Ch. 43 Blueprint

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. b. Allogeneic. c. Monoclonal. d. Autologous.

: 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.

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

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.

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

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.

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

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.

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.

Chelation therapy is begun on a child with -thalassemia major. The purpose of this therapy is to: a. Treat the disease. b. Eliminate excess iron. c. Decrease the risk of hypoxia. 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. PTS: 1 DIF: Cognitive Level: Comprehension REF: 1374 OBJ: Nursing Process: Implementation MSC: Client Needs: Physiologic Integrity

A condition in which the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin is: 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. PTS: 1 DIF: Cognitive Level: Comprehension REF: 1367 OBJ: Nursing Process: Assessment MSC: Client Needs: Physiologic Integrity

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.

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

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.

Which statement best describes -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.

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.

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.

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.

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. PTS: 1 DIF: Cognitive Level: Application REF: 1365 OBJ: Nursing Process: Implementation MSC: Client Needs: Physiologic Integrity

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.

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.

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. PTS: 1 DIF: Cognitive Level: Comprehension REF: 1375 OBJ: Nursing Process: Diagnosis MSC: Client Needs: Physiologic Integrity

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.

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.

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.

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.

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. PTS: 1 DIF: Cognitive Level: Comprehension REF: 1379 OBJ: Nursing Process: Assessment MSC: Client Needs: Physiologic Integrity

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. b. Nausea and vomiting c. Irregular heart rate. 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.

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. 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.

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