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Original Research |

Sleep Phase Delay in Cystic Fibrosis: A Potential New Manifestation of Cystic Fibrosis Transmembrane Regulator Dysfunction

Judy L. Jensen, MS; Christopher R. Jones, MD, PhD; Christiana Kartsonaki, DPhil; Kristyn A. Packer, MA; Frederick R. Adler, PhD; Theodore G. Liou, MD
Author and Funding Information

Conflict of Interest: Drs Jones and Kartsonaki have no conflicts of interest. Ms Jensen, Ms Packer, Prof Adler and Dr Liou receive research funding from the National Heart, Lung and Blood Institute of the National Institutes of Health (NHLBI/NIH R01 HL12552), Bethesda, MD and the Cystic Fibrosis Foundation, Bethesda, MD. Prof Adler receives additional research funding from the US Army Research Office and the National Science Foundation (NSF-DMS 1148230, NSF-DEB 1051491). Ms Jensen, Ms Packer and Dr Liou participate in clinical trials with clinical support through the CF Foundations Therapeutic Development Network (LIOU14Y0) and the Foundation for the National Institutes of Health. The Adult CF Center at the University of Utah receives research support through the NHLBI/NIH, the Ben B and Iris M Margolis Family Foundation of Utah and the Claudia Ruth Goodrich Stevens Endowment Fund. The University of Utah has received specific funding for clinical trials sponsored by Geno, LLC, Gilead Sciences, Inc, Nivalis Therapeutics, Inc, Novartis, Savara Pharmaceuticals, Spiration, Inc and Vertex Pharmaceuticals, Inc within the last three years.

Funding/Support: This work was funded by the NHLBI/NIH, Bethesda, MD (R01 HL 125520), the Cystic Fibrosis Foundation, Bethesda, MD (CC132-16AD, LIOU13A0, LIOU14P0), the Ben B and Iris M Margolis Family Foundation of Utah and the Claudia Ruth Goodrich Stevens Endowment Fund.

aUniversity of Utah Adult Cystic Fibrosis Center, Division of Respiratory, Critical Care and Occupational Pulmonary Medicine, Department of Internal Medicine

bCenter for Quantitative Biology

cDepartment of Neurology, University of Utah, Salt Lake City, UT, United States

dDepartments of Mathematics and Biology, University of Utah, Salt Lake City, UT, United States

eNuffield Department of Population Health, University of Oxford, Oxford, United Kingdom

Correspondence To: Theodore G Liou, MD, 26 North Mario Capecchi Drive, Salt Lake City, UT 84132, USA.


Copyright 2017, . All Rights Reserved.


Chest. 2017. doi:10.1016/j.chest.2017.03.057
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Abstract

Background  Cystic fibrosis (CF) transmembrane regulator (CFTR) protein dysfunction causes CF. Improving survival allows detection of increasingly subtle disease manifestations. CFTR dysfunction in the central nervous system (CNS) may disturb circadian rhythm and thus sleep phase. We studied sleep in adults to better understand potential CNS CFTR dysfunction.

Methods  We recruited participants from April 2012 through April 2015 and administered the Munich Chronotype Questionnaire (MCTQ). We compared free-day sleep measurements between CF and non-CF participants and investigated associations with CF survival predictors.

Results  We recruited 23 female and 22 male adults with CF aged 18-46 years and 26 female and 22 male volunteers aged 18-45 years. Compared to non-CF volunteers, patients with CF had delayed sleep-onset (0.612 hrs, P = 0.015), mid-sleep (1.11 hrs, P < 0.001) and wake-up (1.15 hrs, P < 0.001) times and prolonged sleep latency (7.21 min, P = 0.05) and duration (0.489 hr, P = 0.05). Every hour delay in sleep-onset time was associated with shorter sleep duration by 0.29 hours in CF and 0.75 hours in non-CF (P = 0.007) and longer sleep latency by 0.15 hours in CF and 0.05 in non-CF (P = 0.035). Among patients with CF, FEV1%, prior acute pulmonary exacerbations and weight were independent of all free-day sleep measurements.

Conclusion  CF in adults is associated with marked delays in sleep phase consistent with circadian rhythm phase delays. Independence from disease characteristics predictive of survival suggests that sleep phase delay is a primary manifestation of CFTR dysfunction in the CNS.


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