Succinyl-CoA: 3-oxoacid CoA-transferase (SCOT) deficiency is an inborn error of ketone body utilization, characterized by intermittent ketoacidotic crises and persistent ketosis. The diagnosis was suspected in a patient who presented with hypoglycaemia, ketoacidosis and coma at 4 days of age. The hypoglycaemic tendency was only observed during the first month of life. A novel macromolecular labelling assay in cultured skin fibroblasts using D-3-hydroxy[3-14C]butyrate supported the diagnosis. Subsequently, 9% residual SCOT activity and undetectable cross-reactive protein were noted in fibroblasts and the patient was found to be homozygous for the G324E SCOT gene mutation. By 7 years of age, recurrent episodes of ketoacidosis superimposed on persistent hyperketonaemia had resulted in over 25 hospitalizations requiring intravenous fluid, glucose and sodium bicarbonate therapy. He has had normal growth but developmental delay and attention deficit-hyperactivity disorder. A continuous intravenous glucose infusion at 38 microlmol (6.8 mg)/kg per min reduced plasma total ketone levels from greater than 1.5 mmol/L to less than 0.5 mmol/L after 48 h. This indicates that patients with SCOT deficiency do not always manifest ketosis with administration of a sufficient amount of carbohydrates, but that even under such conditions hyperketonaemia is difficult to eliminate completely. The presence of hypoglycaemia does not exclude the diagnosis of SCOT deficiency in infancy.
Even with the advent of climate change, mainstream environmentalism lacks a robust death ethics, that is, ethical theories and practices for attending directly to what is owed to the unjustly dead and dying. This article draws on Indigenous, Afrofuturist, and feminist science fiction narratives and their correlating lived practices to explore how death ethics for those driven extinct by climate change and other environmental injustices can and ought to go beyond affect, symbolism, and abstraction. It puts forward environmental palliation as an alternative framework for grappling with the injustice of extinction as and in publics. Far from a glorified form of euthanasia, palliation is an ethic and a practice geared toward providing good or better deaths for particular entities under specific conditions of injustice. In death, palliation cedes to remembrance, an ethic and practice for keeping the dead alive in memory so that they can be cared for. When done right, these death ethics are inextricably linked with climate justice for the living and those yet-to-be.
Jonathan Beever and Nicolae Morar’s (2016) article “The Porosity of Autonomy: Social and Biological Constitution of the Patient in Biomedicine” and its accompanying commentaries in the American Journal of Bioethics—though insightful, innovative, and provocative—overlook key interlocutors necessary for any discussion of whether the mid-twentieth-century biomedical principle of autonomy should be revised or revoked. The conversation sparked by “The Porosity of Autonomy” will remain both incomplete and politically untenable so long as there is no meaningful engagement with persons/communities who appeal to the principle of patient autonomy in order to articulate and challenge the conditions of their oppression.
Environmentalism has long placed heavy emphasis on strategies that seek to ensure the environment of today and the future roughly mirror the past. Yet while past-oriented approaches have come under increased scrutiny, environmental ethics in the time of climate change is still largely conceptualized as that which could pull humanity back from the brink of disaster or, at least, prevent the worst of it. As a result, practical and conceptual tools for grappling with what is owed to the dead and dying victims of environmental injustice have been and continue to be woefully underdeveloped. This paper advances scaffolding for robust environmental death ethics that are temporally pluralistic and at home within intergenerational climate justice.
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