Rare Lung Disease Sparks Bicycle Ride for Research

ANAHEIM — Less than two years ago, Paul Mansdoerfer was overweight, out of shape and frequently out of breath until his doctor told him he had only five years to live.

Since diagnosed with a fatal lung disease, Mansdoerfer, 44, of Florida, has embarked on a cross-country sojourn aboard his bicycle to raise thousands of dollars for respiratory research. A leg of that trip ended Sunday night in Anaheim, where he is scheduled to address today’s session of the American Lung Assn.’s international conference.

“I know that whatever research that is developed at this point won’t help me,” Mansdoerfer said Monday during a visit to the conference. “. . . Most people don’t beat this disease, so I am doing this for future generations that should be able to beat this disease and live.”

Mansdoerfer has alpha antitrypsin deficiency-related emphysema, or AAT, a rare illness that slowly destroys the air sacs of the lungs where oxygen enters the blood. When the disease was diagnosed two years ago, the capacity of his lungs was 46% of normal. Now it is 17%.

The inherited disease, which causes a shortness of breath, is sometimes called early-onset emphysema because it can appear when a person is 30 to 40 years old. Today, about 1 in 2,500 people have the illness.

Mansdoerfer, a former civil engineer, says he wants to tell convention-goers about the difficulties he had finding out what he was suffering from. Despite years of doctors’ examinations, his ailment was not diagnosed until 1990 after a series of blood tests.

Before that, Mansdoerfer said, he used to hear from doctors at least twice a year that he had bronchitis. And even though he kicked a pack-a-day cigarette habit, his lung and breathing problems worsened.

“I was misdiagnosed a dozen times as having pneumonia, bronchitis and much more--everything except what I actually had,” Mansdoerfer said. “At first I just accepted the fact I had the disease. Then one day I just freaked out and said I have do something to make a difference.”

Making a difference meant getting in shape. He started an exercise program and lost 100 pounds. Then his wife, Caroline, came home one day with a new bicycle.

“It all clicked,” Mansdoerfer said. “I had to admit it was better than getting a wheelchair.”

After almost a year of daily workouts, the father of three decided to use his new physical prowess in an effort to raise funds and attract attention to the disease that will probably kill him.

In March, Mansdoerfer, who is 6 feet, 2 inches tall and weighs 147 pounds, began pedaling around the perimeter of the United States--a distance of about 15,800 miles. Every week on the road, he takes about $3,000 worth of medication, including a blood thinner that helps increase the blood’s oxygen content. And along the way, bike companies have donated helmets, gloves and other equipment. Hotel chains have given him free rooms.

Mansdoerfer hopes to finish the trek on Oct. 11 by riding 90 miles a day, six days a week while his wife follows behind in a van carrying three types of bikes for various road conditions. So far, his efforts have raised more than $30,000 for research. His goal is $500,000.

The West Coast leg of his trip begins Wednesday. In June, he plans to link up with a group of other bicyclists for the route to Maine.