Sickle Cell Disease

Question 1

Key Vaccines in SCD

Routine Childhood series 2

Answer 1

1. H Flu Type B (Hib)
2. Pneumo conjugated (PCV13 Prevnar)

Question 2

Additional Vaccines Due to Functional Asplenia

4 speciifc timing

Answer 2

1. Meningococcal series + routine boosters
2. Meno Serogroup B (Bexsero, Trumenda) >= 10
3. Pneumo poly (Pneumovax)>= 2 booster 5 years later and at age 65
4. Pneumo conjugated prevnar x 1 in any pt >= 6 years of age if not given in childhood

Question 3

SCD most commonly effects what population?

Answer 3

African Americans

Question 4

How long does it take for symptoms of SCD to develop and why?

Answer 4

2-3 months after birth and fetal Hgb ehich blocks sickling of RBCs

Question 5

What are the two types of Acute Sickle cell crisis and what is the cause?

Answer 5

Caused by vascular occlusion

Most common types VOC: Vasooclussive crisis

or Acute pain crisis

If pain in chest it is acute chest syndrome

Question 6

Common chronic complication of SCD?

Answer 6

Pulmonary HTN

Others but this is underlines

Question 7

SCD causes what issue?

Answer 7

Fibrosis of the spleen causing functional asplenia

Question 8

Non drug treatment for SCD? 2

Answer 8

Blood transfusion no higher than 10 g/dL

Bone marrow transplant

Question 9

Drug tx for SCD? 6

Answer 9

1. Immunizations
2. Abx
3. Analgesics for pain
4. Hydroxyurea
5. L-Glutamine
6. Chelation therapy to reduce iron overload from tranfusion

Question 10

Sepsis and mengingitis can occur in SCD due to what 3 main pathogens?

Answer 10

1. S. Pneumo
2. H. Influe
3. N. Mengingitiis

Question 11

What should be used in children when born?

How long should it be continued?

What is the dosing?

Answer 11

• PCNs Twice daily
• Until 5 yo

Question 12

Pts with severe pain or VOC should be given what?

Answer 12

IV opioids or PCA

Question 13

What is hydroxyurea and what does it do?

Answer 13

Disease modifying agent that stimulates the production of HgbF

Question 14

When is Hydroxyurea indicated? In SCD

Answer 14

Adults who have >=3 acute pain crisis in 1 year

Question 15

How is hydroxyurea taken?

Answer 15

Oral

Question 16

Droxia, Hydrea, Siklos

Answer 16

Hydroxyurea

Question 17

Boxed warning for hydroxyurea?

Answer 17

Myelosuppression

Question 18

2 warnings for hydroxyurea?

Answer 18

1. Fetal embryo toxicity
2. Avoid live vaccines

Question 19

How do you know if hydroxyurea is at toxic levels? 2 labs

Answer 19

ANC<2,000/mm3

Platelet <80,000/mm3

Question 20

What should be monitored for hydroxyurea?

Answer 20

CBC q2-4 wks until stable then 2-3 months

Question 21

What is required for hydroxyurea?

Answer 21

Contraception

Question 22

What supplementation is used for hydroxyurea?

Answer 22

FOlic acid

Question 23

What type of agent is hydroxyurea?

Answer 23

Hazardous

Wear disposable gloves to limit exposure dont open the capsule

Question 24

How long is contraception needed for hydroxyurea?

Answer 24

during and after stopping 1 year males 6 months females

Question 25

What drug interactions for hydroxyurea?

Answer 25

Drugs that cause myelosuppression Limuss Wear sun protection

Question 26

Endari

Answer 26

L-Glutamine

Question 27

Age indication for Endari

Answer 27

\>=5 with SCD

Question 28

What does L-glutamine do for SCD pts?

Answer 28

Reduces the number of acute complications

Question 29

What type of formulation is L-glutamine?

Answer 29

Oral powder mixed with room temp beverage

Question 30

Iron Chelation Tx What isnt used and why?

Answer 30

Defuroxime was used but not any more cause of toxicities and its IV

Question 31

What are the two oral chelators

Answer 31

Deferasirox Deferiprone

Question 32

Who is hydroxyurea also indicated for?

Answer 32

Pts with acute chest syndrome, chronic symptomatic anemia or disability consider in all children \> 9 months