Sickle Cell Disease

Question 1

Immunizations necessary for SCD

Answer 1

Due to functional asplenia
* Hib, PCV13
* MCV4 conjugate series
* Bexsero, Trumenba (Men B)
* PPSV23 (at age 2, 5 yrs later, than 65)

Question 2

Hydroxyurea

Answer 2

• stimulates production of HgbF
• 3+ mod-severe pain crises in 1 year, recurrent acute ches syndrome, chronic asymptomatic anemia or disability, children <9 months of age

Question 3

Droxia, Hydrea, Siklos

Answer 3

Droxia: 15 mg/kg/day
Siklos: 20 mg/kg/day
Max 35 mg/kg/day
CrCl <60: adjust

Question 4

Safety, SE, BW - hydroxyurea

Answer 4

MED GUIDE REQ

BW: myelosuppression (decr WBC, platelets), malignancy (leukemia, skin cancer)
Warnings: fetal tox, avoid live vaccines
Increased risk of pancreatitis, hepatotox, neuropathy when used with antiretroviral (didanosine, stavudine)
ADE: incre LFT, uric acid, BUN, Scr, mouth ulcers, N/V/D, alopecia, low sperm counts (male), hyperpigmentation or atrophy of skin/nails

Monitor: CBC, if tox occurs ANC <2000, plts <80,000, stop hydroxyurea until bone marrow recovers, then restart at 5 mg/kg/day lower

Note: contraception req during tx & 6 months after stopping for females, 12 months males
- hazardous drug! wear gloves when dispensing. Don’t open caps
- folic acid supplementation recommended
- can take 3-6 months for clinical response

DI: don’t use with PIMECROLIMUS, TACROLIMUSS (topical), & other drugs that cause myelosuppression (ex: clozapine, deferiprone, leflunomide, natalizmab, tofacitinib)