July 22nd, 2009

Logo

2009 Walk to Defeat A.L.S.

As most of my friends know, last October my mom died from Lou Gehrig’s disease, officially known as A.L.S. I moved to Bellingham for a year to help take care of her. A.L.S. is an ugly illness. While patients with A.L.S. don’t typically experience a lot of pain, they do lose independence and quality of life. Mom had to eat through a tube, wear a brace to hold her head up, and talk with a computer. I heard that computer so much the last year that I don’t remember much of mom’s actual voice.

My part to see that as few people go through this as possible is to support the Evergreen Chapter of the A.L.S. Association. They fund research into A.L.S. and lobby on behalf of patients. For those who are afflicted, including mom, the A.L.S.A. was a lifeline of support. That computer? Provided by the A.L.S.A. Her hospital bed? Provided by the A.L.S.A. When the family first met with hospice, Mary from the A.L.S.A. was there. When mom saw specialists at Virginia Mason’s neurology center, Mary from the A.L.S.A. was there.

The A.L.S.A. holds the 2009 Walk to Defeat A.L.S.A. in Bellingham in September. Until then, I’m going to write a lot about A.L.S. to educate and to get people to donate money. My family has a team, and I’ve set up a page for donations.

If you donate money, I’ll give you a book. I need shelf space, so why not solve two problems at the same time. Books aren’t just the crappy books either: advance reading copies, autographed copies, hardcovers. Here’s the list of books. More information about mom, how to donate, and how to get your book at my walk page.

Originally published at King Rat. You can comment here or there.

Logo

Bulbar onset

The two most famous cases of A.L.S. are that of Lou Gehrig and Stephen Hawking. Stephen Hawking’s illness is somewhat atypical for A.L.S., in that most patients with the disease have a lifespan of 2 to 5 years after diagnosis. Longer lived patients survive 10 to 15 years. If indeed the underlying disease is A.L.S., Stephen Hawking is the longest surviving patient ever.

Lou Gehrig’s performance as a Yankee started declining in 1938, mostly his power at the plate. By 1939 he was having problems running the bases, fielding balls, and connecting with pitches at all. After 2,130 consecutive games played, Gehrig benched himself. Weeks later he went to the Mayo Clinic where he was diagnosed with A.L.S. Two years later he died.

Stephen Hawking first noticed symptoms when he was rowing. He didn’t have the strength he previously had, and then he started falling down. Over the next 40 years, he has gotten progressively worse. At this point in his life, he uses his cheek to control his speech A.A.C. device.

The first symptoms of A.L.S. that mom noticed were that her tongue and throat felt odd. Not loss of strength in her arms or legs. We (her family) started noticing that she slurred her words, and my brother and his wife talked mom into seeing a doctor. She got her diagnosis in March 2007 and lived 18 months afterward.

The prognosis for a patient with A.L.S. can’t be determined with specificity. Some patients live longer than others, and the reasons why are largely unknown. In two visits to the A.L.S. clinic at Virginia Mason, the doctor there refused to even speculate. For one, he thought knowing lowered the morale of the terminally ill. But the main reason was that there just isn’t any way to predict.

One thing that is known is that people with bulbar onset A.L.S. tend to have symptoms that progress faster than patients with limb onset. Bulbar onset is when the first symptoms show up in the function of swallowing. The medulla oblongata is also known as the bulbar nerves. They control the muscles that effect speaking and swallowing. Typical limb onset A.L.S. affects all the limbs first, sometimes progresses to the bulbar nerves, and lastly affects the diaphragm. For some reason that generally seems to take longer. Bulbar onset affects those nerves first, followed by arms and/or legs, and finally the diaphragm. In mom’s case, the decline in her breathing came concurrently with the loss of function in her arms and legs. And the prognosis, rather than the 2 to 5 years that most limb onset patients might expect, is 18 months to 3 years. Mom’s illness fell at the short end of that range even.

Bulbar onset has a whole different set of challenges than limb onset. Communication and eating are the first set of concerns for bulbar onset patients. Mobility and strength are prime problems for limb onset patients. Mom didn’t lose enough strength in her legs for that to be a serious concern until the last few weeks she lived. Another patient we knew didn’t lose the ability to talk or swallow ever. Because of the differences, in the early stages the same disease doesn’t even resemble itself.


This is one of a number of articles I’m writing to bring up awareness about A.L.S. and to encourage donations to the 2009 Walk to Defeat A.L.S. I’ve set up a donations page to collect money, where donors can claim one of over 100 books as a thank you gift. If you don’t have the money to donate, you can help by publicizing and linking to that page. I’ll be giving away a signed book by Elizabeth Bear for those who help publicize the drive (and more prizes will be forthcoming). More information on that offer can be found here.

Originally published at King Rat. You can comment here or there.