Content area
Full text
The life expectancy for individuals with cystic fibrosis (CF) has dramatically improved over the past 50 years.1 The median length of survival has increased from roughly 1 year in 1950 to > 30 years today. Children born with CF today have an estimated life expectancy of approximately 40 years? There are multiple factors that have contributed to this progress. The development of pancreatic enzyme replacements to treat malabsorption and of effective antipseudomonal antibiotics to suppress airway infection has been crucial. The consolidation of care at Cystic Fibrosis Care Centers sponsored by the US Cystic Fibrosis Foundation, early diagnosis, aggressive disease management, and a host of new therapeutic interventions also have been integral in enhancing quality of life and increasing life expectancy.
The increasing longevity of CF patients has resulted in marked changes in the epidemiology of CF and an aging CF population.3 The most recent Cystic Fibrosis Foundation Patient Registry report indicates a significant increase in the percentage of CF patients classified as adults (>= 18 years), with the total percentage rising from 29.5% in 1988 to 37% in 1998.4 As a result of aging, CF patients are developing a number of CF-related complications as well as "adult" medical problems (eg, reproductive issues) not routinely treated by pediatric pulmonologists. The Cystic Fibrosis Foundation has long recognized the need for internal medicine-trained pulmonologists to care for the growing number of adult CF patients and has recommended, in a...