Hematologic Disorders PREPU

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 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.

dactylitis silent cerebral ischemia acute chest syndrome

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

A nurse is caring for a child diagnosed with von Willebrand disease who is receiving desmopressin as part of the treatment regimen. In relation to desmopressin administration, what assessment finding(s) is important for the nurse to monitor? Select all that apply.

electrolyte balance intake/output heart rate Explanation: Desmopressin is a synthetic antidiuretic hormone that can cause water retention, hyponatremia, and congestive heart failure. It is important for the nurse to closely monitor a client's electrolytes, intake and output, and for signs of congestive heart failure (increased heart rate, wheezing, etc.). While monitoring the level of consciousness and temperature are important assessments for a client with von Willebrand disease, they are not the most important assessments related to desmopressin administration.

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

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.

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 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 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 assessing a 3-year-old child's tissue perfusion. Which assessment finding(s) indicates adequate tissue perfusion in the client? Select all that apply.

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

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

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 cares for the child with the note above. The child requires on-demand factor VIII treatment. Which dosage should the nurse plan to administer during the child's admission?

50 units/kg every 8 hours

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."

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.

57

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 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?

"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?

"The leftover blood in the shoulder is causing hemarthrosis."

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."

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."

A nurse is preparing discharge instructions to the parent of a child diagnosed with beta thalassemia. The child will need to take subcutaneous deferoxamine. As the nurse begins the instruction, the parent says "This is all so overwhelming. Can we do this another time?" How should the nurse respond?

Agree to come back later and discuss the parent's concerns.

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 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 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 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 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.

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.

The nurse is preparing to administer a blood transfusion for a 10-year-old client with beta-thalassemia. What should the nurse do while administering the transfusion? Select all that apply.

Recheck vital signs after the first 15 minutes of the transfusion. Teach the family about the signs and symptoms of a transfusion reaction. Reassess the client at the end of the transfusion. One hour after the transfusion, reassess the client.

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.

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

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.

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

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

The child's fluid intake will improve.