TYPE: Late Breaking Abstract
TOPIC: Genetic and Developmental Disorders
PURPOSE: To determine whether cystic fibrosis (CF) patients on elexacaftor/ivacaftor/tezacaftor have superior outcomes compared to patients not on that therapy upon development of COVID-19 infection.
METHODS: We used the TriNetX database to construct 2 cohorts of patients with cystic fibrosis and COVID-19 infection: One cohort was on active treatment with elexacaftor/ivacaftor/tezacaftor while the other included all CF patients not on that therapy. Primary outcomes included acute respiratory failure, pulmonary collapse, and 6-month mortality.
RESULTS: Patients treated with elexacaftor/ivacaftor/tezacaftor were one-third as likely to suffer pulmonary collapse (RR 0.323, CI 0.159,0.653), and less than 29% as likely to suffer acute respiratory failure (RR 0.289, CI 0.149,0.561) than patients not on that treatment. 6-month mortality was also lower at 0.98% vs 5.417%, P value <0.0001.
CONCLUSIONS: Cystic fibrosis patients who are on treatment with Trikafta (elexacaftor/ivacaftor/tezacaftor) at the time of developing COVID-19 infection have superior outcomes with regards to acute respiratory failure, pulmonary collapse, and 6-month mortality.
CLINICAL IMPLICATIONS: Elexacaftor/ivacaftor/tezacaftor therapy should be considered for any cystic fibrosis patient who is a candidate. Our data indicates that it produces superior outcomes for CF patients who acquire COVID-19 infection.
DISCLOSURE: Nothing to declare.
KEYWORD: Cystic Fibrosis