SCD 2

Question 1

Bone pain (4)

Answer 1

1. More prolonged and constant pain
2. Bone infarction
3. Sickle arthritis
4. Aseptic necrosis of the femur or humerus.

Question 2

Priapism (4)

Answer 2

1. Priapism is due to vaso- occlusion which causes obstruction of venous drainage.
2. Onset between 5-35yrs.
3. Treatment includes pain relief, hydration, exchange transfusion.
4. Multiple episodes can lead to impotency.

Question 3

Stroke (4)

Answer 3

1. Strokes are a blockage of blood flow to a part of the brain caused by the sickle cells.
2. Peak incidence between 2-9 y/o.
3. Cumulative risk of 11% by age 20yrs.
4. Therapy: Chronic Tx to maintain the Hb S < than 30% to prevent subsequent stroke.

Question 4

Stroke and diagnostics (2)

Answer 4

1. CT of the head –> If MRI is not immediately available or practical, noncontrast head CT should be used as a first line test in patients with headache, seizure or focal weakness in whom stroke is a concern.
   * If negative and ongoing concern, MRI should be obtained
2. MRI –> Most sensitive and specific study to define cerebrovascular accidents in children with sickle cell disease.

Question 5

Treatment – Chronic Blood Transfusion (3)

Answer 5

1. Indication for use: Primary prevention of stroke
   * Treat chronic debilitating pain
2. ACS or pulmonary complications
3. Alloimmunization:
   a. 20-25% due to racial difference in donor and recipient population.
   b. Extended phenotyping is required.

Question 6

Transfusion parameters (2)

Answer 6

1. Maintain Hgb S% < 30 and Hgb 10-12

2. Max15cc/kg. Infuse over 4 hours

Question 7

Blood Transfusion and Iron Overload (2)

Answer 7

1. Each unit of blood deposits ~ 250mg Fe.
2. Tests to measure iron stores:
   a. Serum Ferritin
   b. Liver Biopsy
   c. Superconducting Quantum Interference Device (SQUID)

Question 8

Iron chelation (2)

Answer 8

1. The most effective iron chelator, Desferal, removes somewhere between 30-70 mg of iron per day.
2. Desferal must be given SQ or IV
   a. Desferal 40mg/kg/day SQ
   b. Desferal 4grams/24h IV

Question 9

Hydroxyurea Treatment (5)

Answer 9

1. Mechanism of action:
2. ➩ Hgb F – reduces polymerization of Hgb S
3. Indication for use:
   a. Frequent pain crisis
   b. ACS
   c. Priapism
4. Dose: Start at 15mg/kg/day – And then follow escalation map.
   * Max dose 30 mg/kg/day
5. Monthly evaluation. HU is a mild suppressive agent.
   * CBC/diff, Retic, LFT, Renal

Question 10

Hydroxyurea and Hematologic Parameters (4)

Answer 10

1. Hemoglobin may be higher than expected (9-10 g/dL in
2. Patients with HbSS and a low reticulocyte count
3. Macrocytosis is expected.
4. Thrombocytopenia and neutropenia are common side effects and may indicate a supratherapeutic dose.

Question 11

Routine Care (5 with info)

Answer 11

1. Health Care Maintenance
2. Prophylactic Penicillin
   a. 125mg BID for >3 years
3. Proven reduction of strep pneumo by 80%
   a. 2mos-35mos: 125mg/5ml Sig: 1tsp bid
   b. 36mos - : 250mg/5ml Sig: 1tsp bid
4. Immunization Schedule
   a. Pneumovax (PPV23)
   b. Hepatitis A
   c. Meningococcal
5. Preventative Screenings

Question 12

When should prophylactic penicillin should be initiated in children with sickle cell anemia?

Answer 12

2-3 months old

Question 13

What is the expected hemoglobin range for sickle cell anemia? (SS)

Answer 13

6.5 – 9.5 g/dL

Question 14

What is not the direct result of the chronic hemolysis with sickle cell disease?

Answer 14

delayed growth

Question 15

What are the complication occurring in children with either functional or complete asplenia?

Answer 15

bacterial infections