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Management of Critically Ill Patients With Spinal Muscular Atrophy Admitted With Acute Respiratory Failure
Awni M Al-Subu1, Carolyn K Adams2, Scarlett A Dykstra2
1Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin. al-subu@pediatrics.wisc.edu.
Insights
Pediatric patients with spinal muscular atrophy (SMA) types I and II experiencing acute respiratory failure can be successfully managed with noninvasive ventilation (NIV) and aggressive airway clearance, even after intubation.
Area of Science:
- Pediatric critical care medicine
- Neuromuscular disorders
- Respiratory failure management
Background:
- Spinal muscular atrophy (SMA) is a neurodegenerative disease causing progressive muscle weakness and atrophy.
- Pulmonary complications, particularly acute respiratory failure due to impaired airway clearance, are the leading cause of mortality in children with SMA.
- Limited literature exists on managing acute respiratory failure in pediatric SMA patients.
Purpose of the Study:
- To evaluate the effectiveness of noninvasive ventilation (NIV) and aggressive airway clearance in pediatric patients with SMA types I and II experiencing acute respiratory failure.
- To compare outcomes between patients managed with NIV alone versus those requiring invasive mechanical ventilation.
Main Methods:
- Retrospective chart review of pediatric patients with SMA types I or II admitted to a pediatric intensive care unit (PICU) with acute respiratory failure.
- Analysis of patients requiring mechanical ventilation and/or aggressive airway clearance.
- Comparison of outcomes including intubation rates, ventilation duration, extubation success, and length of stay.
Main Results:
- Out of 137 subjects (300 encounters), 49.6% required intubation. Successful extubation to NIV was achieved in 91.1% of intubated patients.
- Patients requiring invasive mechanical ventilation had longer PICU and hospital stays compared to those managed with NIV alone.
- No significant difference in invasive mechanical ventilation duration between SMA type I and type II patients.
Conclusions:
- Noninvasive ventilation (NIV) combined with aggressive airway clearance is a successful treatment strategy for acute respiratory failure in pediatric SMA types I and II.
- Transitional support with NIV following extubation, along with continued aggressive airway clearance, facilitates successful weaning from invasive mechanical ventilation.
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.
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