Quality of life in clinically stable adult cystic fibrosis out-patients: associations with daytime sleepiness and sleep quality

Bouka, A; Tiede, Henning; Liebich, L; Dumitrascu, Rio; Hecker, C; Reichenberger, Frank; Mayer, Kelly; Seeger, W.; Schulz, Riéli Elis

doi:10.1055/s-0032-1306402

Cited by 6 publications

(9 citation statements)

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“…In general, sleep disturbances among CF patients is multifactorial. It has been suggested that increased mucus production and reflux may cause nocturnal cough, anxiety and depression may lead to difficulty falling asleep, and chronic pain may interfere with sleep quality …”

Section: Discussionmentioning

confidence: 99%

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

McCormick

Cho

Lampkin

et al. 2018

Int Forum Allergy Rhinol

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Background Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that improves pulmonary function in cystic fibrosis (CF) patients with at least 1 copy of the G551D CFTR mutation. The purpose of this study is to evaluate the impact of ivacaftor on chronic rhinosinusitis (CRS) symptoms in this population. Methods The G551D Observational (GOAL) study was a multicenter prospective cohort study enrolling CF patients ≥6 years with at least 1 G551D mutation. Subjects were provided 20‐item Sino‐Nasal Outcome Test (SNOT‐20) questionnaires prior to ivacaftor therapy and at 1, 3, and 6 months afterward. The impact on rhinologic (R), psychological (P), sleep (S), and ear/facial (E) quality of life (QOL) domains was evaluated separately. Results Of 153 subjects, 129 (84%) completed all questionnaires. Typical baseline symptom burden was low (75% with scores <1) and degree of improvement (ie, reduced scores) was greater with higher baseline scores. SNOT‐20 decreased, reflecting improvement, at all follow‐up intervals (1 month: [mean change ± standard deviation] –0.25 ± 0.53, p < 0.01; 3 months; –0.29 ± 0.58, p < 0.01; 6 months: –0.21 ± 0.58, p = 0.02), but less than the prespecified minimal clinically important difference (0.8). Significant improvement was observed at 1, 3, and 6 months in the R domain (1 month: –0.24, p < 0.01; 3 months: –0.34, p < 0.01; 6 months: –0.25, p < 0.01) and P domain (1 month: –0.25, p < 0.01; 3 months: –0.32, p < 0.01; 6 months: –0.26, p < 0.01), and 1 and 3 months in the S domain (1 months: –0.35, p < 0.01; 3 months: –0.32, p < 0.01; 6 months: –0.18, p = 0.07). There was no improvement in the E domain at any time point. Conclusion Ivacaftor improves QOL in the R, P, and S domains in G551D CF patients, although QOL instruments validated for CRS may not translate well to CF CRS patients because symptom burden was surprisingly low.

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Section: Discussionmentioning

confidence: 99%

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

McCormick

Cho

Lampkin

et al. 2018

Int Forum Allergy Rhinol

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“…[32][33][34][35][36][37][38] In a study with 67 clinically stable pediatric patients with CF, the authors reported that 26.9% subjects had a SpO 2 < 85% during sleep and only 16.4% did not have any nocturnal desaturation events. [32][33][34][35][36][37][38] In a study with 67 clinically stable pediatric patients with CF, the authors reported that 26.9% subjects had a SpO 2 < 85% during sleep and only 16.4% did not have any nocturnal desaturation events.…”

Section: Discussionmentioning

confidence: 99%

“…[32][33][34][35][36][37][38] In a study with 67 clinically stable pediatric patients with CF, the authors reported that 26.9% subjects had a SpO 2 < 85% during sleep and only 16.4% did not have any nocturnal desaturation events. 33 It has been proposed that nocturnal hypoxemia contributes to the development of PH and ultimately cor pulmonale. 33 It has been proposed that nocturnal hypoxemia contributes to the development of PH and ultimately cor pulmonale.…”

Section: Discussionmentioning

confidence: 99%

“…Previous sleep investigations of CF patients using singlenight polysomnography have reported a poor sleep quality due to sleep-disordered breathing. [32][33][34][35][36][37][38] In a study with 67 clinically stable pediatric patients with CF, the authors reported that 26.9% subjects had a SpO 2 < 85% during sleep and only 16.4% did not have any nocturnal desaturation events. 39 The poor sleep quality in CF patients are associated with impaired in clinical, 39 functional 32,34,[38][39][40] and quality of life.…”

Section: Discussionmentioning

confidence: 99%

“…39 The poor sleep quality in CF patients are associated with impaired in clinical, 39 functional 32,34,[38][39][40] and quality of life. 33 It has been proposed that nocturnal hypoxemia contributes to the development of PH and ultimately cor pulmonale. 41 PH evaluated by Doppler echocardiography, without clinical evidence of cor pulmonale, is now associated with reduced survival in adult CF patients.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings

Ziegler

Perin

Casarotto

et al. 2016

Clinical Respiratory J

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Sleep screening for cystic fibrosis patients: A survey of cystic fibrosis programs

Thomas

Brown

2020

Pediatric Pulmonology

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Objective The prevalence of sleep disorders in patients with cystic fibrosis (CF) is unknown, and no standardized screening or treatment guidelines exist to address sleep disorders in CF. The objective of this study is to characterize current sleep screening practices in adult, pediatric, combined, and affiliate CF programs. Methods A survey was developed in Research Electronic Data Capture and distributed to program directors of accredited CF programs in the United States. Results Eighty‐eight program directors responded (36% adult, 43% pediatric, 16% combined, 3% affiliate, and 1% unidentified). Of the respondents, 68% were part of an academic institution, 24% were associated with an academic institution, and 8% were part of a community or private program. Program sizes ranged from less than 50 to more than 500 patients. Routine or informal sleep screening was not performed in 44% of adults, 29% of pediatricians, and 35% of combined and affiliate programs. Most programs (>80%) have access to otolaryngology and sleep medicine although not all of these programs refer patients for evaluation of sleep disorders. Most program directors (77%) perceive sleep disorders as a problem in CF and would recommend routine sleep screening. Possible barriers to sleep screening included clinic flow, screening fatigue, and lack of recommendations for sleep screening. Conclusions Formal sleep screening is inconsistent among CF care centers although most survey respondents would recommend the inclusion of screening in routine CF care. Future work is needed to further evaluate the impact of sleep disorders in CF and determine best practices for standardization of sleep screening and treatment.

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Quality of life in clinically stable adult cystic fibrosis out-patients: associations with daytime sleepiness and sleep quality

Cited by 6 publications

References 0 publications

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

Ivacaftor improves rhinologic, psychologic, and sleep‐related quality of life in G551D cystic fibrosis patients

Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings

Sleep screening for cystic fibrosis patients: A survey of cystic fibrosis programs

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