Sleep Quality in Adolescents and Adults With Cystic Fibrosis Taking Elexacaftor/Tezacaftor/Ivacaftor

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Sleep Quality in Adolescents and Adults With Cystic Fibrosis Taking Elexacaftor/ Tezacaftor/IvacaftorTo the Editor:Highly effective Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies (HEMTs), including elexacaftor/tezacaftor/ivacaftor (ETI), have dramatically increased the predicted life expectancy of someone born with cystic fibrosis (CF) to 65 years of age. 1 With most people with CF (PwCF) taking HEMT living well into adulthood, there is an urgent need to learn more about the impacts of CF and HEMT on various domains of aging and health-related quality of life, including the impact on sleep.PwCF not taking HEMT experience poor sleep, with likely underlying etiologies including cough, frequent stooling, reflux, chronic pain, and hypoxia. 2 PwCF may also have circadian rhythm disturbances because of defective hypothalamic CFTR function.3 Severity of lung disease is correlated with severity of sleep impairments, with the worst sleep reported by those awaiting lung transplant.4,5 Researchers expected improvements in sleep to follow improvements in pulmonary symptoms for PwCF taking ETI.However, this has not been consistently observed.A few small studies have examined sleep when considering mental health side effects of ETI, finding mixed results.Although some studies show stability or improvement in subjective sleep quality, many show worsening of self-reported sleep quality after ETI initiation.[6][7][8][9][10] Given the far-reaching health consequences of poor sleep, including mental health disorders, obesity, insulin resistance, and increased susceptibility to infection, it is vital to learn more about sleep in PwCF taking ETI.

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