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KMID : 0918520180180020050
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Journal of the Korean Society of Inherited Metabolic Disease
2018 Volume.18 No. 2 p.50 ~ p.54
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Two Cases of Methylmalonic Acidemia where Refusal to Blood Transfusion Led to Death
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Jang Ha-Won
Lee Yong-Wok
Jang Mi-Young
Kil Hong-Ryang
Kim Sook-Za
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Abstract
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Jehovah's Witnesses do not accept blood transfusions, because of their particular interpretation of the Old and New Testaments. When people with such religious convictions are in need of medical care, their faith and belief may become an obstacle for proper treatment, and pose legal, ethical, and medical challenges for the health care providers. We report two inherited metabolic disorder cases in South Korea where the infants died whilst under medical care because of parental refusal of blood transfusions for religious reasons. Case 1 had methylmalonic acidemia, Down syndrome and associated congenital cardiac anomalies requiring surgery. Case 2 had anemia and methylmalonic acidemia requiring dialysis to treat hyperammonemia and metabolic acidosis. For effective medical management, they needed lifesaving
blood transfusions. As a part of alternative treatment, Erythropoietin was administered in both cases. As a result, two babies died from their extremely low hemoglobin and hematocrit. The hemoglobin concentrations below 2.7 g/dL without cardiac problem and 5.4 g/dL with cardiac anomaly complicated by pulmonary hypertension are considered life-threatening hemoglobin threshold. The medical professional must respect and accommodate religious beliefs of the patients who can make informed decisions. However, when parents or legal guardians oppose medical treatment of their babies and incompetent care receivers on cultural and religious grounds, the duty to assist and save persons
exposed to serious danger, particularly life-threatening events must come first.
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KEYWORD
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Blood Transfusion, Methylmalonic acidemia, Jehovah's Witnesses, Anemia
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