47 The Child with a Hematologic Alteration HELPFUL HINT Review the anatomy and physiology of the hematologic system in an anatomy and physiology textbook. MATCHING KEY TERMS Match the term with the correct definition. 1. autoimmune disorder 2. chelation 3. erythropoiesis 4. extramedullary 5. granulocytes 6. hematopoiesis 7. hemolysis 8. hemosiderosis 9. pancytopenia 10. reticulocytes 11. reticuloendothelial system a. Outside of the bone marrow b. Collection of cells capable of phagocytosis c. Reduction in all types of blood cells d. Breakdown of red blood cells (RBCs) e. Increase in tissue iron stores f. Production of RBCs g. Immature RBCs h. Production of blood cells i. Disorder in which the body launches an immunologic response against itself j. Neutrophils, eosinophils, or basophils k. Binding of a metallic ion with a structure that results in inactivation of the ion REVIEW OF THE HEMATOLOGIC SYSTEM 1. Explain the function of the following: a. RBCs: b. White blood cells (WBCs): c. Platelets: Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved. Chapter 47 The Child with a Hematologic Alteration 297
2. Define each term: a. Anemia: b. Polycythemia: c. Lymphopenia: d. Megakaryocytes: IRON-DEFICIENCY ANEMIA (IDA) 1. How does the introduction of cow s milk into the diet before 1 year of age affect the development of IDA? 2. Why are infants with IDA lethargic? 3. Give two reasons why adolescents are prone to IDA. 4. What foods can interfere with iron absorption? SICKLE CELL DISEASE (SCD) 1. Under what three conditions do RBCs change to a sickle shape? a. b. c. 298 Chapter 47 The Child with a Hematologic Alteration Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved.
2. Sickled RBCs cause microvascular occlusion. What does this lead to? Match each complication of sickle cell disease with its clinical manifestation. 3. vaso-occlusive crisis 4. acute chest syndrome 5. dactylitis 6. stroke 7. acute splenic sequestration 8. aplastic crises 9. priapism a. Altered level of consciousness b. Painful, persistent erection of penis c. Pallor, lethargy, and fainting d. Swelling of hands and feet e. Signs of hypovolemic shock f. Fever, cough, and chest pain g. Pain depending on organ or tissues affected BETA-THALASSEMIA 1. Beta-thalassemia is inherited in a(n) pattern. 2. The major complication of the treatment of beta-thalassemia is. 3. In beta-thalassemia, chelation removes from the body. 4. The drug used to manage hemosiderosis is. HEMOPHILIA 1. Describe the parents of a girl with hemophilia. 2. In hemophilia A, the missing blood clotting component is ; in hemophilia B, the missing component is. 3. What are the manifestations of bleeding into the joints? 4. Hemophilia is a genetic disorder carried on the chromosome. VON WILLEBRAND S DISEASE (VWD) Answer as either true (T) or false (F). 1. VWD is an acquired bleeding disorder. 2. Prolonged and excessive bleeding and menorrhagia are signs of VWD. 3. The von Willebrand protein carries coagulation factor IX. 4. VWD is sometimes treated with desmopressin acetate (DDAVP). Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved. Chapter 47 The Child with a Hematologic Alteration 299
IMMUNE THROMBOCYTOPENIC PURPURA (ITP) Answer as either true (T) or false (F). 1. In ITP, autoantibodies are responsible for the destruction of platelets. 2. Children will undergo bone marrow aspirations as part of the diagnostic workup for ITP. 3. Thrombocytopenia, petechiae, and bruising are manifestations of ITP. 4. Splenectomy is the treatment of choice for acute ITP. 5. Chronic ITP is more prevalent among adolescents. DISSEMINATED INTRAVASCULAR COAGULATION (DIC) Answer as either true (T) or false (F). 1. Excessive bleeding and excessive clotting occur at the same time in DIC. 2. In DIC, bleeding occurs because of the depletion of platelets and clotting factors. 3. Excessive bruising and oozing from venipuncture sites are late signs of DIC. APLASTIC ANEMIA 1. In aplastic anemia, the bone marrow stops producing,, and. 2. What are the clinical manifestations of aplastic anemia? 3. Medications used to treat aplastic anemia include steroids, cyclosporin, anti-thymocyte/anti-lymphocyte globulin, and. ABO INCOMPATIBILITY AND HEMOLYTIC DISEASES OF THE NEWBORN 1. ABO incompatibility results when the mother has blood type O and the fetus has either blood type or blood type. 2. Rh incompatibility results when the mother s blood is Rh and the fetal blood is Rh. 3. Rh incompatibility can be prevented by administering RhoGAM to Rh-negative mothers after. HYPERBILIRUBINEMIA Answer as either true (T) or false (F). 1. Neonatal hyperbilirubinemia is referred to as physiologic jaundice. 2. Kernicterus refers to damage to liver cells. 3. Hyperbilirubinemia is more common in term infants. 4. Phototherapy allows unconjugated bilirubin to be removed by the liver and spleen. 5. After an exchange transfusion, phototherapy is no longer necessary. 300 Chapter 47 The Child with a Hematologic Alteration Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved.
SUGGESTED LEARNING ACTIVITIES With one or several other students in a discussion group, review the genetic implications of inherited hematologic disorders by discussing the following questions. 1. Sickle cell disease is an autosomal-recessive disorder. a. When both parents have the trait, what are the chances that a child will have neither the trait nor the disease? a child will have the disease? a child will have the trait? b. When one parent has the disease and the other has the trait, what are the chances that a child will have neither the trait nor the disease? a child will have the disease? a child will have the trait? c. When neither parent has the disease but one has the trait, what are the chances that a child will have neither the trait nor the disease? a child will have the disease? a child will have the trait? 2. Hemophilia is an inherited X-linked recessive disorder. a. When the mother is a carrier and the father does not have hemophilia, what are the chances that a daughter will have the disease? a daughter will be a carrier? a son will have the disease? a son will be a carrier? Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved. Chapter 47 The Child with a Hematologic Alteration 301
b. When the mother is a carrier and the father has hemophilia, what are the chances that a daughter will have the disease? a daughter will be a carrier? a son will have the disease? a son will be a carrier? STUDENT LEARNING APPLICATIONS Enhance your learning by discussing your answers with other students. A 12-year-old girl was admitted to the hospital in a vaso-occlusive sickle cell crisis. Her pain is localized in her right arm and shoulder, and she rates it as 3.5 on a 0-to-5 scale. Her vital signs are slightly elevated, with her oral temperature at 38.2 C. She weighs 40 kg. 1. She is receiving morphine sulfate for pain. As her nurse, what non-pharmacologic measures for pain management might you also suggest to the child? The physician orders IV fluids to run at 2X maintenance. 2. What are the child s basic fluid maintenance requirements for a 24-hour period? What should her hourly IV rate be in order to provide 2X maintenance fluids? 3. You want to assess the child s knowledge about prevention of sickle cell crises. How would you do this? 4. While caring for the child, you notice that she has a productive cough. She also starts to complain about pain in her chest. How would you address this new problem? REVIEW QUESTIONS Choose the correct answer. 1. An example of an autoimmune disorder is a. hemolytic disease of the newborn. b. DIC. c. ITP. d. VWD. 2. If a preschooler with mild hemophilia is experiencing joint pain, the nurse should a. administer children s aspirin. b. apply cold compresses. c. do passive range-of-motion exercises. d. give the child a warm bath in the tub. 302 Chapter 47 The Child with a Hematologic Alteration Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved.
3. Which of the following statements about chelation therapy is true? a. It is used to rid the body of excess sulfate. b. It is used to treat one of the complications of factor VIII therapy. c. It is used to manage hemosiderosis. d. It often results in increased bleeding. 4. An example of a disorder inherited in an autosomaldominant pattern is a. hemophilia A. b. beta-thalassemia. c. VWD. d. ABO-Rh incompatibility. 5. Which of the following statements by an adolescent with iron-deficiency anemia indicates that she needs more teaching about her iron supplement? a. I ll take my pill with orange juice. b. I ll keep the pills out of reach of my younger brother and sister. c. I ll double the dose during my periods. d. It s normal for my bowel movements to be black while I m taking iron. 6. A 4-year-old is admitted to the hospital with sickle cell disease. Her vital signs are temperature of 37.2 C, heart rate 124 bpm, respiratory rate 38 breaths/min, and blood pressure 70/40 mm Hg. She is pale and listless and has splenomegaly. She is experiencing a. aplastic crisis. b. acute chest syndrome. c. a cerebrovascular accident (CVA). d. acute sequestration crisis. 7. An infant receiving phototherapy for hyperbilirubinemia is at increased risk for a. hyperthermia. b. hypothermia. c. dehydration. d. all of the above. 8. A 6-year-old hospital patient complains of a headache. This could be a sign of a serious complication if the child has a. aplastic anemia. b. hemophilia. c. sickle cell disease. d. any of the above. 9. A toddler with hemophilia is at risk for a. altered growth related to poor appetite. b. developmental delay related to activity restrictions. c. infection related to decreased WBCs. d. all of the above. 10. In taking the history of a child with ITP, the nurse is not surprised to discover that a. the child s father has classic hemophilia. b. the child had the flu 2 weeks ago. c. the child fell off a bike last week. d. the child suddenly had a red, raised rash appear today. Copyright 2009, 2005, 2000 by Saunders, an imprint of Elsevier, Inc. All rights reserved. Chapter 47 The Child with a Hematologic Alteration 303
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