Term
| Pts with sicle cell anemia have an increased risk for what infection? |
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Definition
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Term
Prevention of pneumococcal infection in SCD includes:
a. influenza vaccine annual starting at 6mo
b. H. influenzae (Hib) vaccine starting at 2months
c. tetanus toxoid q10y
d. 23valent pneumococcol polysaccharide vaccine, 7-valent pneumococcal conjugated vaccine, and oral PCN
e. meningococcal |
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Definition
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Term
| What is the appropriate PCN prophylaxis regimen? |
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Definition
| PCN 125mg BID starting at diagnosis until 3yo then 250mgBID until 5 |
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Term
| What is the primary indication for chronic transfusion program? |
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Definition
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Term
| How do you treat a vasoocclusive pain crisis? |
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Definition
| hydration, aggressive anaglesic (individualized) |
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Term
What is the appropriate management of SCD pt presenting with fever? All of the following except:
a. cefotaxime or ceftriaxone +/- vanc
b. ibuprofen or apap for fever
c. fluid
d. frequent monitoring
e. pneumococcal vaccine |
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Definition
| E. all of the other answers ARE appropriate |
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Term
| What is the MOA of hydroxyurea in SCD? |
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Definition
| increased the fetal hemoglobin production |
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Term
The most common cause of aplastic crisis is:
a. pneumococcal inf
b. ASPEN syndrome occuring after partial exchange transfusion in pt with priapism
c. parvovirus B19
d. sequestration of RBC in spleen
e. splenectomy |
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Definition
| Parvovirus B19 is the most common cause for aplastic crisis |
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Term
| Hydroxyurea reduces painful crisis but should be monitored for what? |
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Definition
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Term
Analgesic choices for SCD pt with mild to mod pain include all of the following except:
a. NSAIDs
b. APAP
c. narcotics analgesics
d. combo NSAID+narcotic analygesics
e. intramuscular meperidine |
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Definition
| E. meperidine shouldn't be used because it can accumulate and cause neurotoxicity in pt with renal impairment |
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Term
Analgesic choices for SCD pt with severe pain include all of the following except:
a. morphine
b. hydromorphone
c. fentanyl
d. APAP
e. methadone |
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Definition
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Term
Which type of SCD is this?
Painless hematuria and rare aseptic necrosis of bone
vasoocculsivecrises are less common and occur later in life
other complications include ocular disease and prego problems
mild anemia (Hb 10-12 g/dL) |
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Definition
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Term
Which is this?
Rare painless hematuria
normal Hb level
heavy exercise or extreme conditions may provoke gross hematuria and complications |
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Definition
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Term
Which type is this?
pain crisis
microvascuar disruption of organs (spleen, liver, bone marrow, kidney, brain, luing), gallstone, priapism, leg ulcers, anemia (Hb 7-10g/dL) |
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Definition
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Term
Which type is this?
no HbA production
severity similar to SCAnemia
Hb 7-10g/dL with microcytosis |
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Definition
| Sickle cell B0 thalassemia |
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Term
| Which form of sickle cell Beta thalassemia is more severe? |
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Definition
| Beta0 is more severe than beta+ |
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Term
| When do pts become symptomatic and why? |
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Definition
~4-6months
when HbS replaces HbF (fetal hemoglobin) |
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Term
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Definition
painful swelling of hands and feet
"hand-and-foot syndrome" |
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Term
| What is recommended in adult pt, prego, and pt with chronic hemolysis of all ages? |
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Definition
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Term
| What is the max dose of hydroxyurea? |
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Definition
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Term
| What is the O2 saturation goal for SCD? |
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Definition
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Term
| Can you take hydroxyurea if you are prego? |
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Definition
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Term
| What toxicity is seen with hydroxyurea? |
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Definition
ANC<2000cels/mm3
Plt <80000
absolute reticulocyte count <80000 if Hb<9g/dL
Hb <5 or >20% below baseline
increased serum creatinine 50% above baseline
100% increase in ALT |
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