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Another Voice: More attention needs to be paid to sickle cell disease

By Betty W. Carrington

As Congress continues to debate health care insurance coverage formats, great concern is felt by individuals and families impacted by sickle cell disease, an inherited blood disorder that is serious in nature and life-shortening. Identified in 1910 by Chicago physician James B. Herrick, who first published a description of sickled cells in a blood sample from a 20-year-old dental student, the term “sickle cell anemia” was coined.

It is a pre-existing condition that was covered under the Affordable Care Act. Now, debate has been heard about whether pre-existing conditions should be covered, and if so, which ones, and should states be allowed to determine that coverage. Changes are also being discussed about Medicaid and how that should be reduced.

Sickle cell disease is the result of one abnormal gene from each parent, which results in the protein hemoglobin, the substance causing the red color in red blood cells, to go through a chemical process resulting in change from a round red cell into the shape of a sickle.

While the body, specifically the spleen, struggles to produce needed red blood cells, those that are sickle-shaped break down in 18 to 20 days, whereas normal red blood cells remain about 120 days. Most importantly, sickled cells aggregate in the blood vessels, blocking the flow of blood and oxygen delivery to all parts of the body.

The significance is that normal red blood cells take oxygen to all organs and parts of the body. Without oxygen, organs are damaged and disabilities occur beginning with early childhood, including infections, strokes, joint pain and destruction, blindness, kidney failure, acute chest syndrome and constant anemia. Persons so affected suffer painful crises that often necessitate days to weeks of hospitalization. The crises can often interfere with education, work and lifelong plans.

Sickle cell disorders vary in type and severity and are a global issue, affecting primarily people of color. Why is it that since 1910, only one medication has been studied and is in use to reduce the cascade of destructive events during a sickle cell crisis? Why is it that hydroxyurea is the only medication that is available that keeps red blood cells round and increases fetal hemoglobin, which everyone has at some low level since birth? Why is there a paucity of research to find new medications and even a universal cure for sickle cell disease?

Why is it that care for children in hospital pediatric units is available but appropriate care for persons who have aged out of pediatric care must be sought by the individual and is hard to locate? Why is it that sickle cell disease is underresearched, underfunded for hospital care and poorly supported by the general community?

June 19 of every year was declared World Sickle Cell Disease Day by the United Nations in 2008. It is time for this inherited and pre-existing condition to be adequately supported by the general community and any federal health plan developed by Congress.

Betty W. Carrington is second vice president of the Queens Sickle Cell Advocacy Network.

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