Cystic fibrosis (CF) is an autosomal recessive genetic disease, characterized by pulmonary and sinus disease, gastrointestinal and reproductive tract dysfunction. CF patients suffer from chronic repeated cycles of pulmonary bacterial infection, pulmonary exacerbations and chronic lung function decline, which often lead to premature death. Although improved treatment of lung disease has increased survival, the median predicted age for survival is only 37.2 years in the United States (US) (CFF Annual Data Report, 2008) and patients continue to have significant morbidity, including hospitalizations (Ramsey, 1996).