HEMOPHILIA  Gingival bleeding

a. definition  Gastrointestinal bleeding,

which may be manifested by
- Hemopilia refers to a group of hematemesis, occult blood in
congenital coagulation disorders that are the stools, gastric pain, or
characterized by a deficiency or malfunction abdominal pain
of specific clotting factors.  Hematuria
 Pain, parenthesias, or
paralysis resulting from nerve
compression of the
hematomas
 Hemarthrosis (joint bleeding,
b. type swelling and damage)
a.) Hemophilia A, or classic
hemophilia, is caused by a defect e. pathophysiology
in factor VIII (antihemophilic
factor). Hemophilia A is more Hemophilia is an X-linked recessive
common, occurring once per genetic disease that causes abnormal
5000 to 10,000 live male births. bleeding because of a specific clotting factor
malfunction. Factors VIII and IX are
b.) Hemophilia B, or Christmas components of theintrinsic clotting
disease, is caused by a defect in pathway; factor IX is an essential factor and
factor IX (plasma thromboplastin factor VIII is a critical cofactor. Factor VIII
component, or Christmas factor). accelerates the activation of factor X by
Hemophilia B, occurs once per several thousandfold. Excessive bleeding
30,000 live male births. occurs when these clotting factors are
reduced by more than 75%. A deficiency or
c. causes nonfunction of factor VIII causes hemophilia
A; a deficiency or nonfunction of factor IX
- All forms of hemophilia are the causes hemophilia B.
result of an X-linked recessive trait disorder
Hemophilia may be severe,
(see Genetic Considerations).
moderate, or mild, depending on the
Approximately one-third of all hemophiliacs
have no family history of bleeding disorders, degree of activation of clotting factors.
which indicates that there may be factors Patients with severe disease have no
other than heredity involved and that the detectable factor VIII or factor IX activity or
illness is a result of a new mutation. less than 1% of normal. Moderately afflicted
patients have 1-4% of normal clotting
d. s/sx activity; and mildly afflicted patients have
 Persistent and prolonged 5% to 25% of normal clotting activity.
bleeding from small cuts and A person with hemophilia forms a
injuries platelet plug at a bleeding site, but clotting
 Delay of onset of bleeding factor deficiency impairs the ability to form
after injury a stable fibrin clot. Delayed bleeding is
 Subcutaneous ecchymosis more common that immediate
and subcutaneous hematomas hemorrhage.
 concentrates, so their factor levels cannot be
increased. Treatment of this problem is extremely
f. diagnostic tests
difficult and often unsuccessful. Aminocaproic acid
(EACA; Amicar) is a fibrinolytic enzyme inhibitor
a.) Genetic testing that can slow the dissolution of blood clots that do
form; it is very effective as an adjunctive measure
- Identifies genetic alterations after oral surgery. It is also useful in treating mucosal
bleeding. Another agent, desmopressin (eg,
from normal DDAVP), induces a transient rise in factor VIII
levels; the mechanism for this response is unknown.
b.) Assay of factors VIII,IX, XI In patients with mild forms of hemophilia A,
desmopressin is extremely useful, significantly
reducing the amount of blood products required.
- Determines level of activity of However, desmopressin is not effective in patients
essential factors needed for with severe factor VIII deficiency.
blood clotting

c.) Coagulation studies; activated h. 5 nsg. Dx

partial thromboplastin time
(APTT); prothrombin time (PT); a.) Deficient knowledge related to
bleeding time; platelet count
disease process
b.) Acute pain related to bleeding
- Determine for clot formation.
APTT measures the time for into tissues and joints
clot to form in a section of c.) Risk for injury related to altered
clotting cascade affected by clotting factor
factors VIII, IX, and XI d.) Risk for deficient fluid volume
related to bleeding
d.) Thrombin time, x-rays of e.) Ineffective peripheral tissue
hemarthrotic joints, computed perfusion related to impaired
tomography (CT) scan, and blood flow through a major
magnetic resonance imaging vessel caused by bleeding.
(MRI).

g. med. Management i. 5 or more nsg. management base

on its formulating diagnosis
In the past, the only treatment for hemophilia was
infusion of fresh frozen plasma, which had to be
a.) Teach family members or
administered in such large quantities that patients
experienced fluid volume overload. Now factor VIII significant others the
and factor IX concentrates are available to all blood administration of clotting factors
banks. Recombinant forms of these factors have been for prophylactic purposes and if
made available and may diminish the use of factor
concentrates. Patients are given concentrates when
injury occurs.
they are actively bleeding or as a preventive measure b.) Apply ice and pressure to
before traumatic procedures (eg, lumbar puncture, bleeding sites.
dental extraction, surgery). The patient and family are c.) Encourage the client to avoid
taught how to administer the concentrate
intravenously at home at the first sign of bleeding. It activities that may cause trauma
is crucial to initiate treatment as soon as possible so
that bleeding complications can be avoided. A few
patients eventually develop antibodies to the
d.) Quickly regain or maintain a
normal fluid balance during a
bleeding episode
e.) Maintain adequate tissue
perfusion when bleeding
occurs, as shown by a
palpable pulse and normal
skin color and temperature at
and beyond the bleeding site.