Hemophilia By: Renee Marie Alta

Pathophysiology & Etiology A hereditary coagulation disorder that is characterized by a disturbance of the clotting factors. A decrease in the formation of prothrombin activators occurs as a result of the decrease in clotting factors It is an X-linked hereditary trait that affects mainly males, but women are carriers.

Pathophysiology & Etiology Hemophilia A Factor VIII is absent Factor VIII is needed for the conversion of prothrombin to thrombin through thromboplastin component 85% of the total incidence Hemophilia B Exhibits a deficiency of Factor IX with an absence of plasma thromboplastin component (plasma protein) This results in nonformation of thromboplastin

Pathophysiology & Etiology In the past people have developed AIDS from transfusions of Factor VIII concentrate. 90% of older adults affected with hemophilia are seropositive for HIV that was transmitted via replacement therapy. This problem should be eliminated since testing donors for evidence of the HIV virus and there has been recent knowledge that heat treatment of Factor VIII cencentrates destroys the HIV virus.

Clinical Manifestations Internal and external hemorrhage occurs with ecchymosis. It can especially happen in the muscles which will show deformity and in the joints that become ancylosed Hemarthrosis (bleeding into the joint spaces) usually occurs in the knees and elbows. Other manifestations that occur with this is Pain, Edema, and Fever Since this disease has to do with an an individual’s clotting factors small cuts can be fatal.

Assessment Check for abnormal bleeding in response to trauma or surgery Check for joint bleeding causing pain, tenderness, swelling, and limited ROM Tendency to bruise early Note the presence of blood in subcutaneous tissue, urine, or stool Prolonged partial prothrombine (PTT) Bleeding time, prothrombin (PT), and normal platelet count Reports by the patient and family of incidents of ecchymosis and hemorrhage from slight trauma

Medical Management Preventing and treating bleeding and relieving the pain Transfusions and administration of Factor VIII or Factor IX may be prophylactic or to stop hemorrhage. Desmopressin (DDAVP) may be given (a hormone given increases levels of clotting factor VIII).

Medical Management Cryoprecipitate is a clotting factor that is rich in Factor VIII It is waning because of the associated risk of viral disease transmission. With this, home administration is difficult because cryoprecipitate must be stored in cold temperatures. Human derived product is also used to transmit factor VIII and factor IX. They are frozen from plasma (fresh frozen plasma) Treated to inactivate viral diseases There is a slight risk to using human derived product so scientists have used genetic engineering to manufacture factor VIII.

Nursing Interventions & Patient Teaching Genetic counseling-the nice way of saying “don’t have babies” Not to be given aspirin Pain management Teaching to the patient and family. Monitor transfusions of factor VIII concentrate Monitor for pain Monitor neurological status Monitor of urine for hematuria Monitor for bleeding and bleeding precautions. Control bleeds by immobilization, elevation and the application of ice; in addition to adding pressure

Prognosis The average lifespan for a person with hemophilia is was near normal Estimates vary because of the prevalence of HIV in the hemophiliac population (hemophiliacs who received clotting concentrates before 1984 become seropositive for HIV) With recent development of methods to deactivate the virus the risk of contracting HIV from clotting factor concentrates is almost nil. Therefore, the average lifespan for a person with hemophilia is back to normal.