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Background Due to the COVID-19 pandemic, there was an uptake of telehealth in cystic fibrosis care. Previous studies show disparities in telehealth use based on socioeconomic status (SES). We aimed to: (1) understand telehealth use and perceptions and (2) identify the facilitators and barriers to telehealth use among people with CF and their families (PwCF) from diverse racial/ethnic and socioeconomic backgrounds. Methods We conducted an analysis of the 2020 Cystic Fibrosis State of Care surveys completed by PwCF (PFSoC), CF Care Programs (SoC1) and the CF Foundation Patient Registry (CFFPR). Results A total of 424 PwCF and 286 programs responded to the PFSoC and SoC1. Among PwCF, 90% self-identified as White, 6% as Hispanic/Latino, and 2% as Black. Racial/ethnic minorities were less likely to have had a telehealth visit (p=.015). This difference was pronounced among the Hispanic/Latino population (p

OBJECTIVES: Tobacco smoke exposure reduces CFTR functional expression in vitro and contributes to acquired CFTR dysfunction. We investigated whether it also inhibits the clinical benefit of CFTR modulators, focusing on tezacaftor/ivacaftor, approved in February 2018 for individuals with CF age ≥12 years. METHODS: A retrospective longitudinal analysis of encounter-based data from the CF Foundation Patient Registry (2016-2018) compared the slope of change in lung function (GLI FEV(1)% predicted) before and after tezacaftor/ivacaftor initiation in smoke-exposed vs unexposed age-eligible pediatric patients. Tobacco smoke exposure (Ever/Never) was determined from caregiver self-report. Statistical analyses used hierarchical linear mixed modeling and fixed effects regression modeling. RESULTS: The sample included 6,653 individuals with a total of 105,539 person-period observations. Tezacaftor/ivacaftor was prescribed to 19% (1,251) of individuals, mean age 17 years, mean baseline ppFEV(1) 83%, 28% smoke-exposed. Tezacaftor/ivacaftor users who were smoke-exposed had a lower baseline ppFEV(1) and experienced a greater lung function decline. Over two years, the difference in ppFEV(1) by smoke exposure among tezacaftor/ivacaftor users increased by 1.2% (7.6% to 8.8%, p

Background: Few studies have attempted to disentangle the effects of tobacco smoke exposure and socioeconomic factors on lung function decline in pediatric CF. The current study evaluates their contributions longitudinally across the entire U.S. CF care network population. Methods: Data from the CF Foundation Patient Registry were obtained on all individuals who at the end of 2016 were 6-18 years old. Lung function measures (median, highest, and lowest ppFEV1) for each person were calculated at each attained age. Multivariable analyses used mixed modeling to assess the impact of smoke exposure and socioeconomic factors on both initial lung function and change over time. Findings: The sample included 10,692 individuals contributing 57,888 person years. At age 6, ppFEV1 of smoke-exposed children was 4% lower than among unexposed. The deficit persisted through age 18. In fully adjusted mixed models, smoke exposure and socioeconomic factors had independent, additive associations with lung function. Median ppFEV1 declined 2% with smoke exposure, 3% with lower paternal education, 1% with public insurance, and increased 0.2% with each $10,000 annual household income. The effect of smoke exposure on ppFEV1 was larger in disadvantaged children compared to privileged counterparts (2.5% vs 1.5%). Interpretation: Smoke exposure and socioeconomic factors are independent risk factors for decreased ppFEV1 in pediatric CF. Strategies to reduce smoke exposure should be introduced early. Interventions may be prioritized in disadvantaged children, among whom the exposure has a disproportionately large effect. Funding Statement: This study was supported by grants from NIH (P30DK72482, K08HL140190), AHRQ (K12HS023009), and CFF (CC032-14). Declaration of Interests: The authors have no conflicts of interest. Ethics Approval Statement: Written informed consent was obtained from all participants. The study was approved by the Institutional Review Board of the University of Alabama at Birmingham (protocol number 300002076).