REGISTRIES AND CARE OF NEUROMUSCULAR DISORDERS: P.287Mechanically assisted cough: how to keep it simple

Abstract

Mechanical insufflation - exsufflation (MI:E) is a life extending method in neuromuscular patients with cough insufficiency. It supports airway clearance to prevent mucous retention, atelectasis and pneumonia. Some neuromuscular centers (NMC) are still unexperienced and hesitant in its use. In our pediatric NMC we have thus developed a standardized approach to facilitate practical use. Initial routine was based on literature and experience of two large centers. We use only one type of device. MI:E is introduced when cough peak flow is <50% of age-related reference, in ventilated patients and in case of clinical obvious cough insufficiency. We preset 3 different modes for home use. Mode 1 provides only insufflation for daily lung volume recruitment in health. In mode 2 in- and exsufflation are preset for airway secretions or SpO2<95%. In mode 3 expiratory oscillation during exsufflation is added for sticky secretions. Durations of in- and exsufflation are increased with age while pressures for in- and exsufflation are fixed at 30-35 cmH2O and -40 cmH2O. Staff, patients and caregivers are trained in MI:E to use modes depending on health condition of the patient and for emergencies. Retraining is performed regularly. This standardization facilitated teaching of staff, patients and caregivers in MI:E in our and other Austrian NMCs. Acceptance was rapid. Only 10% required adaptations to optimize efficacy. Proactive initiation and empowerment of patients and caregivers through training in applying those 3 different modes prevent emergencies. In 60 patients initiated using MI:E during 4 years no pneumothorax occurred. Only 2 SMA 1 patients died due to severe course of disease. This model could be used as a guide for NMCs with limited specialized staff for and expertise in respiratory management. Further investigations regarding the effects on clinical end points like hospitalisation rate and survival are needed.