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Noninvasive ventilation (NIV) use was initially reported in cystic fibrosis (CF) in 1991 as a bridge to lung transplantation, and over the decades the use of NIV has increased in the CF population. Individuals with CF are prone to various physiologic changes as lung function worsens, and they may benefit from NIV for advanced lung disease. As life expectancy in CF has been increasing due to advances such as highly effective modulator therapy, people with CF are now subject to the same co-morbidities that may benefit from NIV as their age matched cohorts. NIV can improve gas exchange, quality of sleep, exercise tolerance and augment airway clearance in CF, and it is important that CF providers are comfortable with this therapeutic modality. In this review, we will summarize the physiologic basis for NIV use in CF, describe indications for initiation of NIV, and postulate a practical approach for CF clinicians to take fin monitoring patients on NIV. We will discuss aspects unique to people with CF and the use of NIV. We hope that this serves as a resource for CF providers, especially those of us who do not have dedicated training in sleep medicine, as we continue to care for our patient population.

STUDY OBJECTIVES: Centers for Medicare and Medicaid Services (CMS) reimbursement for positive airway pressure (PAP) devices for obstructive sleep apnea treatment is dependent on patients meeting adherence expectations within the first 3 months on therapy. Adherence is defined as usage of the device for at least 4 hours per night on 70% of nights during a consecutive 30-day period. We hypothesize that the adherence pattern may be established beyond this initial period, which may limit the opportunity to treat many patients. METHODS: Treatment and adherence data from PAP devices were monitored via wireless modems for 42 consecutive PAP-naïve military veterans who completed 1 year of nightly monitoring. Their baseline characteristics were as follows: age (mean ± standard deviation) 58.5 ± 12.5 years; body mass index 33.7 ± 5.7 kg/m(2); diagnostic apnea-hypopnea index (pretreatment) 28.1 ± 18.5 events/h; apnea-hypopnea index on PAP: 4.3 ± 3.3 events/h. We examined daily, monthly, quarterly, semiannual, and annual reports, and the best 30-day adherence report for each quarter. RESULTS: In the first 3 months, 19 of 42 participants were adherent by CMS criteria, and 23 of 42 participants were not. Of the 19 adherent participants, 13 remained adherent and 6 became nonadherent or stopped PAP treatment for the remainder of the year. In the 23 initially nonadherent participants, 16 stopped PAP treatment, and 7 participants (30.4%) became adherent (using CMS criteria) during the rest of the year. Thus, PAP adherence during the first 3 months was predictive for the rest of the year in only 68.4%. PAP nonadherence during the first 3 months was predictive for further nonadherence in only 69.6% of the cases. Overall, this led to a 65% sensitivity and 72% specificity of using adherence at 3 months in predicting adherence at 1 year. CONCLUSIONS: CMS adherence criteria affecting PAP coverage are restrictive and can result in the withholding of therapy in many patients who otherwise might become adherent. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov, Title: Remote Monitoring in Obstructive Sleep Apnea, Identifier: NCT01678560, URL: https:// clinicaltrials.gov/ct2/show/NCT01678560 CITATION: Naik S, Al-Halawani M, Kreinin I, Kryger M. Centers for Medicare and Medicaid Services positive airway pressure adherence criteria may limit treatment to many medicare beneficiaries. J Clin Sleep Med. 2019;15(2):245–251.