Sleep in children with cystic fibrosis: More under the covers

Reiter, Joel; Breuer, Oded; Cohen‐Cymberknoh, Malena; Forno, Erick; Gileles‐Hillel, Alex

doi:10.1002/ppul.25462

Cited by 8 publications

(5 citation statements)

References 72 publications

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“…However, sleep architecture (N1, N2, N3 and REM stages) was preserved despite reduced sleep quality. These findings are consistent with data from other studies in children and adult pwCF [ 13 , 19 , 23 , 53 ].…”

Section: Discussionsupporting

confidence: 93%

“…Most current data regarding sleep quality and sleep architecture in pwCF come from studies in children and adolescents. Consistent with existing literature in adult pwCF [ 22 , 24 , 52 , 53 ], we confirmed that these patients have reduced sleep quality (total sleep time, sleep efficiency) and increased sleep latency and wake after sleep onset. There are multiple potential contributors to impaired subjective and objective sleep in pwCF, including nocturnal coughing, pain, chronic rhinosinusitis, CF-related diabetes, and PEG-feeding [ 54 ].…”

Section: Discussionsupporting

confidence: 92%

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Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

et al. 2022

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Background Obstructive sleep apnea (OSA), nocturnal hypoxemia and excessive daytime sleepiness (EDS) are common comorbidities in people with cystic fibrosis (pwCF). Most of the data showing this originates from children and adolescents. The aim of this study was to collect data on sleep parameters, EDS and pulmonary function from a large cohort of adult pwCF. Methods Full overnight polysomnography (PSG) was performed. EDS was determined using the Epworth Sleepiness Scale (ESS). Demographic and clinical data (body mass index [BMI], pulmonary function, capillary blood gases) were collected. Results A total of 52 adult pwCF were included (mean age 30.7 ± 8.0 years, mean percent predicted forced expiratory volume in 1 s [ppFEV1] of 52.1 ± 14.8). Overall AHI was in the normal range (4.5 ± 4.0/h); 21/52 pwCF (40%) had an apnea-hypopnea index > 5/h. Nocturnal hypoxemia was found in 25% of participants and this was associated with ppFEV1 (p = 0.014), awake oxygen saturation (SpO2; p = 0.021) and awake partial pressure of oxygen (pO2; p = 0.003); there were no significant differences in age, lung function and BMI were found for pwCF with versus without OSA (all p > 0.05). Eight pwCF (15%) had an ESS score > 10 (indicating EDS). OSA was best predicted by awake pO2 (area under the curve [AUC] 0.66, p = 0.048), while nocturnal hypoxemia was best predicted by ppFEV1 (AUC 0.74, p = 0.009), awake pO2 (AUC 0.76, p = 0.006) and awake SpO2 (AUC 0.71; p = 0.025). Conclusion OSA, nocturnal hypoxemia and EDS were common in adult pwCF, but no strong predictors were identified. Therefore, we suggest regular PSG and ESS scoring in adult pwCF, regardless of disease severity.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionsupporting

confidence: 92%

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

et al. 2022

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“…Our results show that despite reduced sleep quality, sleep architecture (N1, N2, N3 [slow-wave sleep], and REM stages) is preserved. This is consistent with the results of other studies in children and adult pwCF [7, 22, 46, 47].…”

Section: Discussionsupporting

confidence: 93%

“…PwCF often report disturbed sleep. Nocturnal coughing, chronic rhinosinusitis and nasal polyps, hypoxia, and increased work of breathing negatively affect sleep and lead to abnormal sleep with significantly reduced TST and SE, and increased SL and WASO [21,[44][45][46]. These results are confirmed by the current study, which showed reduced sleep quality in adult pwCF.…”

Section: Discussionsupporting

confidence: 87%

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

et al. 2022

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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.

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“…Furthermore, different from patients with bronchiectasis and mycobacterial infections, patients with CF often manifest poor sleep quality with frequent awakenings and daytime sleepiness, and such disturbances are more frequent with the progression of the disease [ 68 ]. Despite this, SDB prevalence is still under-recognized in this population of patients and may impact disease outcomes [ 69 ].…”

Section: Sleep-disordered Breathing and Cystic Fibrosismentioning

confidence: 99%

Sleep-Disordered Breathing and Chronic Respiratory Infections: A Narrative Review in Adult and Pediatric Population

Faverio

Zanini

Monzani

et al. 2023

IJMS

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Sleep-disordered breathing (SDB) comprises different diseases characterized by abnormal respiratory patterns during sleep including obstructive sleep apnea. SDB prevalence and impact in patients with chronic respiratory infections have been only marginally studied. The purpose of this narrative review is to report the prevalence and impact of SDB in chronic respiratory infections, including cystic fibrosis (CF), bronchiectasis and mycobacterial infections, and explore the possible pathophysiological mechanisms. Common pathophysiological mechanisms, underlying SDB onset in all chronic respiratory infections, include inflammation, which plays a central role, chronic nocturnal cough and pain, excessive production of mucous plugs, presence of obstructive and/or restrictive ventilatory impairment, upper airways involvement, and comorbidities, such as alteration of nutritional status. SDB may affect about 50% of patients with bronchiectasis. The severity of the disease, e.g., patients colonized with P. aeruginosa and frequent exacerbators, as well as comorbidities, such as chronic obstructive pulmonary disease and primary ciliary dyskinesia, may impact SDB onset. SDB may also frequently complicate the clinical course of both children and adults with CF, impacting the quality of life and disease prognosis, suggesting that their routine assessment should be incorporated into the clinical evaluation of patients from the first stages of the disease regardless of suggestive symptoms, in order to avoid late diagnosis. Finally, although the prevalence of SDB in patients with mycobacterial infections is uncertain, extrapulmonary manifestations, particularly nasopharyngeal locations, and concomitant symptoms, such as body pain and depression, may act as atypical predisposing factors for their development.

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Sleep in children with cystic fibrosis: More under the covers

Cited by 8 publications

References 72 publications

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis

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

Sleep-Disordered Breathing and Chronic Respiratory Infections: A Narrative Review in Adult and Pediatric Population

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