Respiratory therapies for Amyotrophic Lateral Sclerosis: A state of the art review

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition noteworthy for upper and lower motor neuron death. Involvement of respiratory motor neuron pools leads to progressive pathology. These impairments include decreases in neural activation and muscle coordination, progressive airway obstruction, weakened airway defenses, restrictive lung disease, increased risk of pulmonary infections, and weakness and atrophy of respiratory muscles. These neural, airway, pulmonary, and neuromuscular changes deteriorate integrated respiratory-related functions including sleep, cough, swallowing, and breathing. Ultimately, respiratory complications account for a large portion of morbidity and mortality in ALS. This state-of-the-art review highlights applications of respiratory therapies for ALS, including lung volume recruitment, mechanical insufflation-exsufflation, non-invasive ventilation, and respiratory strength training. Therapeutic acute intermittent hypoxia, an emerging therapeutic tool for inducing respiratory plasticity will also be introduced. A focus on emerging evidence and future work underscores the common goal to continue to improve survival for patients living with ALS.

Optimizing breathlessness management in amyotrophic lateral sclerosis: insights from a comprehensive systematic review

“…Depending on individualized respiratory function, patients must use NIV for prolonged periods of time, ranging from 8 h/day (overnight while sleeping) up to 24 h/day [ 29 ]. Patients may initially require slightly longer time to adjust to adaptive ventilator modes, adherence is similar over the longer term [ 34 ].…”

“…These devices offer various modes such as bilevel, volume- or pressure-controlled breath delivery, either at specific times or coordinated with spontaneous efforts. Alternatively, they may employ threshold ventilation through adjustable, volume-assured pressure support [ 34 ]. Adaptive ventilator settings enable the device to detect and compensate for lapses in baseline ventilation, even in cases of progressive respiratory weakness [ 34 ].…”

Long-term mechanical ventilation: State of the evidence and future directions

“…The series on long-term ventilation is comprised of three narrative reviews, on the topics of respiratory therapies for people with neuromuscular disease, specifically amyotrophic lateral sclerosis (ALS), 1 transitions in care for ventilator-assisted individuals, 2 and palliation and end of life care in neuromuscular disorders. 3 This series highlights current practices and optimal collaborative care plans for ventilator-assisted individuals, current evidence and avenues for research directions in the area of long-term ventilation.…”

“…Sales de Campos et al 1 provide a comprehensive review of respiratory therapies for individuals with ALS, including non-invasive ventilation (NIV). The benefits of NIV are discussed including improved survival and quality of life.…”

“…It may, however, lead to more cost-effective, and smooth transition process and can be explored further as part of transition models. Sales de Campos et al 1 describe how there has been a shift towards less reliance on pulmonary function testing during the pandemic, in the initiation and monitoring of NIV. Elverson et al 3 described how the pandemic highlighted inequities in access to palliative care, especially for individuals with neuromuscular disorders and malignancies.…”