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Impact of a Standardized Multidisciplinary Quality Improvement Project to Improve Clinical Outcomes in Cystic

Mahitab Morsy Hussein1, Samya Zaki Nasr2, Terez Boshra Kamel1

  • 1Department of Pediatrics, Division of Pediatric Pulmonology, Ain Shams University Pediatric Hospital, Cairo, Egypt.

Pediatric Pulmonology
|April 14, 2026
PubMed

Insights

Quality improvement interventions significantly enhanced lung function and nutrition in children with cystic fibrosis (CF) in low-resource settings. These improvements were further amplified by CFTR modulator therapy, demonstrating a dual benefit for patient outcomes.

Area of Science:

  • Pulmonology and Respiratory Medicine
  • Pediatric Healthcare
  • Quality Improvement Science

Background:

  • Cystic Fibrosis (CF) care in low-resource countries often lags behind international standards, impacting pulmonary outcomes, nutrition, and access to treatments like CFTR modulators.
  • Quality Improvement (QI) methodologies offer a structured framework to address and overcome these disparities in CF care.
  • This study focuses on improving care for pediatric patients with CF in Egypt, a setting with limited resources.

Purpose of the Study:

  • To assess the impact of a 12-month Plan-Do-Study-Act (PDSA)-driven QI intervention on FEV1% predicted (FEV1pp) in pediatric patients with CF.
  • To achieve an absolute increase of at least 5% in mean FEV1pp for patients with baseline FEV1pp ≤ 80%.
  • To differentiate the effects of QI interventions alone versus QI combined with elexacaftor/tezacaftor/ivacaftor (ETI) therapy.

Main Methods:

  • A 12-month study involving 45 pediatric patients (aged 6-21) with CF at Ain Shams University Pediatric Hospital, Egypt.
  • Implementation of sequential PDSA cycles focusing on personalized airway clearance, nutritional support, caregiver education, and telehealth.
  • The study was divided into two phases: Phase 1 (0-6 months, QI only) and Phase 2 (6-12 months), with 56% of patients initiating ETI in Phase 2.

Main Results:

  • In the non-ETI group, mean FEV1pp increased by 7.1% (from 57.9% to 65.0%, p < 0.001), exceeding the 5% target. Mean BMI-for-age Z-scores improved by +0.75 (p < 0.001).
  • Patients initiating ETI in Phase 2 showed greater improvements, with mean FEV1pp rising by 12.2% (from 66.4% to 78.6%, p < 0.001) and mean BMI Z-scores improving by +0.59 (p < 0.001).
  • Significant reductions in antibiotic use were observed throughout the study period.

Conclusions:

  • A tailored, multidisciplinary QI protocol significantly enhanced lung function and nutritional status in pediatric patients with CF in a resource-limited environment.
  • CFTR modulator therapy (ETI) provided additional, clinically meaningful benefits beyond the QI interventions.
  • QI methodologies are effective in improving CF care standards even in settings with resource constraints.
Abstract

Keywords:
BMIFEV1Plan‐Do‐Study‐Act (PDSA) cyclescystic fibrosismultidisciplinary carequality improvement

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