Fast Five Quiz: Are You Prepared to Manage Spinal Muscular Atrophy?

Stephen L. Nelson, Jr, MD, PhD


May 16, 2019

BiPAP offers noninvasive respiratory support for patients with SMA by aiding breathing while improving lung and chest wall development. Compared with continuous airway pressure, BiPAP offers better respiratory muscle resting. Nocturnal BiPAP is often necessary to prevent sleep-disordered breathing and to promote more comfortable sleep. In general, noninvasive respiratory support is preferred over invasive ventilation with tracheotomy.

There is no evidence to support the use of mucolytics, high-frequency chest wall oscillation, or intrapulmonary percussive ventilation for chronic management of patients with SMA.

Other respiratory management strategies for patients with SMA include:

  • Pulse oximetry monitoring, which may reveal issues with mucus plugging or hypoventilation

  • Cough assistance

  • Postural drainage

  • Oral suctioning

For more on complications associated with SMA, read here.


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