Abstract
Critical care management of acute respiratory failure in patients with neuromuscular disease (NMD) such as amyotrophic lateral sclerosis (ALS) is not standardized and is challenging for many critical care specialists. Progressive hypercapnic respiratory failure and ineffective airway clearance are key issues in this patient population. Often at the time of hospital presentation, patients are already supported by home mechanical ventilatory support with noninvasive ventilation (NIV) and an airway clearance regimen. Prognosis is poor once a patient develops acute respiratory failure requiring intubation and invasive mechanical ventilatory support, commonly leading to tracheostomy or palliative-focused care. We focus on this understudied group of patients with ALS without tracheostomy and incorporate existing data to propose a technical approach to the triage and management of acute respiratory failure, primarily for those who require intubation and mechanical ventilatory support for reversible causes, and also for progression of end-stage disease. Optimizing management in this setting improves both quality and quantity of life.Neuromuscular patients with acute respiratory failure require protocolized and personalized triage and treatment. Here, we describe the technical methods used at our single institution. The triage phase incorporates comprehensive evaluation for new etiologies of hypoxia and hypercapnia, which are not initially presumed to be secondary to progression or end-stage neuromuscular respiratory failure. In select patients, this may involve intubation or advanced adjustments of NIV machines. Next, once the acute etiology(s) is identified and treated, the focus shifts: training and use of mechanical airway clearance to optimize pulmonary function, facilitation of NIV wean or successful extubation to NIV, and transition to a stable regimen for home ventilation. The comprehensive protocol described here incorporates multi-institutional approaches and effectively optimizes acute respiratory failure in patients with neuromuscular pulmonary disease.
Highlights
Acute respiratory failure in chronic neuromuscular disease (NMD)Respiratory failure is often an expected course and outcome in patients with NMD
We focus on this understudied group of patients with amyotrophic lateral sclerosis (ALS) without tracheostomy and incorporate existing data to propose a technical approach to the triage and management of acute respiratory failure, primarily for those who require intubation and mechanical ventilatory support for reversible causes, and for progression of end-stage disease
Unique NMD requirements should be met before extubation is considered, and these include: respiratory secretions are manageable with mechanical cough assist, SpO2 ≥ 94% without supplemental oxygen or that oxygen is being tapered off with FiO2 ≤ 40%, chest imaging showing improvement of acute pathology such as infiltrates, and patient is neurocognitively intact with stable cardiopulmonary status (Table 1)
Summary
Respiratory failure is often an expected course and outcome in patients with NMD. In this technical report, we focus on patients with chronic respiratory failure presenting with acute respiratory decompensation. Unique NMD requirements should be met before extubation is considered, and these include: respiratory secretions are manageable with mechanical cough assist, SpO2 ≥ 94% without supplemental oxygen or that oxygen is being tapered off with FiO2 ≤ 40%, chest imaging showing improvement of acute pathology such as infiltrates, and patient is neurocognitively intact with stable cardiopulmonary status (Table 1). If these criteria are met, we recommend extubation directly to NIV utilizing high pressure support and back up respiratory rate. Outcomes, including survival, frequency of exacerbations, length of hospital stay and quality of life, are not well-described, one study suggested that MIE may reduce cough-augmentation treatment time by 17 minutes when added to the standard airway clearance algorithm [20]
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