Ch 58: Sickle Cell Disease Flashcards
iron chelation tx
removes excess iron that builds up from chronic blood tranfusions to prevent iron overload (organ damage). deferoxamine (iron overload antidote) no longer used d/t ADRs and no PO. now deferasirox (Exjade, Jadenu) and deferiprone (Ferriprox) are PO and used and monitored in specialty clinics and pharmacies d/t ADRs
Endari - generic, form, indication, MOA, admin
L-glutamine. PO powder for adults and children 5+ YO to reduce complications of SCD. dec oxidative stress, which can damage sickled RBCs. mix each dose in 8 oz cold or room temp beverage or 4-6 oz of food.
Droxia - generic, MOA, indication
hydroxyurea (Hydrea). MOA: disease mod agent that stim production of HgbF to reduce freq of acute pain crises, eps of acute chest syndrome, and need for blood transfusions. indication: adults with 3+ mod-severe pain crises in 1 yr or pts with severe or recurrent acute chest syndrome, chronic sx anemia, or disability. consider in all children 9+ mo age regardless of disease severity.
Droxia - BBW, warnings, monitor, notes
BBW: myelosuppression (avoid agents that cause addition suppression), malignancy (sun protection for skin cancer). warnings: embryo-fetal tox, avoid live vax. monitor: cbc w diff. contraception req during and 1 yr after tx for males or 6 mo after tx for females. hazardous agent, wear gloves. folic acid supplementation for macrocytosis. clinical response can take 3-6 mo.
analgesics in SCD
mild-mod mgmt: rest, fluids, warm compresses, NSAIDs or APAP. Severe pain and vaso-occlusive crisis: IV admin of opioids of PCA.
non drug tx
blood transfusions, goal Hgb no higher than 10. chelation tx to remove excess iron. only cure is bone marrow transplantation.
infection risk
SCD causes spleen fibrosis –> functional asplenia (dec or absent function). ppx BID pcn PO reduces mortality assoc with invasive pneumococcal in young children (high mortality if <5 YO) –> cont indef if spleen removed or invasive pneumococcal infection despite ppx.
key vax in SCD
routine childhood: h. flu type B (Hib), PCV13/prevnar. additional vax d/t functional asplenia: meningococcal con series + boosters, meningococcal serogroup B (Bexsero/Trumenba), PPSV23/pneumovax 23, PCV13/prevnar 13 x1 in children 6-19 YO and adults if never recieved.
