Keywordsamyotrophic lateral sclerosis, de-efferented state, dementia, end-of-life care, minimally conscious state, persistent vegetative state
With the advent of increasingly sophisticated technology and more powerful pharmacologic agents, it has become possible to prolong the last stages of life in patients with severe and irreversible medical disorders, in whom a long-term favorable outcome cannot be achieved. In some instances, the futile use of sophisticated technology to maintain certain bodily functions in individuals with no prospect of useful recovery has involved major financial outlay, an added burden on patients in advanced stages of incurable disease, and unnecessary and prolonged emotional distress to relatives and friends. Indeed, impersonal advances in modern medicine have led to increasing indignity and fear in many individuals as the end of life draws near, and some will die with unrelieved and uncontrolled symptoms.
Neurologists are often required to look after patients at the end of life either because a primary neurologic disorder reaches a terminal phase or because patients with primary medical disorders develop a catastrophic neurologic complication leading to consultation. The ability to recognize the last hours or days of life, that is, to diagnose “dying,” is an important clinical skill that is a prerequisite to the provision of appropriate care. Such patients are usually obtunded, able to take only sips of fluids, unable to take oral medications, and unresponsive to modifications in their therapeutic regimen; there may be no obvious reversible precipitant of their deterioration. Patient management is optimized when there is agreement between members of the health-care team that the patient is indeed dying. In this circumstance, it is important to avoid giving conflicting messages to patients and their families about the likely course of events.
It is the responsibility of all physicians to provide adequate care for patients who have a terminal disease or are dying. The focus of care changes from attempts to reverse or cure an underlying disease to providing compassionate and sensitive care for patients facing imminent death. Such care may be required for no more than a few hours or for as long as several months, depending on the nature of the underlying disorder. Patients or their surrogates will need to be informed of the severity of their disease, and—depending on the circumstances—this is generally best accomplished gradually and with sensitivity, bearing in mind what they do (and do not) want to know. Patients want hope and reassurance—not necessarily that they will live but that there are options to help their isolation, fear, physical discomfort, and mental distress. Physicians must assume responsibility for helping patients to deal with the bleak uncertainty of the future and to cope with the sense of isolation and dependency that is often experienced with the approach of death. Fears concerning pain or discomfort can often be alleviated by proper explanation, and supportive care by family, friends, and medical attendants may help to diminish otherwise overwhelming feelings of isolation and uncertainty. Some physicians still feel a certain guilt or responsibility for the death of patients, and this colors their approach to patients who are dying. Such attitudes will change only with the wider appreciation that death is but a natural milestone.
With the increasing emphasis on the scientific aspects of modern medicine, the art of the discipline—its human side—is often overlooked. Physicians must be prepared to spend time with their patients to provide support, education, and adequate symptomatic relief and to work with them and their family to enable them to come to terms with events. Unfortunately, however, such discussion and resultant decision-making substantially in advance of death are uncommon. The demands on physicians by patients may be considerable as death approaches. It is especially important for physicians to understand the needs of patients who are unable or unwilling to communicate with them. Patients need to know that they will continue to receive support and care from their physician throughout the process of dying. A cursory glance at patients during the course of a busy ward-round is quite inadequate in this regard. The family, also, may require considerable attention from physicians. This requires a sensitivity to their needs and the ability to communicate fully with them at all times, providing adequate responses to their queries; meeting with them after death has occurred often helps to settle unresolved fears and concerns. The care of terminally ill patients usually requires the cooperation of the entire health-care team, including nursing staff, social workers, physical and occupational therapists, dieticians, and psychotherapists, as well as physicians. The spiritual needs of the family must not be overlooked, and the clergy have an important role in this context.
In many countries, the general public has expressed increasing dissatisfaction with the care received by people who are dying. Fueled by concerns about the quality of end-of-life care, support has grown for the concept that patients with terminal illnesses should have the right to request assistance in dying. The extent to which physicians can assist in this regard is governed by national statutes, and the extent to which physicians choose to become involved in this context is personal and individual. Regardless, many individual physicians support the legalization of medically assisted suicide and voluntary euthanasia, although numerous professional organizations (including the American Academy of Neurology ) are opposed to such an approach. Medically assisted suicide involves providing the means for a patient intentionally to kill himself or herself, whereas voluntary euthanasia consists of the deliberate killing of a patient at his or her request. The provision of appropriate palliative care for dying patients would make such radical alternatives unnecessary in many instances. Pain relief, for example, must be adequate even if, as an unintended side effect, the medications used to control pain lead to some shortening of life in consequence.
Ethicists and educators have emphasized patient autonomy in the decision-making process. However, in a study of 8,308 hospitalized patients, 67 percent ultimately preferred to leave medical decisions to their doctor, even though 97 percent wanted their physician to offer them therapeutic choices and consider their opinions. Thus, both patient autonomy and physician beneficence remain important in guiding clinical care. Some patients with advanced medical disease may seek a particular therapeutic intervention that seems unjustified to their physician. Clinicians should behave in a manner that does not conflict with their own beliefs; if differences with patients and their families cannot be resolved by discussion and explanation, it is sometimes worthwhile to transfer patients—with their consent—to the care of another physician. Physicians have the responsibility of withholding treatment when the distress or other adverse effects of therapy cannot be justified by unrealistic hopes of curing or arresting the underlying medical disorder and of withdrawing life-prolonging maneuvers when these have become too burdensome for patients.
Competent adults have the right to refuse medical treatment or to request its discontinuation. Despite this, many patients dying in hospital continue to receive unwanted interventions such as intensive life support that postpone death. Patients’ refusals of supportive measures are not necessarily indicative of a wish to die: rather, they often signify simply a wish to do without the burden of life-sustaining medical treatment. The goals of such treatment must constantly be reappraised by patients, relatives, and medical staff. Even routine procedures such as radiographs, blood tests, and respiratory care may become unwelcome and tiresome interruptions during the last days of life, and the need for them must be re-evaluated as the patient’s condition deteriorates. When patients do refuse treatment, however, physicians are responsible for ensuring that this is not an impulsive decision but is based on an understanding of the implications of such a choice.
For patients to make informed decisions, it is important that they are properly educated by their physician about their options. They should also be encouraged to use advance directives such as a living will to ensure that their own wishes regarding medical intervention are followed if they later become unable to express their preferences because of physical or mental limitations. A durable power of attorney for health care in the United States gives another person the power to make medical decisions for the patient as necessary. It is important that physicians educate patients about such opportunities and the ability to make advance directives. Clinicians must also properly inform proxy decision-makers about the patient’s disorder, therapeutic options, and prognosis. They are generally expected to make decisions based on what the patient would have wished, and this assumes some knowledge of the patient’s preferences (the substituted judgment standard). Without such knowledge, the proxy can only balance the benefits of any treatment or course of action against the possible burdens (the best interests standard). When the perceived benefits are greater, consent is provided; if the likely burdens exceed any potential benefit, consent is refused.
However, there is a discrepancy between theoretical ethical concepts and clinical reality. A study of dialysis patients revealed that many would allow their designated surrogate to override their advance directives if this was in their interests. Subjects varied greatly in how much leeway they would give surrogates to override advance directives: “no leeway” was given by 39 percent, “a little leeway” by 19 percent, “a lot of leeway” by 11 percent, and “complete leeway” by 31 percent. Another study found that many elderly or seriously ill hospitalized patients (more than 70%) would not want their stated resuscitation preferences to be followed if they were to lose decision-making capacity but preferred that their family and physician make such decisions for them. It seems that proxy decision-makers have the discretion to assess novel and perhaps unforeseen circumstances, make moral judgments, and sometimes make decisions that differ from the patient’s original intent; for example, because the patient’s wishes cannot be applied in certain clinical contexts.
When patients do not have the capacity to make clinical decisions and have no designated surrogate, many states allow a next-of-kin surrogate to be appointed. Decisions by both patient-designated and next-of-kin surrogates are prone to error. A systematic literature review and analysis of 16 eligible studies involving 151 hypothetical scenarios and 2,595 surrogate–patient pairs, which collectively analyzed 19,526 patient–surrogate paired responses, showed that surrogates predicted patients’ end-of-life treatment preferences with only 68 percent accuracy. Surrogates were least accurate in scenarios involving stroke or dementia. Surprisingly, accuracy was not improved when the patient had designated the surrogate or by prior discussion between patients and surrogates about treatment preferences. Nevertheless, surrogates were more accurate than physicians in predicting patients’ treatment preferences.
To better understand the moral obligations of the patient–proxy relationship, Fins and colleagues surveyed 50 patient–proxy pairs and 52 individuals who had been proxies for someone who had died. They used structured vignettes of three separate diseases (one of which was acute stroke), and examined whether respondents believed that proxies should follow explicit instructions and thus act contractually regarding life-sustaining therapy or whether more discretionary (or covenantal) judgments were acceptable. Other variables included the “valence” of initial patient instructions (e.g., “to do nothing” or “to do everything”) and the quality of information available to the proxy. They found that the patient–proxy relationship exists on a contractual to covenantal continuum and that disease course, the clarity of prognosis, instructional valence, and quality of patient instructions led to response differences. The use of interpretative or covenantal judgment was desired by patients and proxies when the prognosis was grim, despite initial instructions to pursue more aggressive care. When hopeful feelings modified initial negative instructions, proxies were more uncertain about the propriety of doing nothing. Nonetheless, patients and proxies intended that negative instructions to be left alone should be heeded. Respondents did not adhere to narrow notions of patient self-determination but made nuanced and contextually informed moral judgments that were not viewed by patients or proxies as violations of the principal’s autonomy. Although respondents were better educated than the general public and were restricted to self-identified, English-speaking Americans of European origin (to avoid the possible confounders of race and ethnicity), these data suggest that advance-care planning should consider both the exercise of autonomy and the interpretative function assumed by the proxy.
Studies indicate that patients would like to discuss advance directives earlier than their physicians and before they are “extremely ill” ( Fig. 62-1 ). Most believe that such discussion should occur when they are still healthy. Both patients and clinicians believe that it is the physician who should initiate the discussion ( Fig. 62-2 ), but physicians are often reluctant or fail to do so. Many patients believe that others (e.g., a spouse or “significant other,” children, or parents) should be brought into the discussion. Most also believe that it should occur before hospitalization and over several visits.
Many healthy subjects consider certain neurologic states to be unacceptable and express the belief that they would not want to remain alive in those circumstances. However, after developing such disability, some patients change their opinion and may experience a better quality of life than previously envisioned. This possibility must be borne in mind when statements made by a patient before their illness are considered.
Decisions about whether to adopt life-sustaining measures often need to be made in a context where the prognosis is uncertain. It is important to note, however, that such decisions can be changed later as the prognosis becomes clearer because no logical distinction exists between withholding and withdrawing life-sustaining measures.
For patients nearing the end of life, decisions have to be made about whether cardiopulmonary resuscitation should be attempted, if the need arises. In patients with terminal diseases, such decisions often bear more on the issue of how a patient will die than on whether death will occur, and it is important for patients and family members to understand this distinction. A decision by a patient (or family members) needs to be made in advance, so that individual wishes can be taken into account in the event that resuscitation is required. In many European countries, physicians take an active role in advising patients and family members about the most appropriate course of action to follow, whereas many physicians in the United States believe that this is an individual decision that is best left to the patient. It is this author’s view that the physician—as the most informed person in the health-care team—should be responsible for ensuring that the patient or surrogate is sufficiently well educated about the issues to make informed decisions, guided by suggestions or recommendations when these are requested of the medical staff.
It is often helpful to discuss the possibility of organ donation with dying patients when this is a significant consideration. This makes it easier for family members, who may otherwise have to be approached about the issue shortly after the death of their relative. A variety of ethnic, religious, and cultural factors affect attitudes concerning organ donation, and these must be respected. Physicians must appreciate that patients are not obliged to donate organs and may choose to limit any donation that they make.
Provision of Care
With their increasing emphasis on surgery and advanced technology, hospitals no longer are able to provide for the long-term care of patients who are dying. By contrast, the needs of the terminally ill are often met more fully in the environment of a hospice, where a multidisciplinary team can focus on their care and support, or at home, if an adequate family structure and medical/paramedical support are available. Care for patients in a home environment requires careful education of the family, reduction of probable causes of stress (such as violence, agitation, or incontinence), support of caregivers by ensuring assistance from other ancillary health-care providers, and provision of periodic respite to the family. The interests of patients nevertheless remain the prime concern. When family members appear preoccupied more with their own welfare than that of a severely demented or dying patient, the possibility of patient abuse may require consideration. Abuse of the weak or elderly is becoming an increasing problem and a matter of grave public health concern.
The care of terminally ill patients should be focused on improving the quality of life rather than simply on extending the duration of life. This aspect has in general been neglected until recently and still receives relatively little attention in the clinical literature or medical school curricula. Palliative medicine constitutes an important emerging medical specialty in which the focus is on the quality of life for patients with advanced and progressive disease. The aim is to ensure that patients are as comfortable as possible, even during the process of dying. Curative treatment is not provided for disorders that do not cause discomfort, even if this leads to some shortening of life. Such an approach, traditionally associated with the care of patients with terminal cancer, is also relevant in the management of patients with a number of neurologic and general medical disorders, such as amyotrophic lateral sclerosis, severe cerebrovascular disease, locked-in syndrome, or advanced dementia. The emphasis is on the control of symptoms. Pain is the most common symptom in dying patients. Pain, discomfort, distress, fatigue, dyspnea, anorexia, nausea, vomiting, cognitive disturbances, and affective disorders all require particular care. When the ability of patients to communicate is impaired, it is necessary to infer the presence of distress by such signs as agitation, restlessness, tachypnea, and tachycardia and to initiate treatment accordingly. Many patients fear that any later inability to communicate their distress will lead to unnecessary and increased suffering. The relatives of dying patients frequently complain that signs of distress were ignored in patients unable to communicate their needs verbally. No justification exists for limiting symptomatic measures in such circumstances; overtreatment is preferable to undertreatment.
Opioids have an important role in providing pain relief when nonopioid analgesia is inadequate. Morphine is also particularly useful in relieving dyspnea. The optimal dose is that providing adequate relief. When it is introduced, morphine is best started in an immediate-release formulation. A starting dose of 5 to 10 mg every 4 hours is often adequate, but higher doses may be needed if patients have previously received another opioid, such as dihydrocodeine. A double dose at bedtime may also be helpful. Rescue supplements of morphine are necessary for “breakthrough” pain. The total daily dose of morphine should be titrated upward rapidly (e.g., by 30 to 50%) if pain relief is inadequate. Once pain is controlled, a modified-release preparation of morphine can be used. Side effects of morphine, especially nausea and constipation, may require concomitant treatment. Delirium resulting from opioid treatment may respond to substitution with an alternative opioid. It may be necessary to administer opioids by a nonoral route in patients with severe vomiting or dysphagia, those with intestinal obstruction or malabsorption, or those who are unable to comply with an oral regimen. Subcutaneous infusion of diamorphine may be used for pain control in dying patients. Anxiety, agitation, or delirium may respond to benzodiazepines or neuroleptic agents. In one study of critically ill patients, it was found that large doses of sedatives and analgesics were administered to relieve pain and distress during the withholding or withdrawal of life-supporting measures, but that death was not hastened by such medication. Supportive therapy is important. Attention to the environment may be rewarding. The intensive care unit, with its harsh light, endless noise, monitoring and other equipment, and frequent alarms, is an overwhelming place that provides no respite for dying patients.
Withdrawal of ventilator support is often particularly distressing for both relatives and medical staff, even though this is ethically no different from limiting other therapeutic or supportive measures. Ventilator withdrawal may be accomplished by extubation or by “terminal weaning” (in which ventilator rate, positive end-expiratory pressure, or tidal volume is gradually reduced with the endotracheal tube in place). Regardless of the method used, patient comfort must be ensured, as must the family’s perception in this regard. For conscious patients requesting sedation for ventilator withdrawal, Brody and colleagues recommend 2 to 4 mg of midazolam intravenously before withdrawal, and 5 to 10 mg of morphine by intravenous bolus for distress during weaning, followed by continuous infusion (50% of bolus dose per hour); in patients with tolerance to such agents because of prior use, higher doses are needed. Others have used pretreatment with the equivalent of 15 mg per hour of intravenous diazepam or 15 mg per hour of intravenous morphine, increasing the dose as necessary to relieve dyspnea and anxiety, even to the point of unconsciousness if required and requested.
Neuromuscular blocking agents should be discontinued before withdrawal of ventilator support, and indeed probably in all dying patients, to allow verbal communication when this is otherwise possible and to permit signs of distress to be manifest so that appropriate treatment can be provided. The masking of such signs by neuromuscular paralysis serves no useful purpose, and the inability to exhibit signs of distress in this context cannot be taken to imply that such distress is absent.
Attention must be devoted to nutrition, hydration, care of the skin, and sphincter function. The nutrition and hydration of dying patients require careful consideration. The points to consider include whether such support should be provided at all, the manner in which it is best provided, and patients’ tolerance of any resulting discomfort, complications, or side effects. There are benefits of withholding nutrition and hydration to shorten life when other treatment is to be discontinued. The artificial provision of nutrition and hydration is a life-sustaining maneuver that does not differ from other maneuvers that supplement or substitute for normal bodily functions. Withdrawal of artificial nutrition and hydration, however, has particular emotional implications for the general public and many clinicians. Such a course therefore demands specific discussion with patients (when communication is possible) and family members, as well as with other members of the health-care team who may be concerned. It is avoided by some clinicians who fear it will add to patients’ discomfort. In fact, however, it is unlikely to lead to much additional distress, and by shortening life may actually limit further discomfort. When distress does follow withdrawal of nutrition or hydration, additional symptomatic measures are necessary. Dryness of the mouth is often relieved by ice chips, glycerin swabs, or petroleum jelly lip balm; withdrawal of medication that may be exacerbating this symptom should also be considered. Mouthwashes may relieve poor oral hygiene, and antimonilial therapy is indicated for candidiasis, antiemetics for nausea, and opiates or benzodiazepines for restlessness or pain.
Thus, the focus is on comfort measures, and non-essential medications and interventions (such as blood tests, intravenous fluids, and the like) are discontinued. Relief of pain, agitation, respiratory difficulty, and other distressing symptoms is assured. There must be good communication with the patient, family, and primary medical practitioner, and the plan of care must be explained to them and understood. Attention should also be focused on religious and spiritual needs.
Various systems, such as the Liverpool Care Pathway in the United Kingdom, have been developed to facilitate care practices and monitor their results. The extent to which they improve care is not entirely clear, although some studies do suggest better outcomes, as by improved documentation of care and reduction in average total symptom burden among the dying.
An attempt has been made to determine the effectiveness of multi-component palliative care for residents of care homes for the elderly and to describe the range and quality of outcome measures, based on a review of existing studies. However, only three studies could be included, all conducted in the United States, and they had several potential sources of bias that rendered interpretation difficult. Further studies are therefore needed that focus on standard outcome measures, assess cost-effectiveness, and are less biased.