About Cystic Fibrosis

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive systems of about 30,000 children and adults in the United States and 70,000 people worldwide. CF is caused by a recessive gene, which means that a child must inherit two copies of the defective CF gene—one from each parent—to have the disease. It is most common in Caucasians, but people of all races can have CF. Because of a defective gene and the protein it produces, the body makes unusually thick, sticky mucus. This mucus clogs the lungs and can lead to life-threatening lung infections. It also can affect the pancreas and block release of enzymes that help the body break down and absorb food. CF may affect other systems as well.

Treatment for CF depends upon the severity of symptoms and the organs involved. Most people with CF must take pancreatic enzyme supplements with every meal to absorb enough calories and nutrients to grow and stay healthy. They must eat a high-calorie, high-fat diet. People with CF also use daily therapy to help clear mucus from the lungs. Other types of treatments include medications to fight lung infections and thin the mucus and improve lung function.

People with CF work hard absolutely every day to stay healthy. Many participate in sports, attend school, work full time, marry and raise children. The bad news is that many don't get the whole ride through life and almost all have to rise above difficult challenges to have a "normal" life. The good news? The average life expectancy for CF is now 37.4 years and over a third of people with CF in the US are now adults. Great progress has been made in the treatment of CF and the prospects are great for even better treatments and eventually a cure.

- Cindy Capen, MSN, RN
Pediatric Pulmonary Center
University of Florida Health Science Center