The A to B of hemophilia and hemophilia treatment
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, is inherited (passed down through families).
Originally, hemophilia was thought to be a single disease. In the 1950s, health care providers discovered that there are actually two forms of the disease: hemophilia A and hemophilia B. Because of this, each disease is treated with its own specific factor replacement medication.
HEMOPHILIA A & B:
WHAT IS HEMOPHILIA A OR FACTOR VIII DEFICIENCY?
Hemophilia A is the most common form of hemophilia, occurring in about 1 in every
People with hemophilia A, also called "classic hemophilia," have a deficiency in clotting
factor VIII, meaning that clotting factor VIII is either missing or is present at a low level.
Factor VIII/IX level
More than 5% of normal
1% to 5% of normal
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 VIII or IX that varies from 50% to 150%.
Treating hemophilia A
It's important to manage hemophilia A by taking steps to stop bleeds as soon as they start. There are several therapies available to treat hemophilia A. Xyntha® Antihemophilic Factor (Recombinant), Plasma/Albumin-Free is a factor VIII replacement product made using a process called recombinant factor technology, meaning that it is not made using donated human blood. XYNTHA is also purified using a state-of-the-art process that is free from human or added animal materials* to reduce the risk of viral contamination. XYNTHA® SOLOFUSE® is the only all-in-one reconstitution device for hemophilia A patients, and it comes with:
- Demonstrated bleed control on demand and before and after surgery in clinical trials
- All-in-one reconstitution, which means no more transfer step and no more vials
- A range of dosing options—250, 500, 1000, 2000, and 3000 IU
- Low 4-mL diluent
- An all-inclusive kit that is ready to go when you are
Visit XYNTHA.com to learn more, and watch XYNTHA SOLOFUSE in action.
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WHAT IS 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.
Treating hemophilia B
It's important to manage hemophilia B by taking steps to stop bleeds as soon as they start. There are several therapies available to treat hemophilia B. One of these is made using recombinant factor technology, meaning that it is made in genetically engineered cells. It's called BeneFix® Coagulation Factor IX (Recombinant), and it has more than 15 years of clinical experience.† BeneFix is designed with viral safety in mind. With BeneFix you get:
- A demonstrated safety record for more than 15 years†
- Established bleed control across a wide range of hemophilia B patients
- Simple reconstitution and a range of doses—including the first 3000-IU dose for hemophilia B patients—all with a low 5-mL diluent
- Enhanced room temperature storage or under refrigeration (2°C to 30°C/36°F to 86°F) for up to 2 years until expiration
To find the most up-to-date information about BeneFix, visit BeneFix.com.
For a comprehensive look at hemophilia B and its treatment, as well as what to expect while living with or caring for someone with hemophilia B, you can download "Living with hemophilia B."
Download "Living with hemophilia B."
†BeneFix was approved February 11, 1997.
GET STARTED ON XYNTHA OR BENEFIX WITH THE PFIZER TRIAL PRESCRIPTION PROGRAM:
Eligible patients can receive a one-time, 1-month supply up to 20,000 IU of Pfizer factor product at no cost.‡
If you have hemophilia A, click here for more information on XYNTHA.
If you have hemophilia B, click here for more information on BeneFix.
HOW IS HEMOPHILIA INHERITED?
Hemophilia is an X-linked genetic disorder. The infographic below helps explain how inherited genes play a role.
The signs and symptoms of hemophilia can include:
- Bleeding following injury
- Spontaneous bleeding (spontaneous means that the bleeding occurs without an apparent cause)
- Pain and swelling resulting from bleeding into joints and muscles
- Blood in the urine or stool
- Heavy bleeding following circumcision
- Unexplained nosebleeds
- Brain bleeds
What Is XYNTHA?
Xyntha® Antihemophilic Factor (Recombinant), Plasma/Albumin-Free is indicated for the control and prevention of bleeding episodes in patients with hemophilia A (congenital factor VIII deficiency or classic hemophilia) and for surgical prophylaxis in patients with hemophilia A.
XYNTHA does not contain von Willebrand factor and, therefore, is not indicated in von Willebrand's disease.
Important Safety Information for XYNTHA
- Call your healthcare provider or go to the emergency department right away if you have any of the following symptoms because these may be signs of a serious allergic reaction: wheezing, trouble breathing, chest tightness, turning blue (look at lips and gums), fast heartbeat, swelling of the face, faintness, rash, or hives. XYNTHA contains trace amounts of hamster protein. You may develop an allergic reaction to these proteins. Tell your healthcare provider if you have had an allergic reaction to hamster protein.
- Call your healthcare provider right away if bleeding is not controlled after using XYNTHA; this may be a sign of an inhibitor, an antibody that may stop XYNTHA from working properly. Your healthcare provider may need to take blood tests to monitor for inhibitors.
- The most common adverse reaction in the safety and efficacy study is headache (24% of subjects) and in the surgery study is fever (43% of subjects). Other common side effects of XYNTHA include nausea, vomiting, diarrhea, or weakness.
- XYNTHA is an injectable medicine administered by intravenous (IV) infusion. You may experience local irritation when infusing XYNTHA after reconstitution in XYNTHA® SOLOFUSE®.
Please see full Prescribing Information for XYNTHA
and XYNTHA SOLOFUSE.
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.
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