, but I just should never do it on purpose. It's been about a week, and so far it has resulted in greatly reduced knuckle cracking, though I won't feel that I am in recovery for many months. Knuckle cracking, like chess, is always there, waiting to suck you back in.

My two-year-old daughter's water-fascination phase has ended. She's been willing for some time now to wash her hands and be done with it. We are now deeply in I-need-a-Band-Aid territory. Everything requires a Band Aid, and there is a spinoff effect that my five-year-old son wants one too.

The mysterious origin of the germs attacking my lovely wife and I is solved. On the night of the 13th, we had some friends of our kids over. The little girl had a cough like the air horn on a truck. My son already had a cough that was getting there too. But I didn't feel a problem in my throat until two days after I ate next to the little girl. She let out a few immense ones while my food was next to her and I was out of the room. Maybe she didn't lift up my chicken and cough under it, but she might as well have. I don't feel miserable yet, I just have a puffy glottis. I may fight it off. We Shall See.

I am going to postpone breakfast this morning until after I have some blood drawn for the latest tests.

...The blood draw was fine. I should know in a week or so about the very long-chain fatty acids. My left grip maintains at 47 (47, 46, 44) and my right is up a tick to 91 (89, 85, 91). Inhale volume is 4490 mL.

...I just got back from the acupuncturist. I asked her about the Lexapro and she said she totally supported my reluctance to take it. She said it is the end molecule of Prozac, and alters your personality, and kills your sex drive. She said it's like using an elephant gun to kill a fly. But when I said that I had the samples and was curious to try it and see if it has an effect on the inappropriate grinning and laughing, she said go ahead and try it. See what it does. She just has qualms about people being on antidepressants for the long term. I do too. I mean, anything that can influence my nervous system in those two ways is far from "very selective." Poison. Maybe not just poison, but maybe even metaphysically dangerous. Then again, I have confidence that I can go through this and the "I" will remain unscathed.

Also, she says she thinks my liver is improving. But that was after I told her I had stopped taking the riluzole.


Tuesday, February 15, 2005

D+7: Valentines Date

Today's left grip is 47 (47, 45, 47), right grip is 88 (88, 82, 85).

I have also included a chart comparing the right and left grip strength over the period from 2/9 to 2/15, inclusive. What I find interesting is that on five of the six days after 2/9, the left and right grip strengths moved in opposite directions, (if we consider a result that stays the same while the other moves in either direction to be "opposite").

Inhale volume is down to 4400 mL, but I have a raw throat and my lovely wife has a sore throat, so I may be fighting off something. Here's a chart of inhale volume covering roughly the same period as the grip strength charts. It starts on 1/14/05 rather than 1/10/05.

So my lovely wife and I went to the checkup at the ALS clinic yesterday, and these are my notes. Sometimes people find my blog entertaining, and I am gratified by that, but I do still use it to keep track of the tons of information I gather. This entry is just such a grab-bag, and I'm sorry if it bores you.

The thing they watch most closely is your Forced Vital Capacity, or spirometry, which is a measure of breathing capacity. My score yesterday was 92 percent of what would be expected for someone just like me who did not have ALS.

When I was diagnosed on 1/28/04, it was 94 percent. Then on 4/5/04, it was 97.3 percent. So I am doing OK in that area, as they don't start to worry until you hit 86 percent or so. At least I think that was the number, I have sent an email to verify. They also said you could have a 6 percent variation and they wouldn't worry. I think that may be the speech they give to people who get low scores.

I talked to them about the riluzole and my bilirubin, and they said that's no reason to stop taking it. They also said it's not a burden on the liver. I asked to hear about the exciting data from people in Ireland and Britain taking riluzole, and whether they are showing better trends, and I was told that nothing has been published, and it's all informal. My own personal bias is that this response proves that riluzole is worthless.

Riluzole requires a blood test for liver function every month for a while when you first go on it, and then every three months during the first year. I spent a year taking that drug without getting my liver function tested at all. Yesterday they gave me a prescription for a blood test for liver function, bilirubin, and cholesterol.

In a fun twist for you science fans, the doctor also decided to have me take a blood test for "very long chain fatty acids," which she says is a definitive test for whether you have an X-linked, rare, hereditary condition that can mimic ALS, called adreno-myeloneuropathy. She said it's a real long shot. But, she said, the syndrome comes with a long life expectancy. I didn't readily find a good definition of it on the web. And the Merck Manual we have says that it mostly strikes adolescent boys. They go spastic and blind. No cure is known. My vision in kindergarten was measured at twice as acute as most people, and when I was in my mid-30s it was tested at 20-20. So I may be going blind. I've been told that I look like a kid, but let's face it, I'm a 42-year-old dad, not a boy. But since the blood test is definitive, I'm willing to try it, if it means I don't have ALS. This raises the topic of Malady Poker as a follow-on to yesterday's Amputation Derby, so ponder that on your own time, because in this post I have to finish making my notes.

The factors she said made her think I might have adreno-myeloneuropathy are:

  1. you are a skinny little bastid, aren't you?

  2. high cholesterol ("lipid disorder")

  3. the tingling and numbness I reported at diagnosis time

  4. visible demylenation in my MRI

She touched my left and right feet and lower leg with a pin, and asked me to say when it felt like a pin. On the right side the pokes she did to the foot felt sharper than going up toward the knee. However on the left leg both regions felt about the same; My impression was that my leg was as sensitive as my foot.

She also twiddled my big toes on both feet and asked me if she had moved it up or down. I believe that I called each of those correctly. After we got home, I asked my lovely wife if she saw any trickery, and she said she saw none.

It turns out that the Mitsumoto book is technical in the first edition, but the second edition, the one we got, is all geared towards patients coping.

I asked the doctor how long I might be able to stay out of a wheelchair, given my trajectory so far. She said the predictive information she has is geared towards survival, but not ambulation. Somehow or other, I did not ask her to predict my survival.

I asked them about whether they'd be involved in Rothstein's upcoming gene trial, and they said no. When I asked them to try to become involved, so that I could participate, they said it was unlikely.

Apparently they will be doing a ceftriaxone trial, and more details should be available by April.

They had this really cool, giant poster on the wall about a patient-driven, horizontal registry for collecting information about people with ALS. But it had no URL on it. We asked practically every person who talked with us if they knew the URL. None did.

They gave me a prescription to find a physical therapist who will show me some stretches. I was told to ask for neck and head exercises to help with the disarthria (difficulty speaking). I was told to ask for someone experienced in rehabilitation with neuromuscular problems, and not just accept any old P.T.

They also gave me some stretches to do, and pitched yoga really hard.

They said that rather than take 1000 I.U of vitamin E twice daily, I should take 400 I.U. only once daily. Apparently there is some research showing possible problems with taking so much E.

The dietitian likes my diet, says it is very healthy (I can't help it, these are the foods that appeal to me). But she said I should eat more, take in more calories. She suggested soy milk, and fruit juices, and bigger portions. Plus plenty of animal protein. Yeah, you know what I'm takin' 'bout. Dead chickens! Also, dis the chickens some more by adding eggs whites to my daily egg, which gives you protein. There is the karmic hit, though, of pouring that egg yolk down the drain. Yeah, I know, I could mail it to a place where they need it, but I fear the response from Pyongyang.

The speech therapist said that my sense of a sudden progression in speech problems in the last few months is not what I think it was. She said that in her experience, the muscles involved with speech decay at a constant rate, and when they cross a threshold where the problem becomes noticeable, people think that it just suddenly happened. The analogy she made was to water boiling. The temperature gradient is constant, but if we were just observing with our eyes, when we saw the bubbles, we might think it was a sudden change. It feels like our speech is in a plateau that suddenly changes, she said, but the deterioration is constant. My speech rate is 189, and on 1/28/04 it was 198. She said I'm holding up very well as far as speech goes, and that I'm 100-percent intelligible. She also said there are no exercises you can do to strengthen the speech muscles; you just stress them when you try to do that.

We also talked with someone about Social Security Disability benefits. Check that form they mail you every year that has your earning history on it, it might tell you what your benefit would be. There is some maximum. When you sign up for Medicare, opt for part A and part B, which costs $77 per month. Medicare can cover medical costs but not prescription drugs. COBRA seems to mean that you can continue your health insurance for 18 months after your employment ends, if you pay the full premium, before they dump you, and it seems to mean that you are by law allowed to shift onto some other luckless insurer, but they can raise the premium a huge amount, like 150 percent.

Again, welcome to America, where the system works perfectly ... if you're rich.

The people from The MDA and ALSA were great, as usual.

We also talked to the doctor about the disinhibition (which they call "lability") that makes me laugh when I'd prefer to be serious (e.g., when giving a stern lecture to a five-year-old). They gave me four weeks worth of free samples of a drug they said is very good at controlling that problem, and said that I should begin seeing results in two or three weeks. It's called Lexipro. It's an antidepressant related to Zoloft. I don't have any mood problems (other than being terminally content), but this one has a "very selective" effect, the doctor said. I'm personally very leery about antidepressants, because I'd prefer to be who I am. Yes, even if I were depressed. Oh yeah, the doctor said it also has one other possible side effect: delayed ejaculation.

It was Valentines Day.

I may try it. And I'm very curious to see if it works.

Should I try Lexapro?


Free polls from


Monday, February 14, 2005

D+6: Amputation Derby!

Here's a fun game: what body part would you be willing to part with in exchange for being thereby cured of ALS?

You might think I'd give up an arm and a leg quite happily so that I would not DIE! But people are always trying to get the best deal for themselves, always scheming and calculating...

See, I have this gut feeling, perhaps totally foolish, that I will live long enough to witness a treatment that will stop the progression of the disease.

So, since a stop-cure is coming anyway, why lose a foot over it? OK, actually, maybe losing a foot today would be worth it because, who knows, in five or 10 years when the cure comes around, I may no longer be able to stand up. So yeah, in that case, it might be worth it.

But in this game, you don't get to make conditionals like that; you don't get to see the future. You just have to place your bet, slap that body part on the table, and await events.

So I'd give up a foot (the left one), because they have great prosthetics these days. Or the externals of both ears, because that's just cosmetic, and I'd still be able to hear just as well even when wearing rubber lobes.

Hands are a different matter. Not willing to lose a hand. Both pinkies, sure. One of the middle fingers, OK. Thumbs? No. OK, maybe the left thumb, since that hand is pretty messed up anyway.

Hey chubmaster! In this game you are not allowed to treat adipose tissue as a "body part!"

Facial disfigurement is pretty disturbing, so I'll just give you my left leg below the knee joint.

But you have to play on the assumption that some mysterious entity will reject your bid if it's not high enough -- and you don't get to make a second offer.

A couple of years from now, if I'm lying on my back, unable to move or speak, I may wish I had been willing to pay more.

But if I'm riding a bike while talking on my jaw-phone a couple of years from now, I'll be feeling pretty smug.

I hope that this kind of flippant post makes clear to you readers what a lucky case of ALS I have had so far. Though it appears to be the same syndrome, it attacks us all in different ways and at different rates. Before those motionless people listening to the ventilator breathing for them, I stand as a complete newcomer to this condition. A billion microscopic dominos now clicking on their run will determine whether, and how soon, I become one of them, laughing at, or probably just ignoring, the bravado of people such as I am now.

We Shall See.

Left grip strength today is 47. My lovely wife and I are going to the ALS clinic for a checkup, so I'll have more numbers for you later.

Sunday, February 13, 2005

D+5: Crib take-down

Yesterday in the morning, before nap time, I disassembled the girl's crib and stowed the parts in the laundry room. She napped in her big-girl bed just fine. When she woke from her nap, she proudly said "I got out of bed!" First time she's had cause to say that.

My lovely wife and I are slightly sad (No more crib! Where is our baby!), but also proud of our little girl. You have to get used to these loss-gains when you are a parent. They just keep growing, and there is no holding them back.

As I've mentioned, my ALS disinhibition usually takes the form of laughing or grinning when I take something seriously. Especially annoying: precisely because I take it seriously. But yesterday it took the form of uncontrollable crying. I was sitting on one couch, watching my son play with trains, when my daughter got on the other couch and crawled over to the arm, which she draped herself over. Way too far over. I shouted her name, and "No!" Her head hit the floor first, those tiny little hands unable to stop her. She flipped on her back and looked at me for a moment as I crossed the room to her, then started to cry. I cried some too. That seemed natural. Then, a few minutes later, when I went upstairs to relax by sorting some laundry, the sorrow of the incident swept over me, and I wound up on the floor sobbing and keening for several minutes, clutching kid clothes in my hands. That part felt out of control. I don't regret it though.

It's been 13 months since diagnosis. The left grip is back "down" to 45 (42, 45, 40). Compare that to 33 and see if you think it is "down!" The right grip is down to 82. The inhale volume is back down to 4500 mL, where it was when we started the ceftriaxone. Maybe it's the mood, the dream I had last night, or yesterday's emotions, or today's breakfast, or just a normal swing in energy levels. We need more data before we have a trend.

Saturday, February 12, 2005

D+4: Timeout

I set up the "youth bed" for my daughter yesterday, and she napped in it like a pro. She slept in it perfectly last night.

Yesterday I also played some chess, and made dinner for my family while my lovely wife took the kids to a play date.

One of the things we do for my son is track his behavior on a chart. He has three metrics: Whether he woke us up in the night, whether he gives his teachers his attention at school when they need it, and whether he sits for dinner. Each metric is marked by a happy or sad face. If he gets four "perfect days" in any seven-day period, he gets some special reward, like an ice cream with mom, or a new battery in his toy train. The system has been in place for many months now. For about a week and a half he seemed to be testing the system to find out if he could get a reward by having no perfect days. The results came in, and then he started getting lots of perfect days. The little train has a battery in it now.

We give him timeouts when needed, and his little sister has always wanted a timeout of her own. She used to get up behind the gate on the stairs that we use to time him out behind, and beam happily while proclaiming that she was "timing out." The other day I had to give her a timeout for real, because she was playing with the gas knobs on the stove. It was her first real timeout, I think. She looked so proud it was as if she were graduating from Vassar. She set her face seriously, but her joy at this graduation was immense. When the timeout was over, she asked for another one.

Left grip is back up to 49 pounds (ALife: 45, 49, 45). Right grip is 90 (90, 87, 86) and inhale volume is back down to 4600 mL. I anticipate there will be daily ups and downs. We need to watch the overall trend.

Read it now, before the Yahoo shuffle makes the link break.


Friday, February 11, 2005

D+3: Bilirubin!

The Aventis site warnings on riluzole say:

"Baseline elevations of several LFTs (especially elevated bilirubin) should preclude the use of RILUTEK."

I have elevated bilirubin, and my blood tests have shown it since my troubles began. The people at the ALS clinic had my blood work results when they prescribed this drug to me.

Before I read the above warning, I did a little experiment...

My breathing score was 4750 two days ago, and yesterday, the morning I skipped the riluzole, it was 4500-4600. So I wondered if maybe the absence of the drug was the reason, and at 3:30 PM I took 100 mg (a full daily dose, usually spread to morning and evening), to see if my score improved.

You could hypothesize that the shortness of breath was anxiety from drug separation. Again, I wouldn't argue with you, because that's a perfectly valid argument. I just don't personally feel that it's true.

The dose did not improve my score at all, so we can rule out lack of riluzole as the cause. OK, if you're Aventis, you can't, but I can, for my purposes.

The blood drawn on December 31, 2003 showed this level of bilirubin:

bilirubin, total: 2.3, normal: 0.0-1.5 mg/dL

The blood drawn on December 19, 2004 showed this level:

bilirubin: 4.5 MG/DL, normal: 0.2-1.5

And, to give props to non-Western medicine where props may be due, when the (English-fluent) new acupuncturist was holding my wrist in that weird way, taking my different-kind-of-pulse (not the normal one), she said I had trouble in my liver. She's never seen my blood work. So maybe it was a lucky guess, or maybe she hit the freakin' nail on the freakin' head.

In other news, I'm pleased that in a short period of time, I organized the acupuncture, probiotics, glyconutrients, ceftriaxone, and the hand dynamometer. And yesterday I finally, at last, at last, filed the forms for Social Security benefits.

Today's metrics are inhale volume 4700 mL, up 100 from yesterday, and left grip 47 pounds, down three pounds from yesterday (ALife: 45, 45, 47). By the way, right grip is at 91 pounds, which is up six from yesterday, yet down two from the day we started the ceftriaxone, and down nine from 1/10/05. ALS has had a much worse effect on the left side of my body, so I assume the variation due to therapy will be greater there.

Good news: No neck muscle twitching since Friday Feb. 4, two days into the ceftriaxone therapy.

Thursday, February 10, 2005

D+2: Riluzole reaction


Here is the left grip strength chart so far. Today my left grip strength is at 50 pounds (ALife: 50.5, 47, 48). Compared to 33 pounds (2/2/05), that's 51 percent stronger. Compared to 38 (1/10/05) pounds, that's 31 percent stronger. Do I feel stronger in my hands? No. But I do feel stronger and better overall. However, I think this is a mind-body response to the good news.

But the breathing is back down to 4600 from yesterday's 4750, though I'll try again after breakfast has cleared out a bit more.

A very strong and athletic wind-surfer we know, who is about my age and who gets exercise all the time, has a left grip of 115 and a right grip of 116. This symmetry is a feature, I think, of people who are in good shape. My wife, who is strong, healthy, thin1 and beautiful, and who competed in college sports, has nearly equal strength in left and right hands too.

Oh, my right hand fell asleep again last night, in that really mild way I have tried to describe. The reason why neither arm did so during the period of the infusions is rather evident, when you think about it: I was much more conscious of my arm positions, even when sleeping, because I did not want to disturb the IV in my arm. It had an effect on how I slept2.

I am the king of rash (but correct!) decisions, and this morning I can't make myself take the riluzole. I have been reading about the possible liver damage, and just between you and me, I don't think it has shown any benefit beyond the three-month extension of the initial trial.

Over a year ago when I was diagnosed, my clinic doctor said: "what we're now seeing, in Ireland and in Britain and in other registries where people are starting the drug earlier, that there's a substantial benefit from the drug. It's very safe, but it is very expensive."

Well, I don't think it's "very safe," and in the past year I didn't heard a peep about that "substantial benefit."

This morning I sent an email and left a voicemail for the patient contact at the ALS clinic, asking for updated data on the ongoing effects of riluzole.

Glutamate is a necessary compound; your body cannot function without it. Normal bodies clear out the glutamate and replace it with new stuff. In the case of ALS, glutamate is not cleared out as much as it should be, and it builds up, damaging the motor neurons. Riluzole suppresses glutamate throughout your whole body. Which, if I may say so, seems kind of stupid. That's like applying a tourniquet to the neck to protect the body from harmful oxidation.

So, in my view, it's a poison, and it doesn't do much for ALS. I can't make myself take it, no, not even until I talk to the experts. I will, however, tell the experts about my decision, and if they can convince me to get back on it, then I will. Or if my metrics take a complete dive, I will.

You might think that that ceftriaxone has made me overconfident. Well, maybe so. But I don't count on the ceftriaxone to save me. I think it is possible that next time I use the ceftriaxone, the benefit will be somewhat less dramatic than it was this time. And so on, and so on, until it has almost no effect. I could be wrong about that, and I hope I am, but I am emotionally prepared for the possibility.

Anyway, nuts to the riluzole. For now.

The "D+[n]" notation in the post header tracks how many days is has been since my last dose of ceftriaxone. The 1/6/05 piece in Nature said that the beneficial effects last a substantial time after dosing (at least seven days in vitro (test tube) and four to 6 weeks in live rodents). The "D+[n]" notation is my way of reminding myself to track the changes.

No, I don't think thin is better. I just threw that in to infuriate people who are obsessed with thinness.

Neither 'affected' nor 'effected' is appropriate here. We should all just learn that there is no such word as the one we are groping for. This space is empty.

Wednesday, February 09, 2005

Can I help you get this drug?

Several people with ALS have emailed me asking how they can convince their doctor to prescribe ceftriaxone. Here is how I responded to one fellow, and I apologize for my lack of expertise, but this is currently all I know:

I am a newcomer to all this medical-bureaucratic poop, since ... I used to be

So I don't know how to convince your doctor to give you ceftriaxone. In my case I have indications of Lyme Disease. There are some other ailments, I bet, that this drug is used to treat. I don't know if you have indications of those.

My neuro and my ALS clinic doctor are different people. I don't think the people at the clinic have much interest in helping me. They are researchers and bureaucrats.

I wish you good luck.

Not trivial

I played with the settings on the self-measured inhalation volume chart so that the data variation would be more apparent, and to show elapsed time since diagnosis. The ups and downs are mostly due to respiratory infections. The broad downward trend is due to ALS, I think. That most recent uptick, from 4500 mL to 4750 mL, is due to the ceftriaxone, I think.

Various people who have told me about their issues over the past year have added "forgive me for complaining, my problems seem so trivial compared to what you are going through."

Well, but I want to hear your issues. I would not want people to stop including me in normal conversation just because of my condition. It's better for me to hear about you than to be isolated. So there.

Today my left grip has increased to 49 pounds (Alife: 49,49,46). The inhale volume was back down to 4500 mL, but I think it's because my tummy was full. Then when I had a chance to digest my breakfast, the inhale volume was up to 4750, the best since 4800 on 10/29/04.

Tuesday, February 08, 2005

Ceftriaxone Day 7: 47 pounds

My method for testing grip strength using the new dynamometer is this: Try it three times within half an hour and take the highest score. Today I got 45, 44, and 47. So today's score is 47.

Mike, P-47s fighter-bombers of the Army Air Force destroyed an enemy position using a low-level attack this morning, so now I'm wearing your watch, driving your car, and movin' into your house!

I am infusing as I type this, and today is the last day until whenever we do this again. I'll be so happy to have this thing out of my arm and be able to shower normally, and make normal gestures, like flicking my hands after I wash them. And wrestle with the kids.

I plan to take daily grip strength readings from here on out. Probably daily inhale scores too. Today my inhale score is 4600 mL. That's the highest since 10/29/04.

Left grip strength of other subjects...
Male architect: 98 lbs.
Female infusion nurse: 65 lbs.
Female cookie baker: 62 lbs.
My lovely wife: 76 lbs.


Irish stew

"Crater Lake" was the phrase I noticed myself having trouble with first, but last night there was an even better one, because we had it for dinner. Irish stew. Try saying that quickly. I can say it OK if I give a big pause to readjust the mouth after "Irish." But if I try to say it quickly, I get "Irisch-too."

A friend writes: "I'm curious why your doctor is only doing 1 week on, then several weeks off. Why wouldn't they do a month or two straight? Perhaps you can explain the logic in your blog."

Well, I can't speak for the doc, but I will tell you my opinion, based on the as-yet unproven belief that the drug helps my ALS. Unproven in the sense that my experience is what they call anecdotal. And, since we've only done it once, it's not yet shown to be reproducible.

Part of my approach is the feeling I have, perhaps false, that I still have a lot of runway left. I mean, I still walk and talk and swallow. If I were much more advanced, or had gotten to this spot very quickly, I would want to do the drug every single day.

But since I do feel like I have runway left, I'm willing to go off the drug for a month, and see if the grip strength declines. And then I want to see what happens to the grip strength when I go back on it.

I feel that if the drug is helping and I stayed on it continuously, at a certain point I'd max out the benefit, and learn nothing. Also, I may possibly prompt my body to down-regulate the expression of the gene that creates glutamate transport protein. My doc reminded me that if you give someone regular testosterone injections, their testicles (if they have them) shrink.

I have this mental image of the motor neuron experiencing a buildup of old glutamate and gradually beginning to function more sluggishly, to send weak signals, slow signals, and misfire by sending signals when not needed (twitching). This could be total nonsense, and the motor neurons could just be dying one by one, in a binary fashion. But the fact that the live rodents, and this live human, could regain strength, argues against the binary model and for the buildup model. The evidence indicates that the nerves were somehow resuscitated, or revived, when the increased levels of GTP-1 cleared out the old glutamate.

Using this imagery, then, we can picture regular monthly infusions of ceftriaxone that stimulate GTP-1 and clean house for the motor neurons. Hopefully this might enhance their survival.

You can argue that my reaction to the ceftriaxone is a placebo effect. And that's fine, I would not debate you. But I know that for my purposes in this world, it's no illusion.

Yet I am such a tech hound that I really want to see that curve. I used to diagnose problems like this all the time when they happened in computers. I want to see if the grip strength stays level for a month, or declines, or even climbs, and if so, exactly how. I love debugging systems.

One of the things we often did to confirm that we understood the problem, and the solution, was to try to recreate the problem, and see if our solution fixed it. Let's say that hundreds of log files are being created every hour, and the system is getting bogged down wasting cycles full-text indexing all of the files. In this analogy, the log file buildup represents the glutamate buildup in my motor neurons. Imagine that we think the solution is to turn off the logging, but we can't do that, because the third-party application server won't allow it. So then we decide to turn off indexing of the log files, and that seems to fix it. Then what we should do, to find out if we have the real solution, is to turn back on the indexing and see if the performance hit recurs, then reapply our solution, and observe the effect.

Naturally, you do this on the backup system. You never want to mess with the production system if you can avoid it.

I always used to say that I could never be a doctor, because someone's life is in your hands, but when you operate on someone's computer, the worst that happens is that the computer dies.

Now I'm debugging a software problem on a live production server. And there is no backup.

I am awake at this awful hour because my daughter has been fussing for over an hour now. My lovely wife, because she wants to take care of me, is doing all the work. My thinking is that I really should set up that youth bed for the girl today, and that will reboot her into a better relationship to sleeping.

Monday, February 07, 2005

Ceftriaxone Day 6: 15 to 33 percent!

Note on 2/9/05 to those who may read this later, to find out what I did. I was infused with one gram of ceftriaxone intravenously, at a hospital. Then the home nursing people came to my house the next day and taught my wife and I how to do it. They placed an IV needle with tube in my arm. We left it there for six more days and I did the infusions myself each day, at one gram per day, using premixed solutions that they provided and left with us. So that was a total of seven days at one gram per day. We may do this again in a month, depending on what my doctor says.

Let's start with the important stuff first: Mike, based on today's mission after breakfast, I am now wearing your watch and driving your second-hand, red Ford Festiva!

I'm infusing right now. I went to the neurologist's office this morning (Monday) to calibrate his hand dynamometer against mine.

The new one I bought is a different type, and it has been saying since it came on the evening of Friday, Feb. 4 that my left grip strength is 44 or 45 pounds. The readout on the doctor's was 38 on Jan. 10, and 33 on Wednesday, Feb. 2, the same morning I started the ceftriaxone treatment. But I assumed that the units were different, so I needed to compare them and get a factor.

The decline from 38 on 1/10/05 to 33 on 2/2/05 amounts to 15 percent. I don't know how to explain it, but I could not top 33.

Each time I use the doctor's dynamometer I pump it three times as instructed and read the score. Then I do this twice more. Then I do it the same way with the other hand. So you wind up with a set of three numbers for each hand. Both the dynamometer he has and the one I have are amazingly consistent in terms of the top score you can hit. You might tire yourself out and go lower, but no matter how hard or how often you try, you can't beat your top score for that day.

This morning, with some dread, I squeezed three on the doc's dynamometer. It read 44. Then I took two more readings, of 40 and 43. Then a few minutes later I squeezed my new dynamometer, which I had brought to the office, and it read 44.

The devices calibrate exactly! [2/25/05 note: This impression is incorrect. The instruments are quite different, and only coincidence made me think they calibrated. However, the strength increase was quite real.]

This means that my earlier readings of 33 and 38 on the doc's dynamometer can be compared to both the current readings it gives, and the readings my dynamometer has been giving over the weekend.

According to our objective measure, my grip strength is stronger. Just like the rodents in the Rothstein study published on Jan. 6 in Nature.

That's great news. And like the rats in the study (if I read it correctly), the effect occurred within about 48 hours of drug application. I'm sorry I did not test myself on the 3rd, but I did not have time to go to the neurologist's office to use his dynamometer (my dynamometer had not yet arrived), and the insertion of the needles that day stressed me so much that I spent the rest of the day recovering.

I have not been testing my right grip strength during this period, because the IV needle is in the right forearm, and I don't want to disturb it with muscle clenching.

If we take the 38 score on 1/10/05 as the baseline, then at 44 I have improved 15.78 percent. If we take the 33 score of 2/2/05 as the baseline, then I have improved 33.3 percent.

Either way, this is an amazing result.

I was so distracted I accidentally injected myself with two cc of heparin instead of one. But I seem to be OK. I did call the home nursing people, and the operator said to call the pharmacist. That's not the right answer. The right answer is: "Here is the supervising nurse today, who will help you." That's the first time the home nursing people have sucked. Up till now they have been great.

Now, being a skeptical mind as I am, I have been trying to find a way to explain this improvement as a combination of psychological stress, and inconsistent technique with the dynamometers. And I think it can be explained that way, but not in a way that fits with my experience.

This is why they do double-blind, controlled studies of groups of patients, to rule out the Maybe Scenario to their highest statistical satisfaction.

And I'm just one guy, and my body, and my ALS, and its rate and severity, are no doubt different from yours, but to me, for my purposes, this is an objectively measured, unexpected increase in grip strength.

I feel just as weak and stumbly as before, but this therapy looks promising to me. I am going to ask my doctor to try two or more grams a day for seven days, in one month's time.

And yes, I am familiar with the Awakenings scenario, where the patients get better for a while, then revert. I'm not about to think of this as a cure. And I do want to see what the data says next time. But unless it has some delayed boomerang effect, it may be adding time to my clock.

We Shall See.



You might wonder how my son copes with the fact that we used to play chase so much, and I no longer run. He's never noticed, because he and his two-year-old sister have developed chase into a daily ritual. Serendipity and utopia all rolled in one.

Sunday, February 06, 2005

Ceftriaxone Day 5

Everything is nominal. I can't say I feel any improvement. But this is half the dose I expected. It has not done me any harm. We are planning an addition to the house to make it disabled-accessible and provide a bath and bedroom I can get to.

I have not noticed the night numbness and tingling during the ceftriaxone period, other than the Wednesday night of 050202, the night following the initial infusion at the hospital. Coincidence, probably.

How am I holding up? I can still use chopsticks. Happy 4703.


Off to the right we see a graph of my self-measured inhale volume, which used to be 5100, dipped to 4300, and now is 4500 mL.

I don't want to be defeatist, but it seems to me that the most likely scenario for me to lose the ability to walk is if I trip and fall, break bone, and then have to be partly or fully immobilized in order to recover, during which point my muscles atrophy severely.

Case in point, yesterday I was standing on the fourth of the concrete steps leading up to our house, talking to my lovely wife, when my feet fumbled and I staggered out of control down the steps and wound up falling on the grass. I considered it a successful fall artfully handled. But it scared my lovely wife, and if there had been children or obstacles in the way, it could have been quite bad.

Breathing is 4505 mL, grip strength is 42. The "05" added to the breathing is because the marker goes just a tad of a hair above 4500.

Once again in an article I see someone calling ALS the worst disease you can get. Well, it's bad, no doubt, but I just got word that someone I know, with his cancer and his chemo, is in such extreme pain right now that he can't walk or talk -- and the painkillers are not powerful enough. And his little boy gets to watch all this, watch his daddy hurt. I think I know what's worse than ALS.


Saturday, February 05, 2005

Ceftriaxone Day 4

All systems nominal. No Herx reaction. Negatory on the squirts.

A friend sent me a link to an article about Olney which mentions something I had not heard yet:

Rothstein is among a handful of researchers developing possible treatments using gene therapy. Later this year, he and his colleagues hope to test in humans a common virus they've engineered to carry a gene that produces a growth factor directly to the brain and spinal cord. This technique significantly extended the lives of ALS-afflicted mice. Rothstein and others also hope to turn stem cells into replacement brain cells.

A buddy who has ALS sent me some links to threads over at braintalk where people are talking about this gene trial:

1, 2, 3, and 4

Infusion steps

To the right is a picture of the bottle that contains a balloon of ceftriaxone. This balloon is in the deflated state, since I already used this one. When full, it's a little ball. Also pictured are the saline (white label) and heparin, (yellow label).

The process is:

  1. remove the netting that restrains the tube and clamp

  2. wash hands with super-antibacterial soap provided

  3. clean the valve at the end of the tube by continuously rubbing it with an alcohol swab for 15 seconds

  4. unclamp

  5. attach saline syringe and inject 2 cc

  6. attach antibiotic unit and let it infuse itself over approximately 30 minutes, until bulb is flat

  7. attach new saline syringe and inject 2 cc

  8. attach heparin syringe and inject 1 cc

  9. clamp

  10. remove the heparin syringe

By the way, the kids have not said anything about the gauzy white retaining sleeve for the IV, even though it sticks out from my sleeve from time to time. I think the little girl has caught sight of it, but in the tumult, she hasn't yet mentioned it. And the boy doesn't even seem to have noticed it. Odd. I also expected them to ask me why I am wearing long-sleeve shirts now, but no. If they do ask, I plan to tell them I am being treated as a precaution against Lyme, given the ambiguous results of my blood test. And no, I won't know what Lyme does to you, it'll just be some "thing," not sure what.

...I got my left squeeze score up to 45 pounds this morning, but only, I think, due to improved technique.

Rear neck muscle twitching 30 seconds yesterday. Did not want that.

Friday, February 04, 2005

Ceftriaxone Day 3

I'm infusing myself now (I seem to be able to handle it, with some fumbling) and am watching Armageddon on DVD.

...Everything went smoothly and I think the printer will recover. When I was preparing to screw the saline onto the line, I prepped it to clear the air bubble, and a bunch of it flew across the room and onto the printer. Then with the heparin, I did the exact same thing. Oops!

That was a fun movie. The theme of trying to save the world through an experiment resonates, since I am trying to stay in this world, as does the idea of wanting to live long enough to see your daughter get married, and like that. And plus I dig space stuff, and as unrealistic as some of it was, it appealed to me. The theme of things getting out of control and just getting too freaky weird as your doom approaches is a good one too. I cried.

5:53 PM: The hand dynamometer just arrived, and my left hand shows a strength of 44 pounds. I do not know what the reading of 33 using the doctor's dynamometer is meant to indicate, pounds, or what. That's why I have to calibrate them. I don't feel any stronger. The manual that came with the dynamometer seems to indicate that for men in my age range, the weakest score for the non-dominant hand (left for me) of typical randomly-sampled men would be 73 pounds. Highest 157. The average given is 116.8. So, hey ... let's hear it for the big 44!

I used to be strong, for my weight, and might well have scored better than average.

My lovely wife can score 65 pounds with her left hand.


Plastic wrap dad

Off to the right we see the annoying IV in question...

All continues to go well, with no squirting. My walking is a refined stagger at all times now (has been since before the ceftriaxone). I remember a few months ago grieving that I could no longer run. Now the issue is walking. Let me just say that running has a threshold, and walking has a much less demanding one.

I'm about to try to wrap my arm in plastic wrap and take a shower.

...Wow, that was awkward. Almost seemed not worth it. It was a mistake to put the IV in the 'good' arm. But I'm not one of those people who laments a decision once made. I understand what our goal was at the time (Get it in, anywhere!), and I'm glad we accomplished it. But next time, left arm. Plastic wrap is what they said to use, but now I see that a plastic bag (open at both ends) plus rubber bands would be better. All that tape cut off my blood supply and tore the hair off my arm upon removal. As it was, I did not do a full shower, just washed the hair (nice and short) and soaped a bit.

CDNPals Mike, I'm wearing your watch now, because a few minutes ago I accomplished a very important mission, and still no hint of the squirts. Quite the opposite.

Also, on the way back from dropping my son at preschool, I got to thinking about how I can't jog, and so as an act of defiance, I 'jogged' for about 40 yards. The sound was: clop-clop clip clop-clop-clop clip clop-clop-clop. All flat-footed, small stride, knees barely lifting. That's the best I can do, and if you want your money back, come see me in Hell when this is all over.

I'll still be wearing your watch, Mike.


Thursday, February 03, 2005

Ceftriaxone Day 2

The home nursing people came by today and put an IV needle in that will stay in until the remaining six days of drug are done, unless they need to change it.

You'd think something as simple as this barely deserves a mention, but my veins were not cooperating today, and they poked me three times to no good end before the fourth one took. And this is my blog, so I am going to complain. I don't like being poked. It makes me feel jaggy and stressed.

Now of course, I get to recover. "From what, whippersnapper? Let me tell you about my bypass!"

Or breast cancer, or other painful things. A couple of needles in the arm are nothing compared to that, but as I said, this is my blog, and this is where I complain.

The good news is that my wife is putting out the trash bins! Just kidding, I actually enjoy that. They told me to 'baby' the arm with the IV, which is my right arm, the stronger one, that I use for lifting those heavy bags of garbage. You try lifting 13 gallons of wet pullups and tell me how easy it is!

Once again just kidding, but the bathroom trash can is quite large and it mostly has wet pullups in it.

This time I was willing to settle for anything that stayed in. But next time I am going to ask them to put it in the left arm. I am wearing a long-sleeve shirt (yes, in warm weather), and using my weak, clumsy left hand it took my five minutes just now to do the button at the wrist. My lovely wife did that sleeve earlier today, but I wanted to check the needle, and she is helping someone go poopie.

Anyway, I am going to get my car smog-checked this afternoon. That should be a nice mission to get me feeling normal again. (But I was unable to do this due to their equipment being broken today).

Still no sign of diarrhea and that's good. I like to think the probiotics are part of the reason.

The waking with numbness and tingling of arms and hands continues, though I characterize it as 'light,' and it feels different from blood deprivation (e.g. sleeping on the limb).

Glyconutients update: The Mannatech "Amrose" glyconutrients came today in a little box that I was hoping contained the hand dynamometer. The idea behind the glycos is that the modern diet is deficient in essential sugars that intercellular processes require. Apparently, our cells are coated with droves of these little sugars. The thing is, I didn't see any references to research showing that any people have cells which lack the requisite panoply of sugars. Or that these individuals suffer any ill effects, such as, oh, let's just say, ALS. But it could be. I slammed three of these pills right away, will do the same tonight, and then I guess taper to one pill twice a day.

Youth bed

My kids are the best. My son is becoming increasingly responsible (for a child!) and my daughter does her part by peeing in her crib. Let me explain: She has internalized the habit of keeping her diaper dry to such an extent that, when she's in her crib and needs to pee, she has twice now removed her garments and her diaper and peed.

Pronto, we need to set up the "youth bed" for her. This is a short little bed whose parts we have stored down in the laundry room ever since my son outgrew it. That way, when she wants to pee, she can get up, go to the potty, and make.

When it comes to potty training, one needs to honor the impulse and not let the wires get crossed to the point where the objective becomes peeing in the crib.

Wednesday, February 02, 2005

Ceftriaxone Day 1

The Ceftriaxone infusion went uneventfully at the local hospital infusion center. They gave me one gram intravenously and there was no allergic reaction. The infusion took place at 11:30 AM. My lovely wife was with me. After the infusion we stopped at the neurologist's office to do another baseline of my grip strength. Left hand 33, right hand 94. The left is the same as last time, the right is down 6 percent from last time. So I may be getting weaker on the right side but I'll bet it's due to something like when my last meal was, or the time of day.

The nurse who will train us on how to do the home infusion will come tomorrow morning.

In a last-minute twist, the home nursing people convinced my doctor that one gram per day would be more appropriate than two. He has today confirmed that he goes along with that. He says that next time we will evaluate whether to up it to two.

I had a pleasing chess victory this morning, and I struggled through and was able to create an animated GIF of the game, so that y'all could see the game rather than its algebraic notation. Safari shows it just fine when opened from my desktop. However when I upload it to Flikr, it no longer animates, neither there nor when blogged. I know that other people have done this successfully. I will try to puzzle it out.


I went and had lunch with the guys at work recently, and it does have an emotional impact. So much of your identity gets caught up in work, if you enjoy what you do and are treated well. I saw the fountain when I walked in this time. I remember having lots of responsibility to solve technical and coding problems, and walking in past that same fountain, feeling cool. Stressed, of course, but ... cool. And I was cool. It was fun to face a big monster and tame it, gradually, over time, with my own smarts.

I also saw one of the guys I used to play basketball with. We were intense, charging up and down the asphalt court playing five on five, for over an hour, in intense heat sometimes, once 114 degrees Fahrenheit. Not much charging on that day. But the point is, the point of team sports is, using your body and your brain together, the brain planning moves, sure, but the body, often just reacting in split seconds, unpremeditated motion, knowing what it can do, what it thinks it can do anyway, and once in a while, once a year maybe, that perfect play, where all the forces and chances line up as your rushing legs and reaching arms, eyes, hands, lungs, even your hair are in rhythm, and you make that perfect pass, the assist, two bodies flashing create a tiny moment of space, and you put the ball through it, and your teammate is there, two hands, eyes locked on the ball, he grabs it, pivots, lifts, scores. Game over, you won. How many steals did you get? How many baskets? I want to play. I want to play again! I miss it. But I did play. At least, I did.

To the right is a graph of the number of pushups I have been able to do, over time since my diagnosis. It's not a complete picture of my decline in strength, because at a certain point I felt too much strain was not good for me, so I gave up on this as a goal.

My cough appears to be greatly reduced. It has so far not gone wild. Sometimes I will have an hour of dry hacking, due, I think, to an irritated voice box. Oh, and now I seem to have a minor eye infection. No kidding. Left eye is all red, some ooze came out yesterday. Good thing I am going on powerful antibiotics today!

Tuesday, February 01, 2005

Theology upate
becky had some comments on the most recent post. and rather than struggle with Haloscan's 1000-character limit, I'm just posting my comments here...


Again, I hope I don't offend you, but since you did ask, here we go:

After I wrote that comment, I worried that you would think I meant joining an organization. Yet I know it is possible to be a Christian without any group to join. By church I meant joining the community of Christ, accepting him.

When you do that, it supposedly has a big impact on your status in the afterlife. What I was trying to highlight was the binary nature of this inclusion. At some point along the line the turnstiles admit one either to heaven or some other place. I believe that this mechanism is a major coercive feature of many historic religions. I find it distasteful, and ultimately demonstrative of the cruelty of the doctrine in question.

> What would it hurt at this point to add
> another adventure to your journey....
> seeking whether or not there is a God??

becky, I sincerely hope that you are not trying to convert me. I have not read the author you mentioned, but I have done enough thinking and reading regarding religion and Christianity to know where I stand. My time is limited on this Earth (and that would be true regardless of ALS), and I see no value in exploring avenues I have already resolved.

What intrigues me is to know whether the perspective from which you read this translates or filters my words into something else, for example, a rejection of love, or hope. I would expect the dominant faiths to do that for you, to categorize me as a lost soul.


Off to the right, as we see a graph of my blood pressure coming back down into the normal range over the past year. Does anyone know if HTML has charting capability? I know it can make tables, but charts?

I arrived at a quippy description of that attitude I keep getting praise for: I'm an optimist because I'm a pessimist. I anticipate how bad things could be, and could become, and I look around and see that they are instead quite good, and my faith is validated.
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