Table 7. Characteristics of Patients who Died.
| Patient No. | NMD | Sex | Age* (yr) | Tracheostomy ( † ) | HMV time/day | Feeding | Hypoxia (n) § | Cause of death ( ¶ ) |
|---|---|---|---|---|---|---|---|---|
| 3 | GSD II | F | 6.0−6.2 | Y (N) | 24 | oral | 1 | RF (home) |
| 4 | SMA I | M | 2.1−6.7 | Y (G) | 24 | PEG tube | 1 | RF (U) |
| 8 | SMA I | F | 0.3−2.1 | Y (N) | 24 | NG tube | 2 | sepsis (home) |
| 13 | CM-MT | M | 0.3−3.3 | Y (N) | 24 | NG tube | 1 | U (U) |
| 17 | SMA I | M | 0.3−1.7 | Y (U) | 24 | NG tube | 1 | U (U) |
| 18 | SMA I | M | 0.6−4.0 | Y (U) | 24 | PEG tube | 1 | RF (home) |
| 22 | CM-FTD | F | 0.5−1.9 | Y (U) | 24 | NG tube | 1 | sepsis (home) |
| 24 | CM | M | 0.8−5.5 | Y (N) | night | oral | 3 | obstruction ǂ (home) |
| 27 | SMA I | M | 0.3−4.8 | Y (U) | 24 | PEG tube | 1 | T-tube obstruction (home) |
| 32 | SMA I | F | 0.6−1.7 | Y (U) | 24 | NG tube | 1 | sepsis (home) |
| 33 | SMA I | F | 0.7−3.3 | Y (U) | 24 | PEG tube | 2 | sepsis (home) |
| 35 | SMA I | F | 0.4−1.6 | Y (U) | 24 | NG tube | 1 | RF (home) |
| 56 | CM-FTD | F | 0.4−0.7 | N | ≥12 | NG tube | 7 | aspiration (home) |
* Age at start of HMV−age at death
† Tracheostomy-associated complication (N, no abnormal finding; G, granulation; U, not evaluated or unknown)
§ Post-HMV episode of hypoxia, including episode that caused death
¶ Where event occurred
ǂ Sudden airway obstruction other than tracheal tube obstruction
NMD = neuromuscular disease; HMV = home mechanical ventilation; GSD = glycogen storage disease; RF = sudden respiratory failure; SMA = spinal muscular atrophy; PEG = percutaneous endoscopic gastrostomy; U = unknown; NG = nasogastric; CM = congenital myopathy; MT = myotubular; FTD = fiber type disproportion