Impact of a Standardized Multidisciplinary Quality Improvement Project to Improve Clinical Outcomes in Cystic Fibrosis: A 12-Month Phased Project at Ain Shams University.

BACKGROUND

In low-resource countries, cystic fibrosis (CF) care often falls below international standards, particularly regarding pulmonary outcomes, airway clearance, nutrition, cystic fibrosis transmembrane conductance regulator (CFTR) modulator availability, and multidisciplinary care. Quality improvement (QI) methodologies provide structured approaches to address these deficiencies.

OBJECTIVE

To evaluate whether a 12 months Plan-Do-Study-Act (PDSA)-driven QI intervention could yield an absolute ≥ 5% increase in mean FEV1pp among people with cystic fibrosis (PwCF) with baseline FEV1pp ≤ 80%, and to distinguish the effects of the QI interventions over 12 months in patients who did not receive elexacaftor/tezacaftor/ivacaftor (ETI) at any time during the projec period (non-ETI group), from those in patients who initiated ETI after 6 months, in order to assess the additional impact of therapy beyond the QI interventions.

METHODS

Between May 2024 and May 2025, 45 PwCF aged 6-21 years were enrolled at Ain Shams University Pediatric Hospital, Egypt, in collaboration with the University of Michigan CF Center. Sequential PDSA cycles implemented personalized airway clearance, intensified nutritional support, caregiver education, and telehealth follow-up. The project comprised Phase 1 (0-6 months, QI-only) and Phase 2 (6-12 months), during which 56% initiated ETI. Outcomes included FEV1pp, BMI-for-age Z-scores, status, and antibiotic use.

RESULTS

Baseline mean age was 9.9 ± 3.5 years, mean FEV1pp 60.6 ± 16.7%, and BMI-for-age Z-score -1.05 ± 1.29. In the non-ETI group, mean FEV1pp increased from 57.9% at baseline to 65.0% at 12 months, yielding an absolute mean gain of +7.1% (p < 0.001), surpassing the predefined target of ≥ 5%, while mean BMI-for-age Z-scores improved from -0.88 to -0.13 (+0.75; p < 0.001). ETI-treated patients demonstrated greater improvements during phase 2, with mean FEV1pp rising from 66.4% to 78.6% (+12.2%; p < 0.001) and mean BMI Z-score improving from -0.64 to -0.05 (+0.59; p < 0.001). Antibiotic use declined significantly over the project period.

CONCLUSIONS

A tailored multidisciplinary QI protocol significantly improved lung function and nutrition in PwCF in a resource-limited setting, with CFTR modulators providing additional clinically meaningful benefits.