“Growing up, my mother worked for our local sickle cell foundation so I have always known people living with sickle cell. When I was asked to be part of this campaign, I said ‘yes,’” R&B crooner Ruben Studdard tells Real Health. This year, Studdard is the spokesperson for The Be Sickle Smart campaign. Sponsored by Novartis Pharmaceuticals Corporation, the national campaign includes a seven-city tour to Jacksonville, Birmingham, Philadelphia, Chicago, St. Louis, Atlanta and Los Angeles. A highlight of the tour, Studdard meets members of the community who also get to attend a CD launch party for the Lighten the Load inspirational album. “So many have come up to me and said that this campaign has given them a voice,” Studdard says.

Researchers estimate that more than 70,000 Americans suffer from sickle cell disease (SCD) and most of them are black. Also, one in 12 African Americans carries the sickle cell disease trait. Here, Willarda V. Edwards, MD, MBA, the SCDAA’S president, and Lanetta Jordan, MD, MPH, SCDAA’s chief medical officer, discuss what you need to know about the illness.

What is sickle cell disease?
 (SCD)is an inherited blood disorder that affects red blood cells. People with SCD have red blood cells that contain mostly hemoglobin S, an abnormal type of hemoglobin.

“The blood cells are typically shaped like a doughnut and can easily go through the vessels,” Dr. Jordan explains. “But with SCD, the blood cells are shaped like sickles that have a hard time traveling through [the blood vessels] or get trapped in the capillaries and block blood vessels.”

How does sickle cell disease affect the body?
When the sickle-shaped cells block small blood vessels, less blood and oxygen reaches certain parts of the body. That in turn leads to tissue damage, which causes the complications of SCD.

“This is a very serious disease with very devastating symptoms,” Jordan warns. “People, including children, can develop hand-foot syndrome (pain, swelling, numbness, tingling, or redness of the hands or feet), enlarged spleens, infections, strokes, develop lung issues and asthma, pulmonary hypertension and gall stones to name a few.”

What are some common misconceptions about sickle cell disease?
“So many people think that this is a rare disease and it’s not,” Edwards says. Jordan adds, “Also, people think that there’s a cure, but there is no cure and people do die from this disease every year.”

How do you know if you have sickle cell disease?
“Nowadays, everywhere in the U.S. it is required to test newborns, so that is when most families will find out if their baby has the disease,” says Edwards. “It’s estimated that one in 500 babies born in the U.S. have sickle cell.”

The test for sickle cell disease won’t hurt your child—it’s a simple relatively painless blood test. If your child does test positive for sickle cell, doctors will start treatment early. “Most likely the doctor will start your infant on a penicillin treatment,” says Edwards. Most infants don’t show signs of the disease until they are four months old.

What are some other treatments for sickle cell disease?
To maintain optimum health levels, treatment of sickle cell disease often includes antibiotics, pain management, intravenous fluids, blood transfusion and surgery. Droxia—a cancer-fighting drug approved to treat sickle cell—helps reduce severe pain, acute chest syndrome and the need for blood transfusions in adult patients.

“While [bone marrow] transplants have cured some people of SCD, that is rare, and gene therapy is still in the future so we don’t want to rely on those alone,” says Jordan. “SCD can be manageable with good preventive care, follow-up care and education.”

And the good news is that people are living longer with the disease—beyond their teen years and into their 50s, which is a vast improvement from decades ago.

What is sickle cell trait?
Sickle cell trait is when a person is a carrier for the trait, but does not express it.
“A person with sickle cell trait who has a baby with someone who is also a carrier has a 25 percent chance of having a baby with sickle cell,” says Edwards.

What is iron overload and how is it related to SCD?
Although blood transfusions can improve the quality of life for people with sickle cell disease, they introduce extra iron into the body, especially for those who have 10 or more transfusions. Because the excess iron does not go away by itself, it can build up causing damage to the heart, liver and other organs.

“You don’t feel iron overload, so we educate families to make sure those with sickle cell know their iron status,” says Jordan. Speak to your doctor about available treatments to eliminate iron overload.

What’s most important for people who do not have sickle cell disease to know?
“Know your trait status,” Jordan says.  “That way if you have the trait, you know there’s a possibility of having a child with the trait or disease and you’ll know what the disease will entail.”

Learn more about SCD and iron overload at sicklecelldisease.org.