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Abstract
Background: Spinal muscular atrophy (SMA) is a neurodegenerative disease that results in progressive muscular atrophy and weakness. The primary cause of morbidity and mortality in these children is pulmonary disease due to poor airway clearance that leads to acute respiratory failure. There is a paucity of literature on the treatment of children with SMA and acute respiratory failure.
Methods: We performed a retrospective chart review of pediatric patients with SMA type I or II admitted to the PICU in a tertiary-care children's hospital with acute respiratory failure who required mechanical ventilation and/or aggressive airway clearance.
Results: The study included 300 unique encounters among 137 unique subjects. All the subjects received airway clearance at home before admission, and 257 encounters (85.7%) were supported with noninvasive ventilation (NIV) before admission. Sixty-eight subjects (49.6%) required endotracheal intubation on admission or at some point during their PICU stay. The median (interquartile range [IQR]) time to intubation was 0 (0–20) h, and the median (IQR) duration of mechanical ventilation was 2 (1–5) d on invasive mechanical ventilation with no statistical difference between type I and type II (P = .89). Of those, 65 (91.1%) were successfully extubated to NIV on the first attempt, whereas 4 subjects (5.8%) required multiple extubation attempts and 3 subjects (4.4%) required subsequent tracheostomy. For the subjects who were intubated, both PICU and hospital length of stay were longer (P < .001) when compared with the subjects managed by NIV alone. The subjects with SMA type I had a longer PICU length of stay, with a median (IQR) of 5 (3–11) d versus 4 (2–7) d (P = .002). The hospital length of stay and duration of invasive mechanical ventilation were not statistically different (P = .055 and P = .068, respectively).
Conclusions: The subjects with SMA types I or II can be treated successfully with NIV and aggressive airway clearance during acute respiratory failure. Similarly, when intubation is required, successful extubation can be achieved with NIV transitional support combined with aggressive airway clearance maneuvers.
- spinal muscular atrophy
- SMA
- mechanical ventilation
- pediatrics
- neuromuscular diseases
- mucociliary clearance
- pediatrics
Footnotes
- Correspondence: Awni M Al-Subu MD, Division of Pediatric Critical Care, Department of Pediatrics, University of Wisconsin School of Medicine & Public Health, 600 Highland Ave, H6/535 CSC, Madison, WI 53792. E-mail: al-subu{at}pediatrics.wisc.edu
The authors have disclosed no conflicts of interest.
This study was conducted at the University of Wisconsin-Madison, Madison, Wisconsin.
Supplementary material related to this paper is available at http://www.rcjournal.com.
- Copyright © 2023 by Daedalus Enterprises
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