By Karen Stokes
Prithu Sundd, PhD
Senior Investigator, Versiti Blood Research Institute (Photo/Versiti)
According to the Versiti Blood Center webpage, Sickle Cell is an inherited condition that affects 1 out of every 400 African American births. It is caused by an abnormal type of hemoglobin (blood protein) that creates red blood cells that have an abnormal “sickle” shape instead of a normal disc shape. Sickle cells carry less oxygen and can interrupt healthy blood flow, eventually leading to tissue and organ damage.
It also states that Sickle Cell patients may require chronic blood transfusions to treat their disease, and many feel the most healthy after they receive blood. Since 44% of African Americans have Ro blood, providing matched Ro blood to sickle cell patients may provide a safer blood transfusion and additional benefits.
“The majority of blood donation is from the Caucasian population. There’s a mistrust that the African American community has that’s why they don’t show up at the blood donation centers. More sickle cell patients would need more African American or folks of African descent to donate blood. With Caucasian blood there’s a mismatch, and it often leads to an elevated response. There is less of a risk if you give blood from an African American or a donor of African ancestry to an African American,” said Dr Prithu Sundd, Senior Investigator, Versiti Blood Research Institute.
“There is a misunderstanding that Sickle Cell is a Black peoples disease. It’s not a Black people’s disease,” Dr Sundd said. “This disease happened because of a mutation that took place in all parts of the world that was affected by malaria.”
Malaria is a serious and life-threatening infection common in certain parts of the world, including sub-Saharan Africa and India.
According to Dr. Sundd, long ago, people in geographic locations prone to malaria particularly Africa, South America and southern Asia—developed a genetic mutation that altered a protein known as hemoglobin in their red blood cells, which provided some level of protection from the disease. People with this genetic mutation were better at recovering from malaria compared to people without the mutation. Over time, the number of people who carried the mutation grew, building a sort of natural defense towards the disease. There’s now 100 million people who carry this mutation.
Sickle Cell Disease (SCD) affects approximately 100,000 Americans.
• SCD occurs among about 1 out of every 365 Black or African-American births.
• SCD occurs among about 1 out of every 16,300 Hispanic-American births.
• About 1 in 13 Black or African-American babies are born with sickle cell trait (SCT).
SCD occurs more often among people from parts of the world where malaria is or was common. It is believed that people who carry the sickle cell trait are less likely to have severe forms of malaria.
There have been some interesting advancements in Sickle Cell Disease research.
“In December, Gene Therapy was approved which is a big step forward in therapy, Gene Therapy allows a patient to show up at the medical center and they harvest stem cells from the patient and then take cells to the lab and remove the gene and they edit a gene or add a gene that starts producing a different kind of hemoglobin which removes the symptoms of the disease and then they take these cells and put them back into the patient so that’s a big step forward, patients don’t need a matched sibling,” said Dr. Sundd.
“It comes with its own limitations right now because the infrastructure has to be expanded so it is accessible to all patients. I don’t think most of the centers can do more than 10 patients a year which is like a drop in the bucket,” he said. “But it’s a big step forward to curing the disease. The vision is that someday we will have a therapy where the patient will come to the medical center and get a shot and the disease will be cured. There are several issues which need to be addressed and we are working on it and it is a work in progress.”
“Patients should know how to keep themselves safe by keeping themselves hydrated, by keeping themselves from smoking. All this helps. African Americans should be more forthcoming in donating blood to blood banks. Most patients because of donations end up getting Caucasian blood which leads to an elevated response and they end up getting more sick. We need African Americans to come forward so the therapy can be more meaningful, “said Sundd.