It’s hard to say how many adults suffer from sickle cell disease in South Carolina because the government only starting tracking new cases of the inherited blood disorder in 2000.
What is sickle cell disease?
Sickle cell disease is a range of hereditary blood disorders, the most common of which is called sickle cell anemia.
The disease affects the body’s red blood cells, distorting the cell’s shape into an “S” or a sickle shape, according to the National Institutes of Health. The distorted shape blocks blood flow, causing pain and internal organ damage.
The federal government estimates one in 500 African-Americans suffer from sickle cell disease. According to 2012 data compiled by the S.C. Department of Health and Environmental Control, 63 infants were born with confirmed cases of sickle cell disease in the state last year.
“It’s a disease characterized by chronic pain, and the older patients get, the more pain they have. The pain is very real and very terrible,” said Dr. Julie Kanter-Washko, MUSC’s new director of sickle cell disease research.
“On top of that, it’s stigmatized. There’s a lot of people out there who still inappropriately think it’s a disease of drug seekers ... which is the most inaccurate representation of sickle cell.”
The average life expectancy for a patient with sickle cell disease is mid-40s, according to the Sickle Cell Disease Association of America.
But the new director of sickle cell disease research at the Medical University of South Carolina wants to get to the bottom of it.
Dr. Julie Kanter-Washko recently moved to the Lowcountry from Louisiana and intends to make MUSC a leader in treating sickle cell disease.
“We are incredibly fortunate to recruit her,” said Pat Votava, an advocate for the MUSC Children’s Hospital.
Kanter-Washko, a trained pediatric hematologist-oncologist and the former director of the Sickle Cell Center of Southern Louisiana at Tulane University, recently discussed her professional goals with The Post and Courier. Her responses have been edited for length.
Q: Are there similarities between Louisiana and South Carolina, with sickle cell disease specifically?
A: The real problem is resources, so when (patients) age out of childhood, they have no resources — this is whether you’re in Atlanta, New Orleans, South Carolina. There’s very few places that continue to maintain their insurance.
The biggest problem is it becomes a Catch-22. So patients, to maintain insurance, will obtain Social Security disability status, which is highly unfortunate because most of them aren’t disabled and a good portion of them want to work and are very capable of it, but they can’t go to work because they’ll lose their insurance and if they lose their insurance, then they’ll be too sick to work. So it’s a really bad Catch-22.
Q: What are the statistics? Do you have any South Carolina specific numbers?
A: So there is no national registry, which is interesting. It’s very different than a lot of the other genetic diseases where we have really good numbers.
We estimate in the United States about 70,000 to 100,000 are affected by sickle cell disease. In South Carolina ... there’s a very large population of patients with sickle cell disease per population number. So compared to Louisiana, which is a bigger state, there are far more affected individuals here.
Q: Why can’t we pinpoint an exact number?
A: By 2000, all 50 states had to diagnose sickle cell disease by newborn screening. It’s a heel stick. Literally you take blood from the heel. It’s done everywhere. There’s an array of disorders you can diagnose with less than 2 milliliters of blood from that heel stick, one of which is sickle cell disease. Each state has its own different panel of diseases that they diagnose by newborn screening, but some of them are mandatory federal law, and sickle cell is one of those.
Q: So we can track how many babies are born in South Carolina now, but that wasn’t required before 2000, so it’s hard to pinpoint the total number?
A: Prevalence data is minimal.
Sickle cell disease, it’s really incredibly underfunded. It’s sad and pathetic, actually, how underfunded it is. It’s a disease that we’ve known about since 1910 ... and yet despite the fact that it’s been around for 103 years, we only have one FDA-approved drug that actually helps the disease and no registry, no really strong national support system and it’s underfunded compared to cystic fibrosis, which is a good comparison because cystic fibrosis is also a genetic disease with mortality that tends to be in that third and fourth decade of life, with limited treatment options as patients get older.
And if you compare their funding ... it’s something like 5 to 1. It’s something like for every $500 a cystic fibrosis patient gets, a sickle cell patient gets $10 — 50 to one. I was off by a factor. It is incredibly — it’s a horrible health disparity.
Q: What made the move to MUSC seem like the right fit?
A: New Orleans is a difficult place to work. It’s just not a progressive atmosphere to improving the care of children, to improving the care of anyone, but specifically children.
Q: Is that Louisiana as a whole, or is that just New Orleans?
A: I think it’s Louisiana as a whole. I think there’s some areas that are improving. They have a fabulous new director of Medicaid — I can’t say enough good things about. I really think she’s going to make improvements.
Q: Is there reason to believe attention paid toward sickle cell disease by the medical community is improving?
A: Sickle cell is a hot topic nationally. Unfortunately the reason it’s a hot topic — I’m glad it’s a hot topic because it’s getting attention finally — but the reason for that is readmission rates. Now, no one wants to pay for 30-day hospital readmissions.
Sickle cell in Louisiana was the second-highest cause of readmission rates. In South Carolina, it’s at the top three. I don’t know what position it holds. From a financial standpoint, which is where a lot of medicine is headed right this second, sickle cell has become a hot topic.
Q: How would you like to change the treatment of this disease?
A: I want to design a bundled care plan ... where patients with sickle cell disease are insured throughout their lives.
It crosses both sides of the party lines. It’s the right thing to do. We should have access to health care. It’s also, on the more conservative side of things, it’s a good economic model. Patients should work. Especially on the very conservative side, everybody wants you to pull up your bootstraps and work, well, nobody is giving these people an opportunity to work.
Reach Lauren Sausser at 937-5598.
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