Jahrbuch für Recht und Ethik [Annual Review of Law and Ethics], Band 4, Duncker & Humblot / Berlin, 1996, pp. 529-552.
Advance Care Planning and the ALS Patients:
A Cross-Cultural Perspective on Advance Directives*
__Because most ALS patients retain cognitive faculties and mental competence throughout the course of the disease, decisions about life-support for the ALS patient are ordinarily framed as issues of treatment refusal by competent patients, at least in Western culture. In the U.S., the rights of ALS patients as competent adults to refuse life support have been recognized in several court cases (Satz, Farrell, Culham).1 This may offer some explanation of why advance directive use among ALS patients has received very little attention compared to other patient groups. It does not diminish, however, the relevance of advance directives nor the broader importance of advance planning for end-of-life decisions. Discussion of advance directives fosters articulation of a more informed and comprehensive treatment plan that includes the patient's own choices for end-of-life care. In addition to promoting self-determination and control over the dying process, advance directives alleviate emotional and psychological burdens of family members who may otherwise face the choice of ventilatory support without knowing the patient's wishes; decrease physician uncertainty about patient's wishes; and offer greater assurance of compliance with the patient's wishes by health care professionals. With ALS patients, participation in decisions can be impaired or impossible despite intact cognitive abilities, for example when speech ability is lost and communication assistance devices prove unavailable or inadequate, or when the patient suffers from the totally locked-in syndrome ("TLS") in an advance stage of the disease. As illustrated by the Leach case,2 in which an ALS patient was hospitalized after suffering a stroke at home, the ALS patient is also susceptible to other illnesses and conditions that can rob him or her of the ability to decide. For all of these reasons advance directives can play a major role in planning ahead for important end-of-life decisions for the ALS patient.3
|II. The Nature of ALS|
__Most ALS patients die of respiratory failure or aspiration pneumonia, although mechanical respiratory support can prolong life through the entire progression of the disease to the TLS.5 Depending on the course of their illness, ALS patients may face decisions not only about mechanical ventilation, but also about other life-sustaining measures, including artificially provided fluids and nutrition, antibiotics and do not resuscitate (DNR) orders.6
|III. Developing A Care Plan:|
___The Physician-Patient-Family Relationship in the U.S., Germany and Japan
|A. The Physician-Patient-Family Relationship|
__The contemporary reign of autonomy which has emerged over the past approximately 25 years should be understood within the larger historical context from which it arose. Prior to autonomy's ascendance, Western medicine was grounded in the moral mission of the Hippocratic principle's directive "to benefit the patient according to the physician's ability and judgment".8 This paternalistic and "custodial" model assumed patients' trust in physicians' skill and judgment, and considered the "good" patient to be one who followed "doctor's orders" without question. The purpose of physician-patient communication was to assist the physician in diagnosis and treatment, not to foster or elicit an informed consent. In fact, the traditional physician-patient encounter has been more strongly characterized as a "history of silence".9 The "old" medical ethics has given way to the "age of patient autonomy", but paternalism and autonomy remain in tension.10
__From the perspective of informed consent analysis the physician-patient interaction has been framed largely in terms of the physician's duty to disclose information needed by the patient to make an informed decision (the way in which American law has framed the physician's duty) and the closely related ethical principle of truth-telling. As a general matter, U.S. physicians disclose to their patients the diagnosis of ALS, as well as information about the likely course of the illness. At least two studies of communication between ALS patients and their physicians support the understanding that most U.S. physicians disclose information about diagnosis, prognosis and treatment options to their ALS patients.11 However, the study by Moss and colleagues also found that significant numbers of patients were not adequately informed about treatment options for respiratory failure.12 One study found that a majority of ALS patients want to learn about their condition and to participate in decision making, though a significant number (30% in the Silverstein study) "preferred to leave decisions about care and treatment to the physician".13 Surprisingly, the Moss study found that 63% of patients did not want to know what the future would hold as the disease progressed. It should be noted that the two studies were conducted in the Northern Illinois and Chicago areas; thus generalizing these findings should be approached with a modicum of caution. We return to this data in the discussion of end-of-life choices and advance directives, below.
__German society shares in principle a commitment to patient autonomy. The ideas of autonomy and self-determination hold a prominent place in German law where the doctrine of informed consent is well-established (see below). Medical ethics and physician behavior, however, are more firmly embedded in paternalism than is the case in the U.S. Patient compliance within the context of a fiduciary relationship in which trust in the physician's judgment has a prominent role - the idea of "compliance-in-trust" - is an apt characterization.14 While many physicians disclose relevant information to their patients and seek patient consent, many others are less forthcoming in the disclosure and discussion of diagnosis, prognosis and treatment options. German physicians caring for ALS patients generally favor telling patients of their diagnosis, but are less willing to discuss the issue of long-term respiratory support. It is worth noting that there appears to be some difference in approach between physicians in West and East Germany. In the former East Germany medicine was and continues to be very paternalistic. Patients rarely question the authority of physicians. Physician paternalism is not the dominant model in West Germany, but appears to have a stronger hold on clinical practice than is the case in the U.S., at least with respect to decisions near the end of life.
__The typical physician-patient relationship in Japan is quite a different story. In the Japanese socio-cultural tradition persons are conceived as existing in a situation of "between-ness" or relatedness. Ningen , the Japanese term for person, is composed of two root Chinese characters that mean "human " "between-ness ". Within this conception of persons the individualistic Western notion at the heart of autonomy is subordinate. The relation between patient and doctor is strongly influenced by the concept of a fiduciary relationship (Shinrai-Kankei ), within which there is great deference to and trust in the professional expertise and role of the physician. "Due to traditional social attitudes, the Japanese have a tendency to trust and adhere to professional advice. Total dependency on those who have expertise is regarded as positive and good. As the Shinrai-Kankei should be respected, it is considered rude to raise questions which might be regarded as casting doubt on the judgment of the expert".15 Family members typically follow a similar pattern of commitment to Shinrai-Kankei. Most families prefer to leave care and treatment decisions, including the choice of artificial ventilation, to their physicians. This deference to physicians' judgment, together with a shared sense of the importance of maintaining hope, has meant that even if family members learned the nature of the disease or treatment, they were not likely to share this information with the patient. Thus, paternalism is dominant in the social and cultural climate of the country; a paternalistic medical tradition in which "doctor knows best" is the norm.
__On this understanding it is quite natural for Japanese physicians to follow a policy of non-truth telling (non-disclosure) with their patients. This has been particularly true of cancer patients and those with terminal illness. Beyond expected deference to professional judgment, many physicians believe that silence serves the patient's good, and that disclosure of a terminal illness, such as cancer, or of a very poor prognosis, will have a negative effect on the patient and cause the patient to give up hope. With experience caring for terminally ill patients over the past decade physician attitudes have been slowly changing. Nonetheless the following features continue to characterize the care and treatment of ALS patients: 1) Physicians in general do not convey the truth about the diagnosis of ALS; 2) ALS is usually classified as an incurable disease with a grim prognosis from respiratory failure; 3) typically, when ALS patients approach the point where they will need a respirator machine they are regarded as in the terminal stage of the disease;16 4) respiratory failure in ALS is considered synonymous with the termination of the disease; 5) a majority of physicians assign a "no cause, no cure, no hope" stigma to ALS. According to a report issued by the Ministry of Health and Welfare of Japan (MHWJ) in 1982, many ALS patients were supposed to be placed on ventilators without knowing the implications of this decision.17
__But there are also signs that the quality of the physician-patient dialogue is changing, that patients and families want more information, are becoming less willing to simply defer to physician expertise, and are less accepting of the use of aggressive measures to prolong life as long as possible. In the recent MHWJ survey (which did not specifically include ALS), 56% of family members thought that disclosing the diagnosis to the patient was appropriate. Twenty percent (20%) of family members believe that treatment to prolong life should be avoided, while 42% stated that it would be better if such treatment were not provided. Together, 62% expressed a negative attitude towards the use of technology to prolong the life of an ALS patient when death is imminent and the dying process painful. (MHWJ, 1 May 1995.) The experience at Tokyo Metropolitan Neurological Hospital (TMNH) for the past decade, where disclosure of diagnosis and prognosis to ALS patients before initiation of ventilatory support has been common practice, is a testament to changing attitudes about truth-telling. Among physicians caring for ALS patients at TMNH, respiratory failure is no longer viewed as the terminal stage of the disease. This physician group believes that care and treatment decisions, such as tube-feedings and respiratory support, should be made after telling the truth to patients and their family members. Informing only family members is considered to place an unnecessary burden on family members.
__The nature of the physician-patient relationship and the value attached to patient autonomy within that relationship plays a critical role in shaping that dialogue and allocating decisional authority. The long clinical course of ALS offers patients and families ample opportunity to develop an understanding of the disease, to share their thoughts and feelings, and to discuss their experience, concerns and options with the physician. As a consequence, there ordinarily is ample time to build a relationship of trust that fosters informed decision making. Physicians' own perceptions of ALS can powerfully influence the communication process. Reluctance to disclose news of an inexorable and "hopeless" illness is understandable, but should not be a barrier to conversation. Most importantly, physicians should strive to be objective in their presentation of information and opinions, and should be attentive to the ways in which their own negative attitudes towards quality of life or home ventilation can influence their choice of language, imagery and stories of experience with other patients. The opportunity to meet with other ALS patients can be especially valuable (a form of peer interaction), as it offers first hand information from the point of view of fellow sufferers about the benefits and burdens of daily life with serious disability, dependency, immobility, and use of respirator.18 A team that makes use of a coordinator/counselor in which patients and families are fully involved has been used successfully in Great Britain.19 National ALS associations, such as the Japan Amyotrophic Lateral Sclerosis Association (JALSA), the Deutsche Gesellschaft für Muskelkranke e.V. (DGM), and the California based ALS Association have played an important role in the education and support of people with ALS.
|B. Respiratory Support and Home Ventilation|
__Home care requires substantial commitments by family members to care for the ALS patient's basic living needs, including bathing, eating, dressing, cooking and shopping. This involves substantial restrictions of personal activities, as well as responsibility for arranging for and scheduling nurses, and dealing with suppliers and insurers. These responsibilities can increase with the patient's decline, and may include learning how to use communication-assistance devices and the cleaning and care of ventilator equipment and gastrostomy feeding. The emotional and psychological strains can be tremendous, and families may experience resentment, anger, feelings of entrapment, and even ill health.20 In addition, home care and home ventilation can be expensive. The average yearly cost of home ventilation in the U.S. has been placed at more than $150,000 per year.21 Private insurance, Medicare and Medicaid cover most of the costs, but generally will not provide 24 hour nursing care which can itself be costly. Some items not typically viewed as "medical", such as communication aids, may not be covered.22 Out-of-pocket expenses borne by families can range upwards of $7,200 a month.23 In sum, home ventilation is a realistic option only for those with comprehensive insurance, financial resources and 24-hour care. It is simply not an option for the millions of uninsured Americans. The financial cost of home ventilation is a less significant factor in Germany and Japan, countries with national health insurance.
__In the Japanese culture the family's obligations as care provider during sickness are deeply embedded in tradition, particularly for long-term care. Patients are expected by their families to strive to live as long as possible. The reality of having this incurable and inexorable disease is so shocking and devastating that withholding the truth from patients, postponing crucial decisions, and total commitment to care and support until the last moment becomes the shared attitude of all family members. This commitment occurs even at great sacrifice to ordinary family life.24 A number of Japanese families of ALS patients have expressed the need for financial support, availability of long-term care facilities and accessibility to home care personnel.
__The burdens of home ventilation for families can be a significant factor in this decision. Moss and colleagues found costs and availability of insurance, together with negative attitudes toward home ventilation among patients, families and physicians to be important factors in this decision.25 This conclusion is consistent with widely shared experience that a major concern for patients in making end-of-life decisions is the burdens imposed on family members. Early and clear discussion of the question of home ventilation is an important part of care and treatment planning. Concerns for family welfare should be openly discussed. The financial costs involved in the home ventilation decision (insurance coverage, financial resources) ought to be a part of this conversation, concerns for the moral propriety of allowing costs to influence decisions to refuse life support notwithstanding.26 The alternative of hospice care should be considered as well.
|C. Withholding and Withdrawing Respiratory Support|
__The withholding/withdrawing issue may also arise in more subtle and less familiar ways in the care of ALS patients. Ventilatory equipment must be maintained, meaning that periodically it must be disconnected, cleaned and kept in good condition. This process might be narrowly construed as a series of stoppings and startings of the ventilator, and may feel that way for care providers. Understanding that "stopping" and "not starting again" are both ethically permissible if consistent with the patient's treatment plan and objectives should help to alleviate concerns in this regard. Brief respite from ventilator dependence can also bring strong reactions from the patient and may trigger re-evaluation of the earlier decision to accept ventilation. A sudden change of mind should be taken as a call to conversation. While competent patients retain the right to change their minds, the choice to discontinue the ventilator should reflect informed and considered judgment. More complex questions arise when the incompetent patient's current requests are contrary to prior settled judgments or a previously issued advance directive.
|D. The Totally Locked-in State|
__Advance care planning should include frank discussion of TLS. As with other of the severe disabilities caused by progressive ALS, the physician-patient-family dialogue should be cognizant of the ways personal quality of life perceptions influence the presentation and understanding of information; physicians should strive to present prognostic information in an objective manner. It should not be assumed that TLS patients are incompetent to make their own decisions, as cognitive capacity might remain intact, and for those who are not totally locked-in some communication may still be possible. Physicians have ongoing obligations to assess patient competence, including being attentive (as at other stages of ALS) to the presence of depression or other psychological states that may impair competence.31
|IV. Advance Directives in Cultural Context|
|A. Legitimation of Advance Directives in Law|
__Concurrent with these judicial developments, state legislatures began to grapple with end-of-life decisions, and began to enact advance directives statutes recognizing the right of individuals to write a living will, proxy directive, or both, to guide health care decisions in the event of future decisional incapacity. The first advance directive law was enacted by California in 1976. All 50 states and the District of Columbia now have legislation in place that expressly gives individuals the right to issue advance directive documents and to have their wishes to refuse treatment in certain situations (in particular when terminally ill or in persistent vegetative state) honored. State laws are complemented by a new federal law, known as the Patient Self-Determination Act (the PSDA) which went into effect in 1991. Largely procedural in its content, the PSDA defers to substantive rights recognized under the laws of the several states. The core mandates of this law, which applies to all health care facilities nationally that receive Medicare and Medicaid funding, are that all persons are to be asked upon admission (or upon coming under the care of a health care provider such as a health maintenance organization) whether they have an advance directive; that this information is to be documented in the medical record; and that patients and families are to be given a statement of their rights concerning advance directives. The PSDA also mandates that facilities maintain written policies and procedures regarding advance directives and the facility's legal obligations, and imposes duties to provide staff and community education. The predominant goal of the PSDA is to foster greater use of and respect for advance directives.33
__German law embodies a similar commitment to the principle of patient autonomy, but the legal significance of autonomy is much less developed than in the U.S. The German constitution's protection of individuals' rights to self-determination has led to development of the doctrine of informed consent which provides that every medical procedure requires the patient's consent. Refusal of treatment, including life support, is legally binding, but it appears that this right is rarely exercised. This rule might be interpreted as giving effect to anticipatory refusals of treatment in an advance directives, but this has yet to be clearly articulated. A 1994 German Supreme Court ruling (Bundesgerichtshof ) mandates that a treatment decision be based upon "the presumed will of the patient at the actual moment", a determination that is to give priority to the patient's prior oral or written expressions, his or her personal values and religious convictions. The ruling also suggests that advance directives may be applicable to situations other than terminal illness when death is near. The 1992 German Care Law recommends designation of a health care proxy, and contains model language for living wills; but there is no federal law that gives legal force and effect to such documents.34 Thus, while recent developments in German law appear to set forth a foundation for evolution of firm commitment to substituted judgment and legitimation of advance directives in law, it is premature to judge whether these roots will grow to mirror the Quinlan legacy.
__Whether advance directives should be given binding effect is uncertain. In contrast to the American jurisprudential system which establishes rules for future behavior and generally makes a judicial ruling binding upon other courts and upon subsequent litigants similarly situated, under German civil law a judicial opinion is binding only upon the parties to the case that has been decided.35 An official policy document issued by the Federal Chamber of Physicians acknowledges that physicians ought to be guided by the presumed will of the patient, but characterizes prior oral and written directives as guidelines only, suggesting that physicians are not bound by patients' advance directives.36 (The stated rationale is that current circumstances may differ from those contemplated by the patient when the directive was written.) Clearly there is a gap between recent legal developments and the attitude of many German physicians. A number of German legal scholars believe that in the German system current law is properly interpreted to give voice to incompetent patients' rights to refuse treatment, different understandings of the precedential force of court decisions notwithstanding.
__The legal climate in Japan stands in stark contrast to that of the U.S. and Germany, both in principle and in practice. The concept of advance directives has not as yet been embraced in Japanese law. While several types of advance directive documents have been issued by voluntary associations, most notably the Japan Society of Dying With Dignity (JSDD), these documents have not been legitimated in law and are not binding upon medical professionals. Japanese legal decisions even in the recent Supreme Court case concerning "Tell Truth to Cancer Patients" (Supreme Court, The Third Minor Division, Makino Case, April 25, 1995) continue to endorse traditional approaches of medical professionals to end-of-life care.37 The principle of patient autonomy on which advance directives are based has yet to win acceptance in Japanese jurisprudence. Moreover, prohibitions against assisting suicide, murder by request and murder with consent found in the Criminal Code of Japan continue to exert a strong influence upon the behavior of physicians who look to the criminal code as a source of justification for prolonging life as long as possible. It is important to note that the Criminal Code of Japan also provides legal protection for physicians who choose to terminate treatment on the ground that continued treatment would be futile or useless.38
|B. Public and Professional Attitudes about Advance Directives|
__Why advance directives are not in greater use may be explained by a number of factors, including 1) natural reluctance to confront death both personally and within the family; 2) lack of appreciation for the importance of such documents; 3) the belief that family authority and/or trust in the physician makes directives unnecessary; 4) the fact that advance directives are not an integral part of the physician-patient relationship (the U.S. included); 5) absence of legal recognition of advance directives (Japan and Germany); and 6) apprehension that a living will gives health care providers reason to deny access to desired care (a concern that has been identified among some minorities in the U.S.). It is also the case (as discussed below) that the inherent limitations of human foresight make advance directives imperfect instruments that do not always provide clear direction for critical treatment decisions, a fact that may diminish confidence in directives, particularly among physicians. The findings of a recent, large multi-medical center study confirm the conclusions of earlier studies and observations that physicians often are unaware of, or ignore, patients' wishes for end-of-life care.42
__The nature and quality of the physician-patient dialogue has an essential role in informing and educating patients and families about the value of advance directives. Physicians often do not raise the issue of advance directives with their patients, and may be unaware of their patients' directives. Though the vast majority of physicians favor use of advance directives,43 there is a significant pattern of physicians not taking responsibility for initiating the advance directive discussion. It has been shown that this pattern persists after enactment of the PSDA.44
__Failure of physicians to raise the issue of advance directives may be attributable to discomfort with the subject of dying, an episodic rather than ongoing physician-patient relationship, and ignorance of advance directive issues or their legal status. It has also been shown that many physicians believe that talking about dying may be discouraging or even harmful to patients; that patients do not want to discuss these issues; or that it is the patient's responsibility to raise the topic. This may be true in individual cases - the factors just noted can make patients reluctant to raise the issue as well - but it is not true of all patients. Growing numbers of patients say they are interested in receiving more information and report dissatisfaction with physician communication on this subject.45
__Data and experience concerning the willingness of physicians, patients and families to openly confront end-of-life decisions is a partial indicator of the place of discussions about advance directives with ALS patients in the U.S. Silverstein and colleagues studied recently diagnosed ALS patients seen at a university neurology clinic. They found that "patients with ALS have definite, stable preferences for receiving information", including information about life-sustaining treatment. Access to information about the disease and its progress is usually highly valued; 81% of ALS patients wanted as much information as possible, whether good or bad. Seventy percent (70%) of ALS patients wanted to participate in decisions about their own medical care.46 Another study by Moss and colleagues suggests that this attitude may not be as prevalent as these numbers suggest. The Moss study found that 63% of patients did not want to know in advance what would happen to them (that is, that they would develop respiratory failure). However, Moss' results may not be indicative of ALS patients' prospective desire for information and participation, as the study population consisted of a small percentage of ALS patients already on home ventilation who responded to this question with the benefit of hindsight.47
__Despite patients' desire for discussion with physicians, such discussions rarely occur. In the Silverstein study, 81% of patients thought their physicians wanted to know their preferences about life support, but only 21% had in fact discussed this topic with their physician.48 Moss and colleagues found that a majority of physicians (58%) favored early discussion of the home ventilation option. But 24% felt this issue should not be discussed until respiratory failure occurred.49 In a recent unpublished survey of 50 ALS patients on home ventilation (from California, West Virginia, Ohio and Kentucky) reported by Moss, only 75% of patients were aware of the likely development of respiratory failure, 47% indicated that they had talked to someone about their wishes for life support in extreme situations only, and fewer than 50% of ALS patients reported that they had discussed this information with their physician.50 These data suggest that most patients are not well prepared to decide about mechanical ventilation. Equally alarming, only 42% make the decision to accept mechanical ventilation in advance of respiratory crisis. Most who are placed on a ventilator without their consent said they would not want to undergo it if they could make the decision over again.51
__Moss also reports the only known data about ALS patients' use of advance directives. In this unpublished study of patients already on home ventilation, 76% of patients had completed an advance directive. Only 6% had directives before the ALS diagnosis, while 34% completed a directive after diagnosis but before starting mechanical ventilation. Thirty-six percent (36%) completed an advance directive after beginning ventilator support. Only 8% of the patient population did not want to issue an advance directive. It is noteworthy, however, that only 50% of ALS patients' advance directives stated specific preferences about stopping respiratory support. Moreover, the wish to limit life support was more often shared with families than with physicians.52
__As noted above, German physicians are inclined to disclose and discuss the diagnosis of ALS, but prefer not to provide information about the possibility of long term respiratory support. The most significant reason is that ALS is seen as a terminal illness with no hope for the future. The limited nature of what medicine has to offer and the perception that living with ALS in its latter stages is a poor quality of life, together with a sense of compassion, all contribute to this attitude among physicians.
__The prevailing attitude among Japanese is that raising the topic of advance directives with ALS patients or even addressing a request for information from patients to enable more informed patient decisions should if possible be avoided. Such a conversation would be regarded as uncomfortable, uneasy, and unkind for both families and patients. It might be possible for families to reluctantly accept the patient's wishes at the end-stage of life. However, generally such a conclusion is not reached until all possible efforts to prolong life have been exhausted. The key concept at issue here is not the problem of truth-telling or respect for patient autonomy, but the common desire, born of a true sense of compassion in the patient-family relationship, to cling to hope until the last possible moment.
__The findings of the 1993 JALSA survey of ALS patients and their families support this understanding. Decisions to start a respirator are rarely initiated by the patient and are most often made by the physician. Requests for respiratory support came from patients only 9% of the time, and only 12% of the time did families initiate this request. But there is also evidence to suggest that decisions to withhold or withdraw a respirator more often belong to the patient. Among surviving family members of ALS patient, 35% reported that this decision had been made by the patient; 25% stated it was a family decision. These findings suggest that autonomy is gradually gaining acceptance among ALS patients, their families and treating physicians. At TMNH, where informing patients of their diagnosis and prognosis is standard practice, almost all patients and family nonetheless prefer to leave the decision about artificial ventilation to their physicians.53
|C. Making Informed Choices|
__Advance directives, specifically living wills, have been criticized for offering limited practical guidance in clinical decision making. Experience with living wills demonstrates the difficulties of interpreting and implementing anticipatory and static written documents that are sometimes written remotely in time, prior to onset of the patient's illness, or without adequate specificity. Clearly, as documents that seek to prospectively address future events, advance directives are subject to the inherent limitations of human foresight, and have too often proven to be vague or ambiguous when actual treatment decisions need to be made. In response to the difficulties encountered with living wills, the proxy directive and increasingly the combined directive have gained widespread acceptance as preferred approaches to advance planning. The chief advantage of the proxy is that it offers the flexibility of a trusted family member or friend who in effect takes the place of the patient in the physician-patient relationship, with authority to make treatment decisions on the patient's behalf and in accordance with the patient's own wishes and best interests (subject to any limitations that may be imposed by law or by the directive itself), and able to respond to changes in the patient's medical condition. The combined directive holds the added advantage of giving the proxy (and others) more specific guidance about the patient's wishes, thereby more firmly assuring respect for one's own wishes and relieving some of the burdens of decision for proxy, family and physician.55
__A disease-specific advance directive developed within the longitudinal context of progression of ALS is less likely to suffer from the difficulties that have sometimes plagued advance directives, in particular living wills. There is some reason to believe that since care and treatment is ordinarily within the context of an established physician-patient-family relationship designation of a proxy is less important as a mechanism for ensuring deference to family authority. This may be most true in Japan. Proxy appointment is nonetheless advisable, as it alleviates professional anxiety about the legalities of looking to family members to authorize termination of life support (a substantial concern in some U.S. states where the law is less hospitable to family decision making). More importantly, studies showing that family members' predictions of patients' preferences are inaccurate more often than is commonly thought further counsels use of the combined approach to ensure respect for personal values and wishes.56
__Personal decisions to terminate (or continue) life support are based on a number of factors, chiefly the value attached to self-determination and control, perceptions of quality of life, and the desire for a dignified dying process. Concern for family welfare can also be an important reason to refuse life support and accept an earlier death. As noted above, substantial burdens on family members, both personal and financial, have an important part in the decisional calculus for the ALS patient. These issues should be openly discussed, not suppressed or discredited. Respect for autonomy, which includes fostering informed decision making, supports honoring refusal of life support irrespective of the patient's reasons.57 The Values History - a question and answer style document designed to elicit deeper understanding of personal views of dignity, dependency, the importance attached to communication, and other matters - may be especially useful for ALS patients as a companion to issuance of an advance directive.58 The story-based approach to assessing patient values is another useful tool that could be adapted to use with ALS patients.59 These tools serve the further purpose of being a vehicle for enhanced understanding among patient, family and physician, and thereby also increase the likelihood that the physician will later respect the advance directive as an accurate reflection of the patient's values and wishes. Those who have written a directive prior to diagnosis of ALS should be encouraged to update their wishes and issue a new, more disease-specific directive in light of this diagnosis.
|D. Respect for Advance Directives|
__Given the uncertain legal status and infrequent use of advance directives in Germany and Japan, whether patients' advance directives will be honored in the respective countries is impossible to assess. (The track record of the JSDD cannot be taken as indicative of the population as a whole, particularly considering that no more than 1% of Japanese actually have advance directives.) However, the U.S. experience suggests that uniform legal legitimation and popular acceptance of advance directives has been a critical but only partial response to the challenges of making advance directives an integral part of advance care planning. Acceptance of and respect for advance directives involves a continuing process of both professional and public education.
|E. When Patients Change Their Minds|
__Obwohl "Advance Directives" bei Patienten mit ALS nicht weit verbreitet sind, können sie eine wertvolle Rolle spielen. Die für Patienten mit ALS typische Erhaltung der geistigen Leistungsfähigkeit, bei zugleich zunehmender Verschlechterung des körperlichen Zustandes über einen relativ langen Zeitraum, bieten den Betroffenen ausgiebig Gelegenheit, Entscheidungen über künftige medizinische Interventionen zu treffen.