Off Topic

(sort of) I resisted posting this here because it is not in keeping with the mission of this blog (I originally posted it on my Facebook page - the more proper venue). However, due to numerous requests and delays in preparing my next intended entry, here goes: My Strings Sing for Renaissance The strings To my Meat Marionette Have been cut! And so we Round up the usual suspects. As they are released One by one I realize that Lying around in bed all day Isn't as decadent as it sounds. The floppy foot Made a Goofy step As it marched up the leg. After the Fall Season I decided to have a seat. Then I Let my fingers do the walking Until they took a hike. I can't even Give myself a hand. And the creeping uneasiness Took my breath away. Unlike Mick Time is not on my side Although I seem to have more Than ever before. Now cyborg is as cyborg does And I wait for The bright near future Where once again Gravity is my friend. © 2010 Eric N. Valor

Tread Lightly

So here I am, minding my own business, when my mother brings her pooches over for a playdate with my two. No worries, I enjoy the sounds of rambunctious dropkick dogs scrambling around on hardwood floors. My mother brings one of hers over to my left side to say hello when my Yorkie jumps up on my right side and does a skidding slide across my abdomen and into my PEG tube. POP! gurgle gurgle gurgle Mom and wife were talking and didn't take notice that this was a serious situation. Since I was not on the computer it took me a few moments to mouth what had happened. When my wife turned back the sheet the tube tumbled out. Now I have gastric goodies and some blood dripping down my side. To their credit neither of them fainted. I am lying there, angry, mentally adding up the cost of transportation and other out-of-pocket for replacement at the hospital. Luckily we reached my doctor on his cellphone, rescuing him temporarily from gardening chores. He explained that the tube was held in place by a balloon on the end which had become semi-deflated and that we should just push it back in. Having already tried that with no success but much pain we were afraid it had started to heal closed in the 45 minutes it took to reach the doctor (a reality for tracheotomy). When we said that, his response was, "Did ya lube it first?" A few globs of medical lubricant left over from a previous trache inspection procedure (tube down nose with camera; trying to forget) and it slips right back into my new second mouth. No worse for wear. Important note: Tread lightly on the PEG.

Role Model

A few days ago, Matt White, who lives with ALS, addressed the Butler NCAA basketball team prior to their Final Four victory over Michigan St. Normally I don't care about basketball (I am a hockey fan) but a Facebook friend posted the Youtube link above and I viewed it. The thing that struck me about Mr. White's address was his promotion of Sir Ernest Shackelton as a role model. If you don't know who he is or don't know his story, please take a moment to read the previous link (most notably the portion on the Trans-Antarctic Expedition). Sir Ernest is a perfect role model for PALS because of his dogged determination, his cool ability to make rational choices in grim situations, and his use of those characteristics to survive a lethal predicament (multiple predicaments, actually). Not only did he survive but he ensured his men would as well. Contrast the experiences of Scott and, worse, Franklin. Even though Shackelton was forced to abandon his original goal, he achieved an even greater goal by surviving. I would like to thank Mr. White for including Sir Ernest in his address and would like to concur. Even though PALS may be forced to abandon their goals, it is my hope that many can join me in "pulling a Shackelton" and doing what is necessary to survive an impossible situation.

CD40L and the MAB

The ALS Therapy Development Institute is a non-profit biotech fully devoted to finding a treatment for ALS. A few years ago they undertook a genome-wide association study (GWAS) on the animal model of ALS (the G93A mouse).The results implicated the costimulatory pathway which links the innate and adaptive immune systems. In an attempt to address that pathway, TDI used a monoclonal antibody against CD40L. This decreased a specific T-Cell interaction which led to the body attacking the axons of the motor neurons. A very good description of the study can be found at the Alzheimer Research Forum (Alzheimers, Parkinsons, and ALS are selective neurodegenerative diseases so research in one can provide clues into the pathways of others). This finding is interesting for a few reasons. For the first, it marks a shift in looking at ALS as a motor neuron disease to seeing it as some other dysfunction in which the neurons are injured. Second, while the treatment described isn't a "cure", it does point towards a significant pathway of disease management. Another important point is the success reported by TDI. Since the development of the murine model of ALS numerous researchers have claimed varying degrees of success in preclinical work only to have the candidate substance fail in human trials. TDI undertook an internal study to figure out why and discovered numerous variabilities for which researchers must account when designing trials. TDI's mouse program is now recognized as the "gold standard" for that model. The fact that they can report measurable and repeatable results is significant. Of course mice aren't men and this isn't a cure, but it does give quite a bit of hope that a truly effective treatment may be within reach. [UPDATE 04-02-2010: TDI discusses this via MP3 podcast.] [UPDATE 04-05-2010: The study is now available free of charge in PDF format. Very good of Nature Publishing Group to make this available to PALS and CALS!

Stem Cells for Dummies

Stem Cells for Dummies is authored by Lawrence S.B. Goldstein, PhD. Dr. Goldstein also co-authored the legislation which created CIRM. The book does, IMHO, a rather good job of presenting complex science in terms which laypeople can easily digest. It starts with a primer on biology as it applies to stem cells and a brief history of their discovery and initial uses. The differences between adult and embryonic cells is explored with frank discussion of the attributes and limitations. The role of these cells in the research and treatment of various diseases is discussed along with a review of the current state of the art (my previous post on stem cells deals with some cutting-edge techniques). There is a detailed section on the ethical and moral controversies surrounding this sensitive topic, and a section on common myths and misconceptions. Stem Cells for Dummies is written from the scientist's viewpoint but takes effort to present in a neutral tone. It is written for anyone who wants to understand stem cell technology without requiring a PhD. Rather than spend weeks cuddling with Google and sifting through the inevitable dreck, drop $10 and receive a great resource from a recognized expert in either dead-tree or electronic form. [disclaimer: I linked to Amazon for convenience and because I use their Kindle for PC application. If you can, support your local bookstores.]

Sausage-fest!

Interesting things happen to one's body due to ALS. One of the most trivial effects I have noticed involves my fingers. I am quadriplegic so my hands spend all their time on a (relatively) flat surface. Grasping or any voluntary flexing of my fingers has been nothing but a memory for years. This allows the skin to tighten and smooth a bit. You know those little wrinkles over your knuckles on each finger? I don't have any anymore! I noticed this during physical therapy a few weeks ago and wondered what these little keratin-tipped bony alien hotdogs were doing at the ends of my hands. Truly a sausage-fest.

Letter from Lou

I recently received a handwritten letter from Lou Gehrig. Obviously it was not addressed to me as the people responsible for creating me had not yet themselves been created. But it does give an intimate glimpse into the life of a remarkable man (even without the disease his accomplishments are legendary). I was struck by the proof of his equally legendary kindness and generosity, and hope to approach that in my own life as I struggle with fellow PALS to achieve the goal that eluded Lou.