Chapter 24: The Child with Hematologic or Immunologic Dysfunction

When both parents have sickle cell trait, which is the chance their children will have sickle cell anemia? a. 25% b. 50% c. 75% d. 100%

a. 25% Sickle cell anemia is inherited in an autosomal recessive pattern. If both parents have sickle cell trait (one copy of the sickle cell gene), then for each pregnancy, a 25% chance exists that their child will be affected with sickle cell disease. With each pregnancy, a 50% chance exists that the child will have sickle cell trait. Percentages of 75% and 100% are too high for the children of parents who have sickle cell trait.

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.

d. central nervous system (CNS) disease. For certain children, CNS prophylactic therapy is indicated. This drug regimen is used to prevent CNS leukemia and will not prevent infection or drug side effects. If the child has a brain tumor in addition to leukemia, additional therapy would be indicated.

A toddler with leukemia is on intravenous chemotherapy treatments. The toddler's lab results are WBC: 1000; neutrophils: 7%; nonsegmented neutrophils (bands): 7%. What is this child's absolute neutrophil count (ANC)? (Record your answer in a whole number.)

ANS: 140 To calculate an ANC for a WBC = 1000; neutrophils = 7%; and nonsegmented neutrophils (bands) = 7%, the steps are Step 1: 7% + 7% = 14%. Step 2: 0.14 1000 = 140 ANC.

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

a. Aplastic anemia 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.

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.

a. Carefully follow universal precautions. 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 children that they will not become infected is a violation of the child's right to privacy.

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

a. Chlorhexidine gluconate (Peridex) c. Antifungal troches (lozenges) d. Lip balm (Aquaphor) 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).

The nurse is teaching parents about the importance of iron in a toddler's diet. Which explains why iron deficiency anemia is common during toddler-hood? 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.

a. Milk is a poor source of iron. 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.

Parents of a child with sickle cell anemia ask the nurse, "What happens to the hemoglobin in sickle cell anemia?" Which statement by the nurse explains the disease process?" a. Normal adult hemoglobin is replaced by abnormal hemoglobin. b. There is a lack of cellular hemoglobin being produced. c. There is a deficiency in the production of globulin chains. d. The size and depth of the hemoglobin are affected.

a. Normal adult hemoglobin is replaced by abnormal hemoglobin. 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. Thalassemia major refers to a variety of inherited disorders characterized by deficiencies in production of certain globulin chains. Iron deficiency anemia affects the size and depth of the color.

The nurse is planning care for an adolescent with AIDS. Which is the priority nursing goal? a. Preventing infection b. Preventing secondary cancers c. Restoring immunologic defenses d. Identifying source of infection

a. Preventing infection Because the child is immunocompromised in association with HIV 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. Preventing secondary cancers 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.

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.

a. Relax any eating pressures. A multifaceted approach is necessary for children with severe stomatitis and anorexia. First, the parents should relax eating pressures. The nurse should suggest that the parents try soft, bland foods; normal saline or bicarbonate mouthwashes; and local anesthetics. The stomatitis is a temporary condition. The child can resume good food habits as soon as the condition resolves.

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.

a. Stop drug infusion immediately. 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.

A young child with human immunodeficiency virus (HIV) 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 carinii pneumonia.

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

The nurse is conducting a staff in-service on inherited childhood blood disorders. Which statement describes severe combined immunodeficiency syndrome (SCIDS)? a. There is a deficit in both the humoral and cellular immunity with this disease. b. Production of red blood cells is affected with this disease. c. Adult hemoglobin is replaced by abnormal hemoglobin in this disease. d. There is a deficiency of T and B lymphocyte production with this disease.

a. There is a deficit in both the humoral and cellular immunity with this disease. Severe combined immunodeficiency syndrome (SCIDS) is a genetic disorder that results in deficits of both humoral and cellular immunity. Wiskott-Aldrich is an X-linked recessive disorder with selected deficiencies of T and B lymphocytes. Fanconi syndrome is a hereditary disorder of red cell production. Sickle cell disease is characterized by the replacement of adult hemoglobin with an abnormal hemoglobin S.

A possible cause of acquired aplastic anemia in children is: a. drugs. b. injury. c. deficient diet. d. congenital defect.

a. drugs. Drugs, such as chemotherapeutic agents and several antibiotics (e.g., chloramphenicol), can cause aplastic anemia. Injury, deficient diet, and congenital defect are not causative agents in acquired aplastic anemia.

Meperidine (Demerol) is not recommended for children in sickle cell crisis because it: a. may induce seizures. b. is easily addictive. c. is not adequate for pain relief. d. is given by intramuscular injection. ANS: A A metabolite of meperidine, normeperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with sickle cell disease are particularly at risk for normeperidine-induced seizures. Meperidine is no more addictive than other narcotic agents. Meperidine is adequate for pain relief. It is available for IV infusion.

a. may induce seizures. A metabolite of meperidine, normeperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with sickle cell disease are particularly at risk for normeperidine-induced seizures. Meperidine is no more addictive than other narcotic agents. Meperidine is adequate for pain relief. It is available for IV infusion.

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

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

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. Finger sticks for blood work instead of venipunctures b. Avoidance of IM injections c. Acetaminophen (Tylenol) for mild pain control d. Soft tooth brush for dental hygiene e. Administration of packed red blood cells

b. Avoidance of IM injections c. Acetaminophen (Tylenol) for mild pain control d. Soft tooth brush for dental hygiene 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.

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

b. Bone involvement 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 changes within the muscles would not cause severe pain.

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 vasoocclusive pain in the legs. e. Notify the health care provider if your child begins to develop symptoms of a cold.

b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. 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 vasoocclusive pain crisis because it vasoconstricts and impairs circulation even more.

Which is often administered to prevent or control hemorrhage in a child with cancer? a. Nitrosoureas b. Platelets c. Whole blood d. Corticosteroids

b. Platelets Most bleeding episodes can be prevented or controlled with the administration of platelet concentrate or platelet-rich plasma. Nitrosoureas, whole blood, and corticosteroids would not prevent or control hemorrhage.

The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. Which is appropriate for the nurse to explain about 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

b. Rarely cause addiction because they are medically indicated 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 are 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 vasoocclusive crisis. Ice is contraindicated because of its vasoconstrictive effects.

The nurse has initiated a blood transfusion on a preschool child. The child begins to exhibit signs of a transfusion reaction. Place in order the interventions the nurse should implement sequencing from the highest priority to the lowest. Provide answer using lowercase letters separated by commas (e.g., a, b, c, d). a. Take the vital signs. b. Stop the transfusion. c. Notify the practitioner. d. Maintain a patent IV line with normal saline.

b. Stop the transfusion. a. Take the vital signs. d. Maintain a patent IV line with normal saline. c. Notify the practitioner. ANS: b, a, d, c If a blood transfusion reaction of any type is suspected, stop the transfusion, take vital signs, maintain a patent IV line with normal saline and new tubing, notify the practitioner, and do not restart the transfusion until the child's condition has been medically evaluated.

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

b. Swimming 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 sport such as soccer and basketball are not recommended.

Which immunization should be given with caution to children infected with human immunodeficiency virus (HIV)? a. Influenza b. Varicella c. Pneumococcal d. Inactivated poliovirus (IPV)

b. Varicella The children should be carefully evaluated before being given 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, pneumococcal, and inactivated poliovirus (IPV) are not live vaccines.

A school-age child is admitted in vasoocclusive 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.

b. adequate hydration and pain management. The management of crises includes adequate hydration, minimization of energy expenditures, pain management, electrolyte replacement, and blood component therapy if indicated. Hydration and pain control are two of the major goals of therapy. The acidosis will be corrected as the crisis is treated. Heparin and factor VIII is not indicated in the treatment of vasoocclusive 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 risk of hypoxia. d. manage nausea and vomiting.

b. eliminate excess iron. 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 effect of the disease management. Decreasing the risk of hypoxia and managing nausea and vomiting are not the purposes of chelation therapy.

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

c. 14-year-old child with a hemoglobin of 10 g/dl 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.

Which 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 c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency disease

c. Acquired immunodeficiency syndrome (AIDS) AIDS is caused by the human immunodeficiency virus (HIV), which primarily attacks the CD4+ T cells. Wiskott-Aldrich syndrome, idiopathic thrombocytopenic purpura, and severe combined immunodeficiency disease are not viral illnesses.

Which should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations? 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. Allow preparation to mix with saliva and bathe the teeth before swallowing.

c. Adequate dosage will turn the stools a tarry green color. 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, then 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.

A school-age child with leukemia experienced severe nausea and vomiting when receiving chemotherapy for the first time. Which is the most appropriate nursing action to prevent or minimize these reactions with subsequent treatments? 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.

c. Administer an antiemetic before chemotherapy begins. 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. Remaining until nausea and vomiting subside will help with this episode, but the child will have the discomfort and be at risk for dehydration. Administering an antiemetic as soon as the child has nausea does not prevent anticipatory nausea.

The nurse is teaching parents of an infant about the causes of iron deficiency anemia. Which statement best describes iron deficiency anemia in infants? a. It is caused by depression of the hematopoietic system. b. It is easily diagnosed because of an infant's emaciated appearance. c. Clinical manifestations are similar regardless of the cause of the anemia. d. Clinical manifestations result from a decreased intake of milk and the premature addition of solid foods.

c. Clinical manifestations are similar regardless of the cause of the anemia. In iron deficiency anemia, the child's clinical appearance is a result of the anemia, not the underlying cause. Usually the hematopoietic system is not depressed in iron deficiency anemia. The bone marrow produces red cells that are smaller and contain less hemoglobin than normal red cells. Children who are iron deficient from drinking excessive quantities of milk are usually pale and overweight. They are receiving sufficient calories, but are deficient in essential nutrients. The clinical manifestations result from decreased intake of iron-fortified solid foods and an excessive intake of milk.

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 b. Bone marrow injection c. IV infusion d. Intra-abdominal infusion

c. IV infusion Bone marrow from a donor is infused intravenously, and the transfused stem cells will repopulate the marrow. Because the stem cells migrate to the recipient's marrow when given intravenously, this is the method of administration.

Which statement best describes -thalassemia major (Cooley 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.

c. Increased incidence occurs in families of Mediterranean extraction. 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 persons of West African descent.

The nurse is conducting a staff in-service on sickle cell anemia. Which 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 adhesion of sickle-shaped cells occurs.

c. Increased red blood cell destruction occurs. 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. Increased adhesion and entanglement of cells occurs.

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

c. Puppet play in the child's room 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.

Which 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. First stage of coagulation process is abnormally stimulated.

c. Unrestricted proliferation of immature white blood cells (WBCs) occurs. Leukemia is a group of malignant disorders of the bone marrow and 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.

Parents of a hemophiliac child ask the nurse, "Can you describe hemophilia to us?" Which response by the nurse 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

c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient 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 inheritance pattern is X-linked recessive. The disorder involves coagulation factors, not platelets, and does not involve red cells or the Y chromosomes.

Iron dextran is ordered for a young child with severe iron deficiency anemia. Nursing considerations include to: a. administer with meals. b. administer between meals. c. inject deeply into a large muscle. d. massage injection site for 5 minutes after administration of drug.

c. inject deeply into a large muscle. Iron dextran is a parenteral form of iron. When administered intramuscularly, it must be injected into a large muscle. Iron dextran is for intramuscular or intravenous (IV) administration. The site should not be massaged to prevent leakage, potential irritation, and staining of the skin.

Several blood tests are ordered for a preschool child with severe anemia. The child is crying and upset because of memories of the venipuncture done at the clinic 2 days ago. The nurse should explain: 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.

c. topical application of local anesthetic can eliminate venipuncture pain. Preschool children are concerned with both pain and the loss of blood. When preparing the child for venipuncture, the nurse will use a topical anesthetic to eliminate any pain. This is a 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. The nurse should not promise one attempt in case multiple attempts are required. Both finger punctures and venipunctures are traumatic for children. Both require preparation.

The nurse is reviewing first aid with a group of school nurses. Which statement made by a participant indicates a correct understanding of the information? a. "If a child loses a tooth due to injury, I should place the tooth in warm milk." b. "If a child has recurrent abdominal pain, I should send him or her back to class until the end of the day." c. "If a child has a chemical burn to the eye, I should irrigate the eye with normal saline." d. "If a child has a nosebleed, I should have the child sit up and lean forward."

d. "If a child has a nosebleed, I should have the child sit up and lean forward." If a child has a nosebleed, the child should lean forward, not lie down. A tooth should be placed in cold milk or saliva for transporting to a dentist. Recurrent abdominal pain is a physiologic problem and requires further evaluation. If a chemical burn occurs in the eye, the eye should be irrigated with water for 20 minutes.

The nurse is conducting a staff in-service on childhood blood disorders. Which describes the pathology of idiopathic thrombocytopenic purpura? a. Bone marrow failure in which all elements are suppressed b. Deficiency in the production rate of globin chains c. Diffuse fibrin deposition in the microvasculature d. An excessive destruction of platelets

d. An excessive destruction of platelets 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.

Which is the most effective pain-management approach for a child who is having a bone marrow aspiration? a. Relaxation techniques b. Administration of an opioid c. EMLA cream applied over site d. Conscious or unconscious sedation

d. Conscious or unconscious sedation Effective pharmacologic and nonpharmacologic measures should be used to minimize pain associated with procedures. For bone marrow aspiration, conscious or unconscious sedation should be used. Relaxation, opioids, and EMLA can be used to augment the conscious or unconscious sedation.

Which is a common clinical manifestation of Hodgkin disease? a. Petechiae b. Bone and joint pain c. Painful, enlarged lymph nodes d. Enlarged, firm, nontender lymph nodes

d. Enlarged, firm, nontender lymph nodes Asymptomatic, enlarged, cervical or supraclavicular lymphadenopathy is the most common presentation of Hodgkin disease. Petechiae are usually associated with leukemia. Bone and joint pain are not likely in Hodgkin disease. The enlarged nodes are rarely painful.

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

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

The nurse is recommending how to prevent iron deficiency anemia in a healthy, term, breast-fed infant. Which should be suggested? a. Iron (ferrous sulfate) drops after age 1 month b. Iron-fortified commercial formula by age 4 to 6 months c. Iron-fortified infant cereal by age 2 months d. Iron-fortified infant cereal by age 4 to 6 months

d. Iron-fortified infant cereal by age 4 to 6 months Breast milk supplies inadequate iron for growth and development after age 5 months. Supplementation is necessary at this time. The mother can supplement the breastfeeding with iron-fortified infant cereal. Iron supplementation or the introduction of solid foods in a breast-fed baby is not indicated. Providing iron-fortified commercial formula by age 4 to 6 months should be done only if the mother is choosing to discontinue breastfeeding.

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

d. Measles, rubella, mumps 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 diphtheria, pertussis, tetanus (DPT) are not live virus vaccines.

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

d. Painful swelling of hands and feet; painful joints A vasoocclusive 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 vasoocclusive phenomena.

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

d. Sudden difficulty in breathing 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 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 emboli. b. allergic reaction. c. hemolytic reaction. d. circulatory overload.

d. circulatory overload. 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.

Myelosuppression, associated with chemotherapeutic agents or some malignancies such as leukemia, can cause bleeding tendencies because of a(n): a. decrease in leukocytes. b. increase in lymphocytes. c. vitamin C deficiency. d. decrease in blood platelets.

d. decrease in blood platelets. 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.