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ALS patients become prisoners in their bodies
By Jessica Ravitz
The Salt Lake Tribune


Lost to Lou Gehrig's diseaseOfficially called amyotrophic lateral sclerosis, but often referred to as Lou Gehrig's disease after the famous New York Yankee who died of it in 1941, ALS is a degenerative disorder that breaks down and eventually kills specific nerve cells. The two affected types of cells, which control muscular movement, are referred to as upper motor neurons and lower motor neurons.
Upper motor neurons, located in the brain, send messages or nerve fibers down the spinal cord to lower motor neurons that, in turn, fire off instructions to muscles. The degeneration and loss of upper motor neurons commonly leads to difficulties with swallowing, speech and movement; increased yawning; urinary urgency; and ease of laughter and tears. The symptoms associated with loss of lower motor neurons generally include muscle weakness, shrinkage or atrophy, cramps and twitching, as well as fatigue.
The brain may stay active and sharp, but often the body cannot, and the ability to communicate plummets. In essence, patients can become prisoners in their own bodies.
Eventually, the progressive disorder weakens breathing muscles, leading to respiratory failure. Mark Bromberg, the neurologist who oversees University of Utah's ALS Clinic, says 50 percent of patients die within two to four years of symptom onset.
"No two people are the same," the doctor says.

The cause of ALS is unknown, although
unproven theories abound. About 5 percent of cases have a familial link, but ALS hits 95 percent of patients at random. The disease affects roughly 30,000 Americans, every year. The median age for symptom onset is 57, but Bromberg - who's been at the U. for more than 12 years and who sees about 45 new cases annually - remembers one patient who came in at 20.

So far, the FDA has approved only one drug treatment for ALS, but its effectiveness is limited. Patients who can afford to take Rilutek, or the generic riluzole, generally live for an extra three to five months, the doctor explains.
There is no known cure for ALS, but Bromberg hopes time and research will discover one. An obstacle, however, is that the special and overwhelming challenges faced by ALS caregivers tend to burn them out, which makes advocacy and fundraising more difficult.
"Unlike cancer or other disorders," he says, "caregivers frequently want to distance themselves as much as possible."

http://www.sltrib.com/news/ci_4966120