Abstract

Abstract Background CFTR modulator Elexacaftor/tezacaftor/ivacaftor (ETI) significantly improves lung function and but its effect on mental health and sleep remains poorly understood. We report sleep, mood and respiratory health outcomes of adolescents with CF with ETI therapy. Methods We conducted a prospective longitudinal study of 31 adolescents (aged 10 to 18 years) from July 2021 to October 2022. Data collected include demographics, pediatric daytime sleepiness scale (PDSS), sleep disturbance scale for children (SDSC), patient health questionnaire-9 (PHQ-9), generalised anxiety disorder-7 (GAD-7) scores and FEV1 percent predicted. Results Paired-T testing demonstrated a significant improvement in FEV1, whereas PDSS, PHQ-9, and GAD-7 scores worsened following ETI therapy, but did not reach statistical significance. SDSC scores marginally reduced. Ten percent of participants developed new onset sleep concerns and new mood concerns developed in 10% following ETI initiation. Conclusion/Outcome This is the first prospective longitudinal study of sleep and mood changes with ETI in adolescents with CF. ETI does not improve sleep and mood in the same way as respiratory health outcomes.

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