Abstract

RationaleLow body mass index (BMI) may influence lung transplant decisions for patients with advanced cystic fibrosis (CF) lung disease. ObjectiveDetermine whether patients with advanced CF lung disease and BMI ≤17 kg/m2 are less likely to be listed for lung transplant or have a higher risk of death without listing compared to those with higher BMI. MethodsUsing merged United Network for Organ Sharing and CF Foundation Patient Registries, we identified adults with onset of advanced lung disease (FEV1 ≤ 40% predicted) between May-2005 and December-2016. We analyzed survival using competing risks regression with cause-specific risks of listing for lung transplant and death without listing. BMI ≤ 17 kg/m2 was our predictor. Measurements and main resultsAmong 5,121 CF patients with advanced lung disease, 23% were listed for lung transplant (n = 1,201), 23% died without listing (n = 1,190), and 44% were alive without listing (n = 2,730) as of December-2016. Patients with BMI ≤ 17 kg/m2 were less likely to be listed for transplant (HR 0.69; 95% CI 0.57, 0.83) and more likely to die without listing (HR 1.63; 95% CI 1.41, 1.88). We identified important regional variations in the likelihood of referral and listing, based on BMI. ConclusionsPatients with advanced CF lung disease and BMI ≤ 17 kg/m2 are less likely to be listed for lung transplant and have a higher risk of dying without listing, compared to those with higher BMI. Regional differences suggest access to transplant for malnourished CF patients may be limited by location.

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