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Cystic fibrosis (CF) was, in the past, typically described as the most common lethal genetic disease in the white population, but the outlook for people with a CF diagnosis has improved dramatically with advancements in treatment, and new therapies are expected to lead to even greater changes. The US Cystic Fibrosis Foundation (CFF) median projected survival age in 2019 (before generalized availability of highly effective modulator therapies, described later, in Management) was 48.4 years (95% CI, 45.9–51.5 years) (Figure 45-1).1  Birth prevalence varies with racial or ethnic background; CF occurs in approximately 1 in 3,000 white Americans...

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