7.HEMOPHILIA

Front

What is hemophilia?

Back

Hemophilia is a rare inherited bleeding disorder in which the blood does not clot normally.

Front

What are the types of hemophilia?

Back

Hemophilia A (factor VIII deficiency) and Hemophilia B (factor IX deficiency).

Front

What are the classifications of hemophilia based on clotting factor levels?

Back

Mild (5-50% of normal factor), Moderate (1-5% of normal factor), Severe (less than 1% of normal factor).

Front

What are the clinical manifestations of hemophilia?

Back

Hemarthrosis, prolonged bleeding from minor trauma, uncontrolled hemorrhage, epistaxis, GI bleeding, hematuria, ecchymoses, pain, hypovolemic shock, and vertigo.

Front

What is hemarthrosis?

Back

Bleeding into joints, which may lead to chronic pain, joint injury, and deformity.

Front

What are the common complications of hemophilia?

Back

Joint damage, viral infection, hematuria, and intracranial hemorrhage.

Front

What is the pathophysiology of hemophilia?

Back

Injury to blood vessels leads to bleeding; lack of clotting factors results in weak platelet plugs and delayed fibrin clots.

Front

What are the diagnostic tests for hemophilia?

Back

CBC and platelet count, coagulation studies, fibrin degradation products, and amniocentesis.

Front

What is the medical management for hemophilia?

Back

Replacement of deficient clotting factors, platelet cells, fresh frozen plasma, desmopressin, antifibrinolytic therapy, and supportive measures.

Front

What nursing interventions are important for a client with hemophilia?

Back

Assess cause of bleeding, monitor skin color and tissue perfusion, administer blood as needed, reposition patient, and minimize skin trauma.