1999 to 2020 saw decrease in cystic fibrosis-related mortality

Cystic fibrosis (CF)-related mortality rates declined and the median age of death among CF decedents increased in the United States from 1999 to 2020, according to a study published online Sept. 12 in Scientific Reports.

Harpreet Singh, R.N., from the Medical College of Wisconsin in Milwaukee, and colleagues examined CF-related mortality trends in the United States from 1999 to 2020 using mortality data from the U.S. Centers for Disease Control and Prevention WONDER database.

The researchers found that CF-related age-standardized mortality rates decreased from 1.9 to 1.04 per million population, with a greater reduction seen in recent years. This trend was seen in both sexes. Over time, the median age of death increased from 24 to 37 years. Decreases in mortality rates were seen across sex, White race, non-Hispanic ethnicity, census regions, and urbanization status. Among patients of non-White race and Hispanic ethnicity, trends were not consistent.

The median age of death was lower among women, non-White races, and Hispanic ethnicity. In 2020, severe acute respiratory syndrome coronavirus 2 infection was the primary cause of death in 1.7 percent of CF decedents.

"Due to the observational nature of this data, we are unable to make causal statements about the exact causes of the significant decrease in CF-related mortality recently," the authors write. "However, a potential contributor to the decreasing CF mortality could be the introduction of cystic fibrosis transmembrane conductance regulator modulator therapy."

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