Practice Question # 636.
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- Question 1 of 1
A nurse teaches a coworker that the treatment for hemophilia will likely include periodic self administration of:Correct
Answer & Rationale:
A person with hemophilia A is deficient in factor VIII; hemophilia B, factor IX; and von Willebrand’s hemophilia, the von Willebrand’s factor and factor VIII. Recombinant forms of the factors are available for the client to self-administer intravenously at home. Although whole blood and fresh frozen plasma contain the deficient factors, periodic administration of factor concentrates are safer. Platelets do not contain the deficient clotting factors.Incorrect
Answer & Rationale:
A person with hemophilia A is deficient in factor VIII; hemophilia B, factor IX; and von Willebrand’s hemophilia, the von Willebrand’s factor and factor VIII. Recombinant forms of the factors are available for the client to self-administer intravenously at home. Although whole blood and fresh frozen plasma contain the deficient factors, periodic administration of factor concentrates are safer. Platelets do not contain the deficient clotting factors.
Hemophilia Study Review
- Hemophilia refers to a group of bleeding disorders resulting from a deficiency of specific coagulation proteins.
- Identifying the specific coagulation deficiency is important so that definitive treatment with the specific replacement agent can be implemented; aggressive replacement therapy is initiated to prevent the chronic crippling effects from joint bleeding.
- The most common types are factor VIII deficiency (hemophilia A or classic hemophilia) and factor IX deficiency (hemophilia B or Christmas disease).
- Hemophilia is transmitted as an X-linked recessive disorder (it may also occur as a result of a gene mutation).
- Carrier females pass on the defect to affected males; female offspring are rarely born with the disorder, but may be if they inherit an affected gene from their mother and are offspring of a father with hemophilia.
- The primary treatment is replacement of the missing clotting factor; additional medications, such as agents to relieve pain or corticosteroids, may be prescribed depending on the source of bleeding from the disorder.
- Abnormal bleeding in response to trauma or surgery (sometimes is detected after circumcision)
- Epistaxis (nosebleeds)
- Joint bleeding causing pain, tenderness, swelling, and limited range of motion
- Tendency to bruise easily
- Results of tests that measure platelet function are normal; results of tests that measure clotting factor function may be abnormal.
- Monitor for bleeding and maintain bleeding precautions.
- Prepare to administer replacement factors as prescribed.
- DDAVP (1-deamino-8-D-arginine vasopressin), a synthetic form of vasopressin, increases plasma factor VIII and may be prescribed to treat mild hemophilia.
- Monitor for joint pain; immobilize the affected extremity if joint pain occurs.
- Assess neurological status (child is at risk for intracranial hemorrhage).
- Monitor urine for hematuria.
- Control joint bleeding by immobilization, elevation, and application of ice; apply pressure (15 minutes) for superficial bleeding.
- Instruct the child and parents about the signs of internal bleeding.
- Instruct parents in how to control the bleeding.
- Instruct parents regarding activities for the child, emphasizing the avoidance of contact sports and the need for protective devices while learning to walk; assist in developing an appropriate exercise plan.
- Instruct the child to wear protective devices such as helmets and knee and elbow pads when participating in sports such as bicycling and skating.