UNC Fights to Give Breath to Cystic Fibrosis Patients

Researchers at the University of North Carolina at Chapel Hill are on the cutting edge of treating the root cause of this disease, however.

Kyle McRee, 14, of Thomasville, has cystic fibrosis. Although the disease can affect other organs, it is most critical in his lungs.

Cystic fibrosis patients lack a micro-thin layer of fluid to lubricate surfaces of their lung's airways. Sticky mucus can then build up and trap bacteria in the airways, leading to infections.

"Patients can have the same bacterial infection for their whole lives, so they never, ever clear it," Dr. Richard Boucher, a UNC professor of medicine, said.

Research at UNC has helped lead to treatments that replace lung lubricant. In one, patients inhale a simple aerated salt solution called hypertonic saline. Gently pounding a cystic fibrosis patient has always helped clear mucus, as have exercise and a vibrating vest.

McRee said he keeps a vest at home, "and I just sit on this little paddle that shakes me for 20 minutes."

UNC researchers have learned why these therapies work: They release a hormone that keeps lung surfaces moist. Researchers are looking for ways to stimulate that hormone more easily and more often.

UNC pediatric pulmonologist Dr. Charles Esther helped develop a method to measure those hormone levels before, during and after stimulation.

A patient breathes into a plastic tube sheathed by a freezing aluminum tube. Breath droplets containing the hormone freeze on the surface of the tube and can be collected, stored and analyzed.

Using a flutter valve also seems to stimulate the hormone and help clear the lungs. A patient breathes into a tube, forcing air into a tear-drop-shaped chamber.

"It's generating a pressure wave that is going into the airway and clearing out some of the mucus, the secretions that build up in CF (cystic fibrosis)," Esther said.

Boucher said he dreams that one day, children will be able to manage cystic fibrosis as easily as they control asthma.

"(The dream) is to have therapies that patients maybe can inhale once in the morning and once in the evening, and you prevent lung disease," Boucher said.

Boucher said that dream may actually happen in McRee's lifetime.