Paper Summary:

This paper presents a detailed overview of sickle cell disease, an inherited disease of the red blood cells, which is present from birth. The paper begins with a brief discussion of the history of the disease, including its discovery. Next the paper investigates the pathophysiological aspects of the disease, presenting the information in easy-to-understand layman terms. The paper then discusses how sickle cell disease affects sufferers physically. Finally the paper presents and evaluates treatment options.

Outline:
Historical Perspective
Pathophysiological Basics of Sickle Cell Disease
Physical Manifestations of Sickle Cell Disease
Treatment options for Sickle Cell Disease
Conclusion

From the Paper:

"Before one can speak in depth on sickle cell disease itself, one must understand the basic pathophysiology which surrounds the condition. Hemoglobin is a protein carried by red cells, which carries oxygen from the lungs for delivery to peripheral tissues. It is composed of two similar proteins, alpha and beta. It is the coordinated action of the alpha and beta globin chains which allow the oxygen transport to occur. These two chains combine to form hemoglobin. During life, except during the very first week of embryonic development, one of the globin chains in an alpha. A developing fetus also has another chain which is a gamma globin; sometimes called non-alpha is present in the fetal circulation. The gamma globin is replaced shortly after birth with the beta, which then chains with the alpha. When two alpha chains combine with two gamma chains, this is called Hemoglobin F, or the common hemoglobin of fetal circulation. Adult hemoglobin, formed of two alpha and beta chains is called Hemoglobin A. If one alpha and one non-alpha chain combine, then this two chain combination is called a dimer and it not functional enough to deliver oxygen to tissues."

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