Hemophilia is the oldest known hereditary bleeding disorder. There
are two types of hemophilia, A and B (Christmas Disease). Low levels
or complete absence of a blood protein essential for clotting causes
         If you ride a skateboard or play a sport, you probably know about
bruises and banged-up knees and  elbows. But for guys with a rare bleeding
disorder called hemophilia (pronounced:  hee-muh-fih-lee-uh), minor cuts and
bruises can be a big deal.

What Is Hemophilia?
Hemophilia is a disease that prevents a person's blood from clotting properly,
which means that  the person bleeds more than a person without hemophilia
does. It's a genetic disorder, which  means it's the result of a change in genes
that's either inherited (passed on from parent to  child) or that happens during
development in the womb. "Hemo" means blood and "philia" means a  tendency
toward. A person who has hemophilia has a tendency to bleed excessively.
Hemophilia  affects mostly boys, although it's very rare: Only about one in every
8,000 boys is born with  hemophilia. The disease can affect people of any race or
For most people, when they get a cut, the body naturally protects itself. Sticky cells
in the  blood called platelets go to where the bleeding is and plug up the hole. This
is the first step  in the clotting process. When the platelets plug the hole, they
release chemicals that attract  more sticky platelets and also activate various
proteins in the blood known as clotting factors.  These proteins mix with the
platelets to form fibers, and these fibers make the clot stronger and  stop the

Our bodies have 12 clotting factors that work together in this process (numbered
using Roman  numerals from I through XII). Having too little of factors VIII (8) and IX
(9) is what causes  hemophilia. A person with hemophilia will only lack one factor,
either factor VIII or factor IX,  but not both.

There are two major kinds of hemophilia. About 80% of cases are hemophilia A,
which is a factor  VIII deficiency. Hemophilia B is when factor IX is lacking.

Hemophilia is classified as mild, moderate, or severe, based on the amount of the
clotting factor  in the person's blood. If someone produces only 1% or less of the
affected factor, the case is  called severe. Someone that produces 2% to 5% has a
moderate case, and someone that produces 6%  to 50% of the affected factor level
is considered to have a mild case of hemophilia. In general,  a person with milder
hemophilia may only bleed excessively once in a while, whereas severe  
hemophilia puts someone at risk for having bleeding problems much more often.

Most people with hemophilia discover they have the condition when they are babies
or young kids.  Sometimes the disease is so mild that a guy doesn't even know he
has it until he has minor  surgery - like getting his tonsils or appendix out - and it's
found in blood tests that doctors  perform before surgery.

What Causes Hemophilia?
Men and women each have 23 pairs of chromosomes (pronounced:
kro-muh-soamz). Women have two X  chromosomes; men have one X and one Y
chromosome. Hemophilia is an X-linked genetic disorder,  which means that it's
passed from mother to son on the X chromosome. If the mother carries the  
gene for hemophilia on one of her X chromosomes, each of her sons will have a
50% chance of  having hemophilia.

Although girls rarely develop the symptoms of hemophilia itself, they can be
carriers of the  disease. For a girl to get hemophilia, she would have to receive
the disease on the chromosome  she receives from her father, who would have
hemophilia, as well as from the X chromosome of her  mother, who would be a
carrier. Although this is not impossible, it is highly unlikely.

What Are the Signs and Symptoms?
If you've just found out you have hemophilia, you probably have a milder form of
the disease.

Symptoms of hemophilia include:
bruises that are unusual in location or number
nosebleeds that won't stop
excessive bleeding from biting a lip or having a tooth pulled
painful or swollen joints
blood in the urine
What Do Doctors Do?
Doctors diagnose hemophilia by performing blood tests. Although the disease
can't be cured, it  can be managed.

A cut or minor wound is usually no big deal for a person with hemophilia, just as
it isn't for  someone without the disease. However, internal bleeding can be
serious. When bleeding occurs in  the joints, muscles, or internal body organs,
treatment is necessary. Patients with more serious  cases of hemophilia often
get regular shots of the factor that they're missing - known as  clotting factor
replacement therapy - to prevent bleeding episodes. The clotting factors are  
transfused to the person through an IV to his vein, and can be given in the
hospital, at the  doctor's office, or at home.

People with moderate or mild cases of hemophilia usually don't need these shots
unless they have  a serious injury or require surgery.

If you have hemophilia, you are probably used to working with a medical team of
hematologists  (doctors who specialize in blood disorders), nurses, and social
workers. Your medical team can  help you learn to recognize how it feels when
you bleed internally (the place where the bleeding  is happening will probably
feel warm and tingly or bubbly). This is important to know because  when you
begin to bleed internally you need to infuse (replace) the missing clotting factor
right  away to make the bleeding stop. Putting off doing so can cause damage to
the joints.

If you will be injecting the clotting factors at home, your treatment team will
probably teach  you and your parents how to mix the clotting factor and inject
it into a vein. If you have bleeds  fairly often, though, your doctor might
consider having you infuse clotting factor regularly to  prevent bleeds from
happening in the first place. If that's the case, you might get a small tube  called
a portacath, or port, implanted in your chest. That way, you never have to
worry about  finding a vein and there's no pain when the infusion is done.

Scientists are working on something called gene therapy for people with
hemophilia. Gene therapy  is an experimental technique that tries to provide the
body with the genetic information it  doesn't have. Hemophilia is considered a
good test for gene therapy because it is caused by only  one defective gene.
Scientists hope that they will be able to provide people with hemophilia with  
the genetic information they need to produce their own missing clotting factors.

Living With Hemophilia
If you have hemophilia, your day-to-day life is probably pretty normal. Exercise is
important for  teens with hemophilia because it makes muscles stronger, which
protects the joints and decreases  bleeds. Swimming and cycling are great
because they don't put pressure on the joints. In fact,  you can participate in
just about any sport, although team sports, such as soccer, basketball, or  
baseball, present a higher risk and all contact sports (like football, boxing, and
hockey) are  off-limits because there's a high risk of injury.

It's also important to maintain a healthy weight because extra pounds can strain
the body. And  don't take any product that contains aspirin, ibuprofen, or
naproxen sodium. All of these can  keep blood from clotting.

If you have hemophilia and want to hang out with other people who have the
disease, think about  going to a camp for teens with hemophilia. You do all the
fun stuff they have at any camp, but  you also learn how to take the very best
control of your condition.

There may be times when you feel really overwhelmed. Try to remember that
you're not alone. Talk  to your mom or dad, your doctor, a nurse, or anyone on
your treatment team. They can help you  deal with the emotional aspects of
having hemophilia.

Having hemophilia doesn't mean you can't work out, have a girlfriend, or do most
of the things  that guys like to do. You just have to be smart about your
condition. Learn everything you can so  you can take care of yourself and make
choices that are right for you.
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informational website, and
should not be used as a  
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Copyrights medgrip.com 2006 Hemophilia Symptom.

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