Comparison of statements from “Consensus Statement for Standard of Care in Spinal Muscular Atrophy”8 to the surveyed participants’ responses toward different respiratory support statements for SMA type I.
| Consensus statement Recommendation | Statement asked in the survey | Consultants | Others | ||||
|---|---|---|---|---|---|---|---|
| Disagree | Neutral | Agreed | Disagree | Neutral | Agreed | ||
| n (%) | |||||||
| “If noninvasive ventilation fails, nonsitters may be intubated and mechanically ventilated as a short-term measure” | “In acute exacerbation, endotracheal intubation is acceptable for SMA type I patients” | 9 (45) | 4 (20) | 7 (35) | 7 (29) | 5 (21) | 12 (50) |
| “If noninvasive ventilation was needed during an acute illness, home noninvasive ventilatory support should be considered.” | “Chronic home Ventilation is acceptable for SMA type I patients” | 8 (40) | 4 (20) | 8 (40) | 8 (33) | 6 (25) | 10(42) |
| “In nonsitters with frequent respiratory infections, tracheotomy and ventilation can be considered but may not improve quality of life or decrease hospitalizations” | “Treating team should offer tracheostomy for SMA type I patients” | 12 (60) | 3(15) | 5 (25) | 9 (37) | 3 (13) | 12 (50) |
| “In nonsitters, care without ventilation support is an option if the burden of management outweighs benefit. Noninvasive ventilation may be used palliatively to facilitate hospital discharge and reduce work of breathing.” | “Will you consider withholding Mechanical Ventilation from SMA type I patients?” | 8 (40) | -------- | 12 (60) | 16 (67) | -------- | 8 (33) |