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The choice of interface used to deliver noninvasive ventilation (NIV) is a critical element in successfully and safely establishing home NIV in people with sleep hypoventilation syndromes. Both patient-related and equipment-related factors need to be considered when selecting an interface. Recognizing specific issues that can occur with a particular style of mask is important when troubleshooting NIV problems and attempting to minimize side effects. Access to a range of mask styles and designs to use on a rotational basis is especially important for patients using NIV on a more continuous basis, those at risk of developing pressure areas, and children., Competing Interests: Disclosure The author reports income related to medical education conducted on behalf of ResMed and Philips, manufacturers of bilevel devices and interfaces for noninvasive ventilation., (Copyright © 2024 Elsevier Inc. All rights reserved.)

This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey., Competing Interests: Declaration of competing interest The authors had no declarations of interest in relation to the current work. Declarations of interest for each author outside the current work are summarised in Supplementary Table 4., (Copyright © 2024. Published by Elsevier B.V.)

The obesity hypoventilation syndrome (OHS) is associated with significant morbidity and increased mortality compared with simple obesity and eucapnic obstructive sleep apnea. Accurate diagnosis and commencement of early and appropriate management is fundamental in reducing the significant personal and societal burdens this disorder poses. Sleep disordered breathing is a major contributor to the developmental of sleep and awake hypercapnia, which characterizes OHS, and is effectively addressed through the use of positive airway pressure (PAP) therapy. This article reviews the current evidence supporting different modes of PAP currently used in managing these individuals., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Obesity hypoventilation syndrome is becoming an increasingly encountered condition both in respiratory outpatient clinics and in hospitalized patients. The health consequences and social disadvantages of obesity hypoventilation syndrome are significant. Unfortunately, the diagnosis and institution of appropriate therapy is commonly delayed when the syndrome is not recognized or misdiagnosed. Positive airway pressure therapy remains the mainstay of treatment and is effective in controlling sleep-disordered breathing and improving awake blood gases in the majority of individuals. Evidence supporting one mode of therapy over another is limited. Both continuous and bilevel therapy modes can successfully improve daytime gas exchange, with adherence to therapy an important modifiable factor in the response to treatment. Despite adherence to therapy, these individuals continue to experience excess mortality primarily due to cardiovascular events compared with those with eucapnic sleep apnea using CPAP. This difference likely arises from ongoing systemic inflammation secondary to the morbidly obese state. The need for a comprehensive approach to managing nutrition, weight, and physical activity in addition to reversal of sleep-disordered breathing is now widely recognized. Future studies need to evaluate the impact of a more aggressive and comprehensive treatment plan beyond managing sleep-disordered breathing. The impact of early identification and treatment of sleep-disordered breathing on the development and reversal of cardiometabolic dysfunction also requires further attention., (Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Question: During an acute exacerbation of cystic fibrosis, is non-invasive ventilation beneficial as an adjunct to the airway clearance regimen?, Design: Randomised controlled trial with concealed allocation and intention-to-treat analysis., Participants: Forty adults with moderate to severe cystic fibrosis lung disease and who were admitted to hospital for an acute exacerbation., Intervention: Comprehensive inpatient care (control group) compared to the same care with the addition of non-invasive ventilation during airway clearance treatments from Day 2 of admission until discharge (experimental group)., Outcome Measures: Lung function and subjective symptom severity were measured daily. Fatigue was measured at admission and discharge on the Schwartz Fatigue Scale from 7 (no fatigue) to 63 (worst fatigue) points. Quality of life and exercise capacity were also measured at admission and discharge. Length of admission and time to next hospital admission were recorded., Results: Analysed as the primary outcome, the experimental group had a greater rate of improvement in forced expiratory volume in 1 second (FEV1) than the control group, but this was not statistically significant (MD 0.13% predicted per day, 95% CI -0.03 to 0.28). However, the experimental group had a significantly higher FEV1 at discharge than the control group (MD 4.2% predicted, 95% CI 0.1 to 8.3). The experimental group reported significantly lower levels of fatigue on the Schwartz fatigue scale at discharge than the control group (MD 6 points, 95% CI 1 to 11). There was no significant difference between the experimental and control groups in subjective symptom severity, quality of life, exercise capacity, length of hospital admission or time to next hospital admission., Conclusion: Among people hospitalised for an acute exacerbation of cystic fibrosis, the use of non-invasive ventilation as an adjunct to the airway clearance regimen significantly improves FEV1 and fatigue., Trial Registration: ANZCTR 12605000437662., (Copyright © 2015. Published by Elsevier B.V.)

Background: Polysomnography findings between matched groups with obstructive sleep apnea (OSA) and OSA plus obesity-hypoventilation syndrome (OHS) before and after continuous positive airway pressure (CPAP), particularly in the extremely severe obese (body mass index [BMI] >or= 50 kg/m2), are unclear., Design: Prospective study of subjects (BMI >or= 50 kg/m2) undergoing diagnostic polysomnography. Subjects with an apnea-hypopnea index (AHI) >or= 15/h underwent a second polysomnography with CPAP. The effect of 1 night of CPAP on sleep architecture, AHI, arousal indexes, and nocturnal oxygenation was assessed. OHS was defined as those subjects with obesity, PaCo2 > 45 mm Hg, and PaO2 < 70 mm Hg in the absence of lung disease., Results: Twenty-three subjects with moderate-to-severe OSA and 23 subjects with moderate-to-severe OSA plus OHS underwent a 1-night trial of CPAP. Both groups were matched for spirometry, BMI, and AHI, but oxygen desaturation was worse in the OSA-plus-OHS group. CPAP significantly improved rapid eye movement (REM) duration (p < 0.005), AHI (p < 0.005), arousal indexes (p < 0.005), and percentage of total sleep time (TST) with oxygen saturation (SpO2) < 90% (p < 0.005) in both groups. In subjects with OSA plus OHS, 43% continued to spend > 20% of TST with SpO2 < 90%, compared to 9% of the OSA group, despite the adequate relief of upper airway obstruction., Conclusions: Extremely severe obese subjects (BMI >or= 50 kg/m2) with moderate-to-severe OSA plus OHS exhibit severe oxygen desaturation but similar severities of AHI, arousal indexes, and sleep architecture abnormalities when compared to matched subjects without OHS. CPAP significantly improves AHI, REM duration, arousal indexes, and nocturnal oxygen desaturation. Some subjects with OHS continued to have nocturnal desaturation despite the control of upper airway obstruction; other mechanisms may contribute. Further long-term studies assessing the comparative role of CPAP and bilevel ventilatory support in such subjects with OHS is warranted.

Background: Supplemental nocturnal oxygen is widely used for hypoxaemic respiratory failure in adults with CF., Methods: In order to determine the factors that predict the development of progressive hypercapnia on oxygen ("failure of oxygen therapy") and the subsequent role of bi-level pressure support ventilation (BVS), we reviewed the outcomes of 39 adults with CF who were treated for hypoxaemic respiratory failure between 1991 and 2002 using strict physiological criteria for the commencement of oxygen and the subsequent commencement of BVS., Results: Twenty of the 39 failed oxygen therapy, 13 of these within 12 months. Baseline PaCO2, rather than age, BMI or FEV1, predicted failure of oxygen therapy within 12 months. A PaCO2>6.5 kPa (49 mm Hg) was significantly associated with failure within 12 months (p=0.04). Twenty patients with mean PaCO2 7.9+/-1.3 kPa (59+/-10 mm Hg) and mean pH 7.38+/-0.05 had a significant reduction in their mean PaCO2 after 1 month of BVS (p=0.007)., Conclusions: Both oxygen and BVS can successfully stabilise patients to transplant. In patients commencing oxygen, baseline PaCO2 is predictive of the development of progressive hypercapnia within 12 months. BVS can successfully attenuate the rise in PaCO2 in the short term.