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Transforming Care in Cystic Fibrosis: The Long-Term Impact of Quality Improvement Projects on Clinical Outcomes

S Karabulut1, M Yuksel Kalyoncu1, M Selcuk Balcı1

  • 1Department of Pediatrics, Division of Pediatric Pulmonology, Marmara University School of Medicine, Istanbul, Turkey.

Pediatric Pulmonology
|March 27, 2026
PubMed

Insights

A quality improvement program improved BMI in children with cystic fibrosis (CF) and maintained adult lung function over five years. These findings highlight the benefits of sustained QI efforts for CF patient outcomes.

Area of Science:

  • Pulmonology
  • Quality Improvement Science
  • Pediatric Medicine

Background:

  • Cystic Fibrosis (CF) requires ongoing management to optimize patient outcomes.
  • Quality Improvement (QI) programs are essential for enhancing standard care in CF centers.
  • Longitudinal data on the impact of QI in specific CF populations, like Turkish patients, is limited.

Purpose of the Study:

  • To assess the 5-year impact of a structured QI program on body mass index (BMI) and lung function (FEV₁) in people with CF (pwCF).
  • To evaluate changes in BMI z-scores and FEV₁ percent predicted (FEV₁pp) in pediatric and adult pwCF.
  • To explore the association between BMI, lung function, and treatment burden.

Main Methods:

  • Retrospective review of medical records for 440 pwCF from January 2018 to December 2022.
  • Utilized data submitted to the European Cystic Fibrosis Society Patient Registry (ECFSPR).
  • Employed linear mixed-effects regression models to analyze time-dependent changes in FEV₁pp.

Main Results:

  • Significant improvement in BMI z-scores observed in pediatric pwCF (p < 0.001); adult BMI remained stable (p = 0.10).
  • No significant linear change in mean FEV₁pp was detected in adjusted models (p > 0.05), but median FEV₁pp increased in adults.
  • Higher BMI correlated with better lung function (p < 0.001) and reduced IV antibiotic use (p < 0.001 for children, p = 0.045 for adults).
  • Estimated mean survival age for the cohort was 46.1 years.

Conclusions:

  • This study presents the first 5-year longitudinal registry data and survival estimates for Turkish pwCF.
  • Sustained, center-wide QI initiatives can maintain or improve clinical outcomes in CF care.
  • QI programs are particularly valuable in settings with restricted access to CFTR modulators.
Abstract

Keywords:
childrencystic fibrosislung functionnutritionpatient registrysurvival analysis

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