Sickle Cell Disease is Such a Complicated Condition Because No Two People Experience the Illness in the Same Way
Sickle cell disease is an inherited red blood cell blood disorder and it’s the most common inherited blood disorder in the United States.
It mostly affects African Americans, but Hispanics, those of Mediterranean descent and even white people can inherit the disease or the trait, meaning they don’t have the condition themselves but can pass it on to their children.
The Centers for Disease Control and Prevention and the National Institutes of Health have said for years that some 100,000 people in the U.S. have sickle cell disease (SCD).
But disease experts point out that number is probably on the low side.
In the Sickle Cell Foundation of Georgia’s (SCFG) first episode of the new TV series “Unveiling Sickle Cell: Beyond the Pain,” host and foundation CEO Tabatha McGee explored the question of how many Georgians and how many Americans overall have the condition with guest Jackie George.
George is the director of advocacy for the SCFG and a certified hemoglobinopathy counselor.
“In the United States, and we’ve been hearing this figure for quite a few years, 100,000. No, it’s more than 100,000 individuals in the United States with sickle cell disease,” George believes.
“Why can’t we find out the true number of individuals living with sickle cell disease here in GA?” McGee asked.
“It may be true in Georgia, but it’s also true in the United States, and this is my personal opinion,” George said. “They call sickle cell an orphan disease. So, when they say an orphan disease it’s not that many, but we know it’s more than that (100,000).”
Another aspect of sickle cell disease that creates complications, especially in treatment, is the disease affects every person differently.
“It varies with each individual and that’s what’s such a big question about sickle cell disease. No two people are alike. You can say that a person with sickle cell disease they’re going to have pain. A person with sickle cell disease may experience acute chest syndrome. They may have a stroke, but everybody’s not going to have that,” George explained.
“Some people with sickle cell disease are on chronic blood transfusion, which means, to prevent a stroke, they have to have a blood transfusion every month. By doing that it can lead to damage to their organs, to their liver, to their heart. So, they have to be monitored,” she continued.
“But the thing about sickle cell is that no two people are alike,” George emphasized.
“No two people are alike, but they may have some of the same problems, so that’s a big problem,” she added.
George recounted a conversation she once had with a warrior, who described the pain from sickle cell disease this way: “Just imagine your tooth aches, the worst tooth ache you’ve ever had in your life being all over your body.”
Sickle cell disease is similar to the name itself. The red blood cells are sickle-shaped and rigid instead of soft and round. That means its harder them to travel through blood vessels and organs.
“What’s happening is those cells are clogging up in any part of the body. Let’s say they clog up the vessels going to the brain, that’s how they can have a stroke. If they’re clogging up in the chest area that’s when they can get that acute chest syndrome which is very, very serious,” George said.
As Tabatha McGee likes to say, “There is hope in every heartbeat.” And it’s true. Progress is being made on treatments for SCD.
In Georgia every newborn is screened for a range of diseases including sickle cell. And it’s important to find out as early as possible if a baby has the illness.
“Now with the newborn screening programs, if these children are born with sickle cell, they’re given a medication that will help those cells not to sickle as much and you want to do it early because the earlier you start, the less problems you will have with organ damage,” George said.
-By Shelby Lin Erdman