Risk Factors and Outcomes of Pulmonary Exacerbations in Infants and Young Children With Cystic Fibrosis

Rationale There are limited data on the risk factors associated with pulmonary exacerbation (PEx) in very young people with CF (VY‐PwCF). The goal of our study was to examine the risk factors associated with PEx in the first 3 years of life and the relationship between PEx in early life and subsequent nutritional and pulmonary outcomes. Methods Using the CF Foundation Patient Registry (CFFPR), we analyzed demographic, clinical and PEx data on PwCF born between 2003 and 2017. Multivariable logistic regression analyzed the probability of a PEx between ages 1 and 3, and later‐life forced expiratory volume in 1 s (FEV1) and body mass index (BMI) z‐scores were modelled using negative binomial and linear regressions, respectively. Results A total of 7,342 patients were included in our analysis. Medicaid insurance status, pancreatic insufficiency, asthma diagnosis before age 3, hypertonic saline use before age 1 and a positive respiratory culture for methicillin‐resistant Staphylococcus Aureus and Stenotrophomonas maltophilia respectively before age 1 were associated with an increased odds of PEx diagnosis. FEV1 at age 6 and BMI z‐scores at age 3 were lower in those who experienced early PEx. Conclusions We identified several risk factors that are associated with PEx diagnosis in the first 3 years of life and found that PEx in the first 3 years negatively impacted ppFEV1 at age 6 and BMI z‐score at age 3. These data will be helpful for clinicians in counselling caregivers of VY‐PwCF on PEx risk factors and identifying patients at higher risk of PEx in the first 3 years of life.