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Sickle cell

Labs
1. SCD diagnosis based on % of Hgb S on electrophoresis; person
with SCD may have 80-100% HbS (% doesnt change during
crisis)
2. The # of RBCs with permanent sticking may be 90% in persons
with SCD (normal < 1%).
Complications during crisis:
Persons with SCD have a normally low Hct at 20-30%; Hct values
decrease further during crisis or when bone marrow fails to
produce cells during stress.
A high reticulocyte count indicates chronic anemia d/t bone
marrow releasing immature RBCs (reticulocytes) to make up for
low Hct.
Bilirubin levels are high d/t Hgb being released from damaged
and dead RBCs; Hgb breaks down into iron and heme molecules;
the heme molecules metabolize into free bilirubin.
A total WBC count is usually high d/t chronic inflammation.
Imaging
X-ray of skull shows Crew cut appearance d/t bone surface cell
destruction and new groth .
X-ray of joints may show necrosis and destruction
Ultrasound, CT, PET, and MRI, show soft tissues and organ
changes d/t poor oxygenation and chronic inflammation
Other DX
ECG changes are r/t the area of the heart that is damaged
Echocardiograms show cardiomyopathy and decreased cardiac
output

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