EXAM 3: HEMATOLOGY
t/f: Prolonged use of oxygen can decrease erythropoiesis.
true
what are influencing factors of severe combined immunidefiecny disease
- hereditary - hormones - self marker molecules issues - viruses - certain drugs
t/f: poorly ventilated airplanes up at high altitudes can precipitate sickle cell anemia to come out worse due to hypoxia
true
what is this? Ischemia and pain caused by sickle-shaped red blood cells that obstruct blood flow to a portion of the body causing ischemia
vaso occlusive crisis
what is this? Defective cell-mediated and humoral immunity where the child has NO immune system
severe combined immunodef. disease
sickle cell anemia is most common in who
African Americans and places where malaria is
what is this? a life threatening issue where blood pools in the spleen which causes it to enlarge and at risk of a stroke
splenic sequestrian
what does it mean that sickle cell anemia is a recessive gene disorder?
that if both parents have it, it is more likely that the child will too
A child with hemophilia A is scheduled for surgery. What precautions should the nurse institute with this child? a. Handle the child gently when transferring to a cart. b. Caution the child not to brush his teeth before surgery. c. Use tape sparingly on postoperative dressings. d. Do not administer analgesics before surgery.
a - the goal of prevention of bleeding episodes is directed toward decreasing the risk of injury. The child should be handled carefully when transferring to a cart. Brushing teeth, use of tape, and giving analgesics will not risk a bleeding episode.
what is this? The body's exaggerated immune response to an internal or surface agent.
allergic reaction
what is the most common immune disorder
allergic reactions
if a child wants to crunch on ice what do you suspect they may have?
anemia
what is this? A condition in which the blood is deficient in red blood cells, in hemoglobin, or in total volume.
anemia
what is this? A condition in which the body stops producing red blood cells; typically caused by infection.
aplastic crisis
A 10-year-old child, diagnosed with hemophilia A, is in the emergency department after experiencing a fall on the school playground. Which of the following laboratory data would the nurse expect to see? a. Leukocyte count 15,000 cells/mm3 b. Partial prothrombin time (PTT) 90 sec (normal 60-70 sec) c. Platelet count 75,000 cells/mm3 d. Prothrombin time 9 sec (normal 11-12.5 sec)
b
A child with a history of fever of unknown origin, excessive bruising, lymphadenopathy, fatigue is exhibiting symptoms most suggestive of? a. Ewing's sarcoma b. Leukemia c. Wilms' tumor d. Neuroblastoma
b
Nursing strategies to improve the growth and development of the child with human immunodeficiency virus (HIV) infection should include what? a. Provide only those foods that the child feels like eating. b. Fortify foods with nutritional supplements to maximize quality of intake. c. Weigh the child and measure height and muscle mass on a daily basis. d. Provide high-fat and high-calorie meals and snacks to meet body requirements for growth.
b
The nurse is caring for a school-age child with severe anemia and activity intolerance. What diversional activity should the nurse plan for this child? a. Playing a musical instrument b. Playing board or card games c. Participating in a game of table tennis d. Participating in decorating the hospital room
b
The regulation of red blood cell (RBC) production is thought to be controlled by which physiologic factor? a. Hemoglobin b. Tissue hypoxia c. Reticulocyte count d. Number of RBCs
b
these S/S match which anemia deficiency: cool skin, pallor, decrease pulses, decreased BP a. increase RBC destruction b. increase RBC loss c. decreased RBC production
b
A child with sickle cell disease is in a vasoocclusive crisis. What nonpharmacologic pain intervention should the nurse plan? a. Exercise as a distraction b. Heat to the affected area c. Elevation of the extremity d. Cold compresses to the affected area
b - Frequently, heat to the affected area is soothing. Cold compresses are not applied to the area because doing so enhances vasoconstriction and occlusion. Bed rest is usually well tolerated during a crisis, although the actual rest obtained depends a great deal on pain alleviation and the use of organized schedules of nursing care. Although the objective of bed rest is to minimize oxygen consumption, some activity, particularly passive range of motion exercises, is beneficial to promote circulation. Usually the best course is to let children determine their activity tolerance. Elevating the extremity will not help in sickle cell disease.
The clinic nurse is evaluating lab results for a child. What recorded hematocrit (Hct) result is considered within the normal range? a. 30% b. 40% c. 50% d. 60%
b - Normal hematocrit (Hct) is 35% to 45%.
What statement is descriptive of most cases of hemophilia? a. X-linked recessive deficiency of platelets causing prolonged bleeding b. X-linked recessive inherited disorder in which a blood clotting factor is deficient c. Autosomal dominant deficiency of a factor involved in the blood-clotting reaction d. Y-linked recessive inherited disorder in which the red blood cells become moon shaped
b - The inheritance pattern in 80% of all 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 blood cells or the Y chromosome.
how do you DX anemia?
get labs and see CBC of RBC les than 10/11 and by looking at the hands and bottom of feet seeing a change in skin color
what is a key diagnostic for sickle cell anemia?
getting cord blood in newborns
how do you treat anemia
With a transfusion of blood or erythropoetin, IV fluids, o2, diet and bed rest
what is the diagnostic of severe combined immunodefiencey disease
history of recurrent infection and not ever fully recovering
when is it most probable that the child will get a splenectomy ?
if they continue to have episodes by age 6
iron deficiency anemia is caused by what?
inadequate supply of diet iron that could be preventable !!!
why do we never give kids demerol?
it causes seizures
what is the purpose of monitoring the reticulocyte count with children who have sickle cell anemia?
it evaluates the function of the bone marrow
what is this? a genetic disorder that causes abnormal hemoglobin, resulting in some red blood cells assuming an abnormal shape
sickle cell anemia
if a child comes in with 4 or more ear infections in 1 year what may you suspect?
the child has severe combined immunidefiecny disease
if a child comes in with a pattern of infections that reoccur what do you think is going on?
the child has severe combined immunidefiecny disease
if the child comes in with two or more episodes of severe sinusitis in one year, what may you suspect?
the child has severe combined immunidefiecny disease
what history of the child can cause them to be more at risk of getting anemia or a hematologic disorder
- low birth weight - gestational diabetes - if vitamin k wasn't give at birth - sleep/wake cycle - bowel elimination cycle
what are foods that are rich in iron?
- red meat - tuna - salmon - eggs - grains - beans and pea - leafy green veggies
what is an allergic reaction
An immunologic hypersensitivity reaction resulting from the unusual sensitivity of a patient to a particular medication; a type of adverse drug event.
What condition occurs when the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia
b
what are S/S of splenic sequestrian
Profound anemia, hypovolemia , and shock
how do we treat vaso occlusive crisis?
#1: fluids and morphine - bed rest, keep warm, blood transfusion, antibiotics
The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. What statement most accurately reflects inheritance of SCA? a. SCA is not inherited. b. All siblings will have SCA. c. Each sibling has a 25% chance of having SCA. d. There is a 50% chance of siblings having SCA.
c
Which of the following describes why iron-deficiency anemia is common during toddler hood? a. Dietary iron can not be started until 12 months of age b. Iron can not be stored during fetal development c. Milk is a poor source of iron d. Fetal iron stores are depleted by one year of age
c
these S/S match which anemia deficiency: pika, murmur, pallor and increased heart rate a. increase RBC destruction b. increase RBC loss c. decreased RBC production
c
how do you treat allergic reactions?
- epi pen - maintain airway
what are key managements of sickle cell anemia
- splenectomy if patient is having splenic sequentrian - blood transfusion or exchange transfusion that decreases ischemia - monitor the reticulocyte count
what can trigger a sickle cell episode?
- sudden change in temperature, which can make the blood vessels narrow. - very strenuous or excessive exercise, due to shortage of oxygen.
what causes aplastic Crisis?
- viral infection - decrease in folic acid
how is kids bone marrow output that have SSD
6-8x greater than norm
A pregnant woman with a family history of hemophilia and who has been seen by a genetic counselor makes the following statements. The nurse must clarify the information in which of the statements? a. "Because the disease is X-linked, only my daugters can be born with hemophilia B." b. "Prenatal testing can be performed to determine whether my fetus has hemophilia B." c. "Children with hemophilia B are lacking one of the important factors needed to clot blood." d. "Some children with hemophilia B have worse bleeding problems than other children with the same genetics."
a
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
a
The nurse is planning care for an adolescent with acquired immunodeficiency syndrome. What is the priority nursing goal? a. Prevent infection. b. Prevent secondary cancers c. Identify source of infection. d. Restore immunologic defenses.
a
What explanation provides the rationale for why iron-deficiency anemia is common during infancy? a. Cow's milk is a poor source of iron. b. Iron cannot be stored during fetal development. c. Fetal iron stores are depleted by 1 month of age. d. Dietary iron cannot be started until 12 months of age
a
What pain medication is contraindicated in children with sickle cell disease (SCD)? a. Meperidine (Demerol) b. Hydrocodone (Vicodin) c. Morphine sulfate d. Ketorolac (Toradol)
a
these S/S match which anemia deficiency: dark urine, hepatomegaly, fatigue a. increase RBC destruction b. increase RBC loss c. decreased RBC production
a
A 5-year-old child is admitted to the hospital in a sickle cell crisis. The child has been alert and oriented but in severe pain. The nurse notes that the child is complaining of a headache and is having unilateral hemiplegia. What action should the nurse implement? a. Notify the health care provider. b. Place the child on bed rest. c. Administer a dose of hydrocodone (Vicodin). d. Start O2 per the hospital's protocol.
a - Any number of neurologic symptoms can indicate a minor cerebral insult, such as headache, aphasia, weakness, convulsions, visual disturbances, or unilateral hemiplegia. Loss of vision is usually the result of progressive retinopathy and retinal detachment. The nurse should notify the health care provider
A toddler has been diagnosed with iron-deficiency anemia. Which of the following information should the nurse educate the parents regarding medication administration? a. Have the child drink orange juice right after he takes his medicine b. Administer the medicine right before his meals c. Crush the tablets and mix the medicine with his applesauce d. Add the iron elixir to his morning bottle
a - because it helps with absorption
The parents of a hospitalized child 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 given as a last resort because of the threat of addiction b. Are often ordered but not usually needed c. Rarely cause addiction because they are medically indicated in a crisis d. Are used only if other pain management efforts are ineffective
c
What are the nursing priorities for a child with sickle-cell disease in vaso-occlusive crisis? a. Intravenous factor for clotting b. Blood transfusions and an increased caloric intake c. Hydration and pain management d. Antibiotics and nebulizer treatments
c
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
A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse? a. Administer 100% oxygen to relieve hypoxia. b. Notify the practitioner because chest syndrome is suspected. c. Infuse intravenous antibiotics as soon as cultures are obtained. d. Give ordered pain medication to relieve symptoms of pain episode.
b - These are the symptoms of chest syndrome, which is a medical emergency. Notifying the practitioner is the priority action. Oxygen may be indicated; however, it does not reverse the sickling that has occurred. Antibiotics are not indicated initially. Pain medications may be required, but evaluation by the practitioner is the priority.
in anticipation of the admission of a child with hereditary spherocytosis (HS) who is experiencing an aplastic crisis, what action should the nurse plan? a. Secure an isolation room. b. Prepare for a transfusion of packed red blood cells. c. Anticipate preoperative preparation for a splenectomy. d. Gather equipment and medication for treatment of shock.
b - aplastic crisis results in a sudden cessation of RBC production by the bone marrow. Hemoglobin and hematocrit values drop rapidly, which results in severe anemia. Transfusion support may be needed, and close monitoring of the child's cardiovascular status is necessary. The nurse should prepare for a transfusion of packed red blood cells initially
what is the number one cause of death with sickle cell anemia
bacterial infections
how can iron deficiency anemia be caused by 12-36 month old babies?
because of the large amount of cow milk that they consume
A child with severe anemia requires a unit of red blood cells (RBCs). The nurse explains to the child that the transfusion is necessary for which reason? a. Allow her parents to come visit her. b. Fight the infection that she now has. c. Increase her energy so she will not be so tired. d. Help her body stop bleeding by forming a clot (scab).
c
A four year old girl is admitted to the hospital with sickle cell disease. Her vital signs are T 37.2 C, HR 124, RR 40, BP 68/40. She is pale, listless and has splenomegaly. She is experiencing a. Vaso-occlusive Crisis b. Acute Chest Syndrome c. Acute Sequestratian Crisis d. Aplastic Crisis
c
A mother states that she brought her child to the clinic because the 3-year-old girl was not keeping up with her siblings. During physical assessment, the nurse notes that the child has pale skin and conjunctiva and has muscle weakness. The hemoglobin on admission is 6.4 g/dl. After notifying the practitioner of the results, what nursing priority intervention should occur next? a. Reduce environmental stimulation to prevent seizures. b. Have the laboratory repeat the analysis with a new specimen. c. Minimize energy expenditure to decrease cardiac workload. d. Administer intravenous fluids to correct the dehydration.
c
The nurse is evaluating the activity tolerance of a 9-month-old with iron-deficiency anemia. The finding that indicates the child is tolerating activity is a. Tachycardia, HR of 150 b. Decreased alertness c. Respiratory rate less than 50 with activity d. Muscle weakness
c
The nurse is teaching a parent of an infant to limit the amount of formula to encourage the intake of iron-rich food. What amount should the nurse teach to the parent? a. 500 ml b. 750 ml c. 1000 ml d. 1250 ml
c
In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxia-ischemia cycle. What information should the nurse share with parents in a teaching plan? a. Encourage drinking. b. Keep accurate records of output. c. Check for moist mucous membranes. d. Monitor the concentration of the child's urine.
c - Children with SCA have impaired kidney function and cannot concentrate urine. Parents are taught signs of dehydration and ways to minimize loss of fluid to the environment. Encouraging drinking is not specific enough for parents. The nurse should give the parents and child a target fluid amount for each 24-hour period. Accurate monitoring of output may not reflect the child's fluid needs. Without the ability to concentrate urine, the child needs additional intake to compensate. Dilute urine and specific gravity are not valid signs of hydration status in children with SCA.
What statement best describes -thalassemia major (Cooley anemia)?a. It is an acquired hemolytic anemia. b. Inadequate numbers of red blood cells (RBCs) are present. c. Increased incidence occurs in families of Mediterranean extraction. d. It commonly occurs in individuals from West Africa
c - Individuals who live near the Mediterranean Sea and their descendants have the highest incidence of thalassemia. Thalassemia is inherited as an autosomal recessive disorder. An overproduction of RBCs occurs. Although numerous, the red blood cells are relatively unstable. Sickle cell disease is common in blacks of West African descent.
The clinical manifestations of sickle cell anemia (SCA) are primarily the result of which physiologic alteration? a. Decreased blood viscosity b. Deficiency in coagulation c. Increased red blood cell (RBC) destruction d. Greater affinity for oxygen
c - The clinical features of SCA are primarily the result of increased RBC destruction and obstruction caused by the sickle-shaped RBCs. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. SCA does not have a coagulation deficit. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension.
A 12-week-gestation African-American woman asks her obstetrician's nurse whether her baby could be born with sickle cell disease. Which of the following replies is appropriate for the nurse to give? a. If either you or the baby's father has sickle cell anemia, your child may be born with the disease b. If the child is a boy, he could have sickle cell anemia, but if the child is a girl, she will definitely be healthy c. It is possible because one out of every 500 African Americans is diagnosed with sickle cell anemia d. The baby could only have sickle cell anemia if both you and the baby's father carry a sickle cell gene
d
A child with hemophilia A will have which abnormal laboratory result? a. PT (ProTime) b. Platelet count c. Fibrinogen level d. PTT (partial thromboplastin time)
d
The nurse is preparing a community outreach program about the prevention of iron-deficiency anemia in infants. What statement should the nurse include in the program? a. Whole milk can be introduced into the infant's diet in small amounts at 6 months. b. Iron supplements cannot be given until the infant is older than 1 year of age. c. Iron-fortified cereal should be introduced to the infant at 2 months of age. d. Breast milk or iron-fortified formula should be used for the first 12 months.
d
What condition is an inherited immunodeficiency disorder characterized by absence of both humoral and cell-mediated immunity? a. Fanconi syndrome b. Wiskott-Aldrich syndrome c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency syndrome (SCIDS)
d
What physiologic defect is responsible for causing anemia? a. Increased blood viscosity b. Depressed hematopoietic system c. Presence of abnormal hemoglobin d. Decreased oxygen-carrying capacity of blood
d
The nurse is caring for a child with hemophilia A. The child's activity is as tolerated. What activity is contraindicated for this child? a. Ambulating to the cafeteria b. Active range of motion c. Ambulating to the playroom d. Passive range of motion exercises
d - Passive range of motion exercises should never be part of an exercise regimen after an acute episode because the joint capsule could easily be stretched and bleeding could recur. Active range of motion exercises are best so that the patient can gauge his or her own pain tolerance. The child can ambulate to the playroom or the cafeteria.
What statement best describes iron deficiency anemia in infants? a. It is caused by depression of the hematopoietic system. b. Diagnosis is easily made because of the infant's emaciated appearance. c. It results from a decreased intake of milk and the premature addition of solid foods d. Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.
d - it is not C because its from an over consumption amount of milk
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 colony-stimulating 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.
The clinic nurse is evaluating lab results for a child. What recorded hemoglobin (Hgb) result is considered within the normal range? a. 9 g/dl b. 10 g/dl c. 11 g/dl d. 12 g/dl
d -Normal hemoglobin (Hgb) determination is 11.5 to 15.5 g/dl
What condition precipitates polycythemia? a. Dehydration b. Severe infections c. Immunosuppression d. Prolonged tissue hypoxia
d -Oxygen transport depends on both the number of circulating RBCs and the amount of normal hemoglobin in the cell. This explains why polycythemia (increase in the number of erythrocytes) occurs in conditions characterized by prolonged tissue hypoxia, such as cyanotic heart defects. Dehydration, severe infections, or immunosuppression will not precipitate polycythemia.
how can iron deficiency anemia be caused by adolescents?
due to the rapid growth and poor diet
