ALS slowly drains Racine lawyer's world

CALEDONIA, Wis.: The house is the same house. The street is the same street. It is Cynthia Murphy's world that is different, shrunken from what it was just a few months ago to what it is now: a few rooms on a single floor of her Caledonia home.

It began a couple of years ago when Murphy had trouble speaking, a difficult problem for most people but crucial for a family lawyer who was in court for divorce trials, modifying judgments and other legal work.

A couple of other local family lawyers had also retired, increasing Cynthia's work load, said Sally Nietzel, Cynthia's sister. Cynthia also had trouble eating and started losing weight.

Cynthia visited doctors, but none had an instant answer. It took about a year to get one, and when it came it was one of the worst that could be expected. She had amyotrophic lateral sclerosis, also known as ALS or Lou Gehrig's Disease. It's a progressive ailment in which motor neurons, the nerve cells that control muscles, gradually degenerate, robbing patients of the ability to move, swallow and even breathe.

``I can't lift my head on my own,'' Cynthia wrote on a pad while lying on the hospital bed that now dominates her living room.

She writes because she cannot speak. The tube that disappears through a hole in her throat prevents that, and her life now depends on several feet of plastic tubing and the hum and sigh of a ventilator.

Her particular type of ALS is called progressive bulbar palsy. It first affects the muscles for speech and swallowing. Her other plastic tube is for feeding herself.

She can move around somewhat, but what she can do is a fraction of what this active person could do a few months ago when she would regularly exercise at one of the local gyms, Sally said.

``I do some stairs now, short flights,'' Cynthia wrote. ``I can't do a lot because of the machine.''

Formulate a question about ALS, and the answer is likely to be, ``We don't know.''
It's not a common disease. Each year there are about two cases for every 100,000 people, and 98 percent of those are ``sporadic,'' meaning there is no clear cause. The other 2 percent are hereditary. It is a mysterious disease. Since it was first clearly described in 1874, medical science hasn't made much progress.

``And indeed I think it's naive to sit down and think that ALS is one disease,'' said Dr. Paul Barkhaus, professor of neurology at the Medical College of Wisconsin and director of the ALS programs at Froedtert Hospital and the Veterans Administration in Milwaukee. It may have more than one cause, he said.

Only one drug has been approved by the U.S. Food and Drug Administration for the treatment of ALS. It's Rilutek, and what it does is interfere with the release of glutamate. That's a naturally occurring chemical which carries a signal from one nerve to another, and it's an excitatory signal. Too much excitation may produce toxins that damage the nerve cell and bring on ALS, Barkhaus said. ``Or at least that's what is thought to be part of the pathway.''

Managing a patient largely consists of making the most of what the patient has and anticipating problems, Barkhaus said. Medical teams try to fulfill patients' spiritual needs and also make sure they understand the reality they're facing. Ventilators help, he said, and barring pneumonia, an infection or a blood clot, they allow longer survival.

Until May, Cynthia's life was moving more or less normally. She had a feeding tube but was still able to go to her office. Then she had a case of pneumonia. A friend who is also a nurse spent a weekend night at Cynthia's house. On that Sunday morning she couldn't wake Cynthia.

``I had no pulse,'' Cynthia wrote on her pad, underlining the last two words.

After talking to the physician in the emergency room, Sally said, ``We basically thought she was dead.''

But she revived, was hooked up to a ventilator, dropped into a coma for a couple of days, and then spent weeks at Kindred Hospital in Greenfield, Sally said. Doctors at the hospital, which specializes in complex medical cases, were unable to help Cynthia breathe on her own. The ALS had affected the diaphragm, the large muscle at the bottom of the rib cage which produces breathing.

She has been on the ventilator since, and that has ended the remains of her legal career. Her son, Matthew, who graduated from law school in 2007, has taken over the practice.
As she adjusted to the disease, Cynthia connected with the ALS Association, an organization formed to advocate for medical research and to help families and patients deal with the disease.

At the end of March a group of people toured the Wasiman Center at the University of Wisconsin-Madison and met with Gov. Jim Doyle to ask for more state funding for research. Cynthia didn't go; it's too difficult, she wrote to the governor, to see other people in other stages of the disease the stages where she may go.

-AP