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Researcher with ALS finds solace in expertise
Sabin Russell, Chronicle Medical Writer

Saturday, March 22, 2008

Researcher with ALS finds solace in expertise
Dr. Richard Olney and his wife, Paula, are treasuring a plateau in the progression of his ALS, or Lou Gehrig's disease. Chronicle photo by Lacy Atkins


From the living room of Dr. Richard Olney's Corte Madera home, sliding glass doors offer a wide-open view of the tidal marsh in front of his house, of passing clouds and of the shorebirds that fly in and fly out.

Inside, the man who once rode mountain bikes and ran triathlons sits nearly motionless in his wheelchair. He watches the world with the same intense intellectual curiosity that made him one of the nation's leading experts in ALS, or amyotrophic lateral sclerosis - popularly known as Lou Gehrig's disease.

Since 2004, ALS has been slowly killing him.

Of unknown origin, the disease is marked by the destruction of motor neurons, the nerve cells that trigger and control muscle movement. It is a progressive and invariably fatal illness. Patients are fully alert to the end. They retain the sense of touch and mercifully experience no abnormal pain, but within two to four years, most patients are unable to move, swallow or breathe.

The terrible irony of Olney's self-diagnosis generated news stories around the world. After colleagues confirmed that he had the disease, he took the same advice he had offered to more than a thousand of his own patients: He put his financial affairs in order, lined up home-based nursing care and prepared for death.

"He hasn't taken a moment to feel sorry for himself or despair," said Dr. Catherine Lomen-Hoerth, director of the ALS center at UCSF, which Olney headed until his illness. He was her colleague and mentor and had an office just down the hall. Now, he is her patient.

Olney's illness began as a weakness in his right knee. For some, the descent is slow and gentle, for others it is steep and fast. By 2005, it was apparent that Olney's was the latter kind.

Stability after fast decline
He lost the ability to walk, to move his arms or legs and then to speak. Doctors thought he had only months to live. But after ALS robbed him of all muscle movement but a slight ability to nod his head, the disease mysteriously stabilized. His condition has remained unchanged for a year and a half.

"I think I have an unusual variant of ALS," Olney told a visitor to his home.

His words were not spoken, but spelled out on a large white placard - a handmade communication system that works like a Ouija board. Laid out across his lap are the letters of the alphabet; the numbers zero to 9; the answers "yes" and "no;" often-used words such as "can," "can't," "feel," "lift" and "want;" and the names of his wife, Paula, his son, Nick, and daughter, Amy.

Olney spells out his thoughts readily, rapidly, using a laser pointer mounted on a pair of eyeglass frames. A subtle turn of the head, a quick nod up or down, and the sharp red light of the pointer hovers over a new word or letter.

His wife, a kidney dialysis nurse who has been married to him for 33 years, is grateful for the reprieve. "We've quit thinking in terms of his dying," she said.

"I have not," Olney quickly spelled out, a mischievous twinkle in his eye.

Paula Olney said the horror of the diagnosis has been harder for her than for her husband. "Thank goodness, he knew everything we'd need," she said.

Knowing what to expect
"It's easier knowing what to expect," Olney said, working his laser pointer. "The best-adjusted patient I worked with had familial ALS. She was a role model for me."

Although the cause of ALS is as mysterious today as it was the 1800s, when the disease was first identified, about 5 to 10 percent of cases are inherited. So families that have experienced the illness are best prepared to deal with it.

Olney's case was not inherited. No one else in his family has suffered from it. He stressed that his illness was not contracted from caring for patients. ALS is not contagious, but it is surprisingly common. About 1 in every 1,000 Americans - more men than women - will develop it in their lifetime. That puts it on a par with better known diseases, such as multiple sclerosis. Yet because ALS is so rapidly fatal, the number of patients living with the disease at any given time - about 30,000 in the United States - is one tenth the number with multiple sclerosis.

Before he lost the ability to walk, Olney arranged to equip his home with ramps and a hospital bed. He bought a special van from the family of a patient who had died. The Olneys bought a big-screen television and rented lots of video, but they still go out to movies, live theater and music concerts.

"We've settled into our lives," Paula Olney said. "The first six months were terrible, because I noticed some new deficit every week. This plateau makes it easier to adjust."

About 10 percent of ALS patients can live 10 years or longer without needing a ventilator. Olney is realistic that chances are slim he will be one of them. He was diagnosed at the age of 55. Now he is 60. "There are exceptions, but generally the younger you are diagnosed, the longer you live," he said.

Olney has formally rejected use of a ventilator. When his breathing fails, he will die.

A failed clinical trial
Only one drug is approved to treat ALS, and in January 2005 Olney was first to sign up for a clinical trial he had designed prior to his illness. It would test whether an AIDS drug, ritonavir, might slow the disease. Olney did not know during the trial whether he was given the real drug, or a placebo, or another medication, hydroxyurea. Both the active drugs - already approved for entirely different uses - were thought to interfere with processes involved in the destruction of nerve cells in ALS patients.

After six months, however, the ritonavir experiment was halted when preliminary data showed that the ALS patients who received that drug might be faring worse. It turned out that Olney was one of them.

While Olney cannot work in his old lab, he keeps up with the literature in the field of ALS research, and he continues to publish. In January, the Journal of Life Sciences published his latest article: "When the Doctor Becomes the Patient."

"I became anxious a few times when thinking about how disabled I would become," he wrote - using a modified computer mouse and specialized software. "My anxiety was relieved when I followed my own advice. I focused on what I was able to do today and realized that more disability would come on gradually, meaning that I would have time to adjust to it."

Olney has a close-knit and caring family, which surrounds him with love and support. He said he is relieved that he has had time to prepare them for a future when he is no longer there. "Prepare for the worst, but hope for the best," he said.

Paula Olney at times is visibly pained by the horrible turn of events. Also, home health care is very expensive, but she feels fortunate that a long-term care insurance policy covers about half the $5,000 a month required. She and their two children continue to be inspired by her husband's tenacity. "He is living out of sheer will," she said.

E-mail Sabin Russell at srussell@sfchronicle.com.

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