Patient stories: A difficult disease made gentler

Patti and Larry Atkins had recently moved to Seaside when Larry's doctors delivered a devastating diagnosis.

For Larry Atkins, doctor visits rarely bring good news, but his mood on this Tuesday is buoyant. Of the 10 specialists and therapists who examined him in a half-day marathon of appointments at Providence ALS Center, each reported that he seemed to be doing pretty well.

For Larry, the goal right now isn't even to get better - it's to delay getting worse. He has amyotrophic lateral sclerosis, or ALS, a degenerative nervous system disease that is gradually robbing him of his ability to walk, talk, swallow, cough and eventually to breathe. Although people with ALS, also called Lou Gehrig's disease, live an average of two to four years after diagnosis, not all cases progress so swiftly.

It's days like these that give Larry, 56, hope that he might be among the fortunate.

Living longer, or just as important, living well are two reasons that Providence ALS Center has established this one-stop multidisciplinary clinic. Instead of having to visit multiple specialists all over town, Larry and his wife, Patti, make the two-hour drive from Seaside to Portland every three months to see all of Larry's medical team in a single afternoon.

"Before this clinic existed people were out there floundering, trying to coordinate their own care," says Lance Christian, M.S.W., executive director of the ALS Association of Oregon and SW Washington. "At the same time they're dealing with a rapidly progressive diagnosis. It's hard to manage everything." The association partners with Providence Brain and Spine Institute and The Oregon Clinic to operate the ALS clinic.

The arrangement involves everyone from neurologists Kimberly Goslin, M.D., Ph.D., and Tracy Sax, M.D., to a pulmonologist and a host of physical, respiratory, speech and occupational therapists. Beyond helping to relieve logistical burdens, regular monitoring helps clinicians catch and treat new problems as they develop, in some cases, prolonging life.

One bad break

In January 2008, Larry was working as a buyer for a home improvement chain when he stumbled in the parking lot. In what felt like slow motion, he lost his balance, slammed into a parked car, then landed hard on the pavement, breaking his hand and nose and cutting his lip.

He had been undergoing tests for an unusual slur in his speech, but doctors couldn't pinpoint a cause. Now the muscles in his legs and feet were growing weak, a sign that his mysterious condition was worsening. At the time, he was living in Lake Oswego and preparing to move to Seaside, where Patti had accepted a public relations job at Providence Seaside Hospital. Larry had planned to transfer to a store there.

After being tested for a range of conditions, including another neurodegenerative disorder, Parkinson's disease, a neurologist at Providence Brain and Spine Institute confirmed in September of that year that Larry had ALS.

"It was hard to wrap my mind around it," says Larry. He speaks slowly, struggling to form consonants. "Essentially, they've given you a death sentence. But they don't give you a date. It could be two years, it could be a year, it could be 40 years."

He tried at first to deny the disease.

"We couldn't even talk about it," says Patti, looking at her husband of 31 years. "You actually didn't want to acknowledge it."

There is no cure for ALS, but despite the bleak outlook, the Atkinses find inspiration where they can. For Larry, it's knowing that physicist Stephen Hawking has lived with the disease since 1963. For Patti, it was a woman standing outside a restroom. The woman's husband couldn't walk unaided, and at the time Larry was still mobile enough to help the man inside.

"She said, 'Isn't this wonderful?' " Patti recalls. "And I was like, 'What's wonderful?' And she said, 'My husband has ALS, but he's had it for 16 years.' "

"Don't ever give up"

In the nearly two years since his diagnosis, the disease has taken a predictable toll. For starters, Larry had to retire.

"I loved to work," he says. "I didn't picture my retirement."

He gave up his driving privileges because of hyper-reflexivity, one of the many symptoms of ALS. He uses a wheelchair most of the time now, and he has a pump that delivers anti-spasticity medication directly into his system. His speech is labored, so he's learning how to use a computer that will eventually become his voice.

The half-day clinics at Providence focus on easing the symptoms of ALS as well as inhibiting its progression. Larry takes special medications, including riluzole, the only FDA-approved drug that slows the advance of ALS.

He wears compression stockings to relieve the swelling caused by inactive leg muscles. He recently got a cough machine to help him get rid of phlegm. And he maintains lung function by manually inflating his lungs through a squeezable plastic bag with a mouthpiece attached.

"One of the problems that ALS patients get into is that their muscles are weak and they don't expand their lungs," says Louis Libby, M.D., a pulmonologist at Providence Portland Medical Center and The Oregon Clinic. "So the parts of the lungs that aren't being used begin to scar over."

Larry's breathing muscles are still relatively strong. In fact, on this Tuesday afternoon in early July, most everything is looking good except for some increased weakness in his legs. His arms remain strong, and he can still swallow his food. Larry's positive three-month report card has given him renewed optimism, at least for today.

"Every day is a gift," he says, after the last of the day's appointments. "Use these gifts that you get as long as you can. Don't ever give up. Continue to fight." Adds Patti: "I honestly can't imagine how we would have struggled through this without our team at the Providence ALS Center. Everyone, from my co-workers at Providence Seaside to the specialists in Portland, has given us the best of care, respect and ongoing support."