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A.

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.

In hemophilia B, there is a defect or


deficiency of factor IX.

Clotting factor malfunction causes


abnormal bleeding owing to impaired
ability to form a fibrin clot.
Signs and symptoms of Hemophilia
Abnormal bleeding
Mild hemophilia-prolonged if
challenge by major surgery or trauma
Moderate Hemophilia-only occasional
spontaneous bleeding episodes
Severe Hemophilia-spontaneous
bleeding; first sign is commonly
excessive bleeding after circumcision
Large subcutaneous and deep
intramuscular hematomas
Complications
Peripheral neuropathy
Pain
Paresthesia
Muscle athropy
Decreased tissue perfusion and
hypovolemic shock
B. Assessment Findings
Prolonged bleeding after minor injury.
At birth after cutting of cord.
Following circumcision.
Following IM immunizations.
Following of baby tooth.
Increased bruising as child learns to crawl and
walk.
Bruising and hematomas but no petechiae.
Peripheral neurophaties (due to bleeding near
peripheral nerves): pain, paresthesias, muscle
atrophy
Hemarthrosis
Repeated bleeding into joint results in a swollen
and painful joint with limited mobility.
May result in contractures and possible
degeneration of joint.
Knees, ankles, elbows, wrists most often affected.

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.

Provide care for hemarthrosis


immobilize joint and control acute bleeding.
Elevate joint in a slightly flexed position
Avoid excessive handling of joint.
Administer analgesics as ordered; pain relief will
minimize increase in pulse rate and blood loss.
Aspirin should not be given because it inhibits
platelet function.
Instruct to avoid weight bearing for 48 hours after
bleeding episode if bleeding is in lower extremities
Provide active or passive ROM exercises after
bleeding has been controlled (48 hours), as long as
exercises do not cause pain or irritate trauma site.
administer factor VIII concentrate,
or DDAVP as ordered
provide client teaching and
discharge planning concerning
prevention of trauma
genetic counseling