Ped success blood stuff

26. A nurse educator is providing a teaching session for the nursing staff. Which of the following individuals is at greatest risk for developing beta-thalassemia (Cooley anemia)? 1. A child of Mediterranean descent. 2. A child of Mexican descent. 3. A child whose mother has chronic anemia. 4. A child who has a low intake of iron.

1

24. Which of the following measures should the nurse teach the parent of a child with hemophilia to do first if the child sustains an injury to a joint causing bleeding? 1. Give the child a dose of Tylenol. 2. Immobilize the joint, and elevate the extremity. 3. Apply heat to the area. 4. Administer factor per the home-care protocol.

4

19. The nurse is caring for a child with sickle cell anemia who is scheduled to have an exchange transfusion. What information should the nurse teach the family? 1. The procedure is done to prevent further sickling during a vaso-occlusive crisis. 2. The procedure reduces side effects from blood transfusions. 3. The procedure is a routine treatment for sickle cell crisis. 4. Once the child's spleen is removed, it is necessary to do exchange transfusions.

1 1. Exchange transfusion reduces the number of circulating sickle cells and slows down the cycle of hypoxia, thrombosis, and tissue ischemia.

25. Which of the following measures should be implemented for a child with von Willebrand disease who has a nosebleed? 1. Apply pressure to the nose for at least 10 minutes. 2. Have the child lie supine and quiet. 3. Avoid packing of the nostrils. 4. Encourage the child to swallow frequently.

1 Applying pressure to the nose may stop the bleeding. In von Willebrand disease, there is an increased tendency to bleed from mucous membranes,

23. The parent of a child with hemophilia is asking the nurse what caused the hemophilia. Which is the nurse's best response? 1. It is an X-linked dominant disorder. 2. It is an X-linked recessive disorder. 3. It is an autosomal dominant disorder. 4. It is an autosomal recessive disorder.

2

28. The nurse is caring for a child diagnosed with thalassemia major who is receiving the first chelation therapy. What information should the nurse teach the parent regarding the therapy? 1. Decreases the risk of bleeding. 2. Eliminates excess iron. 3. Prevents further sickling of the red blood cells. 4. Provides an iron supplement.

2

20. A nurse instructs the parent of a child with sickle cell anemia about factors that might precipitate a pain crisis in the child. Which of the following factors identified by the parent as being able to cause a pain crisis indicates a need for further instruction? 1. Infection. 2. Overhydration. 3. Stress at school. 4. Cold environment.

2 2. Overhydration does not cause a crisis.

17. The nurse is caring for a child with sickle cell disease who is scheduled to have a splenectomy. What information should the nurse explain to the parents regarding the reason for a splenectomy? 1. To decrease potential for infection. 2. To prevent splenic sequestration. 3. To prevent sickling of red blood cells. 4. To prevent sickle cell crisis.

2 2. Splenic sequestration is a lifethreatening situation in children with sickle cell anemia. Once a child is considered to be at high risk of splenic sequestration or has had this in the past, the spleen will be removed.

21. A 10-year-old with severe factor VIII deficiency falls, injures an elbow, and is brought to the ER. The nurse should prepare which of the following? 1. An IM injection of factor VIII. 2. An IV infusion of factor VIII. 3. An injection of desmopressin. 4. An IV infusion of platelets.

2 2. The child is treated with an IV infusion of factor VIII to replace the missing factor and help stop the bleeding.

29. Which of the following should the nurse expect to administer to a child with ITP and a platelet count of 5000/ mm3? 1. Platelets. 2. Intravenous immunoglobulin. 3. Packed red blood cells. 4. White blood cells.

2 Intravenous immunoglobulin is given because the cause of platelet destruction is believed to be an autoimmune response to disease-related antigens. Treatment is usually supportive. Activity is restricted at the onset because of the low platelet count and risk for injury that could cause bleeding.

4. A nurse is caring for a 5-year-old with sickle cell vaso-occlusive crisis. Which of the following orders should the nurse question? Select all that apply. 1. Position the child for comfort. 2. Apply hot packs to painful areas. 3. Give Demerol 25 mg intravenously every 4 hours as needed for pain. 4. Restrict oral fluids. 5. Apply oxygen per nasal cannula to keep oxygen saturations above 94%.

3, 4 3. Tissue hypoxia is very painful. Narcotics such as morphine are usually given for pain when the child is in a crisis. Demerol should be avoided because of the risk of Demerol-induced seizures. 4. The child should receive hydration because when the child is in crisis, the abnormal S-shaped red blood cells clump, causing tissue hypoxia and pain.

1. The nurse is taking care of a child with sickle cell disease. The nurse is aware that which of the following problems is (are) associated with sickle cell disease? Select all that apply. 1. Polycythemia. 2. Hemarthrosis. 3. Aplastic crisis. 4. Thrombocytopenia. 5. Splenic sequestration. 6. Vaso-occlusive crisis.

3, 5, 6

22. Which of the following will be abnormal in a child with the diagnosis of hemophilia? 1. Platelet count. 2. Hemoglobin level. 3. White blood cell count. 4. Partial thromboplastin time.

4

27. A nurse is doing discharge education with a parent who has a child with beta-thalassemia (Cooley anemia). The nurse informs the parent that the child is at risk for which of the following conditions? 1. Hypertrophy of the thyroid. 2. Polycythemia vera. 3. Thrombocytopenia. 4. Chronic hypoxia and iron overload.

4 In beta-thalassemia there is increased destruction of red blood cells, causing anemia. This results in chronic anemia and hypoxia. The children are treated with multiple blood transfusions, which can cause iron overload and damage to major organs.

6. A child with hemophilia A fell and injured a knee while playing outside. The knee is swollen and painful. Which of the following measures should be taken to stop the bleeding? Select all that apply. 1. The extremity should be immobilized. 2. The extremity should be elevated. 3. Warm moist compresses should be applied to decrease pain. 4. Passive range-of-motion exercises should be administered to the extremity. 5. Factor VIII should be administered.

1,2, 5

5. A nurse is caring for a child with von Willebrand disease. The nurse is aware that which of the following is a (are) clinical manifestation(s) of von Willebrand disease? Select all that apply. 1. Bleeding of the mucous membranes. 2. The child bruises easily. 3. Excessive menstruation. 4. The child has frequent nosebleeds. 5. Elevated creatinine levels. 6. The child has a factor IX deficiency.

1,2,3,4, 1. Von Willebrand disease is a hereditary bleeding disorder characterized by deficiency of or defect in a protein. The disorder causes adherence of platelets to damaged endothelium and a mild deficiency of factor VIII. One of the manifestations of this disease is bleeding of the mucous membranes. 2. Bruising is a common manifestation of this disease. 3. Excessive menstruation may be a manifestation of this disease. 4. Frequent nosebleeds are a common manifestation of this disease. Von Willebrand disease is a minor deficiency of factor VIII, so the clinical manifestations will be less severe.

9. The nurse is caring for a child who is receiving a transfusion of packed red blood cells. The nurse is aware that if the child had a hemolytic reaction to the blood, the signs and symptoms would include which of the following? Select all that apply. 1. Fever. 2. Rash. 3. Oliguria. 4. Hypotension. 5. Chills.

1,3, 4

7. Which of the following activities should a nurse suggest for a client diagnosed with hemophilia? Select all that apply. 1. Swimming. 2. Golf. 3. Hiking. 4. Fishing. 5. Soccer.

1-4

8. Which of the following describe(s) idiopathic thrombocytopenia purpura (ITP)? Select all that apply. 1. ITP is a congenital hematological disorder. 2. ITP causes excessive destruction of platelets. 3. Children with ITP have normal bone marrow. 4. Platelets are small in ITP. 5. Purpura is observed in ITP.

2,3,5

30. Which test provides a definitive diagnosis of aplastic anemia? 1. Complete blood count with differential. 2. Bone marrow aspiration. 3. Serum IgG levels. 4. Basic metabolic panel.

2. Definitive diagnosis is determined from bone marrow aspiration, which demonstrates the conversion of red bone marrow to yellow, fatty marrow.