Amyotrophic Lateral Sclerosis and Noninvasive Positive Pressure Ventilatory Support

Abstract Many studies suggest a brief statistical benefit on survival and quality of life by using nasal noninvasive ventilation for patients with amyotrophic lateral sclerosis and other neuromuscular conditions. Indeed, nasal noninvasive ventilation has become synonymous with continuous positive airway pressure and lo-span bilevel positive airway pressure. Nasal noninvasive ventilation, however, may not normalize CO 2 levels and continuous positive airway pressure and O 2 exacerbate hypercapnia and often lead to CO 2 narcosis, intubation, and ultimately tracheostomy or palliative care death. However, a third option can be to offer up to continuous noninvasive ventilatory support and extubation to it. Noninvasive ventilatory support can be effective for full, definitive ventilatory support, even for people with no measurable vital capacity, and has maintained classic amyotrophic lateral sclerosis patients for up to 12 yrs without resort to tracheotomies. Nineteen centers have reported 335 amyotrophic lateral sclerosis patients using continuous noninvasive ventilatory support instead of tracheostomy mechanical ventilation for an average of 14 mos (6 mos to 14 yrs). The noninvasive ventilatory support must also be used in conjunction with mechanical insufflation-exsufflation to clear airway debris and normalize or renormalize ambient air oxyhemoglobin saturation, both to avoid intubation and to facilitate extubation. People with amyotrophic lateral sclerosis satisfying specific criteria, even when continuously dependent on tracheostomy mechanical ventilation, can be decannulated and placed on continuous noninvasive ventilatory support with mechanical insufflation-exsufflation.