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Sickle Cell Disease
UMSOPPY3 SCD Notes
23
Pharmacology
Graduate
04/10/2011

Additional Pharmacology Flashcards

 


 

Cards

Term
Pts with sicle cell anemia have an increased risk for what infection?
Definition
Streptococcus pneumoniae
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

Definition
D
Term
What is the appropriate PCN prophylaxis regimen?
Definition
PCN 125mg BID starting at diagnosis until 3yo then 250mgBID until 5
Term
What is the primary indication for chronic transfusion program?
Definition
Stroke prevention
Term
How do you treat a vasoocclusive pain crisis?
Definition
hydration, aggressive anaglesic (individualized)
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

Definition
E. all of the other answers ARE appropriate
Term
What is the MOA of hydroxyurea in SCD?
Definition
increased the fetal hemoglobin production
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

Definition
Parvovirus B19 is the most common cause for aplastic crisis
Term
Hydroxyurea reduces painful crisis but should be monitored for what?
Definition
bone marrow suppression
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

Definition
E. meperidine shouldn't be used because it can accumulate and cause neurotoxicity in pt with renal impairment
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

Definition
D. APAP
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)

Definition
Sickle Cell Hemoglobin C
Term

Which is this?

 

Rare painless hematuria

normal Hb level

heavy exercise or extreme conditions may provoke gross hematuria and complications

Definition
Sickle cell trait
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)

Definition
Sickle cell anemia
Term

Which type is this?

 

no HbA production

severity similar to SCAnemia

Hb 7-10g/dL with microcytosis

Definition
Sickle cell B0 thalassemia
Term
Which form of sickle cell Beta thalassemia is more severe?
Definition
Beta0 is more severe than beta+
Term
When do pts become symptomatic and why?
Definition

~4-6months

 

when HbS replaces HbF (fetal hemoglobin)

Term
What is dactylitis?
Definition

painful swelling of hands and feet

 

"hand-and-foot syndrome"

Term
What is recommended in adult pt, prego, and pt with chronic hemolysis of all ages?
Definition
Folic acid 1mg QD
Term
What is the max dose of hydroxyurea?
Definition
35mg/kg/day
Term
What is the O2 saturation goal for SCD?
Definition
>92%
Term
Can you take hydroxyurea if you are prego?
Definition
Hell no!
Term
What toxicity is seen with hydroxyurea?
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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