Sicke Cell Anemia

Blue eyes, brown curly hair, cleft-chin, and hemoglobin A/S are traits that make a person normal and a carrier of the sickle cell trait. Blues eyes, brown curly hair, cleft-chin, and hemoglobin S/S means a person has sickle cell Anemia.

What is sickle cell anemia? Dr. Joyce Lloyd, a research professor in the VCU Human and Molecular Genetics Department says sickle cell disease is a genetic mutation of the red blood cells. It occurs when hemoglobin within the red blood cells fail to function normally. Hemoglobin (Hb) is what makes the red blood cells red. It is made up of protein and iron.

In a normal red blood cell the hemoglobin flows freely in a circular motion forming the round shape. Abnormal hemoglobin sticks together and stretches the cell out. The cells become rigid and curves into sickle-like shape. This shape makes it difficult for cells to pass through small blood vessels. They become clogged preventing oxygen and nutrients in the blood from reaching organs and tissues.

Dr. Joyce Lloyd says that a child is diagnosed with sickle cell disease when they inherit the hemoglobin S trait from both parents. When a mother and father both have the sickle cell trait there is a 25 percent chance that their child will have sickle cell disease.

The parents of a child with sickle cell anemia do not have the disease. They are healthy and would just be considered carriers of the trait because there is only a small dose of sickle cell, not enough to change the shape of the cell. Dr. Lloyd explains that their genotype would be made up of hemoglobin A, the normal adult hemoglobin, and hemoglobin S or C, which are sickle cell traits.

Health officials say people who carry the sickle cell trait are said to be at an advantage because their immune system can resist diseases such as Malaria.

“In a country where Malaria is rampant, it is better to be hemoglobin A/S than hemoglobin A/A,” says Dr. Lloyd.

According to the Virginia Department of Health’s Sickle Cell Disease Awareness Program sickle cell is one of the most common genetic disorders in the United States affecting mostly African Americans. It is also found in people from South and Central America, the Middle East, India, Italy, Greece and Turkey.

In Virginia, one in 325 babies born to African-American parents is affected by Sickle Cell Disease. That is 13 percent higher than the national average. It is estimated that more than 155,000 Virginian's have sickle cell trait. It is estimated that more than 4,000 Virginian's are living with Sickle Cell Disease.

People with Sickle cell anemia can suffer from many severe complications. Because the crescent shaped cells can block the flow of blood and oxygen anywhere in the body. Jean Shipman, Program Manager for the Sickle Cell Anemia Awareness Program says that people can suffer from extreme pain from where ever the blood is not flowing. In the circulatory system, if blood stops flowing to the brain, strokes are likely to happen.

“We have young people before there’re four years of age that have had strokes, sickle related strokes,” says Shipman.

Some people experience a breakdown of their major joints, tissue damage and organ damage.

A young woman, Jane, shares her personal story about living with sickle cell anemia on Revolution Health, a website dedicated to different diseases and conditions.

“I was born with sickle cell anemia…Sometimes it can be just downright hard growing up wanting play with your friends and go outside but didn't understand why you just couldn’t, but as you get older you know that you had a lot of hospital visits, stayed in pain then you do research for yourself and discover that sickle cell anemia is your problem.”

It is debatable whether there is a cure for Sickle Cell Anemia. Shipman says yes there is a cure. Bone marrow transplants are the cure for sickle cell disease. But there are consequences to this procedure and because of it Dr. Lloyd says it is a risk.

“If I had to give a short answer, no, not now. It is treatable, but it is not curable.”

Bone marrow transplants are difficult because donors and matches are rare. Also side effects can cause the immune system to react negatively, says Dr. Lloyd.

There are a number of treatments and services provided to help improve the life expectancy of people with sickle cell disease. By two months of age Newborns are treated with Penicillin. Penicillin is one of the key treatments to help fight off infections, says Shipman.

White blood cells, antibodies for infections, are not able to pass once arteries become blocked, so when people with sickle cells get sick their bodies have a difficult time fighting infections. Shipman also mentions Hydroxyurea, an anti-sickling drug that helps the body maintain fetal hemoglobin. It reduces red blood cells from sickling as much. Hydroxyurea is only used on adults.

The sickle cell disease may seem rare to many people, but it is very common, especially in the African American community. A cure is in the works, treatment is saving lives, but the best thing you can do is educate yourself on this disease and become aware.

To find out more information about Sickle Cell Anemia visit the Sickle Cell Disease Awareness Program on the Virginia Department of Health website.