Effect of Triple Combination CFTR Modulator Therapy on Sleep in Adult Patients with Cystic Fibrosis

Welsner M.a· Schulte T.b· Dietz-Terjung S.b· Weinreich G.b· Stehling F.c· Taube C.a,b· Strassburg S.a· Schoebel C.b· Sutharsan S.a

Author affiliations

aDepartment of Pulmonary Medicine, University Hospital Essen – Ruhrlandklinik, Adult Cystic Fibrosis Center, University of Duisburg-Essen, Essen, Germany
bDepartment of Sleep and Telemedicine, University Hospital Essen – Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany
cPediatric Pulmonology and Sleep Medicine, Cystic Fibrosis Center, Children´s Hospital, University of Duisburg-Essen, Essen, Germany

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Article / Publication Details

First-Page Preview

Abstract of Clinical Investigations

Received: January 02, 2022
Accepted: April 27, 2022
Published online: May 20, 2022

Number of Print Pages: 9
Number of Figures: 2
Number of Tables: 2

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

For additional information: https://www.karger.com/RES

Abstract

Background: Sleep-disordered breathing (SDB) and disturbed sleep are common, often underrecognized, comorbidities in people with cystic fibrosis (pwCF). Objectives: We studied the effect of CFTR triple combination therapy elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) on sleep in pwCF. Method: This was a prospective, observational sleep study in clinically stable adult pwCF. All participants underwent overnight polysomnography (PSG), before (T0) and after (T1) initiation of CFTR modulator therapy with ELX/TEZ/IVA. In addition, pulmonary function tests, calculation of BMI, and sweat chloride testing were performed. Results: Twenty-nine pwCF (mean age 32 ± 8 years; 15 female) participated in the study. Mean time between T0 and T1 was 194 ± 21 days. Total sleep time (TST) was 298 ± 40 min, with decreased sleep efficiency (SE) (76 ± 109) and increased sleep latency (SL) (73 ± 38 min). Sleep stages for NREM (N1–3) and REM sleep were within the normal range. Nocturnal respiratory events mainly occur during REM sleep (T0: AHI REM 8.3 ± 9.0/h; ODI REM 9.4 ± 10.6/h), whereas the overall AHI was normal (3.6 ± 3.7/h). After initiation of ELX/TEZ/IVA, we saw significant improvements in ppFEV1 (p < 0.001) and BMI (p < 0.001) and a reduction in sweat chloride levels (p < 0.001). In parallel, there was a reduction in AHI (p = 0.003), ODI (p = 0.001), and nocturnal respiratory rate (p < 0.001), both in total, REM and NREM sleep. Neither TST, SL, SE, nor sleep architecture was influenced (all p > 0.05). Conclusions: Initiation of ELX/TEZ/IVA resulted in significant improvements in SDB in adult pwCF.

© 2022 S. Karger AG, Basel

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First-Page Preview

Abstract of Clinical Investigations

Received: January 02, 2022
Accepted: April 27, 2022
Published online: May 20, 2022

Number of Print Pages: 9
Number of Figures: 2
Number of Tables: 2

ISSN: 0025-7931 (Print)
eISSN: 1423-0356 (Online)

For additional information: https://www.karger.com/RES

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