Management of sickle cell disease: what to do when there is no evidence base

Published on May 16, 2019   34 min

Other Talks in the Category: Diseases, Disorders & Treatments

0:00
I'm Sophie Lanzkron, I am the Director of the Sickle Cell Center for Adults at Johns Hopkins, and I'm going to talk about the "Management of Sickle Cell Disease" today.
0:10
I'm going to start by describing the pathophysiology of vaso-occlusive crises in sickle cell disease, talk a little bit about recognizing the clinical presentation of vaso-occlusive crisis, and then talk about guideline-based care for adults with sickle cell disease.
0:25
I'm going to do that in the context of a patient. So let me introduce you to JP. He's a 23-year-old male with hemoglobin SS disease. He presents reporting typical vaso-occlusive crisis type pain in his arms and legs. He was last hospitalized two weeks ago. His medications include hydromorphone, 4 mg every 4-6 hours as needed, and folic acid 1 mg a day. He's talking to his mom when you walk in the room, when he sees you he crawls into a ball and starts to complain of 8/10 pain.
0:54
Sickle cell anemia is due to a single base substitution of a valine for a glutamine at the sixth amino acid of the gene encoding for the hemoglobin Beta- chain. It affects approximately 100,000 Americans, but millions are affected worldwide. In the United States compared to the general population, it decreases life expectancy by 25-30 years. Interestingly, a recent study shows that the sickle mutation occurred once 7,300 years ago, and the reason that the gene mutations has survived is because having sickle trait provides a significant survival advantage against Falciparum malaria. So if you are born with sickle trait, you're far more likely to survive having malaria than if you don't have sickle trait.
1:36
Here is the hemoglobin molecule. It is made up of two Alpha-chains and two Beta-chains. The mutation we're talking about occurs in the Beta-globin chain.
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Management of sickle cell disease: what to do when there is no evidence base

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