Editor's Notebook
A number I hope reaches zero
I spend a great deal of time working with data. Occasionally, a small set of numbers makes me stop. In the CDC’s national mortality files, I found the following numbers of deaths before the first birthday with cystic fibrosis mentioned on the death certificate: 5 in 2019, 4 in 2020, 8 in 2021, 7 in 2022, 5 in 2023, and 4 in 2024. Within those totals, CF was recorded as the underlying cause in 2, 3, 4, 4, 2, and 3 deaths, respectively. The numbers are small. The losses they represent are not.
Emerging research on prenatal CFTR modulator treatment gives me hope that we may eventually prevent some of these losses. Early clinical reports describe improvement in fetal intestinal complications, including meconium ileus. That is encouraging, although it is not yet evidence that prenatal treatment reduces infant mortality. Treatment during pregnancy also requires careful, individualized assessment of benefits and risks.
During nearly 18 years working with the CF Patient Registry, I rarely encountered recorded infant deaths. Finding these deaths in the national death records brings another part of the CF story into view, one that may not be fully captured in the CF Registry. CDC also maintains linked birth and infant death records, providing another national resource for understanding infant outcomes. These records are not perfect. CF may be unrecognized, omitted, or incorrectly recorded. Nor do the mortality files identify prenatal modulator exposure. With so few deaths each year, a change of one or two can look substantial. Changes in diagnosis, recording, and the number of babies born with CF also matter. A decline would therefore deserve attention, but would not, by itself, establish a treatment effect. Still, I believe these numbers are worth following as prenatal treatment and early CF care evolve. My hope is simple: fewer families experiencing this loss, and more children reaching their first birthday and many birthdays beyond it. I would love to see a year with no infant deaths recorded with CF, and evidence that this reflects real progress rather than missed diagnoses.