Pushing it to the limit to fight ALS
By: Jeffery Kurz, Record-Journal staff
01/31/2009

Dave Zajac / Record-Journal
Jeff Johnson of Wallingford tries to stay positive with his mother Patrica even though suffers from Lou Gehrig's disease. Johnson had been an extremely athletic person having run in many marathons and competitions.



WALLINGFORD - In his living room there are framed photographs of Jeff Johnson running in marathon events, including the Boston Marathon, in which he competed seven times. There's also a number 4 jersey of Lou Gehrig.

Johnson says he was always a Yankees fan, but when it comes to the Iron Horse there is a darker connection. Johnson has amyotrophic lateral sclerosis, the disease that felled the famed Yankee slugger in his prime and is more commonly known as Lou Gehrig's disease.

For a long time, Johnson also had the strength and stamina of Gehrig, who for a long time held the baseball record for most consecutive games played.

His best friend says that Johnson was the athletic type of fellow who would play a softball double-header on a Sunday morning, take a nap, and then go out and run eight miles.

"If I was to describe Jeff in a few words I'd say, 'a great athlete, probably a better man,'" said Tony Leonardi.

The 43-year-old Johnson estimates that over the years he ran in anywhere from 400 to 500 events, starting when he was 12. His best marathon time was two hours and 42 minutes, which he posted in 1984 in Newport, R.I. On the track team at North Branford High School, Johnson ran the mile in four and a half minutes.



In 2004, Johnson posted the best overall time in the four Wallingford running events, so his name is on a brick at Town Hall.

Today, the fingers of both of Johnson's hands clutch inward. His arms hang inertly at his sides. His most pressing problem is eating. Chewing food is a challenge, swallowing is difficult. The appetite is not there. His breathing capacity is at about 70 percent. He can't breathe deeply and "if I ever get a cold, I'll be in tough shape," he said.

He has trouble speaking. "Every word is a tongue twister."

And "everything" is worse at night.

Gehrig's disease started in his legs, Johnson's in his upper extremities. But it hardly matters. Either way, "it's a nasty thing," said Johnson's mother, Pat Johnson.

Johnson ran his last marathon, in Boston, in 2006. A year later he was diagnosed with ALS. When Johnson was diagnosed, he was told he had motor neuron disease, which was a euphemistic attempt, he says, to soften the blow. He came home, looked it up on the Internet and found out it was ALS, with the prospect of living two to five years.

It was a shock, certainly, "but oddly enough, I never got depressed," said Johnson.

"One of the symptoms is uncontrollable periods of laughter or crying," he said. "So I try to concentrate on the laughter, because being depressed is not an option."

No treatment, no cure

Lou Gehrig's is a progressive neurodegenerative disease that hits nerves in the brain and the spinal cord. The degeneration of motor neurons robs the brain of the ability to control muscle movement, eventually leading to death. In the later stages there is typically total paralysis, though the mind remains alert. Thus, it has been described as "life in a glass coffin" until death arrives.

There is no cure, and no treatment that can halt its progress, though there have been developments in the last 15 years that may be promising. A little more than 5,600 people in the United States are diagnosed each year, with an estimated 30,000 Americans having the disease at any given time.

ALS killed Gehrig in 1941. Johnson feels it's high time there was more encouragement for those similarly afflicted.

"The problem is it's so rare," he said.

"People who get it, like me - pretty soon I'm not going to be able to make much noise."

While Johnson is able to raise awareness about ALS, he will.

"I always pushed it to the limit," he said. "Yeah, I miss running and playing sports. But I have to focus on ALS now."

He said he plans to "use the same drive to fight the disease."

The prospect of two to five years from the time of diagnosis is based on statistics, and the experiences of individuals can be varied, said Lauren D'Alessandro, executive director of the ALS Association Connecticut chapter.

How the disease will progress in someone is "very difficult to predict," she said. Her chapter serves about 150 state residents with ALS. D'Alessandro said there are an estimated 300 ALS sufferers in Connecticut.

The advocacy organization funds research, loans medical equipment and provides transportation, she said.

Johnson says he was told his is a slow-progressing disease.

"Common sense tells me to take care of myself as well as I can and the disease will progress slowly," he said.

ALS is more common in men than women, and 55 is the average age at diagnosis, but it has long proven an elusive disease when it comes to research or treatments.

Johnson and his mother are holding out hope for a growth hormone, called Iplex, which was developed to counteract dwarfism but also showed promise in relieving ALS symptoms. The growth hormone was pulled from the market in 2007 following a dispute among developers.

Stephen Byer said his son, Ben, after four days of taking the drug, went from "oatmeal and pudding to a Whopper."

Byer and his wife, Barbara, of Madison, Wisc., are co-directors of the advocacy organization ALS Worldwide. Ben Byer, who died last year at age 37, chronicled his experience with ALS in a documentary film called "Indestructible."

Byer said his son had to stop taking the drug when it was pulled from the market.

In pushing for the availability of Iplex, which is now limited to Italy, Stephen Byer said, "I have nothing to gain from it other than ALS patients like Jeff would live longer from having this."

It remains unclear whether Iplex will provide a significant advancement when it comes to ALS, said Lucie Bruijn, vice president and science director of the national ALS Association.

"At the moment, there isn't any reason to think that Iplex is going to be a blockbuster drug for ALS," she said.

But Bruijn said she considers it "an extremely exciting time" for ALS research.

As noted in an association report titled "Pathways to Hope," ALS has long suffered from dichotomizing attention. While victims include the famous, like Gehrig and astrophysicist Stephen Hawking, its relative rarity compared to other neurodegenerative diseases meant less attention when it came to research.

That changed in 1993, said Bruijn, when the first ALS gene was discovered, which gave researchers a tool to work with and generated "a heightened sense of interest." ALS is now also receiving more attention from pharmaceutical and biotech industries because of its link to other neurodegenerative diseases like Alzheimer's and Parkinson's.

"We know a lot more about the disease than we did before and there are scientists working on the disease," Bruijn said.

"I'm a tough guy"

Johnson's 18-year marriage was a casualty of his affliction. But friends and other family have rallied to his side. His younger sister, Kara, moved into a Wallingford apartment with him, and his mother spends up to six hours a day with him while his sister is working. Brother Mike, a physical fitness trainer, is also supportive by, among other things, taking him to sporting and other events.

But Johnson only sees the youngest of his three daughters, who is with him on weekends, with any regularity. He gave up marathon competition in the 1990s, when his kids were little.

Johnson's first marathon was in East Lyme, when he was 17. He had to fib and say he was 18 to get in the race, he recalled. He's run 16 marathons overall. He loved the challenge, he said. "I love to set goals."

"With Jeff it was always, OK, now I'm going to do better than last time," said his mother.

He returned to marathon running, in Hartford, in 1999, and competed three more times in the Boston marathon.

"To me, it was always about getting into Boston," he said. "To a runner, it's probably one of the best races in the world."

Johnson traces the onset of his disease to a softball game in 2004 when, pitching for Brothers Restaurant, he dove for a ground ball, landed on his left elbow and jammed the shoulder.

"I'm sure that's when it all began," he said.

Displaying an attitude many men would find familiar, Johnson did not seek medical help.

"I'm a tough guy," he said.

But within a month, he was feeling spasms in his left arm, and as time went by he noticed that the fingers of his left hand "didn't work as well."

For two decades, Johnson was a warehouse worker for Gallo wine. But as his left arm continued its stubborn refusal to improve, when he'd lift a case, he found he had to move it to his right arm. At the gym, he had to reduce the weight for his left arm.

"It creeps up on you, so slowly," he said. Initially, he felt the weakness only in his left side, but it spread to his right arm, hands, shoulders and legs. His difficulties today include balance problems, and he has to use a walker.

In the fall of 2006 he was sent to a neurologist and some "very painful" testing. The diagnosis, at Yale-New Haven Hospital, came in March of the following year.

Today, along with the assistance of his family and regular visits from his friends, he gets help six days a week from the Connecticut VNA, which he calls invaluable.

The progress of his disease is monitored by the Hospital for Special Care, in New Britain. His next visit is early this month.

"I don't look forward to those appointments," he said.
"We've known the finality of being fully diagnosed for a while now," said Leonardi, who has been Johnson's friend for 20 years. "I don't want to say you accept it, you just move past it and try to make every day mean something."

Johnson says it's important for him to have something to look forward to, and in his case that usually involves sports, or, perhaps, the latest Bruce Springsteen compact disc, which he was anxious to pick up the other day.

Johnson and Leonardi will head this month to Florida, where Johnson's father lives, and they will all take in the Daytona 500.

"Jeff's a huge NASCAR fan," said Leonardi, who is Middlefield's postmaster.

Other plans in the near future include baseball.

"I'm hoping to go to the new Yankee Stadium," said Johnson.

ALS Worldwide is online at www.alsworldwide.org. The ALS Connecticut chapter is at webct.alsa.org

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