MB4 hematology in children

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MB4 hematology in children
2013-04-09 22:01:18
pediatric hematologic disorders nursing

Hematologic disorders in Peds for mother baby test 4
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  1. When is Fe-deficiency anemia rarely seen?

    before age 4 to 6 mo b/c of maternal Fe stores
  2. When does a baby become at risk for Fe deficiency anemia?
    age 9-24 months mo when maternal iron stores are depleted

    this is when parents start to notice it
  3. Main problem of IDA?

    Why does this occur?
    iron deficiency anemia

    compromised tissue oxygenation - esp to the brain

    B/D of RBC exceeds ability to make new RBC's -> decreased synth of HgB -> decreased ability to carry O2
  4. Why may premature and low birth weight babies have iron deficiency anemia?

    How is this treated?
    b/c they may not have iron stores built up like normal babies do

    put them on iron around 2 mo of age
  5. Why can excessive intake of cow's milk cause?

    iron deficiency anemia

    protein in the milk can cause diarrhea and GI bleeding; also Ca in the milk binds with iron and interferes with its absorption
  6. At what age can a child have cow's milk?

    How much is OK?
    12 mo and over

    should not have more than 24oz per day
  7. Who is most at risk for decreased iron intake?
    adol b/c of poor dietary habits
  8. Who is most at risk for iron deficiency anemia due to iron loss?
    adol girls - menstruation + increased growth rate
  9. 2 periods of increased growth rate that may contribute to iron deficiency anemia?
    • 1. adolescents
    • 2. premature and low birth weight infants
  10. At what age do they start preemies on iron supplements?
    2 months
  11. Why do periods of increased growth cause iron deficiency anemia?
    as they grow their need for RBC's  increases
  12. 5 things that cause iron deficiency anemia?
    • 1. premature/low birth weight
    • 2. excessive intake of cow's milk
    • 3. decreased Fe intake
    • 4. increased Fe lost (menstruation)
    • 5. periods of increased growth rate
  13. S/S of iron deficiency anemia?
    • 1. Skin - pallor, pale, porcelain skin
    • 2. conjunctiva - sclera is pale
    • 3. MM - pale
    • 4. CV - tachycardia, cardiomegaly, heart murmur
    • 5. Respiratory - tachypnea, dyspnea upon minimal exertion
    • 6. CNS - poor school performance
    • 7. Nails - koilonychias/spoon nail (not same as clubbing with COPD)
    • 8. lethargy, fatigue
    • 9. slow, strained movement
    • 10. irritable
    • 11. difficulty sucking b/c no energy
    • 12. sagging limp posture
  14. Why does tachycardia occur in anemia?
    to compensate for decreased RBC & decreased tissue perfusion
  15. When may a heart murmur occur with anemia?

    How is this resolved?

    Teaching for parents?
    with severe anemia

    will go away when anemia is reversed

    tell parents that it is not permanent or a defective heart
  16. Why may a baby/child lose weight due to anemia?
    they don't have enough energy to eat/suck bottle
  17. Koilonychias?
    spoon nail that may occur with long-term anemia
  18. How to assess fatigue and other S/S of anemia?
    • Ask parents about the child's:
    • 1. eating habits
    • 2. crying and movememnts
    • 3. irritability and crying
    • 4. feeding/sucking
    • 5. how much the sleep/nap
  19. Dx evaluation of Fe deficiency anemia?
    1. have them keep a 24 h food dietary Hx including foods eaten & description of child's activities during that day

    2. lab tests: HgB, morphologic changes in RBC (color, size, etc), Fe concentration
  20. Level of HgB considered low?
    6 - 11
  21. RBC appearance in anemia?
    microcytic and hypochromic
  22. Changes in reticulocytes in anemia?
    may be normal or slightly reduced due to decreased stores of iron or could be elevated in severe cases r/t hypoxia eliciting an erythropoietic response
  23. Serum iron level in iron deficiency anemia?
  24. What happens to proteins available to bind iron in iron deficiency anemia?

    What does this do?

    they will increase -> increased iron binding capacity r/t decrease serum iron levels

  25. 2 Tx for iron deficiency anemia?
    • 1. dietary intake
    • 2. supplement
  26. Why are iron supplements necessary in iron deficiency anemia?
    dietary intake is unreliable
  27. 4 foods that inhibit iron absorption?
    • 1. spinach
    • 2. sweet potato
    • 3. coffee
    • 4. tea
  28. 7 foods that are iron rich?
    • 1. egg yolks
    • 2. dried beans & peas
    • 3. cream of wheat
    • 4. dried fruit
    • 5. liver
    • 6. Fe-fortified cereal
    • 7. green leafy veggies:  broccoli; romaine/green leaf lettuce, collard greens, mustard greens
  29. Consideration with children and dried fruits?
    good for iron but can get stuck in teeth and cause cavities - if you can use another source of iron it will be better
  30. How is Fe supplement given?
    tid through a straw, dropper, or syringe

    place at back of mouth and away from teeth

    Brush or wipe off teeth
  31. Consideration with liquid iron supplements?
    can stain teeth- keep away from them and wipe off after admin
  32. Way to increase iron absorption?
    admin with vit C
  33. What vitamin enhances Fe absorption?
    Vit C
  34. What juices have a lot of Vit C?
    • 1. orange juice
    • 2. tomato juice
    • 3. grapefruit juice
  35. Juices with low Vit C juices?
    pear, apple, and peach
  36. What is SCA?
    sickle cell anemia - 1 of the groups of SCD's (sickle cell diseases)
  37. Hemoglobinopathies?
    genetic disease caused by or associated with the presence of one of several forms of abnormal hemoglobin in the blood:  SCD, SCA, etc
  38. SCD?
    sickle cell disease - broad category of hemoglobanemias
  39. 3 common forms of SCD?
    • 1. sickle cell anemia
    • 2. sickle C disease
    • 3. sickle beta-thalassemia syndrome
  40. SCA caused by?
    abnormality in Hgb -> HgbS
  41. Cause of sickle C disease?
  42. Most common SCD in children?
    sickle cell anemia
  43. 2 things all 3 forms of SCD have in common?
    1. all are inheritied autosomal recessive

    2. all are life-long diseases
  44. Normal HgB is HgB ____ and abnormal HgB is _____.
    normal - HgB A

    abnormal - HgbS
  45. What is caused by sickled cells?

    occlusion of vessels -> tissue ischemia distal to the occlusion

    if occlusion is in renal artery will cause renal failure
  46. Who has most incidence of SCA?

    AA - 3 in 1000 have SCA and 1 in 12 have the trait
  47. Ppl at risk for sickle cell anemia?
    • 1. african americans
    • 2. mediteranean-italians and greeks
    • 3. east indian
    • 4. middle eastern
    • 5. caribbean
    • 6. central and south american
  48. Patho of SCA?
    sickled/elongated cell -> stasis of RBC's & increased blood viscosity -> obstructed blood flow (esp. in bifurcations) -> tissue ischemia distal to the obstruction -> acute pain & cell infarction/cell death
  49. Where is obstruction due to SCA likely to occur?
    in bifurcations of vessels
  50. Pain with sickle cell anemia?
    will be extreme and occur wherever the occlusion is located
  51. Where does ischemia with sickle cell occur?
    distal to the occlusion site
  52. 3 tests for SCA?
    • 1. HgB electrophoresis
    • 2. Sickledex - sickle solubility test
    • 3. Reticulocyte count will be elevated
  53. Definitive test for SCA?

    HgB electrophoresis - shows have SCA trait
  54. What does the Sickledex test show?
    sickle solubitily test- detects presence of HgbS but not the trait for SCA
  55. Why is reticulocyte count elevated in SCA?
    a normal RBC lives 120 days/4 months and a sickled RBC lives for 12 days -> need more RBC production
  56. 2 factors that cause the manifestations of SCA?
    1. obstructin caused by the sickled RBC leads to tissue ischemia and infarction of cell death distal to where the obstruction is (can be anywhere)

    2. increased RBC destruction
  57. What is sickle cell crisis?
    a broad term for several acute/exacerbating conditions that occur in with sickle cell anemia
  58. 5 causes of sickle cell crisis?
    • 1. infection
    • 2. dehydration (overwork)
    • 3. hypoxia (increased elevation)
    • 4. trauma
    • 5. general stress
  59. 3 types of sickle cell crisis or complications?
    Which is most common?
    • 1. vaso-occlusive crisis - most common
    • 2. acute sequestration crisis
    • 3. aplastic crisis
  60. 6 S/S and complications of vaso-occlusive crisis?
    • 1. pain - usually in bone and joint but can occur anywhere and is mild to severe
    • 2. dactylitis - inflammation of fingers and toes
    • 3. acute chest syndrome - chest pain, cough
    • 4. fever
    • 5. severe abd pain
    • 6. priapism
    • 7. CVA
  61. Most serious complication of vaso-occlusive crisis?

    7 S/S?

    • 1. altered LOC
    • 2. vision changes
    • 3. headache
    • 4. hemi or monoparesis
    • 5. aphasia/dysphasia
    • 6. ataxia- can't coordinate muscles
    • 7. seizures
  62. Hemiparesis?

    weak or paralyzed on 1 side of the body

    AKA monoparesis
  63. Aphasia/dysphasia?
    difficulty speaking
  64. What is aphasia where pt can talk clear, understand what you said, but does not make any sense?
    expressive aphasia
  65. Receptive aphasia?
    do not understand what is being said
  66. What occurs in carrier state of SCA?
    usually do not have S/S but in extreme stress may have some:  extreme vigorous exercise & high altitudes
  67. 2 characteristics of acute sequestration crisis?
    all of the blood is sequestered in the spleen -> splenomegaly & hypovolemic shock due to all the blood being in spleen
  68. 6 S/S of aplastic crisis?
    • 1. pallor
    • 2. fatigue
    • 3. lethargy
    • 4. HA
    • 5. fainting
    • 6. short of breath or breathlessness
  69. What occurs in aplastic crisis?
    hemolytic anemia (hemolysis) -> decreased HgB-> aplastic crisis
  70. HgB level in aplastic crisis?
    6 to 9 g/dL
  71. 9 Tx management of SCA crisis of any kind?
    • 1. monitor vital signs q 4 h and more frequently prn
    • 2. bed rest
    • 3. O2 sat 96 and up (above 95%)
    • 4. PAIN CONTROL - around the clock
    • 5. incorporate the use of age-appropriate non-pharmacologic pain relief measures
    • 6. Apply heat to painful joints prn
    • 7. HYDRATION - D5W1/2NS 1 to 1/2 times the maintenance rate:  need calories and cells to be hydrated & bloodstream hydrated
    • 8. Strict I&O:  maintain urine output of at least 1mL/kg/h
    • 9. Perform passive ROM
    • 10. Meds:  hydroxyrea, folic acid
    • 11. RBC transfusion last resort
    • 12. instruct to eat foods high in folic acid
  72. Pt with SCA are at risk for ______.

    risk for infection r/t alteration in splenic function from crisis of acute sequestration
  73. What may occur in acute sequestration?
    total splenic dysfunction by age 5 years -> risk for infection
  74. Why are pt's put on bedrest with SCA crisis?
    reduce O2 expenditure
  75. O2 sat for SCA crisis?
    above 95% (96% and up)
  76. D5W 1/2 NS is a _____ solution.
    hypotonic - brain will use the dextrose immediately & will have 1/2 NS left
  77. Why does O2 sat in SCA crisis need to be above 95%?
    to ease WOB & facilitate tissue perfusion & to prevent further sickling

    will not reverse sickling that has already occured
  78. How is SCA crisis pain usually controlled?

    morphine around the clock - maintain a cont. analgesic blood level & reduce metabolic demand for O2 & help to end sickling
  79. Good non-pharm pain relief measures?
    • 1. distraction - age appropriate
    • 2. apply heat to painful joints prn - microwave a wet towel
    • 3. be sympathetic
  80. Why may heat be applied for SCA crisis?
    will increase blood flow (vasodilation) & perfusion to the ischemic tissues
  81. Why is hydration important in SCA crisis?
    reduce the viscosity of blood and alleviate sites of vascular occlusion and prevent further sickling caused by dehydration
  82. Urine output in SCA crisis should be maintained at______.


    to monitor kidney failure that can result from infarction of the renal artery
  83. How are I&O's measured in babies and children?
    • 1. weigh diapers
    • 2. have parents catch it in a hat or urinal
  84. Why is passive ROM performed for SCA crisis pt?
    to promote circulation without exacerbating fatigue and increasing O2 needs
  85. What does hydroxyrea do?
    increases HgB levels
  86. Why may anemia and SCA pt be given folic acid?
    folic acid is necessary for the formation of RBC's
  87. What type of RBC transfusions may be given to SCA crisis pt?

    leukocyte-poor or washed RBC's

    blood that has the WBC's removed as much as possible to try and prevent a transfusion reaction r/t SCA more at risk for transfusion reactions b/c of their repeated exposure to blood transfusions
  88. Why may blood transfusions be done in SCA crisis?
    to maintain adequsate HgB level which increases O2 carrying capacity to aid in further sickling and microvascular ischemia
  89. Nursing consideration for a pt who is receiving a blood transfusion and has had them in the past?
    at risk for transfusion reaction - RN MUST do v/s and stay in room
  90. 8 foods high in folic acid?
    • 1. green leafy veggies
    • 2. broccoli
    • 3. oranges, cantaloupe
    • 4. nuts
    • 5. peanut butter
    • 6. dried beans and peas
    • 7. enriched breads and cereals
    • 8. enriched pastas and grains
  91. When does risk for infection with SCA start?

    What will eventually occur?
    risk for infection starts as early as 6 mo of age and may have total dysfunction by 5 years old - r/t chronic immunocompromised state due to splenic damage
  92. Tx for risk for infection with SCA?
    daily penicillin therapy starting at 2 mo old and cont. until at least 5 years old or throughout childhood
  93. 3 immunizations need with SCA?
    • 1. pneumococcal
    • 2. meningococcal
    • 3. influenza
  94. 12 things to teach pt of child with SCA for home care?
    • 1. prevent dehydration with fluids
    • 2. expect frequent urination
    • 3. provide rest
    • 4. avoid cold and hot
    • 5. avoid infections
    • 6. avoid prolonged exposure to the sun
    • 7. monitor body temp & notify MD of fevers & do not use antipyretics until talked to someone
    • 8. Admin penicillin qd as ordered
    • 9. avoid high elevations
    • 10. S/S of infections - call MD
    • 11. know MD phone number
  95. Why do SCA anemia pt need to avoid cold and hot temperatures?
    cold can cause sickling

    hot can cause dehydration which can lead to sickling
  96. Beta-thalassemia?
    inherited disorder/autosomal recessive that is char by abnormal HgB synth
  97. 3 groups that get beta-thalassemia the most?
    • 1. Mediterranean
    • 2. Asian
    • 3. African American
  98. Onset of beta-thalassemia?
  99. When does beta-thalassemia normally occur/get caught?
    late infancy or early toddlerhood
  100. How long does beta-thalassemia last?
    throughout life
  101. Major complication of beta-thalassemia?
    heart and/or liver failure by 20 to 30 years of age
  102. 3 differences in SCA and beta-thalassemia?
    • 1. heart/liver failure most common in beta-thalassemia
    • 2. growth retardation in beta-thalassemia
    • 3. head morphological changes in beta-thalassemia
  103. Facial char of pt with beta-thalassemia?
    • 1. enlarged head
    • 2. frontal and parietal bossing - enlargement
    • 3. severe maxillary hyperplasia - top bucked
    • 4. malocclusion - misalignment of teeth
    • 5. bronze skin tone
  104. 4 clinical manifestations of beta thalassemia?
    • 1. severe anemia
    • 2. growth maturation retardation
    • 3. char face
    • 4. hepatosplenomegaly
  105. beta-thalassemia AKA?
    thalassemia major & Cooley's anemia
  106. 3 Tx for beta-thalassemia?
    • 1. erythrocyte transfusion
    • 2. chelation therapy - remove heavy metals/iron
    • 3. splenectomy
  107. Why is chelation therapy needed for beta-thalassemia?
    leach off Fe b/c will have overload from too many transfusions
  108. Pt is at risk for _____ if they have had a splenectomy.
  109. Hemophilia?
    X-linked autosomal recessive disorder that occurs predominantly in men and women are mostly carriers
  110. Cure for hemophilia?
    no cure
  111. What does hemophilia cause?
    deficiency in one of factors for blood coagulation
  112. 3 types of hemophilia?
    • 1. hemophilia A - classic - factor VII
    • 2. hemophilia B - factor IX
    • 3. factor XI
  113. Classifications of hemophilia?
    • 1. mild - bleeding occurs with severe trauma
    • 2. moderate - bleeding occurs with minor trauma
    • 3. severe - bleeding occurs with no trauma/SPONTANEOUS bleeding
  114. With each pregnancy a woman carrier of hemophilia has a __% chance of having a hemophilic son.
  115. If a father has hemophilia what will his daughters have?

    What about his sons?
    all of his daughters will be carriers and none of his sons will be affected
  116. 4 clinical manifestations of hemophilia?
    • 1. newborn - umbilical bleeding or prolonged bleeding with circumcision
    • 2. bruises easily
    • 3. epistaxis - nose bleeds
    • 4. bleeding anywhere - most common sites:  muscles and joints
  117. Epistaxis?
    nose bleed
  118. hemarthrosis?
    bleeding in joints that will cause swelling of the joint
  119. 4 components of Dx of hemophilia?
    • 1. Hx of bleeding episodes in newborn or toddler
    • 2. tests of hemostasis
    • 3. tests for blood clotting
    • 4. tests of clotting factor function
  120. 2 tests for hemostasis and results that indicate hemophilia?
    platelet aggregation - prolonged

    bleeding time - increased
  121. 2 tests for blood clotting and results that indicate hemophilia?
    • 1. prothrombin time - PT- increased
    • 2. partial thromboplastin time - PTT- increased
  122. Definitive test for hemophilia?

    What is tested?
    factor assays- test for clotting factor function & will show what factor is being affected
  123. 2 components in therapeutic management of hemophilia?

    1. classification of bleeding will det Tx:  mild, moderate, severe

    2. type of deficient clotting factor - will give factor replacement
  124. Factor replacement therapy in hemophilia?
    replace factor with human blood and/or recombinant clotting factors through IV over 5 minutes

    very expensive 100,000 to 200,000 dollars per year
  125. Therapy for severe bleeding in hemophilia?
    factor replacement IV over 5 min.

    Children may do this themselves at 8 years and above - may go to hemophilia camps

    * RICE - rest for 24 to 48 h, ice, compression, elevation
  126. Tx for mild to moderate bleeding in hemophilia?
    desmospressin/DDAVP - stimulates release of factor VIII for hemophilia A

    route - intranasal - need to keep up with how many sprays used
  127. Why do you need to specify how long child should rest if bleeding occurs?
    don't want them to get stiff
  128. Things to avoid with hemophilia child?
    • 1. toys with sharp edges
    • 2. sharp scissors
    • 3. straight razors
    • 4. NO IM or IV unless necessary
    • 5. no ASA or ibuprofen
    • 6. finger sticks (use venipuncture instead b/c will bleed less)
  129. 7 nursing instructions for hemophilia pt?
    • 1. use soft toothbrush and water irrigating device
    • 2. electric razor
    • 3. wear helmut on bike
    • 4. wear joint pads when playing
    • 5. padded crib sides
    • 7. medical ID bracelet
    • 8. keep on hand at all times: bags of ice or cold packs in freezer
  130. Immune thrombocytopenic purpura?

    idiopathic thrombocyopenic purpura/hemorrhagic disorder
  131. 5 char of immune thrombocytopenic purpura?
    • 1. thrombocytopenia - platelet count below 150,000/mm3
    • 2. purpuric rash
    • 3. normal bone marrow
    • 4. absence of identifiable cause of thrombocytopenia
    • 5. autoimmune response to disease-related antigens
  132. Purpuric rash?
    any rash in wheich blood cells leak into the skin or mucus membranes, usually at mult sites
  133. 2 types of purpuric skin lesions?
    1. petechiae- pinpoint purpuric lesions

    2. ecchymoses- larger hemorrhages into the skin
  134. What usually occurs right before occurrence of immune thrombocytopenic purpura?
    upper resp infections and/or viral infections:  measels, rubella, mumps, chickenpox, 5th disease or parvovirus B12 (slapped face)
  135. 2 classifications of ITP?
    acute - self - limiting course (1 to 3 mo)

    chronic - lasts longer than 6 mo
  136. Dx eval of ITP?
    1. thorough history:  family Hx, recent meds, recent vaccination w/live virus, recent viral infections

    2. low serum platelet count - <150,000
  137. 3 clinical manifestations of ITP?
    1. bruising, petechiae and/or ecchymoses

    2. bleeding gums

    3. heavy menstrual flow
  138. 2 goals in Tx of ITP?
    1. prevent rare, life threatening bleeding events :  IC bleeding

    2. restore platelet count to above 20,000
  139. 4 nursing interventions for ITP?
    • 1. assess for s/s of bleeding from any body system
    • 2. monitor platelet count
    • 3. no IM injections, ASA, or NSAIDS
    • 4 restrict activity at onset:  no bikes, roller blading etc
  140. 3 possible TX for ITP?
    1. for acute may just watch and wait - monitor bleeding and platelet count

    2. oral or IV corticosteroids:  block autoimmune destruction of platelets

    3. IV admin of immunoglobulins (IVIG)
  141. When my splenectomy be performed for ITP?
    if chronic 1 year or longer and doesn't respond to drug therapy

    will try to wait until at least 5 years old