CF-AIR and CF@LANTA primary investigator Rachel Linnemann, MD recently published an impactful journal article which outlines the application of quality improvement methods to improve modulator management and side effect screening at multiple cystic fibrosis (CF) centers across the country. 

Senior author Dr. Linnemann published study results on July 22, 2025, in Pediatric Pulmonology. The article is titled “Improving Modulator Management and Side Effect Screening: A CF Learning Network Innovation Lab.”

The study, funded by the Cystic Fibrosis Foundation and co-led by Dr. Linnemann, collected data from nearly 8000 patient visits occurring at 31 CF centers that participate in the CF Learning Network – a national quality improvement network. Participating sites included the Children’s Healthcare of Atlanta Pediatric CF Program and the Emory Adult CF Program. 

Despite the eligibility of over 90% of people with cystic fibrosis (PwCF) for CFTR modulators, 12% of eligible PwCF are not prescribed these therapies. Through a quality improvement (QI) innovation lab (iLab), the authors investigated gaps in modulator use and identified best practices for modulator initiation, management, and side effect screening. Across 4649 visits evaluated in Phase 1 of the study, the majority of eligible PwCF not taking modulators had a co‐produced reason for deferral, most commonly previous side effects. Across 3236 visits examined in Phase 2 of the study, side effect screening rates improved, with the centerline shifting from 67.1% to 80.8%. Potential side effects were identified in 24.5% of screenings. 

“Side effects have emerged as a topic of critical importance to people with CF and their caregivers. Our results highlight the importance of side effect screening so that these can be identified and addressed, in some cases preventing discontinuation of effective therapies,” said Dr. Linnemann. “The best practices we identified can also be applied as Alyftrek (vanzacaftor-tezacaftor-deutivacafor) and other novel CF therapies are introduced in the clinics.”

Pediatric Pulmonology is the leading global journal for respiratory system health and disease from fetal life through adolescence and adulthood, as well as a principle source for pediatricians in practice and in training, and for pediatric pulmonologists.

Dr. Linnemann serves as the Director of the Cystic Fibrosis Care Center and Co-Director of the Pediatric Cystic Fibrosis Program at Children’s and Emory, and she is also Associate Professor, Department of Pediatrics at the Emory University School of Medicine.

The abstract of the article can be found online here.