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*

Robert S. Olick, Rihito Kimura, Jan T. Kielstein,
Hideaki Hayashi, Marc Riedl and Mark Siegler


__Amyotrophic lateral sclerosis (ALS) is a rare motor neuron disease characterized by progressive loss of motor function and retention of intellectual ability. Most ALS patients ultimately die of respiratory failure, often after reaching a decision not to start or to discontinue ventilatory support. This paper offers a cross-cultural perspective on ethical and socio-cultural issues in advance care planning for ALS patients, with particular emphasis on the role of advance directives for health care. We examine these questions in the different cultural, legal, medical and ethical traditions of Japan, Germany and the U.S. Some aspects of the discussion may be generalizable to other late onset degenerative conditions. The ethical issues raised by advance care planning with ALS patients are especially relevant to other conditions also often characterized by retention of mental capacity over the course of long-term physical deterioration, such as multiple sclerosis (MS) and progressive muscular dystrophy.

__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

__ALS (also known as "Lou Gehrig's disease" in the U.S., as "motor neurone disease" (MND) in Great Britain and as "Charcot's disease" in France) is a crippling disorder that involves large motor neurons of the spinal cord, brainstem, and motor cortex. The clinical manifestations are progressive motor weakness and muscle atrophy. Capacities for sensation and awareness remain intact, and patients ordinarily retain intellectual ability and mental competence (absent complicating conditions) until very late in the course of illness. Degenerative loss of motor neurons in the spinal cord and of muscle control generally leads to quadriplegia and dependence on others for support of daily living activities. ALS is often marked by gradual loss of the ability to speak and swallow caused by progressive motor weakness of throat and facial muscles. The median duration of this invariably fatal disease from diagnosis to death is 4 years, but there are reported cases of some patients living for up to two decades after initial diagnosis. Progression of the disease ordinarily is at a gradual and steady rate. Men are affected by ALS somewhat more frequently than women, with peak onset occurring for most patients in their 50's and 60's. The cause and pathogenesis of ALS are not known; there is no specific cure or treatment. While early deterioration of respiratory capacity is invariably gradual, an accelerated decline occurs during the 12 to 15 months preceding death.4

__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

__As with any serious chronic illness, a plan for care and treatment over time is essential. A care plan is of especial importance for ALS patients because of the long clinical course of illness from time of diagnosis, during which time many treatment choices will need to be made. A critical concern for the ALS patient is to maintain control and independence - to continue to pursue life's activities both large and small to the greatest extent possible. Feeling in charge of one's own life is not only valued for its own sake, it can have a drastic impact on psychological well-being and can prolong survival in ALS patients.7 With an appropriate support network, people with ALS who are highly motivated can live long, productive and rewarding lives despite crippling disability, including living well beyond respiratory failure. At some point the vast majority of ALS patients confront decisions about end of life support. Of particular concern for the ALS patient are whether or not to accept a respirator when respiratory failure develops and whether care will be provided in the home - issues that intersect in the decision about home ventilation. For those on mechanical ventilation whether and when to discontinue the ventilator, and whether to cling to life in a TLS, take on added importance as disease progresses. The content of the care plan will invariably be shaped by the physician-patient-family relationship, and may well be determined by the allocation of authority within that triad. Consequently, understanding the basic moral framework of that relationship in the three cultures lies at the core of any ethical analysis. Of particular import are two interrelated dimensions of this relationship: The allocation of decisional authority, and the quality of communication supporting informed decision making.

A. The Physician-Patient-Family Relationship

__In the U.S., the principle of patient autonomy holds priority status in the physician-patient relationship. Patients have the right to decide about the course of their care and treatment, and physicians have correlative obligations to respect patients' autonomous decisions and to engage in a dialogue which promotes the exercise of autonomy. For the competent patient, autonomy and self-determination find expression in the ethical and legal doctrine of informed consent, which encompasses the right to refuse treatment (the logical corollary of consent), including life-sustaining treatment. This same principle has been extended to decisions in behalf of incompetent patients on the premise that the right of self-determination should not be lost or impaired when the patient becomes unable to personally exercise that right. Through the exercise of future-oriented autonomy a written advance directive extends self-determination to the post-competency period of illness.

__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 Ningen in Japanese, the Japanese term for person, is composed of two root Chinese characters that mean "human Hito in Japanese" "between-ness Aida in Japanese". 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 Shinrai-Kankei in Japanese), 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

__The option of home ventilation is a distinguishing feature of ALS. Home ventilation can offer improved quality of life; patients who have chosen home ventilation generally report being satisfied with this decision. But home ventilation also involves substantial burdens on family members and high financial costs - factors that can strongly influence this decision.

__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

__ALS patients' preferences may change after they have been on a ventilator.27 As a consequence, clear understanding of the withholding/withdrawing distinction is of special importance. The consensus view in medical ethics (also embraced in American law) is that there is no ethical difference between withholding and withdrawing of life support. Once started a ventilator may later be withdrawn. A strong rationale for this position comes from the fear that to require greater justification for withdrawing treatment would result in early decisions not to start potentially beneficial treatment or to continue non-beneficial treatment - in either case doing serious harm to the patient.28 The ALS patient, the family, and the physician should understand that there is always an option to discontinue the ventilator after it has been initiated. Families and health care professionals may still feel that stopping a ventilator once it has been started is a more drastic step because it involves an affirmative action to disconnect the machine. The psychological and emotional dimensions of this issue should be a part of the planning dialogue.

__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

__Some ALS patients face the prospect of life in the totally locked-in state - an end stage of the disease's progression - a condition in which the patient may be conscious, aware and thinking but unable to communicate with the world. With proper medical management long-term survival in TLS is possible; death need not be imminent.29 For many, TLS invokes powerful and frightening image of a "fate worse than death". On the other hand, some TLS patients want life-sustaining treatment continued despite this severe disability and extremely poor quality of life.30

__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

__In the 20 years since New Jersey Supreme Court's seminal opinion in the case of Karen Ann Quinlan (1976)32 the right of both competent and incompetent patients to refuse treatment, including life-sustaining treatment, has become a well-established component of the panoply of patients' rights in health care in the U.S. Quinlan held that an incompetent patient in a persistent vegetative state had the right to refuse the respirator which artificially sustained her life. Finding this autonomy-based right to be grounded in a fundamental constitutional right of privacy, Quinlan also held that the right to refuse treatment is not lost upon loss of competence to decide for oneself, and appointed Karen Quinlan's father as her legal guardian with authority to exercise this right on her behalf. In making this determination Mr. Quinlan was charged with the responsibility of giving priority to what Karen would choose for herself, based upon what was known of her prior expressions and personal values, an approach known in Anglo-American law as the doctrine of "substituted judgment." In strictly legal terms Quinlan was not legally binding beyond the borders of New Jersey, but its reasoning soon began to take hold nationally. With rare exception, courts across the country followed the core approach of Quinlan, recognizing the right of incompetent patients to refuse unwanted medical interventions and finding that family members may exercise this right on the patient's behalf under the rubric of substituted judgment. A number of courts grounded this right in the common law right of self-determination and extended the rationale for the doctrine of informed consent to the situation of incompetent patients, thereby expanding the legal foundation for the incompetent patient's right to refuse treatment.

__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

__Despite uniform legal recognition and overwhelming popular support in the U.S., relatively few people have issued advance directives. Estimates of the prevalence of advance directives range from 5%-20% of the population at any given time.39 Certain groups, such as the elderly, the seriously ill and those afflicted with HIV / AIDS, have shown greater interest in advance directives, but this overall figure has remained relatively constant in the aftermath of the PSDA.40 The prevalence of advance directives in Germany and Japan has been estimated at less than 1% of the population.41

__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

__The studies of ALS patients discussed above are consistent with the general view that advance directives are most useful when a part of good communication between physician and patient. They should not be considered a substitute for physician-patient communication. Several features in some respects unique to ALS suggest that ALS patients can design advance directives that are well-informed, highly useful, and free of many of the difficulties that have plagued the early generation of advance planning documents. The long clinical course and predictable outcome of ALS offers ample opportunity for informed decision making. The advance directive discussion should occur within the context of an ongoing, rather than episodic, physician-patient relationship in which physician and patient come to appreciate each others' values and perspectives. Consequently, ALS patients have the opportunity to tailor their advance directives with substantial insight into the choices that will need to be made - what is sometimes referred to as a "scenario-specific"54 or "disease-specific" advance directive.

__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

__Will advance directives be honored? In the U.S., there are at least two "norms" of behavior among physicians. Many physicians believe there is a (near) absolute legal duty to follow the terms of an advance directive. This belief may be influenced by the perception that following the written directive is the best way to avoid legal entanglements, occasional moral qualms notwithstanding. On the other hand, recent empirical work has identified serious shortcomings in honoring patients' wishes near the end of life,60 and has found that physicians are inclined to follow a directive only when consistent with their own views of what is in the patient's best interests.61Physicians' own perceptions of patients' quality of life can play a significant role in shaping professional resistance to terminating life support. For some respect for autonomy and/or the understanding that the ventilator continues a life of poor quality and forestalls an inevitable decline is reason to respect a refusal of life support. Others firmly adhere to the professional commitment to prolong life. Family attitudes reflect these different views as well. Both physicians and families may have a harder time "letting go" when the patient is still interactive, despite severe disability, immobility and physical dependency. One study found that 25% of the physicians caring for ALS patients "indicated they would be unwilling to stop ventilation at the patient's request".62 Early and ongoing discussion of this issue can identify potentially conflicting values. It can also serve to match the physician's practice style with the patient's desire for information and participation in medical decisions, thereby improving the quality of the physician-patient relationship and improving outcomes.63 When the contrary occurs and conflicts over important issues in patient care persist, it may be advisable for the patient to seek out another physician who shares his or her values. The treating physician should be sympathetic and cooperative in this effort. The experience of the Japan Society for Dying with Dignity ("JSDD"), an organization in which lay membership is limited to the elderly, illustrates the value of matching patients and physicians of like philosophy. A study of their membership found that compliance with advance directive was 96% among physician members of the Society.64

__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

__Silverstein and colleagues note that although ALS patients' preferences about life support tend to remain relatively stable, it is not uncommon for ALS patients to change their minds. The authors note that a change of mind may be due to such factors as experience with the illness, changed perceptions of "quality of life", or a new perspective on the benefits and burdens of life support with realization of the imminence of death.65 Though often managed effectively with medication, it is also not uncommon for respirator-dependent patients to request re-placement of the machine after its removal and the actual experience of air hunger. These factors counsel ever more strongly repeated discussion about life support and periodic re-evaluation of the patient's treatment plan and advance directive to ensure that it reflects his or her current wishes. How strictly patients want their advance directive to be followed should be a part of this discussion. One recent study of dialysis patients suggests that a growing number of patients believe that some degree of flexibility in applying their advance directive is appropriate.66 That patients' preferences may change with actual experience on a ventilator suggests more strongly the special importance of clear understanding that there is no ethical difference between withholding and withdrawing of life support.

V. Conclusion

__Different approaches to advance directive issues in the U.S., Japan and Germany are grounded in differences of culture, history and law; differences reflected in the allocation of authority and the place of autonomy in the physician-patient-family relationship. Although termination of life support for the ALS patient is most often discussed as a decision made by the competent patient, advance directives can play a significant role in formulating a care and treatment plan and in making end-of-life decisions upon loss of competence. The inexorable decline and predictable course of ALS together with substantial time from diagnosis to death offer ample opportunity for physician-patient-family discussion and for informed decision making. These factors suggests that ALS patients have greater opportunity to issue informed and clear advance directives, relative to some other patient groups. This also suggests that ALS patients should have fewer concerns about whether their advance directives will be honored. The most frequent life support decision faced by the ALS patient concerns the respirator, but other questions should be addressed as well. Future study should explore the use of advance directives with ALS patients, the frequency and nature of physician-patient communication about advance directives, and the ways in which patient, family and physician perceptions of ALS affect this dialogue. Several recent studies (not specifically concerned with ALS) have suggested that greater use of advance directives can significantly reduce overall health care expenditures near the end of life;67 there is also evidence to the contrary.68 Future work should be attentive to these and other issues raised by the high cost of care for ALS patients dependent on ventilatory support - including equal access to home ventilation and just allocation of health care resources in an era of increasing concern with cost-containment.


__Die vorliegende Arbeit stellt eine kulturübergreifende Betrachtung der Rolle von "Advance Directives" als Mittel der Vorsorgeverfügung für Patienten mit ALS in Japan, Deutschland und den USA dar. Akzeptanz und Anwendung von "Advance Directives" in den drei Staaten werden maßgeblich durch unterschiedliche kulturelle, juristische und ärztliche Traditionen beeinflußt, welche darüber hinaus sowohl die Diskussion über das Sterben als auch die Zuweisung der Entscheidungsautorität innerhalb der Arzt-Patienten-Familie-Beziehung bestimmen.

__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.

Additional References

Borasio, G. D., "Amyotrophe Lateralsklerose (ALS): Eine Information für Patienten", im Auftrag von der Deutschen Gesellschaft für Muskelkranke e.V. (1994).
Cardy, P., "Ethical Issues in Patient Care and Disease Management", Proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 3-9.
Collopy, B. / Dubler, N. / Zuckerman, C., "The Ethics of Home Care: Autonomy and Accommodation", Hastings Center Report 20 (1990): 1-16.
Earll, L. / Johnston, M. / Mitchell, E., "Coping with Motor Neurone Disease - An Analysis Using Self-Regulation Theory", Proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 21-30.
Fujiki, N. / Macer, D. R. J., eds: Intractable Neurological Disorders, Human Genome Research & Society, Proceedings of the Third International Bioethics Seminar in Fukui, Japan, 19-21 November, 1993 (Eubios Ethics Institute 1994).
Goldblatt, D., "Decisions about Life Support in Amyotrophic Lateral Sclerosis", Seminars in Neurology 4 (1984): 104-110.
Hayashi, H., "ALS no Iryo, Rihabiriteshon o dokangaeruka (How Should We Care for The Patients Suffering from ALS?)", Rigaku Ryoho to Sagyo Ryoho (Japanese Journal of Physical and Occupational Therapy) 21 (10) (Oct. 1987): 643-48.
Ichihara, K. / Ichida, K. / Kasabara, Y., "ALS Kanjya eno Zaitaku Chiimu Apurochi: PT, OT no Tachibakara (Home Visit Team Approach to ALS Patients Living in Community: Physical Therapist's and Occupational Therapist's View", Rigaku Ryoho to Sagyo Ryoho (Japanese Journal of Physical and Occupational Therapy) 21 (10) (Oct. 1987): 649-54.
Meininger, V., "Breaking Bad News in Amyotrophic Lateral Sclerosis", Proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 37-40.
Moore, M., "Dying at Home: A Way of Maintaining Control for the Person with ALS / MND", proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 65-68.
O'Brien, Tony, "Palliative care and taboos within motor neurone disease", Proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 69-72.
Oliver, D. / McMurray, N., "Bereavement - Whose Responsibility?", Proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 73-76.
Oppenheimer, E. A., "Respiratory Management and Home Mechanical Ventilation in Amyotrophic Lateral Sclerosis", in: H. Mitsumoto / P. H. Norris, eds: Amyotrophic Lateral Sclerosis: A Comprehensive Guide to Management, New York: Demos Publications, 1994, pp. 138-165.
Scott, A. / Heughan, A., "A Review of Dysphagia in Four Cases of Motor Neurone Disease", Proceedings of the Third International Symposium on Amyotrophic Lateral Sclerosis / Motor Neurone Disease, Palliative Medicine 7 (4) (Supp. 1993): 41-47.
Sivak, E. D. / Gipson, W. T. / Hanson, M. R., "Long-term Management of Respiratory Failure in Amyotrophic Lateral Sclerosis", Annals of Neurology 12 (1982): 18-23.
Sivak, E. D. / Cordasco, E. M. / Gipson, W. T. / Stelmak, K., "Clinical considerations in the implementation of home care ventilation: observations in 24 patients", Cleveland Clinic Quarterly 50 (1983): 219-225.

Footnotesthe footnotes

Please Email me! Do you have anything to say about the contents of this page?
Please send your opinion to
rihito@human.waseda.ac.jp (Prof. Rihito Kimura)

Return to the contents
Advance Care Planning and the ALS Patient:
A Cross-Cultural Perspective on Advance Directives