Sickle Cell Disease Flashcards Preview

NAPLEX > Sickle Cell Disease > Flashcards

Flashcards in Sickle Cell Disease Deck (20):
0

Whats sickle cell dx (SCD)?

Group of disorders that affect the hemoglobin in RBCs

When tissue is deprived of oxygen-rich blood it's ischemic and painful

1

What's acute episodes of ischemic pain called?

Sickle cell crisis or Vaso-occlusive crisis and is the hallmark of SCD

2

Other common conditions with SCD?

Infection

Pulmonary HTN

Stroke

Anemia

Kidneys

Priapism

Liver enlargement

Gallstones

Spleen becomes nonfunctional due to recurrent episodes of oxygen deprivation

3

Primary drugs used in SCD?

Immunizations

Antibiotics (primarily Penicillin) - to reduce infection risk

Analgesic - to control pain

Folic acid - to help control anemia

Hydroxyurea - to reduce freq of pain episodes & acute chest syndrome, and iron chelation therapy

Blood transfusion

4

Goal of hemoglobin level, post-infusion?

No higher than 10g/dL

5

What's the major cause of death for children with SCD under 5 yrs of age?

Infection

Due to repetitive sickling & infarctions, the spleen becomes fibrotic & eventually shrinks in size

Bacteria proliferates & causes increased risk of infection, including septicemia with encapsulated bacteria

6

Species most often responsible for infection in SCD?

S. Pnuemonia

H. influenza

Salmonella spp

7

Vaccinations for < 2 yrs with SCD?

13-valent pneumococcal conjugate vaccine (Prevnar 13)

H. influenza vaccine

8

Vaccinations for >= 2 yrs with SCD?

23-valent pneumococcal polysaccharide vaccine (Pneumovax) x 2

Admin at least 2 months apart for Prevnar 13 and at 5

Meningococcal conjugate vaccine x 2, give btw 2-6yrs & 5-9yrs

9

What's used for Pneumococcal prophylaxis?

Penicillin V Potassium 125mg PO bid to age 3

Then, 250mg PO bid until at least 5 yrs

OR

Benzathine penicillin 600,000 million units IM Q4 weeks in nonadherent pts from 6 months to 6 yrs

10

What's used for Pneumococcal prophylaxis in penicillin allergy?

Erythromycin 10 mg/kg PO BID

11

What's used for Pneumococcal prophylaxis in non-adherent pts from 6 months to 6 yrs?

Benzathine penicillin 600,000 million units IM Q4 weeks in nonadherent pts from 6 months to 6 yrs

12

When is folic acid recommended in SCD?

SCD + chronic hemolytic anemia

Folic acid 0.1 mg/day up to age 1 yr

Folic acid 0.3 mg/day up to age 1-4 yrs

Folic acid 0.4-1 mg/day from 4 yrs thru adolescence

13

MOA of hydroxyurea?

Stimulates production of HbF, by blocking enzyme ribonucleotide reductase

HbF is form of hgb found in fetus and young infants, which prevent sickling action of RBCs, which is why infants with SCD don't develop sx until HbF levels have dropped

14

SEs of Hydroxyurea (Droxia, Hydrea)?

Leukopenia

Anemia

Thrombocytopenia

Anirexia

Nausea

Diarrhea

Constipation

Hyperpigmentation

Scaling

HA

Dizziness

15

Effect of chronic RBCs transfusion?

Chronic RBC transfusions cause iron overload, which damages liver, heat and other organs

Chelation is used to remove excess iron stores in the body

16

Chelation agent used to reduce iron overload from transfusions?

Deferoxamine (Desferal) injection

Deferasirox (Exjade)

17

SEs of Deferoxamine (Desferal) injection (chelation agent)?

Ototoxicity

Visual impairments

Arthralgia

Acute respiratory distress syndrome (dyspnea, cyanosis and/or
interstitial infiltrates)

Growth failure

Hypersensitivity rxns (e.g. Urticaria, angioedema)

Injection-site rxns (erythema, pruritus)

18

SEs of Deferasirox (Exjade) - (chelation agent)?

HA

Rash

Abdominal pain

Nausea

Arthralgia

Visual impairment

Hepatic dysfxn

Kidney impairment

19

How do u use Deferasirox (Exjade)?

Make an oral suspension in water, juice or apple sauce on an empty stomach

Don't chew or swallow tablet whole, at least 30 min b4 eating

DROP
STIR
DRINK

Decks in NAPLEX Class (73):