Amyotrophic lateral sclerosis (ALS) is a motor neuron disease in adults that invariably results in respiratory failure unless prevented and treated with noninvasive or invasive breathing support and adequate ventilation is maintained.
 

Noninvasive ventilation (NIV) is the standard practice of breathing support for ALS.
 

NIV can be tolerated unless oral secretions become severe due to bulbar impairment (resulting in the progressive inability to swallow saliva).  Therefore, use of NIV is temporary, short-term treatment for those with bulbar ALS.
 

In contrast, tracheostomy and invasive ventilation (TIV) can be tolerated in people with ALS, despite the severity of oral secretions, and may extend survival well beyond respiratory failure. Thus, use of TIV in persons with ALS is referred to as long-term mechanical ventilation (LTMV). ALS itself is not fatal, if TIV maintains adequate ventilation and complications are avoided.
 

People with ALS who are nonbulbar (having the ability to talk, swallow and clear the throat) do not need tracheostomy and invasive breathing support.
 

The validated Oral Secretion Scale (OSS) predicts tolerance of NIV in people with ALS.  Survival correlates with NIV tolerance and hours per day that NIV is used.  The Oral Secretion Scale reliably signals when to initiate hospice or transition to tracheosotomy ventilation.
 

NIV users with nonbulbar ALS do not need a tracheostomy when upper-airway clearance is maintained.
 

Cazzolli PA, Brooks BR, Nakayama, Y, Lewarski JS, McKim DA, Holt SL, Chatburn RL.  The Oral Secretion Scale and prognostic factors for survival in subjects with amyotrophic lateral sclerosis. Respir Care 2020; 65(8):1063-1076.