Chapter 28: The Child with Hematologic or Immunologic Dysfunction Flashcards
Exam 2
- The regulation of red blood cell (RBC) production is thought to be controlled by which physiologic factor?
a. Hemoglobin
b. Tissue hypoxia
c. Reticulocyte count
d. Number of RBCs
ANS: B-Tissue hypoxia
Hemoglobin does not directly control RBC production. If there is insufficient hemoglobin to adequately oxygenate the tissue, then erythropoietin may be released. When tissue hypoxia occurs, the kidneys release erythropoietin into the bloodstream. This stimulates the marrow to produce new RBCs. Reticulocytes are immature RBCs. The retic count can be used to monitor hematopoiesis. The number of RBCs does not directly control production. In congenital cardiac disorders with mixed blood flow or decreased pulmonary blood flow, RBC production continues secondary to tissue hypoxia.
- What physiologic defect is responsible for causing anemia?
a. Increased blood viscosity
b. Depressed hematopoietic system
c. Presence of abnormal hemoglobin
d. Decreased oxygen-carrying capacity of blood
ANS: D-Decreased oxygen-carrying capacity of blood
Anemia is a condition in which the number of red blood cells or hemoglobin concentration is reduced below the normal values for age. This results in a decreased oxygen-carrying capacity of blood. Increased blood viscosity is usually a function of too many cells or of dehydration, not of anemia. A depressed hematopoietic system or abnormal hemoglobin can contribute to anemia, but the definition depends on the decreased oxygen-carrying capacity of the blood.
- A mother states that she brought her child to the clinic because the 3-year-old girl was not keeping up with her siblings. During physical assessment, the nurse notes that the child has pale skin and conjunctiva and has muscle weakness. The hemoglobin on admission is 6.4 g/dl. After notifying the practitioner of the results, what nursing priority intervention should occur next?
a. Reduce environmental stimulation to prevent seizures.
b. Have the laboratory repeat the analysis with a new specimen.
c. Minimize energy expenditure to decrease cardiac workload.
d. Administer intravenous fluids to correct the dehydration.
ANS: C-Minimize energy expenditure to decrease cardiac workload.
The child has a critically low hemoglobin value. The expected range is 11.5 to 15.5 g/dl. When the oxygen-carrying capacity of the blood decreases slowly, the child is able to compensate by increasing cardiac output. With the increasing workload of the heart, additional stress can lead to cardiac failure. Reduction of environmental stimulation can help minimize energy expenditure, but seizures are not a risk. A repeat hemoglobin analysis is not necessary. The child does not have evidence of dehydration. If intravenous fluids are given, they can further dilute the circulating blood volume and increase the strain on the heart.
- A child with severe anemia requires a unit of red blood cells (RBCs). The nurse explains to the child that the transfusion is necessary for which reason?
a. Allow her parents to come visit her.
b. Fight the infection that she now has.
c. Increase her energy so she will not be so tired.
d. Help her body stop bleeding by forming a clot (scab).
ANS: C-Increase her energy so she will not be so tired.
The indication for RBC transfusion is risk of cardiac decompensation. When the number of circulating RBCs is increased, tissue hypoxia decreases, cardiac function is improved, and the child will have more energy. Parental visiting is not dependent on transfusion. The decrease in tissue hypoxia will minimize the risk of infection. There is no evidence that the child is currently infected. Forming a clot is the function of platelets.
- An 8-year-old girl is receiving a blood transfusion when the nurse notes that she has developed precordial pain, dyspnea, distended neck veins, slight cyanosis, and a dry cough. These manifestations are most suggestive of what complication?
a. Air embolism
b. Allergic reaction
c. Hemolytic reaction
d. Circulatory overload
ANS: D-Circulatory overload
The signs of circulatory overload include distended neck veins, hypertension, crackles, a dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema are signs and symptoms of allergic reactions. Hemolytic reactions are characterized by chills, shaking, fever, pain at infusion site, nausea, vomiting, tightness in chest, flank pain, red or black urine, and progressive signs of shock and renal failure.
- What explanation provides the rationale for why iron-deficiency anemia is common during infancy?
a. Cows milk is a poor source of iron.
b. Iron cannot be stored during fetal development.
c. Fetal iron stores are depleted by 1 month of age.
d. Dietary iron cannot be started until 12 months of age.
ANS: A-Cows milk is a poor source of iron.
Children between the ages of 12 and 36 months are at risk for anemia because cows milk is a major component of their diet, and it is a poor source of iron. Iron is stored during fetal development, but the amount stored depends on maternal iron stores. Fetal iron stores are usually depleted by ages 5 to 6 months. Dietary iron can be introduced by breastfeeding, iron-fortified formula, and cereals during the first 12 months of life.
- What statement best describes iron deficiency anemia in infants?
a. It is caused by depression of the hematopoietic system.
b. Diagnosis is easily made because of the infants emaciated appearance.
c. It results from a decreased intake of milk and the premature addition of solid foods.
d. Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.
ANS: D-Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.
In iron-deficiency anemia, the childs clinical appearance is a result of the anemia, not the underlying cause. Usually the hematopoietic system is not depressed. The bone marrow produces red blood cells that are smaller and contain less hemoglobin than normal red blood cells. Children who have iron deficiency from drinking excessive quantities of milk are usually pale and overweight. They are receiving sufficient calories but are deficient in essential nutrients. The clinical manifestations result from decreased intake of iron-fortified solid foods and an excessive intake of milk.
- What information should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations?
a. Give with meals.
b. Stop immediately if nausea and vomiting occur.
c. Adequate dosage will turn the stools a tarry green color.
d. Allow preparation to mix with saliva and bathe the teeth before swallowing.
ANS: C-Adequate dosage will turn the stools a tarry green color.
The nurse should prepare the mother for the anticipated change in the childs stools. If the iron dose is adequate, the stools will become a tarry green color. A lack of color change may indicate insufficient iron. The iron should be given in two divided doses between meals when the presence of free hydrochloric acid is greatest. Iron is absorbed best in an acidic environment. Vomiting and diarrhea may occur with iron administration. If these occur, the iron should be given with meals, and the dosage reduced and gradually increased as the child develops tolerance. Liquid preparations of iron stain the teeth; they should be administered through a straw and the mouth rinsed after administration.
- Therapeutic management of a 6-year-old child with hereditary spherocytosis (HS) should include which therapeutic intervention?
a. Perform a splenectomy.
b. Supplement the diet with calcium.
c. Institute a maintenance transfusion program.
d. Increase intake of iron-rich foods such as meat.
ANS: A-Perform a splenectomy.
Splenectomy corrects the hemolysis that occurs in HS. The splenectomy is generally reserved for children older than age 5 years with symptomatic anemia. Supplementation with calcium does not affect the HS. Additional folic acid can prevent deficiency caused by the rapid cell turnover. A maintenance transfusion program suppresses red blood cell formation. At this time, the risks of transfusion are greater than those of a splenectomy. Iron supplementation does not influence the course of HS.
- What condition occurs when the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin?
a. Aplastic anemia
b. Sickle cell anemia
c. Thalassemia major
d. Iron deficiency anemia
ANS: B-Sickle cell anemia
Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, in which normal adult hemoglobin is replaced by abnormal hemoglobin. Aplastic anemia is a lack of cellular elements being produced. Thalassemia major refers to a variety of inherited disorders characterized by deficiencies in production of certain globulin chains. Iron-deficiency anemia affects red blood cell size and depth of color but does not involve abnormal hemoglobin.
- The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. What statement most accurately reflects inheritance of SCA?
a. SCA is not inherited.
b. All siblings will have SCA.
c. Each sibling has a 25% chance of having SCA.
d. There is a 50% chance of siblings having SCA.
ANS: C-Each sibling has a 25% chance of having SCA.
SCA is inherited as an autosomal recessive disorder. In this inheritance pattern, each child born to these parents has a 25% chance of having the disorder, a 25% chance of having neither SCA nor the trait, and a 50% chance of being heterozygous for SCA (sickle cell trait). SCA is an inherited hemoglobinopathy.
- The clinical manifestations of sickle cell anemia (SCA) are primarily the result of which physiologic alteration?
a. Decreased blood viscosity
b. Deficiency in coagulation
c. Increased red blood cell (RBC) destruction
d. Greater affinity for oxygen
ANS: C-Increased red blood cell (RBC) destruction
The clinical features of SCA are primarily the result of increased RBC destruction and obstruction caused by the sickle-shaped RBCs. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. SCA does not have a coagulation deficit. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension.
- A school-age child is admitted in vasoocclusive sickle cell crisis (pain episode). The childs care should include which therapeutic interventions?
a. Hydration and pain management
b. Oxygenation and factor VIII replacement
c. Electrolyte replacement and administration of heparin
d. Correction of alkalosis and reduction of energy expenditure
ANS: A-Hydration and pain management
The management of crises includes adequate hydration, pain management, minimization of energy expenditures, electrolyte replacement, and blood component therapy if indicated. Factor VIII is not indicated in the treatment of vasoocclusive sickle cell crisis. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels. Also, prolonged oxygen can reduce bone marrow activity. Heparin is not indicated in the treatment of vasoocclusive sickle cell crisis. Electrolyte replacement should accompany hydration. The acidosis will be corrected as the crisis is treated. Energy expenditure should be minimized to improve oxygen utilization. Acidosis, not alkalosis, results from hypoxia, which also promotes sickling.
- A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse?
a. Administer 100% oxygen to relieve hypoxia.
b. Notify the practitioner because chest syndrome is suspected.
c. Infuse intravenous antibiotics as soon as cultures are obtained.
d. Give ordered pain medication to relieve symptoms of pain episode.
ANS: B-Notify the practitioner because chest syndrome is suspected.
These are the symptoms of chest syndrome, which is a medical emergency. Notifying the practitioner is the priority action. Oxygen may be indicated; however, it does not reverse the sickling that has occurred. Antibiotics are not indicated initially. Pain medications may be required, but evaluation by the practitioner is the priority.
- In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxiaischemia cycle. What information should the nurse share with parents in a teaching plan?
a. Encourage drinking.
b. Keep accurate records of output.
c. Check for moist mucous membranes.
d. Monitor the concentration of the childs urine.
ANS: C-Check for moist mucous membranes.
Children with SCA have impaired kidney function and cannot concentrate urine. Parents are taught signs of dehydration and ways to minimize loss of fluid to the environment. Encouraging drinking is not specific enough for parents. The nurse should give the parents and child a target fluid amount for each 24-hour period. Accurate monitoring of output may not reflect the childs fluid needs. Without the ability to concentrate urine, the child needs additional intake to compensate. Dilute urine and specific gravity are not valid signs of hydration status in children with SCA.
- What statement best describes b-thalassemia major (Cooley anemia)?
a. It is an acquired hemolytic anemia.
b. Inadequate numbers of red blood cells (RBCs) are present.
c. Increased incidence occurs in families of Mediterranean extraction.
d. It commonly occurs in individuals from West Africa.
ANS: C-Increased incidence occurs in families of Mediterranean extraction.
Individuals who live near the Mediterranean Sea and their descendants have the highest incidence of thalassemia. Thalassemia is inherited as an autosomal recessive disorder. An overproduction of RBCs occurs. Although numerous, the red blood cells are relatively unstable. Sickle cell disease is common in blacks of West African descent.
- What therapeutic intervention is most appropriate for a child with b-thalassemia major?
a. Oxygen therapy
b. Supplemental iron
c. Adequate hydration
d. Frequent blood transfusions
ANS: D-Frequent blood transfusions
The goal of medical management is to maintain sufficient hemoglobin (>9.5 g/dl) to prevent bone marrow expansion. This is achieved through a long-term transfusion program. Oxygen therapy and adequate hydration are not beneficial in the overall management of thalassemia. The child does not require supplemental iron. Iron overload is a problem because of frequent blood transfusions, decreased production of hemoglobin, and increased absorption from the gastrointestinal tract.
- Iron overload is a side effect of chronic transfusion therapy. What treatment assists in minimizing this complication?
a. Magnetic therapy
b. Infusion of deferoxamine
c. Hemoglobin electrophoresis
d. Washing red blood cells (RBCs) to reduce iron
ANS: B-Infusion of deferoxamine
Deferoxamine infusions in combination with vitamin C allow the iron to remain in a more chelatable form. The iron can then be excreted more easily. Use of magnets does not remove additional iron from the body. Hemoglobin electrophoresis is used to confirm the diagnosis of hemoglobinopathies; it does not affect iron overload. Washed RBCs remove white blood cells and other proteins from the unit of blood; they do not affect the iron concentration.
- In which condition are all the formed elements of the blood simultaneously depressed?
a. Aplastic anemia
b. Sickle cell anemia
c. Thalassemia major
d. Iron deficiency anemia
ANS: A-Aplastic anemia
Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Sickle cell anemia is a hemoglobinopathy in which normal adult hemoglobin is partly or completely replaced by abnormal sickled hemoglobin. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin chains. Iron-deficiency anemia results in a decreased amount of circulating red cells.
