title: Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life.
abstract: The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and cloning of the CFTR gene more than 21 years ago led to the identification of the structure and function of the CFTR chloride channel. New therapies based on the understanding of the function of CFTR are currently under development. The better clinical status and improved survival of patients with CF is not only a result of understanding of the molecular mechanisms of CF but also a result of the development of therapeutic strategies that are based on insights into the natural course of the disease. Current CF treatments that target respiratory infections, inflammation, mucociliary clearance, and nutritional status are associated with improved pulmonary function and reduced exacerbations. Patients benefit from treatment at a specialized CF center by a multidisciplinary dedicated team with emphasis being placed on frequent visits, periodic testing, and monitoring adherence to therapy. The purpose of this review is to survey recent developments in CF care that are responsible for the improved survival and quality of life of patients with CF.
authors.affiliation: Department of Pediatrics and CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors.forename: Malena
authors.initials: M
authors.lastname: Cohen-Cymberknoh
authors.forename: David
authors.initials: D
authors.lastname: Shoseyov
authors.forename: Eitan
authors.initials: E
authors.lastname: Kerem
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications / Q000235:genetics / Q000628:therapy*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D006801:Humans; D007249:Inflammation / Q000150:complications / Q000628:therapy; D008017:Life Expectancy; D009079:Mucociliary Clearance; D011552:Pseudomonas Infections / Q000517:prevention & control; D011550:Pseudomonas aeruginosa; D011788:Quality of Life
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator