Chapter 24 - Hematology

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. 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.

The nurse is caring for an 18 month old who is being treated for iron deficiency anemia. The child's parents ask what most likely led to the development of anemia. Which of the following is the nurse's best response? a. "Your child's body is likely breaking down the red blood cells rapidly" b. "Your child is probably not drinking enough milk" c. "Your child's body is probably not producing too many red blood cells" d. "Your child may be drinking too much milk"

d. In this age group, excessive consumption of milk is a primary cause of iron deficiency anemia.

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 the 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.

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.

A 4 year old with sickle cell disease in vaso-occlusive crisis is admitted. The nurse knows that the primary aim of treatment is to: a. Decrease fever b. Prevent spread of infection to other patients c. Provide for hydration, oxygenation and pain management d. Prevent necrosis of ischemic area

c. A child with sickle cell disease in crisis requires methods to reduce red blood cell sickling such as hydration adn oxygenation. In addition, the child will require pharmacological pain management.

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

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.

Which is the most accurate genetic explanation for a family with hemophilia? a. Y-linked dominant disorder b. Equally distributed among males and females. c. X-linked recessive disorder d. Autosomal recessive disorder

c. X-linked recessive disorder

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, 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.

A child with sickle cell anemia is admitted in a VOC. Which of the following interventions should the nurse expect to see ordered? Select all that apply. a. Cold compress to painful joints b. IV fluids started, oral fluids encouraged. c. Meperidine ordered Q4 for pain d. High-calorie, high-protein diet e. Antibiotics ordered for any existing infection

b, d, e b. IV fluids started, oral fluids encouraged. d. High-calorie, high-protein diet e. Antibiotics ordered for any existing infection

The nurse recognizes which of the following as a clinical manifestation of chronic anemia experienced by children with sickle cell disease? a. Excessive weight gain b. Cardiac murmur c. Early sexual maturation d. Ruddy complexion

b. Chronic anemia is characterized by signs and symptoms that reflect that the body has compensated for the ongoing anemia. Symptoms of chronic anemia include growth retardation, cardiac murmur, delayed sexual maturity and hyperbilirubinemia.

A child is admitted to the pediatric unit. The mother reports that the doctor says her son is anemic. What lab findings/manifestations would the nurse expect to see to confirm the deficiency anemia? a. Cyanosis, due to inadequate O2 saturation of existing hemoglobin b. A decreased reticulocyte count c. A total iron-binding capacity (TIBC) that is elevated above the normal range d. Decreased BP changes, which are an early sign because of the compensatory mechanisms.

c. A total iron-binding capacity (TIBC) that is elevated above the normal range

You are working with a recent graduate on the pediatric unit. You are assigned to take care of an adolescent with beta-thalassemia. The nurse needs more information about this disease if she states which of the following? Select all that apply. a. "We need to check the patient's iron level to make sure he is not anemic." b. "I believe this is most common in those of Hispanic descent, although this patient is Mediterranean." c. "The doctor will be prescribing deferasirox (Exjade) or deferoxamine (Desferal) for chelation therapy." d. "This patient looks much younger than I would expect. I guess he is just a late bloomer." e. "I think a transfusion will be ordered because his hemoglobin level is 9.0."

a, b, d a. "We need to check the patient's iron level to make sure he is not anemic." b. "I believe this is most common in those of Hispanic descent, although this patient is Mediterranean." d. "This patient looks much younger than I would expect. I guess he is just a late bloomer."

Why is meperidine (Demerol) not recommended for children in sickle cell crisis? a. May induce seizures b. Is easily addictive c. Not adequate for pain relief d. Given by intramuscular injection

a. 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.

The mother of a toddler with sickle cell disease calls the clinic to report that the child has a fever of 101.5 F and has been coughing. Which action would the nurse recommend to the parent? a. Have the child evaluated in the emergency room immediately b. Give Tylenol and recheck temp in one hour c. Have child rest at home as the likely cause is a viral illness d. Give clear liquids and aspirin for fever

a. Children with sickle cell disease are at increased risk for infection due to splenic dysfunction. It is essential to promote early diagnosis and treatment of any potentially infectious process. A fever and cough could indicate acute chest syndrome which requires prompt treatment with IV antibiotics.

The nurse is caring for an 8 year old diagnosed with Idiopathic Thrombocytopenic Purpura (ITP). Which of the following is considered a standard of care? a. Intramuscular injections are avoided b. Oral steroids are given when platelet counts are elevated above 150,000 c. Strict bedrest is required when platelet counts fall below 150,000 d. Iron supplementation is a mainstay of therapy

a. Intramuscular injections are avoided due to risk of bleeding. Steroids are given when platelets are decreased but above 20,000. Below 20,000 requires infusion of IVIG. Although trauma is avoided, strict bedrest is not needed. Iton supplementation is not routine in ITP.

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.

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.

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. 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.

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, 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.

Chelation therapy using the drug Desferal is begun on a child with Sickle Cell Disease. 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

b. A complication of the frequent blood transfusions in Sickle Cell Disease 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 teaching parents of a toddler diagnosed with iron-deficiency anemia. Which of the following should be included in the education? a. Give the iron supplement with milk b. Give the iron supplement with citrus fruit or juice c. Monitor for episodes of diarrhea d. Give the iron supplement before meals

b. Iron supplements are absorbed best when given between meals and with citric fruit or juice. Effective compliance with iron supplement therapy will result in stools that are tarry green or black and formed.

The nurse is caring for a toddler being evaluated for anemia. Which of the following might the nurse expect to hear when obtaining the child's history? a. The child has been unusually hungry b. The child has been scratching her arms and legs c. The child recently had a growth spurt d. The child has been complaining of pain in the legs

b. The increased red blood cell breakdown seen in some types of anemia can lead to puritis due to the presence of unconjugated bilirubin. Most children with anemia do not experience an increased appetite. The child with anemia will experience growth retardation. Puritis is a more common complaint than pain.

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. 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.

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 preterm addition of solid foods.

c. 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 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.

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.

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. 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.

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

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.