Cystic fibrosis Cystic fibrosis, also known as cystic fibrosis, is a disabling and fatal genetic disease inherited as an autosomal recessive trait (OMIM #219700). This disorder is lethal by age 2 without treatment, making it the most common lethal inherited disorder of childhood. Today, with treatment, those affected can live 25 to 30 years or more. Within the white population of the United States, CF has an average frequency of 1 in 2000 live births. The frequency is significantly lower among other races (Human Heritage). Cystic fibrosis is characterized by abnormal transport of chloride and sodium ions across the epithelium resulting in thick, viscous secretions. Cystic fibrosis affects the pancreas, liver, intestines, breasts, sexual organs, but most critically the lungs ("Cystic Fibrosis", 2011). The production of thick mucus in the pancreas blocks the ducts that carry enzymes, interfering with proper digestion. It also blocks the airways of the lungs causing difficulty breathing and ultimately developing obstructive lung disease and infections that lead to premature death. Most deaths in cystic fibrosis occur due to lung infections (human heredity). Cystic fibrosis is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene on chromosome 7. CFTR is one of 9 chloride channel proteins now known in humans. Provides instructions and transport of chloride ions into and out of cells. This protein regulates the components of sweat, digestive fluids, and mucus. CFTR is a large gene containing 1,480 amino acids, giving rise to high genetic heterogeneity (Wilschanski, 2010). There are over 1,500 mutations that can produce cystic fibrosis (Bobadilla, Macek, Fine, & Farrell, 2002). However, the majority of the diseased population has a codon, delta F508, deletion in the center of the paper. Ours are used "because they reach the airways quickly and easily". Commonly prescribed inhaled medications include mucolytics, antibiotics, bronchodilators, and anti-inflammatory medications. Mucolytics reduce the viscosity of mucus and loosen it. Antibiotics are the main treatment to treat or prevent lung infections. They can be administered orally, by inhalation or intravenously. Bronchodilators relax the muscles around the airways by widening them. These medicines help improve or prevent lung infections, reduce swelling, thin mucus, and dilate the airways. Anti-inflammatory drugs reduce airway swelling due to ongoing infections. (“Therapies for Cystic Fibrosis”, 2012). “Life expectancy in cystic fibrosis (CF) has improved substantially over the past 75 years, with expected median survival now approaching 40 years (Clancy and Jain, 2012).