Hemophilia Questions & Answers

Q: What is hemophilia?
A: Hemophilia is a rare disorder in which a person’s blood does not clot properly because the body does not produce enough of, or is missing, the blood clotting proteins, Factor VIII or Factor IX. The body depends on these clotting factors to stop bleeding after injury and to promote healing. When a person with hemophilia gets a cut or bleeds internally, he bleeds longer than someone without hemophilia. Internal bleeding is usually into joints and muscles, but can also occur in the brain or other organs.

 

Q: What are the types of hemophilia?
A: Hemophilia A, also called classical hemophilia, occurs when clotting Factor VIII is either absent or not present in sufficient amounts. Hemophilia B, also called Christmas disease, occurs when clotting Factor IX is either absent or not present in sufficient amounts. Hemophilia C is extremely rare, and occurs when clotting Factor XI is either absent or not present in sufficient amounts.

 

Q: Who has hemophilia?
A: Hemophilia primarily affects males. The incidence of hemophilia A is one in 5,000 – 10,000 live male births. Approximately 12,000 Americans have hemophilia A. Hemophilia A is seven times more common than hemophilia B. The incidence of hemophilia B is one in 34,500 men.

 

Q: What are the levels of severity of hemophilia?
A: The severity of hemophilia is dependent on the amount of clotting factor that is either missing or deficient.

Mild hemophilia A patients have Factor VIII levels of 6% to 50% of normal.
Moderate hemophilia A patients have Factor VIII levels of 1% to 5% of normal.
Severe hemophilia A patients have Factor VIII levels of <1% of normal.
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Q: How does blood clot?
A: Bleeding causes a biological “domino effect” in which a series of steps are set in motion. Blood vessels begin to shrink to reduce blood flow. Then, platelets (blood-clotting cells) begin to collect at the injured site to form a temporary plug. In the meantime, clotting factor proteins are sequentially activated in the blood clotting cascade to form a fibrin clot at the wound site, and stopping the bleed. For more information, see the section About Blood.

 

Q: What is clotting factor?
A: Clotting factors are proteins found in blood that work together to make blood clot. They are designated by Roman numerals I through XIII. When the body detects bleeding, clotting factors are switched on in a specific order, each activating the next factor in the blood clotting cascade to ultimately form a fibrin clot. The fibrin acts like a net that holds the platelets together to make a firm blood clot.

Factor VIII is one of the clotting factor proteins that help produce the fibrin clot. In persons with hemophilia A, whose bodies do not make sufficient Factor VIII, fibrin is not made properly so firm blood clots do not form in the wound and bleeding is not easily stopped.

 

Q: Can hemophilia be cured?
A: There is presently no cure for hemophilia, but with proper treatment using clotting factors, everyone, even people with severe hemophilia, can enjoy a full and active life.

 

Q: What are the treatment options for people with hemophilia?
A: The hemophilia community considers infusion (infused into a vein) therapy a safe and excellent option. For patients with hemophilia A, this is known as Factor VIII replacement therapy. This therapy works by substituting the missing or nonfunctioning clotting Factor VIII protein. When clotting factor is administered after an injury, the body begins to use it immediately to form a clot. This therapy is not a cure for hemophilia A–it is a temporary replacement therapy used to treat symptoms of hemophilia. In people without hemophilia, clotting factor is continuously used and remade by the body.

There are many types of Factor VIII concentrates available for replacement therapy. A well-recognized and safe treatment for hemophilia A is an ultra-pure Factor VIII concentrate that is made using recombinant gene technology. Baxter offers therapies for people with hemophilia A. For more information, please see the Therapies and Services section. However, because everybody is different, your doctor or health-care provider will make sure you have the best treatment for your specific needs.

 

Q: What are inhibitors?
A: Inhibitors are proteins called antibodies that are made by our immune system. In general, when our immune system identifies a foreign substance, it makes antibodies that will specifically recognize that substance and destroy it to defend us from harmful disease. In some individuals with hemophilia, the Factor VIII replacement therapy is identified as a foreign substance by their immune system. If this happens, the immune system will make antibodies against Factor VIII. These antibodies will inhibit the ability of the infused Factor VIII to work in the clotting process. This can seriously complicate the treatment of a bleed. The good news is that there are different types of therapies available to successfully treat hemophilia patients who develop inhibitors.

 

Q: Where can I get more information about hemophilia?
A: Hemophilia Galaxy contains a wealth of information and resources related to all aspects of living with and caring for someone with hemophilia. If you are newly diagnosed, you may wish to take a tour of the Hemophilia Galaxy website. Or, visit the Resources section to find organizations and websites that specialize in hemophilia.

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