Description of Positive Airway Pressure Settings in Bulbar Onset Amyotrophic Lateral Sclerosis Patients Requiring Non-invasive Ventilation

Document Type

Conference Proceeding

Publication Date

5-20-2025

Publication Title

Am J Respir Crit Care Med

Abstract

Introduction : Amyotrophic Lateral Sclerosis (ALS) in general and bulbar onset ALS patients in particular are at high risk of developing respiratory failure secondary to neuromuscular weakness. Optimal Non-Invasive Positive Pressure Ventilation (NIPPV) settings are not clearly defined in this subgroup. Methods : A retrospective chart review study was designed to describe NIPPV settings in bulbar onset ALS patients who met initial compliance with NIPPV. Bulbar onset ALS patients were identified from a database including patients diagnosed with Motor Neuron Disease at Henry Ford Hospital from January 1, 2019 to December 31, 2023. Results : A total of 45 patients with bulbar onset ALS were identified and 37 patients were initiated on NIPPV therapy with 26 patients (70%) meeting compliance requirements. Amongst the compliant patients, 14 patients were initiated on Bilevel Positive Airway Pressure - Spontaneous Timed (BPAP-ST) mode and 12 patients on Volume Assured Pressure Support (VAPS) mode. At compliance, mean Positive Airway Pressure (PAP) therapy usage for more than or equal to 4 hours per 24 hours and mean duration of PAP therapy use per 24 hours in the BPAP-ST and VAPS groups was 88% and 5.6 hours versus 81.5% and 5.7 hours respectively. In the BPAP-ST group, mean values of PAP settings and data were as follows: Inspiratory PAP (IPAP) 10.6 cm water, Expiratory PAP (EPAP) 5.7 cm water, backup rate 8.8 breaths per minute, tidal volume 447.3 ml, respiratory rate 17 breaths per minute, minute ventilation 7.5 liters per minute and Apnea Hypopnea Index (AHI) 5.4 per hour. In the VAPS group, mean values of PAP settings and data were as follows: target alveolar ventilation 4.2 liters per minute, back up rate 14 breaths per minute, minimum Pressure Support (PS) 4.6 cm water, maximum PS 13.9 cm water, EPAP 4.3 cm water, median IPAP 11.6 cm water, median EPAP 4.4 cm water, tidal volume 423.9 ml, respiratory rate 16.2 breaths per minute, minute ventilation 7.2 liters per minute and AHI 4.53 per hour. Conclusion : Both BPAP-ST and VAPS modes appear to be well tolerated in bulbar onset ALS patients requiring NIPPV. Further studies are required to analyze settings associated with NIPPV compliance in greater detail in bulbar onset ALS patients.

Volume

211

First Page

1

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