Chapter 43: Hematologic or Immunologic Dysfunction

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 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.

Which statement best 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.

A possible cause of acquired aplastic anemia in children is: A. Drugs B. Injury C. Deficient diet D. Congenital defect

ANS: A Drugs such as chemotherapeutic agents and several antibiotics such as chloramphenicol can cause aplastic anemia. Fanconi syndrome is a primary form of the disorder, which is congenital/present-at-birth and not acquired after birth. Injury, deficient diet, and congenital defect are not causative agents in acquired aplastic anemia.

What is the priority nursing intervention for a child hospitalized with hemarthrosis resulting from hemophilia? A. Immobilization and elevation of the affected joint B. Administration of acetaminophen for pain relief C. Assessment of the child's response to hospitalization D. Assessment of the impact of hospitalization on the family system

ANS: A Immobilization and elevation of the joint will prevent further injury until bleeding is resolved. Although acetaminophen may help with pain associated with the treatment of hemarthrosis, it is not the priority nursing intervention. Assessment of a child's response to hospitalization is relevant to all hospitalized children; however, in this situation, psychosocial concerns are secondary to physiologic concerns. A priority nursing concern for this child is the management of hemarthrosis. Assessing the impact of hospitalization on the family system is relevant to all hospitalized children; however, it is not the priority in this situation.

The most appropriate nursing diagnosis for a child with anemia is: A. Activity intolerance related to generalized weakness B. Decreased cardiac output related to abnormal hemoglobin C. Risk for injury related to depressed sensorium D. Risk for injury related to dehydration and abnormal hemoglobin

ANS: A The basic pathology in anemia is the decreased oxygen-carrying capacity of the blood. The nurse must assess the child's activity level (response to the physiologic state). The nursing diagnosis would reflect the activity intolerance. In generalized anemia no abnormal hemoglobin may be present. Only at a level of very severe anemia does cardiac output become altered. No decreased sensorium exists until profound anemia occurs. Dehydration and abnormal hemoglobin are not usually part of anemia.

A condition in which the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin is: A. Aplastic anemia B. Sickle cell anemia C. Thalassemia major D. Iron deficiency anemia

ANS: B 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. Hemophilia refers to a group of bleeding disorders in which there is deficiency of one of the factors necessary for coagulation. Iron deficiency anemia affects size and depth of color of hemoglobin and does not involve abnormal hemoglobin.

A school-age child is admitted in vaso-occlusive sickle cell crisis. The child's care should include: 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, minimizing energy expenditures, pain management, electrolyte replacement, and blood component therapy if indicated. The acidosis will be corrected as the crisis is treated. Heparin and factor VIII are not indicated in the treatment of vaso-occlusive sickle cell crisis. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels.

The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. The nurse should explain that narcotic analgesics: A. Are often ordered but not usually needed B. Rarely cause addiction because they are medically indicated C. Are given as a last resort because of the threat of addiction D. Are used only if other measures such as ice packs are ineffective

ANS: B The pain of sickle cell anemia is best treated by a multidisciplinary approach. Mild-to-moderate pain can be controlled by ibuprofen and acetaminophen. When narcotics are indicated, they are titrated to effect and given around the clock. Patient-controlled analgesia reinforces the patient's role and responsibility in managing the pain and provides flexibility in dealing with pain. Few if any patients who receive opioids for severe pain become behaviorally addicted to the drug. Narcotics are often used because of the severe nature of the pain of vaso-occlusive crisis. Ice is contraindicated because of its vasoconstrictive effects.

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. Fingersticks for blood work instead of venipunctures B. Avoidance of intramuscular injections C. Acetaminophen for the mild pain control D. Soft toothbrush 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.

The nurse is caring for a child with aplastic anemia. Which nursing diagnoses are appropriate (Select all that apply)? A. Acute pain related to vaso-occlusion B. Risk for infection related to inadequate secondary defenses or immunosuppression C. Ineffective protection related to thrombocytopenia D. Ineffective tissue perfusion related to anemia E. Ineffective protection related to abnormal clotting

ANS: B, C, D These are appropriate nursing diagnosis for the nurse planning care for a child with aplastic anemia. Aplastic anemia is a condition in which the bone marrow ceases production of the cells it normally manufactures, resulting in pancytopenia. The child will have varying degrees of the disease depending on how low the values are for absolute neutrophil count (affecting the body's response to infection), platelet count (putting the child at risk for bleeding), and absolute reticulocyte count (causing the child to have anemia). Acute Pain related to vaso-occlusion is an appropriate nursing diagnosis for sickle cell anemia for the child in vaso-occlusive crisis, but it is not applicable to a child with aplastic anemia. Ineffective Protection related to abnormal clotting is an appropriate diagnosis for a child with hemophilia

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 101.3 or greater occurs C. Give penicillin as prescribed D. Use ice packs to decrease the discomfort of vaso-occlusive 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 vaso-occlusive pain crisis because it vasoconstricts and impairs circulation even more

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.

Which child should the nurse document as being anemic? A. 7-year-old child with a hemoglobin of 11.5g/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: C 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.

The nurse is planning activity for a 4-year-old child with anemia. Which activity should the nurse plan for this child? A. Game of "hide and seek" in the children's outdoor play area B. Participation in dance activities in the playroom C. Puppet play in the child's room D. A walk down to the hospital lobby

ANS: C Because the basic pathologic process in anemia is a decrease in oxygen-carrying capacity, an important nursing responsibility is to assess the child's energy level and minimize excess demands. The child's level of tolerance for activities of daily living and play is assessed, and adjustments are made to allow as much self-care as possible without undue exertion. Puppet play in the child's room would not be overly tiring. Hide and seek, dancing, and walking to the lobby would not conserve the anemic child's energy.

Iron dextran is ordered for a young child with severe iron deficiency anemia. Nursing considerations include: A. Administering with meals B. Administering between meals C. Injecting deeply into a large muscle D. Massaging 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 using the Z-track method. Iron dextran is for intramuscular or intravenous administration; it is not taken orally. The site should not be massaged to prevent leakage, potential irritation, and staining of the skin.

Several blood tests are ordered for a preschool child with severe anemia. She is crying and upset because she remembers the venipuncture done at the clinic 2 days ago. The nurse should explain that: A. Venipuncture discomfort is very brief B. Only one venipuncture will be needed C. Topical application of local anesthetic can eliminate venipuncture pain D. Most blood tests on children require only a finger puncture because a small amount of blood is needed

ANS: C Preschool children are very concerned about both pain and the loss of blood. When preparing the child for venipuncture, a topical anesthetic will be used to eliminate any pain. This is a very traumatic experience for preschool children. They are concerned about their bodily integrity. A local anesthetic should be used, and a bandage should be applied to maintain bodily integrity. A promise that only one venipuncture will be needed should not be made in case multiple attempts are required. Both finger punctures and venipunctures are traumatic for children. Both require preparation.

Which statement most accurately 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 red blood cell destruction 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.

As related to inherited disorders, which statement is descriptive of most cases of hemophilia? A. Autosomal dominant disorder causing deficiency in a factor involved in blood-clotting reaction B. X-linked recessive inherited disorder causing deficiency in 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 disorder involves coagulation factors, not platelets. The disorder does not involve red cells or the Y chromosome.

When teaching the mother of a 9-month-old infant about administering liquid iron preparations, the nurse should include that: 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. Preparation should be allowed 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 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.

Which clinical manifestation should the nurse expect when a child with sickle cell anemia experiences an acute vaso-occlusive crisis? A. Circulatory collapse B. Cardiomegaly, systolic murmurs C. Hepatomegaly, intrahepatic cholestasis D. Painful swelling of hands and feet, painful joins

ANS: D A vaso-occlusive 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 vaso-occlusive phenomena.

An accurate description of anemia is: A. Increased blood viscosity B. Depressed hematopoietic system C. Presence of abnormal hemoglobin D. Decreased oxygen-carrying capacity of blood

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. 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 deceased oxygen-carrying capacity of the blood.

The nurse is recommending how to prevent iron deficiency anemia in a healthy, term, breastfed infant. What should she or he suggest? A. Iron (ferrous sulfate) drops after age 1 month B. Iron-fortified commercial formula can be used by ages 4 to 6 months C. Iron-fortified infant cereal can be introduced at age 2 months D. Iron- fortified infant cereal can be introduced at approximately 6 months of age

ANS: D Breast milk supplies inadequate iron for growth and development after age 5 months. Supplementation is necessary at this time. Iron supplementation or the introduction of solid foods in a breastfed baby is not indicated. Introducing iron-fortified infant cereal at 2 months should be done only if the mother is choosing to discontinue breastfeeding.