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In an ALS patient with FVC 60–70% predicted, normal daytime ABG, and normal nocturnal oximetry, but persistent orthopnea and unrefreshing sleep, do you initiate nocturnal NIV — and if so, how do you navigate the CMS coverage gap?

3 Answers
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Pulmonology · University of Michigan Hospitals and Health Centers

In our assisted ventilation clinic, we lean toward symptom-based initiation for patients with neuromuscular disease, including ALS. There are several strategies for navigating the coverage gap.

We have found that performing both FVC and MIP measurements with supine positioning will often reveal that...

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Pulmonology · UCLA ALS Clinic

I follow the updated CHEST guidelines presented as part of the ONMEP TEP summary from 2021 (Gay et al., PMID 34339685). In patients with FVC <80% with symptoms (particularly orthopnea), I would initiate nocturnal NIV therapy with BIPAP-ST, AVAPS, or AVAPS-AE mode depending on the particular clinical...

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Pulmonology · University of Michigan

This patient with symptomatic orthopnea needs a supine forced vital capacity (FVC) and maximal inspiratory pressure (MIP) measurement. The rule for the 50% threshold for FVC is also applicable in the supine position and often catches the patients who are escaping the criteria in the upright position...

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