Prevalence and Burden of SCD

Ifeyinwa Osunkwo, MD, MPH: Pat, can you discuss the prevalence of sickle cell disease? Not just in the United States but also around the world. Give us insight into the burden of sickle disease: economically, socially, and medically in these different populations.

Patrick McGann, MD, MS: Many people think of this as an uncommon or rare condition. It’s really not, in this country and across the world. As some people probably know, most patients in the United States who have sickle cell disease are Black or African American because the genetic mutation happened in Africa and places where malaria is highly prevalent. In Africa at least 300,000 babies are born every year with sickle cell disease. Most don’t get diagnosed because they don’t have screening as we do here in the United States. I just saw a statistic that 150,000 children are diagnosed with HIV every year. Twice as many children are born with sickle cell disease and considered the global recognition of both conditions. It is not uncommon at all.

In the United States it also is not uncommon. There are about 3000 babies born with sickle cell disease in the United States. Depending on which state you live in, there is newborn screening and there are tests for 30 or 40 conditions—sickle cell disease is among them—no matter what your race or ethnicity. Everyone gets tested for sickle cell. Of the things that are tested in this country, sickle cell disease is by far the most commonly detected condition by newborn screening. It’s more common than hypothyroidism or PKU [phenylketonuria] or cystic fibrosis or anything else. This is not often recognized because it’s a problem of racial minorities. Along with any other health problem in this country, those who are in a racial minority, like Black or African American, are affected by social determinants of health even more profoundly, so patients with sickle cell, on top of having a chronic medical condition, must face those challenges too. There are lots of comparisons to be made between sickle cell and other diseases. Sickle cell disease was detected, or first described, in 1910, but there were no drugs available for it until the 1980s. It was described as the first molecular disease in 1949, and we still have only a few treatments available. It’s a big problem, way bigger than people recognize both here and across the world.

Ifeyinwa Osunkwo, MD, MPH: Thank you so much, Pat. It’s important for people to realize the profound impact sickle cell has on the individuals affected, not just those individuals but their family members, society in general, the economics of health care, and the economics of society.

Transcript Edited for Clarity