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Prevention of Stroke in Sickle Cell Anemia

Published online by Cambridge University Press:  01 January 2021

Robert J. Adams
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Extract

Sickle cell anemia (SCD) is a disease characterized by abnormal hemoglobin (Hb) structure. There is a mutation in the beta-globin gene that changes the sixth amino acid from glutamic acid to valine causing the mutated hemoglobin (HbS) to polymerize reversibly when deoxygenated to form a gelatinous network of fibrous polymers that stiffen and distort the red blood cell (RBC) membrane. This leads to episodes of microvascular vasoocclusion and premature RBC destruction leading to hemolytic anemia. For reasons that are unclear, some children develop a large artery vasculopathy (gradual narrowing and ultimate occlusion causing deprivation of blood to the brain — a stroke in other words) involving the intracranial arteries supplying the brain.

The risk of stroke for a child with SCD is many times greater than that of a healthy child without SCD or heart disease. There is a technique that allows the identification of the children with SCD who have high risk even within this relatively high-risk group. And there is a highly effective preventive treatment.

Type
Symposium
Information
Journal of Law, Medicine & Ethics , Volume 42 , Issue 2 , Summer 2014 , pp. 135 - 138
Copyright
Copyright © American Society of Law, Medicine and Ethics 2014

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