Chapter 25: Hematologic Disorders

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. Notify the health care provider that the parents still have questions. Answer the parents' questions as completely as possible. Encourage the parents to focus their attention on their child.

Answer the parents' questions as completely as possible.

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? Ask the adolescent if the teaching was understood. Provide written materials to reinforce teaching. Request that the adolescent teach the information to the nurse. Provide an opportunity for the adolescent to ask questions.

Request that the adolescent teach the information to the nurse.

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: platelets. plasmin. factor IX. factor VIII.

factor VIII.

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? Elevate the injured area such as a leg or arm. Apply heat to the site of bleeding. Administer factor VIII replacement. Apply direct pressure to the area.

Apply heat to the site of bleeding.

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? Ask the parent if he or she has questions about the plan of care. Provide diversional activities for the child. Contact the health care provider to meet with the parent. Implement strategies to address the child's pain.

Implement strategies to address the child's pain.

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? medication administration occupational therapy home care safety leukopenia precautions

OT

When developing the postoperative plan of care for a child with sickle cell anemia who has undergone a splenectomy, which would the nurse identify as the priority? Impaired skin integrity Risk for delayed growth and development Deficient fluid volume Risk for infection

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.

The nurse is caring for a 2-year-old child with an absolute neutrophil count below 500 cells/mm3 (0.50 ×109/l). What should the nurse include in the child's plan of care? Select all that apply. Avoid vigorous friction when cleansing the skin. Encourage raw fruits and vegetables in the diet. Restrict visitors with known upper respiratory infections. Instruct the parents to have the child wear a mask in public and avoid crowds. Use chlorhexidine preparation on intravenous ports.

Use chlorhexidine preparation on intravenous ports. Restrict visitors with known upper respiratory infections. Instruct the parents to have the child wear a mask in public and avoid crowds.

To prevent further sickle cell crisis, the nurse would advise the parents of a child with sickle cell anemia to: encourage the child to participate in school activities, such as long-distance running. administer an iron supplement daily. prevent the child from drinking an excess amount of fluids per day. notify a health care provider if the child develops an upper respiratory infection.

notify a health care provider if the child develops an upper respiratory infection.

The nurse is performing pain management interventions for an 8-year-old child with sickle cell disease who is in a vaso-occlusive crisis. What will the nurse include in the plan of care? Select all that apply. patient-controlled analgesia (PCA) as prescribed nonsteroidal anti-inflammatory drugs (NSAIDs) meperidine warm compresses to the affected area restricting fluid intake

patient-controlled analgesia (PCA) as prescribed warm compresses to the affected area nonsteroidal anti-inflammatory drugs (NSAIDs)

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: leg ulcers. behavioral addiction. seizures. priapism.

seizures.

The nurse is caring for a 4-year-old with sickle cell anemia. A physical finding the nurse might expect to see in him is: slightly yellow sclerae. depigmented areas on the abdomen. enlarged mandibular growth. increased growth of long bones.

slightly yellow sclerae.Explanation:Many children with sickle cell anemia develop mild scleral yellowing from excess bilirubin from breakdown of damaged cells.

The nurse is assessing a 3-year-old child's tissue perfusion. Which assessment finding(s) indicates adequate tissue perfusion in the client? Select all that apply. cyanosis absent capillary refill 2 seconds urine output 2.2 ml/kg/hr warm skin hemoglobin (Hgb) 8.6 g/dl (86 g/l)A

warm skin cyanosis absent capillary refill 2 seconds urine output 2.2 ml/kg/hr

The nurse is caring for a child with thalassemia who is receiving chelation therapy at home using a battery-operated pump. After teaching the parents about this treatment, which statement by the mother indicates a need for additional teaching? "I must be very careful to strictly adhere to the chelation regimen." "The medication can be administered while my child is sleeping." "I can have the nurse administer the chelation therapy if I am uncomfortable." "The deferoxamine binds to the iron so it can be removed from the body."

"I can have the nurse administer the chelation therapy if I am uncomfortable."

A couple is expecting a child. The fetus undergoes genetic testing and the couple discover the fetus has sickle cell disease. The couple ask the nurse how most commonly happens. Which statement is accurate for the nurse to provide? "Sickle cell disease occurs from a random genetic mutation." "Sickle cell diseas can be passed to the fetus in many ways. We will know more at birth." "Sickle cell disease is passed to a fetus when both parents have the gene." "Sickle cell disease is passed to a fetus when one of the parents has the gene."

"Sickle cell disease is passed to a fetus when both parents have the gene."

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? "Take your child to the emergency department now." "What makes you think your child is in crisis?" "Call 911 and give the child some water while you wait." "Tell me about the symptoms your child is experiencing"

"Tell me about the symptoms your child is experiencing"

A nurse is caring for a child diagnosed with severe hemophilia A. The child is to be treated with an on-demand factor VIII infusion of 50 units/kg every 8 hours for a muscle hemorrhage following a quadriceps injury. The child weighs 30 lb (13.61 kg). Calculate the number units of factor VIII the nurse will infuse every 8 hours. Record your answer using a whole number.

681 Explanation: Severe large muscle hemorrhages such as the quadriceps, require 50 units/kg on-demand factor VIII infusion every 8, 12, or 24 hours. To calculate the number of units required, use the child's weight in kilograms and multiply by the number of units/kg prescribed. 13.61 kg x 50 units/kg = 680.5 units. Rounding to the nearest whole number, the nurse will infuse 681 units every 8 hours.

The nurse is assessing a 4-year-old child with sickle cell disease. For which complication(s) of the client's condition will the nurse assess? Select all that apply. hemochromatosis dactylitis silent cerebral ischemia maxillary prominence acute chest syndrome

dactylitis silent cerebral ischemia acute chest syndrome Explanation: Vaso-occlusive crisis can affect several areas of the body, leading to complications including dactylitis, silent cerebral ischemia, acute chest syndrome, and other conditions. Hemochromatosis and maxillary prominence are caused by beta-thalassemia.

A school-aged child is admitted to the hospital with a vaso-occlusive sickle cell crisis. Which measure in the child's care plan should be given priority? Seeing that the child ingests a protein-rich diet Maintaining fluids through an intravenous line Encouraging the child to take deep breaths hourly Beginning active range-of-motion exercises

Maintaining fluids through an intravenous line Explanation: Sickle cells clump together and prevent normal blood flow. This leads to tissue hypoxia. With a vaso-occlusive crisis, the cells are clumped together and prevent blood flow to the joint or organ. The blood with the clumped sickled cells is very viscous. Adequate hydration is crucial in relieving the problems of a vaso-occlusive crisis. The hydration dilutes the blood and decreases the viscosity. During a crisis the recommended fluid intake (IV and PO) is 150 ml/kg/day. During a vaso-occlusive crisis, the child has severe pain. The goal is to get the pain under control and increase blood flow. Range-of-motion exercises will add to the increased pain during this period of time, so should not be started until crisis in under control. The diet and hourly deep breaths are important, but they are not crucial to correcting the crisis.

A nurse is conducting a class to a group of parents on sickle cell anemia. Which statement by a parent indicates teaching has been effective? "Sickle cell anemia is common in people of Asian descent." "Fluid restriction is necessary to control sickle cell anemia." "The sickle shape of red blood cells decreases oxygen to tissues." "This is a hereditary disease that is transmitted by one affected gene."

"The sickle shape of red blood cells decreases oxygen to tissues." Explanation: The sickle shape of the red blood cells impedes the flow of blood through the vessels, thus causing hypoxia to the tissues. Sickle cell anemia is a hereditary disease but it is autosomal recessive, meaning it requires two genes in order for the disease to be transmitted. Sickle cell anemia is common in people of African, Mediterranean, and Indian descent. Hydration is important to controlling sickle cell anemia. Dehydration is a trigger for 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? "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 values will help us monitor the disease." "These labs are just common labs for children with this disease."

"These values will help us monitor the disease."

A nurse is administering a blood transfusion to a child diagnosed with a hematologic disorder. Fifteen minutes into the transfusion, the child reports severe headache, nausea, and low back pain. There is no evidence of urticaria and vital signs are unchanged from the baseline. What action should the nurse take next? Discontinue the transfusion. Call the health care provider. Administer acetaminophen and antiemetic. Decrease the transfusion rate.

Discontinue the transfusion. Explanation: In the presence of any signs of a transfusion reaction, which includes severe headache, nausea, and low back pain, the next step the nurse should take is to discontinue the transfusion. Calling the health care provider is appropriate but is not the action the nurse should take next. The nurse may need to administer medications to counteract the headache, nausea, and low back pain, but this is not the next action to be taken. The nurse should discontinue the transfusion, not decrease the infusion rate.

The nurse is caring for a client who was diagnosed with a sickle cell crisis and currently reports acute back and joint pain. Upon examination, the nurse noted the following assessments: dry mucous membranes; poor skin turgor; poor capillary refill, and pale nail beds. Which nursing concern will the nurse identify as the priority? acute pain related to effects of sickling altered skin integrity risk related to decreased mobility secondary to pain fluid volume underload related to clustering of sickled cells peripheral tissue perfusion impairment related to the effects of sickled cells

acute pain related to effects of sickling

A nurse caring for an 8-year-old child with a bleeding disorder documents the following nursing diagnosis: Ineffective tissue perfusion related to intravascular thrombosis and hemorrhage. This nursing diagnosis would be most appropriate for a child diagnosed with which condition? iron-deficiency anemia disseminated intravascular coagulation von Willebrand disease hemophilia

disseminated intravascular coagulation

A nurse is providing preoperative care to a child with sickle cell disease. What treatment should the nurse expect to implement prior to surgery? transfusion of packed red blood cells (PRBCs) transfusion of fresh frozen plasma (FFP) deferoxamine administration desmopressin administration

transfusion of packed red blood cells (PRBCs)

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? "We need to wait for your child to express the pain level to us before providing medication." "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." "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 nurse in the emergency department is caring for a 10-year-old female child with sickle cell crisis. Child rates pain 10 on a scale of 0 to 10. Vital signs: 99.8°F (37.6°C); heart rate, 122 beats/min; blood pressure, 92/50 mm Hg; respiratory rate, 26 breaths/min; oxygen saturation, 92% on room air. The nurse receives orders for the child. Click to highlight the order(s) that needs to be implemented immediately. Orders: Administer acetaminophen for headache or temperature greater than 101°F (38.3°C). Administer oxygen to maintain oxygen saturation greater than 95%. Start normal saline continuous intravenous (IV) infusion at 200 ml/hr. Administer 100 mcg/kg morphine IV for pain prn q4 hours. Initiate a regular diet as tolerated.

Administer oxygen to maintain oxygen saturation greater than 95%. Start normal saline continuous intravenous (IV) infusion at 200 ml/hr. Administer 100 mcg/kg morphine IV for pain prn q4 hours

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 Factor XIII Factor V Factor X

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 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? Make time for questions at the end of the teaching session. Give cues as needed while the parent sets up the infusion. Observe the parent set up and administer the infusion. Ask the parent to repeat the instructions step-by-step.

Observe the parent set up and administer the infusion.

The nurse is preparing to administer packed red blood cells (RBCs) to a child with beta-thalassemia. Which action(s) should the nurse take? Select all that apply. Inform the parents that nausea is an expected side effect that will resolve when the transfusion is completed. Use an infusion pump to maintain the infusion rate. Connect the packed RBCs to an infusion set that has already been primed with normal saline. Verify the packed RBCs against the child's blood type. Retrieve the packed RBCs from the unit refrigerator 60 minutes prior to starting the transfusion.

Verify the packed RBCs against the child's blood type. Use an infusion pump to maintain the infusion rate. Connect the packed RBCs to an infusion set that has already been primed with normal saline.