Sickle Cell Disease

Question 1

Define sickle cell disease

Answer 1

RBC have a abnormal hemoglobin called hemoglobin S this causes RBCs to be rigid with a concave “sickle” shape that burst (hemolyze) after 10-20 days

Question 2

S/S of sickle cell disease

Answer 2

chronic pain
avascular necrosis (bone death)
pulmonary HTN
renal impairment
functional asplenia

Question 3

function of a healthy spleen

Answer 3

removal of old or damaged RBCs
aids in immune function
make and store WBC
clear encapsulated pathogens

Question 4

What are the encapsulated organisms

Answer 4

Streptococcus pneumoniae
Haemophilus influenzae
Neisseria meningitidis

Question 5

Non-drug treatment of SCD

Answer 5

blood transfusion

Question 6

Cure for SCD

Answer 6

bone marrow transplant

Question 7

Drug treatment for SCD

Answer 7

immunizations-reduce infection risk
antibiotics-reduce infection risk
analgesics-pain control
hydroxyura or L-glutamine-prevent/reduce complications
chelation-manage iron overload

Question 8

MOA of hydroxyurea

Answer 8

disease-modifying agent that stimulates Hgbf

Question 9

indication of hydroxyurea in SCD

Answer 9

> 3 moderate- severe pain crises in one year

Question 10

warning for hydroxyurea

Answer 10

myelosuppression
fetal toxicity
avoid live vaccines

Question 11

what is required use with hydroxyurea

Answer 11

contraception