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Incidence and Cost of Sickle Cell Disease


Neil B. Minkoff, MD: There are fewer than 100,000 cases of sickle cell disease in the United States annually. Sickle cell disease is a lifelong disease and the severity varies widely from one person to another. While there are effective treatments that can reduce symptoms and prolong life, our panel of experts will discuss the challenges and emerging agents for the treatment of sickle cell disease. I’m Dr Neil Minkoff, the chief medical officer and founder of FountainHead HealthCare in Sudbury, Massachusetts.

Joining me today on this very distinguished panel are Dr Maria Lopes, chief medical director at AMC Health in New York, New York; John Stancil, pharmacy director in the Division of Health Benefits at the North Carolina Department of Health and Human Services in Raleigh, North Carolina; and Dr Ahmar Zaidi, pediatric hematologist-oncologist in the Comprehensive Sickle Cell Center at the Children’s Hospital of Michigan in Detroit, Michigan. Thank you so much for joining us, let’s begin.

I was hoping that we could begin by creating an overview so that we’re all on the same page and to make sure that those who are joining us are all on the same page about sickle cell disease and the differences between that and sickle cell trait. Ahmar, could you go over some of that to help us be grounded in this discussion?

Ahmar U. Zaidi, MD: Absolutely, Neil. Sickle cell disease is a disorder of hemoglobin, which affects the beta globin subunit of hemoglobin. It’s caused by an amino acid substitution that makes hemoglobin more likely to polymerize in states when it’s deoxygenated. The disease requires the inheritance of a gene from both a mom and dad for you to have the full pathophysiologic effect. Sickle cell disease is an umbrella term that includes the inheritance of course of a sickle hemoglobin gene, but also the inheritance of either another sickle hemoglobin gene or an alternate hemoglobin that can interact with a sickle hemoglobin molecule. Sickle cell trait is the term we use to describe somebody who’s only inherited one abnormal sickle hemoglobin gene from one parent and is relatively a benign state.

Neil B. Minkoff, MD: How often does that happen in terms of the predisposition of the 2 different variations? The variation where there’s 1 type of hemoglobin binding with another type.

Ahmar U. Zaidi, MD: As far as, how often does that happen?

Neil B. Minkoff, MD: The comparison between sickle cell trait versus sickle cell disease.

Ahmar U. Zaidi, MD: Got it. As far as incidence goes, Neil, the relative incidence of sickle cell disease in the United States as a whole is about 100,000 to 200,000, in that range. When we talk about the inheritance of trait among African Americans, which is the most largely affected population of individuals, we say that about 1 in 13 African American births will have sickle cell trait, and about 1 in 365 will have disease.

Neil B. Minkoff, MD: I’m going to move over to you Maria and ask if that’s consistent with what you’ve seen as a payer—and you’ve done some work with national payers—and if you’ve seen a geographic distribution pattern?

Maria Lopes, MD, MS: I think as Ahmar pointed out, this is most prevalent in the African American population, Latinos, if we’re dealing with beta value, maybe different populations. But obviously I think one of the challenges as payers is that we may not be really recording, if you will, who has sickle cell trait. So we don’t really know how big the denominator is, and that’s part of the challenge. Many times, we’re reactive to claims, case management, or triggers that are leading up to say a transplant request. Or now you have somebody in the emergency department who’s what we call “a frequent flyer,” and that’s how we often identify them. So I think there’s some missed opportunity perhaps early on to not only identify these patients through claims data, but also opportunities as best practices to connect them to more comprehensive care. As we think about African-American populations and minorities, and as we think about social determinants of health, what is it that we can do? Going back to the geographic variation, rural locations, what is it that we can do to improve access to care?

Neil B. Minkoff, MD: John, do you have anything to add to that?

John C. Stancil, RPh: Our Medicaid program provides healthcare services to about 2.3 million beneficiaries. Of those, approximately 3000 beneficiaries 21 or under have sickle cell disease, and approximately 4000 are adult beneficiaries. And it’s certainly more prevalent in the Southeast. And so in our population, if there are about 100,000 sickle cell disease patients, we represent about 7% of that population.

Neil B. Minkoff, MD: How is that affecting your economic burden? It’s driving cost.

Ahmar U. Zaidi, MD: It’s certainly driving cost, and those costs increase as beneficiaries get older and their disease progresses. Approximately, for the under 21 population, about $7 million was spent in 2018 on prescription cost or medication cost. And almost $15 million for the medical cost, which is mainly hospitalizations and ED [emergency department] visits. As the patient ages or gets older and becomes an adult, those prescription costs increase to nearly $11 million and their medical costs increase to almost $30 million.

Neil B. Minkoff, MD: Across how many patients is that again?

Ahmar U. Zaidi, MD: That’s approximately 7000 patients.

Maria Lopes, MD, MS: There’s actually a recent article on this for the Value in Health that estimates that the economic burden to the United States in total is about $2.98 billion—this may be underestimated—57% of which are hospitalizations or emergency department-related, the rest in outpatients. So there’s certainly a lot of economic burden in terms of what’s happening, and the lifelong journey these patients have.

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