Now, what is this vaso-occlusive crisis? This is a pain crisis.
To the point where - pay attention. 100% SS.
What does that mean? Homozygous.
Which one is this? Is it the trait or the disease? Disease. Good.
And so how will the boards perhaps give you a question here?
Excess opioid consumption.
Uh-oh, who's at risk in the brain? What center? Good.
The respiratory center is at risk.
Shut it down, you'll worry about hypoventilation. Clear?
Let's continue. Acute chest syndrome, all part of your vaso-occlusive crisis.
Hypoxemia caused by the lack of blood flow.
Understand the concept then plug in the organ systems. Stroke. You tell me why.
Vaso-occlusive crisis up in the carotid.
Maybe there isn't sufficient blood flow. No joke.
Autosplenectomy, what happened? Spleen dies. Extravascular hemolysis.
How would you know the spleen is being involved histologically?
It's called the Howell-Jolly body.
You're going to find this very dark basophilic inclusion inside the cell
which to you means, "Oh, no, the spleen is now involved."
You're worried about what kind of organisms? Encapsulated.
Give me one organism that will then start of a cascade
of that list of encapsulated organism.
Streptococcus pneumonia, Klebsiella pneumonia, haemophilus influenzae. Right?
Those are encapsulating.
And then we talked about 100% of those sickle cell disease patients,
autosplenectomized by adulthood.
Painful priapism all part of vaso-occlusive crisis.
What's that mean? For hours there's painful erection.