PEDS Chapter 43: Hematologic and Immunologic Dysfunction

42. A young child with leukemia has anorexia and severe stomatitis. The nurse should suggest that the parents try which intervention? a. Relax any eating pressures. b. Firmly insist that child eat normally. c. Begin gavage feedings to supplement diet. d. Serve foods that are either hot or cold.

ANS: A A multifaceted approach is necessary for children with severe stomatitis and anorexia. First, the parents should relax eating pressures rather than insisting the child eat normally. The nurse should suggest that the parents try soft, bland foods rather than hot or cold foods; normal saline or bicarbonate mouthwashes; and local anesthetics. The stomatitis is a temporary condition; gavage feedings are not necessary. The child can resume good food habits as soon as the condition resolves.

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

30. The nurse is planning care for an adolescent with acquired immunodeficiency syndrome. The priority nursing goal is to: a. Prevent infection. c. Restore immunologic defenses. b. Prevent secondary cancers. d. Identify source of infection.

ANS: A As a result of the immunocompromise that is associated with human immunodeficiency virus infection, the prevention of infection is paramount. Although certain precautions are justified in limiting exposure to infection, these must be balanced with the concern for the child's normal developmental needs. Restoring immunologic defenses is not currently possible. Current drug therapy is affecting the disease progression; although not a cure, these drugs can suppress viral replication, preventing further deterioration. Case finding is not a priority nursing goal.

16. A possible cause of acquired aplastic anemia in children is: a. Drugs. c. Deficient diet. b. Injury. d. Congenital defect.

ANS: A Drugs such as chemotherapeutic agents and several antibiotics such as chloramphenicol can cause aplastic anemia. Fanconi syndrome is a primary form of the disorder, which is congenital/present-at-birth and not acquired after birth. Injury, deficient diet, and congenital defect are not causative agents in acquired aplastic anemia.

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

ANS: A Perinatal transmission accounts for the highest percentage (91%) of HIV infections in children. 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.

48. 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.

44. An adolescent will receive a bone marrow transplant (BMT). The nurse should explain that the bone marrow will be administered by which route? a. Bone grafting c. Intravenous infusion b. Bone marrow injection d. Intraabdominal infusion

ANS: C Bone marrow from a donor is infused intravenously, not intraabdominally, and the transfused stem cells will repopulate the marrow. Because the stem cells migrate to the recipient's marrow when given intravenously, this method of administration is used rather than bone grafting or bone marrow injection.

10. Which clinical manifestation should the nurse expect when a child 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.

7. The nurse is recommending how to prevent iron deficiency anemia in a healthy, term, breastfed infant. What should she or he suggest? 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 infant cereal can be introduced at age 2 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.

22. A child with leukemia is receiving triple intrathecal chemotherapy consisting of methotrexate, cytarabine, and hydrocortisone. The purpose of this is to prevent: a. Infection. b. Brain tumor. c. Drug side effects. d. Central nervous system (CNS) disease.

ANS: D For certain children, CNS prophylactic therapy is indicated. This drug regimen is used to prevent CNS leukemia. This regimen does not prevent infection or drug side effects. If the child has a brain tumor in addition to leukemia, additional therapy would be indicated.

18. An acquired hemorrhagic disorder that is characterized by excessive destruction of platelets is: a. Aplastic anemia. b. Thalassemia major. c. Disseminated intravascular coagulation. d. Idiopathic thrombocytopenic purpura.

ANS: D Idiopathic thrombocytopenic purpura 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.

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.

41. The nurse is administering an intravenous chemotherapeutic agent to a child with leukemia. The child suddenly begins to wheeze and have severe urticaria. Which is the most appropriate nursing action? a. Stop drug infusion immediately. b. Recheck rate of drug infusion. c. Observe child closely for next 10 minutes. d. Explain to child that this is an expected side effect.

ANS: A If an allergic reaction is suspected, the drug should be immediately discontinued. Any drug in the line should be withdrawn, and a normal saline infusion begun to keep the line open. Rechecking the rate of drug infusion, observing the child closely for next 10 minutes, and explaining to the child that this is an expected side effect can all be done after the drug infusion is stopped and the child is evaluated.

35. 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.

31. An inherited immunodeficiency disorder characterized by absence of both humoral and cell-mediated immunity is: a. Severe combined immunodeficiency syndrome (SCIDS). b. Acquired immunodeficiency syndrome. c. Wiskott-Aldrich syndrome. d. Fanconi syndrome.

ANS: A Severe SCIDS is a genetic disorder that results in deficits of both humoral and cellular immunity. Acquired immunodeficiency syndrome is not inherited. Wiskott-Aldrich syndrome is an X-linked recessive disorder with selected deficiencies of T and B lymphocytes. Fanconi syndrome is a hereditary disorder of red cell production.

3. The most appropriate nursing diagnosis for a child with anemia is: 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.

43. The school nurse is informed that a child with human immunodeficiency virus (HIV) will be attending school soon. Which is an important nursing intervention? a. Carefully follow universal precautions. b. Determine how the child became infected. c. Inform the parents of the other children. d. Reassure other children that they will not become infected.

ANS: A Universal precautions are necessary to prevent further transmission of the disease. It is not the role of the nurse to determine how the child became infected. Informing the parents of other children and reassuring other children that they will not become infected is a violation of the child's right to privacy.

50. The nurse is preparing to give oral care to a school-age child with mucositis secondary to chemotherapy administered to treat leukemia. Which preparations should the nurse use for oral care on this child (Select all that apply)? a. Chlorhexidine gluconate (Peridex) b. Lemon glycerin swabs c. Antifungal troches (lozenges) d. Lip balm (Aquaphor) e. Hydrogen peroxide

ANS: A, C, D Preparations that may be used to prevent or treat mucositis include chlorhexidine gluconate (Peridex) because of its dual effectiveness against candidal and bacterial infections, antifungal troches (lozenges) or mouthwash, and lip balm (e.g., Aquaphor) to keep the lips moist. Agents that should not be used include lemon glycerin swabs (irritate eroded tissue and can decay teeth), hydrogen peroxide (delays healing by breaking down protein), and milk of magnesia (dries mucosa).

46. Which home care instructions should the nurse provide to the parents of a child with acquired immunodeficiency syndrome (AIDS) (Select all that apply)? a. Give supplemental vitamins as prescribed. b. Yearly influenza vaccination should be avoided. c. Administer trimethoprim-sulfamethoxazole (Bactrim) as prescribed. d. Notify the physician if the child develops a cough or congestion. e. Missed doses of antiretroviral medication do not need to be recorded.

ANS: A, C, D The parents should be taught that supplemental vitamins will be prescribed to aid in nutritional status. Bactrim is administered to prevent the opportunistic infection of Pneumocystis jiroveci pneumonia. The physician should be notified if the child with AIDS develops a cough and congestion. The yearly influenza vaccination is recommended, and any missed doses of antiretroviral medication need to be recorded and reported.

14. Chelation therapy is begun on a child with b-thalassemia major. The purpose of this therapy is to: a. Treat the disease. c. Decrease the risk of hypoxia. b. Eliminate excess iron. d. Manage 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.

23. A young boy will receive a bone marrow transplant (BMT). This is possible because one of his older siblings is a histocompatible donor. This type of BMT is termed: a. Syngeneic. c. Monoclonal. b. Allogeneic. d. Autologous.

ANS: B Allogeneic transplants are from another individual. Because he and his sibling are histocompatible, the bone marrow transplantation can be done. Syngeneic marrow is from an identical twin. There is no such thing as a monoclonal bone marrow transplant. Autologous refers to the individual's own marrow.

28. A young child with human immunodeficiency virus is receiving several antiretroviral drugs. The purpose of these drugs is to: 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. A condition in which the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin is: a. Aplastic anemia. c. Thalassemia major. b. Sickle cell anemia. 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.

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

20. A boy with leukemia screams whenever he needs to be turned or moved. The most probable cause of this pain is: a. Edema. c. Petechial hemorrhages. b. Bone involvement. d. Changes within the muscles.

ANS: B The invasion of the bone marrow with leukemic cells gradually causes a weakening of the bone and a tendency toward fractures. As leukemic cells invade the periosteum, increasing pressure causes severe pain. Edema, petechial hemorrhages, and muscular changes would not cause severe pain.

11. A school-age child is admitted in vaso-occlusive sickle cell crisis. The child's care should include: a. Correction of acidosis. b. Adequate hydration and pain management. c. Pain management and administration of heparin. d. Adequate oxygenation and replacement of factor VIII.

ANS: B 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. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels.

12. 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 explain that narcotic analgesics: a. Are often ordered but not usually needed. b. Rarely cause addiction because they are medically indicated. c. Are given as a last resort because of the threat of addiction. d. Are used only if other measures such as ice packs are ineffective.

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.

47. 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 (Tylenol) 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.

45. 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.

49. 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 a 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 bedwetting, such as limiting fluids at night. Enuresis is treated as a complication of the disease, such as joint pain or some other symptom, to alleviate parental pressure on the child. Ice should not be used during a vaso-occlusive pain crisis because it vasoconstricts and impairs circulation even more.

27. 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.

38. Which child should the nurse document as being anemic? a. 7-year-old child with a hemoglobin of 11.5 g/dL b. 3-year-old child with a hemoglobin of 12 g/dL c. 14-year-old child with a hemoglobin of 10 g/dL d. 1-year-old child with a hemoglobin of 13 g/dL

ANS: C Anemia is a condition in which the number of red blood cells, or hemoglobin concentration, is reduced below the normal values for age. Anemia is defined as a hemoglobin level below 10 or 11 g/dL. The child with a hemoglobin of 10 g/dL would be considered anemic. The normal hemoglobin for a child after 2 years of age is 11.5 to 15.5 g/dL.

39. 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.

37. Children receiving long-term systemic corticosteroid therapy are most at risk for: a. Hypotension. b. Dilation of blood vessels in the cheeks. c. Growth delays. d. Decreased appetite and weight loss.

ANS: C Growth delay is associated with long-term steroid use. Hypertension is a clinical manifestation of long-term systemic steroid administration. Dilation of blood vessels in the cheeks is associated with an excess of topically administered steroids. Increased appetite and weight gain are clinical manifestations of excess systemic corticosteroid therapy.

13. Which statement best describes b-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. Nursing considerations include: 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.

19. What is most descriptive of the pathophysiology of leukemia? a. Increased blood viscosity occurs. b. Thrombocytopenia (excessive destruction of platelets) occurs. c. Unrestricted proliferation of immature white blood cells (WBCs) occurs. d. The first stage of the coagulation process is abnormally stimulated.

ANS: C Leukemia is a group of malignant disorders of the bone marrow and the lymphatic system. It is defined as an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body. Increased blood viscosity may occur secondary to the increased number of WBCs. Thrombocytopenia may occur secondary to the overproduction of WBCs in the bone marrow. The coagulation process is unaffected by leukemia.

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.

17. 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.

24. A school-age child with leukemia experienced severe nausea and vomiting when receiving chemotherapy for the first time. The most appropriate nursing action to prevent or minimize these reactions with subsequent treatments is to: a. Encourage drinking large amounts of favorite fluids. b. Encourage child to take nothing by mouth (remain NPO) until nausea and vomiting subside. c. Administer an antiemetic before chemotherapy begins. d. Administer an antiemetic as soon as child has nausea.

ANS: C The most beneficial regimen to minimize nausea and vomiting associated with chemotherapy is to administer the antiemetic before the chemotherapy is begun. The goal is to prevent anticipatory symptoms. Drinking fluids will add to the discomfort of the nausea and vomiting. Encouraging the child to remain NPO will help with this episode, but the child will have the discomfort and be at risk for dehydration. Administering an antiemetic after the child has nausea does not avoid anticipatory nausea.

34. The parents of a child with cancer 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 continued.

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: 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.

25. The nurse is preparing a child for possible alopecia from chemotherapy. Which suggestion should be included in the teaching? a. Explaining to the child that hair usually regrows in 1 year. b. Advising the child to expose the head to sunlight to minimize alopecia. c. Explaining to the child that wearing a hat or scarf is preferable to wearing a wig. d. Explaining to the child that, when hair regrows, it may have a slightly different color or texture.

ANS: D Alopecia is a side effect of certain chemotherapeutic agents. When the hair regrows, it may be of different color or texture. The hair usually grows back within 3 to 6 months after the cessation of treatment. The head should be protected from sunlight to avoid sunburn. Children should choose the head covering that they prefer.

1. An accurate description of anemia is: 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.

26. A common clinical manifestation of Hodgkin's disease is: a. Petechiae. b. Bone and joint pain. c. Painful, enlarged lymph nodes. d. Enlarged, firm, nontender lymph nodes.

ANS: D Asymptomatic, enlarged, cervical or supraclavicular lymphadenopathy is the most common presentation of Hodgkin's disease. Petechiae are usually associated with leukemia. Bone and joint pain are not likely in Hodgkin's disease. The enlarged nodes are rarely painful.

32. Several complications can occur when a child receives a blood transfusion. An immediate sign or symptom of an air embolus is: a. Chills and shaking. c. Irregular heart rate. b. Nausea and vomiting. d. Sudden difficulty in breathing.

ANS: D Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Air emboli should be avoided by carefully flushing all tubing of air before connecting to the patient. Chills, shaking, nausea, and vomiting are associated with hemolytic reactions. Irregular heart rate is associated with electrolyte disturbances and hypothermia.

21. Myelosuppression associated with chemotherapeutic agents or some malignancies such as leukemia can cause bleeding tendencies because of a/an: a. Decrease in leukocytes. c. Vitamin C deficiency. b. Increase in lymphocytes. d. Decrease in blood platelets.

ANS: D The decrease in blood platelets secondary to the myelosuppression of chemotherapy can cause an increase in bleeding. The child and family should be alerted to avoid risk of injury. Decrease in leukocytes, increase in lymphocytes, and vitamin C deficiency would not affect bleeding tendencies.

33. 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: a. Air embolism. c. Hemolytic reaction. b. Allergic 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.

40. Which immunization should not be given to a child receiving chemotherapy for cancer? a. Tetanus vaccine c. Diphtheria, pertussis, tetanus (DPT) b. Inactivated poliovirus vaccine d. Measles, rubella, mumps

ANS: D The vaccine used for measles, mumps, and rubella is a live virus and can result in an overwhelming infection. Tetanus vaccine, inactivated poliovirus vaccine, and DPT are not live virus vaccines.