Cystic fibrosis patients living longer, and fuller, lives
By Mary Loftus | Emory Magazine | Aug. 6, 2013
A dire warning was issued in an eighteenth-century German medical text: “Woe is the child kissed on the brow who tastes salty, for he is cursed and soon must die.”
“Even then, they knew about the dangers of this disease,” says pulmonologist Seth Walker, director of Emory’s Adult Cystic Fibrosis Program.
More than thirty thousand people in the US have cystic fibrosis (CF), a genetic disorder of epithelial cells, which form the linings of airways, ducts, and most organs. CF results in especially viscous mucous that makes it harder to breathe and digest food, causes frequent respiratory infections, and can damage the lungs, liver, and intestines.
The disease also produces more salt in sweat, and this sweat test is how many with the disorder are first diagnosed.
Reaching adulthood with CF is a fairly recent development.
In 1955, children with the disorder were not expected to live long enough to attend grade school. In the 1960s and 1970s, patients seldom lived beyond their teenage years. In the 1980s, the average life expectancy was eighteen.
With more research and knowledge about the disease and more aggressive treatments, however, physicians have been able to extend the lifespan of those with CF to the mid- to late thirties. The once-fatal pediatric disease has become a chronic condition, creating new medical demands.
“We started noticing that adults with CF were still being treated by pediatricians, and adult clinics were established,” says Walker.