May 11 2005
“I want to take this opportunity to thank you all for being here today to discuss the issues surrounding Amyotrophic Lateral Sclerosis or ALS. This month is ALS Awareness month and today is the ALS Association's Advocacy Day, so I can think of no better time to be holding this hearing.”
“I also want to thank Senator Specter for agreeing to hold this hearing. This is an important issue that affects many of our constituents and I am pleased that so many folks were able to join us here today, including some of my own constituents.”
“Appearing before us today are two ALS patients, a number of their advocates, both local and celebrity as well as a leading researcher in the field and I look forward to hearing from each of you.”
“ALS is a tragic disease that we know little about and have little or no treatment for at this time. I have seen first hand how rapidly this disease robs one's ability to function and how quickly it can take our loved ones from us. Just last year I met with some Alabamians about ALS and I am sad to report that one of the individuals that we spoke about during that meeting is no longer with us. His beloved wife, however, continues her efforts to promote increased research for ALS and I for one, greatly appreciate her efforts on behalf of those suffering from ALS.”
“Federal funding for ALS research is critical and while we have worked diligently to increase funding, more is left to do.”
“I am hopeful that this hearing today will provide a forum to discuss the issues that must be addressed by researchers. There is so much basic research that must first be done if we are to find the cause and cure of ALS. I also believe that we must continue to work to find immediate opportunities to slow its progression and provide patients with every option available.”
“Again, I hope this hearing will serve to increase awareness of ALS and our need to do more on the behalf of ALS patients and their families. Thank you all again for being here today and I look forward to hearing from each of you,” Shelby remarked.
The hearing included testimony from: Story Landis, Ph.D., Director, National Institute of Neurological Disorders and Stroke; Lucie Bruijn, Ph.D. Science Director and Vice President, The ALS Association; Mr. Eric Obermann, Huntsville, AL; Mr. Rob Borsellino, columnist, The Des Moines Register; Mr. Tommy John, former Major League Baseball player; Mr. David Cone, former Major League Baseball player; and Ms. Kate Linder, actress.
ALS is a progressive, fatal disease that attacks motor neurons in the brain and spinal cord, ultimately resulting in a loss of voluntary muscle control, paralysis and death. The average life expectancy for people with ALS is two to five years from the time of diagnosis, and there is no known cause, cure or means of prevention for the disease.
An estimated 20,000 Americans have ALS, with about 5,000 new cases diagnosed each year. The disease most commonly strikes people between 40 and 60 years of age. Ninety percent of ALS patients die within five years of being diagnosed. Risk factors include an inherited genetic defect, which accounts for 5-10% of cases of familial ALS.
There is no known cure for ALS, nor is there a proven therapy that will prevent or reverse the course of the disorder. Therefore, treatment is aimed at controlling symptoms. The only drug used in the treatment of the disease is Riluzole; the first drug that has been shown to prolong the survival of ALS patients by an average of two to three months. There is no specific clinical test that can identify ALS. Diagnosis is made by physical examination, a thorough patient medical history, and neurological testing.