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General Information
Males inherit hemophilia from their
mothers, and females inherit their
carrier status from their fathers.
Some females who are carriers have
increased tendency to bleed and
although rare, females can have
hemophilia if their fathers have the
disorder and their mothers are
carriers of the genetic disorder.
A group o bleeding disorders where
there is a deficit o one of several
factors in clotting mechanism.
Sex-linked, inherited disorder;
classic forms affects males only.
Types
Hemophilia A: factor VIII deficiency (75 %
of all hemophilia)
Hemophilia B (Christmas Disease): factor
IX deficiency (10-12% of all hemophilia)
Hemophilia C: actor Xi deficiency
(autosomal recessive, affects both sexes)
Only the intrinsic system is involved;
platelets are not affected, but fibrin cloths
are not always from; bleeding from minor
cuts may be stopped by platelets.
If individual has less than 20-30% off
factor VIII or IX, there is an impairment of
clotting and clot is jelly-like.
Bleeding in neck, mouth and thorax
requires immediate professional care.
Pathophysiology
In hemophilia A, there is a deficiency
of, or a defect in, factor VIII
(antihemophilic factor [AHF]), which is
necessary for the formation of
thromboplastin.
Diagnostic Findings
Platelet count normal
Prolonged coagulation time: PTT increased
Anemia
DNA testing for hemophilia A will detect carriers of
the disease
Amniocentesis will diagnose hemophilia prenatally.
C. Medical management
The primary treatment is replacement of
missing clotting factor, such as factor VIII
and IX concentrates.
Amino caproic acid (EACA, Ammicar)
Effective after oral surger
Useful in treating mucosal bleeding
Desmopressin (UDAVP)
Induces a trancient rise in factor VIII level
(especially in hemophilia A, but not in
patient with severe factor VIII deficiency
D. Nursing Interventions
Control acute bleeding episode.
Apply ice compress for vasoconstriction.
Immobilize area to prevent clots from being
dislodged.
Elevate affected extremity above heart level.
Provide manual pressure or pressure dressing for 15
minutes; do not keep lifting dressing to check for
bleeding status.
Maintain calm environment to decrease pulse.
Avoid sutures, cauterization, and aspirin: all
exacerbate bleeding
Administer hemostatic agents as ordered.
fibrin foam
topical application of adrenalin/epinephrine to
promote vasoconstriction.