Corte Madera, Calif. -- 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 the University of California at San Francisco, 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.
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.
"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.
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.
c.2008 San Francisco Chronicle