NCLEX RN Practice Question # 636

NCLEX Quiz.

Practice Question # 636.


Nclex

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Hemophilia Study Review

Description

  • 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.

Nursing Assessment

  • 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.

Nursing Interventions

  • 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.

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