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Dear Editor,
The authors of Management of Critically Ill Patients With Spinal Muscular Atrophy Admitted With Acute Respiratory Failure are to be commended for bringing attention to noninvasive respiratory management of spinal muscular atrophy (SMA).1 However, they understandably misinterpreted references, were unaware of 8 more recent relevant ones, and neglected information that would permit distinguishing their use of noninvasive ventilation (NIV) from full noninvasive respiratory support (NRS) settings. In our center, NRS settings are generally 700–1,500 mL volumes via mouth or other noninvasive interfaces for adults and 16–25 cm H2O for NRS users unable to use a mouthpiece/lip seal for lung volume recruitment or who simply prefer nasal interfaces, including infants. No expiratory positive airway pressure (PAP) is used since it adds inspiratory isometric work; has been reported to be counterproductive, even harmful, for uncomplicated ventilatory pump failure (VPF) patients; and increases risk of aspiration.2
In our 46-year experience with 3,000 NRS users, and in that of other specialized NRS centers that extubate unweanable VPF patients without tracheostomies,3 there has been virtually …
Correspondence: John R Bach MD. E-mail: bachjr{at}njms.rutgers.edu
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