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Nocturnal Hypoventilation
- Formal diagnosis and NIV titration - PSG with
transcutaneous CO2 monitoring
- Depending on patient preference and underlying condition,
empiric NIV also acceptable
- Insurance requires:
- Neuromuscular diagnosis AND no COPD
- FVC <50% predicted OR
- MIP < -60 cm H2O OR
- Daytime pCO2 >45 mmHg OR
- Nocturnal desaturation <88% for >5 minutes
NIV improves survival in DMD
- 5-year survival improved from 8% (historical data) to almost
75% if patients started NIPPV when FVC <1 liter
Simonds et al, Thorax 1998;53:949–952
NIV improves survival in ALS
favor patients with less bulbar involvement
Bourke SC, et. Al. Neurology. 2006;61:171-177
NIV improves survival in ALS
Cohort Analysis – Bulbar patients actually benefitted more
Berlowitz, DJ et al. J Neurol, Neurosurg, Psychiatry 2016;87:280-286
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