Bangor family adapting to a changed life as father's Lou Gehrig's disease progresses
By Meg Haskell
Saturday, April 26, 2008 - Bangor Daily News


BANGOR, Maine - Jim Kingsbury first noticed the stiffness in his right hand almost two years ago, on the day his son Jack was born. Distracted by the engaging activities of fatherhood, he didn't give it much thought at first.

But when the sensation persisted for several weeks and began alternating between stiffness and tingling, tingling and numbness, and when he became aware that he was losing some strength in that hand, he got a little more concerned.

"I figured it was probably carpal tunnel syndrome or tendonitis," he said during a recent interview in the family’s home in Bangor Gardens. Both conditions would be in keeping with his work as a cook and kitchen manager at one of Bangor’s most popular downtown restaurants. Never one to court bad news, though, Kingsbury, who is 46, waited for his annual physical exam before mentioning the problem to his doctor, who ordered some diagnostics.

The tests showed a blockage in the nerve signals to Kingsbury’s right arm. He went to a Portland neurologist, and he started some specialized exercises aimed at building strength and dexterity in the affected hand. But the exercises just made him tired. His hand and arm were twitchy, sore and achy.

It wasn’t until he was referred to a specialist at Tufts Medical Center in Boston that Kingsbury and his wife, Lisa, a secretary at Eastern Maine Healthcare Systems in Brewer, started talking seriously about the possibility that he might have ALS — amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease.

Lou Gehrig, a high-profile ballplayer with the New York Yankees, was diagnosed with ALS in 1939, putting a very familiar face on a disorder most people had never heard of. Gehrig died of complications related to the disease in 1941.

On July 3, 2007, after long weeks of blood work, medical imaging and other tests to rule out other disorders, Jim Kingsbury’s ALS diagnosis was official.

"We were pretty well prepared by the time we heard it," Kingsbury said.

Now, less than a year later, his voice is beginning to thicken and slur a bit, a sign that the disease is progressing. His face is getting a little gaunt. There’s a noticeable loss of muscle in his right hand, and the fingers are curved into a soft claw. A steady twitching is faintly visible in the muscles of his upper arm — but he has to take someone else’s word for it.

"I can’t feel anything there," he said.

ALS is a degenerative disease that affects nerve cells in the brain and the spinal cord. As nerve cells die, the brain’s ability to initiate and control muscle movement is lost. The cause of the disorder is unknown. About 5,600 Americans are diagnosed every year. ALS affects people in every country and of every race.

Men are more likely than women to develop ALS, and people who have served in the military are significantly more likely to develop ALS than those who have not. Some studies have shown that veterans of the Gulf War have nearly twice the average risk of developing ALS.

The average age of onset is 55.

Early on, patients experience loss of strength and the unusual sensations Kingsbury first noticed in his arm. In its later stages, ALS typically causes gradual and eventually near-total paralysis, eliminating the ability to speak, swallow and breathe. In rare cases, the symptoms may reverse themselves, but for most people, survival includes a feeding tube and permanent dependence on an artificial ventilator. The mind remains clear.

It usually takes between two and five years after diagnosis for paralysis to become fully developed.

There is no treatment for ALS, other than a relatively new drug, Rilutek, that in some people slows the progress of the disease, adding a few precious months of independence. Jim Kingsbury is taking this medication, but says he can’t tell if it’s helping or not. Because he has good health care coverage through Lisa’s employer, the cost is only $20 a month. Without insurance, the drug would cost about $1,200 a month.

He gets checked frequently to measure the progress of the disease. His lung capacity and his ability to exhale completely are key measures, along with his weight, strength, dexterity and sensitivity to physical sensations.

There’s not much more his doctors can do.

In the midst of the slow-motion disaster that is ALS, the Kingsburys are determined to make the most of the time that’s left to them.

Each with grown children from earlier marriages, they’re focusing most of their attention now on each other and on blond-haired, blue-eyed Jack, who on a recent evening was lurching around the living room in his mother’s high heels, clutching a cell phone to his small ear.

Lisa still works full time to keep their insurance coverage intact, but Jim has recently given up his cooking job because it takes too much out of him. He still goes in a few hours each week to keep himself busy and earn a few dollars, but there’s no telling how long he’ll be able to keep it up.

He doesn’t qualify for disability. They miss his income.

"Who can be prepared for this?" asked Lisa. "I told him, ‘You just have to get done, and we’ll deal with it.’"

They have begun recording Jim’s voice for a computerized talking machine that will help him communicate when he’s no longer able to speak. They are conferring with other ALS victims about the range of artificial ventilators available, including a relatively low-tech "turtle shell" some people wear when they go to bed to help them breathe through the night.

Later, they will need to talk about putting a tube into Jim’s airway for a permanent ventilator. They will learn more than they ever wanted to know about surgically implanted feeding tubes and other devices.

The Kingsburys have decided against participating in experimental drug trials.

"He could get a placebo, or the drug could make him get worse faster," Lisa said, explaining the decision. "He might have to travel. We have this window of two to five years, and I want to take advantage of all of it."

Briefly, she teared up. "You feel so helpless," she said. "Cancer and other diseases are awful, but at least there are treatment options. With this, there’s nothing you can do except have the progress checked."

Jack staggered by on the spiky 3-inch heels, and everyone smiled.

"I can’t help thinking about how this all started," Lisa said, underscoring the sad irony of the situation. "The day Jack came into the world was the day we all started down this road."



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