Basics
What abnormal Hgb replaces normal Hgb A in sickle cell?
Hgb S
What type of inheritance pattern does sickle cell follow?
autosomal recessive
What is the most common type of crisis?
vaso-occulsive
What is the drug of choice for severe sickle cell pain?
morphine
What oxygen sat goal should be maintained?
> 90%
What happens to RBCs when Hgb S becomes deooxygenated?
They become rigid, sticky, and sickle shaped
If both parents carry the trait, what % chance does the child have SCD?
25%
What 3 symptoms strongly suggest a vaso-occlusive crisis?
pain, fever, swelling
What medication increases fetal hemoglobin and reduces crises?
hydroxyurea
Why are children with SCD given prophylactic antibiotics?
d/t high risk of infection
What major two problems result from sickling?
Vaso-occlusion and hemolysis
What is the difference between sickle cell trait and the disease?
trait = one Hgb S gene
disease = two Hgb S genes
What life threatening crisis involves blood pooling in the spleen?
splenic sequestration
What 3 treatments are given during a crisis?
oxygen, hydration, and analgesics
What assessment is critical d/t stroke risk?
neuro assessment
How long do sickled RBCs live compared to normal RBC?
10-20 days vs 120 days
Why is sickle cell more common in certain populations?
it evolved as a protection against malaria
What crisis causes pulmonary infiltrates and hypoxemia?
Why should demerol NOT be used?
d/t risk of seizures
Why should hot/cold compresses be avoided?
they increase sickling and risk of burns
Why does sickle cell cause chronic anemia?
Due to the premature destruction of RBCs (hemolysis)
At what age do symptoms usually begin in infants and why?
4-6 months when fetal Hbg decreases
What happens during an aplastic crisis?
What is the only potential cure for sickle cell?
Stem cell (bone marrow) transplant
What lab values increase d/t the bone marrow compensating?
Reticulocyte count