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Flashcards in Sickle Cell Disease Deck (27)
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1
Q

Race most affected by sickle cell disease

A

African Americans

2
Q

Pathophysiology of sickle cell disease

A

RBC’s are not shaped correctly; cannot effectively carry oxygen
RBCs hemolyze after 10-20 days

3
Q

leading cause of death in SCD

A

Acute chest syndrome

sickle cell crisis - pain in the chest and signs of infection

4
Q

Why are sickle cell pts at incr risk for infection?

A

Pts spleen loses function early in life

spleen plays a vital role in immune function

5
Q

Non-drug treatment for sickle cell

A

blood transfusions - protects against many life threatening complications
goal Hgb ~ 10

6
Q

Acute complications of sickle cell

A
Anemia
Cholecystitis 
infection
pain crisis 
stroke 
priapism
7
Q

Preferred oral contraception for sickle cell pts

A

progestin only contraception
IUDs

bc pts are at increased risk of stroke

8
Q

What antibiotic is given as prophylaxis in children and infants?

A

penicllin

9
Q

Organisms that are more likely to cause infections in pts with functional asplenia

A

S. pneimoniae
H influenzae
N meningitidis

10
Q

Routine childhood vaccines needed for kids with sickle cell

A

HIB

PCV13 (Prevnar)

11
Q

Vaccines recommended due to functional asplenia

A

Meningococcal conjugate
Meningococcal serogroup B (>/= 10 y/o)
PPSV23 (> 2 y/o, then 5 years later, then > 65)

12
Q

Droxia

Hydrea

A

Hydroxyurea

13
Q

Role of Hydroxyurea

A

stimulates production of HgbF

Reduces frequency of pain crises, need for blood transfusions

Indicated for adults with 3 or more pain crises in 1 year and all kids > 9 months old

14
Q

Hydroxyura boxed warning

A

Myelosuppression

15
Q

Hydroxyurea side effects

A

N/v/d

incr LFTs, uric acid, BUN , SCr

16
Q

Hydroxyurea starting dose

A

15 mg/kg/day as a single dose

20 for kids and children

17
Q

Hydroxyurea monitoring

A

absolute Neutrophil count
goal ANC: 2000-4000

stop therapy if ANC < 2000 and/or PLT < 80,000

18
Q

How long does it take to see a clinical response from hydroyurea?

A

3-6 months

19
Q

can hydroyurea be used in pregnancy?

A

NOOOO
Contraception is required for all pts taking and females must use contraception for 6 months after d/c (12 mons for males)

20
Q

Endari

A

L-glutamine oral powder

21
Q

Role of Endari

A

reduce acute complications of SCD (like hydroxyurea)

22
Q

Endari dosing

A

<30 kg: 5 g BID
30-65 kg: 10 mg BID
> 65 kg: 15 mg BID

23
Q

Endari administration

A

mix with 8 oz of liquid (water, milk, etc)

or 4-6 oz of food (applesauce, yogurt)

24
Q

What is the concern of giving multiple blood transfusions?

A

Iron overload; can damage organs

25
Q

What is used to treat iron overload?

A

Chelating agents

26
Q

Exjade

Jadenu

A

Deferasirox

chelating agent

27
Q

Ferriprox

A

deferiprone

chelating agent

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