Median and range values of ventilatory parameters and adherence to NIV
| Visit | Baseline | Follow-up 1 | Follow-up 2 | Follow-up 3 | Follow-up 4 | Follow-up 5 |
| Time of treatment (months) | – | 4 (1–8) | 7 (4–16) | 13 (7–20) | 21 (11–27) | 30 (21–33) |
| Patients requiring NIV (#) | 4 | 4 | 4 | 4 | 4 | 6 |
| IPAP (cmH2O) | 17 (14–17) | 17 (14–18) | 16 (14–8) | 16 (14–18) | 16 (15–17) | 17 (14–19) |
| EPAP (cmH2O) | 5 (5–6) | 6 (5–6) | 6 (5–6) | 6 (5–6) | 6 (5–7) | 7 (5–8) |
| Use (hours/day) | 8 (5–8) | 8 (5–10) | 8 (4–9) | 8 (6–9) | 8 (8–10) | 8 (6–10) |
EPAP, expiratory positive airway pressure; IPAP, inspiratory positive airway pressure; NIV, non-invasive mechanical ventilation.