Chapter 25 Hematologic Disorders Adaptive Quizzing
A parent has just learned that the infant has Fanconi anemia. The parent tells the nurse "I just do not understand how this could happen." How will the nurse respond? "Fanconi anemia is an acquired disorder that can be caused by certain medications." "It is unfortunate that we really do not understand the cause of this disorder." "This is an autosomal recessive genetic disorder that causes aplastic anemia and other congenital disorders." "This disorder is the result of an autoimmune process that affects the production of the blood's cells."
"This is an autosomal recessive genetic disorder that causes aplastic anemia and other congenital disorders."
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." "The doctor will discuss these findings with you when he comes to the hospital." "I'm really not allowed to discuss these findings with you." "These labs are just common labs for children with this disease."
"These values will help us monitor the disease."
The nurse is caring for a 13-year-old girl with von Willebrand disease. After teaching the adolescent and her parents about this disorder and care, which response by the parents indicates a need for additional teaching? "We should be aware that she may suffer from menorrhagia." "We understand that she may have frequent nosebleeds." "We need to administer Stimate (desmopressin) prior to dental work." "We should administer desmopressin as often as needed."
"We should administer desmopressin as often as needed." Explanation: The parents need to know that desmopressin spray Stimate is used for controlling bleeding; the other brands are used for homeostasis and enuresis. Additionally, Stimate should only be used for 3 days in a row as lessening of the response (tachyphylaxis) occurs with frequent use. Stimate should be used before dental work. Menorrhagia and nosebleeds may occur.
The nurse is caring for a client who is in a sickle cell crisis. The child is hospitalized for pain management during the crisis. The parents tell the nurse that they do not think their child needs any pain medication because the child is sleeping a lot. How should the nurse respond? "I agree. Since your child is sleeping the pain must not be too severe. I will hold his pain medication." "I understand why you think your child is not in pain; sleep is often a way for children to cope with pain." "We need to wait for your child to express the pain level to us before providing medication." "The pain medication is prescribed on a routine basis to keep the pain under control, so I have to give it as prescribed."
"I understand why you think your child is not in pain; sleep is often a way for children to cope with pain."
The parents of a 2-month-old infant have learned that their infant has hemophilia. The parents are visibly upset and ask how this could have happened to them. What is the nurse's best response? "Please do not be upset; it is not your fault. Things like this happen sometimes." "News like this is difficult to hear. Let's talk about what this means for your child." "I understand how you feel. Let's talk about where you go from here." "There is no need to worry. We will teach you how to take care of your child."
"News like this is difficult to hear. Let's talk about what this means for your child."
A nurse is providing teaching on safety to a group of parents whose children are diagnosed with hemophilia. Which statement made by a parent requires follow-up by the nurse? "We make sure our toddler wears a helmet and knee pads." "We had a trampoline but got rid of it after our child was diagnosed." "Our child always wears a helmet and body padding when playing football." "Our child has a medical alert bracelet that is worn at all times."
"Our child always wears a helmet and body padding when playing football."
A nurse is providing teaching to the parents of a child diagnosed with sickle cell anemia. The discussion is focused on precipitating factors for sickle cell crisis. Which statement by the parents requires the nurse to reinforce the teaching? "I make sure our child is up to date on all immunizations." "I make sure my child wears a good warm coat and gloves during winter." "We always take water along when we are on an outing." "Our family is taking a fun hiking trip up in the mountains next week."
"Our family is taking a fun hiking trip up in the mountains next week." Explanation: High altitudes are a contributing factor for sickle cell crisis and should be avoided, as should flights in planes that are not pressurized.
The nurse is preparing a child for discharge following a sickle cell crisis. Which statement by the mother indicates a need for further teaching? "I put her legs up on pillows when her knees start to hurt." "She loves popsicles, so I'll let her have them as a snack or for dessert." "She has been down, but playing in soccer camp will cheer her up." "I bought the medication to give to her when she says she is in pain."
"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.
A parent calls the pediatric clinic and tells the nurse "I think my child is having a sickle cell crisis. Should I bring the child to the office?" What is the nurse's best response? "Call 911 and give the child some water while you wait." "Tell me about the symptoms your child is experiencing" "What makes you think your child is in crisis?" "Take your child to the emergency department now."
"Tell me about the symptoms your child is experiencing"
The parent asks the nurse, "I thought the bleeding was stopped. What is causing this pain?" Based on the note (above), how should the nurse reply? "This is a positive Homan sign, which can occur as the bleed begins to heal." "Immobilization of the shoulder has resulted in a frozen joint." "The leftover blood in the shoulder is causing hemarthrosis." "Telangiectasias have formed in the shoulder due to repeated bleeding episodes."
"The leftover blood in the shoulder is causing hemarthrosis." Explanation: The child is exhibiting signs of hemarthrosis, which can occur after the bleeding is controlled in a joint injury, resulting in swelling and pain with limited movement.
A nurse is caring for a 7-year-old child with hemophilia who requires an infusion of factor VIII. The child is fearful about the process and is resisting treatment. How should the nurse respond? "Will you help me apply this adhesive bandage?" "Please be brave; we need to stop the bleeding." "Would you help me dilute this and mix it up?" "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 child is to use distraction and involve the child in the infusion process in a developmentally appropriate manner. A 7-year-old child is old enough to assist with the dilution and mixing of the factor
A child is hospitalized with complications related to hemophilia. The health care provider has discussed the child's plan of care with the parents, but they continue to ask questions. What action will the nurse take? Reassure the parents that they have been fully briefed on their child's treatment. Answer the parents' questions as completely as possible. Encourage the parents to focus their attention on their child. Notify the health care provider that the parents still have questions.
Answer the parents' questions as completely as possible.
A nurse is preparing a teaching plan for a child with hemophilia and his parents. Which information would the nurse be least likely to include to manage a bleeding episode? Apply direct pressure to the area. Elevate the injured area such as a leg or arm. Administer factor VIII replacement. Apply heat to the site of bleeding.
Apply heat to the site of bleeding. Explanation: Ice or cold compresses, not heat, would be applied to the site of bleeding
The nurse is caring for a child who had a stem cell transplant and is being monitored for engraftment. Which nursing action is priority? Remind parents to contact the child's school. Monitor daily complete blood count (CBC). Ensure neutropenic precautions are in place. Encourage therapeutic play activities.
Ensure neutropenic precautions are in place. Explanation: With stem cell transplants, children are at greatest risk for infection and sepsis. The nurse should ensure neutropenic precautions are used to reduce the change of infection
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 blood factor?
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.
A child with hemophilia A is scheduled for surgery. Which precautions would the nurse institute with this client? Do not allow a dressing to be applied postoperatively. Caution the child not to brush the teeth before surgery. Handle the child gently when transferring to a stretcher. Mark the client's chart to receive no analgesia.
Handle the child gently when transferring to a stretcher.
A nurse has created a plan of care for a hospitalized child receiving treatment for beta-thalassemia. Which client goal should the nurse prioritize? Prescribed medications are well tolerated. Adequate renal function is maintained. Hemoglobin level of 9 g/dl (90 g/l) or higher is achieved. The child engages in developmentally appropriate activities.
Hemoglobin level of 9 g/dl (90 g/l) or higher is achieved. Explanation: The priority goal for this plan of care is a hemoglobin level between 9 and 10 g/dl (90 to 100 g/l). This will ensure adequate tissue perfusion and oxygenation, which will also ensure the child has adequate renal function
The nurse is caring for a 12-year-old boy with sickle-cell disease. During a routine wellness exam, his mother tells the nurse that the boy is becoming rebellious. He responds by telling the nurse that it's because "she never lets me out of her sight!" What would be the best intervention for this family? Advise the mother that she needs to give him freedom. Encourage them to join a support group. Remind him that he needs to take responsibility for his health. Tell them about a camp for children with sickle-cell disease and their parents.
Tell them about a camp for children with sickle-cell disease and their parents.
In caring for a child with sickle cell disease, the highest priority goal is: The child's fluid intake will improve. the caregiver's anxiety will be reduced. the family will verbalize understanding of the disease crisis. the child's skin integrity will be maintained.
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.
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: plasmin. factor VIII. factor IX. platelets.
factor VIII. Explanation: In hemophilia A, the problem is with factor VIII, and in hemophilia B the problem lies with factor IX.
The nurse is caring for a 5-year-old client with sickle cell disease who is receiving hydroxyurea therapy. What should the nurse monitor while caring for the client? Select all that apply. hemoglobin (Hgb) F levels serum ferritin vision and hearing complete blood count (CBC), reticulocyte count, and platelet count growth
hemoglobin (Hgb) F levels complete blood count (CBC), reticulocyte count, and platelet count Explanation: One of the therapeutic effects of hydroxyurea is increasing the level of fetal Hbg; therefore, Hgb F levels should be monitored. Hydroxyurea may also have an effect on the CBC, reticulocyte count, and platelet count, so these results should be monitored as well. Growth, vision and hearing, and serum ferritin should be monitored during deferoxamine therapy, not hydroxyurea therapy.
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: seizures. priapism. leg ulcers. behavioral addiction.
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.
A nurse is providing preoperative care to a child with sickle cell disease. What treatment should the nurse expect to implement prior to surgery? desmopressin administration deferoxamine administration transfusion of packed red blood cells (PRBCs) transfusion of fresh frozen plasma (FFP)
transfusion of packed red blood cells (PRBCs) Explanation: Before surgical procedures, which may lower the oxygen tension, a transfusion of PRBCs is administered with the aim of having a higher concentration of nonsickling vs. sickled red blood cells.
The nurse is teaching an in-service 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? "The disease is most often seen in individuals of Asian decent." "Males are much more likely to have the disease than females." "The trait or the disease is seen in one generation and skips the next generation." "If the trait is inherited from both parents the child will have the disease."
"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 nurse is caring for a child with hemophilia. The parents are upset by the possibility that the child will become infected with hepatitis from the clotting factor replacement therapy. Which response by the nurse would be appropriate? "There are risks with any treatment including using blood products, but these are very minor." "Although factor replacement is expensive, there's more financial strain from missing work if your child has a bleeding episode." "The use of dry heat sterilization has proven to inactivate hepatitis virus, resulting to zero factors transfusion-related incidence of hepatitis infection." "Parents commonly fear the worst; however, the factor will help your child lead a normal life."
"The use of dry heat sterilization has proven to inactivate hepatitis virus, resulting to zero factors transfusion-related incidence of hepatitis infection." Explanation: The nurse needs to emphasize that since the inception of heat treatment of the factor in 1986, there have been no reports of virus transmission from factor infusion.
An 11-year-old child is being prepared for discharge after experiencing a vasoocclusive crisis secondary to sickle cell disease. The child has been prescribed hydroxyurea. After teaching the child and parents about this medication, the nurse determines that the teaching was successful when the parents identify that they will notify the health care provider about which condition? Headache Constipation Infection Gastric upset
Infection Explanation: The adverse effect of hydroxyurea is infection. The drug can cause neutropenia. Therefore, the parents should report signs and symptoms of infection to the health care provider as soon as possible.
The nurse is teaching the family of a child with sickle cell disease how to avoid triggers of hypoxia. What instruction(s) will the nurse include in the teaching? Select all that apply. Seek medical attention promptly for a fever greater than 102°F (38.9°C). Have your child wash hands frequently. Give a dose of vitamin C before administering therapy for sickle cell disease. Avoid high altitudes. Instruct your child to avoid touching the mouth, nose, and eyes.
Avoid high altitudes. Have your child wash hands frequently. Instruct your child to avoid touching the mouth, nose, and eyes.
The nurse is caring for a child with sickle-cell anemia admitted to the pediatric unit. The child reports severe pain and fever. The nurse notes the following laboratory values: white blood cells 18,000/mm3, hemoglobin 6.6 mg/dl (66 g/L), and bilirubin 8 mg/dl (136.83 µmol/L). Which nursing action is priority? Initiate intravenous access. Administer pain medication. Assess the child's temperature. Begin an exchange transfusion.
Initiate intravenous access. Explanation: In a situation where the child is experiencing a sickle cell crisis, a priority nursing action is to initiate intravenous access to begin rehydrating the child to halt the sickling process.
The nurse is caring for a child who has been admitted for a sickle cell crisis. What would the nurse do first to provide adequate pain management? Administer a nonsteroidal anti-inflammatory drug (NSAID) as ordered. Initiate pain assessment with a standardized pain scale. Administer meperidine as ordered. Use guided imagery and therapeutic touch.
Initiate pain assessment with a standardized pain scale. Explanation: The nurse should first initiate pain assessment with a standardized pain scale upon admission and provide frequent evaluations of pain
A nurse is providing care to a child hospitalized for sickle cell anemia. The child begins to exhibit abdominal distension and signs of shock, but reports no pain. For which complication should the nurse assess first? gallstones dactylitis asplenia enuresis
asplenia Explanation: Abdominal distension in a child diagnosed with sickle cell anemia could indicate the development of acute splenic sequestration resulting from the sudden absorption of a vast volume of blood. This is considered a medical emergency requiring a splenectomy; therefore, this is the complication for which the nurse should assess first
Question 4 of 10 A 3-year-old child is hospitalized with a diagnosis of sickle cell anemia and is experiencing a pain crisis. Using the FACES scale, the nurse assesses the child's pain to be a 10 on a scale of 1 to 10. The child is receiving intravenous fluids and oxygen at 2 L/min via nasal cannula. The parent is at the bedside holding the child's hand and has a concerned look. What is the nurse's priority in caring for the child? Provide diversional activities for the child. Contact the health care provider to meet with the parent. Ask the parent if he or she has questions about the plan of care. Implement strategies to address the child's pain. Exit quiz
Implement strategies to address the child's pain.
The nurse is caring for a 5-year-old child with sickle cell disease. What will the nurse include in the client's plan of care? Select all that apply. Maintain oxygen saturation of 93% or higher. Assess for maxillary prominence. Maintain fluid intake at 1,600 ml/m2. Administer deferasirox as prescribed. Monitor for signs and symptoms of infection.
Maintain oxygen saturation of 93% or higher. Monitor for signs and symptoms of infection. Maintain fluid intake at 1,600 ml/m2. Explanation: Maintaining oxygen saturation at 93% or higher, monitoring for signs and symptoms of infection, and maintaining fluid intake at 1,600 ml/m2 will all be included in the plan of care for the child, because these are all measures that the nurse should take to prevent vaso-occlusive crisis.
A nurse is preparing to administer a blood transfusion to a child diagnosed with beta-thalassemia. What action should the nurse take first? Take the child's vital signs. Prime the infusion tubing. Set the infusion pump to the appropriate transfusion rate. Match the unit of blood to the child's identification.
Match the unit of blood to the child's identification. Explanation: In preparing to administer a blood transfusion, the nurse should first match the unit of blood to the child's identification. This is done by two nurses to ensure the child's safety. Once identification is verified, the infusion tubing can be primed and the appropriate transfusion rate set on the infusion pump. Right before the infusion is started, the nurse should assess the child and take a baseline set of vital signs.
A home care nurse is teaching a parent how to administer a clotting factor infusion to their child. How can the nurse best evaluate the effectiveness of the teaching? Ask the parent to repeat the instructions step-by-step. Give cues as needed while the parent sets up the infusion. Observe the parent set up and administer the infusion. Make time for questions at the end of the teaching session.
Observe the parent set up and administer the infusion.
A child diagnosed with hemophilia presents with warm, swollen, painful joints. Which action will the nurse take first? Notify the client's primary health care provider Document the presence of hemarthrosis in the client's chart Assess the client's urine and stool for blood Prepare to administer factor replacement medication
Prepare to administer factor replacement medication Explanation: Many clients with hemophilia have repeated episodes of hemarthrosis or bleeding into the joints, and develop functional impairment of the joints, despite careful treatment. To assist in limiting impairment, the nurse would prepare to administer factor replacement medications, such as plasma, recombinant clotting factor VIII, or a clotting promotor medication.
A nurse is providing teaching on the medication regimen for beta-thalassemia to an adolescent. What is the best way for the nurse to determine if the teaching was successful? Request that the adolescent teach the information to the nurse. Provide written materials to reinforce teaching. Ask the adolescent if the teaching was understood. Provide an opportunity for the adolescent to ask questions.
Request that the adolescent teach the information to the nurse.
The nurse has received morning report on a group of pediatric clients. Which pediatric client will the nurse see first? a child with hemophilia reporting knee pain and edema a child with sickle cell anemia requesting a cool compress a child experiencing a palpable purpural rash and arthralgia a child reporting lethargy with a history of thalassemia major
a child with hemophilia reporting knee pain and edema Explanation: The child with hemophilia should be quickly evaluated when reporting joint pain as this could indicate bleeding.
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? reticulocyte count erythrocyte sedimentation rate hemoglobin electrophoresis peripheral blood smear
hemoglobin electrophoresis Explanation: If the screening test result indicates the possibility of SCA or sickle cell trait, hemoglobin (Hgb) electrophoresis is performed promptly to confirm the diagnosis.
A nurse is preparing a discharge plan for a child diagnosed with Fanconi anemia who has associated congenital defects. What aspect of the plan should the nurse include to address the child's development of orthopedic function? home care safety occupational therapy medication administration leukopenia precautions
occupational therapy Explanation: Occupational therapy will address the growth and development of orthopedic function for a child diagnosed with Fanconi anemia who also has congenital defects
A couple is expecting a child. The fetus undergoes genetic testing and the couple discover the fetus has sickle cell anemia. The couple ask the nurse how this happened. Which statement is accurate for the nurse to provide? "Sickle cell anemia can be passed to the fetus in many ways. We will know more at birth." "Sickle cell anemia is passed to a fetus when one of the parents has the gene." "Sickle cell anemia occurs from a random genetic mutation." "Sickle cell anemia is passed to a fetus when both parents have the gene."
"Sickle cell anemia is passed to a fetus when both parents have the gene." Explanation: Sickle cell anemia is an inherited disease. The recessive gene is passed from both parents who either have the disease or the trait
A nurse is providing care to a child hospitalized with a hematologic disorder. Which nursing intervention(s) will provide support for the family? Select all that apply. Include the family in client care conferences. Tell the family that there is no need to worry about the child. Create opportunities for the family to participate in the child's care. Provide information on community resources related to the child's disorder. Ask the family to leave the child's room during treatments.
Include the family in client care conferences. Provide information on community resources related to the child's disorder. Create opportunities for the family to participate in the child's care.