Table 3.
Recommended parameters for Guiding Discontinuation of Mechanical Ventilation after positive response to Gene Therapy based on the ASPIRO experience
| Assessments | Parameters |
|---|---|
| Gas exchange markers | |
| Sprinting (video record the assessment) |
No distress or SpO2 < 94% or TCO2 > 45 mmHg during respiratory sprinting trial No intercostal retraction, tachypnea, or respiratory paradox |
| Nocturnal respiration monitoring | |
| Transcutaneous CO2 (TcCO2) | 35-45mmHg |
| End Tidal CO2 (ETCO2) | 35-45mmHg |
| Oxygen saturation (SpO2) | > 94% |
| Respiration rate (RR) | Within the age adjusted norms |
| Polysomnogram (PSG) (performed with trach open) | |
| Apnea-hypopnea index (AHI) | < 5 events/hour (lower threshold for NMD than for obstructive processes)[40] |
| TcCO2 | 35–45 mmHg |
| PetCO2 | < 50 mmHg or sleep-related or increase < 10 mmHg above the awake baseline[40] |
| Partial pressure of oxygen (PO2) | Not typically measured |
| Oxygen saturation (SpO2) | > 94%[40] |
| Respiration rate (RR) | Within the age adjusted norms |