Outcome of goal-directed non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I

• DOI: 10.1136/adc.2009.177832
• Publication Date: 2010-06-24T01:11:11Z
• Authors: M. Chatwin; A. Bush; A. K. Simonds
• Keywords: Exsufflation; Decompensation

<h3>Background</h3> There are widely discrepant views on the respiratory management of infants with spinal muscular atrophy (SMA) type I. Typically, is palliative. <h3>Design</h3> A descriptive study interventions and investigations reported that were offered to a cohort 13 children SMA I referred our centre. <h3>Interventions investigations</h3> included sleep studies, provision non-invasive positive pressure ventilation (NIPPV) for ventilatory support/dependency physiotherapy use mechanical insufflation/exsufflation (MI-E). <h3>Results</h3> NIPPV was provided following indications: continuous airways flow driver dependency (n=3), nocturnal hypoventilation enable successful extubation (n=2), in anticipation decompensation oxygen dependency/decompensation (n=2). MI-E used protocol-led extubations (n=9) but not non successes (n=3). essential discharge home patients (n=7) palliation symptoms (n=4). Chest wall shape improved NIPPV. The parents who died (n=5) about these techniques. <h3>Conclusion</h3> can be facilitate home, helpful this group. This symptom goal-directed approach inform medical decision making help make informed choices appropriateness