Purpose: The purpose of this study was to elucidate trends and current situations in end-of-life care for patients with amyotrophic lateral sclerosis (ALS) on non-invasive positive pressure ventilation (NPPV), who elect not to undergo tracheostomy positive-pressure ventilation (TPPV).
Methods: We reviewed the literature using several search methods. We initially searched journal articles published between 2001 and 2015 using the Japan Medical Abstracts Society (ICHUSHI) database and PubMed, using the search terms ALS and/or NPPV. We then searched books, guidelines, comments, or readings that address how to palliate respiratory distress, and other related articles using two search engines: Yahoo! JAPAN and Google Scholar. We conducted searches several times for ten months between January and October 2016. The search terms were ALS, non-invasive positive-pressure ventilation (NPPV) /NIV, withdrawing NPPV to death, terminal care, end-of-life care, and several combinations of these words.
Results: Thirty-four related original and research articles were analyzed. Palliative care during the end of life was addressed in two articles. Palliative care was described for 7 of 20 identified patients in books and journal articles. Furthermore, only a few guidelines and books were found to describe end-of-life care.
Conclusions: The number of patients who use NPPV is increasing. NPPV cannot palliate respiratory failure for patients with aggravated ALS. In our findings, palliative end-of-life care for such patients who choose to remain on NPPV as maximum medical treatment for respiratory failure has received little focus. Development of palliative care at the end-of-life for ALS patients on NPPV is needed.
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