sickle cell disease

Question 1

what happens in sickle cells

Answer 1

RBC dies in 10-20 days instead of 120 days

Question 2

what are some complications of Sicle cell disaeas

Answer 2

Acute- chest syndrome, anemia, cholecystitis, infection, spleen sequestration, multiorgan failure
chronic- leg ulcer, bone death, gall stones, pain, retinopathy

Question 3

what are some non drug treatment for Sickle cell

Answer 3

blood transfusion with goal >10, monitor iron overload
- chelation therapy to reduce iron overload

cure is to do a bonemarrow transplant

Question 4

what are the treatment options for SCD

Answer 4

immunization and abx to reduce infections
analgesics for pain
hydroxyurea adn chelation

Question 5

what are some vaccines recommended for SCD

Answer 5

Prevnar conjugate and poly
meningococcal conjugate
haemophilus influenza

Question 6

hydroxyurea

Answer 6

droxia hydrea
- do not use with immunosupressants
mOA is stimulating production of HGBF
can cause fetal toxicity, avoid live vaccine
folic acid supplements recomended
>3 moderate to severe pain in a year

Question 7

L glutamine

Answer 7

Endari
age>5

Question 8

Voxelotor

Answer 8

Oxbryta FDA approved
MOA- inhibits Hgbs polymerization which is the cause of SCD
age>12 can be mono therapy or added with hydroxyurea

Question 9

Crizanlizumab

Answer 9

Adakveo- FDA approved injection

Question 10

defeoxamine vs deferasirox

Answer 10

deferoxamine not available in oral and infusion requires 8-12 hours