Hematological Alterations

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Hematological Alterations

• NUR 264
• Pediatrics
• Angela Jackson, RN, MSN

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Hematological Alterations Developmental
Differences

• During fetal development, blood cells are
  produced in the liver and the spleen
• In the newborn, blood cells are produced from
  stem cells in the bone marrow
• Full- term newborns are able to store iron in the
  bone marrow and liver tissue for up to 20 weeks
• Premature newborns may use up their iron stores
  within 6-12 weeks

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Iron Deficiency Anemia

• Most common hematologic disorder of infancy and
  childhood
• The body does not have enough iron to synthesize
  the hemoglobin necessary to carry oxygen to the
  tissues
• Affects 9 of toddlers 1-2 years of age
• Affects 4 of children 3-4 years of age
• Incidence rates are higher in low income families

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Iron Deficiency Anemia Pathophysiology

• Dietary iron is absorbed in the small intestine
  and is either passed into the bloodstream or is
  stored as ferritin
• Iron in the blood stream binds to transferrin and
  is delivered to the bone marrow, where it is
  combined with other components to form hemoglobin
• Iron may also be recycled from RBCs that have
  been removed from the bloodstream and catabolized
• When a child does not ingest enough iron,
  hemoglobin synthesis is impaired
• When a child experiences blood loss, there are
  fewer RBCs to be catabolized and the iron in
  these cells cannot be recycled

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Iron Deficiency AnemiaClinical Manifestations

• Mild anemia
• Generally asymptomatic
• May experience symptoms of moderate anemia during
  exertion
• Moderate anemia
• Shortness of breath
• Rapid, pounding heart beat
• Dizziness, fainting, lethargy, irritability
• Severe anemia
• Cardiac murmurs
• Pale skin, mucous membranes, lips, nail beds and
  conjunctiva
• Thinning and early graying of the hair
• Abdominal pain, nausea, vomiting, anorexia,
  low-grade fever
• Decreased physical growth, developmental delay

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Iron Deficiency AnemiaDiagnosis

• History and physical exam
• Laboratory studies
• Decreased hemoglobin, hematocrit, MCV(RBC
  volume), MCH(weight of Hb within a RBC),
  MCHC(concentration of Hb in a RBC), serum iron,
  serum ferritin
• Microcytic, hypochromic RBCs
• Increased Total iron binding capacity
• Normal retic count
• Normal hemoglobin electrophoresis

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Iron Deficiency AnemiaTreatment

• Infants Breast milk or iron-fortified formula
  until 4-6months, then add iron-fortified cereal
• School-age children and adolescents restrict
  amount of milk, encourage iron-rich foods and
  foods high in vitamin C
• Iron supplements
• Infants and preschoolers 3mg/kg/day
• School-age children 60mg/day
• Adolescents 120mg/day

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Iron-Deficiency AnemiaNursing Considerations

• Family Teaching
• Nutrition foods high and iron, and foods high in
  Vitamin C
• Medication dosages and administration
• Side effects of medication
• Follow-up

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Sickle Cell Anemia

• Autosomal recessive disorder
• Occurs in approximately 1 in every 500
  African-American births and 1 in every 1000-1400
  Hispanic American births
• Sickle cell trait is present in 1 in 12
  African-Americans

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Sickle Cell AnemiaPathophysiology

• One amino acid replaces another, resulting in the
  production of sickle hemoglobin (Hb S)
• This form of hemoglobin contains a semi-solid gel
  that caused the RBC to stretch into a sickle
  shape
• These cells are more stiff and less able to
  change shape, and are unable to pass through the
  microcirculation
• Anemia results from increased RBC destruction,
  worsened by the fact that sickled cells die after
  only 10-20 days

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Sickle Cell Anemia Clinical Manifestations

• Vaso-occlusive crisis aggregation of sickled
  cells within a vessel, causing obstruction.
• Pain crisis
• Hand-foot syndrome
• Acute chest syndrome
• Stroke
• Priapism
• Sequestration crisis excessive pooling of blood
  in the liver and spleen. Decreased blood volume
  may result in shock.
• Aplastic crisis decrease in red blood cell
  production. Results in severe anemia
• Pallor, fatigue, shortness of breath
• Delayed growth
• Delayed onset of puberty

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Sickle Cell AnemiaDiagnosis

• Family history and clinical manifestations
• Newborn Screening
• Lab tests
• Increased retic count
• Decreased Hgb Hct and TIBC
• Hemoglobin electrophoresis reveals predominantly
  Hb S
• Normocytic, normochromic, sickle shaped cells
• Normal MCV, MCH, MCHC
• Normal serum iron and serum ferritin

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Sickle Cell AnemiaTreatment

• Primary treatment is prevention of RBC sickling
• Avoidance of fever, infection, acidosis,
  dehydration, constrictive clothing and exposure
  to cold
• Immunization
• Prophylactic oral penicillin until age 5
• Routine blood transfusions for children at high
  risk of CVA

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Sickle Cell AnemiaNursing Considerations

• Promote comfort
• Administer pain medication routinely instead of
  PRN
• Apply heat to painful areas
• Allow child to determine amount of activity
  tolerated
• Provide passive ROM exercises
• Administer IV fluids as ordered to maintain
  hydration
• Prevent infection
• Administer antibiotics as ordered
• Frequent hand washing
• Proper aseptic techniques
• Provide education
• Maintain adequate hydration
• Avoid sources of infection
• Promote proper nutrition
• Signs and symptoms of crisis
• Administration of prophylactic medications

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Hemophilia

• Group of bleeding disorders in which one factor
  in the first phase of coagulation is deficient
• Most common type is hemophilia A (deficiency of
  factor VIII), occurs in approximately 15000
  males
• Hemophilia B (Christmas disease, deficiency of
  factor IX) second most common type, affects
  10-15 of people with hemophilia
• Least common type is hemophilia C (deficiency of
  factor XI)
• Hemophilia A and B are x-linked recessive
  disorders, affecting mostly males. Hemophilia C
  is an autosomal recessive disorder that affects
  males and females equally

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HemophiliaPathophysiology

• One factor of the first phase of the intrinsic
  pathway is deficient
• The body is unable to form clots to repair
  damaged blood vessels
• Bleeding episodes occur

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Hemophilia Clinical Manifestations

• Clinical manifestations vary based on severity of
  the disease
• Mild excessive bleeding only after severe trauma
  or surgery
• Moderate excessive bleeding only after trauma
• Severe excessive bleeding occurs spontaneously
• Hematomas may result from injections or firm
  holding during the first year of life

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HemophiliaClinical Manifestations

• As the child learns to walk, bruising occurs
  easily, and hemarthrosis may develop
• Persistent bleeding from minor lacerations begin
  to occur by 3-4 years of age
• Minor occurrences of hematuria, epistaxis as well
  as major events such as intracranial hemorrhage
  and bleeding into the neck or abdomen may also
  occur

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HemophiliaDiagnosis

• History and physical exam
• Lab tests
• Prolonged PTT
• Normal platelet count, standard bleeding time,
  and PT

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HemophiliaTreatment

• Hemophilia A
• Replacement of missing coagulation factor through
  infusion of recombinant factor VIII concentrates
• May be administered three or four times/week
• Desmopressin (DDAVP) is effective for spontaneous
  bleeding and in preventing bleed prior to dental
  or surgical procedures

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HemophiliaTreatment

• Hemophilia B
• Replacement of factor IX through plasma derived
  concentrates
• DDAVP is not effective
• Hemophilia C Aggressive therapy is usually
  unnecessary because of the mild nature of the
  disease

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HemophiliaNursing Considerations

• Prevent bleeding
• Make the environment as safe as possible for
  infants learning to walk
• Encourage noncontact sports in the school age
  child
• Soft-bristled toothbrushes for mouth care and
  electric razors for shaving
• Prevent IM injections whenever possible
• Wear medic alert bracelet

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Hemophilia Nursing Considerations

• Recognize and manage bleeding
• Make family aware of signs and symptoms of
  internal bleeding such as headache, slurred
  speech, loss of consciousness, black tarry
  stools, hematemesis, hematuria
• Factor replacement should be instituted according
  to established medical protocol
• Apply pressure to the bleeding area for at least
  10-15 minutes
• Immobilize and elevate the area above the level
  of the heart to decrease blood flow
• Apply cold to promote vasoconstriction

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HemophiliaNursing Considerations

• Prevent crippling effects of joint degeneration
  resulting from hemarthrosis
• Elevate and immobilize the joint
• Passive range of motion after the acute phase
• Physical therapy
• Nutrition counseling to maintain optimal weight
• May need orthopedic intervention such as casting,
  traction or aspiration of blood to preserve joint
  function

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HemophiliaNursing considerations

• Family teaching
• Administration of antihemophilic factor
  concentrates
• Methods to prevent or stop bleeding
• Signs and symptoms that indicate an emergent
  situation and the need for immediate physician
  intervention
• Provide support
• Genetic counseling

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Immune Thrombocytopenic Purpura (ITP)

• Acquired disease characterized by
  thrombocytopenia and purpura
• Autoimmune disorder
• Peak incidence between 2 and 4
• 75 of children recover completely in 3 months
• 80-90 have regained normal platelet counts
  within 6 months

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ITPPathophysiology

• Platelets adhere to injured vessel walls, release
  biochemical mediators, and form plugs, blocking
  minute ruptures occurring in the microcirculation
• Inadequate numbers of platelets result in purpura
  under the skin and throughout the tissues
• This occurs as the result of an autoimmune
  process that is triggered by a viral infection
• Antiplatelet antibodies bind to the platelets,
  sequestering and destroying them in the spleen

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ITPClinical Manifestations

• Ecchymoses, general petechial rash occurring 1-4
  weeks following a viral infection
• Asymmetrical bleeding, especially on legs and
  trunk
• Gastrointestinal or urinary tract bleeding may
  occur
• Nose bleeding may be present and difficult to
  control
• Intracranial hemorrhage occurs in only 1 of
  patients

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ITPDiagnosis

• History and clinical presentation
• Peripheral blood smear with very few, large,
  immature platelets
• Bone marrow aspiration will be normal
• Lab tests
• Decreased platelet count
• Prolonged standard bleeding time
• Normal PT and PTT

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ITPTreatment

• Self-limiting condition
• Prevent injury
• Control bleeding
• Immunosuppressive medication if sever symptoms
  are present
• Prednisone
• Gamma globulin

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Questions?