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Understanding Sickle Cell: November 12, 2011

Sickle cell disease occurs in approximately 1 out of every 500 black or African American births, according to the CDC. Most people have heard of it, but may know little about it.

“Contrary to popular belief, people think it’s something you catch. It’s something you’re born with and it usually takes both parents to have the trait,” says Pamela Goldsmith-Denson.

Pamela should know. Her husband has sickle cell and their son inherited the trait. Pamela heads up the Fort Myers Sickle Cell Association. 

“They started doing the baby testings in 1986, that’s when it was mandated. Any child who’s born, when their foot’s pricked in the hospital, sickle cell is one of the diseases that they run they take that blood test,” says Pamela.

Sickle cell is a form of anemia that affects mostly the red blood cells and causes them to change shape.

“What happens with sickle cell in period of what we call, crisis or extreme stress, within the blood, the cells change shape they become sickle shaped. When they become sickle shaped. They are really very rigid so they don’t flow very well through your blood vessels,” says Dr. Emad Salman, a pediatric oncologist/hematologist with Lee Memorial Health System. 

The disease is extremely painful and can result in hospitalizations.

“We give them lots of IV fluids, to hydrate them that way the number of sickle cells that clump together become less and less because there’s more water in between them,” says Dr. Salman.

Drinking water and keeping hydrated is something children with sickle cell disease learn at a young age. But the education process needs to extend to the larger community.

“That’s when we as an association go out and advocate. Any sickle cell client, in order to make those cells move regularly they have to have the oxygen flowing through there and they have to have lots of fluids and so they have to be hydrated at all times,” says Pamela.

There is no cure for sickle cell, but the drug hydroxyurea, which increases hemoglobin, is making a difference.

“We noticed that when you have an increase amount of baby hemoglobin within your red cells, it protects you, you don’t sickle as often,” says Dr. Salman.

Understanding and managing the disease, is helping people with sickle cell live longer, healthier lives.