Source: http://www.detnews.com/ 2005/health/0504/07/A07-130530.htm
By James Ricci / Los Angeles Times Luis Sinco / Los Angeles Times
Understanding the disease as well as he does, Dr. Richard Olney, with the help of his wife, Paula, acquired the equipment that would make his life more tolerable as the disease progressed.
SAN FRANCISCO -- Dr. Richard Olney knows nearly all there is to know about amyotrophic lateral sclerosis, Lou Gehrig's disease. He knows that over a couple of years, it destroys nerves that control muscles, gradually entombing the mind alive in a useless body until, unable to move, speak, swallow or breathe, the patient dies. The founder of the ALS clinic at the University of California-San Francisco and a national figure in the protracted war of research against the mysterious ailment, Olney knows it strikes a victim without warning in the bloom of adulthood. As a doctor noted for the exceptional compassion he shows his patients, Olney also knows the sorrow and dread they and their families must bear. All this knowledge, Olney said, is a comfort now that ALS is ushering him toward his own death in a matter of months. The disease has tightened its grip on him with unusual speed, as if in revenge. It has consigned the 57-year-old runner, bicyclist and hiker to a wheelchair, but it can't surprise him or deter him from trying to make the most of his remaining life. "Maybe a small part of me resents that I'm dying at a relatively young age," he says in a voice the disease has made slow and thin. "But whenever I start thinking of getting it at 55, I think of all the people I have known who have gotten it in their 20s, 30s and 40s."
Olney was known among the young doctors who came to UC San Francisco to learn from him as a soft-spoken, matter-of-fact teacher. No matter how ironic his fate seems to others, he regards it in a practical and analytical way. Every year, one of every 50,000 Americans is diagnosed with the disease, which is not contagious. A person's lifetime risk is about one in 1,000. Given the number of people working in the ALS field, Olney estimates, one of them should come down with the disease every five to 10 years. No one among ALS researchers and caregivers interviewed, however, knows of any other of their number who has been afflicted.
Lives change Understanding the disease as well as he does, Olney, with the help of his wife, Paula, lost no time acquiring the equipment that would make his life more tolerable as his ALS progressed. They had a wheelchair ramp built at their ranch house. They bought a van equipped with hand controls from the family of one of Olney's deceased patients. They had a ceiling track installed from their bedroom to the bathroom in anticipation of the day when he would have to be moved in a sling. They acquired a bed that would automatically turn him when he could no longer turn himself. They bought a motorized wheelchair.
The disease quickly dominated his life. Late last summer, Olney relinquished the directorship of the ALS clinic to a protege, Dr. Catherine Lomen-Hoerth. In late September and early October, while his speech was still relatively steady and strong, he spent 15 hours recording almost 1,700 phrases and sentences in a pair of computer voice programs. They were mostly personal statements -- "Please tell me if you don't understand what I am saying" and "I have Lou Gehrig's disease and cannot speak clearly" and "I love you, Paula" -- for playback after he loses the ability to speak.
By last Thanksgiving, he no longer could manipulate the controls of the van. By Christmas, dragging himself 20 yards on a walker took half an hour. At night he uses a face mask that's part of a device that ensures he receives enough oxygen while asleep. Health declines Of special concern to Lomen-Hoerth, Olney's personal doctor, was the speed with which his breathing power declined. Over about four months, it had dropped from 122 percent of normal to 80 percent, about three times faster than expected and a grim indicator of how much longer he has to live. "When breathing gets down to 50 percent, we usually call hospice in, and after that, it's not likely a person will live six months," Lomen-Hoerth said.
These days, Olney's 5-foot, 10-inch, 190-pound frame must be fitted into the ceiling-hung sling to be ferried from bed to bathroom. Olney can still feed himself, but swallowing thin liquid, which requires a great deal of throat-muscle control, is difficult. His ability to speak has declined markedly in the past month.
Before his diagnosis, Olney wrote a protocol for a new clinical study in which ALS patients would take a combination of an anti-HIV drug and an anti-cancer drug. After he learned he had ALS, he was the first patient to volunteer for the study and was designated "Patient 001." He recently received his first dose. Neither Olney nor his doctor will be told if he's receiving the drugs or placebos.
Earlier this month, however, Lomen-Hoerth found that the precipitous decline in Olney's breathing capacity seems to have slowed, for now. From now until the end, Olney said, he will relish the care his family lavishes on him. Although he will accept a feeding tube when he loses the ability to eat, he will refuse to be placed on a ventilator, even though doing so could keep him alive for years. Low levels of morphine will soften any anxieties. Sleeping pills will ensure slumber. One night he will drift into a peaceful sleep and not awake.
• May 2003: Dr. Richard Olney's right knee began buckling. He thought it was related to an old running injury and, given his work, also considered the remote possibility it might be ALS.
• July 2003: Doctors discover a herniated disc that seemed the cause of the knee trouble, and over the next nine months, Olney underwent three increasingly invasive back surgeries. Afterward, the knee seemed to improve.
• May 2004: While Olney was working out with dumbbells, his right arm started to spasm.
• June 2004: Diagnosed with ALS.