Annals of the American Thoracic Society · 2026
Health inequity in people with cystic fibrosis: can we close the gap?
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Sermet-Gaudelus Isabelle, Orenti Annalisa, Hatziagorou Elpis, Bakkeheim Egil, Naehrlich Lutz, Kerem Eitan, ECFSPR Steering Group
Аннотация
RATIONALE: Although advances in care have improved cystic fibrosis (CF) outcomes in high-income countries (HICs), the situation remains alarming in low-income countries (LICs).
OBJECTIVES: To assess differences in key disease indicators among people with CF (pwCF) based on the socioeconomic status of their country between 2012 and 2022 to suggest practical interventions and evaluate effect of introduction of CFTR modulators on survival.
METHODS: People with CF (pwCF) enrolled in the European Cystic Fibrosis Society Patient Registry (ECFSPR) and carrying at least one F508del variant allele were evaluated in 2017 and in 2022 for percent predicted forced expiratory volume in 1 second (ppFEV1), underweight status, and chronic Pseudomonas aeruginosa (PsA) infection, according to the gross national income per capita divided into 3 terciles (LICs, middle-income countries [MICs], and HICs). Survival was evaluated in the periods 2013-2017 and 2018-2022. Generalized linear models and Cox regression models were fitted.
RESULTS: From the 31 723 pwCF reported in ECFSPR in 2022, 13.5% lived in LICs, 19.9% in MICs, and 66.6% in HICs. PwCF living in LICs had a significantly lower median survival age, reduced ppFEV1, and higher prevalence of PsA infection and underweight status compared with pwCF from MICs and HICs. Data modeling indicated that avoiding underweight status and PsA infection would increase survival by 42 years for pwCF living in LICs. Access to cystic fibrosis transmembrane conductance regulator (CFTR) modulators would further increase their survival by 15 to 29 years depending on their nutrition and infection status, resulting in a survival up to 82 years in the best-case scenario.
CONCLUSIONS: Access to CFTR modulators equalizes survival between LICs and HICs within Europe. Optimizing care practices and social determinants of health remains crucial in LICs.
OBJECTIVES: To assess differences in key disease indicators among people with CF (pwCF) based on the socioeconomic status of their country between 2012 and 2022 to suggest practical interventions and evaluate effect of introduction of CFTR modulators on survival.
METHODS: People with CF (pwCF) enrolled in the European Cystic Fibrosis Society Patient Registry (ECFSPR) and carrying at least one F508del variant allele were evaluated in 2017 and in 2022 for percent predicted forced expiratory volume in 1 second (ppFEV1), underweight status, and chronic Pseudomonas aeruginosa (PsA) infection, according to the gross national income per capita divided into 3 terciles (LICs, middle-income countries [MICs], and HICs). Survival was evaluated in the periods 2013-2017 and 2018-2022. Generalized linear models and Cox regression models were fitted.
RESULTS: From the 31 723 pwCF reported in ECFSPR in 2022, 13.5% lived in LICs, 19.9% in MICs, and 66.6% in HICs. PwCF living in LICs had a significantly lower median survival age, reduced ppFEV1, and higher prevalence of PsA infection and underweight status compared with pwCF from MICs and HICs. Data modeling indicated that avoiding underweight status and PsA infection would increase survival by 42 years for pwCF living in LICs. Access to cystic fibrosis transmembrane conductance regulator (CFTR) modulators would further increase their survival by 15 to 29 years depending on their nutrition and infection status, resulting in a survival up to 82 years in the best-case scenario.
CONCLUSIONS: Access to CFTR modulators equalizes survival between LICs and HICs within Europe. Optimizing care practices and social determinants of health remains crucial in LICs.