Chapter 43

14. In which condition are all the formed elements of the blood simultaneously depressed? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia

ANS: A Aplastic anemia refers to a bone marrow-failure condition in which the formed elements of the blood are simultaneously depressed. Sickle cell anemia is a hemoglobinopathy in which normal adult hemoglobin is partly or completely replaced by abnormal sickle hemoglobin. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin globin chains. Iron deficiency anemia results in a decreased amount of circulating red cells.

4. Which statement best explains why iron deficiency anemia is common during toddlerhood? a. Milk is a poor source of iron. b. Iron cannot be stored during fetal development. c. Fetal iron stores are depleted by age 1 month. d. Dietary iron cannot be started until age 12 months.

ANS: A Children between the ages of 12 and 36 months are at risk for anemia because cow's milk is a major component of their diet, and it is a poor source of iron. Iron is stored during fetal development, but the amount stored depends on maternal iron stores. Fetal iron stores are usually depleted by age 5 to 6 months. Dietary iron can be introduced by breastfeeding, iron-fortified formula, and cereals during the first 12 months of life.

22. What is the priority nursing intervention for a child hospitalized with hemarthrosis resulting from hemophilia? a. Immobilization and elevation of the affected joint b. Administration of acetaminophen for pain relief c. Assessment of the child's response to hospitalization d. Assessment of the impact of hospitalization on the family system

ANS: A Immobilization and elevation of the joint will prevent further injury until bleeding is resolved. Although acetaminophen may help with pain associated with the treatment of hemarthrosis, it is not the priority nursing intervention. Assessment of a child's response to hospitalization is relevant to all hospitalized children; however, in this situation, psychosocial concerns are secondary to physiologic concerns. A priority nursing concern for this child is the management of hemarthrosis. Assessing the impact of hospitalization on the family system is relevant to all hospitalized children; however, it is not the priority in this situation.

23. What is the most common mode of transmission of human immunodeficiency virus (HIV) in the pediatric population? a. Perinatal transmission b. Sexual abuse c. Blood transfusions d. Poor hand washing

ANS: A Infected women can transmit the virus to their infants across the placenta during pregnancy, at delivery, and through breastfeeding. Cases of HIV infection from sexual abuse have been reported; however, perinatal transmission accounts for most pediatric HIV infections. In the past some children became infected with HIV through blood transfusions; however, improved laboratory screening has significantly reduced the probability of contracting HIV from blood products. Poor hand washing is not an etiology of HIV infection.

3. What is the most appropriate nursing diagnosis for a child diagnosed with moderate anemia? a. Activity intolerance related to generalized weakness b. Decreased cardiac output related to abnormal hemoglobin c. Risk for injury related to depressed sensorium d. Risk for Injury related to dehydration and abnormal hemoglobin

ANS: A The basic pathology in anemia is the decreased oxygen-carrying capacity of the blood. The nurse must assess the child's activity level (response to the physiologic state). The nursing diagnosis would reflect the activity intolerance. In generalized anemia no abnormal hemoglobin may be present. Only at a level of very severe anemia does cardiac output become altered. No decreased sensorium exists until profound anemia occurs. Dehydration and abnormal hemoglobin are not usually part of anemia.

13. Chelation therapy is begun on a child with β-thalassemia major with what expected result? a. Treatment of the disease. b. Elimination of excess iron. c. Decreasing the risk of hypoxia. d. Managing nausea and vomiting.

ANS: B A complication of the frequent blood transfusions in thalassemia is iron overload. Chelation therapy with deferoxamine (an iron-chelating agent) is given with oral supplements of vitamin C to increase iron excretion. Chelation therapy treats the side effects of disease management. Decreasing the risk of hypoxia and managing nausea and vomiting are not the purposes of chelation therapy.

18. A young child with human immunodeficiency virus is receiving several antiretroviral drugs. What is the expected outcome of these drug therapies? a. Cure the disease b. Delay disease progression c. Prevent spread of disease d. Treat Pneumocystis jiroveci pneumonia

ANS: B Although not a cure, these antiviral drugs can suppress viral replication, preventing further deterioration of the immune system, and delay disease progression. At this time cure is not possible. These drugs do not prevent the spread of the disease. Pneumocystis jiroveci prophylaxis is accomplished with antibiotics.

8. What term is used to identify the condition in which the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia

ANS: B Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, in which normal adult hemoglobin is replaced by abnormal hemoglobin. Aplastic anemia is a lack of cellular elements being produced. Hemophilia refers to a group of bleeding disorders in which there is deficiency of one of the factors necessary for coagulation. Iron deficiency anemia affects size and depth of color of hemoglobin and does not involve abnormal hemoglobin.

19. Which immunization should be given with caution to children infected with human immunodeficiency virus? a. Influenza b. Varicella c. Pneumococcus d. Inactivated poliovirus

ANS: B The children should be carefully evaluated before giving live viral vaccines such as varicella, measles, mumps, and rubella. The child must be immunocompetent and not have contact with other severely immunocompromised individuals. Influenza, pneumococcus, and inactivated poliovirus are not live vaccines.

11. The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. The nurse should provide what explanation in response to the parent's concern? a. Narcotics are often ordered but not usually needed. b. Narcotics rarely cause addiction when they are medically indicated. c. Narcotics are given as a last resort because of the threat of addiction. d. Narcotics are used only if other measures such as ice packs are

ANS: B The pain of sickle cell anemia is best treated by a multidisciplinary approach. Mild-to-moderate pain can be controlled by ibuprofen and acetaminophen. When narcotics are indicated, they are titrated to effect and given around the clock. Patient-controlled analgesia reinforces the patient's role and responsibility in managing the pain and provides flexibility in dealing with pain. Few if any patients who receive opioids for severe pain become behaviorally addicted to the drug. Narcotics are often used because of the severe nature of the pain of vaso-occlusive crisis. Ice is contraindicated because of its vasoconstrictive effects.

1. A school-age child is admitted in vaso-occlusive sickle cell crisis. The child's care should include which intervention? (Select all that apply.) a. Correction of acidosis b. Adequate hydration c. Pain management d. Administration of heparin e. Replacement of factor VIII

ANS: B, C The management of crises includes adequate hydration, minimizing energy expenditures, pain management, electrolyte replacement, and blood component therapy if indicated. The acidosis will be corrected as the crisis is treated. Heparin and factor VIII are not indicated in the treatment of vaso-occlusive sickle cell crisis.

3. The nurse is planning care for a school-age child admitted to the hospital with hemophilia. Which interventions should the nurse plan to implement for this child? (Select all that apply.) a. Fingersticks for blood work instead of venipunctures b. Avoidance of intramuscular (IM) injections c. Acetaminophen for mild pain control d. Soft toothbrush for dental hygiene e. Administration of packed red blood cells

ANS: B, C, D Nurses should take special precautions when caring for a child with hemophilia to prevent the use of procedures that may cause bleeding, such as IM injections. The subcutaneous route is substituted for IM injections whenever possible. Venipunctures for blood samples are usually preferred for these children. There is usually less bleeding after the venipuncture than after finger or heel punctures. Neither aspirin nor any aspirin-containing compound should be used. Acetaminophen is a suitable aspirin substitute, especially for controlling mild pain. A soft toothbrush is recommended for dental hygiene to prevent bleeding from the gums. Packed red blood cells are not administered. The primary therapy for hemophilia is replacement of the missing clotting factor. The products available are factor VIII concentrates.

2. The nurse is caring for a child with aplastic anemia. Which nursing diagnoses are appropriate? (Select all that apply.) a. Acute Pain related to vaso-occlusion b. Risk for Infection related to inadequate secondary defenses or immunosuppression c. Ineffective Protection related to thrombocytopenia d. Ineffective Tissue Perfusion related to anemia e. Ineffective Protection related to abnormal clotting

ANS: B, C, D These are appropriate nursing diagnosis for the nurse planning care for a child with aplastic anemia. Aplastic anemia is a condition in which the bone marrow ceases production of the cells it normally manufactures, resulting in pancytopenia. The child will have varying degrees of the disease depending on how low the values are for absolute neutrophil count (affecting the body's response to infection), platelet count (putting the child at risk for bleeding), and absolute reticulocyte count (causing the child to have anemia). Acute Pain related to vaso-occlusion is an appropriate nursing diagnosis for sickle cell anemia for the child in vaso-occlusive crisis, but it is not applicable to a child with aplastic anemia. Ineffective Protection related to abnormal clotting is an appropriate diagnosis for a child with hemophilia.

5. Which should the nurse teach about prevention of sickle cell crises to parents of a preschool child with sickle cell disease? (Select all that apply.) a. Limit fluids at bedtime. b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. d. Use ice packs to decrease the discomfort of vaso-occlusive pain in the legs. e. Notify the health care provider if your child begins to develop symptoms of cold.

ANS: B, C, E The most important issues to teach the family of a child with sickle cell anemia are to (1) seek early intervention for problems, such as a fever of 38.5° C (101.3° F) or greater; (2) give penicillin as ordered; (3) recognize signs and symptoms of splenic sequestration, as well as respiratory problems that can lead to hypoxia; and (4) treat the child normally. The nurse emphasizes the importance of adequate hydration to prevent sickling and to delay the adhesion-stasis-thrombosis-ischemia cycle. It is not sufficient to advise parents to "force fluids" or "encourage drinking." They need specific instructions on how many daily glasses or bottles of fluid are required. Many foods are also a source of fluid, particularly soups, flavored ice pops, ice cream, sherbet, gelatin, and puddings. Increased fluids combined with impaired kidney function result in the problem of enuresis. Parents who are unaware of this fact frequently use the usual measures to discourage bed-wetting,

4. Parents of a school-age child with hemophilia ask the nurse, "Which sports are recommended for children with hemophilia?" Which sports should the nurse recommend? (Select all that apply.) a. Soccer b. Swimming c. Basketball d. Golf e. Bowling

ANS: B, D, E Because almost all persons with hemophilia are boys, the physical limitations in regard to active sports may be a difficult adjustment, and activity restrictions must be tempered with sensitivity to the child's emotional and physical needs. Use of protective equipment, such as padding and helmets, is particularly important, and noncontact sports, especially swimming, walking, jogging, tennis, golf, fishing, and bowling, are encouraged. Contact sports such as soccer and basketball are not recommended.

17. Which condition is caused by a virus that primarily infects a specific subset of T lymphocytes, the CD4+ T-cells? a. Wiskott-Aldrich syndrome b. Idiopathic thrombocytopenic purpura (ITP) c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency disease

ANS: C AIDS is caused by the human immunodeficiency virus, which primarily attacks the CD4+ T-cells. Wiskott-Aldrich syndrome, ITP, and severe combined immunodeficiency disease are not viral illnesses.

24. The nurse is planning activity for a 4-year-old child with anemia. Which activity should the nurse plan for this child? a. Game of "hide and seek" in the children's outdoor play area b. Participation in dance activities in the playroom c. Puppet play in the child's room d. A walk down to the hospital lobby

ANS: C Because the basic pathologic process in anemia is a decrease in oxygen-carrying capacity, an important nursing responsibility is to assess the child's energy level and minimize excess demands. The child's level of tolerance for activities of daily living and play is assessed, and adjustments are made to allow as much self-care as possible without undue exertion. Puppet play in the child's room would not be overly tiring. Hide and seek, dancing, and walking to the lobby would not conserve the anemic child's energy.

12. Which statement correctly describes β-thalassemia major (Cooley's anemia)? a. All formed elements of the blood are depressed. b. Inadequate numbers of red blood cells are present. c. Increased incidence occurs in families of Mediterranean extraction. d. Increased incidence occurs in persons of West African descent.

ANS: C Individuals who live near the Mediterranean Sea and their descendants have the highest incidence of thalassemia. An overproduction of red cells occurs. Although numerous, the red cells are relatively unstable. Sickle cell disease is common in blacks of West African descent.

6. Iron dextran is ordered for a young child with severe iron deficiency anemia. What nursing consideration should be considered? a. Administering with meals b. Administering between meals c. Injecting deeply into a large muscle d. Massaging injection site for 5 minutes after administration of drug

ANS: C Iron dextran is a parenteral form of iron. When administered intramuscularly, it must be injected into a large muscle using the Z-track method. Iron dextran is for intramuscular or intravenous administration; it is not taken orally. The site should not be massaged to prevent leakage, potential irritation, and staining of the skin. The administration has no relationship to food since it is not being given orally.

2. Several blood tests are ordered for a preschool child with severe anemia. She is crying and upset because she remembers the venipuncture done at the clinic 2 days ago. The nurse should explain that: a. venipuncture discomfort is very brief. b. only one venipuncture will be needed. c. topical application of local anesthetic can eliminate venipuncture pain. d. most blood tests on children require only a finger puncture because a small amount of blood is needed.

ANS: C Preschool children are very concerned about both pain and the loss of blood. When preparing the child for venipuncture, a topical anesthetic will be used to eliminate any pain. This is a very traumatic experience for preschool children. They are concerned about their bodily integrity. A local anesthetic should be used, and a bandage should be applied to maintain bodily integrity. A promise that only one venipuncture will be needed should not be made in case multiple attempts are required. Both finger punctures and venipunctures are traumatic for children. Both require preparation.

9. Which statement most accurately describes the pathologic changes of sickle cell anemia? a. Sickle-shaped cells carry excess oxygen b. Sickle-shaped cells decrease blood viscosity c. Increased red blood cell destruction occurs d. Decreased red blood cell destruction occurs

ANS: C The clinical features of sickle cell anemia are primarily the result of increased red blood cell destruction and obstruction caused by the sickle-shaped red blood cells. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation.

15. As related to inherited disorders, which statement is descriptive of most cases of hemophilia? a. Autosomal dominant disorder causing deficiency in a factor involved in the blood-clotting reaction b. X-linked recessive inherited disorder causing deficiency of platelets and prolonged bleeding c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient d. Y-linked recessive inherited disorder in which the red blood cells become moon shaped

ANS: C The inheritance pattern in 80% of all of the cases of hemophilia is X-linked recessive. The two most common forms of the disorder are factor VIII deficiency (hemophilia A or classic hemophilia), and factor IX deficiency (hemophilia B or Christmas disease). The disorder involves coagulation factors, not platelets. The disorder does not involve red cells or the Y chromosome.

21. The parents of a child diagnosed with aplastic anemia tell the nurse that a bone marrow transplant (BMT) may be necessary. What should the nurse recognize as important when discussing this with the family? a. BMT should be done at time of diagnosis. b. Parents and siblings of child have a 25% chance of being a suitable donor. c. Finding a suitable donor involves matching antigens from the human leukocyte antigen (HLA) system. d. If BMT fails, chemotherapy or radiotherapy must be continue

ANS: C The most successful BMTs come from suitable HLA-matched donors. The timing of a BMT depends on the disease process involved. It usually follows intensive high-dose chemotherapy and/or radiation therapy. Usually parents only share approximately 50% of the genetic material with their children. A one-in-four chance exists that two siblings will have two identical haplotypes and will be identically matched at the HLA loci. Discussing the continuation of chemotherapy or radiotherapy is not appropriate when planning the BMT. That decision will be made later.

5. When teaching the mother of a 9-month-old infant about administering liquid iron preparations, the nurse should include that information? a. They should be given with meals. b. They should be stopped immediately if nausea and vomiting occur. c. Adequate dosage will turn the stools a tarry green color. d. Preparation should be allowed to mix with saliva and bathe the teeth before swallowing.

ANS: C The nurse should prepare the mother for the anticipated change in the child's stools. If the iron dose is adequate, the stools will become a tarry green color. The lack of the color change may indicate insufficient iron. The iron should be given in two divided doses between meals, when the presence of free hydrochloric acid is greatest. Iron is absorbed best in an acidic environment. Vomiting and diarrhea may occur with iron administration. If these occur, the iron should be given with meals, and the dosage reduced and gradually increased as the child develops tolerance. Liquid preparations of iron stain the teeth. They should be administered through a straw, and the mouth rinsed after administration.

10. Which clinical manifestation should the nurse expect when a child diagnosed with sickle cell anemia experiences an acute vaso-occlusive crisis? a. Circulatory collapse b. Cardiomegaly, systolic murmurs c. Hepatomegaly, intrahepatic cholestasis d. Painful swelling of hands and feet, painful joints

ANS: D A vaso-occlusive crisis is characterized by severe pain in the area of involvement. If in the extremities, painful swelling of the hands and feet is seen; if in the abdomen, severe pain resembles that of acute surgical abdomen; and if in the head, stroke and visual disturbances occur. Circulatory collapse results from sequestration crises. Cardiomegaly, systolic murmurs, hepatomegaly, and intrahepatic cholestasis result from chronic vaso-occlusive phenomena.

1. What is the primary result of anemia? a. Increased blood viscosity. b. Depressed hematopoietic system. c. Presence of abnormal hemoglobin. d. Decreased oxygen-carrying capacity of blood.

ANS: D Anemia is a condition in which the number of red blood cells or hemoglobin concentration is reduced below the normal values for age. This results in a decreased oxygen-carrying capacity of blood. Increased blood viscosity is usually a function of too many cells or of dehydration, not of anemia. A depressed hematopoietic system or abnormal hemoglobin can contribute to anemia, but the definition depends on the deceased oxygen-carrying capacity of the blood.

7. What intervention should the nurse share with parents on how to prevent iron deficiency anemia in a healthy, term, breastfed infant? a. Iron (ferrous sulfate) drops after age 1 month b. Iron-fortified commercial formula can be used by ages 4 to 6 months c. Iron-fortified solid foods are introduced at 3 months d. Iron-fortified infant cereal can be introduced at approximately 6 months of age

ANS: D Breast milk supplies inadequate iron for growth and development after age 5 months. Supplementation is necessary at this time. Iron supplementation or the introduction of solid foods in a breastfed baby is not indicated. Introducing iron-fortified infant cereal at 2 months should be done only if the mother is choosing to discontinue breastfeeding.

16. What name is given to identify an acquired hemorrhagic disorder that is characterized by excessive destruction of platelets? a. Aplastic anemia b. Thalassemia major c. Disseminated intravascular coagulation d. Immune thrombocytopenia

ANS: D Immune thrombocytopenia is an acquired hemorrhagic disorder characterized by an excessive destruction of platelets, discolorations caused by petechiae beneath the skin, and a normal bone marrow. Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin globin chains. Disseminated intravascular coagulation is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasma.

20. An 8-year-old girl is receiving a blood transfusion when the nurse notes that she has developed precordial pain, dyspnea, distended neck veins, slight cyanosis, and a dry cough. These manifestations are most suggestive of what associated adverse reaction? a. Air embolism b. Allergic reaction c. Hemolytic reaction d. Circulatory overload

ANS: D The signs of circulatory overload include distended neck veins, hypertension, crackles, dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Allergic reactions are manifested by urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema. Hemolytic reactions are characterized by chills, shaking, fever, pain at infusion site, nausea, vomiting, tightness in chest, flank pain, red or black urine, and progressive signs of shock and renal failure.