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CPHON Sample Test Items – Answer Key
1. B
Rationale: Children with Fanconi’s anemia may not be diagnosed until they present with myelodysplastic syndrome or acute myelogenous leukemia. It is often associated with a variety of physical anomalies, including abnormalities of the hands and upper extremities. Hereditary spherocytosis is a hemolytic anemia and not associated with malignant transformations. Von Willebrand disease is a clotting disorder and not associated with malignant transformations. Evan’s syndrome is an autoimmune hemolytic anemia and not associated with malignant transformations.
Reference: Kline, N.E. (Ed.) (2008). Essentials of pediatric hematology/oncology nursing: A core curriculum (3rd ed.). Glenview, IL: Association of Pediatric Hematology/Oncology Nurses, p. 298-99.
2. A
Rationale: Myelosuppression is a common side effect of hydroxyurea. Resulting neutropenia puts the patient at increased risk for infection.
Reference: Kline, N.E. (Ed.) (2008). Essentials of pediatric hematology/oncology nursing: A core curriculum (3rd ed.). Glenview, IL: Association of Pediatric Hematology/Oncology Nurses, pp. 66, 81, 122-123, 290.
3. A
Rationale: Priapism can be a very painful experience for patients with sickle cell disease, and therefore pain management is indicated. Ice is never applied to the area due to the risk of further vasoconstriction. Fluids should be encouraged, not restricted. Heparin is not given as priapism is not caused by a clot, but rather by sickle cells becoming stuck in the vasculature of the penis.
Reference: Sickle Cell Disease Care Consortium. (2001). Sickle cell Disease in Children and Adolescents: Diagnosis, Guidelines for Comprehensive Care, and Care Paths and Protocols for Management of Acute and Chronic Complications. Sedona, AZ: Author. Retrieved January 5, 2009 from http://www.dshs.state.tx.us/newborn/pdf/sedona02.pdf. p. 24.
4. D
Rationale: Treatment for splenic sequestration could include all of the listed interventions to assist the patient if warranted; however, transfusion of red blood cells is the most important first step for this patient who is exhibiting signs of cardiovascular compromise.
Reference: Sickle Cell Disease Care Consortium. (2001).Sickle cell Disease in Children and Adolescents: Diagnosis, Guidelines for Comprehensive Care, and Care Paths and Protocols for Management of Acute and Chronic Complications. Sedona, AZ: Author. Retrieved January 5, 2009 from http://www.dshs.state.tx.us/newborn/pdf/sedona02.pdf. pp. 19 – 20.
5. B
Rationale: Fever, increased oxygen demands and a new infiltrate on chest x-ray are the hallmark signs of acute chest syndrome. Pain crisis and pneumonia are risk factors for acute chest syndrome. The patient was admitted with fever, cultures were done upon admission and antibiotics started. Blood cultures may be drawn for persistent of new fevers but are not necessary for the diagnosis of acute chest syndrome. While transfusion is the treatment, along with antibiotics and supplemental oxygen, for acute chest syndrome, the diagnosis must first be established. Acute chest syndrome can lead to the rapid deterioration of the patient; chest x-ray must not wait so diagnosis can be made. Pulmonary function tests are not indicated at this time.
Reference:
Kline, N.E. (Ed.) (2008). Essentials of pediatric hematology/oncology nursing: A core curriculum (3rd ed.). Glenview, IL: Association of Pediatric Hematology/Oncology Nurses, p. 290.
6. C
Rationale: Sepsis is one of the leading causes of death in these patients. Although acute chest syndrome is a leading cause of death in patients with sickle cell, it is caused by the occlusion of lung vessels from sickled cells. The most common cause of aplastic crisis is infection with parvovirus B19. Splenic sequestration occurs when sickled cells are trapped in the spleen.
Reference:
Kline, N.E. (Ed.) (2008). Essentials of pediatric hematology/oncology nursing: A core curriculum (3rd ed.). Glenview, IL: Association of Pediatric Hematology/Oncology Nurses, p. 290.
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