Clinical Outcomes Following SARS-CoV-2 Infection in Pediatric Cystic Fibrosis Patients.

Abstract

Background Cystic fibrosis (CF) is a genetic disorder of the cystic fibrosis transmembrane conductance regulator chloride channel that leads to impaired mucus clearance in the airways, which leads to deteriorations in lung function and chronic respiratory infection. These effects of CF contribute to the hypothesis that patients with CF may be at increased risk of complications when they catch coronavirus disease 2019 (COVID-19), which swept the world in a global pandemic starting in 2019. Overall, however, the role of CF in COVID-19 has not been well studied, particularly in pediatric patients. Methods In this retrospective review, pediatric patients with CF who contracted COVID-19 (3/1/2020-3/1/2023) (N=69) were compared to two equally sized control cohorts of patients with only CF or COVID-19 matched based on demographics and clinical baselines. Occurrences of adverse outcomes (emergency room visits, hospitalizations, CF pulmonary exacerbations, etc.) were assessed for each subject. The mean percentage of predicted forced expiratory volume in 1 second (FEV1%pred) was also assessed for CF patients. Fisher's exact test assesseddifferences between the proportions of subjects who experienced each outcome. Independent two-variable t-testing assessed mean FEV1%pred differences. Analysis was conducted using IBMSPSSStatistics for Windows, Version 29 (Released 2023; IBM Corp., Armonk, New York, United States)with a significance α=0.05. Ad hoc power analysis was conducted using G*Powerv3.1. Results Overall, CF/COVID subjects fared similarly to control groups without either CF or COVID-19 history, including among subgroups stratified based on baseline respiratory function,P. aeruginosacolonization status, and COVID-19 vaccination status. One notable finding was that CF/COVID subjects experienced significantly fewer pulmonary exacerbations compared to CF-only subjects (p=0.004). Conclusion In conclusion, pediatric CF patients performed similarly to their peers without CF with regardto COVID-19and generally did not demonstrate significant deteriorations in pulmonary function following infection. Lower incidence of pulmonary exacerbations in CF/COVID subjects could be explained by stringent monitoring by parents, quarantine, or close pulmonology follow-up. These findings will provide guidance on management and care for pediatric CF patients with COVID-19.