10 Things You Should Know About Sickle Cell Disease

Sickle cell disease is an inherited form of anemia where red blood cells become abnormally long and pointed, similar to the shape of a banana. It affects approximately 100,000 people in the United States and millions worldwide.

In the U.S. it occurs in about one out of every 365 African-American births and more rarely in Hispanic-American births. Although sickle cell disease is not an extremely rare condition, there are some lesser-known facts and misconceptions everyone should know.


It Can Occur in Any Race

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Although sickle cell disease has long been associated with people of African descent, it can be found in many races and ethnic groups, including Spanish, Brazilian, Indian, and even Caucasian. Because of this fact, all babies born in the United States are tested for this condition. 


An Inherited Disease

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Sickle cell disease is not contagious like a cold. People are either born with it or they are not. If you are born with sickle cell disease both of your parents have sickle cell trait (or one parent with sickle cell trait and the other with another hemoglobin trait).

People with sickle cell trait cannot develop sickle cell disease.   


Diagnosed at Birth

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In the United States, every baby is tested for sickle cell disease. This is part of the newborn screen performed shortly after birth. Identifying children with sickle cell disease in infancy can prevent serious complications. 


The Sickle Cell and Malaria Connection

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People with sickle cell trait can be found most heavily in areas of the world that have malaria. This is because sickle cell trait can protect a person from becoming infected with malaria.

This doesn't mean a person with sickle cell trait cannot be infected with malaria, but it is less common than a person without sickle cell trait. 


Not All Types Are Created Equal

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There are different types of sickle cell disease which vary in severity. Hemoglobin SS (also the most common type) and sickle beta zero thalassemia are the most severe followed by hemoglobin SC and sickle beta plus thalassemia.


More Than Just Pain

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There is a lot more to sickle cell disease than just painful crises. Sickle cell disease is a disorder of the red blood cells, which supply oxygen to all the organs.

Because sickle cell disease occurs in the blood, every organ in the body can be affected. Patients with SCD are at risk for stroke, eye disease, gallstones, serious bacterial infections, and anemia, to name a few.  


Children at Risk for Stroke

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Although all people with sickle cell disease are at risk for stroke, children with sickle cell disease have a much higher risk than the children without sickle cell disease.

Because of this risk, physicians who treat children with sickle cell disease use an ultrasound of the brain to screen and determine who is at highest risk of stroke and start treatment to prevent this complication. 


An Antibiotic Changes Life Expectancy

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The antibiotic penicillin is life-saving. People with sickle cell disease are at increased risk of serious bacterial infections.

Starting penicillin twice a day for the first five years of life has changed the course of this condition from something only seen in children into a condition people live into adulthood with.


Treatments Are Available

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There is more than pain medication for treating sickle cell disease. Today, blood transfusions and a medicine called hydroxyurea are changing the lives of people with sickle cell. 

These therapies are allowing people with sickle cell disease to live longer lives with fewer complications. Multiple research studies are ongoing to find additional treatment options.


There Is a Cure

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Bone marrow (also called stem cell) transplantation is the only cure. The best success has come from donors who are siblings whose genetic makeup matches the person with sickle cell disease.

Sometimes types of donors, like unrelated individuals or parents, are used but mostly in clinical research studies. In the coming years, gene therapy looks like a promising treatment.  

If you or your family member has sickle cell disease, it is important to have regular follow-up with a physician to ensure up to date care is provided.

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  1. National Heart, Lung, and Blood Institute. Sickle cell disease.

  2. Gong L, Parikh S, Rosenthal PJ, Greenhouse B. Biochemical and immunological mechanisms by which sickle cell trait protects against malaria. Malar J. 2013;12:317. doi:10.1186/1475-2875-12-317