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Sickle cell disease, a blood disorder in which red cells form a sickle shape, is prevalent in people of African descent as well as people of Mediterranean ancestry.

Sickle cell disease isn’t easy to live with.

It is a group of inherited blood disorders in which red blood cells form a sickle shape rather than a round one.The illness is prevalent in people of African descent as well as people of Mediterranean background.

“It primarily affects people of the African diaspora,” said Dr. Kristina Harrell, director of the sickle cell program at Atrium Health Levine Children’s Hospital, “those who are from Africa or Afro Latinos, but it also affects people through what’s called the Malaria Belt. Places in Africa, the Mediterranean, India where malaria is really prevalent, because the trait was really protective against having malaria or severe malaria symptoms, so it tended to thrive in those areas which is why it mostly affects people in those areas or descended from those areas.”

Sickle cell disorder affects about 100,000 people in the United States with one out of 365 Black babies born with the condition every year, according to the Centers for Disease Control and Prevention.

With September designated National Sickle Cell Awareness Month, there are several events held across the U.S. to raise awareness for the disease.

Sickle cell disease
The main function of red blood cells is to transport oxygen from the lungs to all parts of the body.

Sickle-shaped cells causes blood vessels to be blocked because oxygen is not being carried to certain parts of the body. The disease can cause damage and inflammation to major organs such as to the lungs, heart, kidneys, and liver.

“If you think about it, if you were to hold your breath for a prolonged period of time, how that feels, how painful that feels, after a certain point where you have to just start breathing,” Harrell said. “But in this case, you don't have anything to just immediately go in and break up the blockage and so you have that persistent pain until that blockage starts to get alleviated, or you get pain medicine that can help you deal with that pain.”

One in 13 African Americans are born with sickle cell trait according to the CDC, but they typically will not experience any symptoms. Sickle cell disease forms when both parents of a child have the sickle cell trait.

Even though it is often used interchangeably, sickle cell anemia is a more severe form of sickle cell disease. The only difference is the genetic trait.

The slightest thing can cause a survivor to have a sickle cell crisis, a sudden dull or stabbing pain that can last several hours to a series of days.

“So it might be that they can go out and walk or they can play basketball for 15 minutes, but maybe they can’t do it when it’s hot out,” said Dr. John Strouse, medical director of the adult sickle cell program at Duke Sickle Cell Center. “Or maybe they need to take a break after five minutes when they’re playing basketball. So, people usually have an idea that if they got up for a run for half an hour, that's going to trigger a problem.”

The severity of the disease can vary from childhood to adulthood.

A child with a milder form of the disease may not experience many symptoms or health crises until their early teens and adulthood.

However, in a severe case of the disease, a child can end up having kidney disease, heart disease or even a hip replacement before they are in their 60s.

Diagnoses and treatments
Most people are diagnosed at birth with the disorder.

While there is no cure for sickle cell disease, there are pain medications and other treatments an individual can take.

Some sickle cell anemia survivors can receive a blood transfusion every four weeks in which red cells are replaced, Strouse said. “So, there are some people that will be transfused 13 times a year on a regular schedule as a disease modifying treatment by getting their sickle blood low, can really reduce the problems that you see from sickle cell disease.”
The cost of sickle cell treatments does add up.

“So, for our families whose children had the more severe disease, it's likely that they will reach their out-of-pocket max every year, especially if you have more than one hospitalization or for our patients that are on monthly blood transfusions,” Harrell said.

With more innovations and medication, people with sickle cell disease can live up to their 70s or 80s. But the average lifespan is between 42-47 years old according to the American Society of Hematology.

During the pandemic, sickle cell patients have to be very cautious because COVID can cause them to have more health problems. But overall, they are still able to wear face masks and can get vaccinated.

“They are definitely facing a lot of obstacles in terms of just concern for getting COVID because they can have more severe complications with it,” Harrell said. “If they get COVID and have fickling within their lungs, that’s going to cause more complications, more severe pneumonia, and breathing issues. So, they certainly take all the precautions that they can to prevent it, which includes vaccination for those who are eligible, and wearing a mask and social distancing.”

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