Table 6. Characteristics of Patients with Successful NIV.
| Characteristic | Patient No | |||||||
|---|---|---|---|---|---|---|---|---|
| 12 | 14 | 31 | 34 | 39 | 47 | 59 | 64 | |
| NMD | SMA II | CMD-MN | SMA II | CM | CMD-Ullrich | CM-type I FA | SMA I | SMA I |
| Sex | F | M | F | F | M | F | F | F |
| Age* (yr) | 2.6 | 13.2 | 2.8 | 10.1 | 9.4 | 5 | 1.2 | 0.7 |
| Weight* (kg) (percentile) † | 9.4 (<3p) | U | 10.7 (<3p) | 34.6 (50−75p) | 46.1 (90−97p) | 8.2 (<3) | 8.1 (<3p) | 6.4 (3−10p § ) |
| Planned HMV | N | Y | N | N | N | Y | Y | Y |
| HMV time/day (hr) | ≥12 | <12 | night | night | night | night | 24 | night |
| Use of MIE | N | U | Y | N | Y | Y | Y | Y |
| Hypoxia ¶ (n) | 1 | 0 | 0 | 0 | 0 | 0 | 2 | 2 |
| Feeding | oral | oral | oral | oral | oral | oral | PEG tube | NG tube |
| HMV duration (yr) | 9.7 | 7.3 | 4.2 | 4.1 | 3.0 | 2.0 | 0.6 | 0.3 |
* At the start of HMV
† Body weight percentile for age
§ Body weight percentile for the corrected age
¶ Episode of hypoxia that occurred during home NIV
NIV = noninvasive ventilation; NMD = neuromuscular disease; SMA = spinal muscular atrophy; CMD = congenital muscular dystrophy; MN = merosin-negative; CM = congenital myopathy; FA = fiber atrophy; HMV = home mechanical ventilation; MIE = mechanical in-exsufflator; U = unknown; PEG = percutaneous endoscopic gastrostomy; NG = nasogastric