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Sickle Cell Disease

GLENN ELLIS | 9/19/2016, 9:55 a.m. | Updated on 9/19/2016, 9:55 a.m.
September is National Sickle Cell Awareness Month. First officially recognized by the federal government in 1983, the national awareness month ...
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September is National Sickle Cell Awareness Month. First officially recognized by the federal government in 1983, the national awareness month calls attention to sickle cell disease, a genetic disease.

Most people who are diagnosed with sickle cell anemia are African Americans. About 1 in every 365 African American children are born with it. Sickle cell disease is a blood disorder that’s inherited – meaning it’s passed down from parents to their children. Babies are born with it when they inherit two abnormal genes – one from each parent. These genes cause the body’s red blood cells to change shape.

Sickle cell disease a chronic disorder causing pain in the extremities and back, infections, organ failure and other tissue damage, skin infections, loss of eyesight, severe blood clots, and strokes.

Normal red blood cells are shaped like discs or donuts with the centers partly scooped out. They are soft and flexible so they can easily move through very small blood vessels and deliver oxygen throughout a person’s body.

Sickle cells are stiffer. Instead of being disc shaped, they’re curved like crescent moons or an old farm tool known as a sickle. That’s where the disease gets its name.

While sickle cell disease primarily affects persons of African descent, it is also present in Portuguese, Spanish, French Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks and Cypriots. Sickle cell disease also appears in Middle Eastern countries and Asia.

With 1910 regarded as the date of the discovery of sickle cell disease, 2010 was the 100th anniversary of that discovery, but just what does it mean to say the disease was “discovered”?

The disorder we call “sickle cell disease,” often abbreviated as SCD, had been present in Africa for at least 5,000 years and has been known by many names in many tribal languages. What we call its “discovery” in 1910 occurred not in Africa, but in the United States.

A young man named Walter Clement Noel from the island of Grenada, a dental student studying in Chicago, went to Dr. James B. Herrick with complaints of pain episodes and symptoms of anemia. Herrick was a cardiologist and not too interested in Noel’s case, so he assigned a resident, Dr. Ernest Irons, to the case.

Irons examined Noel’s blood under the microscope and saw red blood cells he described as “having the shape of a sickle.” When Herrick saw this in the chart, he became interested because he saw that this might be a new, unknown disease. He subsequently published a paper in one of the medical journals in which he used the term “sickle shaped cells.”

Originally from Africa and brought to the Americas by the forced immigration of slaves, it is more frequent where the proportion of African descendants is greater. Carriers of the sickle cell trait have some resistance to the often-fatal malaria. This is why it is found more frequently in persons of Middle Eastern, Indian, Mediterranean and African heritage, because those geographic regions are most prone to malaria.