Peds hemetology
The parents of a child with sickle cell disease are both carriers of the gene and ask the nurse how likely it is that they will have another biological child with the disease. What is the correct percent? Fill in the blank.
Rationale: The sickle cell disease occurs if both parents contribute the sickle gene. This factor makes the risk of a biological child having the disease as one in four or 25%.
A nursing student is assigned to care for a child with hemophilia. The nursing instructor reviews the plan of care with the student and asks the student to describe the characteristics of this disorder. Which statement by the student indicates a need for further research? Males inherit hemophilia from their fathers. 2.Females inherit the carrier status from their fathers. 3.Hemophilia A results from deficiency of factor VIII. 4.Hemophilia is inherited in a recessive manner via a genetic defect on the X chromosome.
Ans 1 Rationale: Males inherit hemophilia from their mothers, and females inherit the carrier status from their fathers. Some females who are carriers have an increased tendency to bleed, and although it is rare, females can have hemophilia if their fathers have the disorder and their mothers are carriers of the genetic disorder. Hemophilia 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. Reference(s): Leifer, G. (2015) Introduction to maternity and pediatric nursing (7th ed., pp. 627-628) St. Louis, Elsevier.
A child is admitted to the hospital with sickle cell crisis. The nurse checks this child for which frequent symptom of the disorder? Pain 2.Diarrhea 3.Bradycardia 4.Blurred vision
Ans 1 Rationale: Sickling crisis often causes pain in the bones and joints, accompanied by joint swelling. Pain is a classic symptom of the disease and may require large doses of opioid analgesics when it is severe. The symptoms listed in the other options are not part of the clinical picture. Reference(s): deWit, D. & Kumagai, C. (2013). Medical-surgical nursing: Concepts & practice. (2nd ed., p. 354). St. Louis: Saunders.
Oral iron is prescribed for a child with an iron deficiency anemia, and the nurse provides instructions to the mother regarding the administration of the iron. The nurse instructs the mother to administer the iron in which way? Between meals 2.Just after a meal 3.Just before a meal 4.With a fruit low in vitamin C
Ans 1 between meals Rationale: The mother should be instructed to administer oral iron supplements between meals. The iron should be given with a citrus fruit or juice high in vitamin C because vitamin C increases the absorption of iron by the body. Reference(s): Leifer, G. (2015) Introduction to maternity and pediatric nursing (7th ed., p. 622) St. Louis, Elsevier.
The nurse is caring for a child with a platelet disorder and should expect which prescriptions from the health care provider? Select all that apply. Observe for bleeding. 2.Encourage the child to rest. 3.Aspirin 325 mg orally as needed for pain 4.Provide a hard toothbrush for mouth care. 5.Assist the registered nurse (RN) with blood transfusions.
Ans 1, 2, 5 Rationale: The child with a platelet disorder is at risk for bleeding, so the nurse must observe for any bleeding problems. The child should be encouraged to rest to prevent falls and trauma. Also, packed red blood cells (RBCs) may be prescribed, so the licensed practical nurse nurse must be ready to assist in the care of the child receiving the transfusion. Aspirin should not be given because it interferes with platelet function, and the child should use a soft toothbrush to minimize trauma to the mouth.
A child with sickle cell anemia is being discharged after treatment for a crisis. Which instructions should the nurse reinforce to prevent another crisis from occurring? Select all that apply. Drink plenty of fluids. 2.Avoid foods high in folic acid. 3.Report a sore throat immediately. 4.Use cold packs to relieve joint pain. 5.Restrict all activity to quiet board games. 6.Wash hands before meals and after playing.
Ans 1, 3, 6 Rationale: Sickle cell crisis can be precipitated by cold, dehydration, stress, or infection. Increasing the amount of fluids will reduce the viscosity of blood, thus preventing vascular occlusion. A conscious effort to wash hands can improve the child's health by preventing infection. A sore throat is a sign of an infection and must be reported. It is important to avoid cold temperatures of any kind because this can cause vaso-occlusion. Folic acid avoidance is not necessary. Children need to be encouraged to set Reference(s): Hockenberry, M., & Wilson, D. (2013). Wong's Essentials of pediatric nursing (9th ed., pp. 423, 879-880). St. Louis: Mosby.
The nursing student is presenting a clinical conference and discusses the causative factors related to beta-thalassemia. Which group is at greatest risk of developing this disorder? A child of Mexican descent 2.A child of Mediterranean descent 3.A child whose intake of iron is extremely poor 4.A child breast-fed by a mother with chronic anemia
Ans 2 Rationale: Beta-thalassemia is an autosomal recessive disorder. This disorder is found primarily in individuals of Mediterranean descent. The disease also has been reported in Asian and African populations. Options 1, 3, and 4 are not risk factors for this disorder. Reference(s): Hockenberry, M., & Wilson, D. (2013). Wong's Essentials of pediatric nursing (9th ed., p. 881). St. Louis: Mosby.
The nurse is reinforcing home care instructions to the mother of an infant who has just been found to have hemophilia. The nurse should instruct the mother to do which? 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.
Ans 2 Rationale: Establishment of an age-appropriate safe environment is of paramount importance for hemophiliac clients. Providing a safe environment for an infant includes padding table corners and crib rails, providing extra "joint" padding on clothes, observing a mobile infant at all times, and keeping items that can be pulled down onto the infant out of reach. Use of a soft toothbrush is an appropriate measure for a child with hemophilia, but is not typically necessary for an infant. Rectal temperature
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? Bradycardia 2.Tachycardia 3.Hyperactivity 4.A reddened appearance to the cheeks
Ans 2 Rationale: Signs and symptoms of iron deficiency anemia will vary with the degree of anemia but usually include extreme pallor with porcelain-like skin, tachycardia, lethargy, and irritability. Reference(s): Leifer, G. (2015) Introduction to maternity and pediatric nursing (7th ed., pp. 622-623) St. Louis, Elsevier.
The nurse, caring for a child with aplastic anemia, is reviewing the laboratory results and notes a white blood cell (WBC) count of 6000 cells/mm3 and a platelet count of 20,000 cells/mm3. Which nursing intervention should be incorporated into the plan of care? 1.Encourage naps. 2.Encourage a diet high in iron. 3.Encourage quiet play activities. 4.Maintain strict isolation precautions.
Ans 3 Rationale: Precautionary measures to prevent bleeding should be taken when a child has a low platelet count. These include no injections, no rectal temperatures, the use of a soft toothbrush, and abstinence from contact sports or activities that could cause an injury. Strict isolation would be required if the WBC count was low. Naps and a diet high in iron are unrelated to the risk of bleeding.
The nursing instructor asks a nursing student about the cause of hemophilia. The student correctly responds by telling the instructor which fact about hemophilia? Hemophilia is a Y-linked hereditary disorder. 2.A splenectomy resolves the bleeding disorders. 3.Hemophilia A results from deficiency of factor VIII. 4.A bone marrow transplant is the treatment of choice.
Ans 3 Rationale: The term hemophilia refers to a group of bleeding disorders. The identification of the specific factor deficiencies allows for definitive treatment with replacement agents. Hemophilia A results from a deficiency of factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX. Hemophilia is inherited in a recessive manner via a genetic defect on the X chromosome, not the Y chromosome. Neither a bone marrow transplant nor a splenectomy is used to treat this disorder.
The licensed practical nurse (LPN) is assisting in the admission of a child with suspected sickle cell crisis because of which signs/symptoms noted in this client? Select all that apply. Swollen knee joint 2.Temperature, 97.3° F 3.Pulse,120 beats per minute 4.Peripheral oxygen level of 89% 5.Pain rated as a 6 on a scale of 1 to 10
Ans1,3, 4, and 5 Rationale: Children with sickle cell crisis usually present with one or more swollen joints that are extremely painful (pain rated well above a 5 on a scale of 1 to 10), fever, and low oxygen levels. The heart rate would be increased because of the pain and the lack of normal red blood cells. Reference(s): Leifer, G. (2015) Introduction to maternity and pediatric nursing (7th ed., pp. 624-626) St. Louis, Elsevier.
A child suspected of sickle cell disease is seen in the clinic, and laboratory studies are performed. The nurse reviews the results of the laboratory studies and expects to note which characteristic of this disease? Increased platelet count 2.Increased hematocrit count 3.Increased reticulocyte count 4.Increased hemoglobin count
Ans 3 Rationale: A laboratory diagnosis is established on the basis of a complete blood cell count, examination for sickled red blood cells (RBCs) on the peripheral smear, and hemoglobin electrophoresis. Laboratory studies will show decreased hemoglobin, hematocrit, and platelet count, increased reticulocyte count, and the presence of nucleated red blood cells. Elevated reticulocyte counts occur in children with sickle cell disease because the life span of their sickled RBCs is shortened. Reference(s): Hockenberry, M. & Wilson, D. (2013). Wong's Essentials of pediatric nursing 9th ed., p. 870). St. Louis: Mosby; McKinney, E., James, S., Murray, S., Nelson, K. & Ashwill, J. (2013). Maternal-child nursing (4th ed., pp. 1244-1246). St. Louis: Elsevier.
The nurse is reinforcing home care instructions to the mother of a child with hemophilia. Which activity should the nurse suggest that the child can safely participate in with peers? Soccer 2.Basketball 3.Swimming 4.Field hockey
Ans 3 Rationale: Children with hemophilia need to avoid contact sports and need to take precautions, such as wearing elbow and knee pads and helmets, when participating in other sports. The safest activity that will prevent injury is swimming. Reference(s): Hockenberry, M., & Wilson, D. (2013). Wong's Essentials of pediatric nursing (9th ed., p. 885). St. Louis: Mosby.
The nurse reinforces home care instructions to a client with sickle cell anemia. Which statement by the client indicates a need for further teaching? I'm going to take a painting class." 2."I've learned to knit and sew my own clothes." 3."When I'm feeling better, I'm returning to the soccer team." 4."I'm using a schedule to maintain my increased fluid intake."
Ans 3 Rationale: Clients with sickle cell anemia are advised to avoid strenuous activities. Quiet activities as tolerated are recommended when the client is feeling well. Increasing fluid intake is encouraged to assist in preventing sickle cell crisis. Reference(s): Hockenberry, M., & Wilson, D. (2013). Wong's Essentials of pediatric nursing (9th ed., p. 880). St. Louis: Mosby.
The nurse is reviewing the laboratory results of a child with aplastic anemia and notes that the white blood cell (WBC) count is 2000 cells/mm3, and the platelet count is 150,000 cells/mm3. Which nursing intervention should the nurse incorporate into the plan of care? Avoid unnecessary injections. 2.Encourage quiet play activities. 3.Maintain strict isolation precautions. 4.Encourage the child to use a soft toothbrush.
Ans 3 Rationale: The normal WBC ranges from 5000 to 10,000 cells/mm3 and the normal platelet count ranges from 150,000 to 400,000 cells/mm3. Strict isolation 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.
Laboratory studies are performed on a child suspected of iron deficiency anemia. The nurse reviews the laboratory results, knowing that which finding indicates this type of anemia? A decreased reticulocyte count 2.An elevated red blood cell (RBC) count 3.RBCs that are microcytic and hypochromic 4.An elevated hemoglobin level with a low hematocrit level
Ans 3 Rationale: The results of a complete blood cell count in children with iron deficiency anemia will show low hemoglobin levels and microcytic and hypochromic RBCs. The reticulocyte count is usually normal or slightly elevated. Reference(s): McKinney, E., James, S., Murray, S.,Nelson, K., & Ashwill, J. (2013). Maternal-child nursing (4th ed., pp. 1242-1243). St. Louis: Elsevier.
The nurse reinforces home-care instructions to the parents of a 3-year-old child who has been hospitalized with hemophilia. Which statement by a parent indicates the need for further teaching? "I will supervise my child closely." 2."I will pad the corners of the furniture." 3."I will remove household items that can easily fall over." 4."I will avoid immunizations and dental hygiene treatments for my child."
Ans 4 Rationale: The nurse needs to stress the importance of immunizations, dental hygiene, and routine well-child care. Options 1, 2, and 3 are appropriate statements. The parents are also provided instructions regarding measures to take in the event of blunt trauma (especially trauma that involves the joints), and they are instructed to apply prolonged pressure to superficial wounds until the bleeding has stopped.
The pediatric nursing instructor asks a nursing student to describe the cause of the symptoms that occur in sickle cell disease. Which is the correct response by the nursing student? Bone marrow depression occurs because of the development of sickled cells." 2."Sickled cells increase the blood flow through the body and cause a great deal of pain." 3."The sickled cells mix with the unsickled cells and cause the immune system to become depressed." 4."Sickled cells are unable to flow easily through the microvasculature, and their clumping obstructs blood flow."
Ans 4 Rationale: All the clinical manifestations of sickle cell disease are a result of 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. Options 1, 2, and 3 are inaccurate. Reference(s): McKinney, E., James, S., Murray, S., Nelson, K. & Ashwill, J. (2013). Maternal-child nursing (4th ed., p. 1246). St. Louis: Elsevier.
The nurse reinforces instructions to the mother of a child with sickle cell disease regarding the precipitating factors related to pain crisis. Which, if identified by the mother as a precipitating factor, indicates the need for further teaching? 1.Stress 2.Trauma 3.Infection 4.Fluid overload
Ans 4 Rationale: Pain crisis may be precipitated by infection, dehydration, hypoxia, trauma, or general stress. The mother of a child with sickle cell disease should encourage a fluid intake of 1.5 to 2 times the daily requirement to prevent dehydration. Reference(s): Hockenberry, Wilson (2013), pp. 879-880.
A child suspected of having sickle cell disease (SCD) is seen in a clinic, and laboratory studies are performed. Which laboratory value is likely to be increased in sickle cell disease? Platelet count 2.Hematocrit level 3.Hemoglobin level 4.Reticulocyte count
Ans4 Rationale: A diagnosis is established on the basis of a complete blood count, examination for sickled red blood cells (RBCs) in the peripheral smear, and hemoglobin electrophoresis. Laboratory studies will show decreased hemoglobin and hematocrit levels and a decreased platelet count, an increased reticulocyte count, and the presence of nucleated red blood cells. Increased reticulocyte counts occur in children with SCD because the life span of their sickled RBCs is shortened.
The nurse is reviewing a health care provider's prescription 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 per minute. 5.Provide a high-calorie, high-protein diet. 6.Administer meperidine (Demerol) 25 mg for pain.
Answer 1 and 6 Rationale: Sickle cell anemia is one of a group of diseases called 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, and insufficient oxygen causes the cells to assume a sickle shape; the cells become rigid and clumped together, thus obstructing capillary blood flow. Oral and intravenous fluids are important parts of treatment. Meperidine (Demerol) is not recommended for the child with sickle cell disease because of the risk for normeperidine-induced seizures. Normeperidine, which is a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Therefore, the nurse would question the prescriptions for restricted fluids and meperidine for pain control. Positioning for comfort, avoiding strain in painful joints, oxygen, and a high-calorie, high-protein diet are important parts of the treatment plan.