What Drives ETI Use After CF Lung Transplant in the US?

TOPLINE:

In the US, nearly one third of eligible lung transplant recipients with cystic fibrosis (CF) received new elexacaftor-tezacaftor-ivacaftor (ETI) prescriptions post-transplant. The presence of sinus disease and low BMI were positively associated with ETI prescription; however, these prescribing patterns varied significantly among center types.

METHODOLOGY:

• Researchers conducted a retrospective study to examine the prescription patterns and factors associated with triple-modulator ETI prescription after lung transplantation in patients with CF in the US.
• Data were gathered through December 2022 from a registry of patients at 157 centers who had an ETI-eligible genotype and were not on ETI before lung transplant. The study population included 1666 lung transplant recipients (mean age, 39 years; 50% women).
• Based upon the proportion of lung transplant recipients with CF initiating ETI, centers were classified as — low-prescribing (0-1 patient), middle-prescribing (> 1 but < 50% of patients), and high-prescribing centers (≥ 50% of patients). Centers with less than 10 transplant recipients were labeled “small center.”

TAKEAWAY:

• Overall, 29.3% of recipients received new ETI prescriptions after transplant.
• The presence of sinus disease (odds ratio [OR], 2.12; 95% CI, 1.51-2.99) and a BMI < 18.5 (OR, 1.52; 95% CI, 1.13-2.04) were positively associated with ETI prescription after transplant.
• Receiving care at middle-prescribing (OR, 0.19; 95% CI, 0.14-0.26) or low-prescribing centers (OR, 0.02; 95% CI, 0.01-0.04) significantly reduced the likelihood of ETI prescription compared with that of high-prescribing centers.
• When stratified by center, low BMI was a strong predictor of post-transplant ETI use only at small and low-prescribing centers, whereas sinus disease predicted ETI use only at middle- and high-prescribing centers.

IN PRACTICE:

“We hypothesize low BMI was more important for low-prescribing and small centers because low BMI is probably the most concerning extrapulmonary manifestation of CF to providers and may tip the scale to prescribe if there is reluctance,” the authors of the study wrote.

SOURCE:

This study was led by Nora C. Burdis, Department of Medicine, University of Washington, Seattle. It was published online on July 1, 2025, in the Journal of Cystic Fibrosis.

LIMITATIONS:

This study was limited by difficulty in tracking long-term medication adherence. Variable follow-up practices at CF centers affected data completeness. Additionally, the registry lacked information on symptom severity, limiting the assessment of the relationship between ETI prescription and symptom severity.

DISCLOSURES:

No specific financial support was mentioned in the study; however, the authors reported receiving support from multiple organizations, including the National Institutes of Health, the Cystic Fibrosis Foundation, and the University of Washington. Some authors also declared having financial relationships with multiple pharmaceutical companies.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.