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Sickle Cell Disease 

Sickle Cell Disease
Chapter:
Sickle Cell Disease
Author(s):

Andrew Franklin

DOI:
10.1093/med/9780190226459.003.0084
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date: 23 February 2020

Sickle cell disease, a hemoglobinopathy that affects multiple organ systems, is a complex disease entity that presents unique challenges during the perioperative period. The hallmark of sickle cell disease, vaso-occlusion, results from sickling of erythrocytes containing hemoglobin of abnormal conformation due to genetically mutated beta globin genes. The perioperative clinician must properly care for acute sickle cell crises including acute painful episodes and acute chest syndrome, and safely care for the sickle cell patient through the preoperative, intraoperative, and postoperative phases of surgical treatment. Both acute painful episodes and acute chest syndrome result from vaso-occlusive crises, and early stabilization of these emergencies is crucial to ensuring a positive patient outcome. The singular perioperative objective for the care of sickle cell disease patients is both simple and daunting: to achieve physiologic homeostasis in patients with preexisting multiorgan dysfunction undergoing a series of physiologic insults during and after surgery.

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