Chapter 37 Hematological Disorders

382. The nurse is reviewing a health care provider's prescriptions for a child with sickle cell anemia who was admitted to the hospital for the treatment of vaso-occlusive crisis. Which prescriptions documented in the child's record should the nurse question? Select all that apply. 1. Restrict fluid intake 2. Position for comfort 3. Avoid strain on painful joints 4. Apply nasal oxygen at 2 L/min 5. Provide a high-calorie, high-protein diet 6. Give meperidine (Demerol) 25 mg intravenously every 4 hours for pain

1 Restrict fluid intake 6 Give meperidine (Demerol) 25 mg intravenously every 4 hours for pain Sickle cell anemia is one of a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S. It is caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain. Hemoglobin S is sensitive to changes in the oxygen content of the RBC; insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow. Oral and intravenous fluids are an important part of treatment. Meperidine (Demerol) is not recommended for a child with sickle cell disease because of the risk for normeperidine-induced seizures. Normeperidine, a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. The nurse would question the prescription for restricted fluids and meperidine for pain control. Positioning for comfort, avoiding strain on painful joints, oxygen, and a high-calorie and high-protein diet are also important parts of the treatment plan.

383. The nurse is conducting staff in-service training on von Willebrand's disease. Which should the nurse include as characteristics of von Willebrand's disease? Select all that apply. 1. Easy bruising occurs 2. Gum bleeding occurs 3. It is a hereditary bleeding disorder 4. Treatment and care are similar to that for hemophilia 5. It is characterized by extremely high creatinine levels 6. The disorder causes platelets to adhere to damaged endothelium

1, 2, 3, 4, 6 von Willebrand's disease is a hereditary bleeding disorder characterized by a deficiency of or a defect in a protein termed von Willebrand factor. The disorder causes platelets to adhere to damaged endothelium. It is characterized by an increased tendency to bleed from mucous membranes. Assessment findings include epistaxis, gum bleeding, easy bruising, and excessive menstrual bleeding. An elevated creatinine level is not associated with this disorder.

382. The nurse is reviewing a health care provider's prescriptions for a child with sickle cell anemia who was admitted to the hospital for the treatment of vaso-occlusive crisis. Which prescriptions documented in the child's record should the nurse question? Select all that apply. 1. Restrict fluid intake 2. Position for comfort 3. Avoid strain on painful joints 4. Apply nasal oxygen at 2 L/min 5. Provide a high-calorie, high-protein diet 6. Give meperidine (Demerol) 25 mg intravenously every 4 hours for pain

1, 6 Sickle cell anemia is one of a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S. It is caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain. Hemoglobin S is sensitive to changes in the oxygen content of the RBC; insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow. Oral and intravenous fluids are an important part of treatment. Meperidine (Demerol) is not recommended for a child with sickle cell disease because of the risk for normeperidine-induced seizures. Normeperidine, a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. The nurse would question the prescription for restricted fluids and meperidine for pain control. Positioning for comfort, avoiding strain on painful joints, oxygen, and a high-calorie and high-protein diet are also important parts of the treatment plan.

376. The nursing student is presenting a clinical conference and discusses the cause of beta-thalassemia. The nursing student informs that a child at greatest risk of developing this disorder is which one? 1. A child of Mexican descent 2. A child of Mediterranean descent 3. A child whose intake of iron is extremely poor 4. A breast-fed child of a mother with chronic anemia

2 Beta-thalassemia is an autosomal recessive disorder characterized by the reduced production of one of the globin chains in the synthesis of hemoglobin (both parents must be carriers to produce a child with beta-thalassemia major), This disorder is found primarily in individuals of Mediterranean descent.

375. The nurse is providing home care instructions to the parents of a 10-year-old child with hemophilia. Which sport activity should the nurse suggest for this child? 1. Soccer 2. Basketball 3. Swimming 4. Field hockey

3 Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Children with hemophilia need to avoid contact sports and to take precautions such as wearing elbow and knee pads and helmets with other sports. The safe activity for them is swimming.

379. A 10 y/o child with hemophilia A has slipped on the ice and bumped his knee. The nurse should prepare to administer which prescription? 1. Injection of factor X 2. Intravenous infusion of iron 3. Intravenous infusion of factor VIII 4. Intramuscular injection of iron using the Z-track method

3 Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. The primary treatment is replacement of the missing clotting factor; additional medications, such as agents to relieve pain, may be prescribed depending on the source of bleeding from the disprder. A child with hemophilia A is at risk for joint bleeding after a fall. Factor VIII would be prescribed intravenously to replace the missing clotting factor and minimize the bleeding. Factor X and iron are not used to treat children with hemophilia A.

381. Laboratory studies are performed for a child suspected to have iron deficiency anemia. The nurse reviews the laboratory results, knowing that which result indicates this type of anemia? 1. Elevated hemoglobin level 2. Decreased reticulocyte count 3. Elevated red blood cell count 4. Red blood cells that are microcytic and hypochromic

4 In iron deficiency anemia, iron stores are depleted, resulting in a decreased supply of iron for the manufacture of hemoglobin in RBCs. The results of a complete blood cell count in children with iron deficiency anemia show decreased hemoglobin levels and microcytic and hypochromic red blood cells. The red blood cell count is decreased. The reticulocyte count is usually normal or slightly elevated.

377. A child with beta-thalassemia is receiving long-term blood transfusion therapy for the treatment of the disorder. Chelation therapy is prescribed as a result of too much iron from the transfusions. Which medication should the nurse anticipate to be prescribed? 1. Fragmin 2. Meropenem (Merrem) 3. Metoprolol (Toprol-XL) 4. Deferoxamine (Desferal)

4 Beta-thalassemia is an autosomal recessive disorder characterized by the reduced production of one of the globin chains in the synthesis of hemoglobin (both parents must be carriers to produce a child with beta-thalassemia major). The major complication of long-term transfusion therapy is hemosiderosis. to prevent organ damage from too much iron, chelation therapy with either Exhade of deferoxamine (Desferal) may be prescribed. Deferoxamine is classified as an antidote for acute iron toxicity. Fragmin is an anticoagulant used as prophylaxis for postop DV. Meropenem is an antibiotic. Metoprolol is a beta blocker used to treat hypertension

378. The clinic nurse instructs parents of a child with sickle cell anemia about the precipitating factors related to sickle cell crisis. Which, if identified by the parents as a precipitating factor, indicates the need for further instructions? 1. Stress 2. Trauma 3. Infection 4. Fluid overload

4 Sickle cell crises are acute exacerbations of the disease, which vary considerably in severity and frequency; these include vaso-occlusive crisis, splenic sequestration, hyperhemolytic crisis, and aplastic crisis. Sickle cell crisis may be precipitated by infection, dehydration, hypoxia, trauma, or physical/emotional stress. The mother of a child with sickle cell disease should encourage fluid intake of 1 1/2 to 2 times the daily requirement to prevent dehydration.

374. The nurse analyzes the laboratory results of a child with hemophilia. The nurse understands that which result will most likely be abnormal in this child? 1. Platelet count 2. Hematocrit level 3. Hemoglobin level 4. Partial thromboplastin time (PTT)

4. Partial thromboplastin time Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Results of tests that measure platelet function are normal; results of tests that measure clotting factor function may be abnormal. Abnormal laboratory results in hemophilia indicate a prolonged partial thromboplastin time. The platelet count, hemoglobin level, and hematocrit level are normal in hemophilia.