Two hours a day, Jeff Davis works at staying alive. He inhales a succession of medications, runs on a treadmill and sometimes performs breathing exercises that produce gurgly, crackling sounds.
It's tedious work, crammed into a life that includes a full-time job as a machinist and leisure time with his wife and two young children in the horse country north of Westminster. But it has paid dividends: A generation ago, few people with cystic fibrosis lived past their teens - and Davis is 35.
If there's a miracle in this story, it's that Davis is fairly typical, having soldiered through childhood with a closet full of medications and parents who spent untold hours thumping his torso to keep his lungs clear.
"They told my parents that if I lived to be a teenager, that would be about it," Davis said during a recent visit to Johns Hopkins Hospital's adult cystic fibrosis clinic, established a decade ago. "The fact they even have an adult clinic is pretty amazing."
Affecting 30,000 people in the United States alone, cystic fibrosis is an inherited disease that causes the body to produce thick, sticky mucus that can clog the lungs and damage the pancreas, interfering with the body's ability to absorb nutrients. As a result, victims are prone to dangerous respiratory infections and struggle to gain weight.
In the early 1960s, the median survival age was 10 years. Desperate parents watched frail children cough their way through life and eventually succumb to infections, even malnutrition.
But a steady progression of drugs, medical devices and improvements in disease management has made it possible for patients not only to survive longer, but also to attend college, have careers and raise families. By the early 1980s, median survival had risen to 21 years. Today, it is 37.
"We now expect children to live into adulthood and to be healthy as well," said Dr. Peter Mogaysal, director of the cystic fibrosis center at the Johns Hopkins Children's Center. "Several decades ago, most people with CF died in childhood, and it was the exception to have patients live to be an older adult."
To achieve that, patients face steep challenges - not just lung infections and the likelihood that they will someday die of the disease, but daily treatments that can sideline them from things they'd rather be doing.
