Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation.

People with amyotrophic lateral sclerosis (ALS) usually die from respiratory failure unless they use mechanical ventilation (MV). Many die of respiratory failure without being adequately informed about the available options, such as MV that can provide symptomatic relief and prolong survival. The traditional method of MV used for persons with ALS has been tracheostomy-intermittent positive pressure ventilation (IPPV). However, the advent of nasal-IPPV has provided a new option for relieving respiratory symptoms and prolonging survival among selected individuals. The ALS Association Data on 75 ALS patients using MV is reviewed. Twenty-five patients used nasal-IPPV, all started electively. Survival with nasal-IPPV ranged from 6 to 64 months for non-bulbar patients, or until the onset of severe bulbar dysfunction when nasal-IPPV no longer was effective. Fifteen of these non-bulbar patients used nasal-IPPV from 20 to 24 h daily; one of these patients used nasal-IPPV continuously for 24 h daily for 24 months. One hundred percent of the users indicated they were glad they chose nasal-IPPV. In contrast, 50 have used tracheostomy-IPPV, usually as a result of emergency hospitalization without advance decision making. Twenty-five patients (50%) lived in a sub-acute skilled nursing facility (SNF) and only 18 of these (72%) were satisfied with their quality of life. Patients using tracheostomy-IPPV with good care are able to live many years: 27 of the 50 (54%) are still living, including one patient who is still living after 14 years of MV. In conclusion, home mechanical ventilation with nasal or tracheostomy-IPPV are options for selected people with ALS. Nasal-IPPV offers may advantages; it was only used when MV was planned and desired. Nasal-IPPV can be used unless bulbar impairment is severe.