EAQ: PEDI Hematological Disorders

Based on history and clinical examination, a child is suspected of having sickle cell disease. What finding in the laboratory report adds to the suspicion?

Reticulocyte count 3%

What serious complication of sickle cell disease is similar to pneumonia?

Acute chest syndrome

Which medication is contraindicated with patients with sickle cell disease?

Meperidine

A registered nurse is teaching a group of student nurses about protein disorders and genetic diseases. Which statement made by a student nurse indicates effective learning?

Modifications in globin production cause thalassemia.

A child feels stiffness, tingling and aches in the knee joints and has a tendency toward prolonged bleeding. Which test is the healthcare provider most likely to prescribe?

Partial thromboplastin time

A child is taking a medication that can cause significant hemolysis. What finding in the laboratory report would the nurse infer as causing hemolysis?

Presence of nucleated red blood cells

Which ethnic groups are tested due to the increased incidence of the genetic disorder in the specific ethnic group?

Sickle cell anemia and African-Americans Thalassemia and people of Mediterranean decent Tay Sachs disease and Ashkenazi Jews

The parent of a 4-month-old infant asks the primary health care provider about a substitution for breast milk. What recommendation should the primary health care provider make?

Substituting with iron-fortified formula

Globins protein mutations result in which disorder(s)?

Thalassemias Sickle cell disease

The nurse is caring for a 10-year-old child whose white blood cell count is high. The child asks the nurse about the significance of white blood cells. What is the most appropriate response of the nurse?

White blood cells help keep germs from causing infections.

The nurse is teaching a female, adolescent patient with sickle cell anemia about the complications associated with her disease. Which statement should the nurse include while educating the patient?

Women with sickle cell anemia are at risk for pregnancy-related disorders."

The parent of a 2-year-old child tells the nurse, "My child mostly drinks milk and fruit juice and consumes very little solid food." The nurse further assesses the child for which condition?

Iron deficiency

A registered nurse is teaching about chelation therapy to a group of student nurses. Which statement made by a student nurse indicates a need for further teaching?

It requires the patient be placed on a fluid restriction.

The nursing student understands that the red blood cell is produced through what means?

Erythropoiesis

A 2-month-old formula-fed baby is brought in for a routine checkup. The parent of the baby tells the nurse that a friend has advised her to give fresh cow's milk to the baby instead of formula milk because it has high nutritional value. What is the nurse's best response?

Cow's milk should be avoided before 12 months of age, because it may cause iron-deficiency anemia.

A child with sickle cell anemia has been prescribed deferasirox along with deferoxamine. What is the difference between the two medications?

Deferoxamine is given via an infusion pump; deferasirox is an oral agent.

Parents of an 8-week-old baby with sickle cell disease have come to the hospital for the baby's routine checkup. They tell the nurse that they do not want penicillin prophylaxis for their baby, because the baby is very young. What does the nurse tell them?

"Children with sickle cell disease should take penicillin prophylaxis by 2 months of age." "If a baby is put on penicillin, medical advice is needed if the temperature exceeds 38.3° C."

A child with β-thalassemia is receiving numerous blood transfusions. In addition, the child is receiving deferoxamine therapy. The child's parents ask the nurse what deferoxamine does. What is the most appropriate response by the nurse?

"The medication helps prevent iron overload."

What are the chances that a hemophilic male and a female who carries the gene for hemophilia will have a normal male offspring?

25%

A nurse is caring for a toddler with sickle cell disease. Which complication is likely to be found in this patient?

Aplastic anemia

The nurse is explaining blood components to an 8-year-old child. The nurse, drawing on knowledge of child development, understands that the most appropriate description of platelets is that they do what?

Help the body stop bleeding by forming a clot (scab) over the hurt area

A neonate is suspected of having sickle cell disease. What is the preferred collection source for diagnosis?

blood

A nurse is caring for a newborn with unconjugated hyperbilirubinemia. Which condition may require bottle feeding in the newborn?

Hemolytic disease

Which procedure does the nurse identify as the means of eliminating excess iron in a child with thalassemia major?

Chelation therapy

What are the most common clinical manifestations of hemophilia?

Excessive bruising Hemorrhage from any trauma Prolonged bleeding from or in the body

The nurse observes that the usual dose of analgesic is unable to relieve pain in a 12-year-old child with sickle cell anemia after 3 weeks. What does the nurse conclude from this finding?

The child has developed tolerance to analgesic.

The transcranial Doppler test results of a child with sickle cell anemia indicate that the child has abnormal intracranial vascular flow. What information does the nurse give to the parents?

The child may require multiple blood transfusions.

A 1-day-old breastfed neonate, with the same blood type and Rh type as the mother, has a yellowish facial tone and a bilirubin level of 9.0. What is the most appropriate response by the nurse?

"Your baby is breaking down fetal red blood cells, and bilirubin is a waste product."

The parents of a child with sickle cell anemia are concerned about subsequent children having the disease. Which response by the nurse is most accurate?

"There is a 25% chance of a sibling having sickle cell anemia."

A patient with aplastic anemia is administered antithymocyte globulin (ATG). A portion of the drug accumulates in the extravascular space, causing extravasation. What intervention prevents complications due to this?

Administer medication to prevent tissue damage.

Which is a correct method for reducing pain in young patients undergoing chelation therapy?

Administer the chelating agent with procaine.

What ethnic group has the highest incidence of sickle cell disease?

African Americans

What is true for both sickle cell disease and hemophilia?

Both are genetically transmitted diseases.

What is true for both hereditary spherocytosis and sickle cell disease?

Both cause increased destruction of red blood cells.

What advice should the nurse give to the family of a child who has microcytic hypochromic anemia?

Brush the child's teeth after giving oral iron supplements.

A child with von Willebrand disease suddenly develops epistaxis at school. What should be the priority intervention made by the school nurse?

Calm the child and have him or her sit up and lean forward.

The nurse is assessing the findings of a microscopic study of red blood cell (RBC) morphology. Which assessment finding does the nurse recognize as a sign of sickle cell anemia (SCA)?

Drepanocytes

Why do children with persistent hypoxemia have an increased likelihood of postoperative bleeding compared to normal children?

Due to an increase in their red blood cell count

The school nurse is caring for a boy with hemophilia who fell on his arm during recess. What supportive measures should the nurse use until factor replacement therapy can be instituted?

Elevating the area above the level of the heart

What are the most common clinical features of sickle cell anemia in children?

Gallstones Hematuria Osteomyelitis Hepatomegaly

A child with sickle cell anemia is brought to the hospital with pain in the lower limbs. On examination the child's skin and tongue are pale and the PaO 2 is 98%. What measure should the nurse take to prevent sickling of red blood cells in this patient?

Give intravenous fluids.

A child is diagnosed with iron-deficiency anemia and is treated with iron supplements. The child starts vomiting after ingestion of iron tablets. What instructions does the nurse give to the parent and the child?

Give iron supplements with meals. Decrease dose and then increase to ordered dose as tolerated.

A 4-year-old child is prescribed liquid iron for iron-deficiency anemia. What instructions are given to the parents of this child?

Give the iron using a straw. Brush the child's teeth after administration of iron.

A child with sickle cell disease is brought to the hospital reporting right knee pain. On examination, the nurse finds localized swelling and immediately applies a cold compress to the right knee, massages the knee, and administers ibuprofen for pain relief. The nurse informs the mother that the child may need a high dose of an opioid if there is no relief from pain. Which of the measures taken by the nurse need to be corrected?

Giving cold compression to the affected area

What should the nurse teach the family about caring for a child with sickle cell anemia?

Identify early signs of dehydration. Administer prophylactic penicillin. Encourage the child to drink a lot of fluids. Check the child for proper hand-washing technique.

A 4-month-old infant is brought to the hospital to receive scheduled vaccines. The mother indicates that she stopped giving iron-fortified formula and is giving honey to her infant because the infant is constipated. For which condition is the infant at risk of developing?

Infant botulism Iron deficiency anemia

A child with sickle cell anemia experiences severe chest pain, fever, a cough, and dyspnea. What is the nurse's priority action?

Notifying the practitioner because the child may be experiencing chest syndrome

The nurse is taking the medical history of a child suffering from anemia. What areas should the nurse emphasize? .

Nutrition Eating habits Bowel habits Family history History of chronic infection

How is carbon dioxide carried in the blood?

PaCO 2 combines with certain plasma proteins and hemoglobin PaCO 2 is transported dissolved in the plasma and water of red blood cells PaCO 2 hydrates to form carbonic acid, which dissociates and is carried as bicarbonate and hydrogen ions

What is the primary treatment for hemophilia?

Replacement of missing clotting factor

An infant who is treated with iron supplements has developed constipation. The parents ask the nurse if they can use the low-iron containing formula to help the child. What should the nurse tell them?

They should not switch to low-iron-containing formula or whole milk.