WHEELCHAIR-BOUND but still hopeful

Longtime expert on ALS now knows it all too well

JOHN SCHWARTZ

Dr Richard K. Olney steers his motorised wheelchair towards the front door of the ALS Centre at the University of California, San Francisco.

Like other patients who visit the centre, he has amyotrophic lateral sclerosis, a disease that destroys the nerves that control movement, trapping the victim in an increasingly enfeebled 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 centre, which he founded in 1993. As a neurologist and ALS 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 the hopes of raising awareness about the disease, which has no cure, and money for research and treatment.

Dr Olney, 56, met with a visitor recently in a wide-ranging interview conducted partly in his old office, where so many people got long hours of counselling after learning they had ALS that they call it the kiss-and-cry room. He was joined by his wife, Paula Olney, and by Dr Catherine Lomen-Hoerth, a neurologist he trained at the centre, who has succeeded him as director.

The causes of ALS 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 email 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 favourite teacher in junior high [school] died from ALS,'' 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 ALS remained mentally sharp. And, he said, in the early 1990s, ``advances in molecular biology suggested that ALS might become very treatable during my career.''

Then he got the disease himself.


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What are the odds of an ALS researcher getting the disease that he has devoted his life to studying?

In fact, Dr Olney explained, ALS is not as uncommon as people think. The lifetime chance of getting what is commonly known as Lou Gehrig's disease is actually one in 1,000, or about the same as getting multiple sclerosis.

The difference is that ALS 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, by only 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 HIV patients; the other, hydroxyurea, is used in leukemia and sickle cell anaemia.

Because 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 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 honour, 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.''



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