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Updated: Aug 6, 2026
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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
Published on: April 11, 2018
Cystic Fibrosis Mortality Trends 1999-2024-A CDC Wonder Study
Palak Grover1, Rahul Jain2, Gurleen Kaur3
1Henry Ford Jackson Hospital, Jackson, MI 49201, USA.
Advances in Respiratory Medicine
|July 24, 2026
Summary
Cystic fibrosis (CF) mortality has more than halved since CFTR modulator therapies became available, with significant declines observed since 2016. Further research is needed to understand regional disparities and sex-based trends.
Area of Science:
- Pulmonology
- Genetics
- Public Health
Background:
- Cystic fibrosis (CF) is a genetic disorder impacting multiple organs.
- CFTR modulator therapies have transformed CF treatment since 2012.
- Population-level mortality trends across CFTR modulator eras are not well-established.
Purpose of the Study:
- To assess US cystic fibrosis mortality trends from 1999-2024.
- To evaluate mortality changes across pre-modulator, early modulator, and ETI therapy periods.
- To identify temporal shifts and potential disparities in CF mortality.
Main Methods:
- Retrospective analysis of US death data (1999-2024) using CDC WONDER.
- Calculation of age-adjusted mortality rates (AAMR) per 100,000.
- Segmented log-linear Poisson regression to analyze annual mortality trends and identify change points.
Main Results:
- Total CF deaths decreased by over 50% from the pre-modulator to the ETI period.
- Significant mortality decline observed from 2016-2024 (APC -9.76%/year).
- Regional shifts and changes in sex-based death ratios noted, requiring further investigation.
Conclusions:
- CFTR modulators have dramatically reduced US cystic fibrosis mortality.
- Observed trends suggest potential geographic and sex-based disparities.
- Ecological data cannot confirm causation; other factors may contribute to trends.
