Hemophilia



  1. General information
    1. A group of bleeding disorders where there is a deficit of one of several factors in clotting mechanism
    2. Sex-linked, inherited disorder; classic form affects males only
    3. Types
      1. Hemophilia A: factor VIII deficiency (75% of all hemophilia)
      2. Hemophilia B (Christmas disease): factor IX deficiency (10%-12% of all hemophilia)
      3. Hemophilia C: factor XI deficiency (autosomal recessive, affects both sexes)
    4. Only the intrinsic system is involved; platelets are not affected, but fibrin clot does not always form; bleeding from minor cuts may be stopped by platelets.
    5. If individual has less than 20%-30% of factor VIII or IX, there is an impairment of clotting and clot is jelly-like.
    6. Bleeding in neck, mouth, and thorax requires immediate professional care.
  2. Assessment findings
    1. Prolonged bleeding after minor injury
      1. At birth after cutting of cord
      2. Following circumcision
      3. Following IM immunizations
      4. Following loss of baby teeth
      5. Increased bruising as child learns to crawl and walk
    2. Bruising and hematomas but no petechiae
    3. Peripheral neuropathies (due to bleeding near peripheral nerves): pain, paresthesias, muscle atrophy
    4. Hemarthrosis
      1. Repeated bleeding into a joint results in a swollen and painful joint with limited mobility
      2. May result in contractures and possible degeneration of joint
      3. Knees, ankles, elbows, wrists most often affected
    5. Diagnostic tests
      1. Platelet count normal
      2. Prolonged coagulation time: PTT increased
      3. Anemia
  3. Nursing interventions
    1. Control acute bleeding episode.
      1. Apply ice compress for vasoconstriction.
      2. Immobilize area to prevent clots from being dislodged.
      3. Elevate affected extremity above heart level.
      4. Provide manual pressure or pressure dressing for 15 minutes; do not keep lifting dressing to check for bleeding status.
      5. Maintain calm environment to decrease pulse.
      6. Avoid sutures, cauterization, aspirin: all exacerbate bleeding.
      7. Administer hemostatic agents as ordered.
        1. fibrin foam
        2. topical application of adrenalin/epinephrine to promote vasoconstriction
    2. Provide care for hemarthrosis.
      1. Immobilize joint and control acute bleeding.
      2. Elevate joint in a slightly flexed position.
      3. Avoid excessive handling of joint.
      4. Administer analgesics as ordered; pain relief will minimize increases in pulse rate and blood loss.
      5. Aspirin should not be given because it inhibits platelet function.
      6. Instruct to avoid weight bearing for 48 hours after bleeding episode if bleeding is in lower extremities.
      7. Provide active or passive ROM exercises after bleeding has been controlled (48 hours), as long as exercises do not cause pain or irritate trauma site.
    3. Administer cryoprecipitate (frozen factor VIII) as ordered.
      1. Thaw slowly.
      2. Gently rotate bottle; shaking deteriorates antihemophilic factor.
      3. Infuse immediately when thawed; factor VIII deteriorates at room temperature.
    4. Provide client teaching and discharge planning concerning
      1. Prevention of trauma (see Idiopathic Thrombocytopenic Purpura)
      2. Genetic counseling
        1. when mother is carrier: 50% chance with each pregnancy for sons to have hemophilia, 50% chance with each pregnancy for daughters to be carriers
        2. when father has hemophilia, mother is normal: no chance for children to have disease, but all daughters will be carriers
      3. Availability of support/counseling agencies

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