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Respiratory Management
NIV is CPAP and BiPAP not used for noninvasive ventilatory support (NVS), NVS settings are PIPs of about 20 cm H2O and no EPAP/PEEP. Since we have ALS patients iwth 90% of normal VC who are 24/7 NVS dependent and others with 7% of VC with normal blood gases and asymptomatic without vents, nasal NIV recommendations for VC under 80% or 50% are inappropriate and should normally be based on supine VC anyway and not the sitting VC of PFTs. Most of our MND patients become NVS dependent without ARF or even going to a hospital, not to mention our 18 with SMA1 between 18-30 years old with 0 ml VC and no trach and many DMDs over age 50 who have never been to a hospital. Use NVS not polysomnographic titrations that don't adequately rest muscles during sleep.
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