Toward a Broader Understanding of Cystic Fibrosis Epidemiology and Its Impact on Clinical Manifestations

Abstract

The incidence of cystic fibrosis remains constant in North America and Western Europe is 1 in 3500 live births, but survival and quality of life have improved. The cystic fibrosis population has shifted toward the adult age range with a concomitant shift in the spectrum of complications. Survival increased because of aggressive symptomatic therapy, earlier diagnosis by newborn screening, and the introduction of modulators of the cystic fibrosis transmembrane conductance regulator, so that predicted median survival age is now about 50 years. In the United States, members of low socioeconomic status populations or members of racial or ethnic minorities have benefitted less from these advances.

Keywords: Cystic; Cystic fibrosis; Cystic fibrosis epidemiology; Cystic fibrosis incidence; Fibrosis survival.