Hello and welcome to the Zero to Finals podcast. My name is Tom and in this episode I'm going

to be talking to you about sickle cell anemia. And if you want to follow along with written

notes on this topic, you can follow along at zero definals.com slash sickle cell anemia or in

the hematology section of the zero definals pediatrics book.

So let's get straight into it.

Sickle cell anemia is a genetic condition that causes sickle or crescent shaped red blood cells.

This makes the red blood cells more fragile and easily destroyed, leading to hemolysis and hemolytic anemia.

Patients with sickle cell anemia are prone to various types of sickle cell crisis.

Let's start with some basic pathophysiology. Hemoglobin is the protein in red blood cells that

transports oxygen around the body.

Fetal hemoglobin, or HBF, is usually replaced by adult hemoglobin or HBA around six weeks of age.

Patients with sickle cell disease have an abnormal variant called hemoglobin S.

Hemoglobin S causes red blood cells to become an abnormal

sickle shape. Sickle cell anemia is an autosomal recessive condition where there's an abnormal

gene for the beta globin on chromosome 11. One copy of the gene results in sickle cell trait. Patients that have sickle cell trait are

usually asymptomatic. Two abnormal copies of the gene are required for sickle cell disease.

Let's talk briefly about the relationship between sickle cell disease and malaria.

Sickle cell disease is more common in patients from areas traditionally affected by malaria,

such as Africa, India, the Middle East and the Caribbean.

Having one copy of the gene, meaning they have sickle cell trait,

reduces the severity of malaria.

As a result, patients with sickle cell trait are more likely

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