The Changing Face of Cystic Fibrosis

Aug. 2, 2006 — -- It may seem like a primitive punishment, but for years pounding on a patient's back was one of the few effective treatments for cystic fibrosis.

Cystic fibrosis is a genetic disease where mucus clogs the lungs and pancreas, making breathing and digestion difficult.

The pounding helped loosen the mucus, but it didn't help most patients live past childhood.

In the last few decades, however, more extensive research has led to new treatments that have improved the length and quality of life for CF patients like Anne Governor.

The 25-year-old just had a baby and knows she is more fortunate than other CF victims.

"I've been very lucky," Governor said. "There're people my age that don't do as well."

CF affects 30,000 people in the United States.

Governor was diagnosed with CF as a baby in 1981, when patients were usually dying in their teens.

As a child, she took medication to help her digest food, and later inhaled antibiotics to protect her lungs from infection. She also made sure to stay active to help her lungs stay strong.

"I enjoyed running, and I played fullback when I played soccer," Governor said. "The tiniest person on the team was on defense, and it was a lot of fun."

As Governor grew, so did the average age of survival of CF patients. Today, patients on average live until age 38.

"Now, somewhat around 40 percent of the people with CF are over 18 years of age," said Dr. Rob Horowitz of the University of Rochester Medical Center.

"So yes, it's a childhood disease, but the majority of children with cystic fibrosis become adults with cystic fibrosis."

What is changing the face of cystic fibrosis?

In part, research has found new ways to limit and treat the infections and lung damage caused by thick mucus.

"Daily care, maintenance care, focusing on excellent nutrition, focusing on removing that mucus from the lungs, inhaled antibiotics have clearly offered some benefit to a lot of folks," Horowitz said.

New research has also changed the way doctors approach treating CF patients.

"All of these things are [here] not necessarily to treat someone after they are acutely ill, but to help them remain healthy," said Ann McMullen, a certified pediatric nurse practitioner at the University of Rochester Medical Center. "It's no longer treat them when they're sick, sick, sick, but help them to stay well."