Cystic Fibrosis (CF) is a multisystem disease that affects the lungs, digestive system, sweat glands and the reproductive tract.1 These patients have an atypical pathway of sodium and chloride across secretory epithelia which causes viscous secretions in the lungs and the digestive tract. Pulmonary disease remains the leading cause of morbidity and mortality in CF patients.2 While this article will focus mainly on the pulmonary treatments of the CF patient it is important to recognize that this is a multisystem disease that requires a specific multidisciplinary focused approach to care. There are over 120 CF centers in the United States where patients are seen on a regular basis by clinicians, dieticians, respiratory therapists, nurses, physical therapists and social workers specifically trained in CF care. These centers improve outcomes.3
Every state in the U.S. screens newborns for CF with a blood test that detects a higher than normal level of immunoreactive trypsinogen (IRT). A follow up test used confirm CF is a sweat test, which is considered the gold standard, done at least two weeks after birth. A sweat chloride concentration of greater than 60 mmol/L is indicative of CF. Genetic testing is another diagnostic tool to confirm or rule out CF. This testing and identification of the CF gene is essential in recognition and instituting early, high quality care.