Advances in the Treatment of Sickle Cell Anemia - Episode 2

Burden of Sickle Cell Disease

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Ify Osunkwo, MD, MPH: Biree, let’s talk about the prevalence and the burden of sickle cell disease in the United States and globally.

Biree Andemariam, MD: As many people know, sickle cell disease is really the most common inherited disorder of the red blood cell world-wide. It’s estimated that about 100,000 Americans live with sickle cell disease, but we don’t have a registry, so we’re not sure. That’s based on population estimates. It’s also important to recognize that it’s a global disease, and most of the burden of sickle cell disease is outside of the United States. In fact, it’s estimated that about 300,000 babies are born every year across the globe with sickle cell disease. More than half of the newborns are born in 3 countries, being Nigeria, the Democratic Republic of the Congo, and India.

We have a tremendous burden in this country, in the United States. We have high burden not only in terms of numbers, but also the burden in terms of the impact on individuals living with the disease. I’m sure we’ll get into that. If you talk to patients, one of the things that’s most burdensome is the common manifestation of the disease, being the acute painful crisis episodes, or vaso-occlusive crisis, and that comes out of nowhere. A patient can be feeling fine 1 day and then have a painful episode the next, and it lands them in the hospital or an emergency department.

It causes burdens not only to the individual with the disease, but also their caregivers who might have to take time off from work, and it’s burdensome to society in many ways. It’s very hard to take care of individuals with a chronic condition, in light of recent history of us not having much in the way of treatment options. It’s very expensive. There are estimates that say it costs on an annual basis in the United States about $2 billion, just in the acute care cost associated with sickle cell disease.

Ify Osunkwo, MD, MPH: I can imagine that providers in the community are more likely than not to see a sickle cell patient. Can you speak to what you think the potential is for a doctor in the community getting a new sickle cell patient in their practice?

Biree Andemariam, MD: That’s an excellent question, I think that in the past sickle cell disease was really thought to be mainly a pediatric disease because just a few decades ago children weren’t expected to always make it to adulthood. More recent data suggest that more than 95% of children born with sickle cell disease are expected to survive into adulthood. Which means adult practitioners, whether that be primary care physicians, obstetricians, gynecologists, or adult hematologists, oncologists for that matter, will be seeing more and more individuals with sickle cell disease as the population survives longer.

Ify Osunkwo, MD, MPH: We do have to make sure that we expose and educate our providers about sickle cell disease. I’m going to turn to Dr Shah. Nirmish, how does it present? What are the clinical presentations that sickle cell shows up with, in the emergency department, in the doctor’s office, in the hospital?

Nirmish Shah, MD: I think it’s a good point that was brought up that we’re having more and more patients survive into adulthood. The reason that’s a good point to bring up is that it’s more likely for a practitioner, a hospitalist, a provider in general to see all of the clinical manifestations.

What was brought up is probably the first thing that everyone thinks about, which is pain. When we say vaso-occlusive crisis, we’re talking about a crisis that is causing pain for these patients. If you think about it, sickle cell affects every organ in the body. In that sense we must consider that every organ can be affected by sickle cell disease. Most notably, it’s some of the issues that we really must be concerned about and are extremely problematic, and severe complications include stroke; blood’s not going to the brain, that’s a stroke. If you have blood not going to the lungs, that’s acute chest, 1 of the top causes of death in sickle cell disease. Infection is higher in sickle cell disease, and of course, numerous other complications that are all leading to clinical manifestations that we must consider.

Ify Osunkwo, MD, MPH: The doctors who see them both in primary care and specialty care need to be aware of these potential complications and put the pieces together to truly comprehensively take care of these patients in their practice.

Transcript edited for clarity.