| Obtain anesthesiology and pulmonology consultations before procedures involving general anesthesia or procedural sedation |
| Perform a pulmonary evaluation that includes measurement of forced vital capacity (FVC), maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), peak cough flow (PCF), and oxyhemoglobin saturation measured by pulse oximetry (SpO2) in room air |
| • Measure the blood and/or end-tidal carbon dioxide level if SpO2 is <95% in room air |
| • For patients with DMD who are at increased risk of respiratory complications, defined by an FVC <50% of predicted, and especially for patients at high risk for complications, defined by an FVC <30% of predicted, consider preoperative training in the use of noninvasive positive pressure ventilation (NPPV) |
| • For patients at high risk of ineffective cough, defined in adults by PCF <270 L/minute or MEP <60 cm H2O, consider preoperative training in manual and mechanically assisted cough, emphasizing use of mechanical insufflation-exsufflation with a bronchial secretion clearance device (eg, CoughAssist) |
| Refer the patient to a cardiologist for clinical evaluation and optimization of cardiac therapies |
| Obtain a nutritional assessment, optimize nutritional status, and consider strategies to manage dysphagia |
| Discuss the risks and benefits of general anesthesia or procedural sedation with the patient and guardians, and help them to decide on and implement their decisions regarding resuscitation parameters and, if applicable, advance directives |
