PREPU hematologic disorders

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.

A nurse is providing care to a child who is receiving desmopressin. At the end of a 12-hour shift, the nurse completes an assessment (above). Which finding requires the nurse's intervention?

urine output

The nurse is teaching an in-service program to a group of nurses on the topic of children diagnosed with sickle cell disease. The nurses in the group make the following statements. Which statement is most accurate regarding this condition?

"If the trait is inherited from both parents the child will have the disease."

A child with sickle cell anemia is scheduled for a splenectomy. After the parents receive teaching about the rationale for this surgery, the nurse determines that the teaching was successful when the parents make which statement?

"It will help to decrease the amount of anemia."

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?

"News like this is difficult to hear. Let's talk about what this means for your child."

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 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.

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.

The nurse is working with a child who is in sickle cell crisis. Treatment and nursing care for this child include which actions? Select all that apply.

Administering oxygen Administering analgesics Maintaining fluid intake

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 heat 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?

Ensure neutropenic precautions are in place.

A child with hemophilia A is scheduled for surgery. Which precautions would the nurse institute with this client?

Handle the child gently when transferring to a stretcher.

A nurse is providing care for a child diagnosed with beta-thalassemia who is receiving a blood transfusion. The child reports being bored and asks to go to the playroom. What is the best action for the nurse to take?

Have a child-life specialist find an appropriate activity to occupy the child during the transfusion.

A nurse has created a plan of care for a hospitalized child receiving treatment for beta-thalassemia. Which client goal should the nurse prioritize?

Hemoglobin level of 9 g/dl (90 g/l) or higher is achieved.

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?

Implement strategies to address the child's pain.

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?

"Our child always wears a helmet and body padding when playing football."

A nurse is reviewing the above laboratory results for a 6-year-old child during a pediatric clinic visit. Based on the laboratory results, what question is most appropriate for the nurse to ask the parents?

"What has your child's activity level been like recently?"

Based upon a client's progress note (above), what information will the nurse include in the discharge teaching? Select all that apply.

Maintain adequate hydration. Ensure the ability to obtain medications. Expect to return for follow-up laboratory tests.

A nurse is providing care for a child diagnosed with beta-thalassemia who is to receive a blood transfusion. One unit of packed red blood cells (250 ml) is prescribed at a transfusion rate of 5 ml/kg/hour. The child weighs 11.4 kg. Calculate the rate the nurse will set the infusion pump. Record your answer using a whole number.

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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?

Answer the parents' questions as completely as possible.

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.

A 15-year-old client diagnosed with von Willebrand disease has reached menarche. Based on this fact, what information is most important for the nurse to convey to the client?

Expect menstrual bleeding to be heavy.

A 6-month-old infant diagnosed with hemophilia B presents to the pediatric clinic for routine immunizations. What intervention will the nurse implement for this infant?

Prepare to administer the scheduled vaccines subcutaneously.

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?

disseminated intravascular coagulation

The nurse is caring for a 5-year-old child with a hematologic disorder receiving supplemental oxygen. The child's oxygen saturation level is 94% (0.94). Which action should the nurse take?

Monitor the child's oxygen saturation.

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?

Observe the parent set up and administer the infusion.

The nurse is developing a plan of care for a child who is to have a transfusion. Which would the nurse expect to administer because it is the most common form of transfusion?

Packed red blood cells

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?

Risk for infection

When caring for a 7-year-old client diagnosed with sickle cell anemia, which clinical manifestation will the nurse report to the health care provider first?

facial droop

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:

factor VIII.

A child is diagnosed with sickle cell anemia. Which test will the nurse expect the primary health care provider to prescribe for this client?

hemoglobin

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?

infection

To prevent further sickle cell crisis, the nurse 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.

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?

occupational 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

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)

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?

"This is an autosomal recessive genetic disorder that causes aplastic anemia and other congenital disorders."

The nurse is teaching the parents of a 7-year-old child with sickle cell disease and enuresis. What statement(s) will the nurse include in the teaching? Select all that apply.

"To prevent bedwetting, have your child get up and go to the bathroom periodically at night." "Adequate hydration is essential to maintain blood flow." "Because of your child's condition, the urine output is greater than the capacity of the bladder." "Your child's kidneys are unable to concentrate urine."

The nurse cares for a child with severe hemophilia A with the chart and note above. Based on these notes, what finding(s) indicates that the child has met the expected outcomes for the child's condition? Select all that apply.

Child's PT, PTT, and coagulation factor levels are within target limits. Family expresses understanding of risk prevention measures. Child engages in developmentally appropriate activities. Client demonstrates full range of motion in affected joints.

A health care provider has prescribed hydroxyurea 650 mg for a child diagnosed with sickle cell anemia. The child weighs 65 lb (29.5 kg). The normal recommended dose is 20 mg/kg/day. What action should the nurse take?

Contact the health care provider to lower the dose.

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 understand why you think your child is not in pain; sleep is often a way for children to cope with pain."

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?

"Our family is taking a fun hiking trip up in the mountains next week."

The nurse is preparing a child for discharge following a sickle cell crisis. Which statement by the mother indicates a need for further teaching?

"She has been down, but playing in soccer camp will cheer her up."

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?

"Tell me about the symptoms your child is experiencing"

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?

"The use of dry heat sterilization has proven to inactivate hepatitis virus, resulting to zero factors transfusion-related incidence of hepatitis infection."

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.

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?

Initiate pain assessment with a standardized pain scale.

A child diagnosed with hemophilia presents with warm, swollen, painful joints. Which action will the nurse take first?

Prepare to administer factor replacement medication

In caring for a child with sickle cell disease, the highest priority goal is:

The child's fluid intake will improve.