N.J. man with cystic fibrosis eyes Pittsburgh Marathon again

| Saturday, April 27, 2013, 10:48 p.m.

The first thing Steven Bell does when he wakes up is take a few deep breaths to find out what kind of a day it's going to be.

If he's 100 percent healthy, the air will flow into his lungs, and his chest will rise as his lungs expand before falling back down as he exhales. Inhale, exhale. Expand, contract. Those are the good days.

If he's sick, it will feel like he's breathing through a straw. He'll want his lungs to fill, but it will feel like someone piled cinder blocks on his chest. It could mean an infection. It could mean another trip to the hospital.

Or it could be somewhere in between. Every day is different.

He is 29 years old. That's life with cystic fibrosis.

Bell, however, is far from typical for someone with the progressive and chronic disease that affects the lungs and digestive systems of roughly 30,000 children and adults in the United States.

He runs marathons and runs them faster than a lot of people who don't have to live through daily breathing treatments. Bell, who lives in Hoboken, N.J., and works at a Manhattan advertising agency, ran the Pittsburgh Marathon in 2011 in a personal-record 3:41. On May 5 he's returning to run the half marathon.

“There's really nothing like race day,” Bell said. “You get that anxious, excited, competitive feeling in your stomach. Clearly I'll never win, but you can always be better than yourself, and at this point it's just fun. One thing you hear in the CF world is that to run a race is a celebration that you still have the ability to do so. Even if it's not a great race, even if I'm cramping at mile 21, 98 percent of time I will have a smile on my face.”

It's different in the CF world. Bell's world.

Uncharted territory

Running a marathon stresses the organs and immune system of a healthy individual. For a patient with cystic fibrosis, the situation is more precarious, and Bell landed himself in the hospital for three weeks in 2008 after his first marathon when his body couldn't recover.

A year and a half passed between his first and second marathons, as he, his doctor and nutritionist worked to determine if there should be a second race and, if so, how to run and stay healthy. At the time, Bell knew of no one else with cystic fibrosis who was running full marathons, so there was nowhere to turn for advice.

“I think there was hesitation on all our parts to venture back into that world of marathoning, but there's a conversation that takes place,” he said. “I was a little taken aback with how tough it was on my body, but I've always been a fighter. I wasn't going to let that stop me from continuing to run and compete. And I wasn't happy with my first time. I wanted to be under 4:00, and I finished in 4:53.”

Bell ran his second marathon in 3:57.

He started taking antibiotics before the race to give his body a boost. Patients with cystic fibrosis also lose a significant amount of salt during activity, putting them at risk of dehydration. That was a big part of Bell's problem in his first marathon, so now he takes a salt pill every 20 minutes while running.

Then there is the daily regimen.

Cystic fibrosis causes the body to produce a thick mucus that clogs the lungs and leads to infections, so Bell must undergo 60 to 90 minutes of breathing treatments every day. That includes the use of nebulizers that deliver medication to the lungs and a machine called the vest. He puts it on, hooks it up, and it shakes his upper body to help loosen the mucus that's collected in his lungs overnight.

The other main element to the disease is the inability to break down and absorb food properly. Bell supplements with pancreatic enzyme pills: 12 per meal, and four to six with snacks.

But there's a catch.

On a normal day, Bell said, he would have to consume twice as much food as a non-CF patient to account for his body's inability to absorb nutrients. Marathon training burns 1,000 to 2,000 calories a day, so when he is training he has to take in 5,000 to 6,000 calories per day, and that means taking anywhere from 50 to 70 pills per day.

What's more, because Bell's pancreas has taken such a beating with the disease, he has cystic fibrosis-related diabetes and has to take insulin to control his blood sugar.

“It's an interesting combination, consuming enough food but making sure I'm balancing eating enough with eating what's right and keeping my sugar in check,” he said.

Even as a healthy patient, Bell said, he will wind up in the hospital at least once a year for seven to 10 days followed by two weeks of home IV therapy to treat the inevitable infection.

“In the CF world, we refer to them as tune-ups,” he said. “With the progressiveness of the disease, lung infections come every so often, and there's only so much you can do to fight them off with oral (medication) before the body gets worn down and stronger meds are needed. Some years are great and it's only once. Some years it's three or four.”

Work stress, weight loss, a bad allergy season or a bad flu season could mean the difference between once a year and three or four times a year.

These are inconveniences for healthy adults.

It's different in the CF world. Bell's world.

Up to the challenge

Dr. Joseph Pilewski has been specializing in cystic fibrosis for almost 20 years. As co-director of the Adult Cystic Fibrosis program at the Antonio J. and Janet Palumbo CF Center at Children's Hospital of Pittsburgh, he has had a handful of patients participate in half marathons in recent years. One of his patients also will be running the Pittsburgh Half.

Pilewski doesn't remember talk 10 years ago of cystic fibrosis patients running marathons. It was unheard of 20 years ago, when the median age for life expectancy was 20. Now the median life expectancy is 37. Some patients still die in their teens and 20s, Pilewski said, but he also has patients in their 60s and even one in his 70s. It is a testament, he said, to how far they've come in treating the disease.

They still have a long way to go.

“Really sick patients spend four to six hours per day on respiratory treatments,” Pilewski said. “As it progresses, it's terrible. There's tremendous suffering, and it happens to people in their 20s, 30s and 40s. It's particularly hard when you're that young. Your peers are living very different lives. The majority of your peers are totally healthy.”

Exercise helps, Pilewski said. Those who exercise see a slower progression in the disease. Still, he says, what Bell and the few others like him do borders on heroic.

“CF patients do it for the same reasons everyone else does,” he said. “They do it for the challenge. It's more challenging for them, for sure.”

Bell was three months old when he was diagnosed with cystic fibrosis and 11 years old when he learned that the life expectancy was, at the time, 27 years old. How does an 11-year-old wrap his mind around maybe having only another 16 years to live when his grandparents are in their 70s?

With fear, he said. And with a maturity that no 11-year-old should have to possess as well as a greater appreciation of things healthy people take for granted.

Running, as it is for many people, is his escape from the things that trouble him.

“I run while I still can,” he said. “It's not something I think about all the time. Just like everyone else, I have dreams and aspirations. I want to have family. But it's something certainly that's there. It's reality. While you have the opportunity to do things, it's important to take advantage.”

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