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

Abstract

BACKGROUND:

There are widely discrepant views on the respiratory management of infants with spinal muscular atrophy (SMA) type I. Typically, management is palliative.

DESIGN:

A descriptive study of interventions and investigations is reported that were offered to a cohort of 13 children with SMA type I referred to our centre. Interventions and investigations included sleep studies, provision of non-invasive positive pressure ventilation (NIPPV) for ventilatory support/dependency and for physiotherapy and the use of mechanical insufflation/exsufflation (MI-E).

RESULTS:

NIPPV was provided for the following indications: continuous positive airways pressure flow driver dependency (n=3), nocturnal hypoventilation (n=3), to enable successful extubation (n=2), in anticipation of respiratory decompensation (n=3), and oxygen dependency/decompensation (n=2). NIPPV and MI-E were used for successful protocol-led extubations (n=9) but not non protocol-led successes (n=3). NIPPV was essential for discharge home in patients with ventilatory dependency (n=7) and was used for palliation of respiratory symptoms (n=4). Chest wall shape improved with NIPPV. The parents of children who died (n=5) were positive about the use of these techniques.

CONCLUSION:

NIPPV can be used to facilitate discharge home, and MI-E is helpful in this group. This symptom and goal-directed approach can be used to inform medical decision making and to help parents make informed choices about the appropriateness of respiratory interventions in SMA type I.

• Authors: M Chatwin, A Bush, A K Simonds
• Journal: Archives of disease in childhood (Arch Dis Child) Vol. 96 Issue 5 Pg. 426-32 (May 2011) ISSN: 1468-2044 [Electronic] England
• PMID: 20573738 (Publication Type: Evaluation Study, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Combined Modality Therapy
• Female
• Funnel Chest (etiology, pathology, therapy)
• Home Care Services, Hospital-Based
• Humans
• Infant
• Insufflation (methods)
• Male
• Palliative Care (methods)
• Physical Therapy Modalities
• Positive-Pressure Respiration (methods)
• Respiratory Insufficiency (etiology, therapy)
• Spinal Muscular Atrophies of Childhood (complications)
• Treatment Outcome