Cystic fibrosis: an update for the otolaryngologist

Cystic fibrosis (CF) is an autosomal recessive disorder affecting exocrine gland function. Formerly a deadly disease of infants and children, recent improvements in antibiotics, nutritional therapy, and supportive care have extended the median survival to adulthood. Patients with CF commonly go to an otolaryngologist with a myriad of medical and surgical problems. Fortunately, the treatment and management of CF have rapidly evolved during the past few years, and changes in diagnosis, prevention, and treatment are occurring almost daily. As physicians on the forefront of care for patients with CF, otolaryngologists need to be aware of the new developments in the management of CF. An overview of CF is presented with an emphasis on the current treatment strategy and what the next decade brings for this formerly lethal disease.