Skip to comments.Longtime Expert on A.L.S. Now Knows It All Too Well
Posted on 02/21/2005 9:34:21 PM PST by neverdem
SAN FRANCISCO - Dr. Richard K. Olney steers his motorized wheelchair toward the front door of the A.L.S. Center at the University of California, San Francisco.
Like other patients who visit the center, he has amyotrophic lateral sclerosis, a disease that destroys the nerves that control movement, trapping the victim in an increasingly inert body. It is inevitably fatal, often within a year or two.
Dr. Olney knows these bleak facts better than most because his name is on the door of the center, which he founded in 1993. As a neurologist and A.L.S. researcher, he has written more than 50 scientific papers. As a patient, he is now taking part in a clinical trial that he designed.
He is spending some of the time left to him speaking about his illness, in hopes of raising awareness about the disease, which has no cure, and money for research and treatment.
Dr. Olney, 56, met with a visitor last week in a wide-ranging interview conducted partly in his old office, where so many people got long hours of counseling after learning they had A.L.S. that they call it the kiss-and-cry room. He was joined by his wife, Paula, and by Dr. Catherine Lomen-Hoerth, a neurologist he trained at the center, who has succeeded him as director.
The causes of A.L.S. are still largely unknown; about 10 percent of cases appear to be linked to genetic flaws, while the other 90 percent are a persistent mystery. In an exchange of e-mail messages before the interview, Dr. Olney explained that he was first drawn to the disease as a field of study after becoming a neurologist.
"I found out that the daughter of my favorite teacher in junior high died from A.L.S.," he said. Having also trained as a psychiatrist, he said he was "much more comfortable than most neurologists in relating to patients with fatal disease."
He also liked the fact that people with A.L.S. remained mentally sharp. And, he said, in the early 1990's, "advances in molecular biology suggested that A.L.S. might become very treatable during my career."
Then he got the disease himself. Dr. Olney began noticing problems in July 2003: his right leg was becoming a little stiff, and his movement and coordination were slightly impaired. An extensive neurological evaluation turned up a protruding disk that compressed his spinal cord, and so he underwent back surgery in September of that year. For a month afterward, he seemed to be getting better, but by November, "I was obviously getting worse," he wrote. "The possibility of A.L.S. was raised, and was my expectation, as I underwent another round of testing."
Those tests revealed a large herniated disk that put pressure on the spine, and so he and his doctors continued to hope that he had a spinal problem that could be cured with surgery. He had two more operations, in December 2003 and March 2004.
Then, in mid-May of last year, Dr. Olney said, he noticed mild stiffness and slowness developing in his right arm. He gave himself a common test for A.L.S.: counting how many times he could tap his fingers in 10 seconds. From experience, he knew that he could usually tap 65 times but now he had slowed to 55.
"I knew I had A.L.S. then," Dr. Olney recalled.
On June 11, Dr. Lomen-Hoerth gave him the official diagnosis.
It was, in a way, helpful, said Mrs. Olney, his wife of 30 years, who is a nurse.
"At least you know what you're dealing with, so you're not chasing rainbows any more," she said.
Dr. Olney's condition deteriorated rapidly; he is now in the middle stage of the disease.
He gets around in a $23,000 motorized wheelchair, and sleeps on a special $10,000 bed that tilts him from side to side so that he does not have to wake his wife to turn him over. The costs are covered by insurance.
While his once-crisp speech has become labored and indistinct, he is capable of a slow, tentative smile. And he does smile, often.
For a time, his breathing weakened as well, and he expected to have less than a year to live. That deterioration appears to have leveled off for now.
Losing the ability to breathe independently is the boundary line for A.L.S. patients: they have to decide whether to endure a tracheostomy and let a machine do the breathing for them, or to let their lives end when they can no longer breathe on their own. A ventilator can extend life for years, but more than 90 percent of patients refuse it because of medical complications and greatly increased requirements of care.
Dr. Olney said he did not want the ventilator - a decision that he made in 1993 when he completed a medical form known as an advance directive.
"My primary focus should be on quality of life," he said without sentiment or apparent sadness.
There has already been so much of that. Dr. Olney's mother died last month of congestive heart failure. Paula Olney's mother died last summer of pancreatic cancer; the family had moved her into their home to spend her final days together.
After Dr. Olney's diagnosis, the couple's grown children, Nick and Amy, moved back into the family home to help care for their father.
Nick, who is 27, had graduated from the University of California, San Diego with degrees in biochemistry and theater, and was planning to go to medical school. Amy, who is 24, graduated from the University of Washington in 2003 and had entered a master's program in occupational therapy at the university; she transferred her studies to a college in Oakland.
"A.L.S. brings out either the best or worst in a family," Dr. Olney said. "Usually the best."
What are the odds that an A.L.S. researcher will get the disease that he has devoted his life to studying?
In fact, Dr. Olney explained, A.L.S. is not as uncommon as people think. The lifetime chance of getting what is commonly known as Lou Gehrig's disease is actually 1 in 1,000, or about the same as getting multiple sclerosis.
The difference is that A.L.S. kills so quickly that the number of living patients at any one time is relatively small - which also means that there is not a large population of victims to agitate for research and relief.
Only one drug, Rilutek, has been approved specifically for the disease, and it extends life, on average, only by a couple of months.
As a researcher, Dr. Olney proposed a study on two drugs, already on the market, that had shown promise in mice. One, Ritonavir, is used to treat H.I.V. patients; the other, hydroxyurea, is used in leukemia and sickle cell anemia.
Since the drugs are available for other diseases already, it would be a simple matter for Dr. Olney to get a prescription. Instead, he has opted to join the clinical trial, with the very real possibility that he will receive a placebo instead of a real drug.
Since Dr. Olney set up the trial, he has enough clues to know whether he is in the group testing Ritonavir or hydroxyurea, but he says he does not know whether he is taking the real pill or the placebo.
Clinical trials with placebos offer the best way to judge a drug's effectiveness, and Dr. Olney said he hoped his act would inspire others to take similar risks.
"It's the only way we'll ever make incremental progress," he said.
Dr. Lomen-Hoerth echoed this view.
"He's setting a great example," she said.
And not just for patients. A colleague at the medical school, Dr. Arnold R. Kriegstein, said Dr. Olney's illness was already serving as an inspiration to young researchers, who tend to view the field as depressing and unpromising.
"This is going to be a terrifically motivating event in the lives of clinicians and investigators whose lives he has touched over the years," he said.
Dr. Kriegstein, who spoke at a symposium that the university held in December in Dr. Olney's honor, is a leading researcher in stem cells. He is working to help develop techniques that will allow the versatile cells to replace those ravaged by the disease.
But that, and other promising therapies, are at least 10 years away, Dr. Kriegstein admitted. Dr. Olney, he said, "is, in a sense, fading away as the research effort is really building up."
Mrs. Olney puts it more bluntly. "If Rick lives long enough to finish the trial he started, that will be pretty amazing."
Earlier in the day, the Olneys visited the Neurofit center, one of the few centers in the nation devoted to exercise for patients with neurological disorders.
Albert Seligman, the trainer, put Dr. Olney through sessions on specialized equipment to fight the spasticity that would otherwise stiffen the limbs and to ease Dr. Olney's breathing.
The exercise room is tiny - just 200 square feet carved out of former hallway space, with a stunning view of Golden Gate Park and the Pacific Ocean.
While Dr. Olney strained against the machines, Mrs. Olney walked a brisk three miles on the treadmill.
It was almost like old times.
Until he became ill, the couple led an energetic life, exercising together. They often hiked 8 to 10 miles on Sundays or took 25-mile rides on their mountain bikes.
As they left the building, they ran into one of Dr. Olney's colleagues, who apologized for not having come by for a visit. He explained that he had had a busy schedule of travel, including a recent trip to Switzerland.
"Lucky you!" Mrs. Olney said with a tight smile.
"That used to be our life," she said. "We used to fly all over the world talking about A.L.S. Now we have it."
Paula Olney said she valued the time she had left with her husband, who used to keep such a packed work schedule that they spent time together only on Sundays.
"We have a lot of time to prepare for his passing," she said. "It's not like he cracked up on the freeway, which is how I always thought he would die."
So, she said, "I'll take him any way I can have him, as long as I can have him, but I think he's prepared to die, and I'm prepared for him to die."
In the meantime, she said, "We eke out a little pleasure in every day."
One night, she recalled, they had gone out to dinner and their van broke down. With no wheelchair-accessible cabs in the area, Dr. Olney decided to make the trip of a few miles back to the house in his motorized wheelchair, which has a range of 20 miles. The journey, he said, "was kind of fun."
As Dr. Olney's fame grows, the offers of advice and help increase in number - and in strangeness. Mrs. Olney recalled that one letter writer urgently demanded six of Dr. Olney's neck hairs - "and two from my forearm," Dr. Olney interjected - to prove that the disease was caused by an overabundance of selenium.
Another correspondent offered to adjust his Chi.
"I need to drink my own urine," Dr. Olney said with a smile, remembering another suggestion.
Mrs. Olney said it was a shame that her husband fell ill now, when otherwise he would have had so many productive years ahead of him.
"Too bad he's famous for dying from this disease instead of finding something to help it," she said.
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This is what killed my Grandmother many years ago.
A terrible disease, that quickly or slowly robs one of all their physical abilities. Usually, respiratory arrest is the proximal cause of death.
I'd rather OD on good drugs.
But Dr. Stephen Hawking has had ALS for over 40 years. I recall reading that he is the longest surviving person with ALS.
It progressively leads to death in 2 to 7 years.
I'm glad for Dr. Hawking and Physics. My friend just got the diagnosis not too long ago.
I am totally against euthanasia, but when I think of some of the horrible ways to die, I think I would rather die very quickly.....
I wonder if this suggests the disease can passed between people or if its a really odd coincidence?
However, if somebody has a terminal disease and choses the manner and timing of their own death, I have no problem with it.