Anticoagulation & Blood Disorders: Sickle Cell Disease Flashcards
RBC life span
90-120 days
Sickle Cell disease
inherited RBC disorder
common in African American population
genetic mutation
concave “sickle” shape, RBC burst after 10-20 days
VOC
Vaso-occlusive crisis
most commonly in lower back, legs, hips, abdomen, and chest
can last days to weeks
Females with SCD should use what kind of contraceptives?
progestin only or levonorgestrel IUD
** no estrogen**
Blood transfusions in SCD patients
goal HgB no higher than 10
run risk of iron overload = chelation therapy required if need to remove
Is there a cure for SCD?
Bone marrow transplantation but it isn’t really used due to costs and risks associated with it
Primary disease modifying therapy for SCD?
Hydroxyurea
Infants who screen positive for SCD at birth get……
BID penicillin and treated until age 5
continued indefinitely if remove spleen or invasive pneumococcal infection develops
Analgesics for SCD
IV opioids or PCA
Hydroxyurea is indicated for adults with
> 3 moderate-to-severe pain crises in one year
Hydroxyurea boxed warnings
Myelosuppression
Hydroxyurea warnings
Fetal toxicity, avoid live vaccinations
Hydroxyurea side effects
Inc LFTs
N/V/D
alopecia
hyperpigmentation or atrophy of skin/nails
low sperm count
Hydroxyurea monitoring
CBC w/ differential
if toxicity occurs, hold hydroxyurea until bone marrow recovers
Hydroxyurea special notes
contraception require during and after txm (6 month female/ 12 month males)
hazardous drug, wear gloves when dispensing and wash hands
Folic acid supplementation is recommended
