PEDS Chapter 25: Nursing Care of the Child with a Hematologic Disorder
After teaching the parents of a child diagnosed with pernicious anemia about the disorder and treatment, the nurse determines that the teaching was successful when the parents state which of the following?
"He'll need to have those vitamin shots for the rest of his life." Monthly injections of vitamin B12 are required for life. Although diet is important, diet alone will not cure the anemia. Iron used to treat iron-deficiency anemia can lead to constipation. Bone marrow transplant is used to treat aplastic anemia.
The parents of a 6-year-old male with idiopathic thrombocytopenic purpura (ITP) ask the nurse conducting an assessment of the child what causes the disease. What is the nurse's best response?
"ITP is primarily an autoimmune disease in that the immune system attacks and destroys the body's own platelets, for an unknown reason." Idiopathic thrombocytopenic purpura (ITP) is primarily an autoimmune disease, which is an acquired, self-limiting disorder of hemostasis characterized by destruction and decreased numbers of circulating platelets. Hemophilia A and hemophilia B are distinguished by the particular procoagulant factor that is decreased, absent, or dysfunctional. Iron deficiency anemia occurs when the body's iron stores are depleted. Hereditary spherocytosis (HS) is characterized by loss of surface area on the red blood cell membrane.
The nurse is teaching an inservice program to a group of nurses on the topic of children diagnosed with sickle cell anemia. The nurses in the group make the following statements. Which statement is most accurate regarding sickle cell anemia?
"If the trait is inherited from both parents the child will have the disease." Explanation: When the trait is inherited from both parents (homozygous state), the child has sickle cell disease, and anemia develops. The trait does not skip generations. The trait occurs most commonly in African Americans. Either sex can have the trait and disease.
The nurse is teaching an inservice program to a group of nurses on the topic of children diagnosed with sickle cell anemia. The nurses in the group make the following statements. Which statement is most accurate regarding sickle cell anemia?
"If the trait is inherited from both parents the child will have the disease." When the trait is inherited from both parents (homozygous state), the child has sickle cell disease, and anemia develops. The trait does not skip generations. The trait occurs most commonly in African Americans. Either sex can have the trait and disease.
In discussing the causes of iron deficiency anemia in children with a group of nurses, the following statements are made. Which of these statements is a misconception related to iron deficiency anemia?
"Milk is a perfect food, and babies should be able to have all the milk they want." Babies with an inordinate fondness for milk can take in an astonishing amount and, with their appetites satisfied, may show little interest in solid foods. These babies are prime candidates for iron deficiency anemia. Many children with iron deficiency anemia, however, are undernourished because of the family's economic problems. A caregiver knowledge deficit about nutrition is often present. Because only 10 percent of dietary iron is absorbed, a diet containing 8 to 10 mg of iron is needed for good health. During the first years of life, obtaining this quantity of iron from food is often difficult for a child. If the diet is inadequate, anemia quickly results.
The nurse is reinforcing teaching with a group of caregivers of children diagnosed with iron deficiency anemia. One of the caregivers tells the group, "I give my child ferrous sulfate." Which of the following statements made by the caregivers is correct regarding giving ferrous sulfate?
"My husband gives our daughter orange juice when she takes her ferrous sulfate, so she gets Vitamin C." When ferrous sulfate is administered, it should be given between meals with juice (preferably orange juice, because vitamin C aids in iron absorption). For best re sults, iron should not be given with meals. Ferrous sulfate can cause constipation or turn the child's stools black.
A nurse is providing dietary interventions for a 5-year-old with an iron deficiency. Which response indicates a need for further teaching?
"Red meat is a good option; he loves the hamburgers from the drive-thru." While iron from red meat is the easiest for the body to absorb, the nurse must limit fast food consumption from the drive-thru as they are also high in fat, fillers, and sodium. The other statements are correct.
A child is to receive oral iron therapy in liquid form three times a day. After teaching the parents about administering the iron, which statement indicates a need for additional teaching?
"She can drink the medicine from a medicine cup." Liquid iron can stain the teeth; therefore, the parents should give the liquid iron through a straw or syringe, placing it toward the back of the child's mouth. Iron turns stools dark. To maximize absorption, it is best to give the iron with water or juice between meals.
The nurse is preparing a child for discharge following a sickle cell crisis. The mother makes the following statements to the nurse. Which statement by the mother indicates a need for further teaching?
"She has been down, but playing in soccer camp will cheer her up." Following a sickle cell crisis the child should avoid extremely strenuous activities that may cause oxygen depletion. Fluids are encouraged, pain management will be needed, and the child's legs may be elevated to relieve discomfort, so these are all statements that indicate an understanding of caring for the child who has had a sickle cell crisis.
The nurse is caring for a child with aplastic anemia. The nurse is reviewing the child's blood work and notes the granulocyte count is about 500, platelet count is over 20,000, and the reticulocyte count is over 1%. The parents ask if these values have any significance. Which response by the nurse is appropriate?
"These values will help us monitor the disease." Explanation: This response answers the parent's questions. In the nonsevere form, the granulocyte count remains about 500, the platelets are over 20,000, and the reticulocyte count is over 1%. The other responses do not address what the parents are asking and would block therapeutic communication.
The nurse is caring for a child with aplastic anemia. The nurse is reviewing the child's blood work and notes the granulocyte count is about 500, platelet count is over 20,000, and the reticulocyte count is over 1%. The parents ask if these values have any significance. Which response by the nurse is appropriate?
"These values will help us monitor the disease." This response answers the parent's questions. In the nonsevere form, the granulocyte count remains about 500, the platelets are over 20,000, and the reticulocyte count is over 1%. The other responses do not address what the parents are asking and would block therapeutic communication.
A nurse is caring for a 7-year-old boy with hemophilia who requires an infusion of factor VIII. He is fearful about the process and is resisting treatment. How should the nurse respond?
"Would you help me dilute this and mix it up?" Explanation: The best response for a 7-year-old is to use distraction and involve him in the infusion process in a developmentally appropriate manner. A 7-year-old is old enough to assist with the dilution and mixing of the factor. Asking for help with the band-aid would be best for a younger child. Teens should be taught to administer their own factor infusions. Telling him to be brave is not helpful and does not teach.
A child abruptly develops miniature petechiae over his legs, along with epistaxis and bleeding into the joints. Laboratory results reveal a platelet count of 20,000/mm3. The child is eventually diagnosed with idiopathic thrombocytopenic purpura (ITP). The mother of the child is distraught and asks the nurse what the course of this disorder typically is. Which of the following should the nurse mention?
1 to 3 months In most children, ITP runs a limited, 1- to 3-month course. A few children develop chronic ITP.
The caregiver of a child with sickle cell disease asks the nurse how much fluid her child should have each day after the child goes home. In response to the caregiver's question, the nurse would explain that for the child with sickle cell disease, it is best that the child have:
1,500 to 2,000 mL of fluid per day Explanation: Prevention of crises is the goal between episodes. Adequate hydration is vital; fluid intake of 1,500 to 2,000 mL daily is desirable for a child weighing 20 kg and should be increased to 3,000 mL during the crisis.
The child has been diagnosed with severe iron deficiency anemia. The child requires 5 mg/kg of elemental iron per day in three equally divided doses. The child weighs 47.3 lb (21.5 kg). How many milligrams of elemental iron should the child receive with each dose? Record your answer using a whole number.
36 Explanation: The dose should be calculated using weight in kilograms. 21.5 kg x 5 mg/1 kg = 107.5 mg/day. 107.5 mg/3 doses = 35.8333 mg/dose Rounded to the nearest whole number = 36 mg
A nurse is reviewing the medical records of several children who have undergone lead screening. The nurse would identify the child with which lead level as requiring no further action?
8 mcg/dL A blood lead level less than 10 mcg/dL requires no action. A level of 14 mcg/dL would need to be confirmed with a repeat test in 1 month along with parental education for decreased lead exposure and then a repeat test in 3 months. Levels of 20 mcg/dL and 26 mcg/dL need to be confirmed with a repeat test in 1 week along with parental education and a referral to the local health department for investigation of the home for lead reduction.
Which assessment below would increase your suspicion that iron-deficiency anemia may be present in a child?
A 15-year-old girl constantly sucks ice cubes Iron-deficiency anemia is associated with pica, or the eating of nonfood substances.
A nurse is assessing an 8-year-old child brought to the emergency department by his mother. The child has a history of sickle-cell anemia and reports acute back pain and joint pain. His mucous membranes are dry; skin turgor is poor. Capillary refill is slowed and nail beds are pale. The child is diagnosed with sickle-cell crisis. Which nursing diagnosis would the nurse most likely identify as a priority?
Acute pain related to effects of sickling Although ineffective peripheral tissue perfusion and deficient fluid volume would apply, acute pain would be the priority. Once pain is relieved, the child is able to relax, thus reducing the metabolic demand for oxygen and helping to end the sickling. There is no information to correlate with a nursing diagnosis of ineffective coping.
An 8-month-old girl appears pale, irritable, and anorexic. On blood testing, the red blood cells are hypochromic and microcytic. The hemoglobin level is less than 5 g/100 mL, and the serum iron level is high. Which of the following symptoms should the nurse most expect as a result of excessive iron deposits?
An enlarged spleen The child with thalassemia major may have both an enlarged spleen and liver due to excessive iron deposits and fibrotic scarring in the liver and the spleen's increased attempts to destroy defective RBCs.
A nurse is preparing a teaching plan for a child with hemophilia and his parents. Which of the following would the nurse be least likely to include to manage a bleeding episode?
Apply heat to the site of bleeding. Ice or cold compresses, not heat, would be applied to the site of bleeding. Direct pressure is applied until the bleeding stops. The injured part is elevated unless elevating would contribute to further injury. Factor VIII replacement is given to replace the missing clotting factor.
In understanding the cardiovascular and hematologic systems of the body it is important to know that the blood is made up of plasma, red blood cells, white blood cells, and platelets. These blood cells are formed in which of the following?
Bone marrow Blood is a fluid composed of many elements, including plasma, red blood cells, white blood cells, and platelets. Each of these elements has a different function. These blood cells are formed in the bone marrow. Arteries carry blood away from the heart to the body, and veins collect the blood and return it to the heart. Capillaries are the exchange vessels for the materials that flow through the body. The lymph nodes filter the lymph.
In addition to the child's history, symptoms, and blood studies, which of the following helps to confirm the diagnosis of leukemia?
Bone marrow aspiration In addition to the his tory, symptoms, and laboratory blood studies, a bone mar row aspiration must be done to confirm the diagnosis of leu kemia. Genetic studies are done for hereditary diseases such as sickle cell anemia and hemophilia. The modified Jones criteria are used as a guide to note the manifestations of rheumatic fever, and chest x-rays help in diagnosing congestive heart failure.
Individuals with hemophilia B have a deficiency in factor IX, which can cause excessive blood loss. What is another name for this clotting factor?
Christmas factor Explanation: Factor IX is also known as plasma thromboplastin component or Christmas factor; factor X is Stuart factor; factor VIII is antihemophilic factor; and factor VII is proconvertin.
The nurse is caring for an 18-month-old with suspected iron deficiency anemia. Which lab results confirm the diagnosis?
Decreased hemoglobin and hematocrit, decreased reticulocyte count, microcytosis, and hypochromia, decreased serum iron and ferritin levels and increase FEP level Laboratory evaluation will reveal decreased hemoglobin and hematocrit, decreased reticulocyte count, microcytosis and hypochromia, decreased serum iron and ferritin levels, and increase FEP level. The other findings do not point to iron deficiency anemia.
In a child diagnosed with sickle cell disease, which of the following clinical manifestations would the nurse most likely see?
Decreased hemoglobin level Sickle cell disease causes chronic anemia, with a hemoglobin level of 6 to 9 g/dL (the normal level in an infant is 11 to 15 g/dL). The chronic anemia causes the child to have a poor appetite and severe, acute abdominal pain (caused by sludging, which leads to enlargement of the spleen) and an absence of bowel sounds. A strawberry colored tongue is seen in the diagnosis of Kawasaki disease.
The nurse is caring for a 10-year-old girl with iron toxicity. Which of the following would the nurse expect the physician to order?
Deferasirox Deferasirox is indicated for iron toxicity. It binds with iron, which is removed via the kidneys. Dimercaprol is indicated for blood lead levels greater than 45 mcg/dL. It removes lead from soft tissues and bone, allowing for its excretion via the renal system. Edentate calcium disodium is indicated for blood lead levels greater than 45 mcg/dL. The medication removes lead from soft tissues and bone, allowing for its excretion via the renal system. Succimer is indicated for blood lead levels greater than 45 mcg/dL; it removes lead from soft tissues and bone, allowing for its excretion via the renal system.
After teaching a group of students about hemophilia, the instructor determines that the students have understood the information when they identify hemophilia A as involving a problem with which of the following?
Factor VIII Explanation: In hemophilia A, the problem is with factor VIII, and in hemophilia B it is factor IX. Platelets are problematic in idiopathic thrombocytopenia purpura. Plasmin is involved in the pathophysiologic events of disseminated intravascular coagulation.
A toddler who is beginning to walk has fallen and hit his head on the corner of a low table. The caregiver has been unable to stop the bleeding and brings the child to the pediatric clinic. The nurse is gathering data during the admission process and notes several bruises and swollen joints. A diagnosis of hemophilia is confirmed. This child most likely has a deficiency of which of the following blood factors?
Factor VIII The most common types of hemophilia are factor VIII deficiency and factor IX deficiency, which are inherited as sex-linked recessive traits, with transmission to male offspring by carrier females.
An 11-year-old male is diagnosed with mild hemophilia. Upon assessment, the nurse documents the following factor level for this category of hemophilia:
Factor level of 5% to 50% Explanation: Mild hemophilia is characterized by a factor level of 5% to 50%. People with mild hemophilia experience prolonged bleeding only when injured. Thus, their condition may not be diagnosed unless they have trauma or surgery.
In hemophilia A, the classic form, only females manifest a bleeding disorder.
False The classic form of hemophilia is caused by deficiency of the coagulation component factor VIII, the antihemophilic factor, and transmitted as a sex-linked recessive trait. In the United States, the incidence is approximately 1 in 10,000 white males. A female carrier may have slightly lowered but sufficient levels of the factor VIII component so that she does not manifest a bleeding disorder. Males with the disease also have varying levels of factor VIII; their bleeding tendency varies accordingly, from mild to severe.
Which of the following would the nurse be least likely to assess in a child with a hematologic disorder?
Fever Explanation: Pediatric hematologic alterations usually are characterized by atypical hemostasis, anemia, and/or neutropenia. Fever suggests infection which may or may not be present with a hematologic disorder.
The nurse is caring for a child with leukemia. Which of the following nursing interventions would be the highest priority for this child?
Following guidelines for protective isolation The child with leukemia is susceptible to infection, especially during chemotherapy. Infections such as meningitis, septicemia, and pneumonia are the most common causes of death. To protect the child from infectious organisms, follow standard guidelines for protective isolation. Grouping nursing care to provide rest is important, but not the highest priority. Encouraging the child to share feelings and providing age appropriate activities are important, but psychological issues are a lower priority than physical.
A child is diagnosed with sickle-cell anemia. Which component of the blood, the one responsible for the transport of oxygen, is defective in this disorder?
Hemoglobin Explanation: The component of RBCs that allows them to carry out the transport of oxygen is hemoglobin, composed of globin, a protein, and heme, an iron-containing pigment. Fetal hemoglobin differs from adult hemoglobin; for this reason, diseases such as sickle-cell anemia or the thalassemias, which are disorders of the beta chains, do not become apparent clinically until this hemoglobin change has occurred (at approximately 6 months of age).
A nurse is caring for a newborn whose screening test result indicates the possibility of sickle cell anemia (SCA) or sickle cell trait. The nurse would expect the test result to be confirmed by which lab tests?
Hemoglobin electrophoresis If the screening test result indicates the possibility of SCA or sickle cell trait, hemoglobin (Hgb) electrophoresis is performed promptly to confirm the diagnosis. While Hgb electrophoresis is the only definitive test for diagnosis of the disease, other laboratory testing that assists in the assessment of the disease include reticulocyte count (greatly elevated), peripheral blood smears (presence of sickle-shaped cells and target cells), and erythrocyte sedimentation rate (elevated).
A group of students are reviewing the effects of sickle cell anemia on the various parts of the body. The students demonstrate a need for additional study when they identify which of the following as an effect?
High urine specific gravity Explanation: Low urine specific gravity (hyposthenuria) occurs with sickle cell anemia. Chest syndrome is an acute manifestation of sickle cell anemia. Pulmonary hypertension is a chronic manifestation of sickle cell anemia. Cholelithiasis is a chronic manifestation of sickle cell anemia.
A group of students are reviewing the effects of sickle cell anemia on the various parts of the body. The students demonstrate a need for additional study when they identify which of the following as an effect?
High urine specific gravity Low urine specific gravity (hyposthenuria) occurs with sickle cell anemia. Chest syndrome is an acute manifestation of sickle cell anemia. Pulmonary hypertension is a chronic manifestation of sickle cell anemia. Cholelithiasis is a chronic manifestation of sickle cell anemia. (less)
A nurse is preparing a 7-year-old girl for bone marrow aspiration. Which of the following sites should she prepare?
Iliac crest Bone marrow aspiration provides samples of bone marrow so the type and quantity of cells being produced can be determined. In children, the aspiration sites used are the iliac crests or spines (rather than the sternum, which is commonly used in adults) because performing the test at these sites is usually less frightening for children; these sites also have the largest marrow compartments during childhood. In neonates, the anterior tibia can be used as an additional site.
The nurse preparing a patient for diagnostic testing for disseminated intravascular coagulation knows that the following is a result indicative of this disease:
Increased D-dimer assay Results indicative of disseminated intravascular coagulation include: increased D-dimer assay, decreased antithrombin III, increased fibrogen/fibrin degradation products, and increased fibrinopeptide A level.
Which nursing diagnosis would be most appropriate for a child with idiopathic thrombocytopenic purpura?
Ineffective tissue perfusion related to poor platelet formation Idiopathic thrombocytopenic purpura results in decreased platelets, so bleeding into tissue can occur.
A nurse is teaching the parents of a child with sickle cell disease about factors that predispose the child to a sickle cell crisis. The nurse determines that the teaching was successful when the parents identify which of the following as a factor?
Infection Factors that may precipitate a sickle cell crisis include: fever, infection, dehydration, hot or humid environment, cold air or water temperature, high altitude, or excessive physical activity. Respiratory distress and pallor are general signs and symptoms of a sickle cell crisis. (less)
The child with Thalassemia may be given which of the following classifications of medications to prevent one of the complications frequently seen with the treatment of this disorder.
Iron-chelating drugs Frequent transfusions can lead to complications and additional concerns for the child, including the possibility of iron overload. For these children, iron-chelating drugs such as deferoxamine mesylate (Desferal) may be given. Vitamin and potassium supplements would not be given to treat the iron overload. Factor VIII preparations are given to the child with hemophilia.
The nurse is evaluating the complete blood count of a 7-year-old child with a suspected hematological disorder. Which finding is associated with an elevated mean corpuscular volume (MCV)?
Macrocytic red blood cells (RBCs) Explanation: When the MCV is elevated, the RBCs are larger and referred to as macrocytic. The WBC count does not affect the MCV. The platelet count and Hgb are within normal ranges for a 7-year-old child.
The nurse is evaluating the complete blood count of a 7-year-old child with a suspected hematological disorder. Which finding is associated with an elevated mean corpuscular volume (MCV)?
Macrocytic red blood cells (RBCs) When the MCV is elevated, the RBCs are larger and referred to as macrocytic. The WBC count does not affect the MCV. The platelet count and Hgb are within normal ranges for a 7-year-old child.
A school-aged child is admitted to the hospital with a vaso-occlusive sickle cell crisis. Which measure in his care should be given priority?
Maintaining a fluid intravenous line Dehydration increases sickling of cells, so maintaining fluid balance is important.
A child with sickle cell disease is brought to the emergency department by his parents. He is in excruciating pain. A vaso-occlusive crisis is suspected and analgesia is prescribed. Which of the following would the nurse expect as least likely to be ordered?
Meperidine Explanation: Meperidine is contraindicated for ongoing pain management in a child with vaso-occlusive crisis because it increases the risk for seizures. Analgesics such as morphine, nalbuphine, or hydromorphone are commonly used.
When assessing a child for a possible hematologic disorder, which of the following would the nurse need to keep in mind as most important?
Multiple body sites can be affected. Explanation: The nurse needs to keep in mind that hematologic alterations can affect multiple body sites, so assessment needs to address all body systems. A child's nutritional status may be helpful in assessing certain hematologic disorders such as iron deficiency anemia, but this information is not the most important to remember. Sequelae commonly occur with hematologic alterations, especially chronic conditions such as hemophilia or sickle cell disease. The child's demographic data are important, because some hematologic diagnoses are more commonly associated with a certain age group, sex, race, or geographic location.
A group of nursing students are reviewing the process of blood cell formation. The students demonstrate understanding of this process when they place the following events in the proper sequence.
Multipotent stem cell Myeloid progenitor Megakaryocyte/erythroid progenitor Megakaryocyte Platelets Explanation: The process begins with the multipotent stem cell that then becomes a myeloid progenitor, then a megakaryocyte/erythroid progenitor, a megakaryocyte, and finally platelets.
The nurse is caring for a child with disseminated intravascular coagulation. The nurse notices signs of neurological deficit. Which nursing action is appropriate?
Notify the physician If neurological deficits are assessed, immediate reporting of the findings is necessary to begin treatment to prevent permanent damage.
The nurse is assessing a child and notices pinpoint hemorrhages appearing on several different areas of the body. The hemorrhages do not blanch on pressure. The nurse documents this finding as which of the following?
Petechiae Petechiae are pinpoint hemorrhages that occur anywhere on the body and do not blanch with pressure. Purpura are larger areas of hemorrhage in which blood collects under the tissues and appear purple in color. Ecchymosis refers to areas of bruising. Poikilocytosis refers to the variation in the size and shape of the red blood cells commonly found in children with thalassemia.
The nurse is administering meperidine as ordered for pain management for a 10-year-old boy in sickle cell crisis. The nurse would be alert for which of the following?
Seizures Repeated use of meperidine for pain management during sickle cell crisis increases the risk of seizures when used in children with sickle cell anemia. Behavioral addiction is rarely a concern in the child with sickle cell anemia if the narcotic is used for the alleviation of severe pain. Priapism is a complication of sickle cell anemia unrelated to meperidine administration. Leg ulcers are a complication of sickle cell anemia unrelated to meperidine administration.
A child is diagnosed with iron deficiency anemia. Which diagnostic test would the nurse suspect as being the most sensitive test for determining this disorder?
Serum ferritin Serum ferritin measures the level of ferritin (the major iron storage protein) in the blood and is the most sensitive test for determining iron-deficiency anemia. Hemoglobin electrophoresis measures the percentage of normal and abnormal hemoglobin in the blood and is used to diagnose sickle cell anemia and thalassemia not iron-deficiency anemia. Reticulocyte count measures the amount of reticulocytes in the blood and is used to evaluate the bone marrow's ability to respond to anemia not iron-deficiency anemia. Serum iron level evaluates iron metabolism but is not as sensitive as the serum ferritin test for determining iron-deficiency anemia.
Which of the following statements is most accurate related to sickle cell disease?
Sickle cell disease is genetically inherited. Sickle cell disease is a hereditary trait occurring most commonly in African Americans.
The nurse is examining the hands of a child with suspected iron deficiency anemia. Which finding should the nurse expect?
Spooning of nails A convex shape of the fingernails termed 'spooning' can occur with iron deficiency anemia. Capillary refill in less than 2 seconds, pink palms and nail beds, and absence of bruising are normal findings.
The parents of a child with a bleeding disorder ask the nurse about appropriate activities and sports that they should encourage the child to participate in. Which of the following would be the safest for the nurse to suggest?
Swimming Swimming, a noncontact sport or activity, would be the safest for the nurse to recommend. Soccer and gymnastics may be appropriate; however, these are considered riskier. Rugby would not be recommended because the risks outweigh the benefits.
The nurse is collecting data from the caregivers of a child brought to the clinic setting. The parents tell the nurse that the child's skin seems to be an unusual color. The nurse notes that the child's skin appears bronze-colored and jaundiced. This observation alerts the nurse to the likelihood that this child has which of the following disorders?
Thalassemia In the child with Thalassemia the skin may appear bronze-colored or jaundiced. The child with hemophilia may have bruised areas on the skin. The skin color in children with sickle cell disease may be pale in color, and with Kawasaki disease the child may have a rash on the trunk and extremities.
For the child diagnosed with iron deficiency anemia which of the following would the nurse anticipate would be done in treating this disorder?
The child would be given ferrous sulfate with orange juice between meals. Treatment consists of improved nutrition, with ferrous sulfate administered between meals with juice (preferably orange juice, because vitamin C aids in iron absorption). For best results, iron should not be given with meals.
In caring for a child with sickle cell disease, the highest priority goal is which of the following?
The child's fluid intake will improve. The highest priority goals for this child are maintaining comfort and relieving pain. The child is prone to dehydration because of the kidneys' inability to concentrate urine, so increasing fluid intake is the next highest priority. Other goals include improving physical mobility, maintaining skin integrity, reducing the caregivers' anxiety, and increasing the caregivers' knowledge about the causes of crisis episodes, but these goals are not the highest priority.
In von Willebrand's disease, girls exhibit unusually heavy menstrual flow.
True von Willebrand's disease, an inherited autosomal dominant disorder, affects both sexes and is often referred to as angiohemophilia. Along with a factor VIII defect, there is also an inability of the platelets to aggregate and the blood vessels to constrict to aid in coagulation. Bleeding time is prolonged, with most hemorrhages occurring from mucous membrane sites. Epistaxis is a major problem, because all children tend to rub or pick at their nose as a nervous mechanism. In girls, menstrual flow is unusually heavy and may cause embarrassment from stained clothing.
A 14-year-old girl who is a vegetarian has recently developed anemia. Blood smear results show large, fragile, immature erythrocytes. She claims to take an iron supplement regularly and is surprised to learn that she is anemic, as she is otherwise healthy. The nurse recognizes that which of the following is the likely cause of this type of anemia?
Vitamin B12 deficiency Explanation: Vitamin B12 is necessary for the maturation of RBCs. Pernicious anemia results from deficiency or inability to use the vitamin, resulting in RBCs that appear abnormally large and are immature megaloblasts (nucleated immature red cells). Thus, pernicious anemia is one of the megaloblastic anemias. In children, the cause is more often lack of ingestion of vitamin B12 rather than poor absorption. Adolescents may be deficient in vitamin B12 if they are ingesting a long-term, poorly formulated vegetarian diet as the vitamin is found primarily in foods of animal origin.
A nurse is providing care for a child with disseminated intravascular coagulation (DIC). Which of the following would alert the nurse to possible neurologic compromise?
Widely fluctuating blood pressure A key aspect of the nurse's role is to assess the child for signs and symptoms of impaired tissue perfusion in the various body systems that may be affected by DIC. Unstable or abnormal blood pressure such as wide fluctuations in blood pressure or unequal pupil size may suggest neurologic compromise. Hematuria would suggest renal compromise. Petechiae would be indicative of bleeding into the skin.
When planning care for a child with idiopathic thrombocytopenic purpura, you plan to teach her
not to pick or irritate her nose. Without adequate platelets, children bleed easily from lesions.
To prevent further sickle cell crisis, you would advise the parents of a child with sickle cell anemia to
notify a health care provider if the child develops an upper respiratory infection. Reduction of oxygen and dehydration lead to increased sickling of cells. Early prevention of these with respiratory illness is important.
A nurse in the emergency department is examining a 6-month-old with symmetrical swelling of the hands and feet. The nurse immediately suspects:
sickle cell disease. Symmetrical swelling of the hands and feet in the infant or toddler is termed dactylitis; aseptic infarction occurs in the metacarpals and metatarsals and is often the first vaso-occlusive event seen with sickle cell disease. Symmetrical swelling of the hands and feet are not typically seen with the other conditions listed
You care for a 4-year-old with sickle cell anemia. A physical finding you might expect to see in him is
slightly yellow sclerae. Many children with sickle cell anemia develop mild scleral yellowing from excess bilirubin from breakdown of damaged cells.
A 3-year-old female is brought to the ER by her parents and presents with bruising and mucous membrane bleeding from the nose and mouth. The nurse knows that these symptoms are indicative of:
von Willebrand disease The primary clinical manifestations of von Willebrand disease are bruising and mucous membrane bleeding from the nose, mouth, and gastrointestinal tract; bleeding may be severe and lead to anemia and shock. Deep bleeding into joints and muscles, like that seen in hemophilia, is rare, except with type III von Willebrand disease.
A 10-month-old has been admitted to the hospital with severe hemolytic anemia and chronic hypoxia. The nurse notes icteral sclerae, jaundice of the skin, and frontal and maxillary bossing. The nurse interprets these findings as most likely indicating which of the following?
β-Thalassemia major Severe hemolytic anemia and chronic hypoxia, icteral sclerae, jaundice of the skin, and frontal and maxillary bossing are signs and symptoms of β-thalassemia major. Hemophilia is manifested by clotting dysfunctions. von Willebrand disease is manifested by abnormal clotting. Sickle cell anemia involves abnormal hemoglobin that leads to significant anemia and acute and chronic symptoms.
The young boy has had his spleen surgically removed. Which statements by the boy's parents prior to discharge indicates that an adequate amount of learning has occurred?
• "If he gets a fever, I'm going to call our physician right away." • "Before he goes to the dentist, we'll make sure he gets antibiotics." • "He's going to need several vaccines." • "He's going to get really good at washing his hands." Explanation: The caregivers should seek medical treatment promptly for any clinical manifestations associated with an infection. The child should receive prophylactic antibiotics. The child should be provided with immunization against the following organisms: Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae type B. The child should be taught techniques to reduce the transmission of infection. The child should wear his medic alert bracelet all the time.
The nurse is working with a child who is in sickle cell crisis. Treatment and nursing care for this child includes which of the following. Select all that apply.
• Administering oxygen • Administering analgesics • Maintaining fluid intake Explanation: Treatment for a crisis is supportive for each presenting symptom, and bed rest is indicated. Oxygen may be administered. Analgesics are given for pain. Dehydration and acidosis are vigorously treated. Prognosis is guarded, depending on the severity of the disease.
The nurse is reviewing the laboratory test results of a child with thalassemia. Which result would the nurse expect to find with the hemoglobin electrophoresis? Select all that apply.
• Hemoglobin A2 • Hemoglobin F Explanation: In thalassemia, the hemoglobin electrophoresis would reveal the presence of hemoglobin F and A2 only. Hemoglobin S would be found with sickle cell disease.
A 5-year-old boy is diagnosed with congenital aplastic anemia. Which of the following symptoms should the nurse expect in this child? (Select all that apply.)
• Pallor • Easy bruising • Fatigue • Cyanosis Explanation: When symptoms begin, a child appears pale, fatigues easily, and has anorexia from the lowered RBC count and tissue hypoxia. Because of reduced platelet formation (thrombocytopenia), the child bruises easily or develops petechiae (pinpoint, macular, purplish-red spots caused by intradermal or submucous hemorrhage). A child may have excessive nosebleeds or gastrointestinal bleeding. As a result of a decrease in WBCs (neutropenia) a child may contract an increased number of infections and respond poorly to antibiotic therapy. Observe closely for signs of cardiac decompensation such as tachycardia (not bradycardia), tachypnea (not bradypnea), shortness of breath, or cyanosis from the long-term increased workload of all these effects on the heart.
