Methods to Improve Expiratory Cough Flow and Lung Volume in Children With NMDs

The non-invasive bipap ventilator will be used in the following respiratory treatment: bipap assisted inspiratory breaths (BAIB). The patient will be encouraged to take deep breaths at least 1.5 times greater than resting tidal volume. The pressure assistance provided by the bipap starts at 10cm H2O. Patients already on bipap will use their home settings. Starting pressure is then increased as necessary to meet the maximal inspiratory goal volume in steps of 2 up to a maximum 20cm H2O. Once the ideal pressure of support levels are established, the child will perform 3 cycles of 5 deep breaths. After each cycle the patient will pause and perform huffing and assisted coughing. The patient will be suctioned as necessary.

A commercial mechanical insufflator-exsufflator will be used in the following treatment: Mechanical insufflation-exsufflation (MI-E). The pressure will be administered by a face mask. The optimal pressure for the test will start at 10 cm H2O and will in crease in steps of 5 to a maximum of 25 cm H2O. The insufflation phase will take 3 seconds followed by a 2 second exsufflation phase. Three cycles of treatment will be used. Between each cycle the subject will pause and perform huffing and assisted coughing. The patient will be suctioned as necessary.

A commercial high frequency percussive oscillator will be used in the following treatment: combined oscillation and nebulisation. Pressures will be delivered by a face mask. At higher oscillation frequencies it is usually necessary to support the cheeks. Pressure will be gradually be increased to accustom the child to a maximum of 25cm H2O. This standard maximal pressure will ideally be used for every child. A treatment cycle will consist of 3 minutes at a frequency of 2 Hz and pressure of 15cm H2O followed by 3 minutes of 4 Hz at 20cm H2O, then 3 minutes of 5 Hz at 25cm H2O. Throughout each cycle, the patient will receive nebulized saline through the machine. After each cycle the patient will pause and perform huffing and assisted coughing. The patient will be suctioned as necessary.