wong's SCA and hemophilia

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.

ANS: 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.

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.

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 and depth of the color.

A young boy will receive a bone marrow transplant (BMT). This is possible because one of his older siblings is a histocompatible donor. Which is this type of BMT called? a. Syngeneic b. Allogeneic c. Monoclonal d. Autologous

ANS: B Allogeneic transplants are from another individual. Because he and his sibling are histocompatible, the BMT can be done. Syngeneic marrow is from an identical twin. There is no such thing as a monoclonal BMT. Autologous refers to the individuals own marrow.

A school-age child is admitted in vasoocclusive sickle cell crisis. The childs 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, 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.

The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. Which is appropriate for the nurse to explain about 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 are given around the clock. Patient-controlled analgesia reinforces the patients 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 vasoocclusive crisis. Ice is contraindicated because of its vasoconstrictive effects.

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 childrens outdoor play area b. Participation in dance activities in the playroom c. Puppet play in the childs 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 childs energy level and minimize excess demands. The childs 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 childs room would not be overly tiring. Hide and seek, dancing, and walking to the lobby would not conserve the anemic childs energy.

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

ANS: C 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 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.

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.

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.

Which is the most effective pain-management approach for a child who is having a bone marrow aspiration? a. Relaxation techniques b. Administration of an opioid c. EMLA cream applied over site d. Conscious or unconscious sedation

ANS: D Effective pharmacologic and nonpharmacologic measures should be used to minimize pain associated with procedures. For bone marrow aspiration, conscious or unconscious sedation should be used. Relaxation, opioids, and EMLA can be used to augment the conscious or unconscious sedation.

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.

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 adhesionstasisthrombosisischemia 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.

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, 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 childs 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.

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, 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 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.

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 childs condition has been medically evaluated.