Chapter 44: Nursing Care of a Family when a Child has a Hematologic Disorder

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 can have the nurse administer the chelation therapy if I am uncomfortable."* The nurse needs to emphasize to the mother that therapy must be maintained at home to continuously decrease the iron levels in the child's body. Family members need to be taught to administer deferoxamine subcutaneously with a small battery-powered infusion pump over a several-hour period each night (usually while the child is sleeping).

The nurse begins administering blood to a pediatric client with hemoglobinopathy. During the transfusion, the nurse notes: a rash on the child's chest, face, and extremities; temperature 101.8°F (38.8°C); respirations 34 breaths/minute; and the child reports nausea. Which actions will the nurse take? Select all that apply.

Call the child's primary health care provider. Stop the blood transfusion. Monitor the child's urine output. Assess the child's vital signs. Administer only IV normal saline (NS). Based on the findings, the nurse would suspect an adverse reaction to the blood transfusion. The nurse would immediately stop the transfusion, administer NS IV to the client, send the blood and tubing to the laboratory, and notify the health care provider. The nurse would continue to monitor the child by assessing vital signs and monitor urine output as a decrease in kidney function could indicate acute kidney failure.

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."* Sickle cell disease is an inherited disease. The recessive gene is passed from both parents who either have the disease or the trait. There is no need for further testing to determine the cause. There are no other ways to pass the disease other than through genetics. Informing the parents that the gene was passed from both parents is most informative. Sickle cell anemia is not a dominant disease, which is passed when only one parent has the gene, nor is caused by a random mutation.

The nurse is caring for a 2-year-old with sickle cell anemia and instructing the parents on the manifestations of the disease. Which statement by the mother indicates a need for further teaching?

*"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.

A 1-year-old child is diagnosed with pernicious anemia due to lack of intrinsic factor and is prescribed vitamin B12 injections. After teaching the child's parents about this treatment, the nurse determines that the teaching was successful based on which statement?

*"We will give the injection once a month for the rest of our child's life."* If the anemia is identified as being caused by a diet deficient in vitamin B12, temporary injections of vitamin B12 will reverse the symptoms. If the anemia is caused by a lack of the intrinsic factor, lifelong monthly intramuscular injections of vitamin B12 may be necessary.

A nurse is providing care to a child with idiopathic thrombocytopenic purpura with a platelet count of 18,000/mm3 μl (18,000 x 109/L). Which medication would the nurse most likely expect to be ordered?

*Intravenous immune globulin* Intravenous immune globulin would be used to treat idiopathic thrombocytopenic purpura. Folic acid is used to treat folic acid deficiency anemia. Dimercaprol is used to remove lead from the soft tissue and bone to allow for excretion by the kidneys. Deferoxamine is used to treat iron toxicity.

When assessing a child for a possible hematologic disorder, which would the nurse need to keep in mind as most important?

*Multiple body sites can be affected.* The nurse needs to keep in mind that hematologic alterations can affect multiple body sites, so assessment needs to address all body systems. A child's nutritional status may be helpful in assessing certain hematologic disorders such as iron deficiency anemia, but this information is not the most important to remember. Sequelae commonly occur with hematologic alterations, especially chronic conditions such as hemophilia or sickle cell disease. The child's demographic data are important, because some hematologic diagnoses are more commonly associated with a certain age group, sex, race, or geographic location.

A nurse is providing care for a child with disseminated intravascular coagulation (DIC). What would alert the nurse to possible neurologic compromise?

*Widely fluctuating blood pressure* A key aspect of the nurse's role is to assess the child for signs and symptoms of impaired tissue perfusion in the various body systems that may be affected by DIC. Unstable or abnormal blood pressure (such as wide BP fluctuations) or unequal pupil size may suggest neurologic compromise. Hematuria would suggest renal compromise. Petechiae would be indicative of bleeding into the skin.

A child with hemophilia A has had repeated episodes of hemarthrosis. Which assessment finding is most important to consider?

*decreased ROM* Repeated bleeding into a joint causes cartilage erosion and joint space narrowing, decreased range of motion, and proximal muscle weakening; disabling arthropathy may follow.

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 what as a factor?

*infection* 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 is receiving a blood transfusion. Which sign or symptom would alert the nurse that the child is experiencing a hemolytic reaction?

*lower back pain* With a hemolytic transfusion reaction, lower back or abdominal pain is noted. Urticaria may be seen with a hemolytic reaction or an allergic reaction. Respiratory distress is associated with an allergic reaction. Diaphoresis is associated with a febrile reaction.

A child diagnosed with idiopathic thrombocytopenic purpura (ITP) is scheduled to receive an infusion of intravenous immunoglobulin (IVIG) due to low platelet counts. Prior to the infusion, the nurse administers acetaminophen to the child. The nurse would explain to the parents that acetaminophen is administered to obtain which expected outcome?

*to decrease fever produced from the medication* For the child with ITP, the administration of IVIG is warranted, if the platelet counts decrease below 10,000 mm3 (10 ×109/L). IVIG produces large amounts of antibodies and will improve the platelet count. IVIG is considered a blood product. During administration, antipyretics may be given to reduce fever and pain from the flulike symptoms that can develop. Antihistamines are given to prevent or reduce chills. The nurse would monitor fever, vital signs, and any other adverse reactions that could occur from this medication or any blood product.

A 14-year-old girl who is a vegetarian has recently developed anemia. Blood smear results show large, fragile, immature erythrocytes. She claims to take an iron supplement regularly and is surprised to learn that she is anemic, as she is otherwise healthy. As the nurse considers all of the data in the case, which anemia will the nurse discuss when collaborating with the primary healthcare provider?

*vitamin B12 deficiency* Vitamin B12 is necessary for the maturation of RBCs. Pernicious anemia results from deficiency or inability to use the vitamin, resulting in RBCs that appear abnormally large and are immature megaloblasts (nucleated immature red cells). Thus, pernicious anemia is one of the megaloblastic anemias. In children, the cause is more often lack of ingestion of vitamin B12 rather than poor absorption. Adolescents may be deficient in vitamin B12 if they have a long-term, poorly formulated vegetarian diet, as the vitamin is found primarily in foods of animal origin. Since the client is taking iron, iron deficiency anemia is ruled out. The blood cells in a client with sickle cell anemia are crescent-shaped and do not display the characteristics noted. There are no symptoms of blood loss or acute bleeding, as the client is otherwise healthy.