OnlineMedEd: Pediatrics - "Sickle Cell Disease" Flashcards
What is the “normal” adult hemoglobin?
Hgb A1 (alpha 2 beta 2)
Alpha hemoglobin is on chromosome ______.
16
Beta is on 11. (Think of beta as the number 2 letter in the Greek alphabet and 1 + 1 = 2.)
Beta hemoglobin is on chromosome _______.
16
Fetal hemoglobin is _________.
alpha 2 gamma 2
Think of how babies say “mAMMA.”
What two situations induce sickling?
Hypoxia and acidosis
Those with sickle cell disease have chronically high levels of _____________.
unconjugated bilirubin
This can lead to increased risk of pigmented gallstones.
What hematologic value will be high in those with SCD?
Reticulocyte count
They can make RBCs just fine. It’s a destruction problem.
Those with SCD often need to take what supplements?
Folate and iron
Note, however, that if they receive transfusions then they might become iron overloaded.
What is the Hgb nomenclature for full-on sickle cell disease?
HgbSS
What is the most common organism that causes osteomyelitis in those with SCD?
S. aureus
They are at increased risk of Salmonella osteomyelitis, but Staph is still the most common.
What non-infectious orthopedic malady are those with SCD at increased risk for?
Avascular necrosis of the hip
How should avascular necrosis of the hip be treated?
Conservative management first: NSAIDs, crutches
If this fails, then surgical stabilization.
Acute chest syndrome and focal neurologic deficits are both indications for _______________.
exchange transfusion
For vasooclusive crises that are not FNDs or ACS, you should give ____________.
IVF, O2, and opiates
Also, consider an infectious trigger.
True or false: those with HgbSC disease do not sickle or have vasooclusive crises.
True
