Sickle Cell Disease

Question 1

sickle cell - spleen

Answer 1

due to abnormal shape, can cause a decrease in oxygen level at certain tissue…

healthy spleen removes old or damaged RBC. in SCD spleen becomes fibrotic and shrinks due to to repetitive sickling and infractions.. functional asplenia or absent spleen
increased risk of serious infections

Question 2

sickle cell goal Hgb

Answer 2

10g/dl, no higher
blood transfusion can help

only cure is bone marrow transplantation

Question 3

key vaccines in sickle cell disease

Answer 3

routine childhood series
- Hib
- PCV13
additional vax for asplenia
- meningococcal conjugate series polus routine boosters
- meningococcal serogroup b (Bexsero, trumenba)
- PCV20, or PCV15 8 weeks then PPSV23

Question 4

Hydroxyurea

Answer 4

Droxia, Hydrea, Siklos
primary disease-modifying therapy for SCD
boxed warning: myelosuppression
fetal toxicity, avoid live vax
CBC w/differential every 2-4 weeks if tox occurs (ANC<2,000, platelet <80,000) hold med

SE: hyperpigmentation or atrophy of the skin, nails, low sperm count

contraception required during and after therapy (6 month female and 12 months male)
hazard drug wear gloves!! wash hands
add folic acid supplementation to prevent macrocytosis
CrCl<60 dose adjust