Sickle Cell Disease

Question 1

Target population of SCA

Answer 1

African American

Question 2

When do sx develop for SCA

Answer 2

2-3 months due to baby still having HgbF

Question 3

Acute complication of SCA

Answer 3

Vas-occlusive crisis (acute pain crisis)
ACS
Anemia
Cholecystitis
Infecrion
Priapism
Splenic sequestration
Stroke
Multiorgan failure

Question 4

Chronic complications od SCA

Answer 4

Avascular necrosis (bone death)
Depressiona nd stress
Gallstones
Leg ulcers
Pain
Pregnancy complications
Pulmonary HTN
Priapism
Renal impairment
Retinopathy

Question 5

Pathogens that target SCA? Why?

Answer 5

Strep. pneumo
H flu
Neisseria meningitis

Functional asplenia

Question 6

Non drug treatment for SCA

Answer 6

Blood transfusions
goal Hgb level should not be higher 10 post infusion → iron overload

Question 7

What is the only cure for SCA

Answer 7

Bone marrow transplantation

Question 8

How to remove excess iron from blood transfusion?

Answer 8

Chelation therapy

Question 9

PO chelating drus

Answer 9

Deferasirox (Exjade, Jadenu)
Deferiprone (Ferriprox)

Question 10

Anaglesics for SCA

Answer 10

IV opioids or PCA

Question 11

MOA of hydroxyurea

Answer 11

Stimulates the production of HgbF

Question 12

When is HU indicated

Answer 12

≥1 modnerate-severe pain crisis

Question 13

Hydroxyurea

Answer 13

Droxia, Hydra, Siklos

Question 14

Vaccines recommended for SCA

Answer 14

Hib
PCV 15 → ≥8wks then PPSV23 or PCV 20 (once)
MenACWY
MenB

Question 15

Prophylaxis for children

Answer 15

PO Penicillin BID and treated until age 5

Question 16

BBW of HU

Answer 16

Myelosuppression

Question 17

Warning of HU

Answer 17

Fetal toxicity, avoid live vaccines

Contraception required

Hazardous drugs → wear gloves and wash hands

Folic acid is recommended

Question 18

Monitoring and dosing of HU

Answer 18

CBC with diffntial Q2-4W

Toxicity: ANC <2000, platelet <80,000

Round doses to the nearest capsule size

Question 19

MOA of L-glutamine

Answer 19

Reduce acute complications of SCD → ↓ oxidative stress and damaged sickled RBCs

Question 20

ADR of L-glutamine

Answer 20

Constipation, farts, nausea, HA, pan, cough

Question 21

Counseling on L-glutamine

Answer 21

Mix each 8 oz of cold or room temp beverage

Or 4-6 oz of food

Question 22

Voxelotor MOA

Answer 22

Inhibits HubS polymerization → prevents RBC sickling

Question 23

MOA of Crizanlizumab

Answer 23

Monoclonal antibody that reduces the frequency of VOC

Binds and inhibits the P-selcetin that involves the adhesion of sickled erythrocyte to vessels