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| This article is from the May 26, 1996 edition of the Eau Claire Leader-Telegram newspaper, in Eau Claire, Wisconsin. |
"I've got to figure out if I want to get on a new drug, if what I've got left is even worth saving."-- Todd Gilbert, who takes Rilutek, a new drug that extends the life of ALS patients a few months
By Susan Barber
Leader-Telegram staff
Richard Todd Gilbert is going to die. A new drug, riluzole, may give him a few more months to live.
And for people with amyotrophic lateral sclerosis -- who generally live about three years after diagnosis -- a lot of living can be done in a few months.
Going on the drug was not an easy choice for Todd, of Eau Claire. In October he traveled to Mayo Clinic in Rochester, Minn., to be examined and interviewed for potential inclusion as part of a medical trial.
"I've got to figure out if I want to get on a new drug, if what I've got left is even worth saving," Todd said then, his shaking hands motioning to take in the motorized wheelchair he uses because of paralysis in his legs.
A longtime smoker, Todd lit up a cigarette sitting outside Mayo Clinic's lobby -- right after Dr. Jasper Daube told him he must quit to be in the study.
"I went to have a cigarette to contemplate the depth of the situation," Todd said, his droll humor coming through.
"I fully realize I have to do it because of (the need for a controlled study)," he said, then added with a laugh, "I'll eat cranberry muffins dipped in butter instead."
Todd agreed to try riluzole, sold only under the brand name Rilutek. Shortly thereafter, in January, the federal Food and Drug Administration approved its sale by prescription.
Riluzole does not cure the fatal neuromuscular disease nor even ease particular symptoms. But it successfully extended the lives of a trial group of patients by an average of three months, and side effects were minimal.
The difficult thing about prescribing Rilutek, says Dr. Donn Dexter, Todd's neurologist at Midelfort Clinic, is that no one knows how ALS starts or how the drug works.
ALS -- commonly known as Lou Gehrig's disease for the New York Yankees baseball player who achieved fame in the 1920s and '30s and died of the disease in 1941-- actually was recognized and documented more than 125 years ago by a French neurologist.
While about 5 percent of ALS cases are hereditary, for most the cause is unknown. Several theories hold that nerve cells are damaged by overexposure to glutamate, a chemical important to normal brain functions. Riluzole aims to block harmful effects of glutamate on motor nerve cells.
Because it's impossible to know what a patient's decline would have been without riluzole, the drug's benefits are hard to pin down.
"You can't tell," Dexter says. "We're to take on faith that the drug works."
For a drug that costs $600 a month, that's a lot of faith, says Todd, who has noted no difference in his declining health.
The limited knowledge on ALS is frustrating for Todd. While he knows ALS is not to be confused with AIDS -- another disease identified by an acronym, -- Todd says the two diagnoses share a common denominator: "They kill ya; that's all they know."
ALS, at least, gives its unwilling hosts some time to prepare. While the disease robs its victims of movement, it usually doesn't affect their minds. One of the greatest minds of our time, British physicist Stephen Hawkings, was deprived of his ability to walk 20 years ago and his power of speech 10 years ago by ALS. But Hawkings continues to impress audiences with Einsteinian thoughts on quantum theory of radiation that he shares through the help of a computer and voice synthesizer.
"I've been impressed with the incredible courage of people faced with these diagnoses," Dexter says. "With a big stroke, for example, they may never say goodbye to anyone. ... (With ALS) you can take time to organize your life, make decisions about how your life will end. If your brain's intact, you can be like Stephen Hawkings if you wish."
For Todd's part, he's using his time to benefit others. He's made advocacy for people with any disability a priority. He's traveled to Madison, Appleton and Wausau for hearings. "The greed out there is what makes me the most upset, and they're actually willing to rob the handicapped to get it," he says.
He actively supports ALS research. "The apathy on ALS is almost total unless you have it or have a direct blood relative with it. The general public thinks I'll never get it or my kids," says Todd, who strongly disagrees with the reported 30,000 cases of ALS nationwide. Todd puts the number closer to 145,000 and believes there are clusters of ALS in northwest Wisconsin.
Todd also is a peer counselor with the Center for Independent Living for Western Wisconsin in Menomonie and attends meetings of Eau Claire County's Special Transportation Board. He's active in the Northwest Wisconsin ALS Support Group, but he says its more for others than himself.
"I'm not there to get support; I'm there to give it," Todd says. "I'm bullheaded and aggressive. I give them hope, and I make them laugh."
That's evident at the May 5 meeting. About 14 people sit in a room at the Chippewa Valley Bible Church in Chippewa Falls. They come from as far east as Wausau and as far north as Trade Lake.
Some are there in wheelchairs because they have ALS; some are pushing chairs of their loved ones with the disease. A mother and two children finally are feeling strong enough to attend since their husband and father died of ALS last August.
When Todd comes in, a little late, he looks dashing in a cowboy hat and bolo tie. It's not long before he cracks a joke, and laughter breaks the group's polite chatter.
"If you can't look at life facetiously, how can you face it?" Todd asks rhetorically. "I could be the first wheelchair comic. I can't be a stand-up comic."
They start by sharing news of the past month. Todd is pleased to come bearing computer equipment the group purchased for another member. Todd, a self-taught computer expert, offered his assistance in purchasing and setting up the new system.
Group leader Sally Krohn interrupts Todd's technospeak. Every third month the group brings in a speaker, and on this day Dr. Gareth Parry, a Minneapolis neurologist, will discuss new medication.
With Rilutek, "It's the first time I've been actually able to treat this disease," Parry says. "I've been treating patients but doing nothing to change their disease."
At his clinic in Minneapolis, "the whole mood has changed," Parry says. "There's a sense of urgency."
And that's not just because of Rilutek. Parry describes other drugs being tested that slow decline in breathing and strength. New ways of delivering drugs to the brain also are being researched.
"I think we are going to see a tremendous explosion in the drugs that may help ALS," Parry says.
"It's a very exciting time scientifically for patients with ALS. We have hope for the first time in 100 years. "
Hope, however, has its limits.
"We would regard as a fabulous success any drug that stopped progress (of the disease) and left you where you were," Parry says.
"Where you were."
Looking around the room, the impact of that statement means different things to people at different stages in the disease's relentless toll.
Attending a support group for people who get worse instead of better can be difficult for all involved. Krohn, the group's leader who also is a physical therapist, says it's hard for her too. But she's confident the shared emotional support and coping skills outweigh the loss the members feel.
"You see the benefits so much that it makes all the difference," Krohn says. "You see they're going to die whether I'm around them or not so if I can help, it's worthwhile."
The members quiz Parry about different drugs and vitamins. Like many people touched by a serious illness, they've become their own best experts, analyzing information on their disease. Todd tells Parry his cluster theory, but Parry is unconvinced. "There's a misconception that for every 100,000 people ... (ALS) should be equally distributed," Parry says. "That's not true. It doesn't necessarily mean there's something there or it doesn't necessarily not mean there's something there."
He gives the example of the San Francisco 49ers who have had three teammates with ALS. "It's a statistical phenomenon," Parry says.
It's Todd's turn to be unconvinced. But he does tell Parry something the doctor already knows -- that ALS patients trade drugs with each other to try to maximize the relatively short time they have after diagnosis.
"Through the Internet or access with each other, people are doing multiple-drug studies on their own," Todd says.
It's illegal for prescription drugs to be used by anyone other than the person for whom they were prescribed. It's risky too because of drug interactions and possible allergies.
Todd won't confirm or deny whether he's ever exchanged medications with anyone.
But he tells Parry what many ALS patients feel, "I've got nothing to lose."