Inpatient and PICU Clinical Pathway for Management of Patients with Neuromuscular Compromise and Respiratory Failure

Goals and Metrics
Provider Resources
Patients with Neuromuscular Conditions
Affecting Respiratory Status
Admitted to
Medical/Surgical Unit
Admitted to PICU with
Acute Respiratory Failure
Pre-procedure gene therapy or Post-op or non-respiratory failure diagnosis
Respiratory
distress
Continue baseline settings and secretion clearance therapies
NIV and Airway Clearance Weaning Strategies – Pulmonary Floor
NIV and Airway Clearance Weaning Strategies – PICU
Invasive Ventilation Weaning Strategies
Extubate to NIV
  • Peri-extubation to NIV
  • Immediate Post-extubation Airway Clearance
  • Frequent MI-E (CoughAssist) treatments may be needed
  • CAT call if:
    • Increased PEEP by 2cmH2O
    • Acute FiO2 > 40% for > 1 hr, despite PRN secretion clearance
    • Unable to sprint BID for 2 hrs
    • Clinician Concern
Maximize Non-Invasive Ventilation (NIV)
Consider Invasive Ventilation
Initially or after maximizing NIV based on trajectory
Artificial Airway
  • Persistent tachycardia
  • Fatigue
  • Diaphoresis
  • Increased airway clearance needs
  • Impaired gas exchange
  • FiO2 40-60% not weaning
  • IPAP > 24 / EPAP > 10 or above baseline

Definitions

  • 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
Transition to chronic management: Determine long term plan for airway clearance
First- and Second-line Management Strategies
Disposition
Posted: October 2017
Last Revised: October 2024
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