Cystic fibrosis is the most common autosomal recessive fatal disease among whites. Life expectancy is now at 31 years of age. The major cause of morbidity and mortality is chronic progressive lung disease. Lung disease occurs early in cystic fibrosis, suggesting the need for early and aggressive treatment of any pulmonary symptoms and malnutrition. This treatment is ideally performed within a cystic fibrosis center, and new therapies are being sought for management of the early stages of disease.