Hemophilia

By: Ava Foudeh

What is it?

There are two types of Hemophilia, A and B. Both are

caused by deficiencies in the amount of clotting factor
in the blood (VIII or IX). When the blood does not have
enough of one of these or is missing one clotting
factor, the bleeding may end very slowly or may not
stop at all. The two types of Hemophilia are linked
together by their similar clinical pictures and their
similar inheritance patterns.
The most dangerous part about having Hemophilia is
internal bleeding. If internal bleeding is left untreated it
can lead to deformity, disability or even death. In a
Hemophiliac the bleeding continues until either it clots
long enough for it to heal or the person will bleed to
death.

History of Hemophilia
The

earliest references to hemophilia can be

found in second century Jewish writings.
The first attempts to treat hemophilia was by
replacing the clotting factory with blood plasma
taken from pigs and cows.
In the 1970s scientists found two approaches to
the disease
One that was called prophylaxis required injecting
doses of the clotting factor on a regular basis
The second was to inject the factor whenever the
bleeding occurred

How do you get it?

Hemophilia

is a genetic disease and is passed

on by the X chromosome (the chromosome that
carries the clotting factor).
If a boy gets the X chromosome that carries the
hemophilia gene he will become a hemophiliac.
If a girl get the gene, she will become the carrier
of the gene, not showing symptoms of the
disease though she may have a long or heavy
menstrual cycle. The carrier has a 50% chance
of passing the gene on to her children every time
she gets pregnant.

How do you get it ctd.

How do you know if you have it?

When

a baby starts to crawl the parents may

notice bruises on stomach, chest, buttock, and
back.
The baby may also be fussy, not wanting to walk
or crawl
Other symptoms include long nosebleeds,
excessive bleeding from biting down on the lips
or tongue, excessive bleeding following a tooth
extraction, excessive bleeding following surgery
and blood in the urine.

Testing for Hemophilia

There are two ways to test
for Hemophilia before
birth though both may
cause a sight risk of a
miscarriage
One is to take a fragment
of tissue from the placenta
and look at the genetic
structure. The DNA can
be examined to see if the
child has Hemophilia.

Another way is to insert a thin

needle though the mothers
abdominal wall. The needle
has optical fibers and a system
which enables the doctor to
see the womb and fetus. With
the image, the doctor can
insert a needle into the
umbilical cord and draw out a
small amount of fetal blood.
After the child is born, several
blood tests may be carried out
to determine if the child has it.

Daily cost of Having Hemophilia

Because

Hemophilia is a life long disease

if you have it you need life long treatment.
It will cost any where from $75,000 to
$125,000 a year to treat severe
hemophilia.
That is approximately 31 times an average
nonhemophiliac pays for health care over
the course of a year.

Average life span of people with

Hemophilia
Year

Average age at the time

of death

Treatments available at
time

Before 1938

11

none

Before 1968

20

Plasma or Whole blood

transfusions

1968

Less than 40

Cryoprecipitate

1983

64

Freeze dried clotting

factors

1988

40 ( impact of aids)

Same

1999

Normal life span

Factors produced by
genetic engineering

Average life span ctd.

This is a diagram of the joints most commonly

affected by Hemophilia. It most often occurs at the
knees, hips, ankles, shoulders, and elbows

The most common muscles that bleed with

Hemophilia are those in the the upper arm, upper leg
(front and back), the calf and the front of the groin

Daily life with Hemophilia

Children

can not play with toys with sharp

or hard edges.
Sports or other out-side activities may lead
to injuries.
The patients will most likely want to clean
their teeth properly so diseases that can
lead to bleeding in the gums will not occur.

Facts About Hemophilia

About 17,000 people in the

USA have the disease.
400,000 people through out
the world have it in 40
countries.

Each case of Hemophilia is

different.
It Is called the royal disease
because it was passed down
from Queen Victoria and
descended down the royal
blood line.

Hemophilia may lead to

arthritis because bleeding into
the joints can inflame the joint
lining and destroy cartilage.
The Hemophiliac will not bleed
any faster than a normal
person, the bleeding just will
not stop as quickly.
Ordinary bruises are rarely a
serious problem but cuts and
abrasions may bleed for days
(with out treatment).

Bibliography

Willet, Edward 1959. Enslow Publishers, Inc. Diseases and

People: Hemophilia. Pgs 128.
Hemophilia: Information provided by NIH & NHLBI
http://www.medceu.com/tests/hemophilia.htm
Hemophilia. Funded by Josiah Macy Jr. foundation
http://www.ygyh.org/hemo/whatisit.htm
"Hemophilia," Microsoft Encarta Online Encyclopedia 2004
http://encarta.msn.com/encnet/refpages/RefArticle.aspx?
refid=761570021
Hemophilia A and B. Last updated June 23, 2004 Written by
Hadi Sawaf http://www.emedicine.com/ped/topic962.htm
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