HEMOPHILIA

Written by :
Anggun Nurhalida
C. 14201.09.039

Nandita Milda Hidayat

C. 14201.09.056

SEKOLAH TINGGI ILMU KESEHATAN MUHAMMADIYAH

TASIKMALAYA
2013
 HEMOPHILIA

A. Introduction
Blood is the fluid found in all living things (except plants). It has
function to send a high-level substances and oxygen needed by body tissues.
abnormalities in the human circulatory system can occur due to congenital,
example is hemophilia. Hemophilia is blood disorders difficult to freeze
because of heredity / decrease.

B. Meaning
Hemophilia is a blood clotting disorder effect of blood clotting factor
caused by cromosomal damage X. Blood in haemophiliacs can not be frozen
by themselves normally. Blood clotting process is running very slow nothing
like those with normal blood. Normally there are 13 blood clotting factors,
people with haemophilia lack a factor of VIII and XI.
Hemophilia is a genetic disorder usually inherited of the mechanism
of blood clotting. Depending on the degree of the disorder present in an
individual, excess bleeding may occur only after specific, predictable events
(such as surgery, dental procedures, or injury), or occur spontaneously, with
no known initiating event.

C. Causes
Hemophilia is caused by an X-linked recessive disorder involving a
lack of genetic factors VIII and XI functional clotting of blood.

D. Sign / Symptom
1. Signs and symptoms of spontaneous bleeding may include :
a. Large or deep bruises
b. Joint pain and swelling caused by internal bleeding
c. Unexplained and excessive bleeding or bruising
d. Blood in your urine or stool
e. Prolonged bleeding from cuts or injuries or after surgery or tooth
extraction
 f. Nose bleeds without a known cause
g. Tightness in your joints
h. In infants, unexplained irritability
i. Unusual bleeding after immunizations
2. Emergency signs and symptoms of hemophilia may include :
a. Sudden pain, swelling, and warmth of large joints, such as knees,
elbows, hips and shoulders, and of the muscles of your arms and
legs.
b. Bleeding from an injury, especially if you have a severe form of
hemophilia
c. Painful, lasting headache
d. Repeated vomiting
e. Extreme fatigue
f. Neck pain
g. Double vision

E. Treatment
a. Desmopressin

Desmopressin (DDAVP) is a man-made hormone used to treat

people who have mild to moderate hemophilia A. DDAVP isn't used to
treat hemophilia B or severe hemophilia A. DDAVP stimulates the
release of stored factor VIII and von Willebrand factor; it also increases
the level of these proteins in your blood. Von Willebrand factor carries
and binds factor VIII, which can then stay in the bloodstream longer.

DDAVP usually is given by injection or as nasal spray. Because

the effect of this medicine wears off if it's used often, the medicine is
given only in certain situations. For example, you may take this
medicine prior to dental work or before playing certain sports to prevent
or reduce bleeding.

b. Antifibrinolytic Medicines

Antifibrinolytic medicines (including tranexamic acid and

aminocaproic acid) may be used with replacement therapy. They're
usually given as a pill, and they help keep blood clots from breaking
 down. These medicines most often are used before dental work or to
treat bleeding from the mouth or nose or mild intestinal bleeding.

c. Gene Therapy
Researchers are trying to find ways to correct the faulty genes that
cause hemophilia. Gene therapy hasn't yet developed to the point that
it's an accepted treatment for hemophilia. However, researchers
continue to test gene therapy in clinical trials.
d. Treatment of a Specific Bleeding Site
Pain medicines, steroids, and physical therapy may be used to
reduce pain and swelling in an affected joint. Talk with your doctor or
pharmacist about which medicines are safe for you to take.

F. Closing

Hemopilia is a hereditary disease. If you already have this disease its

must regularly take medication to keep it controlled. Prevention of much
bleeding is to limit strenuous activities should not be an accident that resulted
in injuries due to injury in people who have the disease difficult to cure
hemopilia. Other precautions can also be done when we are going to do the
wedding, it helps us to know our spouse does have a genetic disease or not
because if you have a hereditary disease would have decreased as well to our
children someday.