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Improvements in Clinical Outcomes in Children with Cystic Fibrosis aged Six and 16 years

B P Finn1, S R Millar2, K Cronin3

  • 1Department of Paediatrics and Child Health, Cork University Hospital.

Irish Medical Journal
|May 16, 2022
PubMed

Insights

Outcomes for cystic fibrosis (CF) patients have significantly improved over 17 years. Lung function (FEV1) and body mass index (BMI) increased, while chronic pseudomonas infections decreased in children and adolescents.

Area of Science:

  • Pediatric Pulmonology
  • Cystic Fibrosis Research
  • Clinical Outcomes

Background:

  • Cystic Fibrosis (CF) impacts multiple organs, primarily the lungs.
  • Long-term outcomes for CF patients have historically been poor, with significant morbidity and mortality.
  • Assessing trends in key health indicators is crucial for evaluating care improvements.

Purpose of the Study:

  • To evaluate improvements in health outcomes for pediatric CF patients over a 17-year period.
  • To analyze changes in FEV1, BMI, and chronic Pseudomonas aeruginosa infection rates.
  • To determine if clinical outcomes have improved in CF patients aged six and 16 years.

Main Methods:

  • Retrospective observational study utilizing a prospectively maintained CF patient database.
  • Inclusion of patients aged six and 16 years from Cork University Hospital.
  • Analysis of data from three distinct time periods: 2002-2007, 2008-2013, and 2014-2018.

Main Results:

  • Significant improvements observed in mean FEV1 and BMI for 16-year-olds across the study periods, particularly in 2014-2018.
  • Mean FEV1 for six-year-olds also showed improvement over time.
  • A dramatic reduction in the percentage of patients with chronic Pseudomonas status was noted in the 2014-2018 group (4.2%) compared to earlier periods (37.9% and 51.6%).

Conclusions:

  • Notable improvements in FEV1 and BMI at ages six and 16 indicate better patient health.
  • A significant decline in chronic Pseudomonas infections suggests effective management strategies.
  • These findings highlight positive trends in CF patient care and outcomes over the past 17 years.

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