Amyotrophic lateral sclerosis and mechanical ventilation: experience in a pulmonology outpatient clinic

Amyotrophic lateral sclerosis(ALS) is a progressive neurodegenerative disorder that affects respiratory muscles. Respiratory failure is the most common cause of death, and usually happens in 3 to 5 years. It's very important to give support for respiratory function, by mechanical ventilation(MV), which can improve quality of life and survival. A restrospective, descriptive study was conducted between 2021 and 2023, in a follow-up of all ALS patients. Clinical data, pulmonary function test and level III sleep study were obtained. We collect information about prognosis and treatment. 69 patients, 51% male, with a mean age of disease onset 59,4820;85 years, and most patients presented with spinal symptoms. 92,8% initiated noninvasive mechanical ventilation(NIMV) in a sleep lab, and the principal criteria for initiation were symptoms(55%). During follow-up, 21,7% initiated mouthpiece ventilation(MPV), which is an option for daytime ventilation when there is disease progression. 43,4% were submitted to percutaneous endoscopic gastrostomy(PEG). Time from diagnosis to death was 44±40 months(Fig.1). Every patient was accompanied by palliative care since the diagnosis. ALS patients usually die from consequence of respiratory failure. NIMV can provide symptomatic relief and prolong survival. Further studies are needed to establish the most adequate timing for the initiation of NIMV, to obtain the maximum benefit.