Cystic fibrosis (CF) is a genetic disease affecting lungs, digestive system, reproductive system and sweat glands. Life expectancy has increased from only a few years in 1938, when the disease was first described, to approximately 37 years today. Identification of the mutated gene in 1989 initiated research into therapies targeted at the underlying defect. Dr. James L. Kreindler of Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, provided a review.
Damage to the pancreas was the first recognized manifestation of cystic fibrosis. Today, pancreatic enzyme replacement therapy enables patients to survive with adequate nutritional status. The healthy pancreas produces amylase, lipase, and protease that are necessary for digestion. Approximately 90 percent of CF patients can't produce sufficient amounts of these enzymes on their own.
“Survival of infancy and early childhood because of better nutrition resulted in lung disease rather than malnutrition being the most common cause of mortality in CF,” Kreindler reported.