Starting and stopping the ventilator for patients with amyotrophic lateral sclerosis

Only a minority of patients who have ALS require, request, and receive assisted or supported ventilation. Usually, when a mechanical ventilator is needed, nonsurgical methods can be used for prolonged periods of time. Appropriately timed discussions can reduce the need for emergency management of breathing failure. The doctrine of informed consent applies to decisions about life support. It involves both the physician (to exercise clinical judgment on behalf of the patient) and the patient (to make personal decisions). They must interact. The patient's firm decision must be clear but need not be in the form of a "living will," and it does not need to be sought repeatedly or reiterated endlessly. Just as a considered decision cannot be arbitrarily overthrown in a time of crisis, neither can a change of mind be willfully ignored. In practice, this may test the capability of even the most experienced and understanding physician, and may result in less-than-ideal outcomes, as our examples show. As in any other area of medical practice, personal experience teaches valuable lessons. Unfortunately, even extended publications discussing clinical management of ALS have failed to address the subject of discontinuing ventilatory support, and ethicists have not always been helpful. Bernat and Beresford have, however, successfully summarized the ethical issues involved. Failure to sustain breathing mechanically or withdrawing artificial support of breathing from a requesting patient who, in the terminal stage of ALS, has become unable to breathe without a mechanical ventilator cannot be called assisted suicide, mercy killing, or either passive or active euthanasia. It is allowing a competent person to die naturally of the incurable illness that afflicts him. The state has no legal interests to be served by intervening in the process just described, which bears no relationship to issues of malpractice, much less to criminal negligence or homicide. Neurologists have not uniformly understood these points, as demonstrated by previous publications addressing the issue and by the findings of our own survey of neurologists who have special experience in the area of neuromuscular diseases. In regard both to starting and to stopping the ventilator, we believe strongly that it is time to lay aside the moral, legal, and ethical conflicts that have needlessly delayed or prevented physicians from complying with the resolute decisions that competent patients have made about their own lives. We urge doctors to act in these cases, as in all others, with their best medical judgment.(ABSTRACT TRUNCATED AT 400 WORDS)