PEDS HEMATOLOGICAL/ONCOLOGY

Which of the following is correct regarding prognostic factors for determining survival for a child newly diagnosed with ALL? 1. The initial white blood cell count on diagnosis. 2. The race of the child. 3. The amount of time needed to initiate treatment. 4. Children aged 12 to 15 years.

1. Children with a normal or low white blood cell count who do not have non-T, non-B acute lymphoblastic leukemia and who are CALLA-positive have a much better prognosis than those with high cell counts or other cell types.

Which of the following laboratory tests will be ordered to determine the presence of the human immunodeficiency virus antigen in an infant whose mother is HIV+ ? 1. CD4 cell count. 2. Western blot. 3. IgG levels. 4. p24 antigen assay.

4. Detection of HIV in infants is confi rmed by a p24 antigen assay, viral culture of HIV, or polymerase chain reaction.

The nurse admits a 1-year-old child to the hospital with the diagnosis of sickle cell crisis. The nurse explains to the parents that which condition leads to local tissue damage during a sickle cell crisis? □ 1. autoimmune reaction complicated by hypoxia □ 2. lack of oxygen in the red blood cells □ 3. obstruction to circulation □ 4. elevated serum bilirubin concentration

3. Characteristic sickle cells tend to cause "log jams" in capillaries. This results in poor circulation to local tissues, leading to ischemia and necrosis. The basic defect in sickle cell disease is an abnormality in the structure of the red blood cells. The erythrocytes are sickle shaped, rough in texture, and rigid. Sickle cell disease is an inherited disease, not an autoimmune reaction. Elevated serum bilirubin concentrations are associated with jaundice, not sickle cell disease.

Where is the primary site of origin of the tumor in children who have neuroblastoma? 1. Bone. 2. Kidney. 3. Abdomen. 4. Liver.

3. Neuroblastoma tumors originate from embryonic neural crest cells that normally give rise to the adrenal medulla and the sympathetic nervous system. The majority of the tumors arise from the adrenal gland or from the retroperitoneal sympathetic chain. Therefore, the primary site is within the abdomen.

The nurse is caring for a child who is receiving extensive radiation as part of the treatment for Hodgkin disease. Which intervention should be implemented? 1. Administer pain medication prior to the child ' s going to radiation therapy. 2. Assess the child for neuropathy since this is a common side effect. 3. Provide adequate rest, because the child may experience excessive malaise and lack of energy. 4. Encourage the child to eat a low-protein diet while on radiation therapy.

3. The most common side effect is extensive malaise, which may be from damage to the thyroid gland, causing hypothyroidism.

A child with leukemia is receiving chemotherapy and is complaining of nausea. The nurse has been giving the scheduled antiemetic. Which of the following should the nurse do when the child is nauseated? 1. Encourage low-protein foods. 2. Encourage low-caloric foods. 3. Offer the child ' s favorite foods. 4. Offer cool, clear liquids.

4. Cool, clear liquids are better tolerated. Milk-based products cause secretions to be thick and can cause vomiting.

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, anti-thymocyte 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.

Which foods should the nurse encourage a parent to offer to a child with iron-deficiency anemia? □ 1. cereal, milk, and yellow vegetables □ 2. potato, peas, and chicken □ 3. macaroni, cheese, and ham □ 4. pudding, green vegetables, and rice

2. Potatoes, peas, chicken, green vegetables, and fortified cereal contain significant amounts of iron and therefore would be recommended. Milk and yellow vegetables are not good iron sources. Rice by itself also is not a good source of iron. Macaroni, cheese, and ham are not high in iron. While pudding (made with fortified milk) and green vegetables contain some iron, the better diet has protein and iron from the chicken and potato.

Which of the following can be a manifestation of leukemia in a child? Select all that apply. 1. Leg pain. 2. Fever. 3. Excessive weight gain. 4. Bruising. 5. Enlarged lymph nodes.

1,2,4,5 1. The proliferation of cells in the bone marrow can cause leg pain. 2. Fever is a result of the neutropenia. 4. A decrease in platelets causes the bruising. 5. The lymph nodes are enlarged by the infiltration of leukemic cells.

A 5-year-old is admitted to the hospital with complaints of leg pain and fever. On physical examination, the child is pale and has bruising over various areas of the body. The health-care provider suspects that the child has ALL. The nurse informs the parent that the diagnosis will be confirmed by which of the following? 1. Lumbar puncture. 2. White blood cell count. 3. Bone marrow aspirate. 4. Bone scan.

3. The diagnostic test that confi rms leukemia is microscopic examination of the bone marrow aspirate.

The most important reason health-care providers prescribe a combination of antiretroviral drugs to children with HIV is to delay: 1. Progression to AIDS. 2. Enable treatment of multiple symptoms. 3. Recurrence of symptoms. 4. Drug resistance.

4. A combination of antiretroviral medications is prescribed for a child who is HIV positive to delay development of drug resistance. HIV drugs work on different stages of the HIV life cycle to prevent reproduction of new virus particles.

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 child's 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 child's 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 statement best describes b-thalassemia major (Cooley anemia)? a. It is an acquired hemolytic anemia. b. Inadequate numbers of red blood cells (RBCs) are present. c. Increased incidence occurs in families of Mediterranean extraction. d. It commonly occurs in individuals from West Africa.

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

What information should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations? a. Give with meals. b. Stop 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.

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

What statement best describes iron deficiency anemia in infants? a. It is caused by depression of the hematopoietic system. b. Diagnosis is easily made because of the infants emaciated appearance. c. It results from a decreased intake of milk and the premature addition of solid foods. d. Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.

ANS: D 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. The bone marrow produces red blood cells that are smaller and contain less hemoglobin than normal red blood cells. Children who have iron deficiency 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.

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.

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

ANS: D The signs of circulatory overload include distended neck veins, hypertension, crackles, a dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema are signs and symptoms of allergic reactions. 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 nurse is conducting staff in-service training on von Willebrand's disease. Which should the nurse include as characteristics of von Willebrand's disease? Select all that apply. 1. Easy bruising occurs. 2. Gum bleeding occurs. 3. It is a hereditary bleeding disorder. 4. Treatment and care are similar to that for hemophilia. 5. It is characterized by extremely high creatinine levels. 6. The disorder causes platelets to adhere to damaged endothelium.

Answer: 1, 2, 3, 4, 6 Rationale: von Willebrand's disease is a hereditary bleeding disorder characterized by a deficiency of or a defect in a protein termed von Willebrand factor. The disorder causes platelets to adhere to damaged endothelium. It is characterized by an increased tendency to bleed from mucous membranes. Assessment findings include epistaxis, gum bleeding, easy bruising, and excessive menstrual bleeding. An elevated creatinine level is not associated with this disorder.

The nurse is instructing the parents of a child with iron deficiency anemia regarding the administration of a liquid oral iron supplement. Which instruction should the nurse tell the parents? 1. Administer the iron at mealtimes. 2. Administer the iron through a straw. 3. Mix the iron with cereal to administer. 4. Add the iron to formula for easy administration.

Answer: 2 Rationale: In iron deficiency anemia, iron stores are depleted, resulting in a decreased supply of iron for the manufacture of hemoglobin in red blood cells. An oral iron supplement should be administered through a straw or medicine dropper placed at the back of the mouth, because the iron stains the teeth. The parents should be instructed to brush or wipe the child's teeth or have the child brush the teeth after administration. Iron is administered between meals because absorption is decreased if there is food in the stomach. Iron requires an acid environment to facilitate its absorption in the duodenum. Iron is not added to formula or mixed with cereal or other food items.

The nurse is providing home care instructions to the parents of a 10-year-old child with hemophilia. Which sport activity should the nurse suggest for this child? 1. Soccer 2. Basketball 3. Swimming 4. Field hockey

Answer: 3 Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Children with hemophilia need to avoid contact sports and to take precautions such as wearing elbow and knee pads and helmets with other sports. The safe activity for them is swimming.

Laboratory studies are performed for a child suspected to have iron deficiency anemia. The nurse reviews the laboratory results, knowing that which result indicates this type of anemia? 1. Elevated hemoglobin level 2. Decreased reticulocyte count 3. Elevated red blood cell count 4. Red blood cells that are microcytic and hypochromic

Answer: 4 Rationale: In iron deficiency anemia, iron stores are depleted, resulting in a decreased supply of iron for the manufacture of hemoglobin in red blood cells. The results of a complete blood cell count in children with iron deficiency anemia show decreased hemoglobin levels and microcytic and hypochromic red blood cells. The red blood cell count is decreased. The reticulocyte count is usually normal or slightly elevated.

The nurse is caring for a child with leukemia. The nurse should be aware that children being treated for leukemia may experience which of the following complications? Select all that apply. 1. Anemia. 2. Infection. 3. Bleeding tendencies. 4. Bone deformities. 5. Polycythemia.

1, 2, 3. 1. Anemia is caused by decreased production of red blood cells. 2. Infection risk in leukemia is secondary to the neutropenia. 3. Bleeding tendencies are from decreased platelet production.

A child with hemophilia A fell and injured a knee while playing outside. The knee is swollen and painful. Which of the following measures should be taken to stop the bleeding? Select all that apply. 1. The extremity should be immobilized. 2. The extremity should be elevated. 3. Warm moist compresses should be applied to decrease pain. 4. Passive range-of-motion exercises should be administered to the extremity. 5. Factor VIII should be administered.

1, 2, 5. 1. Measures are needed to induce vasoconstriction and stop the bleeding, including immobilization of the extremity. 2. Measures are needed to induce vasoconstriction and stop the bleeding. Treatment should include elevating the extremity. 5. Hemophilia A is a defi ciency in factor VIII, which causes delay in clotting when there is a bleed. Giving a dose of Factor VIII concentrate will assist in the clotting process.

The nurse is caring for a child who is receiving a transfusion of PRBCs. The nurse is aware that if the child has a hemolytic reaction to the blood, the signs and symptoms would include which of the following? Select all that apply. 1. Fever. 2. Rash. 3. Oliguria. 4. Hypotension. 5. Chills.

1, 3, 4. 1. Hemolytic reactions include fever, pain at insertion site, hypotension, renal failure, tachycardia, oliguria, and shock. 3. Hemolytic reactions include fever, pain at insertion site, hypotension, renal failure, tachycardia, oliguria, and shock. 4. Hemolytic reactions include fever, pain at insertion site, hypotension, renal failure, tachycardia, oliguria, and shock.

Which of the following factors need(s) to be included in a teaching plan for a child with sickle cell disease? Select all that apply. 1. The child needs to be taken to a physician when sick. 2. The parent should make sure the child sleeps in an air-conditioned room. 3. Emotional stress should be avoided. 4. It is important to keep the child well hydrated. 5. It is important to make sure the child gets adequate nutrition.

1,3,4,5 1. Seek medical attention for illness to prevent the child from going into a crisis. 3. Stress can cause a depressed immune system, making the child more susceptible to infection and crisis. Parents and children are advised to avoid stress. 4. The child needs good hydration and nutrition to maintain good health. 5. The child needs good hydration and nutrition to maintain good health.

Which statement by the parent of a toddler most suggests that the child is at risk for iron-deficiency anemia? □ 1. "He drinks over four glasses of milk per day." □ 2. "He must drink over 10 oz (300 mL) of apple juice per day." □ 3. "He refuses to eat more than two different kinds of vegetables." □ 4. "He does not like meat, but he will eat small amounts of it."

1. Milk is a poor source of iron. Toddlers should have between two and three servings of milk per day. Iron-deficiency anemia can be caused when excessive milk intake of more than 32 oz (1 L)/day intake displaces iron-rich food in the diet. While 6 oz (300 mL) is the recommended daily limit for apple juice, it does contain more iron than milk. Food preferences vary among children. It is acceptable for the child to refuse foods as long as the diet is balanced and contains adequate calories.

A school-age child with leukemia will be taking vincristine. The nurse should encourage the child to eat what kind of diet? □ 1. high-residue □ 2. low-residue □ 3. low-fat □ 4. high-calorie

1. Vincristine may cause constipation, so the client should be encouraged to eat a high-residue (fiber) diet. The other diets do not help with constipation that can occur while receiving vincristine.

Which of the following describes idiopathic thrombocytopenia purpura (ITP)? Select all that apply. 1. ITP is a congenital hematological disorder. 2. ITP causes excessive destruction of platelets. 3. Children with ITP have normal bone marrow. 4. Platelets are small in ITP. 5. Purpura is observed in ITP.

2,3,5 2. ITP is characterized by excessive destruction of platelets. 3. The bone marrow is normal in children with ITP. 5. ITP is characterized by purpura, which are areas of hemorrhage under the skin.

Which of the following should the nurse expect to administer to a child with ITP and a platelet count of 5000/mm 3? Select all that apply. 1. Platelets. 2. Intravenous immunoglobulin. 3. Packed red blood cells (PRBCs). 4. White blood cells. 5. Prednisolone.

2,5 2. Intravenous immunoglobulin is given because the cause of platelet destruction is believed to be an autoimmune response to disease-related antigens. Treatment is usually supportive. Activity is restricted at the onset because of the low platelet count and risk for injury that could cause bleeding. 5. Treatment in the acute phase is often symptomatic, and prednisolone, IVIG, and anti-D antibody are often given. This tends to shorten the course because the disease tends to resolve over time. Focus on the cause of ITP and which cells are affected.

After the nurse teaches the parent of a child newly diagnosed with leukemia about the disease, which description if given by the parent best indicates understanding the nature of leukemia? □ 1. "Leukemia is an infection resulting in increased white blood cell production." □ 2. "Leukemia is a type of cancer characterized by an increase in immature white blood cells." □ 3. "Leukemia is an inflammation associated with enlargement of the lymph nodes." □ 4. "Leukemia is an allergic disorder involving increased circulating antibodies in the blood."

2. Leukemia is a neoplastic, or cancerous, disorder of blood-forming tissues that is characterized by a proliferation of immature white blood cells. Leukemia is not an infection, inflammation, or allergic disorder.

Which test provides a defi nitive diagnosis of aplastic anemia? 1. Complete blood count with differential. 2. Bone marrow aspiration. 3. Serum IgG levels. 4. Basic metabolic panel.

2. Defi nitive diagnosis is determined from bone marrow aspiration, which demonstrates the conversion of red bone marrow to yellow, fatty marrow.

Which of the following is the best method to prevent the spread of infection to an immunosuppressed child? 1. Administer antibiotics prophylactically to the child. 2. Have people wash their hands prior to contact with the child. 3. Assign the same nurses to care for the child each day. 4. Limit visitors to family members only.

2. Hand washing is the best method to prevent the spread of germs and protect the child from infection.

A 10-year-old with severe factor VIII deficiency falls, injures an elbow, and is brought to the ED. The nurse should prepare which of the following? 1. An IM injection of factor VIII. 2. An IV infusion of factor VIII. 3. An injection of desmopressin. 4. An IV infusion of platelets.

2. The child is treated with an IV infusion of factor VIII to replace the missing factor and help stop the bleeding.

What are the clinical manifestations of non-Hodgkin lymphoma? 1. Basically the same as those in Hodgkin disease. 2. Depends on the anatomical site and extent of involvement. 3. Nausea, vomiting, abdominal pain. 4. Behavior changes, jaundice, dry mouth.

2. The clinical manifestations include symptoms of involvement. Rarely is a single sign or symptom diagnostic. Metastasis to the bone marrow or central nervous system may produce manifestations of leukemia.

Which of the following should be done to protect the central nervous system from the invasion of malignant cells in a child newly diagnosed with leukemia? 1. Cranial and spinal radiation. 2. Intravenous steroid therapy. 3. Intrathecal chemotherapy. 4. High-dose intravenous chemotherapy.

3. Giving chemotherapy via lumbar puncture allows the drugs to get to the brain and helps prevent metastasis of the disease.

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

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

Therapeutic management of a 6-year-old child with hereditary spherocytosis (HS) should include which therapeutic intervention? a. Perform a splenectomy. b. Supplement the diet with calcium. c. Institute a maintenance transfusion program. d. Increase intake of iron-rich foods such as meat.

ANS: A Splenectomy corrects the hemolysis that occurs in HS. The splenectomy is generally reserved for children older than age 5 years with symptomatic anemia. Supplementation with calcium does not affect the HS. Additional folic acid can prevent deficiency caused by the rapid cell turnover. A maintenance transfusion program suppresses red blood cell formation. At this time, the risks of transfusion are greater than those of a splenectomy. Iron supplementation does not influence the course of HS.

A school-age child is admitted in vasoocclusive sickle cell crisis (pain episode). The child's care should include which therapeutic interventions? a. Hydration and pain management b. Oxygenation and factor VIII replacement c. Electrolyte replacement and administration of heparin d. Correction of alkalosis and reduction of energy expenditure

ANS: A The management of crises includes adequate hydration, pain management, minimization of energy expenditures, electrolyte replacement, and blood component therapy if indicated. 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. Also, prolonged oxygen can reduce bone marrow activity. Heparin is not indicated in the treatment of vasoocclusive sickle cell crisis. Electrolyte replacement should accompany hydration. The acidosis will be corrected as the crisis is treated. Energy expenditure should be minimized to improve oxygen utilization. Acidosis, not alkalosis, results from hypoxia, which also promotes sickling.

18. 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.

What condition occurs when the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia

ANS: B Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, in which normal adult hemoglobin is replaced by abnormal hemoglobin. Aplastic anemia is a lack of cellular elements being produced. Thalassemia major refers to a variety of inherited disorders characterized by deficiencies in production of certain globulin chains. Iron-deficiency anemia affects red blood cell size and depth of color but does not involve abnormal hemoglobin.

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.

The clinical manifestations of sickle cell anemia (SCA) are primarily the result of which physiologic alteration? a. Decreased blood viscosity b. Deficiency in coagulation c. Increased red blood cell (RBC) destruction d. Greater affinity for oxygen

ANS: C The clinical features of SCA are primarily the result of increased RBC destruction and obstruction caused by the sickle-shaped RBCs. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. SCA does not have a coagulation deficit. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension.

The nurse is reviewing a health care provider's prescriptions for a child with sickle cell anemia who was admitted to the hospital for the treatment of vaso-occlusive crisis. Which prescriptions documented in the child's record should the nurse question? Select all that apply. 1. Restrict fluid intake. 2. Position for comfort. 3. Avoid strain on painful joints. 4. Apply nasal oxygen at 2 L/minute. 5. Provide a high-calorie, high-protein diet. 6. Give meperidine, 25 mg intravenously, every 4 hours for pain.

Answer: 1, 6 Rationale: Sickle cell anemia is one of a group of diseases termed hemoglobinopathies, in which hemoglobin A is partly or completely replaced by abnormal sickle hemoglobin S. It is caused by the inheritance of a gene for a structurally abnormal portion of the hemoglobin chain. Hemoglobin S is sensitive to changes in the oxygen content of the red blood cell; insufficient oxygen causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow. Oral and intravenous fluids are an important part of treatment. Meperidine is not recommended for a child with sickle cell disease because of the risk for normeperidine-induced seizures. 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. The nurse would question the prescription for restricted fluids and meperidine for pain control. Positioning for comfort, avoiding strain on painful joints, oxygen, and a high-calorie and high-protein diet are also important parts of the treatment plan.

The nursing student is presenting a clinical conference and discusses the cause of β-thalassemia. The nursing student informs the group that a child at greatest risk of developing this disorder is which of these? 1. A child of Mexican descent 2. A child of Mediterranean descent 3. A child whose intake of iron is extremely poor 4. A breast-fed child of a mother with chronic anemia

Answer: 2 Rationale: β-Thalassemia is an autosomal recessive disorder characterized by the reduced production of 1 of the globin chains in the synthesis of hemoglobin (both parents must be carriers to produce a child with β-thalassemia major). This disorder is found primarily in individuals of Mediterranean descent.

A 10-year-old child with hemophilia A has slipped on the ice and bumped his knee. The nurse should prepare to administer which prescription? 1. Injection of factor X 2. Intravenous infusion of iron 3. Intravenous infusion of factor VIII 4. Intramuscular injection of iron using the Z-track method

Answer: 3 Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. The primary treatment is replacement of the missing clotting factor; additional medications, such as agents to relieve pain, may be prescribed depending on the source of bleeding from the disorder. A child with hemophilia A is at risk for joint bleeding after a fall. Factor VIII would be prescribed intravenously to replace the missing clotting factor and minimize the bleeding. Factor X and iron are not used to treat children with hemophilia A.

The nurse analyzes the laboratory results of a child with hemophilia. The nurse understands that which result will most likely be abnormal in this child? 1. Platelet count 2. Hematocrit level 3. Hemoglobin level 4. Partial thromboplastin time

Answer: 4 Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Results of tests that measure platelet function are normal; results of tests that measure clotting factor function may be abnormal. Abnormal laboratory results in hemophilia indicate a prolonged partial thromboplastin time. The platelet count, hemoglobin level, and hematocrit level are normal in hemophilia.

The clinic nurse instructs parents of a child with sickle cell anemia about the precipitating factors related to sickle cell crisis. Which, if identified by the parents as a precipitating factor, indicates the need for further instruction? 1. Stress 2. Trauma 3. Infection 4. Fluid overload

Answer: 4 Rationale: Sickle cell crises are acute exacerbations of the disease, which vary considerably in severity and frequency; these include vaso-occlusive crisis, splenic sequestration, hyperhemolytic crisis, and aplastic crisis. Sickle cell crisis may be precipitated by infection, dehydration, hypoxia, trauma, or physical or emotional stress. The mother of a child with sickle cell disease should encourage fluid intake of 1.5 to 2 times the daily requirement to prevent dehydration.

A child with β-thalassemia is receiving long-term blood transfusion therapy for the treatment of the disorder. Chelation therapy is prescribed as a result of too much iron from the transfusions. Which medication should the nurse anticipate being prescribed? 1. Fragmin 2. Meropenem 3. Metoprolol 4. Deferoxamine

Answer: 4 Rationale: β-Thalassemia is an autosomal recessive disorder characterized by the reduced production of 1 of the globin chains in the synthesis of hemoglobin (both parents must be carriers to produce a child with β-thalassemia major). The major complication of long-term transfusion therapy is hemosiderosis. To prevent organ damage from too much iron, chelation therapy with either deferasirox or deferoxamine may be prescribed. Deferoxamine is classified as an antidote for acute iron toxicity. Dalteparin is an anticoagulant used as prophylaxis for postoperative deep vein thrombosis. Meropenem is an antibiotic. Metoprolol is a beta blocker used to treat hypertension.

The nurse is caring for a child with sickle cell disease who is scheduled to have a splenectomy. What information should the nurse explain to the parents regarding the reason for a splenectomy? 1. To decrease potential for infection. 2. To prevent splenic sequestration. 3. To prevent sickling of red blood cells. 4. To prevent sickle cell crisis.

2. Splenic sequestration is a life-threatening situation in children with sickle cell disease. Once a child is considered to be at high risk of splenic sequestration or has had this in the past, the spleen will be removed.

Which intervention should be implemented after a bone marrow aspiration? 1. Ask the child to remain in a supine position. 2. Place the child in an upright position for 4 hours. 3. Keep the child nothing by mouth for 6 hours. 4. Administer analgesics as needed for pain.

4. Children may experience minor discomfort after the procedure, and analgesics should be given as needed.

A nurse is doing discharge education with a parent who has a child with betathalassemia (Cooley anemia). The nurse informs the parent that the child is at risk for which of the following conditions? 1. Hypertrophy of the thyroid. 2. Polycythemia vera. 3. Thrombocytopenia. 4. Chronic hypoxia and iron overload.

4. In beta-thalassemia, there is increased destruction of red blood cells, causing anemia. This results in chronic anemia and hypoxia. The children are treated with multiple blood transfusions, which can cause iron overload and damage to major organs.

Which of the following activities should a nurse suggest for a client diagnosed with hemophilia? Select all that apply. 1. Swimming. 2. Golf. 3. Hiking. 4. Fishing. 5. Soccer.

1, 2, 3, 4. 1. Children with hemophilia should be encouraged to take part in noncontact activities that allow for social, psychological, and physical growth, such as swimming. 2. Children with hemophilia should be encouraged to take part in noncontact activities that allow for social, psychological, and physical growth, such as golf. 3. Children with hemophilia should be encouraged to take part in noncontact activities that allow for social, psychological, and physical growth, such as hiking. 4. Children with hemophilia should be encouraged to take part in noncontact activities that allow for social, psychological, and physical growth, such as fi shing.

Which of the following measures should the nurse implement to help with the nausea and vomiting caused by chemotherapy? Select all that apply. 1. Give an antiemetic 30 minutes prior to the start of therapy. 2. Continue the antiemetic as ordered until 24 hours after the chemotherapy is complete. 3. Remove food that has a lot of odor. 4. Keep the child on a nothing-by-mouth status. 5. Wait until the nausea begins to start the antiemetic.

1, 2, 3. 1. The first dose should be given 30 minutes prior to the start of the therapy. 2. Antiemetic should be administered around the clock until 24 hours after the chemotherapy is completed. 3. It is also helpful to remove foods with odor so that the smell of the food does not make the child nauseated.

A nurse is caring for a child with von Willebrand disease. The nurse is aware that which of the following is a clinical manifestation of von Willebrand disease? Select all that apply. 1. The child bruises easily. 2. Excessive menstruation. 3. The child has frequent nosebleeds. 4. Elevated creatinine levels. 5. Elevated blood pressure.

1, 2, 3. 1. Von Willebrand disease is a hereditary bleeding disorder characterized by deficiency of or defect in a protein. The disorder causes adherence of platelets to damaged endothelium and a mild deficiency of factor VIII. One of the manifestations of this disease is bleeding of the mucous membranes. 2. Excessive menstruation may be a manifestation of this disease. 3. Frequent nosebleeds are a common manifestation of this disease.

Which of the following can lead to a possible diagnosis of human immunodeficiency virus (HIV) in a child? Select all that apply. 1. Repeated respiratory infections. 2. Intermittent diarrhea. 3. Excessive weight gain. 4. Irregular heartbeat. 5. Poor weight gain.

1, 2, 5. 1. Symptoms of HIV include frequent respiratory infections. The symptoms present based on the underlying cellular immunodeficiency-related disease. 2. Symptoms of HIV include intermittent diarrhea. The symptoms present based on the underlying cellular immunodeficiency-related disease. 5. Symptoms of HIV include poor weight gain.

A nurse is caring for a 15-year-old who has just been diagnosed with non-Hodgkin lymphoma. Which of the following should the nurse include in teaching the parents about this lymphoma? Select all that apply. 1. The malignancy originates in the lymphoid system. 2. The presence of Reed-Sternberg cells in the biopsy is considered diagnostic. 3. Mediastinal involvement is typical. 4. The disease is diffuse rather than nodular. 5. Treatment includes chemotherapy and radiation.

1, 3, 4, 5. 1. Non-Hodgkin disease originates in the lymphoid system. 3. Mediastinal involvement is typical. 4. The disease is diffuse rather than nodular. 5. Treatment includes chemotherapy and radiation.

Which of the following best describes the action of chemotherapeutic agents used in the treatment of cancer in children? Select all that apply. 1. Suppress the function of normal lymphocytes in the immune system. 2. Are alkylating agents and are cell-specifi c. 3. Cause a replication of DNA and are cell-specifi c. 4. Interrupt cell cycle, thereby causing cell death. 5. Prednisone is a natural hormone.

1, 4, 5. 1. All chemotherapy is immunosuppressive, because most childhood cancers affect the immune system. 4. Mitotic inhibitors, such as vincristine (Oncovin), stop cell division but can also damage cells in all phases of the cell cycle. 5. The corticosteroids are natural hormones that can be used to prevent nausea and allergic reactions. They are given with other chemotherapeutic agents.

School-age children with cancer often have a body image disturbance related to hair loss, moon face, or debilitation. Which of the following interventions is (are) most appropriate? Select all that apply. 1. Encourage them to wear a wig similar to their own hairstyle. 2. Emphasize the benefi ts of the therapy they are receiving. 3. Have them play only with other children with cancer. 4. Use diversional techniques to avoid discussing changes in the body because of the chemotherapy. 5. Help them find a "special friend" who understands what they are experiencing.

1, 5. 1. Wearing a wig is a good way for the child to keep personal identity despite the loss of hair. 5. Children with cancer and body-image changes oftentimes need assistance in expressing themselves, which a "special friend" can help with.

A child diagnosed with leukemia is receiving allopurinol (Zyloprim) as part of the treatment plan. The parents ask why their child is receiving this medication. What information about the medication should the nurse provide? 1. Helps reduce the uric acid level caused by cell destruction. 2. Helps make the chemotherapy more effective. 3. Helps reduce the nausea and vomiting associated with chemotherapy. 4. Helps decrease pain in the bone marrow.

1. Allopurinol (Zyloprim) reduces serum uric acid. When there is lysis of cells from chemotherapy, there will be an increase in serum uric acid.

Which of the following measures should be implemented for a child with von Willebrand disease who has a nosebleed? 1. Apply pressure to the nose for at least 10 minutes. 2. Have the child lie supine and quiet. 3. Avoid packing of the nostrils. 4. Encourage the child to swallow frequently.

1. Applying pressure to the nose may stop the bleeding. In von Willebrand disease, there is an increased tendency to bleed from mucous membranes, leading to nosebleeds commonly from the anterior part of the nasal septum.

The nurse is caring for a child with a diagnosis of ALL who is receiving chemotherapy. The nurse notes that the child's platelet count is 20,000/mm 3 . Based on this laboratory finding, what information should the nurse provide to the child and parents? 1. A soft toothbrush should be used for mouth care. 2. Isolation precautions should be started immediately. 3. The child's vital signs, including blood pressure, should be monitored every 4 hours. 4. All visitors should be discouraged from coming to see the family.

1. Because the platelet count is decreased, there is a signifi cant risk of bleeding, especially in soft tissue. The use of the soft toothbrush should help prevent bleeding of the gums.

The nurse is caring for a child with sickle cell disease who is scheduled to have an exchange transfusion. What information should the nurse teach the family? 1. The procedure is done to prevent further sickling during a vaso-occlusive crisis. 2. The procedure reduces side effects from blood transfusions. 3. The procedure is a routine treatment for sickle cell crisis. 4. Once the child's spleen is removed, it is not necessary to do exchange transfusions.

1. Exchange transfusion reduces the number of circulating sickle cells and slows down the cycle of hypoxia, thrombosis, and tissue ischemia.

The parent of a teen with a diagnosis of Hodgkin disease asks what the child's prognosis will be with treatment. What information should the nurse give to the parent and child? 1. Clinical staging of Hodgkin disease will determine the treatment; long-term survival for all stages of Hodgkin disease is excellent. 2. There is a considerably better prognosis if the client is diagnosed early and is less than 5 years of age. 3. The prognosis for Hodgkin disease depends on the type of chemotherapy. 4. The only way to obtain a good prognosis is by chemotherapy and bone marrow transplant.

1. Long-term survival for all stages of Hodgkin disease is excellent. Early-stage disease can have a survival rate greater than 90%, with advanced stages having rates between 65% and 75%.

The parent of a 4-year-old brings the child to the clinic and tells the nurse the child's abdomen is distended. After a complete examination, a diagnosis of Wilms tumor is suspected. Which of the following is most important when doing a physical examination on this child? 1. Avoid palpation of the abdomen. 2. Assess the urine for the presence of blood. 3. Monitor vital signs, especially the blood pressure. 4. Obtain an accurate height and weight.

1. Palpating the abdomen of the child in whom a diagnosis of Wilms tumor is suspected should be avoided because manipulation of the abdomen may cause seeding of the tumor.

During a health history the nurse learns that a pediatric client seldom eats foods high in iron. Which physical assessment findings would suggest that the child has developed iron-deficiency anemia? Select all that apply. □ 1. decreased heart rate □ 2. pale skin □ 3. swollen tongue □ 4. systolic murmur □ 5. yellowed sclera

2, 3, 4. Pale skin is one of the most common physical findings associated with iron-deficiency anemia. Lower levels of myoglobin lead to soreness and swelling of the tongue. Low levels of hemoglobin force the heart to work harder to pump blood. Tachycardia and systolic murmurs may result. Anemia presents as an elevated heart rate not decreased. Yellowed sclera is consistent with hemolytic anemia.

Which of the following is a reason to perform a lumbar puncture on a child with a diagnosis of leukemia? Select all that apply. 1. Rule out meningitis. 2. Assess the central nervous system for infi ltration. 3. Give intrathecal chemotherapy. 4. Determine increased intracranial pressure. 5. Stage the leukemia.

2,3 2. A lumbar puncture is done to determine whether the cancer cells have entered the CNS, but this would not be routine unless the child was symptomatic. 3. Chemotherapy can also be given through a lumbar puncture (spinal tap).

A transfusion of packed red blood cells has been prescribed for a 1-year-old with sickle cell anemia. The infant has a 25-gauge IV infusing dextrose with sodium and potassium. Using the situation, background, assessment, and recommendation (SBAR) method of communication, the nurse contacts the health care provider (HCP) and makes which recommendation? □ 1. starting a second IV with a 22-gauge catheter to infuse normal saline with the blood □ 2. using the existing IV, but changing the fluids to normal saline for the transfusion □ 3. replacing the IV with a 22-gauge catheter to infuse the prescribed fluids □ 4. starting a second IV with a 25-gauge catheter to infuse normal saline with the transfusion

2. The best evidence indicates that a catheter as small as 27 gauge may safely be used for transfusion in children, but blood must be infused with normal saline, not dextrose. A 1-year-old should be able to maintain his or her blood glucose for the 2-hour duration of the infusion without the need for a second IV.

At a wellness check, the nurse monitors the routine laboratory values of an asymptomatic school-age client with sickle cell anemia. The reports reveal that the child has a hemoglobin of 10 g/100 mL (100 g/1 L). The nurse plans the client's care based on which interpretation of the hemoglobin level? □ 1. The child will most likely need a blood transfusion for the low hemoglobin. □ 2. This hemoglobin level is a typical finding in children with this disease. □ 3. The folic acid dose may need to be increased to improve hemoglobin production. □ 4. Additional tests are needed to determine if a sequestration crisis is causing the low hemoglobin.

2. Between crises, hemoglobin levels between 6 and 9 g/100 mL (60 to 90/L) are typical for children with sickle cell anemia. The decision to transfuse a child must be weighed against the risks. Transfusions are most often considered to treat life-threatening sickle cell complications, to keep HbS levels within a desired range, or as prophylaxis before surgery. Oral folic acid is frequently prescribed to rebuild hemolyzed RBCs. It would be appropriate for the nurse to verify that the client was taking folic acid as prescribed before making any further interpretations. Clients with sequestration crisis present with symptoms including pain and signs and symptoms of hypovolemia.

The parent of a child with hemophilia is asking the nurse what caused the hemophilia. Which is the nurse ' s best response? 1. It is an X-linked dominant disorder. 2. It is an X-linked recessive disorder. 3. It is an autosomal dominant disorder. 4. It is an autosomal recessive disorder.

2. Hemophilia is transmitted as an X-linked recessive disorder. About 60% of children have a family history of hemophilia. The usual transmission is by a female with the trait and an unaffected male.

The nurse is teaching the parents of a child with sickle cell disease. What information should the nurse give the family on how to prevent sickle cell crisis? □ 1. Exercise in cool temperatures. □ 2. Drink at least 2 quarts of fluids per day. □ 3. Avoid contact sports. □ 4. Take anti-inflammatory medications before exercising.

2. Increasing fluid intake and being well hydrated will help prevent cell stasis in the small vessels. Restricting fluids causes stasis of red blood cells and promotes obstruction and increases the chance of sickling with hypoxia and pain to the part that is involved. Clients with sickle cell disease should avoid exercising in cool temperatures or swimming in cold water. While contact sports are not recommended because of bleeding risks, they do not cause sickle crisis. Taking an anti-inflammatory medication before exercising does not prevent sickle cell crisis.

The parent of a 2-year-old who is HIV + questions the nurse about placing the child in day care. Which of the following is the best response? 1. The child should not go to day care until older, because there is a high risk for transmission of the disease. 2. The child can go to day care without restrictions and should be allowed to participate in all activities. 3. The child can go to day care but should avoid physical activity. 4. The child may go to day care, but the parent must inform all the parents at the day care that the child is HIV + .

2. The child can attend day care without any limitations but should not attend with a fever.

A child has completed treatment for leukemia and comes to the clinic for a checkup with the parents. The parents express to the nurse that they are glad their child has been cured of cancer and is safe from getting cancer later in life. Which of the following should the nurse consider in responding? 1. Childhood cancer usually instills immunity to all other cancers. 2. Children surviving one cancer are at higher risk for a second cancer. 3. The child may have a remission of the leukemia but is immune to all other cancers. 4. As long as the child continues to take steroids, there will be no other cancers.

2. The most devastating late effect of leukemia treatment is development of secondary malignancy.

An 18-month-old male is brought to the clinic by his mother. His height is in the 50th percentile, and his weight is in the 80th percentile. The child is pale. The physical examination is normal, but his hematocrit level is 20%. Which of the following questions should assist the nurse in making a diagnosis? Select all that apply. 1. "How many bowel movements a day does your child have?" 2. "How much did your baby weigh at birth?" 3. "What does your child eat every day?" 4. "Has the child been given any new medications?" 5. "How much milk does your child drink per day?"

3, 5 3. A diet history is necessary to determine the nutritional status of the child and whether the child is getting sufficient sources of iron. 5. By asking how much milk the child consumes, the nurse can determine whether the child is filling up on milk and then not wanting to take food.

Which of the following is the most effective treatment for pain in a child with sickle cell crisis? Select all that apply. 1. Meperidine (Demerol). 2. Aspirin. 3. Morphine. 4. Behavioral techniques. 5. Acetaminophen (Tylenol) with codeine.

3,4,5 3. Morphine is the drug of choice for a child with sickle cell crises. Usually the child is started on oral doses of acetaminophen (Tylenol) with codeine. When that is not suffi cient to alleviate pain, stronger narcotics are prescribed, such as morphine. Ketorolac (Toradol) may be indicated for short-term use for moderate-severe pain. 4. Behavioral techniques such as positive self-talk, relaxation, distraction, and guided imagery are helpful when pain is occurring. 5. Usually the child is started on oral doses of acetaminophen (Tylenol) with codeine when pain is described as mild to moderate.

A nurse is caring for a 5-year-old with sickle cell vaso-occlusive crisis. Which of the following orders should the nurse question? Select all that apply. 1. Position the child for comfort. 2. Apply hot packs to painful areas. 3. Give meperidine (Demerol) 25 mg intravenously every 4 hours as needed for pain. 4. Restrict oral fluids. 5. Apply oxygen per nasal cannula to keep oxygen saturations above 94%.

3,4,5 3. Tissue hypoxia is very painful. Narcotics such as morphine are usually given for pain when the child is in a crisis. Meperidine (Demerol) should be avoided because of the risk of Demerol-induced seizures. 4. The child should receive hydration because when the child is in crisis, the abnormal S-shaped red blood cells clump, causing tissue hypoxia and pain. 5. Oxygen is of little value unless the tissue is hypoxic. The objective of treatment is to minimize hypoxia.

The nurse is taking care of a child with sickle cell disease. The nurse is aware that which of the following problems is (are) associated with sickle cell disease? Select all that apply. 1. Polycythemia. 2. Hemarthrosis. 3. Aplastic crisis. 4. Thrombocytopenia. 5. Vaso-occlusive crisis.

3,5 3. Aplastic crisis, temporary cessation of red blood cell production, is associated with sickle cell anemia. 5. Vaso-occlusive crisis is the most common problem in children with sickle cell disease.

The parent of a child diagnosed with Wilms tumor asks the nurse what the treatment plan will be. The nurse explains the usual protocol for this condition. Which information should the nurse give to the parent? 1. The child will have chemotherapy and, after that has been completed, radiation. 2. The child will need to have surgery to remove the tumor. 3. The child will go to surgery for removal of the tumor and the kidney and will then start chemotherapy. 4. The child will need radiation and later surgery to remove the tumor.

3. Combination therapy of surgery and chemotherapy is the primary therapeutic management. Radiation is done depending on clinical stage and histological pattern.

Which of the following will be abnormal in a child with the diagnosis of hemophilia? 1. Platelet count. 2. Hemoglobin level. 3. White blood cell count. 4. Partial thromboplastin time (PTT).

4. The abnormal laboratory results in hemophilia are related to decreased clotting function. Partial thromboplastin time is prolonged.

The nurse is discharging a child who has just received chemotherapy for neuroblastoma. Which of the following statements made by the child ' s parent indicates a need for additional teaching? 1. "I will inspect the skin often for any lesions." 2. "I will do mouth care daily and monitor for any mouth sores." 3. "I will wash my hands before caring for my child." 4. "I will take a rectal temperature daily and report a temperature greater than 101°F (38.3°C) immediately to the health-care provider."

4. Monitoring the child ' s temperature and reporting it to the physician are important, but the temperature should not be taken rectally. The risk of injury to the mucous membranes is high. Rectal abscesses can occur in the damaged rectal tissue. The best method for taking the temperature is axillary, especially if the child has mouth sores.

Which of the following is the most common opportunistic infection in children infected with human immunodeficiency virus (HIV)? 1. CMV. 2. Encephalitis. 3. Meningitis. 4. Pneumocystic pneumonia.

4. Pneumocystis jiroveci pneumonia is the most common opportunistic infection that can occur in HIV-infected children, and such children are treated prophylactically for this.

Prednisone is given to children who are being treated for leukemia. Why is this medication given as part of the treatment plan? 1. Enhances protein metabolism. 2. Enhances sodium excretion. 3. Increases absorption of the chemotherapy. 4. Destroys abnormal lymphocytes.

4. Prednisone is used in many of the treatment protocols for leukemia because there is abnormal lymphocyte production. Prednisone is thought to destroy abnormal lymphocytes.

The nurse expects which of the following clinical manifestations in a child diagnosed with SCID? 1. Prolonged bleeding. 2. Failure to thrive. 3. Fatigue and malaise. 4. Susceptibility to infection.

4. SCID is characterized by an absence of cell-mediated immunity, with the most common clinical manifestation being infection in children from age 3 months. These children do not usually recover from these infections.

What explanation provides the rationale for why iron-deficiency anemia is common during infancy? a. Cow's milk is a poor source of iron. b. Iron cannot be stored during fetal development. c. Fetal iron stores are depleted by 1 month of age. d. Dietary iron cannot be started until 12 months of age.

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

A nurse instructs the parent of a child with sickle cell disease about factors that might precipitate a pain crisis in the child. Which of the following factors identified by the parent as being able to cause a pain crisis indicates a need for further instruction? 1. Infection. 2. Overhydration. 3. Stress at school. 4. Cold environment.

2. Overhydration does not cause a crisis.

Which action indicates that the parents of a 12-month-old with iron-deficiency anemia understand how to administer iron supplements? Select all that apply. □ 1. administering iron supplements in combination with fruit juice □ 2. scheduling iron supplements with meals □ 3. verbalizing the need to report dark stools □ 4. brushing the child's teeth after administering the iron supplements □ 5. decreasing the dietary intake of foods fortified with iron

1, 4. Parent teaching concerning a child with iron-deficiency anemia should include directions about giving iron combined with fruit juice, in divided doses, between meals, and with a dropper for a 12-month-old or through a straw for older toddlers. Iron stains teeth, so brushing the teeth and administering liquid iron through a dropper or straw are necessary to prevent staining the teeth. Iron should not be given with milk, antacids, or tea and should be administered on an empty stomach. Iron will cause the stool to become black or green, which is normal and does not need to be reported. However, light-colored stools indicate the iron is not being absorbed and should be reported.

The nurse and parents plan for the discharge of a child with leukemia who is receiving dactinomycin and vincristine. Which intervention should the nurse include in the teaching plan? □ 1. Encourage increased fluid intake. □ 2. Keep the child out of the sun. □ 3. Monitor the child's heart rate. □ 4. Observe the child for memory loss.

1. Dactinomycin and vincristine both cause nausea and vomiting. Oral fluids are encouraged, and antiemetics are given to prevent dehydration. Avoiding sun exposure is not necessary because photosensitivity is not associated with these drugs. Heart rate changes and memory issues also are not associated with either of these two drugs.

Which of the following confi rms a diagnosis of Hodgkin disease in a 15-year-old? 1. Reed-Sternberg cells in the lymph nodes. 2. Blast cells in the blood. 3. Lymphocytes in the bone marrow. 4. VMA in the urine.

1. A lymph node biopsy is done to confi rm a histological diagnosis and staging of Hodgkin disease. The presence of Reed- Sternberg cells is characteristic of the disease.

A nurse educator is providing a teaching session for the nursing staff. Which of the following individuals is at greatest risk for developing beta-thalassemia (Cooley anemia)? 1. A child of Mediterranean descent. 2. A child of Mexican descent. 3. A child whose mother has chronic anemia. 4. A child who has a low intake of iron.

1. Beta-thalassemia is an inherited recessive disorder that is found primarily in individuals of Mediterranean descent. The disease has also been reported in Asian and African populations.

The nurse is caring for a child being treated for ALL. Laboratory results indicate that the child has a white blood cell count of 5000/mm 3 with 5% polys and 3% bands. Which of the following analyses is most appropriate? 1. The absolute neutrophil count is 400/mm 3 , and the child is neutropenic. 2. The absolute neutrophil count is 800/mm 3 , and the child is neutropenic. 3. The absolute neutrophil count is 4000/mm 3 , and the child is not neutropenic. 4. The absolute neutrophil count is 5800/mm 3 , and the child is not neutropenic.

1. The calculated absolute neutrophil count is 400/mm 3 (0.08 × 5000), and the child is neutropenic because the count is less than 500/mm 3 .

Which signs and symptoms of leukemia would lead the nurse to suspect the client has thrombocytopenia? Select all that apply. □ 1. fever □ 2. petechiae □ 3. epistaxis □ 4. anorexia □ 5. bone pain □ 6. dyspnea

2, 3. Children with acute lymphocytic leukemia have a reduced platelet count (thrombocytopenia), reduced red blood cell count (anemia), and reduced white blood cell count (neutropenia) because of unrestricted proliferation of immature white blood cell. Chemotherapy is used to treat leukemia and contributes to thrombocytopenia, neutropenia, and anemia. Clients with thrombocytopenia are at risk for bleeding. Petechiae (small red or purple spots on the skin) and epistaxis (nose bleeds) are both signs of bleeding. A fever is a result of a decreased white blood cell count. Anorexia and dyspnea (shortness of breath) are a result of a decreased red blood cell count. Bone pain is a result of stress on the bone related to the unrestricted proliferation of the leukemic blast cells.

The nurse receives a call from a parent of a child with leukemia in remission. The parent says the child has been exposed to chickenpox and has never had it. Which of the following responses is most appropriate for the nurse? Select all that apply. 1. "You need to monitor the child's temperature frequently and call back if the temperature is greater than 101°F (38.3°C)." 2. "The child has had two varicella immunizations as an infant but is no longer immune after chemotherapy." 3. "You need to bring the child to the clinic for a varicella immunoglobulin vaccine." 4. "Your child will need to be isolated for the next 2 weeks." 5. "Your child may develop chicken pox lesions about 14 to 21 days after exposure."

2,3 2. Chickenpox exposure is a real concern for a child who is immunocompromised, and action needs to be taken. 3. The child should receive varicella zoster immune globulin within 96 hours of the exposure.

The nurse is caring for a child diagnosed with thalassemia major who is receiving the first chelation therapy. What information should the nurse provide to the parent regarding the therapy? Select all that apply. 1. Decreases the risk of bleeding. 2. Eliminates excess iron. 3. Prevents further sickling of the red blood cells. 4. Provides an iron supplement. 5. Hydration is necessary for the process to be effective.

2,5 2. Chelation therapy is used to rid the body of excess iron stores that result from frequent blood transfusions. 5. Hydration is necessary for the process to be effective.

A diagnosis of hemophilia A is confirmed in an infant. Which instruction should the nurse provide the parents as the infant becomes more mobile and starts to crawl? □ 1. Administer one-half of a children's aspirin for a temperature higher than 101°F (38.3°C). □ 2. Sew thick padding into the elbows and knees of the child's clothing. □ 3. Check the color of the child's urine every day. □ 4. Expect the eruption of the primary teeth to produce moderate to severe bleeding.

2. As the hemophilic infant begins to acquire motor skills, falls and bumps increase that risk of bleeding. Such injuries can be minimized by padding vulnerable joints. Aspirin is contraindicated because of its antiplatelet properties, which increase the infant's risk for bleeding. Because genitourinary bleeding is not a typical problem in children with hemophilia, urine testing is not indicated. Although some bleeding may occur with tooth eruption, it does not normally cause moderate to severe bleeding episodes in children with hemophilia.

A school-age child with leukemia is taking immunosuppressive drugs. What health maintenance recommendation should the nurse include in the teaching plan? □ 1. Monitor the child's temperature at school. □ 2. Avoid any live attenuated vaccines. □ 3. Take daily vitamin and mineral supplements. □ 4. Stay away from other children.

2. Children who are immunosuppressed should not receive any live attenuated vaccines. Clients who are immunosuppressed and are given live attenuated vaccines such as measles, mumps, rubella, and oral polio vaccine can develop severe forms of the diseases for which they are being immunized, which can result in death. Inactivated vaccines may be given if necessary, but the client is not able to adequately produce needed antibodies, and it is recommended that immunizations be delayed for 3 months after the immunosuppressive drugs have been discontinued. It is unnecessary to monitor the child's temperature at school unless the child shows symptoms of an illness. Vitamin and mineral supplements are not normally given in conjunction with immunosuppressive drugs. When the client is immunosuppressed, the client should avoid only persons who have an infection.

An adolescent client is admitted to the hospital with the diagnosis of acute lymphocytic leukemia. Which signs and symptoms require the most urgent nursing intervention? □ 1. fatigue and anorexia □ 2. fever and petechiae □ 3. swollen neck lymph glands and lethargy □ 4. enlarged liver and spleen

2. Fever and petechiae associated with acute lymphocytic leukemia indicate a suppression of normal white blood cells and thrombocytes by the bone marrow and put the client at risk for other infections and bleeding. The nurse should initiate infection control and safety precautions to reduce these risks. Fatigue is a common symptom of leukemia due to red blood cell suppression. Although the client should be told about the need for rest and meal planning, such teaching is not the priority intervention. Swollen glands and lethargy may be uncomfortable, but they do not require immediate intervention. An enlarged liver and spleen do require safety precautions that prevent injury to the abdomen; however, these precautions are not the priority.

After doing well for a period of time, a child with leukemia develops an overwhelming infection. The child's death is imminent. Which statement offers the nurse the best guide in making plans to assist the parents in dealing with their child's imminent death? □ 1. Knowing that the prognosis is poor helps prepare parents for the death of children. □ 2. Parents are especially grieved when a child does well at first but then declines rapidly. □ 3. Parents' trust in health care personnel is most often destroyed by a death that is considered untimely. □ 4. It is more difficult for parents to accept the death of an older child than that of a toddler.

2. It has been found that parents are more grieved when optimism is followed by defeat. The nurse should recognize this when planning various ways to help the parents of a dying child. It is not necessarily true that knowing about a poor prognosis for years helps prepare parents for a child's death. Death is still a shock when it occurs. Trust in health care personnel is not necessarily destroyed when a death is untimely if the family views the personnel as having done all that was possible. It is not more difficult for parents to accept the death of an older child than that of a younger child.

The mother tells the nurse she will be afraid to allow her child with hemophilia to participate in sports because of the danger of injury and bleeding. After explaining that physical fitness is important for children with hemophilia, which activity should the nurse suggest as ideal? □ 1. snow skiing □ 2. swimming □ 3. basketball □ 4. gymnastics

2. Swimming is an ideal activity for a child with hemophilia because it is a noncontact sport. Many noncontact sports and physical activities that do not place excessive strain on joints are also appropriate. Such activities strengthen the muscles surrounding joints and help control bleeding in these areas. Noncontact sports also enhance general mental and physical well-being. Falls and subsequent injury to the child may occur with snow skiing. Basketball is a contact sport and therefore increases the child's risk for injury. Gymnastics is a very strenuous sport. Gymnasts frequently have muscle and joint injuries that result in bleeding episodes.

A child with hemophilia presents with a burning sensation in the knee and reluctance to move the body part. The nurse collaborates with the care team to provide factor replacement and implements which intervention? □ 1. administers an aspirin-containing compound □ 2. institutes rest, ice, compression, and elevation □ 3. begins physical therapy with active range of motion □ 4. initiates skin traction immobilization

2. The child is displaying symptoms of bleeding in the joint, and factor replacement is indicated. The RICE method is used as a supportive measure to help control the bleeding. Aspirin-containing compounds contribute to bleeding and should never be used to control pain. Physical therapy is instituted after acute bleeding to prevent further damage. Orthopedic traction is considered in some rare cases during the rehabilitation phase, but not the acute phase.

The nurse is caring for a 10-year-old with leukemia who is receiving chemotherapy. The child is on neutropenic precautions. Friends of the child come to the desk and ask for a vase for the flowers they have brought with them. Which of the following is the best response? 1. "I will get you a special vase that we use on this unit." 2. "The flowers from your garden are beautiful but should not be placed in the room at this time." 3. "As soon as I can wash a vase, I will put the flowers in it and bring it to the room." 4. "Get rid of the flowers immediately. You could harm the child."

2. A neutropenic client should not have fl owers in the room because the fl owers may harbor Aspergillus or Pseudomonas aeruginosa. Neutropenic children are susceptible to infection. Precautions need to be taken so that the child does not come in contact with any potential sources of infection. Fresh fruits and vegetables can also harbor molds and should be avoided. Telling the friend that the fl owers are beautiful but that the child cannot have them is a tactful way not to offend the friend.

When caring for a child with lymphoma, the nurse needs to be aware of which of the following? 1. The same staging system is used for lymphoma and Hodgkin disease. 2. Aggressive chemotherapy with central nervous system prophylaxis will give the child a good prognosis. 3. All children with lymphoma need a bone marrow transplant for a good prognosis. 4. Despite high-dose chemotherapy, the prognosis is very poor for most children.

2. The use of aggressive combination chemotherapy has a major impact on the survival rates for children with a diagnosis of lymphoma. Because there is usually bone marrow involvement, there is a need for central nervous system prophylaxis.

A school-age client with hemophilia A has fallen and badly bruised his knee. Which action should the nurse do first to manage the client's hemarthrosis? □ 1. Use active range of motion to prevent immobility. □ 2. Apply cold packs to promote vasoconstriction. □ 3. Apply pressure and immobilize the joint. □ 4. Notify the health care provider (HCP) of the injury.

3. Application of pressure and immobilization of the affected limb are the first priority. Pressure is required to stop the bleeding, and immobilization aids in reducing swelling and pain. Active range of motion is recommended after the bleeding is controlled. The application of cold packs can be helpful in diminishing swelling and pain. Cold packs will also promote vasoconstriction, which can help reduce the bleeding. The health care provider (HCP) should be informed of the bleeding episode after initial measures to control the bleeding are implemented

The nurse assists with conscious sedation of a school-age client undergoing a bone marrow biopsy. What is the nurse's most important responsibility during the procedure? □ 1. administering the topical anesthetic □ 2. keeping the parents informed □ 3. monitoring the client □ 4. recording the procedure

3. During conscious sedation, the client may lose protective reflexes, and adequate respiratory and cardiac function may be impaired. At every procedure, there must be one health care professional whose sole responsibility is to monitor the client. Topical agents must be given in advance of the procedure to be effective. During the procedure, the nurse would not leave the child to speak with the parents. While the procedure would be documented according to the facility's protocols, proper monitoring of the client is the intervention most associated with reducing risks

Which medication prescription to help relieve pain in a child with leukemia should the nurse question? □ 1. hydromorphone □ 2. acetaminophen with codeine □ 3. ibuprofen □ 4. hydrocodone

3. Ibuprofen prolongs bleeding time and is contraindicated in clients with leukemia. Nonnarcotic drugs other than ibuprofen or aspirin, such as acetaminophen, may be prescribed to control pain and may be used in combination with codeine or hydrocodone if pain is more severe. Hydromorphone may also be used for severe pain.

A school-age client with leukemia is receiving cyclophosphamide. The nurse should assess the client for which adverse effect of cyclophosphamide? □ 1. photosensitivity □ 2. ataxia □ 3. cystitis □ 4. cardiac arrhythmias

3. Cystitis is a potential adverse effect of cyclophosphamide. The client should be monitored for pain on urination. Photosensitivity, ataxia, and cardiac arrhythmias are not adverse effects associated with cyclophosphamide.

A teen is seen in clinic for a possible diagnosis of Hodgkin disease. The nurse is aware that which of the following symptoms should make the health-care provider suspect Hodgkin disease? 1. Fever, fatigue, and pain in the joints. 2. Anorexia with weight loss. 3. Enlarged, painless, and movable lymph nodes in the cervical area. 4. Enlarged liver with jaundice.

3. Enlarged, painless, and movable lymph nodes in the cervical area are the most common presenting manifestations of Hodgkin disease.

The nurse is instructing the parent of a child with HIV about immunizations. Which of the following should the nurse tell the parent? Select all that apply. 1. Hepatitis B vaccine will not be given to this child. 2. Members of the family should be cautioned not to receive the varicella vaccine. 3. The child will need to have a Western blot test done prior to all immunizations. 4. Pneumococcal and influenza vaccines are recommended. 5. Meningitis immunization.

4,5 4. Immunizations against childhood illnesses are recommended for children exposed to or infected with HIV. Pneumococcal and influenza vaccines are recommended at their scheduled times. 5. Meningococcal immunizations can be administered on the regular schedule.

The nurse creates a teaching plan for the family of a child with hemophilia who receives recombinant antihemophilic factor. Which problem is most important for the nurse to teach the family to report immediately? □ 1. yellowing of the skin □ 2. constipation □ 3. abdominal distention □ 4. hives

4. Administration of antihemolytic factor (recombinant) is a biosynthetic preparation of factor VIII that carries the risk of severe allergic reaction. Signs include hives, difficulty breathing, tachycardia, chills, and fever. Originally, factor VIII preparations were derived from large pools of human plasma and carried the risk of hepatitis, but recombinant preparations do not. Antihemolytic factor (recombinant) is not associated with constipation or abdominal distention.

After teaching a child with leukemia about a scheduled bone marrow aspiration, the nurse determines that the teaching has been successful when the child identifies which place as the site for the aspiration? □ 1. right lateral side of the right wrist □ 2. middle of the chest □ 3. distal end of the thigh □ 4. back of the hipbone

4. Although bone marrow specimens may be obtained from various sites, the most commonly used site in children is the posterior iliac crest, the back of the hipbone. This area is close to the body's surface but removed from vital organs. The area is large, so specimens can easily be obtained. For infants, the proximal tibia and the posterior iliac crest are used. The middle of the chest or sternum is the usual site for bone marrow aspiration in an adult. The wrist, chest, and thigh are not sites from which to obtain bone marrow specimens.

A nurse is proving anticipatory guidance to the family of a school-age child with acute lymphocytic leukemia. Which recommendation should the nurse make? □ 1. home schooling for 2 years □ 2. avoiding all athletic activities □ 3. encouraging trips to the shopping mall □ 4. being treated as "normal" as much as possible

4. Any child with a chronic illness should be treated as normally as possible. Unless the child has severe bone marrow depression, he or she should be allowed to go to school with others and can go to the mall. If the child is in remission, athletic activities are allowed.

Which beverage should the nurse plan to give a child with leukemia to relieve nausea? □ 1. orange juice □ 2. weak tea □ 3. plain water □ 4. carbonated soda

4. Carbonated beverages ordinarily are best tolerated when a child feels nauseated. Many children find cola drinks especially easy to tolerate, but noncola beverages are also recommended. Orange juice usually is not tolerated well because of its high acid content. Tea may also be too acidic, and many children do not like tea. Water does not relieve nausea.

What is the most appropriate method to use when drawing blood from a child with hemophilia? □ 1. Use finger punctures for lab draws. □ 2. Prepare to administer platelets. □ 3. Apply heat to the extremity before venipunctures. □ 4. Schedule all labs to be drawn at one time.

4. Coordinating labs to minimize sticks reduces trauma and the risk of bleeding. Fingersticks in general are more painful and associated with more bleeding than are venipunctures. In hemophilia, platelets are typically normal. Heat would increase vasodilatation and increase bleeding.

The nurse teaches the family of child with leukemia about preventing infections. How should the nurse explain to the parents why their child is at risk for infections? □ 1. "Abnormal platelets lead to bruising and bleeding." □ 2. "There are an insufficient number of circulating white blood cells." □ 3. "The number of red blood cells is inadequate for carrying oxygen." □ 4. "Immature white blood cells are incapable of handling an infectious process."

4. In leukemia, although there is an increased number of immature white blood cells, they are unable to combat infection. Lack of mature white blood cells puts a child with leukemia at risk for infection. The major morbidity and mortality factor associated with leukemia is infection resulting from the presence of granulocytopenia. Decreased red blood cells are not directly caused by infection. While platelets play a role in the body's response to infection, bleeding does not directly cause infections.

Which of the following measures should the nurse teach the parent of a child with hemophilia to do first if the child sustains an injury to a joint causing bleeding? 1. Give the child a dose of acetaminophen (Tylenol). 2. Immobilize the joint and elevate the extremity. 3. Apply heat to the area. 4. Administer factor per the home-care protocol.

4. Administration of factor should be the first intervention if home-care transfusions have been initiated.

The parents of a child with sickle cell disease ask the nurse why their child's hemoglobin was normal at birth but now the child has S hemoglobin. Which response by the nurse is appropriate? □ 1. "The placenta prevents the passage of the hemoglobin S from the mother to the fetus." □ 2. "The red bone marrow does not begin to produce hemoglobin S until several months after birth." □ 3. "Antibodies transmitted from you to the fetus provide the newborn with temporary immunity." □ 4. "The newborn has a high concentration of fetal hemoglobin in the blood for some time after birth."

4. Sickle cell disease is an inherited disease that is present at birth. However, 60% to 80% of a newborn's hemoglobin is fetal hemoglobin, which has a structure different from that of hemoglobin S or hemoglobin A. Sickle cell symptoms usually occur about 4 months after birth, when hemoglobin S begins to replace the fetal hemoglobin. The gene for sickle cell disease is transmitted at the time of conception, not passed through the placenta. Some hemoglobin S is produced by the fetus near term. The fetus produces all its own hemoglobin from the earliest production in the first trimester. Passive immunity conferred by maternal antibodies is not related to sickle cell disease, but this transmission of antibodies is important to protect the infant from various infections during early infancy.

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 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.

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 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.