• Giancarlo Ottonello, Chiara Mastella, Alessia Franceschi, Daniela Bosticco, Andrea Wolfler, Marina Pedemonte, Irene Esposito, and Cesare Gregoretti.
• U.O. Anestesia e Rianimazione IRCCS G Gaslini, Genoa, Italy.
• Am J Phys Med Rehabil. 2011 Nov 1;90(11):895-900.

ObjectiveThe aim of this study was to report the outcomes of an oximetry protocol using up to continuous full ventilator-setting noninvasive ventilation (NIV) and mechanically assisted coughing (MAC) to avoid episodes of acute respiratory failure and hospitalizations for children with spinal muscular atrophy type 1 under 3 yrs of age.DesignThis study was a retrospective chart review of 16 patients with spinal muscular atrophy type 1 under 3 yrs of age consecutively referred for respiratory decompensations resulting in continuous NIV dependence and oxyhemoglobin desaturation. An avoided hospitalization was defined by the need for continuous NIV using high span bilevel positive airway pressure and reversal of desaturations by MAC in the home setting. The protocol included training and equipping parents to use NIV, MAC, and basic life support.ResultsThere were 49 acute episodes (1.20/patient per year), of which 43 met the criteria for an avoided hospitalization. Therefore, only six episodes (0.15/patient per year) required hospitalization, and four required endotracheal intubation (0.1/patient per year). Three of the four were extubated after 4, 9, and 15 days, respectively, to full NIV support and aggressive MAC. The fourth patient, for whom NIV could not be provided, underwent an emergency intubation at home and died at the age of 40 mos.ConclusionsA protocol including high span bilevel positive airway pressure along with MAC to expel airway secretions and normalize oxyhemoglobin saturation can be used by trained caregivers to avoid episodes of acute respiratory failure and hospitalization for children with spinal muscular atrophy type 1 under 3 yrs of age.

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