Remember the money you put into that donation canister marked for cystic fibrosis research?

Your donations have helped give more years of life to children who 30 years ago would have died before finishing fifth grade, says a University of Iowa Carver College of Medicine researcher. Today, though no cure yet exists, research and care advances have resulted in most children with cystic fibrosis surviving to adulthood.

Cystic fibrosis is an inherited disorder resulting from a mistake in the genes. In Iowa, it occurs in about one in every 2,000 live births, says Dr. Miles Weinberger, head of the UI pediatric allergy and pulmonary division and the UI Cystic Fibrosis Center. The error causes abnormalities in various glands and organs in the body.

One of the organs involved in cystic fibrosis is the pancreas, which contains glands that produce digestive enzymes. The deficiency of pancreatic enzymes in children with the disease can now be treated successfully with medication.

The other glands involved--the mucous glands in the lungs and breathing passages--are much more resistant to treatment, Weinberger says. In cystic fibrosis, production of abnormally thick mucus prevents the respiratory tract from protecting itself from bacteria. As a result, chronic lung infections lead to progressive damage to the airways. Once this tissue destruction advances to the point that the lungs can't function well enough to support life, the cystic fibrosis patient dies. Longer survival appears to be a result of earlier diagnosis leading to earlier treatment, Weinberger says. Physicians are becoming more aware of the disease and spotting milder cases. In the past, mild cases often were misdiagnosed, he says.

A new development in care of cystic fibrosis is the addition of cystic fibrosis to the newborn screening program in Iowa and other states. Diagnosing CF before symptoms occur enables initiation of treatment before damage has occurred.

Survival also is increasing due to more vigorous support measures. These include mechanical treatments to clear the airways of thick mucus and antibiotics to combat lung infections.

The underlying abnormality that causes cystic fibrosis is not correctable, however, Weinberger says. Treatment only slows the progression of the disease and improves the quality and duration of the patient's life. Treatment cannot reverse damage that has already occurred, he notes.

The UI Cystic Fibrosis Center, one of about 100 in the country, is the only unit in Iowa involved in research and teaching in addition to patient care. Over 200 patients participate in the program at UI Hospitals and Clinics. The oldest patient is over 60, and many in their 20s and 30s are doing well, Weinberger says.