Sickle Cell Disease Practice Questions

25 multiple-choice questions on Sickle cell disease for paramedic and EMT students, from Chapter 25 (Hematologic Emergencies). Read each question, pick your answer, then tap Show answer to check it and read the explanation.

25 practice questions

1. Patients with sickle cell disease often take folic acid daily because:

   1. A. Folate stops pain crises directly
   2. B. Rapid red cell turnover uses up folate
   3. C. It replaces iron lost in the urine
   4. D. It prevents red cells from sickling in crises
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   Answer: B. Rapid red cell turnover uses up folate

   Constant hemolysis forces the marrow to make red cells quickly, which uses up folate. Folate does not prevent sickling or crises.

2. Sickle cell disease is inherited in which pattern?

   1. A. Autosomal dominant
   2. B. X-linked recessive
   3. C. Mitochondrial inheritance
   4. D. Autosomal recessive
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   Answer: D. Autosomal recessive

   A child needs two sickle genes to have the disease. One gene causes sickle cell trait, which is usually harmless.

3. In the United States, sickle cell disease most commonly affects people of:

   1. A. African descent
   2. B. Northern European descent
   3. C. East Asian descent
   4. D. Native American descent
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   Answer: A. African descent

   It also occurs in people of Mediterranean, Middle Eastern, Indian, and Hispanic ancestry.

4. Sickling of red cells is triggered by:

   1. A. Low platelets and bleeding
   2. B. Overhydration and alkalosis
   3. C. Hypoxia, dehydration, cold
   4. D. High iron and B12 levels
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   Answer: C. Hypoxia, dehydration, cold

   Low oxygen, acidosis, dehydration, infection, cold, and stress all promote sickling. Low platelets and bleeding do not make hemoglobin S polymerize, fluids and alkalosis work against sickling, and iron or B12 levels do not trigger a crisis.

5. A 25-year-old with sickle cell disease has hip pain for months that worsens with walking. A likely cause is:

   1. A. Avascular necrosis of the femoral head
   2. B. Gout crystals deposited in the hip joint
   3. C. A hip dislocation from minor trauma
   4. D. Hemarthrosis from low platelets
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   Answer: A. Avascular necrosis of the femoral head

   Repeated vaso-occlusion cuts off blood to the femoral head, and the bone dies and collapses. It is common in sickle cell disease. Platelets are usually normal in sickle cell disease.

6. The most common reason sickle cell patients call EMS is:

   1. A. An aplastic red cell crisis
   2. B. A splenic sequestration crisis
   3. C. A vaso-occlusive pain crisis
   4. D. Iron overload from transfusion
   Show answer

   Answer: C. A vaso-occlusive pain crisis

   Pain crises are recurrent and can be severe. Treat pain promptly and believe the patient's report of pain.

7. A sickle cell patient in a pain crisis is not dehydrated. The main risk of giving large volumes of IV fluid is:

   1. A. Increased sickling from the diluted blood
   2. B. Fluid overload and acute chest syndrome
   3. C. A rapid rise in the hemoglobin
   4. D. Hypoglycemia from diluted glucose
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   Answer: B. Fluid overload and acute chest syndrome

   Give fluids for dehydration, but routine large boluses add little and can cause pulmonary edema and acute chest syndrome. Dilution lowers, not raises, the hemoglobin and does not increase sickling.

8. A 9-month-old with sickle cell disease has painful swelling of both hands and feet. This is most likely:

   1. A. Juvenile idiopathic arthritis
   2. B. An allergic reaction to new formula
   3. C. Cellulitis from an insect bite
   4. D. Dactylitis from vaso-occlusion
   Show answer

   Answer: D. Dactylitis from vaso-occlusion

   Hand-foot syndrome is often the first sign of sickle cell disease in infants, as sickled cells block blood flow in the small bones. Treat pain and transport.

9. Patients with sickle cell disease dehydrate easily because their kidneys:

   1. A. Cannot concentrate urine well
   2. B. Make too much erythropoietin
   3. C. Retain extra sodium and water
   4. D. Stop making urine during a crisis
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   Answer: A. Cannot concentrate urine well

   Sickling in the kidney's medulla damages the concentrating mechanism, so patients keep passing dilute urine even when dry. Dehydration then promotes more sickling.

10. For acute chest syndrome or stroke in sickle cell disease, the hospital often uses exchange transfusion to:

    1. A. Raise the platelet count quickly
    2. B. Remove excess iron from the liver
    3. C. Replace clotting factors that were lost
    4. D. Lower the share of sickle hemoglobin
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    Answer: D. Lower the share of sickle hemoglobin

    Exchange transfusion removes the patient's sickle cells and replaces them with normal donor cells, lowering hemoglobin S to below about 30% without making the blood too thick.

11. Splenic sequestration crisis is most dangerous in:

    1. A. Older adults
    2. B. Young children
    3. C. Pregnant adults
    4. D. Teenagers
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    Answer: B. Young children

    It mainly affects infants and young children, whose spleens are still large enough to trap a large share of the blood volume. It can kill within hours.

12. A child with sickle cell disease has a suddenly enlarging spleen, pallor, and tachycardia. You suspect:

    1. A. Acute chest syndrome
    2. B. Vaso-occlusive crisis
    3. C. Splenic sequestration
    4. D. Iron deficiency
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    Answer: C. Splenic sequestration

    This is a life-threatening emergency. Treat for shock and transport rapidly.

13. Most adults with sickle cell disease have:

    1. A. A nonfunctioning spleen
    2. B. An enlarged, firm spleen
    3. C. A normally functioning spleen
    4. D. An overactive, hyperfunctioning spleen
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    Answer: A. A nonfunctioning spleen

    Repeated infarctions scar and shrink the spleen (autosplenectomy), usually by adolescence.

14. A fever in a sickle cell patient is an emergency because of:

    1. A. Risk of febrile seizures
    2. B. A high chance of stroke
    3. C. Risk of overwhelming sepsis
    4. D. Bleeding triggered by the fever
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    Answer: C. Risk of overwhelming sepsis

    Without a working spleen, encapsulated bacteria such as pneumococcus can cause rapid sepsis. Children need antibiotics quickly. Febrile seizures are a concern in young children generally, not the reason fever is an emergency in sickle cell disease, and fever does not cause bleeding.

15. Aplastic crisis in sickle cell disease is often triggered by:

    1. A. Influenza vaccine
    2. B. Parvovirus B19
    3. C. Iron supplements
    4. D. Cold weather
    Show answer

    Answer: B. Parvovirus B19

    Parvovirus temporarily stops red cell production. With red cells already short-lived, hemoglobin drops quickly.

16. Sickle cell disease greatly increases the risk of which neurologic emergency in children?

    1. A. Guillain-Barré syndrome
    2. B. Bell palsy
    3. C. Migraine headache
    4. D. Stroke
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    Answer: D. Stroke

    Children with sickle cell disease have a high stroke risk. Any new weakness, speech change, or seizure requires rapid transport.

17. Priapism in a male sickle cell patient is:

    1. A. A urologic emergency
    2. B. A normal finding
    3. C. An early sign of dehydration
    4. D. Caused by low platelets
    Show answer

    Answer: A. A urologic emergency

    Sickled cells trap blood in the penis. Prolonged priapism can cause permanent damage.

18. Why are sickle cell patients prone to gallstones?

    1. A. They lack bile salts
    2. B. Their liver makes too much cholesterol
    3. C. Chronic hemolysis raises bilirubin
    4. D. Iron forms stones
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    Answer: C. Chronic hemolysis raises bilirubin

    High bilirubin from constant red cell breakdown forms pigment stones.

19. Sickle cell trait (one gene) usually:

    1. A. Causes frequent pain crises
    2. B. Causes no symptoms
    3. C. Causes severe anemia
    4. D. Requires monthly transfusion
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    Answer: B. Causes no symptoms

    Trait is usually benign. Rarely, extreme exertion, dehydration, or altitude can cause complications.

20. The approximate life span of a sickled red cell is:

    1. A. 60 to 90 days
    2. B. 120 days
    3. C. 180 days
    4. D. 10 to 20 days
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    Answer: D. 10 to 20 days

    Sickled cells are destroyed quickly, causing chronic hemolytic anemia.

21. Which medication is commonly used long term to reduce sickle cell crises?

    1. A. Warfarin
    2. B. Prednisone daily
    3. C. Furosemide
    4. D. Hydroxyurea
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    Answer: D. Hydroxyurea

    Hydroxyurea increases fetal hemoglobin, which reduces sickling.

22. A sickle cell patient reports pain is at its usual crisis level but vital signs are normal. You should:

    1. A. Withhold analgesia as drug-seeking
    2. B. Delay care until labs return
    3. C. Treat pain per protocol
    4. D. Tell them to drink water at home
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    Answer: C. Treat pain per protocol

    Patients may not appear distressed despite severe pain. Pain should be treated promptly. Normal vital signs do not rule out a crisis.

23. Which complication can cause sudden vision loss in sickle cell disease?

    1. A. Glaucoma from long-term steroids
    2. B. Retinal artery occlusion
    3. C. Age-related cataracts
    4. D. Conjunctivitis
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    Answer: B. Retinal artery occlusion

    Sickling in retinal vessels can cause infarction and retinal detachment.

24. A sickle cell patient with chest pain, cough, and SpO2 of 89% should first receive:

    1. A. Oxygen
    2. B. Aspirin 324 mg chewed
    3. C. A 2-liter saline bolus
    4. D. Oral fluids
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    Answer: A. Oxygen

    Correct hypoxia promptly, since it worsens sickling and is a feature of acute chest syndrome. Chest pain here is from acute chest syndrome, not a coronary clot, so aspirin is not the first step. A large saline bolus can worsen lung fluid and acute chest syndrome, so fluids are given cautiously.

25. Pregnant patients with sickle cell disease are at increased risk for:

    1. A. Lower risk of infection
    2. B. Crises and clotting
    3. C. Fewer crises
    4. D. Higher hemoglobin
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    Answer: B. Crises and clotting

    Pregnancy increases the frequency of crises, infection, preeclampsia, and thrombosis.