Learn what hemophilia is, what can cause it, and what treatment is needed.
What is hemophilia?
In hemophilia, blood does not clot properly. This usually happens because your body does not have enough of a certain kind of clotting factor. This makes it harder for bleeding to stop. People with hemophilia may bleed a lot after cuts, during surgery, or even after a fall. Some people have abnormal bleeding inside their bodies for no clear reason.
are two main types of hemophilia:
Hemophilia A is caused
by a lack of active clotting factor VIII (8). About 1 out of every 5,000 male
babies is born with hemophilia A.1
Hemophilia B (Christmas disease) is caused by a lack of active
clotting factor IX (9). It is less common and affects 1 out of 30,000 male
Hemophilia usually runs in families and almost always affects males. In rare cases, a person may get a type
that does not run in the family. This is called acquired hemophilia, and it affects both males and females.
What causes hemophilia?
Hemophilia A and B are caused by a flaw in a part of a gene. This flaw affects how much clotting
factor a person has and how well it works.
With acquired hemophilia, clotting
factors don't work right because the body makes antibodies that attack
What are the symptoms?
Symptoms of hemophilia include:
Bleeding into a joint or muscle, which causes
pain and swelling.
Bleeding that is not normal after an injury or
Blood in the urine.
Bleeding after dental
Some people with milder types of the
disease may not have symptoms until later in life. But most of the time, hemophilia symptoms are
noticed during infancy or childhood. Symptoms
noticed in infants include:
Bleeding into the muscle, which causes a deep
bruise after the baby gets a routine vitamin K shot.
Bleeding that goes on for a long time after the infant's heel is pricked to draw blood for newborn screening tests.
goes on for a long time after a baby is circumcised.
Bleeding in the scalp or brain after a difficult delivery or after special devices (vacuum or forceps) are used to help deliver the baby.
How is hemophilia diagnosed?
If your doctor thinks that you or your child may have a problem with blood clotting, he or she will take a blood sample. The sample will be used in tests that check for the amount of clotting factor. If the level is low, then more tests will find out the type of hemophilia and how severe it is.
How severe the disease is depends on how much clotting
factor is produced and when bleeding most often occurs.
Clotting factor level is at least 5% of normal. This type might not be noticed
unless there is a lot of bleeding after a major injury or surgery.
Moderate hemophilia: Clotting factor level
is 1% to 5% of normal. Bleeding normally follows a fall, sprain, or
Severe hemophilia: Clotting factor
level is less than 1% of normal. Bleeding often happens one or more times a
week for no reason.
If hemophilia runs in your family and you are planning to have children, ask your doctor about tests that can show if you are a carrier. (Only females can be
carriers.) This will allow you to make informed decisions about pregnancy and prenatal care.
How is it treated?
Hemophilia can be
treated by replacing missing blood clotting factors. This is called clotting factor replacement therapy. Clotting factor concentrate is injected into a vein. Replacement therapy can prevent or treat bleeding episodes.
You may need to take medicines, such as desmopressin (for example, DDAVP or Stimate), that help prevent bleeding. You might take medicines at certain times, such as before you have surgery or dental work. Some people also need pain medicine to help with pain from joint damage.
You can live a normal life with treatment. Hemophilia treatment centers
are available at most large medical centers. They are an excellent resource to
help you and your family get the best care for this condition.
What can you do at home?
You can take steps at home
to prevent bleeding episodes and improve your health.
Learn how to recognize bleeding episodes so you can start treatment right away.
Stay at a healthy weight. Additional stress on joints can trigger bleeding episodes.
Exercise with care.
Choose activities, such as swimming, that do not put too much pressure on your joints.
Don't take nonprescription medicines unless your doctor tells you
to. And don't take aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs), such as
ibuprofen and naproxen. These can affect the clotting action of your
Chitlur M, Kulkarni R (2013). Hemophilia and related conditions. In ET Bope, RD Kellerman, eds., Conn's Current Therapy 2013, pp. 803–810. Philadelphia: Saunders.
How this information was developed to help you make better health decisions.