[Mechanical ventilation for amyotrophic lateral sclerosis--Making a comparison between hospital and home care]

In Japan, the traditional method of mechanical ventilation (MV) used for patients with amyotrophic lateral sclerosis (ALS) has been positive pressure ventilation with tracheostomy (PPV). Survival after starting PPV can be for many years. The purpose of our study is to report our findings on 25 patients (22.5%) using PPV for thirteen years. A total of 91 ALS patients without PPV died during in past thirteen years. Four patients with PPV lived at home and their families usually served as primary caregivers. Other five patients with PPV lived in our hospital, because they were lacked willing, competent or available caregivers at home, and they had not sufficient informed consent about use of mechanical ventilation or not. The quality of MV care provided by properly trained family members and home helper was as good as care provided by home-visited nurse. Quality of life in ALS patients with home MV were significantly increased comparing to hospital MV. Few patients chose nasal intermittent PPV. Most patients with ALS do not want to use MV. They need assistance with palliative care and planning for emergencies, so that PPV can be avoided when respiratory failure occurs. Before a decision is made to use PPV, the patient with ALS and family should understand that death related to ALS can be prevented with home MV and good care. However, if PPV is used, ongoing tracheal suctioning will be required, immobility will progress, or needed resources and cost are high, with the result that family might be burdened with heavy responsibility. Thorough informed consent are necessary.