Hematological Peds

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

aplastic anemia diagnostics (3)

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

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

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

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)

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

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

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 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

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

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

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

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

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

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 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

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 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

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

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

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 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

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

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

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