Managing Cystic Fibrosis: Strategies that Increase Life Expectancy and Improve Quality of Life.
The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and cloning of the CFTR gene over 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 as 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 pulmonar...