What is Hemophilia?
Hemophilia occurs when a protein in the
blood, which is needed for the process of
forming a blood clot, is missing or reduced.
This certain type of protein is called a
clotting factor. When there is not enough of
this clotting factor in a person’s body, they
do not stop bleeding as quickly as they
should. A person with hemophilia is at risk
to bleed longer, not faster.
Hemophilia A occurs when there is a lack
of the clotting protein called factor VIII
(eight). Hemophilia B occurs when there is
a lack of the clotting protein called factor IX
Bleeding and Clotting
How does bleeding start and stop?
1. Bleeding starts when a capillary is
injured and blood leaks out.
2. The capillary tightens up to help
slow the bleeding.
3. Then blood cells called platelets
make a plug to patch the hole.
4. Next, many clotting factors in
plasma (part of the blood) work
together to form a clot over the plug.
This makes the plug stronger and
stops the bleeding.
In hemophilia, the missing clotting factor
makes it difficult to form a clot, so bleeding
continues longer than usual, not faster.
Levels of Hemophilia
The level of severity depends on the amount
of clotting factor that is missing from a
person’s blood. Normal clotting factor
levels are 50-150%.
Mild: A factor level of 5-50%
Bleeding occurs only after a major
injury, surgery or dental work
Moderate: A factor level of 1-5%
Bleeding occurs after the above and
after smaller injuries
Severe: A factor level of <1%
Bleeding can occur after the above
but can also occur for no clear reason.
A person’s level of hemophilia does not
change over time. The level of hemophilia
is the same among family members. If your
family has a mild level, then all members
who have hemophilia will also have a mild
level. However family members can have
different patterns of bleeding.
Hemophilia is passed down through families
ξ When the father has hemophilia,
none of the sons will have
hemophilia but all of the daughters
will be carriers.
ξ Women who have the hemophilia
gene are called carriers. They
sometimes show signs of
hemophilia, and they can pass it on
to their children. For each child,
there is a 50% chance that a son will
have hemophilia and a 50% chance
that a daughter will be a carrier.
ξ Women can only have hemophilia if
their father has hemophilia and their
mother is a carrier. This is very
ξ Sometimes hemophilia can occur
when there is no family history. This
is called a spontaneous mutation and
occurs in about 30% of new cases of
Types of bleeds and symptoms
Severe, life threatening bleeds –seek
medical help right away
ξ Head – symptoms may include
headache, neck ache, irritability,
dizziness or difficulty walking,
sleepiness, sensitivity to light,
nausea, vomiting, or loss of
ξ Eye- symptoms may include pain
around the eye, swelling, change in
skin color around the eye, or change
ξ Neck and Throat – symptoms may
include trouble swallowing or
breathing, different speech or crying,
skin color changes around the neck,
neck swelling or tongue swelling.
ξ Abdominal/Gastrointestinal (GI) –
symptoms may include blood in
vomit or vomit that looks like coffee
grounds, black or tar colored stools,
nausea or abdominal pain.
Joint and muscle bleeds
ξ Bleeding occurs most often inside
joints and muscles. When a bleed
occurs blood builds up in the joint or
muscle space. This build up of blood
causes swelling and pain. It takes
time for the body to reabsorb the
blood. The enzymes that are
released to reabsorb the blood also
can cause breakdown of healthy
tissue inside joints. Treating a joint
or muscle bleed as soon as possible
will limit the amount of blood that
builds up and decrease the likelihood
of long-term problems.
ξ Early signs- a hard hit to the area, a
bubbling or tingling feeling inside
the joint, a warm feeling inside the
muscle or joint, or a baby who is
upset or crying for no reason you can
see (not because of hunger, thirst, or
a need to be held).
ξ Late signs- the skin over the muscle
or joint feels warmer than other skin,
swelling, pain, stiffness, signs of
poor blood flow surrounding the
joint or muscle such as cool, numb,
or pale skin, or a young child who
won’t straighten or use an arm or leg.
ξ Mouth and Nose
ξ Cuts and scrapes
ξ Rest, ice, compression, elevation
ξ Clotting factor concentrate
Factor VIII concentrate for
hemophilia A, Factor IX concentrate
for hemophilia B given into a vein to
stop or prevent bleeding.
ξ Acetaminophen (Tylenol®) may be
used for pain. Avoid aspirin
products, ibuprofen (Motrin®,
Advil®), and naproxen ( Aleve® ).
ξ DDAVP (Desmopressin) can only be
used for a mild factor VIII
deficiency. It can be given either by
IV or inhaled through the nose
ξ Antifibrinolytic medicines (Amicar,
Lysteda) are used to treat mouth and
nose bleeding. They are most often
taken by mouth and are often used
before and after dental work.
ξ First Aid for minor cuts, bruises and
Prophylaxis with Factor Concentrate can
prevent joint damage and decrease the
frequency of bleeding in children and adults
with severe hemophilia. Prophylaxis is done
on a regular schedule, often 3 times a week
or every other day, and is usually done at
home. When prophylaxis begins at a young
age children will often have a port placed in
the chest until the veins become easier to
Inhibitors occur when the body’s immune
system reacts to the clotting factor that is
infused. The immune system sees the factor
as a foreign substance and forms inhibitors,
or antibodies, to destroy the factor. Most
inhibitors develop in the first 75 exposures
to clotting factor, with the greatest risk
occurring in the first 10-20 treatments.
Inhibitors are most common in severe
Hemophilia A, about 25-30% of children
with Hemophilia A develop inhibitors. Only
about 1-6% of people with Hemophilia B
develop inhibitors. The most common sign
of an inhibitor is bleeding that is
uncontrolled with typical doses of factor.
Inhibitors are diagnosed by doing a simple
blood draw. They are most often treated
with high dose factor concentrate or
bypassing agents such as Factor VIIa or
Activated Prothrombin complex
Everyone with a bleeding disorder should
have a good level of physical activity.
Being active improves joint and muscle
function and can have a positive effect on
psychological and emotional well-being.
The amount of activity that someone with a
bleeding disorder takes part in will depend
on their own skill level. Sports such as
swimming, dancing and walking are
Contact sports with a high risk of injury,
including football, hockey and boxing, are
strongly discouraged. Proper training in any
activity is important for safety.
This includes learning the rules and using
the correct gear such as helmets, pads, and
UWHC Comprehensive Program for Bleeding Disorders: 866-737-6707.
Your health care team may have given you this information as part of your care. If so, please use it and call if you
have any questions. If this information was not given to you as part of your care, please check with your doctor. This
is not medical advice. This is not to be used for diagnosis or treatment of any medical condition. Because each
person’s health needs are different, you should talk with your doctor or others on your health care team when using
this information. If you have an emergency, please call 911. Copyright © 10/2016 University of Wisconsin Hospitals
and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF#6535