Chapter 24: The Child with Hematologic or Immunologic Dysfunction

The nurse is teaching parents about the importance of iron in a toddler's diet. Which explains why iron deficiency anemia is common during toddler-hood? 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.

a. Milk is a poor source of iron. 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.

The nurse is planning care for an adolescent with AIDS. Which is the priority nursing goal? a. Preventing infection b. Preventing secondary cancers c. Restoring immunologic defenses d. Identifying source of infection

a. Preventing infection Because the child is immunocompromised in association with HIV infection, the prevention of infection is paramount. Although certain precautions are justified in limiting exposure to infection, these must be balanced with the concern for the child's normal developmental needs. Preventing secondary cancers is not currently possible. Current drug therapy is affecting the disease progression; although not a cure, these drugs can suppress viral replication, preventing further deterioration. Case finding is not a priority nursing goal.

suddenly begins to wheeze and have severe urticaria. Which is the most appropriate nursing action? a. Stop drug infusion immediately. b. Recheck rate of drug infusion. c. Observe child closely for next 10 minutes. d. Explain to child that this is an expected side effect.

a. Stop drug infusion immediately. If an allergic reaction is suspected, the drug should be immediately discontinued. Any drug in the line should be withdrawn, and a normal saline infusion begun to keep the line open. Rechecking the rate of drug infusion, observing the child closely for next 10 minutes, and explaining to the child that this is an expected side effect can all be done after the drug infusion is stopped and the child is evaluated.

The nurse is conducting a staff in-service on inherited childhood blood disorders. Which statement describes severe combined immunodeficiency syndrome (SCIDS)? a. There is a deficit in both the humoral and cellular immunity with this disease. b. Production of red blood cells is affected with this disease. c. Adult hemoglobin is replaced by abnormal hemoglobin in this disease. d. There is a deficiency of T and B lymphocyte production with this disease.

a. There is a deficit in both the humoral and cellular immunity with this disease. Severe combined immunodeficiency syndrome (SCIDS) is a genetic disorder that results in deficits of both humoral and cellular immunity. Wiskott-Aldrich is an X-linked recessive disorder with selected deficiencies of T and B lymphocytes. Fanconi syndrome is a hereditary disorder of red cell production. Sickle cell disease is characterized by the replacement of adult hemoglobin with an abnormal hemoglobin S.

Meperidine (Demerol) is not recommended for children in sickle cell crisis because it: a. may induce seizures. b. is easily addictive. c. is not adequate for pain relief. d. is given by intramuscular injection.

a. may induce seizures. A metabolite of meperidine, normeperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with sickle cell disease are particularly at risk for normeperidine-induced seizures. Meperidine is no more addictive than other narcotic agents. Meperidine is adequate for pain relief. It is available for IV infusion.

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

d. Painful swelling of hands and feet; painful joints 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.

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

d. Sudden difficulty in breathing 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.

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: a. air emboli. b. allergic reaction. c. hemolytic reaction. d. circulatory overload.

d. circulatory overload. 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.

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

B 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.

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

D 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.

For children who do not have a matched sibling bone marrow donor, the therapeutic management of aplastic anemia includes what intervention? a. Antibiotics b. Antiretroviral drugs c. Iron supplementation d. Immunosuppressive therapy

D It is thought that aplastic anemia may be an autoimmune disease. Immunosuppressive therapy, including antilymphocyte globulin, antithymocyte globulin, cyclosporine, granulocyte colonystimulating factor, and methylprednisone, has greatly improved the prognosis for patients with aplastic anemia. Antibiotics are not indicated as the management. They may be indicated for infections. Antiretroviral drugs and iron supplementation are not part of the therapy.

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.

C 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.

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%

a. 25% 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.

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

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 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.

The school nurse is informed that a child with human immunodeficiency virus (HIV) will be attending school soon. Which is an important nursing intervention? a. Carefully follow universal precautions. b. Determine how the child became infected. c. Inform the parents of the other children. d. Reassure other children that they will not become infected.

a. Carefully follow universal precautions. Universal precautions are necessary to prevent further transmission of the disease. It is not the role of the nurse to determine how the child became infected. Informing the parents of other children and reassuring children that they will not become infected is a violation of the child's right to privacy.

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

b. Avoidance of IM injections c. Acetaminophen (Tylenol) for mild pain control d. Soft tooth brush for dental hygiene 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.

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.

b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. 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.

The nurse has initiated a blood transfusion on a preschool child. The child begins to exhibit signs of a transfusion reaction. Place in order the interventions the nurse should implement sequencing from the highest priority to the lowest. Provide answer using lowercase letters separated by commas (e.g., a, b, c, d). a. Take the vital signs. b. Stop the transfusion. c. Notify the practitioner. d. Maintain a patent IV line with normal saline. ANS: b, a, d, c If a blood transfusion reaction of any type is suspected, stop the transfusion, take vital signs, maintain a patent IV line with normal saline and new tubing, notify the practitioner, and do not restart the transfusion until the child's condition has been medically evaluated.

b. Stop the transfusion. a. Take the vital signs. d. Maintain a patent IV line with normal saline. c. Notify the practitioner. ANS: b, a, d, c If a blood transfusion reaction of any type is suspected, stop the transfusion, take vital signs, maintain a patent IV line with normal saline and new tubing, notify the practitioner, and do not restart the transfusion until the child's condition has been medically evaluated.

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

b. Swimming 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 sport such as soccer and basketball are not recommended.

Which immunization should be given with caution to children infected with human immunodeficiency virus (HIV)? a. Influenza b. Varicella c. Pneumococcal d. Inactivated poliovirus (IPV)

b. Varicella The children should be carefully evaluated before being given live viral vaccines such as varicella, measles, mumps, and rubella. The child must be immunocompetent and not have contact with other severely immunocompromised individuals. Influenza, pneumococcal, and inactivated poliovirus (IPV) are not live vaccines.

A school-age child is admitted in vasoocclusive 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.

b. adequate hydration and pain management. 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 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.

A young child with human immunodeficiency virus (HIV) is receiving several antiretroviral drugs. The purpose of these drugs is to: a. cure the disease. b. delay disease progression. c. prevent spread of disease. d. treat Pneumocystis carinii pneumonia.

b. delay disease progression. Although not a cure, these antiviral drugs can suppress viral replication, preventing further deterioration of the immune system and delaying disease progression. At this time, cure is not possible. These drugs do not prevent the spread of the disease. P. carinii prophylaxis is accomplished with antibiotics.

Chelation therapy is begun on a child with -thalassemia major. The purpose of this therapy is to: a. treat the disease. b. eliminate excess iron. c. decrease risk of hypoxia. d. manage nausea and vomiting.

b. eliminate excess iron. 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.

The nurse is recommending how to prevent iron deficiency anemia in a healthy, term, breast-fed infant. Which should be suggested? a. Iron (ferrous sulfate) drops after age 1 month b. Iron-fortified commercial formula by age 4 to 6 months c. Iron-fortified infant cereal by age 2 months d. Iron-fortified infant cereal by age 4 to 6 months

d. Iron-fortified infant cereal by age 4 to 6 months Breast milk supplies inadequate iron for growth and development after age 5 months. Supplementation is necessary at this time. The mother can supplement the breastfeeding with iron-fortified infant cereal. Iron supplementation or the introduction of solid foods in a breast-fed baby is not indicated. Providing iron-fortified commercial formula by age 4 to 6 months should be done only if the mother is choosing to discontinue breastfeeding.

The nurse is teaching parents of an infant about the causes of iron deficiency anemia. Which statement best describes iron deficiency anemia in infants? a. It is caused by depression of the hematopoietic system. b. It is easily diagnosed because of an infant's emaciated appearance. c. Clinical manifestations are similar regardless of the cause of the anemia. d. Clinical manifestations result from a decreased intake of milk and the premature addition of solid foods.

c. Clinical manifestations are similar regardless of the cause of the anemia. In iron deficiency anemia, the child's clinical appearance is a result of the anemia, not the underlying cause. Usually the hematopoietic system is not depressed in iron deficiency anemia. The bone marrow produces red cells that are smaller and contain less hemoglobin than normal red cells. Children who are iron deficient 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.

Which statement best describes -thalassemia major (Cooley anemia)? a. All formed elements of the blood are depressed. b. Inadequate numbers of red blood cells are present. c. Increased incidence occurs in families of Mediterranean extraction. d. Increased incidence occurs in persons of West African descent.

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. An overproduction of red cells occurs. Although numerous, the red cells are relatively unstable. Sickle cell disease is common in persons of West African descent.

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

c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient 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.

Several blood tests are ordered for a preschool child with severe anemia. The child is crying and upset because of memories of the venipuncture done at the clinic 2 days ago. The nurse should explain: 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.

c. topical application of local anesthetic can eliminate venipuncture pain. Preschool children are concerned with both pain and the loss of blood. When preparing the child for venipuncture, the nurse will use a topical anesthetic to eliminate any pain. This is a 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. The nurse should not promise one attempt in case multiple attempts are required. Both finger punctures and venipunctures are traumatic for children. Both require preparation.

The nurse is reviewing first aid with a group of school nurses. Which statement made by a participant indicates a correct understanding of the information? a. "If a child loses a tooth due to injury, I should place the tooth in warm milk." b. "If a child has recurrent abdominal pain, I should send him or her back to class until the end of the day." c. "If a child has a chemical burn to the eye, I should irrigate the eye with normal saline." d. "If a child has a nosebleed, I should have the child sit up and lean forward."

d. "If a child has a nosebleed, I should have the child sit up and lean forward." If a child has a nosebleed, the child should lean forward, not lie down. A tooth should be placed in cold milk or saliva for transporting to a dentist. Recurrent abdominal pain is a physiologic problem and requires further evaluation. If a chemical burn occurs in the eye, the eye should be irrigated with water for 20 minutes.

The nurse is conducting a staff in-service on childhood blood disorders. Which describes the pathology of idiopathic thrombocytopenic purpura? a. Bone marrow failure in which all elements are suppressed b. Deficiency in the production rate of globin chains c. Diffuse fibrin deposition in the microvasculature d. An excessive destruction of platelets

d. An excessive destruction of platelets Idiopathic thrombocytopenic purpura is an acquired hemorrhagic disorder characterized by an excessive destruction of platelets, discolorations caused by petechiae beneath the skin, and a normal bone marrow. Aplastic anemia refers to a bone marrow-failure condition in which the formed elements of the blood are simultaneously depressed. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin globin chains. Disseminated intravascular coagulation is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasma.

Wiskott-Aldrich syndrome

thrombocytopenia, eczema, and immunodeficiency of selective functions of B and T lymphocytes