POINT: Is Noninvasive Ventilation Always the Most Appropriate Manner of Long-term Ventilation for Infants With Spinal Muscular Atrophy Type 1? Yes, Almost Always

Spinal muscular atrophy (SMA) is an inherited disease of anterior horn cells. It varies in severity from type 1A (SMA1; or type 0), for which continuous noninvasive ventilatory support (CNVS) and enteral nutrition are required before 6 months of age (10% of cases); to typical, for which CNVS and enteral nutrition are needed by 18 months (75%) of age; to mild, when CNVS and enteral nutrition are subsequently needed (15%).1,2 Without respiratory support, SMA1 has 50% mortality by 7 months of age and 90% by 1 year of age.3

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