Sickle Cell Disease Flashcards

1
Q

Key Vaccines in SCD

Routine Childhood series 2

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

Additional Vaccines Due to Functional Asplenia

4 speciifc timing

A
  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
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3
Q

SCD most commonly effects what population?

A

African Americans

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4
Q

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

A

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

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5
Q

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

A

Caused by vascular occlusion

Most common types VOC: Vasooclussive crisis

or Acute pain crisis

If pain in chest it is acute chest syndrome

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6
Q

Common chronic complication of SCD?

A

Pulmonary HTN

Others but this is underlines

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7
Q

SCD causes what issue?

A

Fibrosis of the spleen causing functional asplenia

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8
Q

Non drug treatment for SCD? 2

A

Blood transfusion no higher than 10 g/dL

Bone marrow transplant

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9
Q

Drug tx for SCD? 6

A
  1. Immunizations
  2. Abx
  3. Analgesics for pain
  4. Hydroxyurea
  5. L-Glutamine
  6. Chelation therapy to reduce iron overload from tranfusion
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10
Q

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

A
  1. S. Pneumo
  2. H. Influe
  3. N. Mengingitiis
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11
Q

What should be used in children when born?

How long should it be continued?

What is the dosing?

A
  • PCNs Twice daily
  • Until 5 yo
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12
Q

Pts with severe pain or VOC should be given what?

A

IV opioids or PCA

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13
Q

What is hydroxyurea and what does it do?

A

Disease modifying agent that stimulates the production of HgbF

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14
Q

When is Hydroxyurea indicated? In SCD

A

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

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15
Q

How is hydroxyurea taken?

A

Oral

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16
Q

Droxia, Hydrea, Siklos

A

Hydroxyurea

17
Q

Boxed warning for hydroxyurea?

A

Myelosuppression

18
Q

2 warnings for hydroxyurea?

A
  1. Fetal embryo toxicity
  2. Avoid live vaccines
19
Q

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

A

ANC<2,000/mm3

Platelet <80,000/mm3

20
Q

What should be monitored for hydroxyurea?

A

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

21
Q

What is required for hydroxyurea?

A

Contraception

22
Q

What supplementation is used for hydroxyurea?

A

FOlic acid

23
Q

What type of agent is hydroxyurea?

A

Hazardous

Wear disposable gloves to limit exposure dont open the capsule

24
Q

How long is contraception needed for hydroxyurea?

A

during and after stopping 1 year males 6 months females

25
Q

What drug interactions for hydroxyurea?

A

Drugs that cause myelosuppression

Limuss

Wear sun protection

26
Q

Endari

A

L-Glutamine

27
Q

Age indication for Endari

A

>=5 with SCD

28
Q

What does L-glutamine do for SCD pts?

A

Reduces the number of acute complications

29
Q

What type of formulation is L-glutamine?

A

Oral powder mixed with room temp beverage

30
Q

Iron Chelation Tx

What isnt used and why?

A

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

31
Q

What are the two oral chelators

A

Deferasirox

Deferiprone

32
Q

Who is hydroxyurea also indicated for?

A

Pts with acute chest syndrome, chronic symptomatic anemia or disability

consider in all children > 9 months