Coagulation and Bleeding Disorders
According to the National Hemophilia Foundation, Hemophilia is a genetic disorder marked by prolonged bleeding due to low levels or absence of one of the coagulation factors needed to form blood clots.
Caused by a faulty gene that impairs the body's ability to produce enough factor to cause clotting, hemophilia affects approximately 1 in 8000 males.
In the most common types of hemophilia, levels of either factor VIII (eight) or factor IX (nine) are low or missing.
von Willebrand disease (VWD) is a hereditary disorder characterized by abnormally slow clotting of the blood.
Patients with von Willebrand disease may have spontaneous and prolonged bleeding from the nose and gums. VWD occurs in about 1 in 100 people and is equallly likely to affect both males and females.
CSL Behring offers a wide range of plasma-derived and recombinant therapies and pharmaceuticals for the treatment of bleeding disorders. We also provide a variety of support services specially targeted to address the needs of people with bleeding disorders, their families, and their healthcare providers.
Hemophilia A
Hemophilia A is a deficiency of factor VIII. About 85% of people with hemophilia have hemophilia A.
People with hemophilia A need to increase the level of factor VIII in their blood. This is done with what is known as "factor replacement therapy."
One type of therapy involves injection of replacement factor VIII directly into their blood, a process called "infusion." Some people with the mild type of hemophilia A can be infused with the chemical DDAVP (desmopressin acetate) or use a nasal spray containing highly concentrated DDAVP. DDAVP causes the release of factor VIII from storage sites in the body.
Hemophilia B
Hemophilia B is a deficiency of factor IX. About 15% of people with hemophilia have hemophilia B.
People with hemophilia B need to increase the level of factor IX in their blood. This can also be accomplished with "factor replacement therapy."
People with this type of hemophilia must inject replacement factor IX directly into their blood, a process known as infusion.
von Willebrand Disease
von Willebrand disease (VWD) is a hereditary disorder characterized by abnormally slow clotting of the blood.
Patients with von Willebrand disease may have spontaneous and prolonged bleeding from the nose and gums. VWD occurs in about one in 100 persons and is equally likely to affect both males and females.
von Willebrand disease occurs when one of the coagulation factors, called von Willebrand factor, is not sufficiently present in the blood or does not work properly.
The lower the level of active von Willebrand factor in a person's blood, the more severe the disease. In many patients with von Willebrand disease, another coagulation factor, factor VIII, is also affected.
For more information about Hemophilia A, Hemophilia B, and von Willebrand disease, and the CSL Behring products available to treat these disorders, please visit these CSL Behring websites:
www.allaboutbleeding-us.com
www.corifact.com
www.helixatefs.com
www.Hemophiliamoms.com
www.humate-p.com
www.riastap.com
www.stimate.com
Important Safety Information for Corifact
Corifact®, FXIII Concentrate (Human), is intended for routine preventive treatment of congenital Factor XIII deficiency. No controlled studies have been done demonstrating a direct benefit of Corifact in the treatment of bleeding events.
Corifact must be given by a healthcare professional through an intravenous injection. Your physician will monitor your Factor XIII levels throughout treatment with Corifact.
Do not use Corifact if you have experienced severe, immediate sensitivity reactions (including shock) to any component of Corifact. If during or after a Corifact infusion, you notice hives, rash, tightness of the chest, wheezing, shock or other undesirable reactions, let the treatment administrator or your physician know as soon as possible. In addition, reduced blood pressure has been known to occur around the time of infusion.
In clinical studies, adverse reactions reported in more than 1% of patients following treatment with Corifact were sensitivity reactions (including allergy, rash, itching, and skin redness), chills or fever, joint pain, headache, and an increase in liver enzymes.
Corifact is made from human blood plasma, and the risk of transmitting infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.
Please see full prescribing information for Corifact.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Important Safety Information for Helixate FS
Helixate® FS, Antihemophilic Factor (Recombinant), is a recombinant factor VIII treatment indicated for the control and prevention of bleeding episodes and peri-operative management in adults and children (0-16 years) with hemophilia A. Helixate® FS is also indicated for routine prophylaxis to reduce the frequency of bleeding episodes and the risk of joint damage in children with hemophilia A with no preexisting joint damage.
The most serious adverse reactions are systemic hypersensitivity reactions and the development of high-titer inhibitors necessitating alternative treatments to antihemophilic factor. The most common adverse reactions observed in clinical trials were inhibitor formation in previously untreated or minimally treated patients, skin-associated hypersensitivity reactions, infusion site reactions, and central venous access device (CVAD) line-associated infections.
Helixate® FS is contraindicated in patients who have manifested life-threatening immediate hypersensitivity reactions, including anaphylaxis, to the product or its components, including mouse or hamster proteins.
Please see the full prescribing information for Helixate FS.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Important Safety Information for Humate-P
Antihemophilic Factor/von Willebrand Factor Complex (Human), Humate-P? is approved to treat and prevent bleeding in adult patients with hemophilia A (classical hemophilia). Humate-P also treats spontaneous or trauma-induced bleeding episodes in adults and children with von Willebrand disease (VWD) and prevents excessive bleeding during and after surgery in patients with mild, moderate or severe VWD. Humate-P is not known to prevent spontaneous bleeding episodes.
Do not take Humate-P if you have had extreme sensitivity or an allergic response to antihemophilic or von Willebrand factor preparations. Your doctor will monitor you for events related to abnormal blood clotting.
Humate-P is made from human blood and could contain infectious agents. The risk that these agents may transmit disease cannot be completely eliminated, but has been reduced by screening plasma donors and testing donated plasma for certain viruses, and by inactivating and/or removing viruses during manufacturing.
In studies, more than 5% of patients reported the following adverse reactions to Humate-P: allergic/anaphylactic reactions, including hives, chest tightness, rash, itching, and swelling. The most common adverse reactions after surgery were bleeding at the wound or infusion site, and nosebleeds.
Please see full prescribing information for Humate-P.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Important Safety Information for Stimate Nasal Spray
Stimate® (desmopressin acetate) Nasal Spray, 1.5 mg/mL is a treatment used to stop some types of bleeding in people with mild hemophilia A or mild to moderate von Willebrand disease (VWD) Type 1. Stimate Nasal Spray should not be used in children under 11 months of age.
All patients using Stimate Nasal Spray are at risk of water intoxication, fluid overload and low sodium levels in the blood. Follow your healthcare provider’s instructions on limiting the amount of fluid you drink when using Stimate Nasal Spray, as too much fluid intake can lead to serious adverse reactions, including seizures, coma, and even death. Fluid restrictions are especially important for children and elderly patients, as they are at higher risk for these reactions.
See the patient information leaflet in the prescribing information for Stimate Nasal Spray for symptoms that could mean your blood sodium level is low—including headache, hallucinations, confusion, restlessness, weight gain and muscle spasms. Immediately report any of these symptoms to your physician or, if necessary, an emergency department. Also contact your doctor immediately if you have uncontrolled bleeding.
Before being prescribed Stimate Nasal Spray, make sure your doctor knows about all your medical conditions and about any medications you are taking. Use Stimate Nasal Spray exactly as your healthcare provider has instructed.
Side effects of Stimate Nasal Spray generally come from having too much water in the body. The most common include facial flushing, nasal congestion, runny nose, nose bleed, sore throat, cough, and upper respiratory infections. Tell your healthcare provider if you experience a side effect that does not go away.
Please see full prescribing information for Stimate Nasal Spray, which includes the patient information leaflet.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Important Safety Information for RiaSTAP
RiaSTAP®, Fibrinogen Concentrate (Human) is indicated for the treatment of congenital deficiency of fibrinogen, a key component in the blood clotting process. No controlled trials have demonstrated a direct benefit of RiaSTAP in treating bleeding episodes. RiaSTAP is not indicated for patients with sufficient fibrinogen that does not function properly (dysfibrinogemia).
RiaSTAP was approved based on a test that measures the firmness of a blood clot. This test is thought to be a likely predictor of efficacy in bleeding episodes, though a direct correlation has not been established.
If you have had an anaphylactic or severe systemic reaction to human fibrinogen preparations, you should not take RiaSTAP. Your doctor will monitor for early signs of blood clotting episodes and allergic or hypersensitivity reactions, and may discontinue administration if necessary.
RiaSTAP is made from pooled human plasma. Products made from human plasma may contain infectious agents, such as viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
In clinical studies, the most serious adverse reactions reported in those receiving RiaSTAP were clotting episodes and allergic-anaphylactic reactions. The most common adverse reactions observed were allergic reactions, including chills, fever, nausea, and vomiting.
Please see full prescribing information for RiaSTAP.
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.