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Issues & Controversies of Sickle Cell Disease

Sickle cell disease is a serious and life threatening genetic disease. According to the National Human Genome Research Institute it is the most common inherited blood disorder in the United States, affecting more than 80,000 people. Because of its severity, and because it affects primarily the African American community, the topic of sickle cell disease is a hotbed of controversy. But for those affected, the pain and difficulty they face everyday are just as big an issue.

  1. Sickle Cell

    • Sickle cell disease is a genetic mutation of hemoglobin, a protein in your red blood cells. Mutated hemoglobin forms stiff, stick-like structures that cause red blood cells take on a sickle shape. This shape can cause the cells to stick together and become backed up, leaving organs starved for oxygen.

    Crisis

    • A sickle cell crisis happens when malformed red blood cells block the flow of blood to an organ or part of the body. A crisis can happen suddenly, without any warning and can happen in many parts of the body. The lungs, leading to severe chest pain and fits of coughing up blood. The spleen, which cleans and filters blood can be affected, resulting in high risk of infection. The bones, joints, stomach, and eyes are also very often affected in a sickle cell crisis.

    Treatments

    • Treatments include blood transfusions to replace sickled cells and antibiotics for frequent bacterial infections. The anti-tumor drug hydroxeurea stimulates the production of fetal hemoglobin which helps prevent sickling. This drug has proven successful in treating sickle cell disease.

    Cure

    • Bone marrow transplant, however complicated and risky, is the only cure for sickle cell disease. Bone marrow must be donated by a matched sibling, but according to the National Human Genome Research Institute because siblings are so often carriers of the disease there is only an eighteen percent chance of a healthy, sibling match.

    African Americans

    • World wide, sickle cell disease is most common in west and central Africa where, according to SickleCellDisease.org, twenty-five percent of the population carries the gene. SickleCellDisease.org also states that one to two percent of all children are born with some form of the disease. In the United States, the sickle cell gene is carried by one in twelve African American adults according to the National Human Genome Research Institute. They also state that a parent with the defective gene has a twenty-five percent chance of passing it on to their children.

    Genetics

    • Genetic testing is a sensitive issue to anyone suffering from a serious genetic disease. Issues arise with health care, life insurance, and even in the workplace. Because this disease is so serious and can be fatal, genetic testing is often discussed as a preventative measure. But according to a paper written by Tim Freisen from North Dakota Sate University, there are many ethical questions raised by genetic testing. Issues arise ranging from who decides who gets tested and what should the consequences be for sickle cell carriers. Freisen states that mass testing done by the government in the seventies raised fears about eugenics. As we rocket into the modern era, it is unlikely debates about genetic testing will become any less intense. Those carrying the sickle cell trait can only hope a surefire cure makes testing less controversial.

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