ABSTRACT
Cystic fibrosis (CF) is the most common fatal genetic disease, but with better understanding of the disease and aggressive treatment, CF patients who once died as infants are living well into adulthood. CF is an autosomal recessive disease that results in multisystem dysfunction, including chronic sino-pulmonary disease, pancreatic exocrine and endocrine insufficiency, hepatobiliary disease, gastrointestinal dysfunction, and male infertility. The classic CF phenotype is a patient diagnosed in infancy as a result of failureto-thrive or recurrent pneumonias who goes on to have chronic bronchiectasis with difficult-to-treat pathogens and eventually dies of respiratory failure as an adolescent or young adult. Better genotype diagnosis of the disease has shown that there is actually a wide range of CF phenotypes that do not necessarily correlate with the genotype. And, more significantly, better treatment for the manifestations of CF has led to increased survival. In the United States, there are about 30,000 CF patients with almost 40% of them older than 18 years of age. The mean life expectancy is 32 years, and there are increasing numbers of patients in their fifth or sixth decade of life (1,2).
