From: Comprehensive approach to weaning in difficult-to-wean infantile and juvenile-onset glycogen-storage disease type II patients: a case series
Months of ERT (28 days/month)
Proportion of ventilator time
MRC scale
Invasive BIPAP%
NIV %
UL
LL
0.0
100
0
4-
0.5–4.5
4.5–5.5
4
>4.5a
4+