Cystic Fibrosis

Cystic fibrosis (CF) is genetic disorder caused by abnormalities in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) resulting in excessive mucus accumulation in various organ systems. Pulmonary disease is the leading cause of morbidity and mortality in people with CF. Mainstays of CF management have been targeted at improving lung function and nutrition as well as decreasing respiratory infections. Newer therapies called CFTR modulators have better targeted the specific abnormalities seen in people with CF resulting in fewer hospitalizations and a focus on optimizing outpatient care.