Non-Invasive Ventilation in Myasthenia Gravis
Clinical Indicators for NIV Initiation
- Myasthenia gravis patients should receive non-invasive ventilation when they demonstrate signs of respiratory distress with a PCO2 > 45 mmHg, forced vital capacity < 15-20 ml/kg, or oxygen saturation < 92% while awake, as recommended by the Centers for Disease Control and Prevention 1
- Forced vital capacity (FVC) < 15-20 ml/kg or < 1 liter is an indicator for NIV initiation, according to the European Respiratory Society 2
- Maximum inspiratory pressure (MIP) < 30 cm H2O is a criterion for NIV initiation, as stated by the Centers for Disease Control and Prevention 1
- Maximum expiratory pressure (MEP) < 40 cm H2O is another indicator for NIV initiation, as recommended by the Centers for Disease Control and Prevention 1
- Oxygen saturation < 92% while awake is a sign of respiratory distress that requires NIV, according to the American Thoracic Society 3, 4
Monitoring Requirements
- Arterial blood gas analysis to evaluate PaO2, PaCO2, and pH is necessary for initial assessment, as recommended by the Centers for Disease Control and Prevention 1
- Spirometry measurements including FVC, MIP, and MEP are required for initial assessment, according to the Centers for Disease Control and Prevention 1
NIV Settings for Myasthenia Gravis
- Initial inspiratory positive airway pressure (IPAP): 8-12 cmH2O is recommended, as stated by the British Thoracic Society 7
- Initial expiratory positive airway pressure (EPAP): 3-5 cmH2O is suggested, according to the British Thoracic Society 7
- FiO2: Start at 40% and titrate to maintain SpO2 > 92%, as recommended by the American College of Chest Physicians 6
Predictors of NIV Success vs. Failure
- Intact bulbar function is a favorable factor for NIV success, as stated by the Centers for Disease Control and Prevention 1
- Severe bulbar dysfunction with risk of aspiration is a risk factor for NIV failure, according to the Centers for Disease Control and Prevention 1
Special Considerations
- Select appropriate mask size and type to minimize air leakage and skin breakdown, as recommended by the British Thoracic Society 7
- Consider alternating between nasal and full-face masks for prolonged use, according to the British Thoracic Society 7
- Failure to improve after 1-2 hours of optimized NIV is an indication for intubation, as stated by the American College of Chest Physicians 6
- Deteriorating level of consciousness is a sign that requires consideration of intubation, according to the British Thoracic Society 7
Pitfalls and Caveats
- NIV should not be used in patients with severe bulbar dysfunction due to risk of aspiration, as recommended by the Centers for Disease Control and Prevention 1
- Oxygen therapy alone should not be used to treat hypoventilation without ventilatory assistance, according to the American Thoracic Society 3, 4