Sickle Cell Disease

Question 1

Race most affected by sickle cell disease

Answer 1

African Americans

Question 2

Pathophysiology of sickle cell disease

Answer 2

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

Question 3

leading cause of death in SCD

Answer 3

Acute chest syndrome

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

Question 4

Why are sickle cell pts at incr risk for infection?

Answer 4

Pts spleen loses function early in life

spleen plays a vital role in immune function

Question 5

Non-drug treatment for sickle cell

Answer 5

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

Question 6

Acute complications of sickle cell

Answer 6

Anemia
Cholecystitis 
infection
pain crisis 
stroke 
priapism

Question 7

Preferred oral contraception for sickle cell pts

Answer 7

progestin only contraception
IUDs

bc pts are at increased risk of stroke

Question 8

What antibiotic is given as prophylaxis in children and infants?

Answer 8

penicllin

Question 9

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

Answer 9

S. pneimoniae
H influenzae
N meningitidis

Question 10

Routine childhood vaccines needed for kids with sickle cell

Answer 10

HIB

PCV13 (Prevnar)

Question 11

Vaccines recommended due to functional asplenia

Answer 11

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

Question 12

Droxia

Hydrea

Answer 12

Hydroxyurea

Question 13

Role of Hydroxyurea

Answer 13

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

Question 14

Hydroxyura boxed warning

Answer 14

Myelosuppression

Question 15

Hydroxyurea side effects

Answer 15

N/v/d

incr LFTs, uric acid, BUN , SCr

Question 16

Hydroxyurea starting dose

Answer 16

15 mg/kg/day as a single dose

20 for kids and children

Question 17

Hydroxyurea monitoring

Answer 17

absolute Neutrophil count
goal ANC: 2000-4000

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

Question 18

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

Answer 18

3-6 months

Question 19

can hydroyurea be used in pregnancy?

Answer 19

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

Question 20

Endari

Answer 20

L-glutamine oral powder

Question 21

Role of Endari

Answer 21

reduce acute complications of SCD (like hydroxyurea)

Question 22

Endari dosing

Answer 22

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

Question 23

Endari administration

Answer 23

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

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

Question 24

What is the concern of giving multiple blood transfusions?

Answer 24

Iron overload; can damage organs

Question 25

What is used to treat iron overload?

Answer 25

Chelating agents

Question 26

Exjade

Jadenu

Answer 26

Deferasirox

chelating agent

Question 27

Ferriprox

Answer 27

deferiprone

chelating agent