Welcome back to the Internet Book of Critical Care Podcast. I'm here with Adam Thomas, and we're going to talk about sickle cell acute chest syndrome.

And this is one of those things that we might see a lot or we might see not so much. Josh and I were talking offline here, but in both our training, we saw more of this. So today we'll

cover the pathophysiology, the definition diagnosis, and then we'll talk about the management

and treatment, and treatment is going to be cool because we all heard about textbooks with

sickle cell crisis where you do exchange transfusions, but you're not always in a place that can do

that. So Josh, let's get straight into the pathophysiology around sickling transfusions, but you're not always in a place that can do that. So, Josh,

let's get straight into the pathophysiology around sickling in general. But we're going to focus on

acute chest syndrome in general because that's the ICU level why people get in a lot of trouble.

Yeah, so just to start off a little bit, just some nomenclature here, this confused me somewhat.

So sickle cell anemia is a genetic disorder, most common genetic disease in the

world that involves a mutation in the hemoglobin gene, which causes hemoglobin to polymerize

and essentially cause the RBCs to take on this crescentic sickle shape, which can cause them

to clot and obstruct capillars. Now, there are also patients with sickle cell disease who are

compounded heterozygo to have one copy of a sickle hemoglobin gene

and another copy of hemoglobin C or beta thalcemia. And those patients can still manifest

sickling and clinically behave very similar to sickle cell anemia. So generally we're going to be

talking about sickle cell disease, which is this umbrella term for all of these different patients.

But mostly sickle cell disease gets into trouble when you start getting vaso-oclusive crises,

at least from a critical care perspective.

So let's talk about what will bring patients in with vaso-eclusive crises, knowing that a lot of

these patients manage at home themselves with a really good pain control, really

good hydration and standing orders of antimicrobial.

...