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This chapter is from the book

This chapter is from the book

Hemophilia

In hemophilia, there is a deficiency of one of the factors necessary for blood coagulation. An abnormal clotting pattern occurs, resulting in an ineffective clot. Hemophilia is inherited as a sex-linked disorder. It is an x-linked recessive disorder. The mother passes this disorder to her male children. When a female inherits the gene from her father, she has a 50% chance of transmitting it to her son. Approximately 40% of patients with hemophilia have no hereditary genetic links. Clients lacking factor VIII have hemophilia A (Classic hemophilia); clients lacking factor IX have hemophilia B (Christmas disease). The discussion that follows focuses on Factor VIII deficiencies because they are the most common (75% of cases).

There are varying degrees of severity associated with hemophilia that relate to the amount of Factor VIII activity. Severe hemophilia occurs when there is 1% of Factor VIII activity. This client can have spontaneous bleeding without trauma. Moderate hemophilia happens with 1%–5% of Factor VIII activity and bleedings occurs with trauma. The last degree of severity is the mild form that occurs with 5%–50% of Factor VIII active. Clients with mild hemophilia bleed with severe trauma or when surgery is performed. Signs or symptoms of hemophilia include

  • Bleeding and bruising easily.
  • Prolonged bleeding from any orifice or anywhere in the body.
  • Hemorrhaging from minor cuts or with teeth eruption.
  • Joint hemorrhages or hemarthrosis. Early signs are stiffness, tingling or aching in the joint, and inability to move the joint. Other symptoms are warmth, redness, swelling, and pain.
  • Post-operative hemorrhaging.
  • Epistaxis.
  • Hematuria.
  • Internal bleeding. Spinal cord hematomas can lead to paralysis.
  • Intracranial bleeding.

The primary goals of treatment for clients with hemophilia are to promote adequate blood clotting and to prevent and treat complications or problems associated with the disease. Nursing measures for goal accomplishment include the following:

  • Administration of prescribed Factor VIII concentrate (monoclonal) and recombinant Factor VIII concentrate (sold as a drug, not a drug product); factor replacement might be administered prophylactically
  • Control localized bleeding
    • Topical coagulants
    • Institute R-I-C-E (rest, ice, compression, elevation) treatment
  • Manage pain (ibuprofen is used with caution due to its platelet inhibition properties)
  • No rectal temperatures
  • Monitor the administration of blood transfusions as ordered
  • Administer prescribed desmopressin acetate (DDAVP) for mild hemophilia (increases Factor VIII by releasing factors from storage sites)
  • Consult with physical therapy after bleeding under control for muscle and joint strengthening; no passive range of motion due to possibility of rebleed
  • Reinforce teaching both the family and client on prevention of injuries
    • Medic-Alert bracelet
    • Padding of furniture corners, if toddler
    • Avoid contact sports (suggest swimming or golf)
    • Signs and symptoms of hemarthrosis
    • Use of soft toothbrush and regular dental visits
    • Avoid aspirin
    • Genetic counseling
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