Management of the Adult Patient with Cystic Fibrosis

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Abstract
Cystic fibrosis (CF) is an autosomal recessive genetic disease that results in impaired fluid and electrolyte transport across epithelial surfaces. More than 7 million white Americans are asymptomatic carriers of the CF gene, which results in a child afflicted with CF in 1 of every 2500 live births. Advances in the care and understanding of this disease process have extended the life expectancy remarkably in the last 2 decades, and the future is bright for new and innovative treatments to continue this trend. Previously a disease of the pediatric population and cared for primarily by pediatric subspecialists, CF is now a challenge to adult medical and surgical subspecialists. Respiratory, gastrointestinal, upper respiratory, infectious, nutritional, and reproductive complications in this patient population mandate a foundation of knowledge and a coordinated multidisciplinary approach to provide optimal care. Clin Pulm Med 1995;2(2):75-87

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