NCLEX - Pediatrics - Hematology

A child is brought to the emergency department after falling from a high swing and landing on the back. The nurse notes that the client also has hemophilia. Based on the client's history and the nature of the injury, which should the nurse assess for first? 1.Blood in the urine 2.Oxygen saturation 3.Presence of headache 4.Presence of slurred speech

Correct Answer: 1 Rationale: Because the kidneys are located in the flank region of the body, trauma to the back area can cause hematuria, particularly in the child with hemophilia. The nurse would be most concerned about the child's airway and respiratory rate if the child sustained an injury to the neck region. Headache and slurred speech are associated with head trauma.

A 2-year-old boy with a diagnosis of hemophilia is admitted to the hospital with bleeding into the joint of the right knee. Which intervention should the nurse plan to implement with this child? 1.Measure the injured knee joint every shift. 2.Take the temperature by rectal method only. 3.Administer acetylsalicylic acid for pain control. 4.Immobilize the joint and apply moist heat to the joint.

Correct Answer: 1 Rationale: Interventions for bleeding into the joint include measuring the injured joint to assess for progression of the bleeding. This provides objective rather than subjective data, which are needed to determine if the bleeding is increasing. Rectal temperatures can cause tissue trauma, causing further bleeding. The application of heat and the administration of acetylsalicylic acid will increase bleeding.

Oral iron is prescribed for a child with iron deficiency anemia. The nurse provides instructions to the mother regarding the administration of the iron. The nurse should instruct the mother to administer the medication in which way? 1.Between meals 2.Just before a meal 3.Just after the meal 4.With a fruit low in vitamin C

Correct Answer: 1 Rationale: The mother should be instructed to administer oral iron supplements between meals. The iron should be given with a citrus fruit or juice high in vitamin C because vitamin C increases the absorption of iron by the body.

The nurse is providing instructions to the mother of a 3-year-old child with hemophilia regarding care of the child. Which statement by the mother indicates a need for further teaching? 1."I need to cancel the upcoming dental appointment that I made for my child." 2."If my child gets a cut, I should hold pressure on it until the bleeding stops." 3."I should check the house and remove any household items that can easily fall over." 4."I should move furniture with sharp corners out of the way and pad the corners of the furniture."

Correct Answer: 1 Rationale: The nurse needs to stress the importance of immunizations, dental hygiene, and routine well-child care. The remaining options are appropriate care measures. The mother is instructed regarding actions in the event of blunt trauma, especially trauma involving the joints, and is told to apply prolonged pressure to superficial wounds until the bleeding has stopped.

The home care nurse is providing safety instructions to the mother of a child with hemophilia. Which instruction should the nurse include to promote a safe environment for the child? 1.Eliminate any toys with sharp edges from the child's play area. 2.Allow the child to use play equipment only when a parent is present. 3.Allow the child to play indoors only, and avoid any outdoor play or playgrounds. 4.Place a helmet and elbow pads on the child every day as soon as the child awakens.

Correct Answer: 1 Rationale: The nurse should instruct the mother to remove toys with sharp edges that may cause injury from the child's play area. It is not necessary to restrict play if safety measures have been implemented. It is not necessary that the child be restricted from outdoor play activity, but the activities that the child participates in should be monitored. Requiring that the child wear a helmet and elbow pads immediately on awakening and throughout the day is not necessary; however, these items should be worn during activities that could cause injury.

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

Correct 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 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.

Correct 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.

A child is brought to the emergency department after being accidentally struck in the lower back region with a baseball bat. When gathering assessment data, the nurse discovers that the child has hemophilia. The nurse should immediately assess for which data? 1. Slurred speech 2. Presence of hematuria 3. Complaints of headache 4. Change in respiratory rate

Correct Answer: 2 Rationale: Because the kidneys are located in the flank region of the body, trauma to the back area can cause hematuria, particularly in a child with hemophilia. The nurse would be most concerned about the child's airway and respiratory rate if the child had sustained an injury to the neck region. Slurred speech and headache are associated with head trauma.

An 11-year-old child is admitted to the hospital in vaso-occlusive sickle cell crisis. The nurse plans for which priority treatments in the care of the child? 1.Splenectomy, correction of acidosis 2.Adequate hydration, pain management 3.Frequent ambulation, oxygen administration 4.Passive range-of-motion exercises, adequate hydration

Correct Answer: 2 Rationale: During vaso-occlusive sickle cell crisis, the care focuses on adequate hydration and pain management. Adequate hydration with intravenous normal saline and oral fluids maintains blood flow and decreases the severity of the vaso-occlusive crisis. Analgesics for pain management are necessary during a vaso-occlusive crisis. Splenectomy would not be done with a vaso-occlusive crisis. Acidosis is not present. Oxygen can be administered to increase tissue perfusion but is not the priority treatment for a vaso-occlusive crisis. Passive range of motion is not recommended; bed rest is prescribed initially.

The nurse is providing home care instructions to the mother of an infant who has just been found to have hemophilia. The nurse should tell the mother that care of the infant should include which appropriate measure? 1.Use aspirin for pain relief. 2.Pad crib rails and table corners. 3.Use a soft toothbrush for dental hygiene. 4.Use a generous amount of lubricant when taking a temperature rectally.

Correct Answer: 2 Rationale: Establishment of an age-appropriate, safe environment is of paramount importance for hemophiliacs. Providing a safe environment for an infant includes padding table corners and crib rails, providing extra padding on clothes to protect the joints, observing a mobile infant at all times, and keeping items that can be pulled down onto the infant out of reach. Use of a soft toothbrush is an appropriate measure for a child with hemophilia but is not typically necessary for an infant. Rectal temperature measurements and the use of aspirin are contraindicated in hemophiliacs because of the risk of bleeding.

The nursing student is assigned to care for a child with hemophilia. The nursing instructor reviews the plan of care with the student. Which intervention on the student written plan of care requires correction? 1.Measure circumference of injured joints. 2.Blood transfusion of packed red blood cells. 3.Monitor temperature with oral thermometers. 4.Intravenous administration of recombinant factor.

Correct Answer: 2 Rationale: Hemophilia is a lifelong hereditary blood disorder associated with deficiency of clotting factors. It is inherited in a recessive manner via a genetic defect on the X chromosome. Hemophilia A results from a deficiency of factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX. Blood product transfusion is not the treatment of choice over administering recombinant factors intravenously. Measuring circumference of injured joints is appropriate to assess for enlarging hematomas or bleeding under the skin. The nurse should avoid taking rectal temperatures to decrease the risk for injury.

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.

Correct 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.

A child with sickle cell anemia who is in vaso-occlusive crisis is admitted to the hospital. Which health care provider prescription would assist in reversing the vaso-occlusive crisis? 1.Monitor pulse oximetry. 2.Begin intravenous fluids. 3.Administer oxygen by face mask. 4.Monitor vital signs and respiratory status.

Correct Answer: 2 Rationale: Increased fluid volume reduces the viscosity of the blood, preventing further vascular occlusion and further sickling caused by dehydration. Pulse oximetry and vital sign monitoring may be components of care, but they are actions that relate to monitoring the client versus treating. The intravenous fluids, however, will treat the condition. Vaso-occlusive crisis treatment includes analgesic and fluid administration. Oxygen may help relieve symptoms of respiratory distress, but analgesics and fluids treat the condition.

The pediatric nursing instructor asks a nursing student to prioritize care for a child diagnosed with sickle cell disease. Which student response correctly identifies the priority of care? 1.Fatigue 2.Hypoxia 3.Delayed growth 4.Avascular necrosis

Correct Answer: 2 Rationale: Sickle cell disease is 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. Hypoxia causes the cells to assume a sickle shape, and the cells become rigid and clumped together, obstructing capillary blood flow and leading to a vaso-occlusive crisis. All the clinical manifestations of sickle cell anemia result from the sickled cells being unable to flow easily through the microvasculature, and their clumping obstructs blood flow. With reoxygenation most of the sickled red blood cells resume their normal shape. Fatigue is a result of hypoxia; hypoxia should be addressed first. Avascular necrosis of the hips and shoulders and delayed growth are general manifestations of sickle cell disease.

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

Correct 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. Options 1, 3, and 4 are incorrect.

The nurse is collecting data on a 12-month-old child with iron deficiency anemia. Which finding should the nurse expect to note in this child? 1.Cyanosis 2.Bronze skin 3.Tachycardia 4.Hyperactivity

Correct Answer: 3 Rationale: Clinical manifestations of iron deficiency anemia will vary with the degree of anemia but usually include extreme pallor with a porcelain-like skin, tachycardia, lethargy, and irritability.

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

Correct 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.

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

Correct 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 is caring for a child with a diagnosis of hemophilia, and hemarthrosis is suspected because the child is complaining of pain in the joints. Which measure should the nurse expect to be prescribed for the child? 1.Range-of-motion exercises to the affected joint 2.Application of a heating pad to the affected joint 3.Application of a bivalved cast for joint immobilization 4.Nonsteroidal antiinflammatory drugs for the pain

Correct Answer: 3 Rationale: In an acute period, immobilization of the joint would be prescribed. Range-of-motion exercise during the acute period can increase the bleeding and would be avoided at this time. Heat will increase blood flow to the area, so it would promote increased bleeding to the area. Nonsteroidal antiinflammatory drugs (NSAIDs) can prolong bleeding time and would not be prescribed for the child.

A child with a diagnosis of sickle cell disease is being admitted for the treatment of vaso-occlusive crisis. The nurse prepares for the admission anticipating which prescription for the child? 1.NPO status 2.Meperidine for pain 3.Intravenous fluids 4.Intubation to administer oxygen

Correct Answer: 3 Rationale: Intravenous fluid and increased oral fluids are a component of the treatment plan for the child with vaso-occlusive crisis. Management of the severe pain that occurs with vaso-occlusive crisis includes the use of opioid analgesics, such as morphine sulfate and hydromorphone. Meperidine is contraindicated because of its side effects and the increased risk of seizures with its use. Oxygen is administered when hypoxia is present and the oxygen saturation level is less than 95%. Intubation is not necessary to treat vaso-occlusive crisis.

A nursing student is assigned to care for a child with sickle cell disease (SCD). The nursing instructor asks the student to describe the causative factors related to this disease. Which statement by the student indicates a need for further research? 1.SCD is an autosomal recessive disease. 2.Children with the HbS (sickle cell hemoglobin) trait are not symptomatic. 3.If each parent carries the trait, the child will carry the trait, and the probability of the child having the disease is 75%. 4.If one parent has the HbS trait and the other parent is normal, there is a 50% chance that each offspring will inherit the trait.

Correct Answer: 3 Rationale: SCD is an autosomal recessive disease. Children with the HbS trait are not symptomatic. If one parent has the HbS trait and the other parent is normal, there is a 50% chance that each offspring will inherit the trait. If each parent carries the trait, there is a 25% chance that their child will be normal, a 50% chance that the child will carry the trait, and a 25% chance that each child will have the disease.

A child arrives at the emergency department with a nosebleed. On assessment, the nurse is told by the mother that the nosebleed began suddenly and for no apparent reason. What is the initial nursing action? 1.Insert nasal packing. 2.Prepare a nasal balloon for insertion. 3.Ask the child to sit down and lean forward, and apply pressure to the nose. 4.Place the child in a semi Fowler's position, and apply ice packs to the nose.

Correct Answer: 3 Rationale: The initial nursing action for a child with a nosebleed is to have him or her sit down, ask the child to lean forward, and apply pressure to the nose for 5 to 10 minutes. Ice or cool compresses may also be applied to the nose and face. Placing the child in semi Fowler's position would cause swallowing of blood. Inserting nasal packing and preparing a nasal balloon are not appropriate initial interventions. A nasal packing or nasal balloon may be used if conservative measures fail.

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

Correct 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 nurse is reviewing the laboratory results of a child with aplastic anemia and notes that the white blood cell count is 2000 mm3 (2 × 109/L) and that the platelet count is 150,000 mm3 (150 × 109/L). Which intervention should the nurse incorporate into the plan of care? 1.Avoid unnecessary injections. 2.Encourage quiet play activities. 3.Maintain strict neutropenic precautions. 4.Encourage the child to use a soft toothbrush.

Correct Answer: 3 Rationale: The normal white blood cell (WBC) count ranges from 5000 to 10,000 mm3 (5 to 10 × 109/L)and the normal platelet count ranges from 150,000 to 400,000 mm3 (150 to 400 × 109/L). Strict neutropenic procedures would be required if the WBC count were low to protect the child from infection. Precautionary measures to prevent bleeding should be taken when a child has a low platelet count. These include no injections, no rectal temperatures, use of a soft toothbrush, and abstinence from contact sports or activities that could cause an injury.

The nurse provides instructions regarding home care to the parents of a 3-year-old child hospitalized with hemophilia. Which statement, if made by the parent, indicates a need for further instructions? 1."We will supervise our child closely." 2."We will pad corners of the furniture." 3."We will avoid having our child receive immunizations." 4."We will remove household items that can easily fall over."

Correct Answer: 3 Rationale: The nurse needs to stress the importance of immunizations, dental hygiene, and routine well-child care. The remaining options are appropriate. The parents also are instructed in the measures to implement in the event of blunt trauma, especially trauma involving the joints, and taught to apply prolonged pressure to superficial wounds until the bleeding has stopped.

A child is seen in the health care clinic for complaints of fever. On data collection, the nurse notes that the child is pale, tachycardic, and has petechiae. Aplastic anemia is suspected. The nurse should prepare the child to obtain which specimen that will confirm the diagnosis? 1.Platelet count 2.Granulocyte count 3.Red blood cell count 4.Bone marrow biopsy

Correct Answer: 4 Rationale: Although the diagnosis of aplastic anemia may be suspected from the child's history and from the results of a complete blood count, a bone marrow biopsy must be performed to confirm the diagnosis.

A 12-year-old child with newly diagnosed thalassemia is brought to the clinic exhibiting delayed sexual maturation, fatigue, anorexia, pallor, and complaints of headache. The child seems listless and small for age and has frontal bossing. What should the nurse expect to note on review of the results of the laboratory tests? 1.Macrocytosis and hyperchromia 2.Excessive red blood cell production 3.Excessive mature erythrocyte proliferation 4.Deficient production of functional hemoglobin

Correct Answer: 4 Rationale: Defective hemoglobin is produced as a result of genetically deficient beta-polypeptide. This hemoglobin is unstable, disintegrates, and damages the erythrocytes. Rapid destruction of the red cells stimulates rapid production of immature red cells, and the net gain is less than optimally functioning red cells. Iron from the red blood cell destruction is stored in the tissues, causing multiple problems. In thalassemia, immature erythrocytes proliferate, not mature ones. This is a progressive anemia. The nurse also would note microcytosis and hypochromia.

The pediatric nurse educator provides a teaching session to the nursing staff regarding hemophilia. Which statement regarding this disorder should the nurse plan to include in the discussion? 1.Males inherit hemophilia from their fathers. 2.Hemophilia is a Y-linked hereditary disorder. 3.Females inherit hemophilia from their mothers. 4.Hemophilia A results from deficiency of factor VIII.

Correct Answer: 4 Rationale: Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins. Hemophilia A results from a deficiency of factor VIII. Males inherit hemophilia from their mothers, and females inherit the carrier status from their fathers. Hemophilia is inherited in a recessive manner via a genetic defect on the X chromosome. Hemophilia B (Christmas disease) is a deficiency of factor IX.

Oral iron supplements are prescribed for a 6-year-old child with iron deficiency anemia. Which beverage is the best option to recommend with iron administration? 1.Milk 2.Water 3.Apple juice 4.Orange juice

Correct Answer: 4 Rationale: Vitamin C (ascorbic acid) increases the absorption of iron by the body. The mother should be instructed to administer the medication with a citrus fruit or juice high in vitamin C. From the options presented, the correct option is the only one that identifies the food highest in vitamin C.

The nurse is monitoring the laboratory values of a child with leukemia who is receiving chemotherapy. The nurse prepares to implement bleeding precautions if the child becomes thrombocytopenic and the platelet count is less than how many cells/mm3? 1.200,000 mm3 (200 × 109/L) 2.180,000 mm3 (180 × 109/L) 3.160,000 mm3 (160× 109/L) 4.150,000 mm3 (150 × 109/L)

Correct Answer: 4 Rationale: If a child is thrombocytopenic, precautions need to be taken because of the increased risk of bleeding. The precautions include limiting activity that could result in head injury, using soft toothbrushes, checking urine and stools for blood, and administering stool softeners to prevent straining with constipation. Additionally, suppositories and rectal temperatures are avoided. The normal platelet count ranges from 150,000 to 400,000 mm3 (150 to 400 × 109/L).

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

Correct 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.

A child with a diagnosis of sickle cell anemia and vaso-occlusive crisis is complaining of severe pain, selecting number 8 on the 1 to 10 pain scale. Which medication would the nurse expect to be prescribed for pain control? 1.Ibuprofen 2.Meperidine 3.Acetaminophen 4.Morphine sulfate

Correct Answer: 4 Rationale: Morphine sulfate is the medication of choice for severe pain for the child with sickle cell anemia. Opioids such as morphine sulfate provide systemic relief. Ibuprofen decreases inflammation locally. Meperidine has neurological adverse effects and can cause seizures and should be avoided. Acetaminophen would not provide adequate pain relief.

The nurse is caring for a child with hemophilia and is reviewing the results that were sent from the laboratory. Which result should the nurse expect in this child? 1.Shortened prothrombin time (PT) 2.Prolonged PT 3.Shortened partial thromboplastin time (PTT) 4.Prolonged PTT

Correct Answer: 4 Rationale: PTT measures the activity of thromboplastin, which is dependent on intrinsic factors. In hemophilia, the intrinsic clotting factor VIII (antihemophilic factor) is deficient, resulting in a prolonged PTT. The results in the remaining options are incorrect. The PT may not necessarily be affected in this disorder.

A child in whom sickle cell anemia is suspected is seen in a clinic, and laboratory studies are performed. The nurse checks the laboratory results, knowing that which value would be increased in this disease? 1.Platelet count 2.Hematocrit level 3.Hemoglobin level 4.Reticulocyte count

Correct Answer: 4 Rationale: Sickle cell anemia is 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. A diagnosis is established on the basis of a complete blood count, examination for sickled red blood cells in the peripheral smear, and hemoglobin electrophoresis. Laboratory studies will show decreased hemoglobin level and hematocrit, a decreased platelet count, an increased reticulocyte count, and the presence of nucleated red blood cells. Reticulocyte counts are increased in children with sickle cell disease because the life span of their sickled red blood cells is shortened.

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

Correct 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 1½ to 2 times the daily requirement to prevent dehydration.

The pediatric nurse educator is providing a teaching session to nursing staff about hemophilia. Which statement should the nurse educator include? 1."Acetylsalicylic acid is given for pain control." 2."Hemarthrosis is the result of synovial cavity aspiration." 3."Total joint rest along with ice pack application continues for 72 hours after factor VIII is administered." 4."Affected prepubescent girls should be counseled concerning menorrhagia, which may be life-threatening."

Correct Answer: 4 Rationale: The female offspring of an affected male and a carrier female is at risk for hemorrhage once puberty is attained and menstrual cycles begin, and depending on the severity of the hemophilia, a hysterectomy or ablation may be performed. The remaining options are incorrect statements. Aspirin is not routinely given to young children and would not be given to a child with a bleeding disorder because of its effects on platelet aggregation. Hemarthrosis is the result of bleeding into the joint cavity, not of aspiration. Seventy-two hours is too long for the joint to be rested because maintenance of mobility is a primary concern once the bleeding episode has been arrested.

The nurse provides instructions to the mother of a child with sickle cell disease. Which statement by the mother indicates a need for further teaching? 1."I need to be sure that my child has adequate rest periods." 2."I will take my child's temperature and watch for a fever." 3."I need to encourage my child to drink large amounts of fluids." 4."I know my child must spend as much time as possible in the sun."

Correct Answer: 4 Rationale: The nurse should instruct the mother to encourage fluid intake 1.5 to 2 times the daily requirements. Adequate rest periods should be provided, and the child should not be exposed to cold or heat stress. The mother should be taught how to take the child's body temperature and how to use a thermometer properly. Sources of infection should be avoided, as should prolonged exposure to the sun.

The nurse on the pediatric unit is caring for a child with hemophilia who has been in a motor vehicle crash. Which assessment finding, if noted in the child, indicates the need for follow-up? 1.The child maintains affected joints in an immobilized position and denies pain at this time. 2.The child's urine is noted to be clear and light yellow and is negative for red blood cells. 3.The child maintains bruised joints in an elevated position; the bruises noted are beginning to turn yellow. 4.The child is drowsy and difficult to arouse; previously the child was able to respond to questions effectively.

Correct Answer: 4 Rationale: When caring for a child with hemophilia who has sustained injuries, the nurse should monitor for signs of internal bleeding. One sign of internal bleeding is change in level of consciousness, which could indicate intracranial hemorrhage. Additional signs of bleeding include pain, tenderness, and bruising of the affected area and hematuria. Denial of pain of affected joints, clear and light yellow urine that is negative for red blood cells, and bruises that are beginning to turn yellow are not signs of internal or external bleeding.

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 to be prescribed? 1.Fragmin 2.Meropenem 3.Metoprolol 4.Deferoxamine

Correct 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 Exjade or deferoxamine may be prescribed. Deferoxamine is classified as an antidote for acute iron toxicity. Fragmin is an anticoagulant used as prophylaxis for postoperative deep vein thrombosis. Meropenem is an antibiotic. Metoprolol is a beta blocker used to treat hypertension.