Dr. Bernat is professor of neurology and medicine at Dartmouth Medical School. He was the former chair of the AAN Ethics Law & Humanities Committee and is the author of Ethical Issues in Neurology, 3rd ed. (Lippincott Williams & Wilkins, 2008).
Q: A 62-year-old man with advanced amyotrophic lateral sclerosis (ALS) was admitted to the intensive care unit (ICU) with severe pneumonia treated with intubation and ventilation. The ICU staff determined that he had little chance for survival. Treatment orders included Do-Not-Resuscitate (DNR) but his wife insisted that all life-sustaining treatments be used. Given his poor prognosis, is it appropriate for the neurologist to discuss the costs of life-sustaining treatments with his wife?
DR. JAMES L. BERNAT: This situation raises several medical and ethical issues worthy of consideration. Accepted best-practice clinical guidelines provide that neurologists caring for ALS patients should discuss patients' preferences for life-sustaining therapy before they are needed by conducting so-called advance care planning. Earlier in the course of the illness, the patient's neurologist should discuss the eventual need to insert a gastrostomy feeding tube when pulmonary function declines to below a certain threshold. Additionally, neurologists should educate the patient about respiratory support options that will become necessary including noninvasive positive-pressure ventilation, such as BiPAP, and tracheal positive-pressure ventilation. His neurologist also should explain palliative care options if he refuses respiratory support at any time. The previously prevailing physician-centered decision-making model, in which neurologists determined which patients should be intubated, has been replaced by a patient-centered decision-making model in which informed patients make these decisions for themselves.
Despite the mild cognitive impairment often present in patients with advanced ALS, most ALS patients retain decision-making capacity throughout their illness. Therefore, the neurologist can discuss whether to initiate or continue life-sustaining therapy directly with the patient throughout the illness. Communication barriers resulting from paralysis and endotracheal intubation can be overcome with the assistance of occupational therapists providing letter boards or computer-assisted communication devices and respiratory therapists inserting fenestrated tracheostomy tubes that permit speech.
The opinion of a family member on whether life-sustaining therapy should be begun or continued is relevant but should not be permitted to overrule a capacitated patient's right to consent to or refuse therapies. Family members who consistently oppose a patient's decision require counseling to understand the reasons for their opposition. The capacitated patient's decision takes precedence is the case of a conflict with a family member. There are very few situations in which it is ethically justified for physicians to overrule a capacitated patient's refusal of life-sustaining therapy.
The patient's psychological state is important to assess. Severely depressed patients, especially those who feel hopeless and who believe that their lives are meaningless, are more likely to refuse life-sustaining therapy than other equally ill patients. Reactive depression is insufficient grounds for a physician to invalidate a severely ill patient's refusal of therapy but psychotic depression, although rarely present even in advanced ALS, may be grounds for psychiatric intervention prior to ordering extubation. Many ALS patients who refuse life-sustaining therapy state that they do not necessarily wish to die but are willing to die if it is the only means to be relieved of the burden of tracheal positive-pressure ventilation. Surveyed ALS patients consistently express the concern that they want to avoid being an emotional or financial burden on their loved ones.
In this case, we are told that the patient's chance for survival is very small. But it is ambiguous if that dismal prognosis refers to survival with or without continued tracheal positive-pressure ventilation. This point requires clarification. We are also told that the patient was made DNR but it is unclear if this order was written after discussing the matter with the patient, the family, or neither. Ideally, whether or not to undergo cardiopulmonary resuscitation (CPR) status is an important element of advance care planning that should be discussed with an ALS patient prior to its necessity. Given the patient's current endotracheal intubation and ventilation, a DNR order usually indicates withholding cardiac defibrillation, chest compressions, and vasopressor drugs.
At this point, the neurologist should engage in a conversation with the intubated patient, particularly if this discussion had not been conducted previously. Treatment options should be discussed including the current likelihood of weaning from the ventilator once the pneumonia has been treated, and the likelihood of successfully relying on noninvasive positive-pressure ventilation technologies such as BiPAP. To consent for current treatment, patients need to know whether they are likely to be able to be successfully weaned to BiPAP or they will require tracheal positive-pressure ventilation.
It is especially important for the neurologist to describe the provision of ideal palliative care to ease dying if the patient insists on extubation that is likely to result in his death. There are published guidelines for providing excellent palliative care during terminal ventilator weaning. These include the judicious administration of morphine to combat air hunger and of benzodiazepines to provide light sedation to make death free from suffering. Terminal ventilator weaning is usually conducted in the hospital but may be done at home if the necessary logistical support can be arranged through hospice.
The expense of continued medical care is a legitimate topic of discussion. Many physicians lack information on the actual costs of tests, treatments, and hospitalizations they routinely order. These financial data are important to patients and family members who must pay or share payment for them. Whether health insurance pays for home tracheal positive-pressure ventilation has been shown to be the most important factor in patients' decisions to have home ventilator therapy. But it would be wrong for physicians to wield the costs of care as a bludgeon to intimidate or coerce patients and family members into agreeing with the physician's decision.
Some physicians and nurses find it psychologically difficult to order extubation even after tracheal positive-pressure ventilation has been validly refused by an ALS patient. Other physicians also may be uncertain or fearful of medico-legal consequences when a patient dies after refusing life-sustaining therapy or believe that this activity violates their personal moral code. Some of these obstacles are amenable to education about clinical practice guidelines and others resolve with more experience in conducting this activity. But if a physician remains unwilling to order extubation after further tracheal positive-pressure ventilation has been validly refused by an ALS patient — on the grounds that it violates the physician's personal moral code — the physician should attempt to transfer the patient to another physician who is willing to carry out the patient's wishes. While it is true that physicians are not required to perform medical practices that they regard as unethical, their conscientious opting out of stopping therapy that a capacitated patient has validly refused should not deprive a patient of the right to refuse life-sustaining therapy.
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