The following sample questions are representative of actual test content, difficulty level, and question format. The answer key follows the set of questions.

Directions: Each question or incomplete statement below is followed by four suggested answers or completions. Select the one that is best in each case.

1. A child with misshapen thumbs is newly diagnosed with myelodysplastic syndrome. The nurse anticipates that the work-up will include testing for:
   
   A.         Evan’s syndrome.
   B.         Fanconi’s anemia.
   C.         hereditary spherocytosis.
   D.         von Willebrand disease.
   
   
2. An adolescent with sickle cell disease is started on hydroxyurea.  The nurse reviews the side effects of this drug including the patient’s increased risk for:
   
   A.         infection.
   B.         insomnia.
   C.         cardiac arrhythmias.
   D.         interstitial pneumonitis.
   
   
3. Which of the following is an appropriate intervention for a patient with sickle cell disease experiencing priapism that has lasted four hours?
   
   A.         Administration of opioid analgesics
   B.         Application of ice packs to the penis
   C.         Intravenous heparin boluses
   D.         Restriction of fluid intake
   
   
4. A five-year-old patient with sickle cell disease presents with splenic sequestration, oxygen saturation value of 95%, temperature 37.5°C (99.5°F), heart rate 148, respirations 28, and blood pressure 90/36.  The patient’s lab values include a white blood cell count 5,000/mm3, hemoglobin 5.2, and platelet count 175,000.
   
   What is the most important initial intervention?
   
   A.         Administration of intravenous antibiotics
   B.         Initiation of oxygen via nasal cannula
   C.         Ten breaths on an incentive spirometer
   D.         Transfusion of  10 ml/kg packed red blood cells
   
   
5. A patient admitted for fever and vaso-occlusive crisis develops an increased respiratory rate and retractions. Which of the following would most likely be ordered?
   
   A.         Blood cultures
   B.         Chest x-ray
   C.         Pulmonary functions
   D.         Type and crossmatch
   
   
6. In patients with sickle cell disease, immunization with the 23-valent-pneumococcal vaccine has been instrumental in decreasing deaths from:
   
   A.         acute chest syndrome.
   B.         aplastic crisis.
   C.         sepsis.
   D          splenic sequestration.

Answer Key