Inpatient and PICU Clinical Pathway for Management of Patients with Neuromuscular Compromise and Respiratory Failure
- Core Management and Planning
Respiratory, skin and positoining, nutrition, consults - Airway Clearance Management
- Mechanical Strategies
Suction, Cough Assist, CPT, IPV, HFCWO (Vest) - Pharmacologic Strategies
Mucolytic agents - Oxygenation Goals
SpO2 > 95% in room air
- Mechanical Strategies
Baseline respiratory status
Admitted to
Pulmonary Service
Pulmonary Service
Respiratory distress
Admitted to PICU with
Acute Respiratory Failure
Acute Respiratory Failure
Continue baseline support and secretion clearance therapies
Optimize NIV
- NIV Management Strategies
- Airway Clearance Management
- Critical Assessment Team indications
- Increased EPAP by ≥ 2 cm H2O
- FiO2 requirement > 0.40 for > 1 hr, despite PRN secretion clearance
- Inability to sprint for
2 hrs BID, if applicable
Maximize NIV
- NIV Management Strategies
- Airway and Secretion Clearance
- Airway Clearance Management
Invasive Ventilation Considerations
Initially or after maximizing
NIV based on trajectory
NIV based on trajectory
Invasive Ventilation
Transition to chronic management: Determine long term plan for airway clearance
Airway Clearance Management: Mechanical, Pharmacologic Interventions
Airway Clearance Management: Mechanical, Pharmacologic Interventions
Definitions
- NIV
Non-invasive ventilation - 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 - Pe
Exsufflation pressure - Pi
Insufflation pressure
Evidence
- Special Considerations in the Respiratory Management of Spinal Muscular Atrophy
- Diagnosis and Management of Spinal Muscular Atrophy: Part 2: Pulmonary and Acute Care; Medications, Supplements and Immunizations; Other Organ Systems; and Ethics
- Airway Clearance in Patients with Neuromuscular Disease
CHOP Programs
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