Cystic Fibrosis

CF is the most common genetic disease in the Unites States affecting about 30,000 children and adults (70,000 worldwide). The genetic defect renders certain organs of the body susceptible to obstruction due to thick mucus secretions. The most severe manifestation is in the lung where thick secretions lead to chronic lung infections which require a daily regimen of drug treatments and chest physical therapy to help clear airway secretions. Thick secretions in the pancreas compromise food absorption leading to nutritional deficiencies that can be partially overcome with nutritional supplements and oral enzyme replacement. Advances in the clinical management of CF have improved the prognosis, although current life expectancy is 37.4 years of age (median). There is no cure for CF, however, lung transplantation is the only life saving treatment in those with end stage lung disease.