Initiation and Withdrawal of Invasive Ventilation for Patients with Amyotrophic Lateral Sclerosis: A Narrative Literature Review

Abstract

Decisions regarding invasive ventilation with tracheostomy (TIV) in patients with amyotrophic lateral sclerosis (ALS) involve serious ethical issues. Cultural differences in the attitudes of patients, caregivers, and physicians toward TIV initiation and withdrawal decisions have been analyzed based on a narrative review approach, comparing the situation between Japan and the U.S. Three main issues were identified regarding the implementation of TIV. The first is the lack of Advance Care Planning. Second, some patients may choose TIV based on the wishes of their physicians or caregivers, even if the patients themselves do not want TIV in the Japanese context. Third is the influence of patient associations, which advocate for the protection of patients’ rights. Next, this study identifies the following issues related to TIV discontinuation. The main concern here is cultural differences in legislation and ethical intuitions regarding the discontinuation of TIV. The treatment guidelines for patients with ALS advise physicians to reassure patients that TIV can be withdrawn at any point. However, TIV withdrawal is not explicitly discussed in Japan. Moreover, Japanese ALS treatment guidelines state that ventilation withdrawal is currently impossible, due to a lack of legal support. Most Japanese physicians have told patients that they are not allowed to stop ventilation via such a request. Unlike in the U.S., withholding and withdrawing ventilators are not ethically equivalent in Japan. In conclusion, the decision-making process regarding TIV is difficult, not only for the patients and caregivers, but also for physicians. Even if patients are legally entitled to refuse unwanted treatment, there have been cases in which Japanese physicians have felt an ethical dilemma in stopping TIV for patients with ALS. However, few studies have investigated in detail why physicians oppose the patient’s right to discontinue TIV in Japan.