Faculty Collaboration Database

Cystic fibrosis-related mortality in the United States from 1999 to 2020: an observational analysis of time trends and disparities. Sci Rep 2023 Sep 12;13(1):15030

Date

09/13/2023

Pubmed ID

37699961

Pubmed Central ID

PMC10497589

DOI

10.1038/s41598-023-41868-x

Scopus ID

2-s2.0-85170692540 (requires institutional sign-in at Scopus site)   22 Citations

Abstract

Cystic fibrosis transmembrane conductance regulator modulators have revolutionized cystic fibrosis (CF) care in the past decade. This study explores the CF-related mortality trends in the US from 1999 to 2020. We extracted CF-related mortality data from the CDC WONDER database. CF age-standardized mortality rates (ASMRs) were identified by ICD-10 code E84 and were stratified by demographic and geographical variables. Temporal trends were analyzed using Joinpoint modeling. CF-related ASMRs decreased from 1.9 to 1.04 per million population (p = 0.013), with a greater reduction in recent years. This trend was replicated in both sexes. The median age of death increased from 24 to 37 years. CF mortality rates decreased across sex, white race, non-Hispanic ethnicity, census regions, and urbanization status. Incongruent trends were reported in non-white races and Hispanic ethnicity. A lower median age of death was observed in women, non-white races, and Hispanic ethnicity. SARS-CoV-2 infection was the primary cause of death in 1.7% of CF decedents in 2020. The national CF-related mortality rates declined and the median age of death among CF decedents increased significantly indicating better survival in the recent years. The changes were relatively slow during the earlier period of the study, followed by a greater decline lately. We observed patterns of sex, ethnic, racial, and geographical disparities associated with the worsening of the gap between ethnicities, narrowing of the gap between races and rural vs. urban counties, and closing of the gap between sexes over the study period.

Author List

Singh H, Jani C, Marshall DC, Franco R, Bhatt P, Podder S, Shalhoub J, Kurman JS, Nanchal R, Uluer AZ, Salciccioli JD

Authors

Rose Franco MD Professor in the Medicine department at Medical College of Wisconsin
Jonathan S. Kurman MD Associate Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adult
Cystic Fibrosis
Female
Humans
Male
United States
Young Adult