1. Scheduled visit to NIV centre	Timing to visit the reference centre between 3-12 months according to disease progression
2. Screening MPV	The screening is first based on symptoms (dyspnea), then PaCO2 and SpO2, speed progression and severe drop in FVC
3. Indication MPV	Timing for the choice of the ventilator (ideally with a dedicated MPV software)
4. Psychological and educational preparation	This step includes the psychological preparation + the demonstration of the material + explanations on the impact of MPV: social impact, facilitating eating, speaking, protecting respiratory progression, etc.)
5. Choices material/settings:	A similar tubing is desirable for night and daytime
5A. Choice MPV tubing	A passive tubing include a single tubing, active tubing includes an exhalation valve
5B. Choice MPV dedicated	A dedicated mode includes the availability of: PEEP at 0, high sensitive trigger via flow interruption, rate at 0, all alarms OFF
6. Pre-settings MPV	Pre-settings occur before the first trial with MPV when patient is not connected
6A. Advised MPV mode	Volume is 1st choice mode and allows: air-stacking, leaks NIV without untimely leak compensation
6B. Suggested tidal volume	Volume is presented as “starting value (range)”. For a child, starting values can be 300 mL, for an adult DMD: 500-600mL, for an ALS patient: 1000mL
6C. Suggested inspiration time	Inspiration time is presented as “starting value (range)”
6D. Suggested trigger	MPV dedicated trigger is a high sensitive flow interruption trigger, classic flow trigger (high or medium sensitive) is less sensitive
6E. Evaluation of NIV dependence	In many countries in Europe, NIV use >16/24 hours defines NIV-dependence
6F. Suggested rate	Comfortable free time can be estimated as >4-6 consecutive hours without NIV
6G. Suggested disconnection alarm	Apnoea alarms may be set in the very dependent patients (example: alarm after 5 minutes with apnoea)
6H. Choice interface	Choice of the mouthpiece according to patient preference after trial with both pieces
6I. Choice tubing support	Custom support on the shoulders is advised for dependent patients
7. MPV trial	The trial & titration can be done in a 2 hour session
8. Monitoring during MPV initiation	Monitoring at initiation is advised during a 20-30 minutes trial. Primary focus is comfort and the way they breath and less on CO2 and saturation
9. Pressure mode	Pressure mode is not advised as the first choice Pressure mode is possible (ST or PSV) with dedicated mode solely. This mode is sometimes preferred by children Pressures can be set at 12-20cmH2O according to patient comfort Pressure mode is not adequate when the patient maintain MPV continuously in his mouth Lip seal around MPV is required. Air-stacking is not possible
10. Long-term monitoring	Long-term monitoring is highly variable among countries and centres It is also recommended to monitor the MIC-VC difference this is to ensure a compliant thorax. When the MIC-VC difference decreases, education is essential to ensure patients optimise the MIC regularly to ensure stretching of the patients thorax to maintain maximal recruitable lung volumes