Clinical Course and Prognosis

Life expectancy has greatly increased from a predicted survival of 16 years in 1970 to more than 40 years for patients born in the 1990s.5 The average age of patients in the Cystic Fibrosis Foundation Registry is now more than 16 years, and almost 45% of CF patients in the 2006 Registry annual report are over 18 years old with the oldest

being age 78.

The clinical course varies greatly among patients because of the multiple genetic mutations and heterogeneous profile of the AF508 mutation. Some patients develop severe lung disease early in childhood and reach end-stage lung disease by their teens, whereas others maintain near-normal lung function into adulthood. Newly diagnosed adults tend to present with chronic respiratory symptoms but usually have milder lung disease, less frequent Pseudomonas infection, and less severe pancreatic insuffi-


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