Abstract
Previous reports on spinal muscular atrophy type 1 (SMA-1) underestimate potential survival because of failure to optimally use noninvasive respiratory muscle aids including mechanically assisted coughing and noninvasive ventilation (NIV) at full support settings. We report our center’s experience in prolonging survival for these patients. We focus on early initiation of nasal noninvasive ventilation, mechanically assisted coughing, and pulse oximetry monitoring during acute respiratory tract infections to guide use of assistive technologies. Seventeen SMA-1 patients with ventilation via tracheostomy are living, with a mean age of 78.2 (range 65–179) months. Ten died at a mean age of 61.6 (range 16–270) months of age. Twenty five of 27 were not able to regain autonomous breathing ability after the tracheostomy. None of the 21 who had not developed the ability to verbalize before undergoing tracheotomy did so after tracheotomy. Six patients had comprehendible speech at the time of tracheotomy and retained ...
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