Peds PrepU: Chapter 25
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: a) 1,000 to 1,200 mL of fluid per day b) 2,500 to 3,200 mL of fluid per day c) 1,500 to 2,000 mL of fluid per day d) 300 to 800 mL of fluid per day
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.
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? a) Apply direct pressure to the area. b) Administer factor VIII replacement. c) Apply heat to the site of bleeding. d) Elevate the injured area such as a leg or arm.
Apply heat to the site of bleeding. Explanation: 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.
Individuals with hemophilia B have a deficiency in factor IX, which can cause excessive blood loss. What is another name for this clotting factor? a) Stuart factor b) Christmas factor c) Proconvertin d) Antihemophilic 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.
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? a) Pallor b) Infection c) Fluid overload d) Respiratory distress
Infection Explanation: 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
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? a) Nalbuphine b) Morphine c) Meperidine d) Hydromorphone
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
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? a) Factor VIII b) Plasmin c) Platelets d) Factor IX
a. 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 school-aged child is admitted to the hospital with a vaso-occlusive sickle cell crisis. Which measure in his care should be given priority? a) Encouraging him to take deep breaths hourly b) Maintaining a fluid intravenous line c) Beginning active range-of-motion exercises d) Seeing that he ingests a protein-rich diet
b. Maintaining a fluid intravenous line Explanation: Dehydration increases sickling of cells, so maintaining fluid balance is important.
You care for a 4-year-old with sickle cell anemia. A physical finding you might expect to see in him is a) increased growth of long bones. b) slightly yellow sclerae. c) enlarged mandibular growth. d) depigmented areas on the abdomen
b. slightly yellow sclerae. Explanation: Many children with sickle cell anemia develop mild scleral yellowing from excess bilirubin from breakdown of damaged cells.
To prevent further sickle cell crisis, you would advise the parents of a child with sickle cell anemia to a) encourage the child to participate in school activities, such as long-distance running. b) prevent the child from drinking an excess amount of fluids per day. c) notify a health care provider if the child develops an upper respiratory infection. d) administer an iron supplement daily
notify a health care provider if the child develops an upper respiratory infection. Explanation: Reduction of oxygen and dehydration lead to increased sickling of cells. Early prevention of these with respiratory illness is important
A nurse is reviewing laboratory test results from several children, looking specifically at their thrombocyte levels. The nurse would identify that the child with which platelet level might be at risk for bleeding? Select all that apply. a) 80,000 per cubic millimeter b) 175,000 per cubic millimeter c) 110,000 per cubic millimeter d) 234,000 per cubic millimeter e) 287,000 per cubic millimeter
• 80,000 per cubic millimeter • 110,000 per cubic millimeter Explanation: Normal thrombocyte level ranges from 150,000 to 300,000 per cubic millimeter. Therefore, a child with a thrombocyte level of 80,000 and 110,000 per cubic millimeter would be at risk for bleeding.
The nurse is reviewing the results of a clotting study for a healthy 6-year-old. Which of the following would the nurse document as a normal prothrombin finding? a) 16.0 to 18.0 seconds b) 6.0 to 9.0 seconds c) 11.0 to 13.0 seconds d) 21.0 to 35.0 seconds
11.0 to 13.0 seconds Explanation: The nurse would identify a prothrombin time of 11.0 to 13.0 seconds as normal for a healthy child. A result of 21.0 to 35.0 seconds would be the expected range for partial thromboplastin time and activated partial thromboplastin time. Findings of 6.0 to 9.0 seconds and 16.0 to 18.0 seconds are outside the normal range.
The nurse is caring for a child in sickle cell crisis. To best promote hemodilution, the nurse would expect to administer how much fluid per day intravenously or orally? a) 110 mL/kg of fluids b) 120 mL/kg of fluids per day c) 150 mL/kg of fluids d) 130 mL/kg of fluids per day
150 mL/kg of fluids Explanation: To promote hemodilution in sickle cell crisis, the nurse would provide 150 mL/kg of fluids per day or as much as double maintenance, either orally or intravenously
Complications associated with bleeding most often involve joints and muscles. Adjunct measures to control bleeding include: a) Compression b) Lowering extremities c) Heat d) Exercise
Compression Complications associated with bleeding most often involve joints and muscles. Adjunct measures include rest, ice, compression, and elevation (RICE). In addition, corticosteroids such as prednisone may be used to reduce inflammation in the joint.
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? a) Factor X b) Factor V c) Factor XIII d) Factor VIII
Factor VIII Explanation: 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: a) Factor level less than 1% b) Factor level of 1% to 5% c) Factor level of 5% to 50% d) Factor level greater than 50%
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.
Which of the following would the nurse be least likely to assess in a child with a hematologic disorder? a) Anemia b) Fever c) Abnormal hemostasis d) Neutropenia
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.
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 of the following lab tests: a) Peripheral blood smear b) Hemoglobin electrophoresis c) Erythrocyte sedimentation rate d) Reticulocyte count
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).
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? a) Poikilocytosis b) Purpura c) Ecchymosis d) Petechiae
Petechiae Explanation: 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.
In hemophilia A, the classic form, only females manifest a bleeding disorder. a) False b) True
a. False Explanation: 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.
The nurse is caring for a 10-year-old boy with hemophilia. He asks the nurse for suggestions about appropriate physical activities. Which activity would the nurse most likely recommend? a) Wrestling b) Football c) Soccer d) Basebal
d. Baseball Explanation: Children with hemophilia should stay active. Good physical activities would be swimming, baseball, basketball, and bicycling (with a helmet). He would still need to be careful about falls and sliding into base. Intense contact sports like football, wrestling, and soccer should be avoided
To prevent further sickle cell crisis, you would advise the parents of a child with sickle cell anemia to a) encourage the child to participate in school activities, such as long-distance running. b) administer an iron supplement daily. c) prevent the child from drinking an excess amount of fluids per day. d) notify a health care provider if the child develops an upper respiratory infection.
d. notify a health care provider if the child develops an upper respiratory infection. Explanation: Reduction of oxygen and dehydration lead to increased sickling of cells. Early prevention of these with respiratory illness is important
The nurse is providing family education for the prevention or early recognition of vaso-occlusive events in sickle cell anemia. Which response by a family member indicates a need for further teaching? a) "We must be compliant with vaccinations and prophylactic penicillin." b) "We must watch for unusual headache, loss of feeling, or sudden weakness." c) "We should call the doctor for any fever over 100°F." d) "We need to seek medical attention for abdominal pain."
"We should call the doctor for any fever over 100°F." Explanation: The nurse must emphasize that ANY febrile illness requires immediate attention. Fever causes dehydration, which can trigger problems in a child with sickle cell anemia. Seeking medical attention for abdominal pain; watching for unusual headache, loss of feeling, or sudden weakness; and compliance with vaccinations are appropriate.
The nurse is providing family education for the prevention or early recognition of vaso-occlusive events in sickle cell anemia. Which response by a family member indicates a need for further teaching? a) "We must be compliant with vaccinations and prophylactic penicillin." b) "We need to seek medical attention for abdominal pain." c) "We must watch for unusual headache, loss of feeling, or sudden weakness." d) "We should call the doctor for any fever over 100°F."
"We should call the doctor for any fever over 100°F." Explanation: The nurse must emphasize that ANY febrile illness requires immediate attention. Fever causes dehydration, which can trigger problems in a child with sickle cell anemia. Seeking medical attention for abdominal pain; watching for unusual headache, loss of feeling, or sudden weakness; and compliance with vaccinations are appropriate.
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? a) "Will you help me apply this band-aid?" b) "Please be brave; we need to stop the bleeding" c) "Would you help me dilute this and mix it up?" d) "Would you like to administer the infusion?"
"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 boy with hemophilia A is scheduled for surgery. Which of the following precautions would you institute with him? a) Do not allow a dressing to be applied postoperatively. b) Caution him not to brush his teeth before surgery. c) Handle him gently when transferring him to a stretcher. d) Mark his chart for him to receive no analgesia
Handle him gently when transferring him to a stretcher. Explanation: Gentle handling can reduce bruising. Analgesia will be needed postoperatively; IM injections are contraindicated because of potential bleeding.
When developing the postoperative plan of care for a child with sickle cell anemia who has undergone a splenectomy, which of the following would the nurse identify as the priority? a) Impaired skin integrity b) Risk for delayed growth and development c) Risk for infection d) Deficient fluid volume
Risk for infection Explanation: Removal of the spleen places the child at significant risk for infection. Although the child's skin integrity is disrupted due to the surgery, this is not the priority nursing diagnosis. Loss of fluids occurs during surgery and adequate hydration is important to prevent a sickle cell crisis, but this diagnosis is not the priority in the postoperative period. Although the child is at risk for delayed growth and development, the priority postoperatively is to prevent infection
In caring for a child with sickle cell disease, the highest priority goal is which of the following? a) The family caregivers' anxiety will be reduced. b) The child's skin integrity will be maintained. c) The family will verbalize understanding of of the disease crisis. d) The child's fluid intake will improve
The child's fluid intake will improve. Explanation: 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.
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: a) Hemophilia b) von Willebrand disease c) Chronic iron deficiency anemia d) Disseminated intravascular coagulation
b. von Willebrand disease Explanation: 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.
The nurse is caring for a 2-year-old with sickle cell anemia and describing the acute and chronic manifestations of sickle cell anemia to his mother. Which statement by the mother indicates a need for further teaching? a) "Aplastic crisis is a life-threatening acute manifestation of sickle cell anemia." b) "Delayed growth and development and delayed puberty are chronic manifestations." c) "The acute manifestations, like splenic sequestration, are most often life-threatening." d) "Bone infarction, dactylitis, and recurrent pain episodes are acute manifestations.
"The acute manifestations, like splenic sequestration, are most often life-threatening." Splenic sequestration is a life-threatening acute manifestation of sickle cell anemia, but some of the chronic manifestations of the disease, such as pulmonary hypertension and restrictive lung disease, are also often life-threatening. Aplastic crisis is a life-threatening acute manifestation. Bone infarction, dactylitis, and recurrent pain episodes are acute manifestations; delayed growth and development and chronic puberty are chronic manifestations.
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? a) Priapism b) Leg ulcers c) Behavioral addiction d) Seizures
Seizures Explanation: 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.
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? a) Swimming b) Gymnastics c) Rugby d) Soccer
Swimming Explanation: 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.
In von Willebrand's disease, girls exhibit unusually heavy menstrual flow. a) False b) True
b. True Explanation: 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.
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? a) "The disease is most often seen in individuals of Asian decent." b) "The trait or the disease is seen in one generation and skips the next generation." c) "If the trait is inherited from both parents the child will have the disease." d) "Males are much more likely to have the disease than females."
c. "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 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? a) "She loves popsicles, so I'll let her have them as a snack or for dessert." b) "I put her legs up on pillows when her knees start to hurt." c) "She has been down, but playing in soccer camp will cheer her up." d) "I bought the medication to give to her when she complains of pain."
c. "She has been down, but playing in soccer camp will cheer her up." Explanation: 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.
Choice Multiple question - Select all answer choices that apply. 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. a) Promoting exercise and activity b) Administering analgesics c) Administering oxygen d) Maintaining fluid intake e) Preventing injury and bleeding episodes
• 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.
