PICU Pathway for SMA Patient Admitted
with Acute Respiratory Failure

SMA Patient:
Core Assessments & Management

Admitted to PICU with Acute Respiratory Failure

Maximize Non-invasive Ventilation (NIV):

Artificial Airway

Consider Invasive Ventilation
(initially or after maximizing NIV based on trajectory)

  • Persistent tachycardia
  • Fatigue
  • Diaphoresis
  • Increased airway clearance needs
  • Impaired gas exchange
  • FiO2 40-60% not weaning
  • IPAP > 24 / EPAP > 10 or above baseline
  • Peri-extubation to NIV
  • Immediate Post-extubation
  • Airway Clearance
  • Frequent MI-E (CoughAssist) treatments may be needed


  • IPAP: Inspiratory positive airway pressure
  • EPAP: Expiratory positive airway pressure
  • HFCWO/HFCWC (Vest®): High-frequency chest wall oscillation/high-frequency chest wall compression
  • IPV: Intrapulmonary percussive ventilation
  • CPT: Chest physiotherapy
  • MI-E: Mechanical insufflation-exsufflation (CoughAssist)
  • Pe: Exsufflation pressure
  • Pi: Insufflation pressure
Posted: October 2017
Revised: September 2021
Authors: O. Mayer, MD; H. Panitch, MD; L. Rhodes, RRT; C. Dominick, RRT; H. Wolfe, MD; K. Martin, MD; N. McGowan, RRT; M. Bernstein, RN