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Rationale: Clinical variables associated with shortened survival in patients with advanced-stage cystic fibrosis (CF) are not included in the lung allocation score (LAS).Objectives: To identify variables associated with wait-list and post-transplant mortality for CF lung transplant candidates using a novel database and to analyze the impact of including new CF-specific variables in the LAS system.Methods: A deterministic matching algorithm identified patients from the Scientific Registry of Transplant Recipients and the Cystic Fibrosis Foundation Patient Registry. LAS wait-list and post-transplant survival models were recalculated using CF-specific variables. This multicenter, retrospective, population-based study of all lung transplant wait-list candidates aged 12 years or older from January 1, 2011, to December 31, 2014, included 9,043 patients on the lung transplant waiting list and 6,110 lung transplant recipients between 2011 and 2014, comprising 1,020 and 677 with CF, respectively.Measurements and Main Results: Measured outcomes were changes in LAS and lung allocation rank. For CF candidates, any Burkholderia sp. (hazard ratio [HR], 2.8; 95% confidence interval [CI], 1.2-6.6), 29-42 days hospitalized (HR 2.8; CI 1.3-5.9), massive hemoptysis (HR 2.1; CI 1.1-3.9), and relative drop in FEV1 ≥30% over 12 months (HR 1.7; CI 1.0-2.8) increased wait-list mortality risk; pulmonary exacerbation time 15-28 days (1.8; 1.1-2.9) increased post-transplant mortality risk. A relative drop in FEV1 ≥10% in chronic obstructive pulmonary disease (COPD) candidates was associated with increased wait-list mortality risk (HR 2.6; CI 1.2-5.4). Variability in LAS score and rank increased in patients with CF. Priority for transplant increased for COPD candidates. Access did not change for other diagnosis groups.Conclusions: Adding CF-specific variables improved discrimination among wait-listed CF candidates and benefited COPD candidates.

Rationale: Referrals for lung transplant and transplant rates in the United States are lower than in Canada for patients with advanced cystic fibrosis (CF) lung disease. Further study of factors limiting access are needed to optimize referral and transplant for this population. Objectives: To determine the effect of socioeconomic position, while accounting for disease severity, on the likelihood of wait-listing for lung transplant in the United States. Methods: A case–control study of 3,110 patients (1,555 wait-listed, 1,555 never wait-listed) in the linked CF Foundation Patient Registry/Scientific Registry of Transplant Recipients was performed with 1:1 matching for age, forced expiratory volume in 1 second, and year. Logistic regression was performed with univariate and multivariate analyses accounting for eight clinical factors (sex, oxygen use, body mass index, hemoptysis, forced vital capacity, methicillin-resistant Staphylococcus aureus, multidrug-resistant Pseudomonas aeruginosa, and i.v. antibiotic days) and six socioeconomic factors (race, marital status, education, health insurance, median zip code income, and distance to transplant program). The CF Health Score and Socioeconomic Barrier Score were created based on summation of variables. Interactions between scores were calculated. Results: We found an inverse relationship between the probability of wait-listing and CF Health Score and Socioeconomic Barrier Score. As the CF Health Score decreased (less healthy), the probability of wait-listing increased by 69.3% from a score of 7 to 2. As the Socioeconomic Barrier Score decreased (fewer barriers), the probability of wait-listing increased by 31.7% from a score of ≥5 to 1). Regardless of illness severity, socioeconomic barriers presented an impediment to wait-listing. Individuals with higher Socioeconomic Barrier Scores accessed transplant about half as often as those with lower scores at the same level of medical severity. Analysis of interactions demonstrated a higher probability of wait-listing for individuals with moderate health severity and fewer social barriers compared with sicker individuals with more socioeconomic barriers. Conclusions: Accrual of socioeconomic barriers limits access to lung transplant irrespective of disease severity, a finding of substantial concern for patients with CF and for transplant providers. Future interventions can focus on this at-risk population early in the disease course.