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.

Kindle for PC

Back in November of 2009, Amazon released its Kindle for PC software. The program is free and available for Windows 7, XP, and Vista. A future version for Mac is promised. While I have never used a physical Kindle (holding anything but my breath is no longer an option for me), the software appears to recapitulate most of the function of the device. The most important part for me (and for all advanced-stage PALS) is that it behaves very well with my optically-controlled assisted communication system. I use the ERICA from ERT. ERT was recently purchased by Dynavox but the K4PC should still work on that platform as well as the Tobii. Downloading and installing is very simple. Once installed you need to register the "device" to your Amazon account (like the physical Kindle each registered software counts as one of the 5 devices on which you are allowed to share your purchases). If you don't have an Amazon account you will need to set one up (I recommend letting an assistant do the data entry here although I was able to do this myself). For those who already have a Kindle, you should see your previous purchases sync to the software. If this is your only device, new purchases from Amazon will appear within 30 seconds of purchase. Each page has large, easy to read type and the size and spacing can be adjusted for comfort. To either side of the page are white arrows to turn the page. They only appear on mouse-over, and if you click even near them you will hit the target. They are spaced far enough away from the text that accidental page turn is almost impossible. If you close the application with a book open your place is saved automagically. Other bookmarks are easily set. The software is considered beta at the time of writing this post but works very well for me. Updates are automatic but can be set to manual. For software not expressly written for handicapped users it satisfies some important requirements. I am very happy to be reading books again!

Stem Cells

There has been a bit of action recently in terms of stem cells, with one human trial in MND which commenced this month and another which hopes to commence soon. There are a two ways for stem cells to be of benefit: Trophic support where the grafted cells support existing neurons by secreting nourishing (trophic) proteins such as GDNF and BDNF and regenerative where new replacement neurons are grown. Each approach has advantages and technical (biological) hurdles. As I said, trophic is secretion of neurotrophic ("nerve-feeding") factors. It can also occur from the implanted cells just being a close friend to the diseased neurons. A benefit of trophic therapy is that the graft cells do not need to differentiate or grow. Cells modified to become GDNF "mini-pumps" have been shown to fully protect motor neurons in animal models of ALS. Unfortunately, however, in that study the protection did not extend to the axon. While the cell body was healthy, it made no contact with muscle which is functionally the same thing as the whole cell dying. In the trial underway in the United States, Neuralstem is using fetal stem cells. While they claim their product will differentiate into neurons and glials, they only claim to be pursuing a trophic therapy. In at least one animal trial, the implanted cells extended the life of the model (it is worth noting that due to variability in the model, the ten extra days could just be statistical noise). The cells were found to fully integrate into the host central nervous system. Another group, TCA Cellular Therapy is using autologous cells taken from the bone marrow. Both of these trials involve spinal implantation. A planned trial in Israel will also use autologous cells but will do the implantation in muscle to try axonal uptake. Regenerative therapy has its own set of issues. While the ability for stem cells to become neurons has long been demonstrated, the new neurons still need to make proper axonal connections to muscle (spine -> muscle) and within the brain to other neurons (motor cortex -> spine). In 2006, a study by Johns-Hopkins achieved that in test animals. The process required not only implantation of prepared cells but also significant treatment on the muscular side of the connection (sharp readers may notice similarities to procedures discussed above). A much more recent study from Stanford showed that proper preparation of the stem cells is critical for proper behavior of the cells after implantation. The cells prepared with retinoic acid (as in the Johns-Hopkins study) failed to create proper connections whereas cells prepared by co-culture with stromal cells grew robustly and made proper connections from the motor cortex to the spinal cord, bypassing neurons from other cortexes (visual, etc.). Caveates of the Stanford study is that the work was done on very young animals (about a week old) so fully adult animals may present a different environment, and that requirements for cortex -> cord connections may be different from spine -> muscle. On most of the studies above, the links are to press releases. This is done to indicate existence and to give limited information. Copyright prevents me from republishing any studies (except for the PLOS link which is intentionally placed in the Public Domain. The reader should also note that my technical training is in hardware and software, not wetware.

A Very Special Olympics

People often ask, "How do you do this?" The simple answer is, I have no choice. There is only one alternative and that only interests me in terms of rhetoric not experience. But there is a deeper answer. Anyone who knows me will tell you that I can be one stubborn cuss. My grandmother used to say that I would argue with a fence post. I have always lived on my terms (often to my detriment) and this presented challenges which I learned to overcome. My chosen profession (Information Technology) gave me unique and complex challenges every day. Motor Neuron Disease is just another challenge. I see no reason why this one can't be overcome. MND tries to define you: how you look, how you act, how you live, and how you die. I have defined myself and am not about to change. Instead of MND defining me, I define it. It is hurdle to jump. It is a hill to ski. It is just another event in which I compete. Like an Olympic athlete, technology gives me the equipment to compete. Assistive equipment allows me mobility, helps with personal tasks, and keeps me nearly as engaged with the external world as when I was healthy. And it keeps me alive. As with Olympic athletes, I am part of a team. I have a personal team of family, caregivers and doctors without whom I could not succeed. These are my coaches, trainers, and equipment handlers. I compete for them as well as for myself. And I am competing for the Gold. That is why I do this: I intend to win.

I'm Not Dead Yet!

It has been a long time since my last post. Blame the holidays a bit. But the blame lies in the website being hideously difficult for me to update. I had intended to use Blogspot as the "archive" but found it so much easier to edit that I looked for ways to automagically include it into a site in which I had no administrative access to the server. A clever use of the "object" tag (for those who are interested) permitted this. I have some interesting news to report in future posts so please check back. I promise no more 4 month absences! Thank you all for your support.

Radio Show

11/02/2009
A few weeks ago my mother Joan participated in a half-hour radio interview with P.J. Ochlan of KGIL in Los Angeles. Since I can't talk I had prepared some points to cover. Mr. Ochlan opted to read them verbatim and they are shown below (followed by a link to the show which you can download and play with Windows Media Player or similar program):
While I have an immediate financial need, this opportunity should be also used to inform the public. If not for the short average survival the number of concurrent PALS would be much higher and more visible like cancer or alzheimers. The NIH funding for ALS research is 10% of that for other diseases like atherosclerosis which already have effective treatment and are not fatal.
It would be nice to have Medicare support for home caregivers. Necessary skills are minimal (basic trach care, personal care) but are the daily difference between life and death. This is already supported in most or all European nations.
I am still a useful human, trying to use my skills and training to continue to assist others while researching the applicable biology and physiology to better understand what is happening to me. The mental acuity of PALS is very rarely impacted.
It is now clear that ALS is a multi-system disease which means more than just neurons are involved. Research into ALS therefore can be applied to many other diseases.
The show can be downloaded here.