(319) Fatigue and Poor Sleep Quality Still Prevalent Even in Setting Of Elexacaftor/Tezacaftor/Ivacaftor

Background: There is growing acknowledgement that patient reported outcomes (PROs), including non-respiratory symptoms, are important to address in clinical care. Small, single site studies have shown fatigue, poor sleep quality, pain, and emotional distress (i.e., depression and anxiety) are prevalent among adults with cystic fibrosis (AwCF). In other chronic illnesses these symptoms co-occur and are associated with worse health and poorer quality of life. The goal of this study was to report baseline findings of a multisite longitudinal study investigating at the prevalence of these symptoms and their association with CF outcomes including respiratory symptoms and lung function.

Methods: Participants were recruited from two large adult CF centers. Eligibility criteria included CF diagnosis, ≥18 years old, on modulator therapy 4+ weeks (if prescribed) and psychiatric medications 8+ weeks (if prescribed), not pregnant or < 6mo postpartum, no antibiotic treatment ±14 days of screening, English speaking, and having technology to complete online surveys. After consenting, participants were sent a survey link that included the following measures: Fatigue Severity Scale (FSS), Pittsburg Sleep Quality Index (PSQI), Patient Health Questionnaire (PHQ8), Generalized Anxiety Disorder (GAD7), PROMIS Pain Intensity, Cystic Fibrosis Respiratory Symptom Diary 7 –Day Recall (CFRSD-7). The study is ongoing with recruitment and baseline surveys are expected to be completed by August 2022.

Results: To date, 164 AwCF, out of a planned 255, have completed the baseline survey (mean age=37 (SD=12, range 20-79) years old, 54% Female, 90% Non-Hispanic White, 87% prescribed CF modulator therapy). Clinically elevated scores ranged from 15% (PROMIS Pain Intensity) to 80% (PSQI; TABLE 1). As expected fatigue, pain, sleep quality, depressive symptoms, anxiety, pain and respiratory symptoms were all statistically significantly correlated with each other (ranging from r=0.20 (GAD7 & PROMIS Pain Intensity) to r=0.71 (PHQ8 & GAD7)), including all being correlated with respiratory symptoms.

Conclusions: While increased prevalence of depression and anxiety are well established among AwCF, this study found that 4 of 5 AwCF reported poor sleep quality while almost half experienced clinical levels of fatigue despite high penetration of modulator therapy (predominantly elexacaftor/tezacaftor/ivacaftor). These non-respiratory symptoms can be treated with pharmacologic and non-pharmacologic approaches such as Cognitive Behavioral Therapy. Our findings suggest it may be beneficial for CF clinics to incorporate routine assessments for non-pulmonary symptoms using PROs into clinical care and to ensure there are team members, including mental health professionals, available to provide services.

Acknowledgements: This work was supported by the Cystic Fibrosis Foundation (RIEKER19AO)

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