Life expectancy for people with sickle cell disease appears to be declining, and the same systemic racism being protested around the nation could be at least partly responsible, a local expert says.
“If you compare other orphan diseases, like cystic fibrosis or hemophilia, which have less cases than sickle cell, and then compare funding and research, it’s a night-and-day difference,” said Dr. Alan R. Anderson, director of Prisma Health Upstate’s Comprehensive Sickle Cell Disease Program.
The U.S. Food and Drug Administration defines an orphan disease as a condition that affects fewer than 200,000 people nationwide.
“That directly goes along with … this same prejudice, racial disparities that we see in our political-social landscape,” he said. “It’s about time we recognize this is a serious problem.”
Anderson points to a National Institutes of Health study that concluded that life expectancy dropped from 42 for men and 48 for women in the early 1990s to 42 for women and 38 for men in 2005 — even as life expectancy increased for people with other chronic conditions.
And a Sept. 1 New England Journal of Medicine article reports that while cystic fibrosis affects a third fewer than sickle cell, it receives seven to 11 times the funding. The authors added that “the development of disease-modifying therapies has stagnated because of inadequate research funding, attributable at least in part to structural racism.”
Sickle cell disease (SCD) is a genetic disorder of the red blood cells that primarily affects African Americans, though people from Hispanic, southern European, Middle Eastern, southern Asia, or Asian Indian backgrounds can also get the disease. Both parents must carry the genes for their child to get the disease.
While healthy cells are round, in people with SCD they resemble a sickle, or C shape, and are also hard and sticky, clogging the flow of blood through the vessels, according to the U.S. Centers for Disease Control and Prevention. Symptoms include pain, infections and stroke, and typically begin when a child is around 5 months old.
Treatments include medicines that can reduce complications and extend life, the agency reports, but the only cure is a risky bone marrow or stem cell transplant from a close match, like a brother or sister.
The state Department of Health and Environmental Control says that despite improvements in treatments, SCD remains “a life-limiting disease with multi-organ complications that reduces the quality of life of impacted individuals especially as the person ages.”
DHEC also reports that SCD has suffered from “decades of poor disease awareness and lack of funding compared to other inherited disorders.”
Historically, Anderson said, while children are cared for via Medicaid, adults often lack insurance so they have no medical home. And along with the funding disparity, there is a lack of training for medical professionals about SCD.
“This is not rocket science,” he said. “If you focus on sickle cell disease like you do on diabetes and other chronic diseases, we will see reductions in acute care needs that will ultimately manifest in life expectancy.”
DHEC has an SCD plan that calls for improving access to care, sustainable funding and increasing the educational awareness of medical professionals about SCD.
“It’s going to take more research and more advocacy and, I believe, policy changes,” said the Rev. Sean Dogan of Long Branch Baptist Church in Greenville. “We need that same momentum for SCD as well.”
Anderson said that Prisma’s program has stepped up preventive health strategies and treatments for people of all ages while reducing ER visits and hospitalizations by about 50%, which will hopefully increase life expectancy.
Last year, it launched Camp Crescent to provide SCD patients and their families a respite from the disease. But the pandemic prevented that this year.
So organizers are holding a block party from 4-6 p.m. on Sept. 19 where people can safely gather for some fun and to raise awareness, said Dogan.
“It’s going to be an exciting event … to raise awareness [and] celebrate those who have SCD and their caregivers,” he said. “The more awareness the community has, the more support it will give. And our community is a very generous community.”
Modeled on the drive-by birthday parties so popular during the pandemic, it will feature patients and their families driving by the entrance of Prisma’s Cancer Center on Faris Road, he said. There, they’ll find encouragement, well wishes and fun activities like quizzes with prizes, he said.
Meanwhile, on Sunday, blood drives to benefit patients will be held in the area, including one at Long Branch from 10 a.m. to 2 p.m., he said.
To learn more about the event, go to http://events.r20.constantcontact.com/register/event?oeidk=a07eh9btc8wd9af19f2&llr=76g5y7tab or https://www.ghschildrens.org/programs/camp-crescent/.
While the exact number of people with SCD is unknown, it’s estimated they number about 100,000 nationwide.
South Carolina doesn’t track SCD numbers, but between 1991 and 2017, 1,884 infants were born with it, DHEC said. Another 56,607 were born with sickle cell trait, which means they don’t have symptoms but can pass the gene on to their children.