Hematological Peds

aplastic anemia diagnostics (3)

-CBC = pancytopenia (deficiency of erythrocyes, leukocytes, thrombocytes) -bone marrow aspiration (yellow, fatty bone marrow)

parent teaching: increasing absorption of iron supplements (3)

-take between meals (to ↑ absorption) -take with vitamin C (ex: OJ) (to ↑ absorption) -do not give with milk or antacids (they ↓ absorption)

iron deficiency anemia risk factors (5)

-premature birth (↓ iron stores) -excessive intake of cow's milk in toddlers -poor dietary intake -GI malabsorption -adolescents (poor diet, rapid growth, menses)

sickle cell crisis triggers (4)

-stress -trauma -infection -dehydration

iron deficiency anemia symptoms (5)

-tachycardia -pallor -fatigue, weakness -spoon-shaped fingernails -↓ Hgb and Hct

Immune Thrombocytopenic Purpura (ITP)

-Bleeding disorder characterized by increased destruction of platelets in the spleen -When the rate of platelet destruction exceeds the rate of platelet production, the number of circulating platelets decreases and blood clotting slows

ITP treatments (4)

-IV immunoglobulin (IVIG) -Corticosteroids -IV anti-D immunoglobulin -Splenectomy if chronic

parent education: hemophilia (4)

-avoid contact sports -protective equipment (helmets, knee and elbow pads) -signs of internal bleeding -control bleeding using RICE (rest, ice, compression, elevation)

aplastic anemia treatments (3)

-bone marrow transplant -anti-lymphocyte globulin (immunosuppressive med) -anti-thymocyte globulin (immunosuppressive med)

sickle cell splenic sequestration

-caused by pooling and clumping of blood in the spleen -hypovolemia, hypovolemic shock

sickle cell vaso-occlusive crisis

-caused by stasis of blood with clumping of cells in the microcirculation, ischemia, and infarction -fever; painful swelling of hands, feet, joints; abdominal pain

side effects of iron supplements (3)

-constipation -black stools -foul aftertaste

hemophilia medications (4)

-factor VIII -DDAVP vasopressin (more for management, prevention) -corticosteroids (hematuria, hemathrosis, synovitis) -NSAIDs (synovitis)

sickle cell treatment (3)

-hydration -oxygenation -pain management

sickle cell hyperhemolytic crisis

-increased rate of RBC destruction -anemia, jaundice, reticulocytosis

aplastic anemia symptoms (6)

-petechiae -purpura -pallor -bleeding -tachycardia -weakness, fatigue

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."

1. "I need to cancel the upcoming dental appointment that I made for my child." NCLEX

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

1. Between meals NCLEX

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

1. Blood in the urine (kidneys) NCLEX

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.

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. 6. The disorder causes platelets to adhere to damaged endothelium. NCLEX

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.

1. Eliminate any toys with sharp edges 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.) NCLEX

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.

1. Measure the injured knee joint every shift. NCLEX

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.

1. Restrict fluid intake. 6. Give meperidine, 25 mg intravenously, every 4 hours for pain. (Oral and IV 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.) NCLEX

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.

1.Restrict fluid intake 6.Give meperidine, 25 mg intravenously, every 4 hours for pain 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 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

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.

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

2. Adequate hydration, pain management NCLEX

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.

2. Administer the iron through a straw. (stains teeth) NCLEX

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.

2. Begin intravenous fluids. (Increased fluid volume reduces the viscosity of the blood, preventing further vascular occlusion and further sickling caused by dehydration.) NCLEX

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.

2. Blood transfusion of packed red blood cells. (Blood product transfusion is not the treatment of choice over administering recombinant factors intravenously.) NCLEX

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

2. Hypoxia NCLEX

Which of the following describe(s) 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. ITP causes excessive destruction of platelets. 3. Children with ITP have normal bone marrow. 5. Purpura is observed in ITP 1. ITP is an acquired hematological condition that is characterized by excessive destruction of platelets, purpura, and normal bone marrow along with increase in large, yellow platelets. 2. ITP is characterized by excessive destruction of platelets. 3. The bone marrow is normal in children with ITP. 4. Platelets are large, not small. 5. ITP is characterized by purpuras, which are areas of hemorrhage under the skin. NCLEX

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.

2. Pad crib rails and table corners. NCLEX

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

2. Presence of hematuria (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.) NCLEX

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

2.A child of Mediterranean descent NCLEX

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

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.

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."

3. "We will avoid having our child receive immunizations." NCLEX

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

3. Application of a bivalved cast for joint immobilization (other options could increase bleeding) NCLEX

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.

3. Ask the child to sit down and lean forward, and apply pressure to the nose. NCLEX

A client in sickle cell crisis is admitted to the emergency department. What are the priorities of care in order of importance? 1. Nutrition, hydration, electrolyte balance 2. Hydration, pain management, electrolyte balance 3. Hydration, oxygenation, pain management 4. Hydration, oxygenation, electrolyte balance

3. Hydration, oxygenation, pain management Critical Thinking

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.

3. If each parent carries the trait, the child will carry the trait, and the probability of the child having the disease is 75%. (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.) NCLEX

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

3. Intravenous fluids (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.) NCLEX

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

3. Intravenous infusion of factor VIII (hemophilia A = factor VIII, hemophilia B = factor IX) NCLEX

The nurse is reviewing the laboratory results of a child with aplastic anemia and notes that the white blood cell count is 2000 mm3 and that the platelet count is 150,000 mm3. 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.

3. Maintain strict neutropenic precautions. -normal WBC = 5,000-10,000 mm3 -normal platelets = 150,000-400,000 mm3 (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.) NCLEX

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

3. Swimming (no contact sports) NCLEX

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

3. Tachycardia (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.) NCLEX

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

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.

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

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 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."

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 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."

4. "Affected prepubescent girls should be counseled concerning menorrhagia, which may be life-threatening." (Hemarthrosis is the result of bleeding into the joint cavity, not of aspiration.) NCLEX

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."

4. "I know my child must spend as much time as possible in the sun." NCLEX

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

4. Bone marrow biopsy NCLEX

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

4. Deferoxamine NCLEX

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

4. Deficient production of functional hemoglobin NCLEX

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

4. Fluid overload (dehydration is a precipitating factor) NCLEX

he 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.

4. Hemophilia A results from 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.) NCLEX

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

4. Reticulocyte count NCLEX

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

4. Morphine sulfate (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.) NCLEX

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

4. Orange juice (vitamin C enhances body's absorption of iron) NCLEX

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

4. Partial thromboplastin time NCLEX

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

4. Prolonged PTT (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.) NCLEX

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

4. Red blood cells that are microcytic and hypochromic NCLEX

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.

4. The child is drowsy and difficult to arouse; previously the child was able to respond to questions effectively. (One sign of internal bleeding is change in level of consciousness, which could indicate intracranial hemorrhage.) NCLEX

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

A

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

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

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

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.

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

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

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

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

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

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.

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

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

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

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

A nurse is providing teaching about epistaxis to the parent of a school-age child. Which of the following should the nurse include as an appropriate action to take when managing an episode of epistaxis? (Select all that apply.) A. Press the nares together for at least 10 min. B. Breathe through the nose until bleeding stops C. Pack cotton or tissue into the naris that is bleeding D. Apply a warm cloth across the bridge of the nose E. Insert petroleum into the naris after the bleeding stops

A. Press the nares together for at least 10 min. C. Pack cotton or tissue into the naris that is bleeding ATI

A nurse is providing teaching about the management of epistaxis to a child and his family. Which of the following positions should the nurse instruct the child to take when experiencing a nosebleed? A. Sit up and lean forward B. Sit up and tilt the head up C. Lie in a supine position D. Lie in a prone position

A. Sit up and lean forward ATI

Which should the nurse teach about prevention of sickle cell crises to parents of a preschool child with sickle cell disease? (Select all that apply.) a. Limit fluids at bedtime. b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. d. Use ice packs to decrease the discomfort of vasoocclusive pain in the legs. e. Notify the health care provider if your child begins to develop symptoms of a cold.

ANS: B, C, E The most important issues to teach the family of a child with sickle cell anemia are to (1) seek early intervention for problems, such as a fever of 38.5° C (101.3° F) or greater; (2) give penicillin as ordered; (3) recognize signs and symptoms of splenic sequestration, as well as respiratory problems that can lead to hypoxia; and (4) treat the child normally. The nurse emphasizes the importance of adequate hydration to prevent sickling and to delay the adhesion-stasis-thrombosis-ischemia cycle. It is not sufficient to advise parents to "force fluids" or "encourage drinking." They need specific instructions on how many daily glasses or bottles of fluid are required. Many foods are also a source of fluid, particularly soups, flavored ice pops, ice cream, sherbet, gelatin, and puddings. Increased fluids combined with impaired kidney function result in the problem of enuresis. Parents who are unaware of this fact frequently use the usual measures to discourage bedwetting, such as limiting fluids at night. Enuresis is treated as a complication of the disease, such as joint pain or some other symptom, to alleviate parental pressure on the child. Ice should not be used during a vasoocclusive pain crisis because it vasoconstricts and impairs circulation even more.

1. Which child should the nurse document as being anemic? a. 7-year-old child with a hemoglobin of 11.5 g/dl b. 3-year-old child with a hemoglobin of 12 g/dl c. 14-year-old child with a hemoglobin of 10 g/dl d. 1-year-old child with a hemoglobin of 13 g/dl

ANS: C Anemia is a condition in which the number of red blood cells, or hemoglobin concentration, is reduced below the normal values for age. Anemia is defined as a hemoglobin level below 10 or 11 g/dl. The child with a hemoglobin of 10 g/dl would be considered anemic. The normal hemoglobin for a child after 2 years of age is 11.5 to 15.5 g/dl.

The nurse is conducting a staff in-service on sickle cell anemia. Which describes the pathologic changes of sickle cell anemia? a. Sickle-shaped cells carry excess oxygen. b. Sickle-shaped cells decrease blood viscosity. c. Increased red blood cell destruction occurs. d. Decreased adhesion of sickle-shaped cells occurs.

ANS: C The clinical features of sickle cell anemia are primarily the result of increased red blood cell destruction and obstruction caused by the sickle-shaped red blood cells. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. Increased adhesion and entanglement of cells occurs.

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

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

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

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

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

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

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

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

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

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.

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

A nurse is caring for an infant whose screening test reveals that he might have sickle cell disease. Which of the following tests should be performed to distinguish if the infant has the trait or the disease? A. Sickle solubility test B. Hemoglobin electrophoresis C. Complete blood count D. Transcranial Doppler

B. Hemoglobin electrophoresis ATI

A nurse is preparing to administer iron dextran IM to a school-age child who has iron deficiency anemia. Which of the following actions by the nurse is appropriate? A. Administer the dose in the deltoid muscle B. Use the Z-track method when administering the dose C. Avoid injection more than 2 mL with each dose D. Massage the injection site for 1 min after administering the dose

B. Use the Z-track method when administering the dose ATI

In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxia-ischemia 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.

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.

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.

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.

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.

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

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.

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).

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.

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.

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

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.

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

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

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

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

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

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

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

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 caring for a child who has been admitted for a sickle cell crisis. Which of the following would the nurse do first to provide adequate pain management? A) Administer a nonsteroidal anti-inflammatory drug as ordered. B) Use guided imagery and therapeutic touch. C) Administer meperidine as ordered. D) Initiate pain assessment with a standardized pain scale.

D) Initiate pain assessment with a standardized pain scale.

A nurse is providing teaching to the parent of a child who has a new prescription for liquid oral iron supplements. Which of the following statements by the parent indicates an understanding of the teaching? A. "I should take my child to the emergency department if his stools become dark." B. "My child should avoid eating citrus fruits while taking the supplements." C. "I should give the iron with milk to help prevent an upset stomach." D. "My child should take the supplement through a straw."

D. "My child should take the supplement through a straw." (stains teeth) ATI

hemophilia B

Factor IX deficiency

hemophilia A

Factor VIII deficiency

The nurse is caring for a child with a low platelet count. What skin assessments would alert the nurse to bleeding? (Select all that apply.) a. Petichiae b. Purpura c. Ecchymosis d. Hematoma e. Lymphadenopathy

a. Petichiae b. Purpura c. Ecchymosis d. Hematoma

A school-age child is admitted in vasoocclusive sickle cell crisis. The child's care should include: a. correction of acidosis. b. adequate hydration and pain management. c. pain management and administration of heparin. d. adequate oxygenation and replacement of factor VIII.

b. adequate hydration and pain management.

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

d. Painful swelling of hands and feet; painful joints

sickle cell aplastic crisis

extreme anemia as a result of decreased RBC production, typically triggered by an infection with a virus

iron supplementation for infants

iron-fortified cereals and formula

parent teaching: taking iron supplements

liquid iron stains the teeth - take through a straw and brush teeth after administration