Saunders sickle cell and hemophilia

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.

16 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 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 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 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 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.

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

A nurse is completing a history and physical on a 3-year old child who is admitted for a surgical repair of Tetralogy of Fallot (TOF). Which of the following manifestations of the condition should the nurse expect? (Select all that apply) a. Polycythemia b. Hypertension c. Clubbing of nail beds d. Failure to thrive e. Pallor f. Murmur

A. Polycythemia (Child's body produces extra blood cells in attempt to increase the oxygen carrying capacity in the blood) C. Clubbing of the nail beds D. Failure to Thrive - results from child expending more energy to eat than it is able to take in F. A long, systolic murmur is usually seen in these patients

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

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

The 8 year old child diagnosed with sickle cell anemia tells the nurse that her family is planning a skiing trip. Which action should the nurse take? a. Take no action because this sounds like an enjoyable trip b. Talk to the parents about taking the child to the mountains c. Tell the child she cannot go skiing because of her disease d. Suggest the child talk to her parents about going on this trip

B. High altitudes have decreased oxygen, which could lead to a sickle cell crisis; therefore, the nurse should discuss this with the parents. The nurse should not have the 8 year old directly talk to the parents.

A nurse is discharging a child with sickle cell anemia after an acute crisis episode. Which of the following should the nurse teach the child's parents to do? a. Monitor the child's temperature daily. b. Restrict outdoor play activity to 1 hr per day c. Encourage the child to drink lots of fluids. d. Have the child eat a high-protein diet.

C. Preventing dehydration is an important step in preventing a sickle cell crisis. The nurse should give the parents a specialized amount of fluid that the child should drink each day.

The adolescent who is diagnosed with anemia has the following lab values: RBC 3mm, WBC 8.9mm, and platelets 150mm. Which intervention should the nurse implement? a. Place the child in reverse isolation b. Continue to monitor the child's lab values c. Administer eryhtropoietin (Epogen), a biological response modifier d. Institute bleeding precautions for the client

C. The client's RBC count is low, Therefore, the nurse should administer Epogen, a biological response modifier that stimulate the production of RBC. (normal is usually between 4-5)

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.

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 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.

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 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 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 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 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.

8. A patient is being tested for sickle cell disease. As the nurse, you know the ________ will assess for abnormal hemoglobin on the red blood cell, but will not differentiate between sickle cell disease and sickle cell trait. Therefore, the patient will need to have what other test to determine this? A. dithionite test; hemoglobin electrophoresis B. hemoglobin electrophoresis; sickledex C. edrophonium test, dithionite test D. sickledex; edrophonium test

The answer is A.

12. A mother brings in her 8 month-old child to the ER. The mother reports the baby has recently started being extremely fussy, has a fever, and swelling in the hands and feet. The child is diagnosed with sickle cell disease. As the nurse you know that the swelling in the hands and feet in the infant is termed? A. Dactylitis B. Erythromelaglia C. Dyshidrotia D. Phalitis

The answer is A. Dactylitis (also called hand-foot syndrome) occurs mainly in infants who are newly diagnosed with sickle cell anemia.

11. You're providing education to a patient with sickle cell anemia who is taking Hydroxyurea. You will make it priority to tell the patient to? A. Consume foods high in calcium and potassium B. Avoid sick people and maintain strict hand hygiene C. Take this medication with at least 8 oz of water D. Monitor your blood glucose level daily

The answer is B. This medication can lower the white blood cell count. Therefore, the nurse should make it priority to tell the patient to avoid infection by avoiding sick people and performing hand hygiene regularly.

14. You're educating the parents of a 12 year-old, who was recently treated for sickle cell crisis, on ways to prevent further sickle cell crises in the further. Which statement by the parents demonstrates they understood your instructions? A. "We will limit fluid intake during the day to 1-2 L a day." B. "Cold showers are best to help with pain associated with sickling." C. "We will avoid traveling to high altitude locations." D. "It is important we refuse all future vaccinations unless absolutely necessary."

The answer is C. Remember sickle cell crisis can be caused by blood loss, illness (it's important the patient is up-to-date with all vaccinations), high altitudes, stress, dehydration, elevated temperature, or extreme cold temperatures. All options are wrong except C.

9. During an outpatient well visit with a patient who has sickle cell anemia, you make it PRIORITY to assess the patient's? A. hemoglobin A1C level B. heart rate C. reflexes D. vaccination history

The answer is D. Patients will sickle cell anemia are at risk for infection because of spleen compromise. Many patients with SCA experience splenomegaly because blood flow is compromised to the spleen due to sickling of RBCs and the spleen is overworked from recycling the old RBCs (remember a patient with sickle cell anemia does NOT have long-living RBCs...the RBCs tend to die in 20 days rather than 120 days). Therefore, vaccination history is very important. The patient should be up-to-date with the flu, pneumococcal, and meningococcal vaccines.

10. An 18 year-old male is taking Hydroxyurea for treatment of sickle cell anemia. Which options below indicate this medication is working successfully? Select all that apply: A. The patient needs fewer blood transfusions. B. The patient experiences diuresis. C. The patient experiences an increase in fetal hemoglobin (Hbg F). D. The patient experiences a decrease in hemoglobin S.

The answers are A and C. This medications actually treats cancer, but it will help with SCA in that it will help create fetal hemoglobin hgb F (this helps decrease sickling episodes) and helps with anemia (decreasing the need for so many blood transfusions).