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Abstract Introduction Respiratory failure is the most common cause of death in patients with several types of neuromuscular disease (NMD), including myotonic dystrophy type 1 (MD1). Non-invasive ventilation (NIV) is the cornerstone of treatment, but consistent use and acceptability remain a challenge. While nap-titrations have improved CPAP adherence in obstructive sleep apnea (OSA), we present a case where increased use and optimization of therapy was achieved through a daytime nap NIV titration. Report of case(s) 47-year-old man with MD1, obesity , and mild OSA with long-standing PAP intolerance presented with dyspnea and hypoxia. Blood gas was suggestive of hypoventilation and work-up for hypoxia including CT chest with pulmonary artery protocol and echocardiogram with bubble were unremarkable. BIPAP and supplemental oxygen were applied with subsequent improvement. Pulmonary function testing was also consistent with hypoventilation, featuring MIP of 14 cmH2O (15% predicted), FVC of 3.30L (65% predicted), and no diffusion impairment. Shortly after discharge, patient was transitioned to NIV with iVAPS with EPAP 5 cmH2O, PS 4-12 cmH2O, TV 6cc/kg, and RR 12 with 4L oxygen. Despite consistent use, ongoing hypoxia was noted on home oximetry testing. The patient was then brought into the sleep lab for a daytime NIV titration study, or “NIV Nap.” During this 2-hour study, he was found to have persistent obstructive events, and was titrated to BIPAP S/T with EPAP 10 cm H2O, IPAP 16 cm H2O, RR 16, and I-time of 0.8-1.5 seconds which achieved optimal therapy for his OSA and hypoventilation. Following this titration, he was able to weaned off of nocturnal oxygen and reported improved symptoms. At 30-day follow-up, remote data demonstrated NIV usage 100% of days, 4-hour/day usage of 87%, residual AHI of 1.3/hr, average TV of 519 mL, and average MV of 10.8 L/min. Conclusion While daytime nap titrations have been used with good effect in patients with OSA, this tool has been underutilized in patients with neuromuscular disease and complex hypoventilation. Daytime “NIV Naps” can allow for increased acceptability of treatment while assuring safe and effective NIV therapy, and in this case demonstrated an increase in use. Support (if any) N/A
Thetford et al. (Sat,) studied this question.