Sickle Cell Disease Awareness Article

Monique Simpson, RN

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Sickle Cell Disease, a hereditary condition, poses a significant threat to children, particularly in Africa. This genetic anomaly leads to the production of abnormal hemoglobin and misshapen red blood cells, resulting in excruciating pain and various health issues such as painful crisis episodes, severe anemia, and stroke.

When both parents carry the sickle cell gene, there is a 25% likelihood, or a 1 in 4 chance, of their child being born with sickle cell disease.

Sickle Cell Disease is the most prevalent monogenic (single-gene) disorder worldwide. It is also the most frequent genetic ailment in the United States, affecting 1 in 500 African Americans. Approximately 1 in 13 African Americans carry the sickle cell trait.

In the U.S., the sickle cell disease population is approximately 100,000 and is expected to rise. However, the heaviest burden of SCD is observed in sub-Saharan Africa, where over 75% of all cases occur. In countries like Cameroon, Republic of Congo, Gabon, Ghana, and Nigeria, prevalence ranges between 20% and 30%, while in northern Uganda, it reaches as high as 45%.

Nigeria, Africa's most populous nation, harbors the world's largest SCD population. One in three babies born with SCD globally is Nigerian. Over 40 million Nigerians carry the sickle cell gene, and approximately 150,000 babies are born with SCD in the country annually. Unfortunately, it's reported that 100,000 of these babies do not survive beyond their 5th birthday.

Interestingly, the sickle cell trait provides some defense against malaria. However, those inheriting the gene from both parents lack this protection and experience severe SCD effects, often succumbing before reproductive age.

In many countries where SCD is a public health concern, there are no national programs to control it. Essential patient care facilities are often absent, systematic SCD screening is uncommon, and diagnoses are usually made when severe complications arise.

Treatment options are specialized and costly, including medications, blood transfusions, and stem cell transplants—the latter being the sole known cure for sickle cell anemia.

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