Changing Epidemiology of Pediatric Pulmonary Exacerbations in Cystic Fibrosis

Yaron Fireizen, Department of Pediatrics, Rady Children's Hospital, University of California San Diego, San Diego, California, USA.
Mohamoud Ahmed, University of Colorado School of Medicine, Aurora, Colorado, USA.
Timothy Vigers, Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
Kathryn Akong, Department of Pediatrics, Rady Children's Hospital, University of California San Diego, San Diego, California, USA.
Julie Ryu, Department of Pediatrics, Rady Children's Hospital, University of California San Diego, San Diego, California, USA.
Andrea Hahn, Department of Pediatrics, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Hani Fanous, Department of Pediatrics, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Anastassios Koumbourlis, Department of Pediatrics, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, District of Columbia, USA.
Pornchai Tirakitsoontorn, Department of Pediatrics, Children's Hospital of Orange County, Division of Pulmonology, University of California Irvine, Orange, California, USA.
Antonio Arrieta, Division of Infectious Diseases, Children's Hospital of Orange County, University of California Irvine, Orange, California, USA.
Elizabeth B. Burgener, Department of Pediatrics, Children's Hospital of Los Angeles, Division of Pulmonology, Keck School of Medicine at University of Southern California, Los Angeles, California, USA.
Jonathan Koff, Section of Pulmonary, Critical Care & Sleep Medicine, Department of Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.
Jonathan D. Cogen, Department of Pediatrics, Seattle Children's Hospital, Division of Pulmonary and Sleep Medicine, University of Washington, Seattle, Washington, USA.
Drake C. Bouzek, Department of Pediatrics, Seattle Children's Hospital, Division of Pulmonary and Sleep Medicine, University of Washington, Seattle, Washington, USA.
Elin Hanley, Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
Allison Keck, Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
Dayna Stout, Rady Children's Hospital, San Diego, California, USA.
John Bradley, Department of Pediatrics, Rady Children's Hospital, University of California San Diego, San Diego, California, USA.
Scott D. Sagel, Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.

Document Type

Journal Article

Publication Date

3-1-2025

Journal

Pediatric pulmonology

Volume

60

Issue

3

DOI

10.1002/ppul.71019

Keywords

airway infection; children; epidemiology; health disparities; pulmonary exacerbations

Abstract

RATIONALE: The introduction of elexacaftor/tezacaftor/ivacaftor (ETI), a highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy, to younger ages and the COVID-19 pandemic have significantly reduced pulmonary exacerbations requiring hospitalization among children with CF. OBJECTIVE: To assess demographic and clinical characteristics of children and young adults with CF hospitalized for pulmonary exacerbations before and after pediatric ETI approval. METHODS: A retrospective chart review was conducted at five United States CF Foundation-accredited care centers. Hospitalization data from children and young adults with CF in 2018 and 2022 were analyzed. RESULTS: Hospitalizations decreased from 471 cases (241 individuals) in 2018 to 163 cases (110 individuals) in 2022. The racial distribution shifted, with more hospitalized patients identifying as people of color in 2022 (28% vs. 14%; p = 0.018). A greater proportion of hospitalized children in 2022 had two non-F508del mutations compared with children hospitalized in 2018 (38% vs. 19%) and were less likely to be infected with methicillin-resistant Staphylococcus aureus (MRSA). Comparing 2022-2018, children on CFTR modulator therapy, including ETI (76%), showed reduced infections with Pseudomonas aeruginosa and Achromobacter xylosoxidans. CONCLUSIONS: The decline in hospitalizations for pulmonary exacerbations likely reflects the benefits of ETI therapy, as a higher proportion of children and young adults hospitalized in 2022 had two non-F508del mutations and were not eligible for ETI. A greater percentage of those hospitalized in 2022 identified as belonging to minority racial groups, highlighting ongoing health disparities in the ETI era. Additionally, there were notable changes in the microbiological characteristics between 2018 and 2022.

Department

Pediatrics

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