About hemophilia B: Signs and symptoms of factor IX deficiency
What is hemophilia?
Hemophilia is a type of bleeding disorder that causes the blood to take a long time to clot. This can cause abnormal bleeding, or bleeding that won’t stop. People with hemophilia have too little—or even none—of a protein in the blood called clotting factor. Hemophilia most often affects males, and in most cases, it is inherited (passed down through families).
Originally, hemophilia was thought to be a single disease. In the 1950s, doctors discovered that there were actually two forms of the disease: hemophilia A and hemophilia B. While the symptoms are similar, the clotting factor that is missing in people with hemophilia A is different than the clotting factor that is missing in people with hemophilia B. Because of this, they are treated with different medications.
Hemophilia B or factor IX deficiency
People with hemophilia B have a deficiency in clotting factor IX. Hemophilia B is sometimes called Christmas disease after Stephen Christmas, the first patient described with this disease. It is the second most common form of hemophilia, occurring in about 1 in 20,000-50,000 people. A 2008 global survey by The World Federation of Hemophilia reported that there were about 3,900 people with hemophilia B in the United States and 22,000 worldwide.
Signs and symptoms
The signs and symptoms of hemophilia B can include:
- Bleeding following injury or surgery
- Spontaneous bleeding (spontaneous means that the bleeding occurs without an apparent cause)
- Bruising
- Pain and swelling resulting from bleeding into joints and muscles
- Blood in the urine or stool
- Heavy bleeding following circumcision
- Unexplained nosebleeds
Classification
| Hemophilia Severity | Factor IX Level |
|---|---|
| Mild | More than 5% of normal |
| Moderate | 1% to 5% of normal |
| Severe | Less than 1% of normal |
Hemophilia is classified as mild, moderate, or severe, depending on the level of clotting factor in the blood. While more severe hemophilia is usually diagnosed during infancy, a mild form may not be diagnosed until adulthood.
People without hemophilia normally have a range of factor IX that varies from 50% to 150%.
If you or your child has hemophilia B, it’s important to stay current and to know all you can about the therapies available. Together, you and your health care provider can come up with a treatment plan to help ensure long-term health and wellness.
Treating hemophilia B
It's important to manage hemophilia B by taking steps to stop bleeds as soon as they start and by putting together a treatment plan to help avoid bleeds altogether. There are several therapies available to treat hemophilia B. One of these is made using recombinant DNA technology, meaning that it is not made with donated human blood. It's called BeneFix® Coagulation Factor IX (Recombinant), and it has more than 14+ years of clinical experience.* BeneFix is designed with viral safety in mind. To find the most up-to-date information about BeneFIX, visit BeneFIX.com.
*BeneFix was approved February 11, 1997.
What Is BeneFIX?
BeneFIX® Coagulation Factor IX (Recombinant) is an injectable medicine that is used to help control and prevent bleeding in people with hemophilia B. Hemophilia B is also called congenital factor IX deficiency or Christmas disease.
BeneFIX is NOT used to treat hemophilia A.
Important Safety Information for BeneFIX
- BeneFIX is contraindicated in patients who have manifested life-threatening, immediate hypersensitivity reactions, including anaphylaxis, to the product or its components, including hamster protein.
- Call your health care provider right away if your bleeding is not controlled after using BeneFIX.
- Allergic reactions may occur with BeneFIX. Call your health care provider or get emergency treatment right away if you have any of the following symptoms: wheezing, difficulty breathing, chest tightness, your lips and gums turning blue, fast heartbeat, facial swelling, faintness, rash or hives.
- Your body can make antibodies, called “inhibitors,” which may interfere with the effectiveness of BeneFIX.
- If you have risk factors for developing blood clots, such as a venous catheter through which BeneFIX is given by continuous infusion, BeneFIX may increase the risk of abnormal blood clots. The safety and efficacy of BeneFIX administration by continuous infusion have not been established.
- Some common side effects of BeneFIX are nausea, injection site reaction, injection site pain, headache, dizziness and rash.
Please see full Prescribing Information for BeneFIX.
