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Long Time, No Write


It’s been a long time since I’ve posted anything to Bachblog. Several (all three?) bachblogophiles have recently asked have I misplaced my keyboard or something like that. One even lamented the lack of anything new from Adelbert B. Brain! And more than a few of you have asked about the elephant.

Has the cat got my keyboard?

The cat has my keyboardWhy the lack of blogtivity? It’s not for lack of time, that’s for sure. I’ve just neglected it, that’s all.

Joann has been on sabbatical for almost a year now and we’ve kept busy together. It’s still my intention to post something about our winter trips to Belize and to Florida. If nothing else, I will be updating my lifebird accounts: I’ve added 85 birds to my list since the cranes of 9/11.

To help fill the very long gap between posts, I’m gone ahead and posted a very long, very tedious account my performance in a November Scrabble tournament. I wrote this shortly after the event, but thought better of publishing it because it is very long and very tedious. Now I’ve gone and posted it anyway. I will be playing in another tournament this coming weekend, and I’ll try to post a short, scintillating account of it soon.

Here’s a promise. When Joann returns to teaching full time in the fall, I’ll either start sleeping in every day until noon, or spend more time futzing with this blog. Maybe both.

The elephant

ALS is the elephantThe elephant in the room is of course ALS. What’s up with that now?

I haven’t “kicked it” in either sense: I still have ALS–it didn’t “get better” or turn out to be a really, really bad cold–and I’m still nearly six feet above ground.

To keep playing that broken record, my progression is a slow one. I’m now approaching three years from the onset of my symptoms and was given a definite diagnosis 14 months ago. In most respects I am doing very well.[1] I participate in the ALS forum of Patients Like Me and occasionally read other on-line ALS forums. I know, by sad experience, that most of those whose symptoms first appeared at about the same time as mine are not as fortunate. A few have died.

My legs remain strong with only minor changes in strength and coordination. The atrophy I noticed in my left leg a year ago has changed little. My breathing as measured by “forced vital capacity” (FVC) has not declined at all. My ability to swallow has not been affected.

Not all news is good, however. My tongue shows definite signs of involvement. It fasciculates (twitches) and has lost some of its muscle. I can sound a little tongue-tied when I’m tired, but it’s very minor at this point and most people don’t notice it. Still, this is my first definite sign of bulbar involvement and is unwelcome.[2]

Other changes include the fact that, for the first time, I test positive for the Babinski sign.[3] This indicates a progression of upper motor neuron involvement.

The most notable changes, though, are in my right hand and arm. My symptoms, you may recall, first appeared in my left hand and arm. I am still able to use my left arm to some extent, but it is very weak and pretty close to useless for most tasks. Now my right is following. Fine motor skills in the hand are deteriorating rapidly (I type mostly with one finger now[4]), and the arm and hand fatigue very quickly. I’m having more trouble adapting to these changes than I did to those in my left because I’m right-handed and–dammit!–it’s the only arm I have left.

So this is not great news. It’s hard to know how fast things will go from here, but it is possible, perhaps probable, that I will not be driving, using binoculars, or playing tournament Scrabble in a year. My clumsy use of utensils is already making it difficult for me whenever I try to make a good impression on a pretty young waitress. I’m sure this won’t be any easier when Joann starts cutting my food and makes me wear a bib.

On the other hand (literally!), my left arm and hand weakened fairly quickly and then seemed to plateau. If I see a similar plateau on my right side soon, I will be quite pleased. I’m still in a promising drug trial that will end in September.[5] If I’m on the drug now, it may be helping to slow my progression (is it responsible for the plateau in left arm and lack of progression to legs, diaphragm, etc?). If I’m on the placebo, I should get the real thing in September (will it further slow my already-slow progression)?

There are always more questions than answers.

Recent ALS Bites:
DATE TITLE
05/12/2017 The Eyes Have It
08/04/2016 YFALS National Corntoss Challenge
07/18/2016 Scopolamine Blues
03/06/2016 Dragon Me Down
02/12/2016 Five Years In

   or see the  Complete ALS Bites

Notes:

  1. I have mentioned ALS’s sobering prognosis before. [^]
  2. Motor neurons of the bulbar region enervate muscles that control speech, chewing and swallowing. [^]
  3. See Wikipedia’s Plantar reflex page for more on the Babinski reflex. [^]
  4. I am also learning to use Dragon Naturally Speaking to dictate some of what I write, including parts of this post. [^]
  5. I am in Biogen’s “EMPOWER” trial of dexpramipexole. There is a fair amount of cautious optimism in the air about the drug, which the company hopes will reduce the rate of progression in ALS patients by an average of about 35%. Biogen stock may be a good investment. I wrote more about this trial in Guinea Piggin’. [^]

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