Sickle Cell Disease Flashcards
Sickle Cell Disease pathophys
RBCs are crescant shaped because they contain an abnormal hemoblin S (for sickle cell)
-these sickled RBC burst within 10-20 days (normal RBCs last for ~120 days)
-they are unable to transport oxygen properly
Vaso-occulsive crisis (VOC)
acute pain crisis
-pain in lower back, legs, hips, abs, chest
caused from vascular occulsion leading to ischemia
Complications with sickle cell disease?
Acute
Acute chest syndrome
Anema
Cholecystitis
Organ failure
Priapism
Spleen sequestration
Stroke
VOC
Chronic
Avascular necrosis
Leg uclers
gallstones
pain
fetal death
pulmonary HTN
renal impairment
retinopathy
Sickle Cell disease is immunocomprisable. What are the common infections in these patients?
Streptococcus pneumoniae
Haemophilus influenzae
Neisseria meningitidis
What is the only cure for sickle cell disease?
Bone Marrow Transplant
What vaccinations are recommended for sicle cell disease patients>
influenza
pneumococcal
meningococcal
What is the mechanism of Hydroxyurea in SCD?
stimulates production of the correct hemoglobin (HgbF).
When is hydroxyurea indicated?
-adults with >/= 3 moderate-severe pain crisis in 1 year
-considered in all children >9 months
Hydroxyurea SE
BW: myelosuppression
Warning: fetal toxicity!!!!
avoid live vaccines
**contraception required during treatment
**Hazardous Drug!!!
**Folic acid supplementation is recommended
When a SCD patient receives too many blood transfusions and needs therapy for iron overload, what is the recommendation?
Chelation therapy of iron
-IV deferoxamine (less common)
PO deferasirox
PO deferiprone
What SCD drug works by inhibiting hemoglobin S polymerization?
Voxelotor
What oral powder can be used to treat children or adults >/= 5 years old with SCD?
L-glutamine
What monoclonal antibody is FDA approved to treat sickle cell disease?
Crizanlizumab
