Copyright 2004 The Austin American Statesman
Austin American-Statesman (Texas)
October 24, 2004 Sunday
SECTION: NEWS; Pg. A1
LENGTH: 1212 words
HEADLINE: Defying his limits;
Austinite stubbornly seeks options in fight against Lou Gehrig's disease
BYLINE: Mary Ann Roser, AMERICAN-STATESMAN STAFF

Burly, outdoorsy and hard-driving, Stuart Cohen of Austin couldn't figure out why, at age 45, he was suddenly tired all the time. Then he noticed his right middle finger twitched involuntarily at times.

"Being from New York, people joked that it was from overuse," he said with an accent that 24 years in Texas can't disguise. He laughed.

Laughter doesn't come as easily as it once did. Nothing does.

In February 2001, a few months after Cohen noticed the symptoms, doctors diagnosed him with amyotrophic lateral sclerosis, or ALS. Also known as Lou Gehrig's disease -- for the New York Yankees legend who died of it in 1941 -- the disease attacks nerve cells in the brain and spinal cord that control the muscles. Patients grow weaker as their muscles waste; they become paralyzed. Ultimately, they can't breathe. Most live two to five years.

Cohen, now 48 and nearly a decade younger than the average ALS patient at diagnosis, has a slow-moving form of the disease and no family history of it. Since his diagnosis, he has lost strength and begun wearing a calf-high brace on his right leg to help him walk. He had hoped experimental drugs from clinical trials might slow -- maybe even halt -- his decline.

But this doggedly independent bachelor who once made a good living selling high-end men's clothing said his hope is gone. The two drug trials he is participating in are ending, and he is not eligible for another drug he desperately wants to try. He's had the disease too long. Most trials won't admit patients who have had ALS more than two or three years because of the risk they will die during the trial, Cohen's doctor said.

'A slap in the face'

Cohen is known as an activist in ALS circles. He encourages other patients and is a regular at support groups and fund-raisers for ALS research. He took part in Saturday's ALS walk in Austin and had musician friends play for the crowd at St. Stephen's Episcopal School.

"I can't sit back and wait to die," said Cohen, a singer/songwriter who wears his brown hair in a ponytail. "I fought and lobbied for these trials. To be excluded is a slap in the face."

Dr. Jeffrey Rothstein, who has conducted many ALS trials, said the goal isn't to exclude anyone but to measure whether the drug is working. People such as Cohen who have the slow-moving form of ALS won't change much during a yearlong trial, and "we won't learn anything from them," said Rothstein, a professor of neurology and neuroscience at Johns Hopkins University in Baltimore.

Cohen said others who have been diagnosed within the past two or three years could have a slower form of the illness, so he doesn't buy Rothstein's argument. He thinks scientists should be studying people like him to better understand ALS.

"I don't have three years," Cohen said. "Now is where I want to stabilize. I don't want to stabilize in a wheelchair."

His doctor, Carlayne Jackson, an associate professor of neurology at the University of Texas Health Science Center at San Antonio, said Cohen makes excellent points, but she agrees that it is best to study drugs in people whose disease is progressing faster in order to see whether the drug is helping alleviate the symptoms.

Any drugs proven to work would probably be put on a fast track for other patients, said Jackson, an ALS researcher who heads the South Texas ALS Clinic, one of three in Texas. Also, gene therapy -- targeting defective genes that have been identified as contributing to the disease in some patients -- is stepping closer to reality, she said. Rothstein expects trials next year.

"This is an incredibly devastating disease, especially for someone at the prime of their life," Jackson said of Cohen. It's people like him who motivate people like us to work harder."

Although 5 percent to 10 percent of ALS patients have a relative with the illness, scientists don't know what causes most cases. Nor is there a cure for the disease, which is expected to become more prevalent as baby boomers age. About 30,000 Americans, including about 70 or 80 in the Austin area, live with ALS today.

'Time is marching on'

Cohen measures his decline in subtle ways. For example, the bottle top he screwed on two months ago is too hard to open now. He has trouble holding onto things and uses a rubber tube over his toothbrush to better grasp it. He relies on an ultra-thin guitar pick because it is more flexible and doesn't make him as tired when he strums the strings.

He can't take the one ALS drug that the Food and Drug Administration has approved, Rilutek, because of side effects.

Under Jackson's direction, Cohen is finishing a trial of creatine, a dietary supplement that might or might not help muscle fatigue, and a trial of Celebrex, a drug commonly used for arthritis. The Celebrex trial results should be out this week, said Jackson.

Cohen said he didn't know whether Celebrex or creatine is helping. He still takes them, along with vitamins E and C and other antioxidants.

Not everyone in a clinical trial gets the trial drug. Some get a sugar pill, or placebo. Most times, the trial drug is ineffective, Rothstein said. Sometimes, it is harmful.

Stem cell therapy -- which could help rejuvenate damaged cells -- holds the best hope, Rothstein said, but that won't be available for a long time.

Dr. Lewis Rowland, a professor of neurology at Columbia University Medical Center in New York, said, "I don't know of any drug out there in clinical trials that's promising, but we can take better care of patients living with ALS."

Advances in technology and medicine are extending patients' lives and making them more comfortable, said employees of the ALS Association's South Texas Chapter. Machines help patients breathe at night, feeding tubes help them eat, and more aggressive drug therapy, such as using a form of Botox, helps stop patients from drooling or choking.

Meanwhile, Cohen said the disease has gradually moved to the left side of his body, and despite his frequent jokes, he's depressed.

"Every day I realize I can't do something else . . . and time is marching on," he said.

A constant reminder

Cohen's garage holds camping, caving and fishing gear he can no longer enjoy. He can't work and is now on Medicare. In one of his songs, "Living in Limbo," he sings, "While I may look fine to the naked eye, underneath there's a storm going on."

On Wednesday night, his 20-minute open-mike set at Gino's Italian Grill left him sweating and with tired arms.

His cat and his music make him happy, he said, but playing guitar "reminds me of my limitations."

In a back room of his North Austin house, he sits under posters of Frank Zappa and spends hours on the Web every day, combing it for information about ALS and news about drug trials. He subscribes to numerous online mailing lists and news and medical sites, and brings information to his doctors. His appetite for knowledge about ALS seems insatiable.

Cohen doesn't know what else to do but search.

"We all want to make a mark in this world," he said. "My mark suddenly has a time stamp on it."

For more information about ALS programs and services in Central Texas, contact the ALS Association's South Texas Chapter toll-free at (877) ALS-HOPE (257-4673) or see the Web site at www.alsa-south-tx.org
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