Sickle Cell Disease Flashcards
What’s acute episodes of ischemic pain called?
Sickle cell crisis or Vaso-occlusive crisis and is the hallmark of SCD
Other common conditions with SCD?
Infection
Pulmonary HTN
Stroke
Anemia
Kidneys
Priapism
Liver enlargement
Gallstones
Spleen becomes nonfunctional due to recurrent episodes of oxygen deprivation
Primary drugs used in SCD?
Immunizations
Antibiotics (primarily Penicillin) - to reduce infection risk
Analgesic - to control pain
Folic acid - to help control anemia
Hydroxyurea - to reduce freq of pain episodes & acute chest syndrome, and iron chelation therapy
Blood transfusion
Goal of hemoglobin level, post-infusion?
No higher than 10g/dL
What’s the major cause of death for children with SCD under 5 yrs of age?
Infection
Due to repetitive sickling & infarctions, the spleen becomes fibrotic & eventually shrinks in size
Bacteria proliferates & causes increased risk of infection, including septicemia with encapsulated bacteria
Species most often responsible for infection in SCD?
S. Pnuemonia
H. influenza
Salmonella spp
Vaccinations for < 2 yrs with SCD?
13-valent pneumococcal conjugate vaccine (Prevnar 13)
H. influenza vaccine
Vaccinations for >= 2 yrs with SCD?
23-valent pneumococcal polysaccharide vaccine (Pneumovax) x 2
Admin at least 2 months apart for Prevnar 13 and at 5
Meningococcal conjugate vaccine x 2, give btw 2-6yrs & 5-9yrs
What’s used for Pneumococcal prophylaxis?
Penicillin V Potassium 125mg PO bid to age 3
Then, 250mg PO bid until at least 5 yrs
OR
Benzathine penicillin 600,000 million units IM Q4 weeks in nonadherent pts from 6 months to 6 yrs
What’s used for Pneumococcal prophylaxis in penicillin allergy?
Erythromycin 10 mg/kg PO BID
What’s used for Pneumococcal prophylaxis in non-adherent pts from 6 months to 6 yrs?
Benzathine penicillin 600,000 million units IM Q4 weeks in nonadherent pts from 6 months to 6 yrs
When is folic acid recommended in SCD?
SCD + chronic hemolytic anemia
Folic acid 0.1 mg/day up to age 1 yr
Folic acid 0.3 mg/day up to age 1-4 yrs
Folic acid 0.4-1 mg/day from 4 yrs thru adolescence
MOA of hydroxyurea?
Stimulates production of HbF, by blocking enzyme ribonucleotide reductase
HbF is form of hgb found in fetus and young infants, which prevent sickling action of RBCs, which is why infants with SCD don’t develop sx until HbF levels have dropped
SEs of Hydroxyurea (Droxia, Hydrea)?
Leukopenia
Anemia
Thrombocytopenia
Anirexia
Nausea
Diarrhea
Constipation
Hyperpigmentation
Scaling
HA
Dizziness
Effect of chronic RBCs transfusion?
Chronic RBC transfusions cause iron overload, which damages liver, heat and other organs
Chelation is used to remove excess iron stores in the body
