Use of the Intermittent Abdominal Pressure Ventilation to guarantee speech in a tracheostomized Amyotrophic Lateral Sclerosis patient

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Passive versus active circuit during invasive mechanical ventilation in subjects with amyotrophic lateral sclerosis

BACKGROUND: Until recently, it has been considered essential to maintain the use of a double-limb circuit in patients with amyotrophic lateral sclerosis (ALS) to avoid rebreathing expired air during invasive mechanical ventilation. Currently, life-sustaining home ventilators can work with a single, lighter circuit that is easier to manage. Our aim was to evaluate the effectiveness and safety of a single-limb circuit with intentional leaks (passive circuit) in comparison with a circuit with an expiratory valve (active circuit), in subjects with ALS who use invasive home ventilation. METHODS: We conducted a retrospective single-center study. The enrolled subjects were divided into 2 groups according to the type of exhalation port. The aim of the study was to compare arterial blood gases; nocturnal oxygen saturation recordings; and the occurrence of adverse events, both clinical and technical events. In addition, we compared the rate of mortality and unplanned hospital admissions that occurred within a year after discharge from the hospital. RESULTS: Forty-three subjects were included in our study: 23 who used a passive circuit and 20 who used an active circuit. No significant difference in nocturnal and diurnal gas exchanges was detected. The incidence of adverse events was significantly higher in the active circuit group (85% in active circuit vs 30% in passive circuit, P < .001). However, by splitting the adverse events into 2 categories, clinical and technical, the technical events were significantly more frequent in the active circuit group. None of these events led to hospital admission or death. CONCLUSIONS: The passive circuit was shown to be as effective and safe as the active circuit during home invasive ventilation in the subjects with amyotrophic lateral sclerosis. A future randomized controlled study is necessary to confirm these results and to extend indications to other pathologies. Key words: mechanical ventilation; chronic respiratory failure; tracheostomy; respiratory therapy; neuromuscular diseases; amyotrophic lateral sclerosis (ALS). [Respir Care 2018;63(9):1132\u20131138. \ua9 2018 Daedalus Enterprises]

Optimizing the noninvasive ventilation pathway for patients with amyotrophic lateral sclerosis/motor neuron disease: a systematic review

Objective: To systematically review quantitative and qualitative literature on optimal provision of noninvasive ventilation (NIV) for patients with amyotrophic lateral sclerosis/motor neuron disease (MND). Methods: A systematic search of electronic databases, together with supplementary search methods was used to identify relevant literature from the last 20 years. Studies of any empirical design with an English abstract were eligible for inclusion. Data from documents meeting our criteria were extracted and synthesized using narrative and thematic synthesis. A patient pathway of care model was used to integrate data and provide a process perspective to the findings. Results: While the importance of individualizing care was highlighted, factors optimizing use for all patients include: specialized multi-disciplinary team service provision; determining need using respiratory function tests in addition to symptom report; providing adequate information for patients and their family; paying attention to the role of carers in decision-making; adequately managing secretions; considering the most advantageous place of initiation; optimizing the interface, machine mode, and settings for patient comfort and effectiveness; providing supportive interventions where appropriate; regular monitoring and adjustment of settings; and providing opportunities for ongoing discussion of patient wishes. Conclusions: Optimizing use of NIV in people with MND requires consideration of multiple factors as part of a process throughout the patient pathway. Current guidelines predominantly focus on the initiation of NIV and may underplay psychosocial factors. We have made evidence-based recommendations for each step in the pathway, which may help improve optimal uptake, usage, quality of life, and survival outcomes in patients with MND