Advance directives for oral feeding in dementia: a response to Shelton and Geppert.

In a recent paper in JME, Shelton and Geppert use an approach by Menzel and Chandler-Cramer to sort out ethical dilemmas about the oral feeding of patients in advanced dementia, ultimately arguing that the usefulness of advance directives about such feeding is highly limited. They misunderstand central aspects of Menzel's and Chandler-Cramer's approach, and in making their larger claim that such directives are much less useful than typically presumed, they fail to account for five important elements in writing good directives for dementia and implementing them properly: (1) Directives should be paired with appointment of trusted agents. (2) Appointed agents' authority can be greatly weakened without advance directives to guide them. (3) Directives' implementation does not require clinically precise assessment of dementia's stage. (4) Palliative support is typically required for withholding of oral feeding to be compassionate. (5) The central purpose of stopping feeding is often not the avoidance of suffering but not prolonging unwanted life.

Clinical Guidelines for Voluntarily Stopping Eating and Drinking (VSED).

As the care of patients with serious illness increasingly emphasizes clarifying goals of care, exploring quality of life, and minimizing patients' symptom burden, voluntarily stopping eating and drinking (VSED) has emerged as a topic of increasing interest for patients who face a diminishing quality of life. It is an option for those with serious illness that is legal in every state in the country, but for which there are few published comprehensive guidelines-and none specific to the American medical system-even as public awareness and the number of inquiries regarding this action increase. In addition to the ethical questions raised by the practice and support of VSED, there are also clinical, logistical, institutional, social, religious, spiritual, and administrative considerations for clinicians who are asked to respond to patients' inquiries about VSED and who discuss this option in end-of-life care. With these clinical guidelines, we seek to provide practical recommendations for clinicians who consider providing support to their patients who contemplate and/or undertake this effort to hasten death.

How Should Willingness-to-Pay Values of Quality-Adjusted Life-Years Be Updated and According to Whom?

Before updating any willingness-to-pay (WTP) per quality-adjusted life-year (QALY) threshold, a few points must be recognized. Ethical justification for using WTP thresholds and QALYs lies in incorporating the preferences of those whose treatment could be affected by resulting resource allocations. For WTP thresholds, such justification depends on the sufficiency of a match between a group-members of an insurance pool from which health care payments and services are drawn-and those whose health care is potentially affected. For QALYs, that justification depends on eliciting the right persons' preferences to inform quality-adjustment ratings; on balance it should be from those who have the conditions being rated. Because the value of simply being alive is not adequately accounted for, how life extension and quality improvement are combined in constructing the QALY is its most significant shortcoming as a measure. Although updating WTP thresholds might be better than not updating them, this manuscript suggests why drawing on a less fundamentally flawed concept than the conventional QALY is more important.

Germline MUTYH Mutation in a Pediatric Cancer Survivor Developing a Secondary Malignancy.

Radiotherapy-induced second malignant neoplasms (SMNs) are a severe late complication in pediatric cancer survivors. Germline mutations in tumor suppressor genes contribute to SMNs; however, the most relevant germline variants mediating susceptibility are not fully defined. The authors performed matched whole-exome sequencing analyses of germline and tumor DNA from 4 pediatric solid tumor survivors who subsequently developed radiation-associated SMNs. Pathogenic and predicted deleterious germline variants were identified for each patient and validated with Sanger sequencing. These germline variants were compared with germline variants in a cohort of 59 pediatric patients diagnosed with primary sarcomas. Pathway analysis was performed to test for similarities in the germline variant profiles between individuals diagnosed with SMNs or primary sarcomas. One index patient was found to have a pathogenic germline monoallelic mutation in the MUTYH gene, which encodes the base excision repair enzyme adenine DNA glycosylase. This specific germline mutation is associated with a form of familial adenomatous polyposis, a new diagnosis in the patient. Germline-level genetic similarity exists between SMN-developing patients and patients developing primary sarcomas, with relevant genes involved in signal transduction and DNA repair mechanisms. The authors identify a germline MUTYH mutation in a pediatric cancer survivor developing an SMN. Germline mutations involving specific pathways such as base excision repair may identify individuals at risk for developing SMNs. The composition of germline variants in individual patients may enable estimates of patient-specific risk for developing SMNs. The authors anticipate that further analyses of germline genomes and epigenomes will reveal diverse genes and mechanisms influencing cancer risk.

Advance Directives for Refusing Life-Sustaining Treatment in Dementia.

Aid-in-dying laws in the United States have two important restrictions. First, only patients who are terminally ill, defined as having a prognosis of six months or less to live, qualify. Second, at the time the patients take the lethal medication, they must be competent to make medical decisions. This means that an advance directive requesting aid in dying for a later time when the patient lacks decision-making capacity would be invalid. However, many people are more concerned about avoiding living into severe dementia for years-a time when they will lack decision-making capacity-than they are about preventing suffering or the loss of dignity or autonomy for a few months at the end of life. Gillian Bennett is an example of someone determined not to live into severe dementia. She opted for preemptive suicide in 2014, explaining why in a letter she posted online: "Every day I lose bits of myself, and it's obvious that I am heading toward the state that all dementia patients eventually get to: not knowing who I am and requiring full-time care." A major problem with Bennett's solution, however, is that the individual is likely thereby to be giving up some "good time." A legal alternative to preemptive suicide is to create an advance directive stating the circumstances under which one wants not to receive any lifesaving or life-sustaining treatment, even the most basic and noninvasive. This option is our focus in this paper: how to create effective advance directives to avoid living into severe dementia. To be relevant to progressive dementia, the directive would need to state what kinds of care should be withheld and when. At the same time, advance directives for severe dementia face serious challenges. Before addressing these, we review the normative force of directives themselves.

Long-term outcomes of adult medulloblastoma patients treated with radiotherapy.

Medulloblastoma (MB) is rare in adults and treatment guidelines are consequently not well-established. Few modern series have reported long-term follow-up and treatment sequelae. We examined long-term outcomes of adult MB patients at one institution. Records of 29 consecutive patients (18 male, 11 female) aged ≥ 18 years who received radiotherapy (RT) for primary MB from 1990 to 2016 were reviewed. Median age at diagnosis was 28 years (range 18-72 years). Seventeen patients were standard risk and 12 were high risk. Nineteen patients had gross total resection, seven had subtotal resection, and three had biopsy only. Median craniospinal irradiation and boost doses were 36 Gy (range 23.4-39.6 Gy) and 55.8 Gy (range 54-59.4 Gy), respectively. Of 24 patients receiving chemotherapy, 20 received concurrent + adjuvant and 4 received adjuvant only. At median follow-up of 9.0 years (range 1.1-20.5 years), five patients recurred: four in the posterior fossa and one in both the posterior fossa and above the tentorium. Five patients died: two of disease progression and three after possible treatment complications (seizure, lobar pneumonia, and multifactorial sepsis). At last follow-up, 23 patients were alive with no evidence of disease. Long-term effects include executive dysfunction (n = 17), weakness/ataxia (n = 16), and depression/anxiety (n = 13). Kaplan-Meier estimates of 10-year overall survival and failure-free survival are 83% (95% confidence interval [CI] 59-93%) and 79% (CI 55-91%), respectively. Despite encouraging disease control in this cohort, long-term sequelae may limit quality of life. Multimodality pediatric regimens using lower RT doses may be considered to reduce treatment-related morbidity.

Merits, Demands, and Challenges of VSED.

VSED demands willful determination from those who do it. The requisite willfulness is an ethical strength, providing greater assurance of voluntariness. Dying by VSED has another major merit: its pace provides time for the person and loved ones to embrace death in a shared appreciation of life. Support from professional caregivers is important in making dying by VSED comfortable. If acknowledged by its providers, this need for professional support has ancillary benefits: accurate information is more likely to be communicated to patients, and professional caregivers help normalize VSED and minimize abuses. VSED faces a moral challenge when it is categorized as suicide. In an obvious sense, VSED is suicide: the person's intention is to die, and the means employed assuredly causes death. Being a form of suicide does not imply that VSED is morally impermissible. Pursued to hasten death in a last stage of life, it is a response to "terminal" situations. This contributes greatly to VSED's moral acceptability when perceived as suicide.

Advance directives, dementia, and withholding food and water by mouth.

People with dementia who are no longer competent have limited control over how their lives end. But an advance directive to withhold food and water by mouth could be used to ensure that one does not live for years in severe dementia. Such directives are arguably already legal.

Advance directives, dementia, and physician-assisted death.

Physician-assisted suicide laws in Oregon and Washington require the person's current competency and a prognosis of terminal illness. In The Netherlands voluntariness and unbearable suffering are required for euthanasia. Many people are more concerned about the loss of autonomy and independence in years of severe dementia than about pain and suffering in their last months. To address this concern, people could write advance directives for physician-assisted death in dementia. Should such directives be implemented even though, at the time, the person is no longer competent and would not be either terminally ill or suffering unbearably? We argue that in many cases they should be, and that a sliding scale which considers both autonomy and the capacity for enjoyment provides the best justification for determining when: when written by a previously well-informed and competent person, such a directive gains in authority as the later person's capacities to generate new critical interests and to enjoy life decrease. Such an extension of legalized death assistance is grounded in the same central value of voluntariness that undergirds the current more limited legalization.

Justice and fairness: a critical element in U.S. health system reform.

The case for U.S. health system reform aimed at achieving wider insurance coverage in the population and disciplining the growth of costs is fundamentally a moral case, grounded in two principles: (1) a principle of social justice, the Just Sharing of the costs of illness, and (2) a related principle of fairness, the Prevention of Free-Riding. These principles generate an argument for universal access to basic care when applied to two existing facts: the phenomenon of "market failure" in health insurance and, in the U.S., the existing legal guarantee of access to emergency care. The principles are widely shared in U.S. moral culture by conservatives and liberals alike. Similarly, across the political spectrum, the fact of market failure is not contested (though it is sometimes ignored), and the guarantee of access to emergency care is rarely challenged. The conclusion generated by the principles is not only that insurance for a basic minimum of care should be mandatory but that the scope of that care should be lean, efficient, and constrained in its cost.