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Living for Today, Locked in a Paralyzed Body
By JOHN SCHWARTZ and JAMES ESTRIN
Published: November 7, 2004
http://www.nytimes.com/2004/11/07/health/07ALS.html?oref=login&oref=login&th
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Dr. Jules Lodish welcomes visitors to the downstairs bedroom of his
Bethesda, Md., home with a robotic greeting that bursts from his
computer's speaker.
Ten years of living with amyotrophic lateral sclerosis, or A.L.S., a
progressive, paralyzing disease, have stilled nearly every muscle; he
types with twitches of his cheek, detected by a sensor clipped to his
glasses.
But ask him how he feels about his life, and Dr. Lodish, his eyes
expressing the intensity denied to his body, responds: "I still look
forward to every day."
A.L.S., or Lou Gehrig's disease, is often described as a kind of living
death in which the body goes flaccid while the mind remains intact and
acutely aware. The prospect of being trapped in an inert body and being
totally dependent on others drives many sufferers to suicide.
When Attorney General John Ashcroft attacked an Oregon law allowing
doctor-assisted suicide in 2001 - a case that is still working its ways
through the legal system - patients with the disease were among those
who supported the law in court.
But while the legal case and much of the national attention has focused
on the issue of the right to die, less is known about those patients who
want to live, and, like Dr. Lodish, will go to extraordinary lengths to
do so.
With adequate medical care, patients often can live for years relatively
free of physical pain from the disease itself. "It's more a sort of
existential, psychic sort of pain," said Dr. Leo McCluskey, a
neurologist in Philadelphia who treats many people with the disease.
As a result, patients and their families are forced, on a daily basis,
to take stock of the meaning and quality of their lives and to make
repeated decisions about how much is too much.
"With A.L.S., you have a choice about when to stop treatment," letting
nature take its course, said Dr. Linda Ganzini, a professor of
psychiatry at Oregon Health and Science University in Portland, who has
studied patients making end-of-life decisions.
What keeps many patients alive, experts say, is a sense of having
unfinished business - perhaps a milestone "like getting the last kid off
to college," said Dr. Mellar P. Davis, a professor of hematology and
medical oncology at the Cleveland Clinic.
Those patients who do best are those who have insurance that covers
nursing and medical care, allowing them to avoid some of the major
health risks associated with the disease, and family members who can
cover the hours when expert help is unavailable.
Many patients, Dr. Ganzini said, have deep religious beliefs that help
sustain them, and they are able, "to find hope in the future, find
meaning and tolerate the daily ongoing losses that they are
experiencing."
As Dr. Davis put it, "Quality of life becomes a moving target - what was
one day an unacceptable quality of life becomes an acceptable quality of
life."
Dr. Lodish's body sits limp in a wheelchair and his tongue lolls; a
machine breathes for him through a tracheostomy tube in his throat. He
lost the ability to talk more than three years ago, he says, then jokes,
"but not the ability to be annoying."
At a time when even business executives fall into the shorthand of
teenagers' text messages - "r u going 2 the mtg?" - he refuses to cut
corners, communicating in eloquent sentences in person and via e-mail.
There are no half measures for Dr. Lodish, a hematologist and oncologist
who devised his own intricately detailed treatment regimen. He wrote a
30-page guide for his nurses that sets standards for a sterile
environment that go beyond hospital practices, rules that have helped
him avoid the infections that kill many patients.
When he could no longer eat, he did the research to come up with a
recipe for the nutrient blend that flows down his feeding tube - even
determining that the ingredients were kosher - and he typed the two-page
guide to its preparation, twitch by twitch, with a special program on
the laptop that helps him to choose whole words or phrases from
scrolling lists.
This wire to the world keeps him connected to his family and friends,
and allows him to remain an important part of their lives. He continues
to provide medical consultation, and now advises patients with A.L.S.
and their families on how to organize their own care and use the
communication devices he has mastered.
"One irony is with many people I communicate more now than when I was
well," Dr. Lodish said.
By holding on, he said, he has been able to see many of life's
milestones, including the marriages of two of his three children.
When his older daughter, Elizabeth Lester, became pregnant with the
first grandchild, she asked her father to make the official family
announcement.
"He still plays the same role for me," she said, "I still consult him on
financial matters and other kinds of things."
Dr. Lodish said that his own determination to live comes, in part, from
his long experience in treating cancer patients, who often feel that a
diagnosis is a death sentence.
"I spent my career getting people to live with their illnesses until
they died," he said, "if they weren't cured."
Now, he says, "My illness has validated my approach to my caring for
people." His wife, Carolyn, says she and her husband draw comfort from
each other and from family jokes that have worn thin with repetition and
age.
"We all say he's a better dancer than he used to be," Mrs. Lodish said
with a tired smile.
Choosing Death
Not everyone with the disease makes the same choice as Dr. Lodish.
Patients with the illness, Dr. Ganzini said, are 25 times as likely to
die by doctor-assisted suicide as people with other diseases, she said.
Dr. McCluskey, the neurologist in Philadelphia, said that at least 90
percent of patients with A.L.S. decided to die when they could no longer
breathe on their own, although medical science can extend their lives
much longer. For many, the tracheostomy surgery to implant a breathing
tube marks a dividing line between living naturally and being kept alive
artificially, and introduces serious medical complications and new
burdens of care on family members.
Without a tracheostomy, patients' lungs stop working on their own. The
level of carbon dioxide builds up in the bloodstream and the patient
goes to sleep. Morphine is given to ease suffering and anxiety. Within
hours - sometimes within minutes - sleep gives way to coma and coma to
death.
Once a tracheostomy has been performed, however, a patient who wants to
end his life has to make an active decision to turn off the ventilator.
Dr. McCluskey discusses death with every patient, he said, but the
conversations begin in earnest when breathing begins to become impaired
and the choices are not so hypothetical. After they have come to a
decision, he revisits the issue again and again, giving them
opportunities to change their minds.
Most patients decide that they want a feeding tube, Dr. McCluskey said,
but the vast majority turn down a breathing tube. Most of those who do
request mechanical ventilation leave instructions in advance to turn off
the machine if they come to the point that all connection to the outside
world ends. He calls this the "locked-in state."
Dr. Lodish has not yet reached that point, but he said that when he did,
he could imagine deciding that he had had enough of living with his
illness.
"It would have to interfere with my ability to relate to people," he
said.
The crucial element, Dr. McCluskey said, is choice.
"That's what this whole conversation is about - to empower people," he
said, "even though they are so debilitated and sick, they are actually
in control."
Needed for Longevity
Dr. John R. Bach, a respiratory expert at University Hospital in Newark,
said that more patients might choose to live longer if their doctors
knew more about the disease and the best treatments - and about hope.
"Physicians invariably tell these patients that they have a year or two
to live and that there's no hope," he said.
Dr. Bach said he encouraged patients to think in terms of the longest
possible life with the disease, and he has developed techniques to put
off the insertion of a tracheostomy tube as long as possible, including
mechanized girdles that mimic breathing, and masks and mouthpieces that
keep the air flowing in and out of the lungs.
Speaking of the withdrawal of medical support, Dr. Bach said, "I have
nothing against euthanasia" for patients who truly want to die. But, he
said, "you should not allow euthanasia for the first two years," because
many patients reach an accommodation with the illness.
"Do you know what happiness is?'' Dr. Bach says, citing a survey that
showed patients on ventilators tend to rank their level of satisfaction
with life at 5.1 out of a possible score of 7; the average person in the
same surveys has a score of 5.5. "Happiness is reality divided by
expectations."
Most days, Gregory MacDonald faces his disease with his own blend of
black humor and commitment, and says that he wants to live for years to
come. Other days he is not so sure.
A.L.S. has ravaged his once-muscular frame. He is unable to move from
the neck down. On one of the recent good days, Dr. Bach cheered him with
the opinion that if his lung capacity remained stable, Mr. MacDonald
could survive without a tracheostomy for another 10 or 15 years.
He was joyful. But an acquaintance who had driven him to see the doctor
was incredulous. Mr. MacDonald recalled that she asked, "You want to
stay like that for 10 or 15 years?"
"Absolutely!" he responded.
Just a few weeks later, however, came a bad day. He choked on a pill and
did not have the strength to cough it up or work it down. The panicky
and miserable ordeal lasted for nearly an hour, and he saw a dwindling
existence ahead.
"Is life worth it at all costs?" he asked. "If I'm exchanging carbon
dioxide, is that the only criterion?"
A school administrator, Mr. MacDonald was an energetic outdoorsman
before the disease struck him. He does not fully buy into his doctor's
sunny outlook.
"It's a living nightmare, and you can't imagine anything worse," he
said. "But from my life experience, I can guarantee you that things can
always get worse."
Once an avid guitarist - he played finger-picking country and blues -
the first signs of illness came, he said, when "I couldn't play basic
things." The initial diagnosis, in February 2002, was delivered by a
doctor who detailed the physical losses to come and eventual death in a
clipped, clinical monologue that ended with his handing Mr. MacDonald a
box of tissues and walking out.
"If it weren't for the kids, I'd have gone off a bridge," he says.
Today he sits in his Tuckahoe, N.Y., apartment on a recliner, a
52-year-old man who can say with a smile, "With the exception of a fatal
disease, I'm in almost perfect health."
He punctuates his conversation with a frequent turn of the head to sip
air from a mouthpiece.
He says that the good days outnumber the bad days, but that the bad days
can be intense. Recently, he won a long dispute with his insurance
company, allowing him 12 hours a day of professional nursing assistance
instead of the 4 hours a day of the low-skilled care he had before. The
difference in his quality of life, he said, has "given me a whole new
outlook."
Friends and family, including his girlfriend, Julia Stephenson, and his
former wife, Catherine Beazer, help fill in the hours that his insurance
does not cover.
"It is in a sense a family disease," Dr. Davis of the Cleveland Clinic
said.
Mr. MacDonald is tied into the daily lives of his 13-year-old son,
Dylan, and his 11-year-old daughter, Cheyenne, whose custody he shares
with Ms. Beazer.
Mr. MacDonald also suffers from the litany of medical problems that come
with the disease: frozen joints that have to be kept supple through
physical therapy, swollen toes that weep blood and can easily become
infected. A hand left resting on his leg, over time, can cause agony as
gravity forces the fingernail into his skin - a pain that he says can
sometimes be worse than the time on a fishing boat when he accidentally
slashed his hand with a gutting knife. He still allows himself small
treats - a martini in the evening ("Medicinal," he says) and one cigar a
week.
When he listens to the songs he once played, it can evoke a physical
memory of playing that strikes him unexpectedly.
"A specific tone in a song, a specific cord change can do that - can
make me quite weepy," he said.
Before his musical ability drifted away, he recorded a song that he had
written for Dylan and Cheyenne. The cassette sits in a safe.
At the back of Mr. McDonald's mind is a grim calculus of life, death and
pensions. He stopped working last spring after completing the school
year in a wheelchair, and will retire when his months of previous unused
sick days run out in January. If he should die before retirement, his
children will receive three years' salary as a death benefit. Over the
next three years, the death benefit diminishes and then vanishes.
Mr. McDonald says he wants to be there for his children, but he also
wants to be more than a mere "living presence." He wants, he says, a
life and a role in their lives that is "not only dignified but
meaningful."
"If I can't eat and I can't speak, quite frankly I can't imagine wanting
to entertain that for very long," he said.
But he also has trouble envisioning dying. "When you have so much to
live for, the thought of it becoming unbearable doesn't - it never
overshadows the fact that I can't imagine saying goodbye."
Meaning for Others
Finding meaning in life is not only the fundamental challenge for A.L.S.
patients, said Dr. Adele Zinberg, a psychiatrist who works with the
homeless and who has lived with the disease for seven years.
"A lot of what I do in my work is help people find meaning - everyone
needs it, whether it's through their family or their work or some other
cause," said Dr. Zinberg, who can operate a powered wheelchair.
"Everyone has to feel that their life has meaning."
She says that some patients ask, "How can I talk to you about my
problems when you have your own problems?"
She tells them: "Mine seem more obvious. But we all have our own
challenges."
Dr. Lodish also counsels other people who have suffered life blows. He
recalled that a doctor friend recently asked his advice about ways to
help a friend who was deeply depressed after a serious automobile
accident.
"Much of this boils down to whether or not one can hang on to who one
is," he wrote in response. He said that he was still what he had always
been: a father, a husband, a friend.
"In fundamental ways, I feel totally unchanged," he said.
"Quintessentially, I have found that ambulation, movement, swallowing,
eating, talking, breathing, and self care are not me. They are
substantial physical losses; but they are not me."
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