notes Flashcards
What is the most common sickle cell crises?
vasoocclusive crisis or acute pain crisis
Why are sickle cell pts at risk for an infection?
fibrosis at the spleen causes functional asplenia (decreased or absent splenic function)
what is the non-drug txt of sickle cell disease? What is the goal and risks?
Blood transfusions
Hgb goal is no greater than 10g/dL post infusion.
Risk of frequent blood transfusion is iron overload which requires chelation therapy.
what is the major cause of death in children <5 years who have SCD?
infection (sepsis and meningitis from s. pneumo, h. influenzae and n. meningitidis)
which antibiotic is given prophylactically to reduce mortality associated w/ invasive pneumococcal infection?
penicillin prophylactically
infants diagnosed with SCD are put on what antibiotic? what regimen?
PCN BID until age 5 years old
What do patients w/ severe pain and VOC from SCD require?
IV opioids or PCA
What kind of agent is hydroxyurea and MOA in SCD?
It is a disease modifying agent that stimulates HgbF
Who is indicated for Hydroxyurea in SCD?
adults with 3 or more moderate to severe acute pain crises in 1 year
hydroxyurea brand
Droxia
Hydrea
BW for hydroxuyrea
myelosuppression
warnings for hydroxuyrea
fetal-embryo toxicity, avoid live vaccines, skin cancer
monitoring hydroxuyrea
CBC w/ differential
when should hydroxyurea be held?
ANC <2000 or platelets <80,000
How long do men and women have to wait to have children?
6 months after txt for women
12 months after txt for males
How should hydroxyurea be handled?
wear gloves and wash hands. It is a hazardous agent
What supplement should be given with hydroxyurea and why?
folic acid for macrocytosis
hydroxyurea and ARVT
increased risk for pancreatitis, hepatotoxicity, hepatic failure, severe peripheral neuropathy, especially stavudine and didanosine
hydroxyurea should be avoided with what drugs?
pimecrolimus, topical tacrolimus, myelosuppressive drugs, clozapine, leflunomide, tofacitinib, deferiprone, natalizumab
L-glutamine brand
Endari
How do you administer Endari?
in 8 oz of cold or room temperature liquid or 4-6 oz of food
historically, what was used to in chelation therapy. Why is it not as commonly used?
deferoxamine
IV or SC only - no PO therapy
What are the available PO chelation therapy medications?
deferasirox (Exjade, Jadenu)
deferiprone (Ferriprox)
