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

to be going through sickle cell anemia. And if you want to follow along with written notes on this

topic, you can follow along at zero definals.com or in the hematology section of the

zero to finals medicine book. But let's get straight into it.

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

And a sickle shape is a sort of crescent shape or a moon shape.

And this makes the red blood cells more fragile and more easily destroyed, which leads to a

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

crisis that we're going to talk about later. So what's the path of physiology? Well, hemoglobin is the

protein in red blood cells that transports oxygen, fetal hemoglobin or

HBF, is usually replaced by hemoglobin A or HBA at around six weeks of age, and patients with sick

or cell disease have an abnormal variant called hemoglobin S or HBS.

And HBS causes red blood cells to be an abnormal sickle-shaped.

It's an autosomal recessive condition where there is an abnormal gene for beta-globin on the

chromosome 11.

And one copy of the gene leads to something called sickle cell trait.

And patients with sickle cell trait are usually asymptomatic

and they're just carriers of the abnormal gene.

Two abnormal copies are required for full sickle cell disease.

Briefly let's talk about the relation to malaria and sickle cell disease. Briefly let's talk about the relation to malaria

and sickle cell disease is more common in patients

from areas traditionally affected by malaria,

...