CH 54 hemophilia Flashcards
Hemophilia is a bleeding disorder seen almost exclusively in __.
males.
The underlying cause is a genetically based deficiency of clotting factors.
Hemophilia has two forms:
hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency).
Hemophilia may be severe, moderate, or mild, depending on ___.
the degree of clotting factor deficiency
Patients with severe hemophilia may experience ___,
whereas those with mild hemophilia may experience ___.
lifethreatening hemorrhage in response to minor trauma
little or no excessive bleeding
Repeated bleeding in the knee and other joints can cause ___.
permanent joint damage
The cornerstone of hemophilia treatment is replacement
therapy with __.
factor VIII (hemophilia A) or factor IX (hemophilia B)
Replacement therapy may be done (2)..
prophylactically (to prevent bleeding and thus prevent joint injury) or on demand (to stop an ongoing bleed or to prevent excessive bleeding during surgery)
Clotting factor products are made in two basic ways:
extraction from donor plasma and production in cell culture using recombinant DNA technology
All clotting factor concentrates, whether plasma derived
or recombinant, are __.
equally effective
All clotting factor concentrates in use today are __.
very safe:
They carry no risk of transmitting HIV/AIDS and little or no risk of transmitting hepatis or CJD.
However, because recombinant factors are, in theory, slightly safer than plasma-derived factors, they are considered the treatment of choice.
As a rule, clotting factors are given by __.
slow IV push.
Continuous infusion may also be done, but only by a clinician with special training.
Clotting factor dosage depends primarily on the __.
site and severity of the bleed
A dose of 1 unit of factor VIII/kg will raise the plasma
level of factor VIII activity by __.
2%, whereas 1 unit of factor IX/kg will raise the plasma level of factor IX activity by only 1%.
Although we can monitor the activity of clotting factors
in blood to help guide treatment, dosage is ultimately
determined by __.
the clinical response
For prophylaxis, clotting factor concentrates are administered on a __.
regular schedule, usually every other day to 3 times a week for factor VIII and twice a week for factor IX.
With both factors, the goal is to maintain plasma factor levels above 1% of normal.
To facilitate frequent IV administration during prophylaxis,
a ___ can be installed.
central venous access device
Clotting factor concentrates can cause __.
allergic reactions.
Mild reactions can be managed with an antihistamine (e.g., diphenhydramine [Benadryl]).
The most severe reaction—anaphylaxis—is treated with subQ epinephrine.
For some patients with mild hemophilia A, bleeding can
be stopped with __.
desmopressin, a drug that promotes the release of stored factor VIII.
Desmopressin does not release factor IX, and so cannot treat hemophilia B.
Two drugs—(2)—can suppress fibrinolysis and can promote hemostasis in hemophilia A and hemophilia B.
aminocaproic acid and tranexamic acid
These antifibrinolytic agents are more effective for preventing recurrent bleeding than for stopping an ongoing bleed.
Development of ___ is a serious complication of hemophilia therapy.
inhibitors (antibodies that neutralize factor VIII or factor IX)
(2) are preferred agents for controlling bleeding when inhibitors of factor VIII or factor IX are present.
Activated factor VII (factor VIIa) and AICC
People with hemophilia should avoid __.
aspirin and other traditional NSAIDs because these agents suppress platelet aggregation and promote GI ulceration and bleeding.
Second-generation NSAIDs (COX-2 inhibitors) are probably safe.
