Two Cases of Methylmalonic Acidemia where Refusal to Blood Transfusion Led to Death

종교상의 이유로 수혈을 거부하여 사망한 메틸 말로닌산 혈증 환아 2례

  • Jang, Ha Won (Department of Pediatrics, Chungnam National University Hospital) ;
  • Lee, Yong Wook (Department of Pediatrics, Chungnam National University Hospital) ;
  • Chang, Meayoung (Department of Pediatrics, Chungnam National University Hospital) ;
  • Kil, Hong Ryang (Department of Pediatrics, Chungnam National University Hospital) ;
  • Kim, Sook Za (Department of Pediatrics, Chungnam National University Hospital)
  • 장하원 (충남대학교병원 소아청소년과) ;
  • 이용욱 (충남대학교병원 소아청소년과) ;
  • 장미영 (충남대학교병원 소아청소년과) ;
  • 길홍량 (충남대학교병원 소아청소년과) ;
  • 김숙자 (충남대학교병원 소아청소년과)
  • Published : 2018.08.30

Abstract

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 life-saving 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.

Keywords

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