Background: New 2023 CF liver disease (CFLD) guidelines advocate for additional screening in people with cystic fibrosis PwCF, including biennial abdominal ultrasound. As a first step towards effective and equitable guidelines implementation, we examined our current practice of CFLD screening and hepatobiliary involvement (HBI) evaluation. We identified characteristics of PwCF at-risk for incomplete screening and factors affecting evaluation. Methods We retrospectively reviewed medical records of PwCF aged 0-21yrs, with native liver and ≥2 outpatient CF clinic visits 2017-23. Logistic regression was used to identify characteristics associated with incomplete screening and with HBI. Results Amongst 112 PwCF at our center: 37% self-reported as mixed race, 27% as Hispanic; 53% had public insurance. Incomplete lab screening was identified in 19% of our cohort. GGT was the most frequently missed component (17%). Hispanics and publicly insured people were more likely to have incomplete screening. Of the 112, 45 met criteria for HBI. Demographics did not predict HBI. Five with CF and HBI had the full hepatitis workup recommended by the new guidelines. Those with HBI documented (42%) were more likely to receive additional workup. PwCF who were seen by a gastroenterologist were more likely to have additional diagnostic work-up for HBI. Conclusions: One in five PwCF at our center were incompletely screened for CFLD, with Hispanics and publicly insured at higher risk. Accurate diagnosis and adequate documentation are the first steps to identifying HBI in PwCF. A dedicated CF gastroenterologist is key to completing CFLD screening and liver diagnosis.

Background: As the population of people with CF (pwCF) continues to age, attention is shifting towards addressing the unique challenges teenagers and adults face, including substance use. Changing attitudes and legality regarding marijuana and CBD may influence their use among pwCF, but data on their prevalence, reasons for use, and administration methods are lacking. Objective: Investigate marijuana, cannabidiol (CBD), e-cigarette, and cigarette usage among pwCF and explore differences in demographics, disease severity, and CFTR modulator use between current and non-users. Methods: This cross-sectional study used a one-time electronic survey to assess marijuana, CBD, e-cigarette, and cigarette use in pwCF aged >13 years. Demographic and clinical characteristics were compared between current users and non-users. The association between current substance use and CFTR modulator use was analyzed using logistic regressions. Results: Among 226 participants, 29% used marijuana, 22% used CBD, 27% used e-cigarettes, and 22% used cigarettes. Current users of all substances were more likely to be college-educated, Black, or aged 29-39 years than non-users. Current e-cigarette users were 2.9 times more likely to use CFTR modulators (95% CI 0.98-11.00, p=0.08) and current marijuana users were 2.5 times more likely to use CFTR modulators compared to non-users, adjusted for confounders. Current users of CBD, e-cigarettes, and cigarettes were more likely to have an abnormal mental health screen compared to non-users. A high proportion of never-users of marijuana and CBD expressed interest in using. Conclusion: Substance use is more prevalent among pwCF than previously reported and needs to be addressed by healthcare providers.