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Sickle Cell Disease: Dogma, Science, and Clinical Care

Source: www.uic.edu
Topic: Sickle Cell Disease
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Sort Desciption: Sickle cell anemia is a lifelong, dynamic, sometimes debilitating disease. Patients with sickle cell disease often present for surgical management of conditions unrelated or directly attributable to the consequences of the illness. ...

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Sickle cell anemia is a lifelong, dynamic, sometimes debilitating disease. Patients with sickle cell disease often present for surgical management of conditions unrelated or directly attributable to the consequences of the illness. In addition to the usual childhood maladies for which surgical attention is required, children and adults may suffer osteonecrosis, solid organ infarction, pulmonary hypertension, and heart failure. As early as preschool age, these patients are considered immunocompromised secondary to splenic infarction. A small subset of patients with sickle cell disease requires repeated hospitalization and care for multiple associated morbidities. Most historical recommendations regarding the treatment of sickle cell patients in the perioperative period appear to be prudent and rational, and they are generally accepted by our community with little controversy, except with regard to decisions about exchange transfusion therapy. Recommendations to keep the operative arena warm, the patient well hydrated, well oxygenated, and without systemic acidosis are readily accepted. However, the minimal hemoglobin concentration or maximal percent of sickle hemoglobin consistent with perioperative safety are not so well established. Vichinsky et al. (1) published their findings in a comparative trial of conservative versus aggressive transfusion regimens in the perioperative management of sickle cell disease. They concluded that an aggressive protocol of transfusion did not enhance the safety of the patients in the perioperative period. In both groups, approximately 10% of the patients suffered acute chest syndrome despite a difference of hemoglobin S values of 31% versus 59% between the groups. ...

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