[BobbyB]

Thursday, December 7, 2006
The world's strongest man
Augie Nieto is losing muscle to Lou Gehrig's disease. Now, he's tougher than ever.


REDEDICATION OF LIFE: Augie Nieto, 48, founder of Life Fitness poses for a photo at his house in Corona del Mar on Wednesday.
By GAUTAM NAIK

The Wall Street Journal
While water-skiing in Vietnam's Mekong Delta about two years ago, Augie Nieto fell several times. His muscular arms suddenly went slack and he couldn't haul himself up.

Nieto, of Corona del Mar, was diagnosed with amyotrophic lateral sclerosis, or ALS. About 30,000 Americans have the ailment, also known as Lou Gehrig's disease. They progressively lose control of limbs, speech and the ability to breathe. Most die within five years.

The diagnosis was a shock to Nieto, who had built his life around exercise. As a young man he founded what became Life Fitness, one of the world's biggest makers of exercise equipment. "I remember wondering what I had done to deserve this death sentence," says Nieto, 48.

After an initial bout of despair, he decided to fight. He assembled a team of doctors, geneticists and ALS experts and gave them a mission: Identify the genetic basis of the disease in order to seek a treatment.

In just nine months, the effort has yielded some clues to a better understanding of ALS. Its findings were released at an ALS conference in Japan last week. Though the chances of halting Nieto's deteriorating condition are slim, researchers already are using the new understanding to study existing drugs, to see if any might help him and others.

Nieto is one of a wave of wealthy patients bankrolling a new technology to jump-start the search for a cure: machines that sift through DNA at lightning speed. They identify tiny genetic differences that show up more often in people with an intractable illness than in others. Finding these differences is a first step toward understanding what underpins a disease, which can pave the way for new treatments.

Using the new machines is costly, and government funding is scarce. Now, privately funded gene quests are under way in a number of maladies, including autism, Parkinson's disease and Alzheimer's.

The Cure Alzheimer's Fund, sparked by three families touched by the disease, raised $3 million for a gene search conducted by Rudolph Tanzi, a geneticist at Harvard Medical School. Today, says Tanzi, science has "the ability to find the key genetic players in a disease. It's expensive, and families are stepping up to the plate." In the case of ALS, at least two other privately funded studies besides Nieto's are in progress, at Harvard and Johns Hopkins University.

It's too soon to tell whether the gene approach will pay off. Some efforts have been disappointments. The Michael J. Fox Foundation for Parkinson's Research paid close to $3 million to scan genes of Parkinson's patients. An initial report identified 13 gene suspects, but follow-up studies didn't confirm a role for them.

Some doctors worry that the research is too rushed and could even harm patients – such as by prompting hopes that any discoveries will rapidly translate to treatments. Others say an approach focused on genes may overlook environmental and lifestyle factors that also can play a role.

Like Nieto's search for ALS genes, his quest to build a fitness business began with a personal problem: He was a chubby teenager. To keep the weight off and to help others do the same, he opened a gym while he was at Claremont McKenna College.

Soon he learned about a stationary exercise bike, developed as a medical device, which could crudely record the user's heart rate. He sold his gym after college, bought marketing rights to the bike, and drove 5,000 miles across the country in a motor home trying to get health clubs to buy the yellow "Lifecycle." He sold just 11 in nine months.

Unfazed, he teamed up with the bike's developer in 1980. They sent free bikes to 50 big health clubs. The clubs installed them, they proved popular, and Life Fitness of Chicago grew rapidly. In 1997 Nieto, who then co-owned the business with an investment fund, sold it for $310 million. He traveled the world indulging his taste for physical adventure – fast cars, scuba diving and snowmobiling.

In mid-2004, he found himself struggling to lift his usual weights. After he had more trouble during the water-skiing trip months later, doctors diagnosed ALS.

"Your first reaction is denial, then anger," he says. "Then you get ready to fight."

Nieto met with a several doctors and ALS researchers, who had varying theories about his condition. He was given an antibiotic, which caused sun exposure to turn his skin several shades darker. He was prescribed the breast-cancer drug Tamoxifen, which gave him hot flashes. Neither seemed to help him.

Taking matters into his own hands, Nieto drew up plans for an ALS foundation, Augie's Quest

A year ago, Nieto visited Translation Genomics Research Institute, or TGen, a nonprofit group in Phoenix that uses gene screening to seek targets for various diseases. A TGen senior researcher, Dietrich Stephan, told Nieto a screen could be done quickly.

"After banging your head against the doors of academics, it was like a breath of fresh air," Nieto says.

His foundation, through the MDA, offered $650,000 to TGen for a hunt for ALS-related genes. TGen matched the amount, and the three groups agreed to a deal in March 2006.

In late September, a dozen members of the team met at the MDA's Tucson headquarters to study these genes. Using their laptops, with each scientist initially looking at just one of the 50 genes, they spent two days scouring scientific literature for clues.

"We turned the cell phones off and really tried to figure this out," says Stephan. The effort narrowed the 50 to about 25 genes that appeared to play a bigger role.

Nieto takes the only U.S.-approved ALS drug, Rilutek, which can prolong survival for several months, and also a drug that can reduce mood swings in patients with ALS or MS. His wife, Lynne, says of the recent TGen findings: "It's the first time I've had hope."

Nieto's doctor asked TGen to retrieve Nieto's genetic data after the gene screen. Stephan did so, but here a problem cropped up. Stephan soon realized that federal law requires keeping the identity of samples in such research secret. "I thought we could just flip him his [gene data]but we can't do that," he says.

Nieto and his physician persisted, Stephan says, so TGen is trying to help them out by having the same gene test done on Nieto's blood again, in another lab. "We are trying to accommodate [him]as best we can," Stephan says. "We want to give something he can sink his teeth into."

Nieto sold his silver Ferrari after he was diagnosed with ALS. To avoid stairs, he recently had an elevator installed in his cliff-side house. He still exercises an hour a day but can no longer play golf or feed himself.

Nieto says his mind is as sharp as ever and he isn't stopping. His latest project: to financially kick-start a new institute that focuses on cutting-edge ALS research.

"The business of ALS is a blast," Nieto says. "The disease sucks."


http://www.ocregister.com/ocregister...le_1375931.php