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Spinal Muscular Atrophy Type 1: Management and Outcomes by John R. Bach; J. Scott Baird; Daniel Plosky; Jose Navado; Brian Weaver is a Medicine article available to read on EtoBox.

What is Spinal Muscular Atrophy Type 1: Management and Outcomes about?

Our objectives were to describe survival, hospitalization, speech, and outcomes related to respirator needs for spinal muscular atrophy type 1 (SMA1) patients, using noninvasive or tracheostomy ventilation. From 65 SMA patients referred to our clinic since 1996, we chose 56 SMA1 patients who developed respiratory failure before age 2 years. Patients either had tracheostomy tubes (group A), or used noninvasive ventilation and assisted coughing; a previously reported extubation protocol (group B) was used as needed. Sixteen patients underwent tracheostomy at 10.8 +/- 5.0 months of age, 33 were in group B, and 7 others died without life-support interventions. Compared to group B, group A patients had fewer hospitalizations until age 3 years, but more after age 5, and 15 of 16 lost all spontaneous breathing tolerance posttracheostomy and could not speak. One group A patient died at 16 months of age, and the others were 73.8 +/- 57 months of age (the oldest was 19 years old). Two group B patients died at 6 and 13 months, respectively, whereas the other 31 were 41.8 +/- 26.0 months (and up to 8.3 years) old. Three of 31 in group B required high-span positive inspiratory pressure plus pos

Who reads Spinal Muscular Atrophy Type 1: Management and Outcomes?

Author

John R. Bach; J. Scott Baird; Daniel Plosky; Jose Navado; Brian Weaver

Publisher

John Wiley and Sons; Wiley (John Wiley & Sons); John Wiley & Sons Inc.; Wiley (ISSN 8755-6863)

Published

2002

Language

EN

Field

Medicine (Health Sciences)