Sitting in his motorized wheel chair, Richard Olney unfolds the computer on his lap and taps several keys to demonstrate how he may communicate in the future.
“I have Lou Gehrig's disease and I have trouble speaking,” he says, as his voice is projected from the computer. His voice is very clear, just modestly affected by the disease.
He recorded 1,700 phrases about six months earlier, well before he needed an interpreter - Paula, his wife of 30 years - to clarify much of what he says.
Its just one of several steps he has taken to prepare himself for the anticipated decline in his physical abilities. More than most patients, he knew what to expect. This is because he is a neurologist and founder of the ALS Treatment and Research Center the University of California, San Francisco (UCSF). Over his 29-year career, he has diagnosed amyotrophic lateral sclerosis (ALS) in more than 1,000 patients. But on June 11, 2004, Dr. Olney, 57, learned that he himself had ALS, and was about to become a patient at the center he founded.
Like most people who get the disease, he had no family history, no reason to expect he would get it.
Active in Public Education
In March, he received the Public Education Award from the American Academy of Neurology Foundation, honoring him “for sharing his personal experience with ALS to inform people everywhere about the disease.”
His disease has not dampened his zeal. Since his diagnosis, he has been busy speaking to the national media. Assisted by Paula and his adult children, Nick and Amy, he has told his story to many print and broadcast outlets. He taped a public service announcement for the ALS Association last September.
Something they could fix
His diagnosis was an emotional roller-coaster ride. He first noticed something amiss in May of 2003. ''My right knee started to ache and pop and seemed less stable. I saw a sports medicine doctor because I thought I had a running injury. In August, I recognized spasticity in my right leg - slowness and stiffness. He checked his reflexes, and discovered the ones in his right leg were abnormal. He underwent magnetic resonance imaging tests (MRIs) of his head, neck and back. The tests found a ruptured part of a disc was pushing on his spinal cord. “We were so relieved that they had found something that they could fix,” Paula said.
But his condition worsened following his first spinal surgery.
Then, in May 2004, he experienced stiffness and incoordination (spasticity) in his arms. Early the next month, the ALS diagnosis was confirmed.
Now, he receives the very comprehensive care that the UCSF ALS center provides to its patients. The center is one of only 15 U.S. centers recognized internationally for the quality of research and treatment, Dr. Olney says. Unfortunately, he adds, most people live too far away from most ALS centers.
Most see a primary care physician and are less likely to receive medications that can treat symptoms of the disease, including spasticity and bladder urgency, and quinine for muscle cramps. He also takes a low-dose antidepressant, antioxidant vitamins and coenzyme Q10. People with ALS also can benefit from psychotherapy and riluzole (Rilutek).
Choosing to Take Part in Research
Long before he contemplated the possibility that he'd ever have ALS, Dr. Olney set up a clinical trial at UCSF to determine if three drug therapies - low-dose ritonovir, high-dose ritonovir or hydroxy urea - might slow progression of the disease. The trial compares them to a placebo, an inactive substitute. As a patient, he became the first to enroll. None of the participants, including Dr. Olney, know whether they will receive the experimental drugs or a placebo. “The only way we will ever know if drugs are effective is to have placebo-control trials so I wanted to make a statement with my behavior to reinforce this,” Dr. Olney says. At the end of six months, he and the other patients will learn what they are taking and can choose to change or continue their regimen.
What Matters Most
Meanwhile, what matters most now, Dr. Olney and Paula say, is that he enjoys life, which includes bird watching from the back of their Marin County home, occasional hikes (in his wheel chair), visits by family and friends, keeping fit on specialized equipment and other leisurely pastimes, like watching movies. And, of course, telling his story and the story of ALS.
An Endowment Fund to honor Richard K. Olney has been established to support the work of the ALS Treatment and Research Center at UCSF. To make a donation, call (415) 514-2612 ore-mail firstname.lastname@example.org.
FOR MORE INFORMATION
The American Academy of Neurology's journal Neurology publishes special articles for patients. To access “Treatment and Quality of Life for People with ALS,” go to www.neurology.org, click on “Patient Pages” and scroll down to May 2004.