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.
thanks for posting this. I’m going to post a thing on my blog in the next day or two to publicize it.
My special aunt was just (July 2009) diagnosed with ALS (bulpar symptoms started in March 2008) drs. This helps to understand what to expect
my mother has ALS and she struggles with extreme saliva production..Even to the point of gagging her at times..Any suggestions? home remedies? Her doctor has prescribed two different medications but neither has helped, in fact the problem seems to be getting worse..
My friend has bulbar-onset ALS and experiences extreme salavation as well. The best remedy is a suction machine, but she was told that a Dramamine patch behind the ear has been known to help. It has worked for her. She was also told that Botox injections behind the neck are helpful too and should be covered by insurance with a Dr’s perscription. My friend hasn’t tried this option. Best of luck to you and your mother.
thank you so much for posting this. my mom has exactly the same thing, bulbar onset ALS and has received stem cell treatment, electro shock therapy, and rilutek for it.
My husband recently died of bulbar-onset ALS. At age 67 his original diagnosis was dementia/alzheimer’s disease. 6 months later he had a dramatic amount of weight loss, muscle wasting, and then his speech became slurred…fasciculations were noticable in his thigh..the dr. changed the diagnosis to ALS with frontemporal lobal dementia. He lived only 5 months after the diagnosis. For those of you with loved ones suffering from this disease..I hope you have a strong support system. don’t hesitate to as for help with their care…it is a difficult journey for everyone….
My heart goes out to all of those who are personally affected by this disease. I will do what I can to raise awareness and concern. Thank you for your eloquent posting about your mom. My sympathies to you and your family.
Hello, we just received a confirmed diagnosis of ALS for my mom. She started a few years ago. It got very bad to the point where her nutritional needs were not being met and her speech had worsened. She was put on a feeding tube in Nov. 2010. EMG results kept showing negative for ALS. I am realizing now that this is probably b/c her ALS is in the form of Bulbar Onset. I am struggling with this and am so glad to have found this forum
sorry I wanted to edit my reply after I wrote it but I had entered it already.
I wanted to say that my mom started a few years ago with swallowing diffiuculty and it got so bad that her nutritional needs were not being met and her speech was deteriorating.
I have the bulbar onset and noticed slurred speech in July 2011. As of Feb 1, 2012 I can not speak, produce saliva at alarming rate, and have almost lost use of hands and arms. No neck strength. Constant fascicilations, and upper body muscles atrophying daily. In short….Fading fast.