Cystic fibrosis: current trends in respiratory care

Abstract

Cystic fibrosis is a genetic disease that typically produces malnutrition and chronic respiratory infections. Prolonged bronchial obstruction, infection, and inflammation result in bronchiectstasis and permanent lung damage. Most cystic fibrosis patients die because of this progressive respiratory disease. Thus, in the absence of a cure, effective respiratory therapy is the primary means to extend and improve the quality of life for the cystic fibrosis patient. Aerosol therapy, airway clearance techniques, and noninvasive ventilation can all improve quality of life and possibly extend survival. Close patient monitoring with pulmonary function testing, chest radiography, and induced sputum can result in earlier treatment, potentially reducing permanent lung damage. Earlier diagnosis has prevented serious complications through early initiation of preventive therapies such as improved nutrition.