CH 54 hemophilia

Question 1

Hemophilia is a bleeding disorder seen almost exclusively in __.

Answer 1

males.

The underlying cause is a genetically based deficiency of clotting factors.

Question 2

Hemophilia has two forms:

Answer 2

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

Question 3

Hemophilia may be severe, moderate, or mild, depending on ___.

Answer 3

the degree of clotting factor deficiency

Question 4

Patients with severe hemophilia may experience ___,

whereas those with mild hemophilia may experience ___.

Answer 4

lifethreatening hemorrhage in response to minor trauma

little or no excessive bleeding

Question 5

Repeated bleeding in the knee and other joints can cause ___.

Answer 5

permanent joint damage

Question 6

The cornerstone of hemophilia treatment is replacement

therapy with __.

Answer 6

factor VIII (hemophilia A) or factor IX (hemophilia B)

Question 7

Replacement therapy may be done (2)..

Answer 7

prophylactically (to prevent bleeding and thus prevent joint injury) or on demand (to stop an ongoing bleed or to prevent excessive bleeding during surgery)

Question 8

Clotting factor products are made in two basic ways:

Answer 8

extraction from donor plasma and production in cell culture using recombinant DNA technology

Question 9

All clotting factor concentrates, whether plasma derived

or recombinant, are __.

Answer 9

equally effective

Question 10

All clotting factor concentrates in use today are __.

Answer 10

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.

Question 11

As a rule, clotting factors are given by __.

Answer 11

slow IV push.

Continuous infusion may also be done, but only by a clinician with special training.

Question 12

Clotting factor dosage depends primarily on the __.

Answer 12

site and severity of the bleed

Question 13

A dose of 1 unit of factor VIII/kg will raise the plasma

level of factor VIII activity by __.

Answer 13

2%, whereas 1 unit of factor IX/kg will raise the plasma level of factor IX activity by only 1%.

Question 14

Although we can monitor the activity of clotting factors
in blood to help guide treatment, dosage is ultimately
determined by __.

Answer 14

the clinical response

Question 15

For prophylaxis, clotting factor concentrates are administered on a __.

Answer 15

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.

Question 16

To facilitate frequent IV administration during prophylaxis,

a ___ can be installed.

Answer 16

central venous access device

Question 17

Clotting factor concentrates can cause __.

Answer 17

allergic reactions.

Mild reactions can be managed with an antihistamine (e.g., diphenhydramine [Benadryl]).

The most severe reaction—anaphylaxis—is treated with subQ epinephrine.

Question 18

For some patients with mild hemophilia A, bleeding can

be stopped with __.

Answer 18

desmopressin, a drug that promotes the release of stored factor VIII.

Desmopressin does not release factor IX, and so cannot treat hemophilia B.

Question 19

Two drugs—(2)—can suppress fibrinolysis and can promote hemostasis in hemophilia A and hemophilia B.

Answer 19

aminocaproic acid and tranexamic acid

These antifibrinolytic agents are more effective for preventing recurrent bleeding than for stopping an ongoing bleed.

Question 20

Development of ___ is a serious complication of hemophilia therapy.

Answer 20

inhibitors (antibodies that neutralize factor VIII or factor IX)

Question 21

(2) are preferred agents for controlling bleeding when inhibitors of factor VIII or factor IX are present.

Answer 21

Activated factor VII (factor VIIa) and AICC

Question 22

People with hemophilia should avoid __.

Answer 22

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.