Cystic fibrosis (CF) affects an estimated 30,000 people in the United States1 and is caused by a mutation in the gene encoding a protein called CF transmembrane regulator (CFTR). This protein functions as a chloride channel in epithelial cells of multiple organ systems. The mutation results in a dysfunctional or absent CFTR channel and a decrease in chloride secretion, which ultimately results in increased viscosity of secretions. Children with CF are less able to clear pulmonary secretions and become colonized with bacterial pathogens that regularly cause exacerbation of lung disease. The hallmarks of CF are recurrent pulmonary exacerbations and declining pulmonary function. Respiratory failure is the cause of death in more than 90% of people with CF. However, there are also extrapulmonary manifestations of CF that affect both health and quality of life. These include CF related diabetes, liver disease, and bone disease. The median age of survival for patients with CF has been increasing steadily and is currently more than 37 years.1 With this improvement in life expectancy, it has become increasingly important to address the long-term complications of CF.