loading page

Outcomes of infants born during the first 9 years of CF newborn screening in the United States: successes and the need for improvement
  • +4
  • Stacey Martiniano,
  • Alexander Elbert,
  • Philip Farrell,
  • Clement Ren,
  • Marci Sontag,
  • Runyu Wu,
  • Susanna McColley
Stacey Martiniano
University of Colorado at Denver - Anschutz Medical Campus
Author Profile
Alexander Elbert
Cystic Fibrosis Foundation
Author Profile
Philip Farrell
University of Wisconsin School of Medicine and Public Health
Author Profile
Clement Ren
Indiana University System
Author Profile
Marci Sontag
CI International
Author Profile
Runyu Wu
Cystic Fibrosis Foundation
Author Profile
Susanna McColley
Ann and Robert H Lurie Children's Hospital of Chicago
Author Profile

Abstract

Introduction: Newborn screening (NBS) for cystic fibrosis (CF) was implemented in all US states and DC by 2010. This hypothesis generating study was designed to form the basis of additional research and to plan quality improvement initiatives. The aims were to describe the outcomes of infants with CF born during the first 9 years of universal NBS. Methods: We included participants in the CF Foundation Patient Registry born 2010-2018 with age at first CF event (first sweat test, clinic visit or hospitalization) by age 365 days. We assessed age of center-reported diagnosis, age at first CF event, demographics and outcomes for three consecutive 3-year cohorts born in 2010-2012, 2013-2015, and 2016-2018. Results: In 6354 infants, median age at diagnosis was earlier than median age at first CF event, which decreased from 1st cohort to 3rd cohort. Weight-for-age (WFA) was < 10th percentile in about 40% of infants at the first CF Center visit. Median WFA z-score at 1-2 years was > 0 but height-for-age (HFA) z-score was < 0 through age 5-6 years. The second cohort had a higher HFA z-score than the first cohort at age 5-6 years. Pseudomonas aeruginosa infection rates decreased over time. About 1/3 of infants were hospitalized in the first year of life across cohorts. Conclusion: Over 9 years of CF NBS, median age at first CF event decreased. CF NBS had positive health impacts but improving nutritional deficits and reducing infant hospitalizations remain targets for improvement.

Peer review status:UNDER REVIEW

17 Feb 2021Submitted to Pediatric Pulmonology
18 Feb 2021Submission Checks Completed
18 Feb 2021Assigned to Editor
18 Feb 2021Reviewer(s) Assigned
27 Mar 2021Review(s) Completed, Editorial Evaluation Pending
28 Mar 2021Editorial Decision: Revise Minor
25 May 20211st Revision Received
25 May 2021Submission Checks Completed
25 May 2021Assigned to Editor
25 May 2021Reviewer(s) Assigned
07 Jun 2021Review(s) Completed, Editorial Evaluation Pending