Chapter 24: Hematologic or Immunologic Dysfunction

The nurse is administering a unit of blood to a child. What are signs and symptoms of a transfusion reaction? (Select all that apply.) a. Chills b. Shaking c. Flank pain d. Hypothermia e. Sudden severe headache

ANS: A, B, C, E Signs and symptoms of a transfusion reaction include chills, shaking, flank pain, and sudden severe headache. Hyperthermia, not hypothermia, occurs.

The school nurse is caring for a boy with hemophilia who fell on his arm during recess. What supportive measures should the nurse use until factor replacement therapy can be instituted? A. Apply warm, moist compresses. B. Apply pressure for at least 1 minute. C. Elevate the area above the level of the heart. D. Begin passive range-of-motion unless the pain is severe.

ANS: C The initial response should include elevation of the arm to minimize bleeding. Cold should be applied to the arm. This will aid in vasoconstriction, minimizing blood loss. Pressure is effective in small areas but would not be as effective for an extremity. Passive range-of-motion is not recommended. The child can perform active range-of-motion after the bleeding episode has resolved.

The nurse is teaching parents of a child being discharged from the hospital after a splenectomy about the risk of infection. What should the nurse include in the teaching session? (Select all that apply.) a. Avoid obtaining the pneumococcal vaccination for the child. b. Avoid obtaining the meningococcal vaccination for the child. c. The child should receive prophylactic penicillin for certain procedures. d. Have the child immunized with the Haemophilus influenzae type b vaccination. e. Notify the health care provider if your child develops a fever of 38.5 C (101.3 F).

ANS: C, D, E Because of the risk of life-threatening bacterial infection after splenectomy, these children are immunized with the pneumococcal, meningococcal, and H. influenzae type b vaccines before surgery and receive prophylactic penicillin for several years after splenectomy. The parents should be instructed in the importance of seeking immediate medical attention if their child develops a fever of 38.5 C (101.3 F) or higher as a common sign of infection or postsplenectomy sepsis.

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.

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.

A 5-year-old child is admitted to the hospital in a sickle cell crisis. The child has been alert and oriented but in severe pain. The nurse notes that the child is complaining of a headache and is having unilateral hemiplegia. What action should the nurse implement? a. Notify the health care provider. b. Place the child on bed rest. c. Administer a dose of hydrocodone (Vicodin). d. Start O2 per the hospitals protocol.

ANS: A Any number of neurologic symptoms can indicate a minor cerebral insult, such as headache, aphasia, weakness, convulsions, visual disturbances, or unilateral hemiplegia. Loss of vision is usually the result of progressive retinopathy and retinal detachment. The nurse should notify the health care provider.

What is the most important nursing consideration when caring for a child with sickle cell anemia? A. Teach the parents and child how to minimize crises. B. Refer the parents and child for genetic counseling. C. Help the child and family to adjust to a short-term disease. D. Observe for complications of multiple blood transfusions.

ANS: A Children and their families need specific instructions on how to minimize crises, including preventing infections; maintaining adequate hydration; and addressing environmental concerns, such as avoidance of extreme cold. Genetic counseling is important, but teaching care for the child is a priority. Sickle cell anemia is a long-term, chronic illness. Multiple blood transfusions are an option for some children with sickle cell disease. The priority is that the child and the parents are properly prepared to manage the chronic disease.

What pain medication is contraindicated in children with sickle cell disease (SCD)? a. Meperidine (Demerol) b. Hydrocodone (Vicodin) c. Morphine sulfate d. Ketorolac (Toradol)

ANS: A Meperidine (pethidine [Demerol]) is not recommended. Normeperidine, a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with SCD are particularly at risk for normeperidine-induced seizures.

What rationale explains why prolonged use of oxygen should be discouraged in a child with anemia? a. Prolonged use of oxygen can decrease erythropoiesis. b. Prolonged use of oxygen can interfere with iron production. c. Prolonged use of oxygen interferes with a childs appetite. d. Prolonged use of oxygen can affect the synthesis of hemoglobin.

ANS: A Oxygen administration is of limited value, because each gram of hemoglobin is able to carry a limited amount of the gas. In addition, prolonged use of supplemental oxygen can decrease erythropoiesis. Prolonged use of oxygen does not interfere with iron production, a childs appetite, or affect the synthesis of hemoglobin.

A child with hemophilia A is scheduled for surgery. What precautions should the nurse institute with this child? a. Handle the child gently when transferring to a cart. b. Caution the child not to brush his teeth before surgery. c. Use tape sparingly on postoperative dressings. d. Do not administer analgesics before surgery.

ANS: A The goal of prevention of bleeding episodes is directed toward decreasing the risk of injury. The child should be handled carefully when transferring to a cart. Brushing teeth, use of tape, and giving analgesics will not risk a bleeding episode.

You are working with a recent graduate on the pediatric unit. You are assigned to take care of an adolescent with B-thalassemia. The nurse needs more information about this disease if she states which of the following? Select all that apply: A. "We need to check the patients iron level to make sure he is not anemic." B. "I believe this is the most common in those of Hispanic descent, although this patient is Mediterranean." C. "The doctor will be prescribing deferasirox (Exjade) or deferoxamine (Desferal) for chelation therapy." D. "This patient looks much younger than I would expect. I guess he's just a late bloomer." E. "I think a transfusion will be ordered, because his hemoglobin level is 9.0."

ANS: A, B, D

What are signs and symptoms of anemia? (Select all that apply.) a. Pallor b. Fatigue c. Dilute urine d. Bradycardia e. Muscle weakness

ANS: A, B, E Signs and symptoms of anemia include, pallor, fatigue, and muscle weakness. Tachycardia, not bradycardia, and dark urine, not dilute, are signs and symptoms of anemia.

The clinic nurse is evaluating causes for iron deficiency caused by inadequate supply of iron. What should the nurse recognize as causes for iron deficiency caused by an inadequate iron supply? (Select all that apply.) a. Prematurity b. Slow growth rate c. Excessive milk intake d. Severe iron deficiency in the mother e. Exclusive breastfeeding of infant from birth to 3 months

ANS: A, C, D Causes for iron deficiency caused by an inadequate supply of iron include prematurity, excessive milk intake, and severe iron deficiency in the mother. Rapid growth rate, not slow, and exclusive breastfeeding of infant after 6 months, not from birth to 3 months, can be causes of inadequate supply of iron.

The nurse is caring for a 14-year-old child with disseminated intravascular coagulation (DIC). What clinical manifestations should the nurse expect to observe? (Select all that apply.) a. Petechiae b. Chronic diarrhea c. Hepatosplenomegaly d. Bleeding from openings in the skin e. Hypotension f. Purpura

ANS: A, D, E, F Some clinical manifestations of DIC are petechiae, bleeding from openings in the skin, hypotension, and purpura. Hepatosplenomegaly and chronic diarrhea are clinical manifestations of human immunodeficiency virus (HIV) infection in children.

Care for the child with acute idiopathic thrombocytopenic purpura (ITP) includes which therapeutic intervention? a. Splenectomy b. Intravenous administration of anti-D antibody c. Use of nonsteroidal anti-inflammatory drugs (NSAIDs) d. Helping child participate in sports

ANS: B Anti-D antibody causes an increase in platelet count approximately 48 hours after administration. Splenectomy is reserved for chronic severe ITP not responsive to pharmacologic management. NSAIDs are not used in ITP. Both NSAIDs and aspirin interfere with platelet aggregation. The nurse works with the child and parents to choose quiet activities while the platelet count is below 100,000/mm3.

Iron overload is a side effect of chronic transfusion therapy. What treatment assists in minimizing this complication? a. Magnetic therapy b. Infusion of deferoxamine c. Hemoglobin electrophoresis d. Washing red blood cells (RBCs) to reduce iron

ANS: B Deferoxamine infusions in combination with vitamin C allow the iron to remain in a more chelatable form. The iron can then be excreted more easily. Use of magnets does not remove additional iron from the body. Hemoglobin electrophoresis is used to confirm the diagnosis of hemoglobinopathies; it does not affect iron overload. Washed RBCs remove white blood cells and other proteins from the unit of blood; they do not affect the iron concentration.

A child with sickle cell disease is in a vasoocclusive crisis. What nonpharmacologic pain intervention should the nurse plan? a. Exercise as a distraction b. Heat to the affected area c. Elevation of the extremity d. Cold compresses to the affected area

ANS: B Frequently, heat to the affected area is soothing. Cold compresses are not applied to the area because doing so enhances vasoconstriction and occlusion. Bed rest is usually well tolerated during a crisis, although the actual rest obtained depends a great deal on pain alleviation and the use of organized schedules of nursing care. Although the objective of bed rest is to minimize oxygen consumption, some activity, particularly passive range of motion exercises, is beneficial to promote circulation. Usually the best course is to let children determine their activity tolerance. Elevating the extremity will not help in sickle cell disease.

Nursing strategies to improve the growth and development of the child with human immunodeficiency virus (HIV) infection should include what? a. Provide only those foods that the child feels like eating. b. Fortify foods with nutritional supplements to maximize quality of intake. c. Weigh the child and measure height and muscle mass on a daily basis. d. Provide high-fat and high-calorie meals and snacks to meet body requirements for growth.

ANS: B HIV infection often leads to marked failure to thrive and multiple nutritional deficiencies. Nutritional management may be difficult because of recurrent illness, diarrhea, and other physical problems. The nurse should implement intensive nutritional interventions if the childs growth begins to slow or weight begins to decrease. Fortifying foods with nutritional supplements will maximize quality of intake. The child does not need to be weighed daily, and high-fat meals and snacks should not be encouraged.

The regulation of red blood cell (RBC) production is thought to be controlled by which physiologic factor? a. Hemoglobin b. Tissue hypoxia c. Reticulocyte count d. Number of RBCs

ANS: B Hemoglobin does not directly control RBC production. If there is insufficient hemoglobin to adequately oxygenate the tissue, then erythropoietin may be released. When tissue hypoxia occurs, the kidneys release erythropoietin into the bloodstream. This stimulates the marrow to produce new RBCs. Reticulocytes are immature RBCs. The retic count can be used to monitor hematopoiesis. The number of RBCs does not directly control production. In congenital cardiac disorders with mixed blood flow or decreased pulmonary blood flow, RBC production continues secondary to tissue hypoxia.

The clinic nurse is evaluating lab results for a child. What recorded hematocrit (Hct) result is considered within the normal range? a. 30% b. 40% c. 50% d. 60%

ANS: B Normal hematocrit (Hct) is 35% to 45%.

The nurse is caring for a school-age child with severe anemia and activity intolerance. What diversional activity should the nurse plan for this child? a. Playing a musical instrument b. Playing board or card games c. Participating in a game of table tennis d. Participating in decorating the hospital room

ANS: B Plan diversional activities that promote rest but prevent boredom and withdrawal. Because short attention span, irritability, and restlessness are common in anemia and increase stress demands on the body, plan appropriate activities such as playing board or card games. Playing a musical instrument, participating in a game of table tennis, or decorating the hospital room would cause undue exertion.

A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse? a. Administer 100% oxygen to relieve hypoxia. b. Notify the practitioner because chest syndrome is suspected. c. Infuse intravenous antibiotics as soon as cultures are obtained. d. Give ordered pain medication to relieve symptoms of pain episode.

ANS: B These are the symptoms of chest syndrome, which is a medical emergency. Notifying the practitioner is the priority action. Oxygen may be indicated; however, it does not reverse the sickling that has occurred. Antibiotics are not indicated initially. Pain medications may be required, but evaluation by the practitioner is the priority.

A child is status post hematopoietic stem cell transplantation (HSCT) and is preparing for discharge home. Based on the nurse's knowledge of HSCT, which concepts are important to include in the discharge teaching plan of care? Select all that apply. A. Preparing the child to return to school within 6 weeks B. Keeping the child on a high-calcium diet C. Avoiding live plants and fresh vegetables D. Avoiding influenza vaccinations E. Practicing good hygiene

ANS: B, C, E Children should have a diet high in calcium or be placed on calcium supplements to reduce the risk of osteopenia. Live plants and fresh vegetables should be avoided because they carry bacteria. Practicing good hand hygiene is essential to prevent the spread of infection. Children cannot return to school for 6-12 months after HSCT. Either in-hospital or home schooling is required. Children and their families should be encouraged to get yearly influenza vaccination.

Critical safety considerations that must be included if parenteral iron injections are used as part of therapy include Select all that apply. A. type and cross match. B. no massage following injection. C. multiple injections are preferred over intravenous route. D. no more than 1 ml should be given via injection. E. there is no need for a test dose administration if administered intravenously.

ANS: B, D There is no need to perform a type and cross for iron replacement therapy. Administration via injection is done into a large muscle mass using Z track technique with no massage following. Preference is for intravenous route over intramuscular route if more than one injection is needed. No more than 1 ml is given via injection and a test dose is recommended if using intravenous route to high potential for allergic reaction.

A child with sickle cell anemia (SCA) is admitted in a vaso-occlusive crisis (VOC). Which of the following interventions should the nurse expect to see ordered? Select all that apply: A. Cold compresses to painful joints B. IV fluids started, and oral fluids encouraged C. Meperidine ordered every 4 hours for pain D. High-calorie, high -protein diet E. Antibiotics ordered for any existing infection

ANS: B, D, E

A child is admitted to the pediatric unit. The mother reports that the doctor says her son is anemic. What lab findings/manifestations would the nurse expect to see to confirm iron deficiency anemia? A. Cyanosis, due to inadequate oxygen saturation of existing hemoglobin B. A decreased reticulocyte count C. A total iron-binding capacity (TIBC) that is elevated above the normal range D. Decreased blood pressure changes, which are an early sign because of the compensatory mechanisms

ANS: C

Which is the most accurate genetic explanation for a family with hemophilia? A. It is a Y-linked dominant disorder B. It is equally distributed among males and females C. It is an X-linked recessive disorder D. It is an autosomal recessive disorder

ANS: C

A child has been diagnosed with aplastic anemia and undergoing therapeutic treatment. Therapeutic therapy would focus on A. palliative treatment to maintain comfort. B. initiation of steroid therapy. C. anticipation of bone marrow transplant. D. asking parents if they want to consider organ donation.

ANS: C Although a clinical diagnosis of aplastic anemia can lead to increased morbidity and mortality treatment measures focusing on immunosuppressive therapy, removal of potential exacerbating etiology, and bone marrow replacement. Palliative treatment methods may be included but they are not the mainstay of therapeutic management. Steroid therapy is not indicated as that may lead to an increased susceptibility to infection. Asking the parents about organ donation at this time may cause considerable distress and anxiety.

In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxiaischemia cycle. What information should the nurse share with parents in a teaching plan? a. Encourage drinking. b. Keep accurate records of output. c. Check for moist mucous membranes. d. Monitor the concentration of the childs urine.

ANS: C Children with SCA have impaired kidney function and cannot concentrate urine. Parents are taught signs of dehydration and ways to minimize loss of fluid to the environment. Encouraging drinking is not specific enough for parents. The nurse should give the parents and child a target fluid amount for each 24-hour period. Accurate monitoring of output may not reflect the childs fluid needs. Without the ability to concentrate urine, the child needs additional intake to compensate. Dilute urine and specific gravity are not valid signs of hydration status in children with SCA.

What medication is classified as an antiretroviral? a. Dapsone (Aczone) b. Pentamidine (Pentam) c. Didanosine (Videx) d. Trimethoprimsulfamethoxazole (Bactrim)

ANS: C Classes of antiretroviral agents include nucleoside reverse transcriptase inhibitors (e.g., zidovudine, didanosine, stavudine, lamivudine, abacavir), nonnucleoside reverse transcriptase inhibitors (e.g., nevirapine, delavirdine, efavirenz), and protease inhibitors (e.g., indinavir, saquinavir, ritonavir, nelfinavir, amprenavir, lopinavir, ritonavir). Dapsone, pentamidine, and Bactrim are anti-infectives.

A child has frequent nose bleeds without warning producing varying amount of blood. Inspection of the nose area reveals irritated mucosa but no evidence of picking or trauma. The child has no history of allergic rhinitis or upper respiratory infections. Denies taking any medication, either prescribed or over the counter. Based on this information the nurse would: A. maintain wait full management as most nose bleeds originate from anterior portion of nose. B. suggest that the patient use humidification to prevent dryness of nasal passages. C. consider referral to ENT for evaluation for other comorbidities. D. increase fluid content to maintain humidification as this may be due to environmental dryness.

ANS: C Even though the majority of nose bleeds are considered to be from the anterior portion of the nose, the fact that the patient has frequent nose bleeds without warning in the absence of irritation or disease states alerts the nurse that there may be other underlying comorbidities such as vascular disorders, leukemia, thrombocytopenia, and clotting factor deficiencies. As such the patient should be referred for further evaluation.

What condition is an acquired hemorrhagic disorder that is characterized by excessive destruction of platelets? a. Aplastic anemia b. Thalassemia major c. Idiopathic thrombocytopenic purpura d. Disseminated intravascular coagulation

ANS: C Idiopathic thrombocytopenic purpura is an acquired hemorrhagic disorder characterized by an excessive destruction of platelets, discolorations caused by petechiae beneath the skin, and 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 chains. Disseminated intravascular coagulation is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasma.

The nurse is teaching the family of a child, age 8 years, with moderate hemophilia about home care. What should the nurse tell the family to do to minimize joint injury? a. Administer nonsteroidal anti-inflammatory drugs (NSAIDs). b. Administer DDAVP (synthetic vasopressin). c. Provide intravenous (IV) infusion of factor VIII concentrates. d. Encourage elevation and application of ice to the involved joint.

ANS: C Parents are taught home infusion of factor VIII concentrate. For moderate and severe hemophilia, prompt IV administration is essential to prevent joint injury. NSAIDs are effective for pain relief. They must be given with caution because they inhibit platelet aggregation. A factor VIII level of 30% is necessary to stop bleeding. DDAVP can raise the factor VIII level fourfold. Moderate hemophilia is defined by a factor VIII activity of 4.9. A fourfold increase would not meet the 30% level. Ice and elevation are important adjunctive therapy, but factor VIII is necessary.

The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. What statement most accurately reflects inheritance of SCA? a. SCA is not inherited. b. All siblings will have SCA. c. Each sibling has a 25% chance of having SCA. d. There is a 50% chance of siblings having SCA.

ANS: C SCA is inherited as an autosomal recessive disorder. In this inheritance pattern, each child born to these parents has a 25% chance of having the disorder, a 25% chance of having neither SCA nor the trait, and a 50% chance of being heterozygous for SCA (sickle cell trait). SCA is an inherited hemoglobinopathy.

The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. Which response by the nurse is most accurate? A. "SCA is not inherited." B. "All siblings will have SCA." C. "There is a 25% chance of a sibling having SCA." D. "There is a 50% chance of a sibling having SCA."

ANS: C SCA is inherited as an autosomal recessive disorder. In this inheritance pattern, there is a 25% chance that each subsequent child will have the disorder. SCA is an inherited hemoglobinopathy. In autosomal recessive disorders, there is a chance that 25% of the children

Sickle cell disease (SCD) occurs through a genetic mutation. Based on the understanding of this genetic form of transmission, the nurse understands that A. there are no carrier states associated with this disease. B. the disease is transmitted as part of a sex-linked mutation. C. SCD refers to a group of congenital disease expressions. D. it is a relatively uncommon disease as it is expressed as an autosomal recessive gene trait.

ANS: C SCD refers to a group of heredity disease states in which there is variants exhibited in both heterozygous and homozygous expressions. It is expressed as an autosomal recessive trait and as such there are carrier states. It is not transmitted as a sex-linked mutation. It is one of the most common genetic disorders globally.

A mother states that she brought her child to the clinic because the 3-year-old girl was not keeping up with her siblings. During physical assessment, the nurse notes that the child has pale skin and conjunctiva and has muscle weakness. The hemoglobin on admission is 6.4 g/dl. After notifying the practitioner of the results, what nursing priority intervention should occur next? a. Reduce environmental stimulation to prevent seizures. b. Have the laboratory repeat the analysis with a new specimen. c. Minimize energy expenditure to decrease cardiac workload. d. Administer intravenous fluids to correct the dehydration.

ANS: C The child has a critically low hemoglobin value. The expected range is 11.5 to 15.5 g/dl. When the oxygen-carrying capacity of the blood decreases slowly, the child is able to compensate by increasing cardiac output. With the increasing workload of the heart, additional stress can lead to cardiac failure. Reduction of environmental stimulation can help minimize energy expenditure, but seizures are not a risk. A repeat hemoglobin analysis is not necessary. The child does not have evidence of dehydration. If intravenous fluids are given, they can further dilute the circulating blood volume and increase the strain on the heart.

A child with severe anemia requires a unit of red blood cells (RBCs). The nurse explains to the child that the transfusion is necessary for which reason? a. Allow her parents to come visit her. b. Fight the infection that she now has. c. Increase her energy so she will not be so tired. d. Help her body stop bleeding by forming a clot (scab).

ANS: C The indication for RBC transfusion is risk of cardiac decompensation. When the number of circulating RBCs is increased, tissue hypoxia decreases, cardiac function is improved, and the child will have more energy. Parental visiting is not dependent on transfusion. The decrease in tissue hypoxia will minimize the risk of infection. There is no evidence that the child is currently infected. Forming a clot is the function of platelets.

What immunoglobulin pattern does the nurse expect in a child recently diagnosed with Wiskott-Aldrich syndrome? a. Diminished levels of IgG b. Diminished levels of IgA c. Diminished levels of IgM d. Diminished levels of IgE

ANS: C The level of IgM is diminished early in the course of the disease, but levels of IgG, IgA, and IgE may be elevated initially.

A child with β-thalassemia is receiving numerous blood transfusions. In addition, the child is receiving deferoxamine (Desferal) therapy. The child's parents ask the nurse what deferoxamine does. The most appropriate response by the nurse is A. the medication helps to prevent blood transfusion reactions. B. the medication stimulates red blood cell production. C. the medication provides vitamin supplementation. D. the medication helps to prevent iron overload.

ANS: D A side effect of hypertransfusion therapy is often iron overload. Deferoxamine is an iron-chelating drug that binds excess iron; therefore, it can be excreted by the kidneys. It does not prevent blood transfusions nor stimulate red bell production. It is not a vitamin supplement.

What physiologic defect is responsible for causing anemia? a. Increased blood viscosity b. Depressed hematopoietic system c. Presence of abnormal hemoglobin d. Decreased oxygen-carrying capacity of blood

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

Question 2 of 23 A child is coming to the clinic with his parents for a sports physical. Upon reviewing the patient's history, there is a notation that the child has been recently treated six months ago for immune thrombocytopenia purpura (ITP). Based on this notation, the nurse would provide this recommendation? A. The child should not play any sports or participate in any physical activity while in school. B. The child should take art classes rather than participate in sporting events. C. The child will have to repeat blood work before any determination can be made. D. The child can play non-contact sports but will have to be monitored if any bruising or bleeding should occur.

ANS: D Contact sports should be avoided in a patient who has had prior treatment for ITP. The nature of ITP being idiopathic, it is important to protect the patient from impending trauma related to contact. However, the child as part of normal growth and development should be able to engage in non-contact sports activities. Being that the treatment was recent and that ITP is idiopathic, prospective management and monitoring should be included in the plan of care at this time.

For children who do not have a matched sibling bone marrow donor, the therapeutic management of aplastic anemia includes what intervention? a. Antibiotics b. Antiretroviral drugs c. Iron supplementation d. Immunosuppressive therapy

ANS: D It is thought that aplastic anemia may be an autoimmune disease. Immunosuppressive therapy, including antilymphocyte globulin, antithymocyte globulin, cyclosporine, granulocyte colony-stimulating factor, and methylprednisone, has greatly improved the prognosis for patients with aplastic anemia. Antibiotics are not indicated as the management. They may be indicated for infections. Antiretroviral drugs and iron supplementation are not part of the therapy.

The majority of children in the United States with human immunodeficiency virus (HIV) infection acquired the disease by which means? a. Through sexual contact b. From a blood transfusion c. By using intravenous (IV) drugs d. Perinatally from their mothers

ANS: D More than 90% of the children with HIV under 13 years who were reported to the Centers for Disease Control and Prevention acquired the infection during the perinatal period. With intervention, the number of children infected can be decreased. Sexual contact and IV drug use are the leading causes of infection in the 14- to 19-year age group. This number is less than the number of cases in the under 13-year age group. Transfusion has accounted for 3% to 6% of all pediatric acquired immunodeficiency syndrome cases to date. Before 1985 and routine screening of donated blood products, children with hemophilia were at great risk from pooled plasma products.

The clinic nurse is evaluating lab results for a child. What recorded hemoglobin (Hgb) result is considered within the normal range? a. 9 g/dl b. 10 g/dl c. 11 g/dl d. 12 g/dl

ANS: D Normal hemoglobin (Hgb) determination is 11.5 to 15.5 g/dl.

What condition precipitates polycythemia? a. Dehydration b. Severe infections c. Immunosuppression d. Prolonged tissue hypoxia

ANS: D Oxygen transport depends on both the number of circulating RBCs and the amount of normal hemoglobin in the cell. This explains why polycythemia (increase in the number of erythrocytes) occurs in conditions characterized by prolonged tissue hypoxia, such as cyanotic heart defects. Dehydration, severe infections, or immunosuppression will not precipitate polycythemia.

The nurse is caring for a child with hemophilia A. The childs activity is as tolerated. What activity is contraindicated for this child? a. Ambulating to the cafeteria b. Active range of motion c. Ambulating to the playroom d. Passive range of motion exercises

ANS: D Passive range of motion exercises should never be part of an exercise regimen after an acute episode because the joint capsule could easily be stretched and bleeding could recur. Active range of motion exercises are best so that the patient can gauge his or her own pain tolerance. The child can ambulate to the playroom or the cafeteria.

The nurse is explaining blood components to an 8-year-old child. Based on the nurse's knowledge of child development, the most appropriate description of platelets is that they A. help keep germs from causing infection. B make up the liquid portion of blood. C. carry the oxygen you breathe from your lungs to all parts of your body. D. help your body stop bleeding by forming a clot (scab) over the hurt area.

ANS: D Platelets are involved in homeostasis. White blood cells help protect the body from infection. The liquid portion of blood id known as plasma. Red blood cells are involved in oxygenation of tissues in the body.

The nurse is preparing a community outreach program about the prevention of iron-deficiency anemia in infants. What statement should the nurse include in the program? a. Whole milk can be introduced into the infants diet in small amounts at 6 months. b. Iron supplements cannot be given until the infant is older than 1 year of age. c. Iron-fortified cereal should be introduced to the infant at 2 months of age. d. Breast milk or iron-fortified formula should be used for the first 12 months.

ANS: D Prevention, the primary goal in iron-deficiency anemia, is achieved through optimal nutrition and appropriate iron supplements. The American Academy of Pediatrics recommends feeding an infant only breast milk or iron-fortified formula for the first 12 months of life. Whole cows milk should not be introduced until after 12 months, iron supplements can be given during the first year of life, and iron-fortified cereals should not be introduced until the infant is 4 to 6 months old.

What condition is an inherited immunodeficiency disorder characterized by absence of both humoral and cell-mediated immunity? a. Fanconi syndrome b. Wiskott-Aldrich syndrome c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency syndrome (SCIDS)

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

A child with hemophilia A will have which abnormal laboratory result? a. PT (ProTime) b. Platelet count c. Fibrinogen level d. PTT (partial thromboplastin time)

ANS: D The basic defect of hemophilia A is a deficiency of factor VIII. The partial thromboplastin time measures abnormalities in the intrinsic pathway (abnormalities in factors I, II, V, VIII, IX, X, XII, HMK, and KAL). The prothrombin time measures abnormalities of the extrinsic pathway (abnormalities in factors I, II, V, VII, and X). Fibrinogen level is not dependent on the intrinsic pathway. Platelets are not affected with hemophilia A.

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.

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 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.

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.

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.

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.

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.

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.

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.

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. 4-year-old child with hemoglobin of 10 g/dl d. 1-year-old child with a hemoglobin of 13 g/dl

c. 4-year-old child with 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 and are related to a reduction in the amount of oxygen available to tissues. 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.

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.

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 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.

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 nurse is caring for a 12-year-old child with b-thalassemia. What clinical manifestations should the nurse expect to observe? (Select all that apply.) a. Anorexia b. Unexplained fever c. Enlarged spleen or liver d. Bronzed, freckled complexion e. Precocious sexual development

ANS: A, B, C, D The clinical manifestations of b-thalassemia include anorexia; unexplained fever; an enlarged spleen or liver; and a bronzed, freckled complexion. There is delayed sexual maturation, not precocious.

The nurse is preparing to administer a unit of packed red blood cells to a hospitalized child. What is an appropriate action that applies to administering blood? a. Take the vital signs every 15 minutes while blood is infusing. b. Use blood within 1 hour of its arrival from the blood bank. c. Administer the blood with 5% glucose in a piggyback setup. d. Administer the first 50 ml of blood slowly and stay with the child.

ANS: D The nurse should administer the first 50 ml of blood or initial 20% of volume (whichever is smaller) slowly and stay with the child. Vitals signs should be taken 15 minutes after initiation and then every hour, not every 15 minutes. Blood should be used within 30 minutes, not 1 hour. Normal saline, not 5% glucose, should be the IV solution.

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.

The clinic nurse is evaluating causes for iron deficiency due to impaired iron absorption. What should the nurse recognize as causes for iron deficiency due to impaired iron absorption? (Select all that apply.) a. Gastric acidity b. Chronic diarrhea c. Lactose intolerance d. Absence of phosphates e. Inflammatory bowel disease

ANS: B, C, E Causes for iron deficiency due to impaired iron absorption include chronic diarrhea, lactose intolerance, and inflammatory bowel disease. Gastric alkalinity, not acidity, and the presence, not absence, of phosphates can be causes of impaired iron absorption.

What therapeutic intervention is most appropriate for a child with b-thalassemia major? a. Oxygen therapy b. Supplemental iron c. Adequate hydration d. Frequent blood transfusions

ANS: D The goal of medical management is to maintain sufficient hemoglobin (>9.5 g/dl) to prevent bone marrow expansion. This is achieved through a long-term transfusion program. Oxygen therapy and adequate hydration are not beneficial in the overall management of thalassemia. The child does not require supplemental iron. Iron overload is a problem because of frequent blood transfusions, decreased production of hemoglobin, and increased absorption from the gastrointestinal tract.