WHEN PAULA OLNEY became anxious about a medical complication in the birth of her grandson last month, she sought reassurance from her husband, Dr. Richard Olney of Corte Madera.

Lou Gehrig’s disease — amyotrophic lateral sclerosis — has robbed Dr. Olney of virtually every voluntary muscle function, yet he remains a pillar of strength for his wife and their two adult children.

“He’s the one I go to to calm me down,” his wife said. “He can’t stand up, can’t walk, can’t talk, can’t chew. But he can think. despite his incapacitation, he still helps all of us with the major decisions we make in our lives.”

When he was diagnosed with the disease in 2004 at age 55, Olney’s illness garnered national attention because of a cruel irony. at the time, he was working as a neurologist and director of the University of California at San Francisco’s ALS Treatment and Research Center, which he founded in 1993.

“That’s why it was such a big news story,” his wife said. “It was like the brain surgeon himself getting a brain tumor.”

Strapped in a wheelchair and able to communicate only with the assistance of a computerized speech synthesizer, Dr. Olney’s strength is not immediately apparent. it manifests itself gradually as one watches him doggedly manipulate the synthesizer. he painstakingly constructs sentences by moving his gaze over a computer tablet and selecting, sometimes a word or phrase, but usually a single letter at a time.

“ALS is more common than most people think,” Olney said, using the machine. the halting voice that emerges is his own, prerecorded with the prescience that came from watching countless patients succumb to the deadly disease.

According to the National Institute of Neurological Disorders and Stroke, only about 20,000 to 30,000 people in the United States have ALS, even though an estimated 5,000 people in the country are diagnosed with the disease each year.

Dr. Catherine Lomen-Hoerth, who took over as director of UCSF’s ALS Treatment and Research Center after being trained by Olney, said the number of people with ALS is misleading because the disease is so lethal.

“If people lived longer with ALS, it would be as common as multiple sclerosis,” Lomen-Hoerth said. Multiple sclerosis affects more than 350,000 people in the United States and 2.5 million worldwide.

Most people who have ALS die from respiratory failure, usually within three to five years from the onset of symptoms. About 10 percent of ALS patients, however, survive for 10 or more years without use of a ventilator.

While a ventilator might extend his life for years, Olney has already decided against it.

There is no cure for ALS. in clinical trials, the only drug approved for treatment, riluzole, has prolonged survival by several months, mainly in patients who have difficulty swallowing. Scientists have so far been unable to determine what causes the disease, which in 90 to 95 percent of cases seems to occur at random with no clearly associated risk factors. About 5 to 10 percent of ALS cases appear to be inherited.

In the case of Olney, who has no known incidence of the disease in his family, symptoms progressed quickly at first.

“Richard’s an anomaly,” his wife said, “He lost his ability to walk, his voice and fine motor skills, like typing, all in about one year. Then he plateaued.”

Rather than dwell on what he can no longer do, Olney has stayed focused on what he can do, as well as anyone in the country. with the assistance of his son, Nicholas, who recently graduated from medical school, Olney is racing against the clock to complete a research paper on ALS using data he accumulated before he became ill.

The ticking clock almost stopped in March, when breathing problems landed Olney in UCSF’s intensive care unit for several days. Since then, he has been forced to take nourishment through a tube to avoid choking.

“Then he got this horrendous cold that lasted five weeks,” his wife said. “We know he’s living on borrowed time, and every day is important.”

Olney said there are two reasons he is determined to complete his research paper.

Firstly, he said, “I want to write a paper with Nick.” also, he said, “I think there are many forms of ALS, and this paper documents several different forms.”

Olney hopes that the paper will demonstrate that neurophysiological tests conducted in the early stages of ALS can be utilized to predict how quickly patients will lose muscle control.

Lomen-Hoerth said, “He’s a very smart man and he enjoys being active and being able to contribute to science. I think that keeps him going.”

In the early stages of his illness, Olney himself became a guinea pig in a UCSF clinical trial of a promising ALS drug. the drug, however, proved ineffective, and Olney resolved himself to his fate.

“Some people get wrapped up in all these alternative treatments,” said Nicholas Olney. “Dad quickly focused on making the most of every day.”

Oselinda Ames, one of Olney’s caregivers, met and married a former ALS patient of Olney’s, David Ames, after Ames traveled to Abadiania, Brazil, to visit a spiritual healer who calls himself John of God. the couple were later married and returned to the United States where Oselinda cared for Ames until his death.

“I’m happy to work with Rick because he reminds me a lot of David,” Ames said. “David enjoyed every single day.”

When he’s not working on his paper, Olney exchanges emails with other ALS patients, updates his Facebook page, watches movies, listens to books on audio tape and attends concerts and plays. his wife recently took him to see a new favorite of hers: Lady Gaga.

“He’s not angry and he’s not depressed,” she said. “Can you believe it?”

Lomen-Hoerth said many ALS patients cope with their disease surprisingly well.

“I’m continually amazed by my patients, and their incredible endurance,” Lomen-Hoerth said. “In general, ALS patients have a very low incidence of depression and tend to be quite hopeful. I think the lack of pain helps.”

Although Olney is not traditionally religious, he said spirituality is one source of his strength.

“I feel God’s presence especially in nature,” he said.

And while Olney said his greatest pleasure these days is spending time with family and friends, he also has his dreams.

He said, “I can run, walk, hike and jog in my dreams.”

Contact Richard Halstead via email at

lou gehrig’s disease facts

• Amyotrophic lateral sclerosis, or ALS, is a disease of the nerve cells in the brain and spinal cord that results in a progressive loss of voluntary muscle movement.• ALS affects approximately five out of every 100,000 people worldwide.• in at least 90 percent of cases there are no known risk factors. About 5 to 10 percent of ALS cases appear to be inherited.• ALS leaves the senses unaffected and only rarely affects bladder or bowel function, or a person’s ability to think.• There is no known cure for ALS, and sufferers rarely live beyond 10 years without aid of a ventilator.

Sources: National Institute of Neurological Disorders and Stroke, National Center for Biotechnology Information