Ward Class

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Ward Class is an online resource for student nurses. Created with the aim of helping student nurses cope with the demands of nursing school and clinicals, it offers free downloads, notes, sample NCPs, sample drug studies, study aids, news and updates, and practical tips to its users.

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Hematologic Disorders: Hemophilia


  • an inherited interference in blood coagulation
  • sex-linked, hereditary disorder; classic form affects males only
  • types:
    • hemophilia A (factor VIII deficiency)
      • this is a deficiency in factor VIII, the antihemophilic factor, which is transmitted as a sex-linked recessive trait
      • comprises 75% of all hemophilia cases
      • males with this type of hemophilia have varying bleeding tendencies, from mild to severe
    • hemophilia B (Christmas Disease, factor IX deficiency)
      • accounts for 15% of hemophilia cases
      • transmitted as a sex-liked recessive trait, affects males
    • hemophilia C (factor XI deficiency)
        • a deficiency in plasma thromboplastin, transmitted as an autosomal recessive trait in both sexes
        • occurs in Jewish children; symptoms are milder compared to other types of hemophilia
Assessment Findings
  • prolonged bleeding after minor injuries
    • at birth, after cutting of cord
    • following circumcision
    • following immunizations
    • following loss of baby teeth
    • heavy bruising, esp. in lower extremities as child begins to walk
  • bruising and hematomas with no petechiae
  • nosebleeds with no obvious cause
  • pain, paresthesias, muscle atrophy
  • swollen and painful joints with limited mobility (due to repeated bleeding)
  • blood in urine or stool
  • emergency signs and symptoms include:
    • Sudden pain, swelling, and warmth of large joints, such as knees, elbows, hips and shoulders, and of the muscles of your arms and legs
    • Bleeding from an injury, especially if you have a severe form of hemophilia
    • Painful, lasting headache
    • Repeated vomiting
    • Extreme fatigue
    • Neck pain
    • Double vision
  • diagnostic tests:
    • normal platelet count
    • normal prothrombin time
    • increased PTT (>45 sec.)
    • anemia
Medical Management
  1. Mild hemophilia A. Treatment may involve slow injection of the hormone desmopressin (DDAVP) to stimulate a release of clotting factors to stop bleeding. Desmopressin may be given via nasal spray.
  2. Moderate to severe hemophilia A or hemophilia B. Treatment is done by the administration of appropriate clotting factor.
  3. Hemophilia C.Plasma infusions are given to stop bleeding episodes.
Nursing management
  1. control acute bleeding
    • apply ice compress-->promotes vasoconstriction
    • immobilize affected area-->prevents clots from being dislodged
    • elevate affected extremity above heart level
    • provide manual pressure or apply pressure dressing for 15 minutes
    • maintain calm environment-->decreases pulse
    • avoid exacerbation of bleeding-->no sutures, cauterization, aspirin
    • administer hemostatic agents as ordered-->fibrin foam, topical epinephrine
  2. provide care for hemarthrosis (bleeding into a joint)
    • immobilize joint, avoid excessive handling of joint
    • elevate joint in slightly-flexed position
    • administer pain medications as ordered--
    • instruct patient to avoid weight-bearing activities for 48 hours if bleeding is in lower extremities
    • provide passive or active ROM excercises after bleeding has been controlled
  3. administer factor VIII concentrate or Desmopressin (DDAVP) as ordered
  4. provide teachings concerning:
    • prevention of trauma-->no contact sports
    • importance of good dental hygiene-->to avoid need for tooth extraction
    • avoidance of certain medications-->aspirin, herbal meds that can cause bleeding such as willow (salix)
    • genetic counseling-->when mother is carrier, there is a 50% chance with each pregnancy for sons to have hemophilia and for daughters to be carriers
    • availability of community support
Prognosis

Prognosis is very difficult to generalize because the disease comes in varying severity. Complications of hemophilia includes: deep internal bleeding, damage to joints, infection due to multiply transfusions, and adverse reactions to clotting factor treatment.

Prevention

Because it is a hereditary disorder, hemophilia cannot be prevented.

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