Chapter 24 Hematology

In which of the conditions 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 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 sickle hemoglobin. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin globin chains. Iron-deficiency anemia results in a decreased amount of circulating red cells. DIF: Cognitive Level: Understand REF: p. 800 TOP: Integrated Process: Nursing Process: Assessment MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 19.

The nurse is teaching parents about the importance of iron in a toddler's diet. Which explains why iron-deficiency anemia is common during toddlerhood? a. Milk is a poor source of iron. b. Iron cannot be stored during fetal development. c. Fetal iron stores are depleted by age 1 month. d. Dietary iron cannot be started until age 12 months.

ANS: A Children between the ages of 12 and 36 months are at risk for anemia because cow's 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 age 5 to 6 months. Dietary iron can be introduced by breastfeeding, iron-fortified formula, and cereals during the first 12 months of life. DIF: Cognitive Level: Understand REF: p. 789 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 5.

When both parents have sickle cell trait, which is the chance their children will have sickle cell anemia? a. 25% b. 50% c. 75% d. 100%

ANS: A Sickle cell anemia is inherited in an autosomal recessive pattern. If both parents have sickle cell trait (one copy of the sickle cell gene), then for each pregnancy, a 25% chance exists that their child will be affected with sickle cell disease. With each pregnancy, a 50% chance exists that the child will have sickle cell trait. Percentages of 75% and 100% are too high for the children of parents who have sickle cell trait. DIF: Cognitive Level: Analyze REF: p. 791 TOP: Integrated Process: Nursing Process: Assessment MSC: Area of Client Needs: Physiologic Integrity: Reduction of Risk Potential 11.

Parents of a child with sickle cell anemia ask the nurse, "What happens to the hemoglobin in sickle cell anemia?" Which statement by the nurse explains the disease process? a. Normal adult hemoglobin is replaced by abnormal hemoglobin. b. There is a lack of cellular hemoglobin being produced. c. There is a deficiency in the production of globulin chains. d. The size and depth of the hemoglobin are affected.

ANS: A 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 the size, depth, and color of hemoglobin. DIF: Cognitive Level: Apply REF: p. 791 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 10.

Chelation therapy is begun on a child with -thalassemia major. What is the purpose of this therapy? a. Treat the disease b. Eliminate excess iron c. Decrease risk of hypoxia d. Manage nausea and vomiting

ANS: B A complication of the frequent blood transfusions in thalassemia is iron overload. Chelation therapy with deferoxamine (an iron-chelating agent) is given with oral supplements of vitamin C to increase iron excretion. Chelation therapy treats the side effect of the disease management. Decreasing the risk of hypoxia and managing nausea and vomiting are not the purposes of chelation therapy. DIF: Cognitive Level: Understand REF: p. 799 TOP: Integrated Process: Nursing Process: Implementation MSC: Area of Client Needs: Physiologic Integrity: Pharmacologic and Parenteral Therapies 18.

A school-age child is admitted in vasoocclusive sickle cell crisis. What should be included in the child's care? a. Correction of acidosis b. Adequate hydration and pain management c. Pain management and administration of heparin d. Adequate oxygenation and replacement of factor VIII

ANS: B The management of crises includes adequate hydration, minimization of energy expenditures, pain management, electrolyte replacement, and blood component therapy if indicated. Hydration and pain control are two of the major goals of therapy. The acidosis will be corrected as the crisis is treated. Heparin and factor VIII are 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. DIF: Cognitive Level: Apply REF: p. 796 TOP: Integrated Process: Nursing Process: Implementation MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 15.

The nurse is planning care for a school-age child admitted to the hospital with hemophilia. Which interventions should the nurse plan to implement for this child? (Select all that apply.) a. Finger sticks for blood work instead of venipunctures b. Avoidance of IM injections c. Acetaminophen (Tylenol) for mild pain control d. Soft tooth brush for dental hygiene e. Administration of packed red blood cells

ANS: B, C, D Nurses should take special precautions when caring for a child with hemophilia to prevent the use of procedures that may cause bleeding, such as IM injections. The subcutaneous route is substituted for IM injections whenever possible. Venipunctures for blood samples are usually preferred for these children. There is usually less bleeding after the venipuncture than after finger or heel punctures. Neither aspirin nor any aspirin-containing compound should be used. Acetaminophen is a suitable aspirin substitute, especially for controlling mild pain. A soft toothbrush is recommended for dental hygiene to prevent bleeding from the gums. Packed red blood cells are not administered. The primary therapy for hemophilia is replacement of the missing clotting factor. The products available are factor VIII concentrates. DIF: Cognitive Level: Apply REF: p. 806 TOP: Integrated Process: Nursing Process: Implementation MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 2.

Which should the nurse teach about prevention of sickle cell crises to parents of a preschool child with sickle cell disease? (Select all that apply.) a. Limit fluids at bedtime. b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. d. Use ice packs to decrease the discomfort of vasoocclusive pain in the legs. e. Notify the health care provider if your child begins to develop symptoms of a cold.

ANS: B, C, E The most important issues to teach the family of a child with sickle cell anemia are to (1) seek early intervention for problems, such as a fever of 38.5° C (101.3° F) or greater; (2) give penicillin as ordered; (3) recognize signs and symptoms of splenic sequestration, as well as respiratory problems that can lead to hypoxia; and (4) treat the child normally. The nurse emphasizes the importance of adequate hydration to prevent sickling and to delay the adhesion-stasis-thrombosis-ischemia cycle. It is not sufficient to advise parents to "force fluids" or "encourage drinking." They need specific instructions on how many daily glasses or bottles of fluid are required. Many foods are also a source of fluid, particularly soups, flavored ice pops, ice cream, sherbet, gelatin, and puddings. Increased fluids combined with impaired kidney function result in the problem of enuresis. Parents who are unaware of this fact frequently use the usual measures to discourage bedwetting, such as limiting fluids at night. Enuresis is treated as a complication of the disease, such as joint pain or some other symptom, to alleviate parental pressure on the child. Ice should not be used during a vasoocclusive pain crisis because it vasoconstricts and impairs circulation even more. DIF: Cognitive Level: Apply REF: p. 797 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Health Promotion and Maintenance

Parents of a school-age child with hemophilia ask the nurse, "Which sports are recommended for children with hemophilia?" Which sports should the nurse recommend? (Select all that apply.) a. Soccer b. Swimming c. Basketball d. Golf e. Bowling

ANS: B, D, E Because almost all persons with hemophilia are boys, the physical limitations in regard to active sports may be a difficult adjustment, and activity restrictions must be tempered with sensitivity to the child's emotional and physical needs. Use of protective equipment, such as padding and helmets, is particularly important, and noncontact sports, especially swimming, walking, jogging, tennis, golf, fishing, and bowling, are encouraged. Contact sports such as soccer and basketball are not recommended. DIF: Cognitive Level: Apply REF: p. 801 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Health Promotion and Maintenance 3.

Iron dextran is ordered for a young child with severe iron-deficiency anemia. What nursing considerations should be included? a. Administer with meals b. Administer between meals c. Inject deeply into a large muscle d. Massage injection site for 5 minutes after administration of drug

ANS: C Iron dextran is a parenteral form of iron. When administered intramuscularly, it must be injected into a large muscle. Iron dextran is for intramuscular or intravenous (IV) administration. The site should not be massaged to prevent leakage, potential irritation, and staining of the skin. DIF: Cognitive Level: Apply REF: p. 790 TOP: Integrated Process: Nursing Process: Implementation MSC: Area of Client Needs: Physiologic Integrity: Pharmacologic and Parenteral Therapies 8.

The nurse is conducting a staff in-service on sickle cell anemia. Which describes the pathologic changes of sickle cell anemia? a. Sickle-shaped cells carry excess oxygen. b. Sickle-shaped cells decrease blood viscosity. c. Increased red blood cell destruction occurs. d. Decreased adhesion of sickle-shaped cells occurs.

ANS: C The clinical features of sickle cell anemia are primarily the result of increased red blood cell destruction and obstruction caused by the sickle-shaped red blood cells. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. Increased adhesion and entanglement of cells occurs. DIF: Cognitive Level: Apply REF: p. 791 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 12.

Parents of a hemophiliac child ask the nurse, "Can you describe hemophilia to us?" Which response by the nurse is descriptive of most cases of hemophilia? a. Autosomal dominant disorder causing deficiency in a factor involved in the blood-clotting reaction b. X-linked recessive inherited disorder causing deficiency of platelets and prolonged bleeding c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient d. Y-linked recessive inherited disorder in which the red blood cells become moon-shaped

ANS: C The inheritance pattern in 80% of all of the cases of hemophilia is X-linked recessive. The two most common forms of the disorder are factor VIII deficiency, hemophilia A or classic hemophilia; and factor IX deficiency, hemophilia B or Christmas disease. The inheritance pattern is X-linked recessive. The disorder involves coagulation factors, not platelets, and does not involve red cells or the Y chromosomes. DIF: Cognitive Level: Understand REF: p. 801 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 21.

Which should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations? a. They should be given with meals. b. They should be stopped 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 The nurse should prepare the mother for the anticipated change in the child's stools. If the iron dose is adequate, the stools will become a tarry green color. The lack of the 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, then 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. DIF: Cognitive Level: Apply REF: p. 789 TOP: Integrated Process: Teaching/Learning MSC: Area of Client Needs: Physiologic Integrity: Pharmacologic and Parenteral Therapies 7.

Which clinical manifestation should the nurse expect when a child with sickle cell anemia experiences an acute vasoocclusive crisis? a. Circulatory collapse b. Cardiomegaly, systolic murmurs c. Hepatomegaly, intrahepatic cholestasis d. Painful swelling of hands and feet; painful joints

ANS: D A vasoocclusive crisis is characterized by severe pain in the area of involvement. If in the extremities, painful swelling of the hands and feet is seen; if in the abdomen, severe pain resembles that of acute surgical abdomen; and if in the head, stroke and visual disturbances occur. Circulatory collapse results from sequestration crises. Cardiomegaly, systolic murmurs, hepatomegaly, and intrahepatic cholestasis result from chronic vasoocclusive phenomena. DIF: Cognitive Level: Understand REF: p. 791 TOP: Integrated Process: Nursing Process: Assessment MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 13.

Which child should the nurse document as being anemic? a. 7-year-old child with a hemoglobin of 11.5 g/dl b. 3-year-old child with a hemoglobin of 12 g/dl c. 14-year-old child with a hemoglobin of 10 g/dl d. 1-year-old child with a hemoglobin of 13 g/dl

ANS: D Anemia is a condition in which the number of red blood cells, or hemoglobin concentration, is reduced below the normal values for age. Anemia is defined as a hemoglobin level below 10 or 11 g/dl. The child with a hemoglobin of 10 g/dl would be considered anemic. The normal hemoglobin for a child after 2 years of age is 11.5 to 15.5 g/dl. DIF: Cognitive Level: Understand REF: p. 789 TOP: Integrated Process: Nursing Process: Assessment MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 2.

Several complications can occur when a child receives a blood transfusion. Which is an immediate sign or symptom of an air embolus? a. Chills and shaking b. Nausea and vomiting c. Irregular heart rate d. Sudden difficulty in breathing

ANS: D Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Air emboli should be avoided by carefully flushing all tubing of air before connecting to patient. Chills, shaking, nausea, and vomiting are associated with hemolytic reactions. Irregular heart rate is associated with electrolyte disturbances and hypothermia. DIF: Cognitive Level: Understand REF: p. 810 TOP: Integrated Process: Nursing Process: Assessment MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 29.

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. Of what are these manifestations most suggestive? a. Air emboli b. Allergic reaction c. Hemolytic reaction d. Circulatory overload

ANS: D The signs of circulatory overload include distended neck veins, hypertension, crackles, dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Allergic reactions are manifested by urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema. 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. DIF: Cognitive Level: Apply REF: p. 811 TOP: Integrated Process: Nursing Process: Assessment MSC: Area of Client Needs: Physiologic Integrity: Physiologic Adaptation 30.