Use of volume-targeted non-invasive bilevel positive airway pressure ventilation in a patient with amyotrophic lateral sclerosis

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease in which most patients die of respiratory failure. Although volume-targeted non-invasive bilevel positive airway pressure (BPAP) ventilation has been studied in patients with chronic respiratory failure of various etiologies, its use in ALS has not been reported. We present the case of a 66-year-old woman with ALS and respiratory failure treated with volume-targeted BPAP ventilation for 15 weeks. Weekly data downloads showed that disease progression was associated with increased respiratory muscle weakness, decreased spontaneous breathing, and increased use of non-invasive positive pressure ventilation, whereas tidal volume and minute ventilation remained relatively constant.

Figure 1. Ventilator data for a 15-week period in an amyotrophic lateral sclerosis patient on bilevel positive airway pressure ventilation. Note the increased duration of daily use of ventilation with decreased ability to trigger breaths spontaneously (i.e., increased reliance on timed ventilator-delivered breaths) over time. As can be seen, minute ventilation remained relatively constant. *Ventilator support increased between weeks 8 and 9; week 10 reflects this increase for the first complete week.

Figura 1. Dados do ventilador durante um período de 15 semanas em uma paciente com esclerose lateral amiotrófica tratada com ventilação com dois níveis de pressão positiva nas vias aéreas. Nota-se o aumento da duração do uso diário de ventilação com a diminuição da capacidade de desencadear ciclos respiratórios espontaneamente (isto é, o aumento da dependência dos ciclos cronometrados gerados pelo ventilador) ao longo do tempo. Como se pode observar, a ventilação minuto manteve-se relativamente constante. *O suporte ventilatório aumentou entre a 8ª e a 9ª semana; a 10ª semana reflete esse aumento pela primeira semana completa.