Sometimes it's easy to pinpoint the moment things go wrong.
For fitness executive Augie Nieto, it started with a backache in 2004. Other signs followed: weakness, slowed speech, involuntary muscle spasms in his right arm, difficulty shaving. After being unable to hold on to a tow-rope while water skiing in early 2005, Nieto went to the Mayo Clinic.
Several tests and a few days later, Nieto returned home to Corona Del Mar, Calif., with a dreadful verdict. Doctors were fairly certain he had amyotrophic lateral sclerosis, also known as ALS, or Lou Gehrig's disease. He was 47.
Augie's Quest, co-written with T.R. Pearson, is about Nieto's battle with ALS and his mission to find, if not a cure, at least an effective treatment for the disease that's killing him.
ALS is a fatal disease that attacks motor neurons in the brain and the spinal cord, leaving the neurons unable to control muscle movement. As the neurons lose function, the patient becomes paralyzed. Because ALS victims go on thinking, seeing and hearing as well as they did before ALS, they are essentially trapped in a degenerating body. Most patients die of respiratory failure within three to five years.
Not only is there no cure, there is only one FDA-approved drug for ALS, and it, at best, extends life for a mere two or three months.
Nieto, who built a fortune by popularizing the Lifecycle exercise bike, had the means to seek no less than seven second opinions from coast to coast.
A few short months and an unsuccessful suicide attempt later, Nieto digested what he learned.
No one knew what caused ALS, and research efforts were scattershot, underfunded and largely conducted in an uncoordinated vacuum, he decided. "That's no way to run a business," he says.
Nieto doesn't dwell on the larger issue of the state of health care in the USA, but its dysfunction is revealed time and again.
ALS is classified as an orphan disease, meaning there are fewer than 200,000 cases annually. In fact, there are only about 30,000 Americans living with ALS at any given time. The 1982 Orphan Drug Act gives market advantages for companies willing to develop drugs for orphan diseases, including no limit on pricing to ensure profitability. This gives pharmaceutical companies a powerful incentive.
Nieto believes he can use this to convince drug companies that ALS can be a profitable disease. "Available market drives everything," Nieto says. "This gives us the leverage to fund construction of a real drug."
Who's us? Nieto has joined forces with a few ALS groups. By essentially turning a fitness-industry convention into a fundraiser, Nieto was able to raise more than $1 million and prove his clout to the Muscular Dystrophy Association, which has an ALS division.
Nieto has also proclaimed himself as a face of ALS, saying that as in business, it's important for the disease to have a "brand." Now in a wheelchair and experiencing speech difficulties, he's appeared in The Wall Street Journal, on the Today show and on Jerry Lewis' Labor Day MDA telethon. Mitch Albom, who profiled his professor Morrie Schwartz's ALS ordeal in Tuesdays with Morrie, writes the introduction to Augie's Quest.
Another visible ALS patient, Stephen Heywood, featured in the compelling documentary So Much So Fast, died of the disease a year ago at 37. Heywood's brother, James, formed a slightly renegade, ALS-focused non-profit biotechnology company after Stephen's 1998 diagnosis in a bid to save his life. That group, now called the ALS Therapy Development Institute (ALSTDI), tests drugs on genetically engineered mice and has reviewed every major ALS drug study conducted by academia.
The two groups — ALSTDI and MDA — had agreed to start over with ALS research, working to dismantle the disease at a molecular level, at an estimated cost of $60 million. Enter Nieto and his fundraising muscle.
Augie's Quest's third-person voice serves to detach much of the emotion from a heartbreaking illness, while still managing to convey the urgency of finding a breakthrough.