Sickle Cell Quiz: Can You Manage The Crisis?

1. Which complication is the leading cause of death in children with sickle cell disease?

Acute chest syndrome

Splenic rupture

Stroke from vasculopathy

Renal failure

2. During a sickle cell crisis, which assessment finding indicates adequate pain control?

Pain reduced to manageable level; patient resting comfortably

Complete pain elimination

Patient sleeping deeply from sedation

No complaints reported to nursing staff

3. A 3-year-old with sickle cell disease has jaundice, dark urine, and pale mucous membranes. What is the likely cause?

Hemolytic anemia from RBC destruction

Liver hepatitis

Biliary obstruction

Kidney disease

4. What is the recommended oxygen saturation target for a child in sickle cell crisis?

≥95% (maintain normoxia, avoid hyperoxia)

100% (maximize oxygen delivery)

85-90% (avoid pulmonary edema)

Any level above 75%

5. A child with sickle cell disease presents with headache, altered mental status, and focal neurological deficits. What is the priority?

Stat CT or MRI; evaluate for stroke

Give pain medication and observe

Perform lumbar puncture for meningitis

Increase oxygen to 100%

6. Which preventive measure is most effective in reducing sickle cell disease complications in children?

Penicillin prophylaxis and vaccinations

Avoiding all physical activity

Frequent blood transfusions

Strict dietary restrictions

7. A 4-year-old with sickle cell disease has sudden severe pain in the legs and cannot bear weight. Which diagnosis is most likely?

Bone infarction (vaso-occlusive crisis)

Fracture from trauma

Muscular dystrophy

Leukemia

8. What is the significance of a child with sickle cell disease having a functional asplenia?

Increased risk of infections, especially from encapsulated organisms

No significant clinical impact

Improved oxygen carrying capacity

Reduced pain during crises

9. Hydroxyurea is prescribed for a child with sickle cell disease. What is its primary therapeutic mechanism?

Increases fetal hemoglobin (HbF) production

Destroys sickled red blood cells

Prevents infection

Reduces pain sensation

10. A child with sickle cell disease has a sudden onset of severe abdominal pain, vomiting, and abdominal distension. What should be ruled out first?

Splenic sequestration or infarction

Appendicitis

Gastroenteritis

Lactose intolerance

11. A 7-year-old with sickle cell disease presents with severe chest pain, fever, and dyspnea. What is the most likely diagnosis?

Acute chest syndrome

Splenic sequestration

Pneumonia only

Congestive heart failure

12. A 6-year-old with sickle cell disease has a fever of 101.5°F and appears well. What is the recommended action?

Urgent evaluation and blood cultures; empiric antibiotics

Observe at home with acetaminophen

Schedule office visit within 24 hours

Give antipyretics and recheck in 4 hours

13. Hand-foot syndrome (dactylitis) in sickle cell disease typically occurs in which age group?

Infants and toddlers (6 months to 2 years)

School-age children (6-12 years)

Adolescents (13-18 years)

Young adults only

14. Which medication is the standard first-line treatment for vaso-occlusive pain crisis?

IV opioids (morphine or hydromorphone)

Acetaminophen alone

NSAIDs exclusively

Aspirin for blood thinning

15. A child with sickle cell disease is in crisis. Which fluid management strategy is most appropriate?

IV hydration at 1.5 times maintenance rate

Restrict fluids to prevent edema

Oral fluids only

Normal saline bolus followed by restriction

16. What is the primary mechanism of pain in a vaso-occlusive crisis?

Tissue hypoxia from RBC sickling and vascular occlusion

Infection of bone marrow

Spleen enlargement pressing on nerves

Direct hemolysis of red blood cells

17. Acute splenic sequestration in sickle cell disease is characterized by which clinical finding?

Massive splenomegaly with sudden anemia

Spleen atrophy in young children

Painless abdominal distension

Elevated white blood cell count only

18. A 5-year-old with sickle cell disease has a hemoglobin level of 5 g/dL and appears lethargic. What is the priority nursing intervention?

Prepare for blood transfusion

Start oxygen at 2 L/min

Administer oral iron supplements

Encourage fluid intake

19. Which finding is most characteristic of vaso-occlusive crisis in sickle cell disease?

Severe pain in bones, joints, and abdomen

Sudden hepatomegaly without pain

Gradual onset weakness over weeks

Painless jaundice

Sickle Cell Quiz: Can You Manage the Crisis?