Indications and Usage
Antihemophilic Factor (Human), Koāte^®-DVI is indicated for the treatment of classical hemophilia (hemophilia A) in which there is a demonstrated deficiency of activity of the plasma clotting factor, factor VIII.

Koāte^®-DVI provides a means of temporarily replacing the missing clotting factor in order to correct or prevent bleeding episodes, or in order to perform emergency and elective surgery on individuals with hemophilia. Koāte^®-DVI contains naturally occurring von Willebrand factor, which is co-purified as part of the manufacturing process.

Koāte^®-DVI has not been investigated for efficacy in the treatment of von Willebrand disease, and hence is not approved for such usage.

Learn about the Talecris screening and product safety program for manufacturing Koāte^®-DVI

About Hemophilia
Hemophilia is a genetic disorder characterized by the deficiency or absence of one of the proteins (or factors) in blood plasma that is responsible for clotting. Low levels of one of these plasma proteins result in bleeding and difficulty clotting in the affected individual. There are more than a dozen clotting factors in our bodies. The most common deficiency of a clotting protein is factor VIII although any one of these can be deficient or absent. The second most common deficiency is Factor IX deficiency.

Factor VIII Deficiency – Hemophilia A, Classic hemophilia

Factor IX Deficiency – Hemophilia B, Christmas Disease

There are factor VIII and IX concentrates that are manufactured to treat these two types of hemophilia.

Treatment for hemophilia
The treatment of hemophilia involves replacement of the clotting factor that is missing from the person's circulation. One such factor replacement therapy is Koāte^®-DVI, Antihemophilic Factor (Human) that is produced by Talecris Biotherapeutics.

Manufacturers like Talecris have developed concentrates containing clotting factors VIII and IX that are used for the treatment of persons with hemophilia. These clotting factor concentrates are packaged in a freeze-dried formulation that requires reconstitution with sterile water for injection before being infused into the vein of a person with hemophilia.

Hereditary factors 
The genetic coding for hemophilia is carried on the X chromosome. Females have two X-chromosomes and contribute a copy of one to each of their offspring. If they are carrying the abnormal gene for hemophilia on one of their X-chromosomes, they will not have hemophilia, but they will be considered a carrier of hemophilia. If a male child receives the abnormal gene, that child will have hemophilia. 

 

Males have an X and a Y chromosome and whichever chromosome they contribute to their child will determine the gender (sex) of that child. If a man has hemophilia and has daughters, all of his daughters will be obligate carriers because they will have his X' chromosome for hemophilia.

Occurrence
There are approximately 17,000 people with hemophilia in the United States. Although there is genetic transmission of hemophilia, approximately one third of all persons with hemophilia have no family history of this disorder. There are genetic counselors available at Hemophilia Treatment Centers that can assist families with family planning and prenatal diagnosis.

Hemophilia-related bleeding 
In young children, the most common hemophilia-related bleeding is related to activities such as crawling, walking, and climbing. Common sites of bleeding are joints, muscles, and soft tissues. Life-threatening bleeding can occur in the head, spine, near the airway (neck or below the tongue), within the gastrointestinal system or other sites within the central nervous system, and should be treated as an emergency.

Common bleeding sites:
Joints
Joints are the most common site of bleeding in hemophilia (also called hemarthrosis). The most common location within the joint is bleeding in the synovium (lining of the joint). If not treated, the bleeding continues until the joint space is filled. In young children, bleeding into joints does not usually occur until they become ambulatory.

Most persons with hemophilia will experience bleeding into other joints, such as knees, ankles, and elbows, but bleeding in any joint can occur. If there is repeated bleeding into the same joint, risk for development of chronic joint disease occurs. Joint bleeding is the most common hemophilia related problem.

Muscles
Bleeding into muscles is the second most frequent site of hemophilia-related bleeding. Muscle bleeding can occur in any part of the body. If not treated, muscle bleeding can lead to significant blood loss from the circulation into a muscle.

Soft tissue
Bleeding can occur under the skin causing bruising and "lumps" or hematomas. This bleeding does not typically require treatment with factor replacement unless it occurs in a small space such as around the eye or in a finger or toe.

Other sites of bleeding:
Urinary bleeding (hematuria)
Blood in the urine may occur spontaneously in persons with hemophilia. If these episodes are painless, do not cause significant blood loss, and resolve on their own, they are usually not cause for concern. If however, these episodes continue, lead to significant blood loss, are associated with pain, or recur frequently, they should be evaluated.

Nose bleeding (epistaxis)
Nose bleeding in persons with hemophilia is unlikely to occur more frequently than for people without hemophilia. If it occurs frequently, results in significant blood loss, or interferes with activities, it should be evaluated and treated.

Lacerations
Cuts or lacerations requiring sutures should be treated with factor replacement therapy in persons with hemophilia. During suture removal, the person with hemophilia should also receive factor replacement therapy.

Life-Threatening Bleeding
If bleeding occurs in the head, spine, near the airway (neck or below the tongue), within the gastrointestinal system or other sites within the central nervous system, it should be treated immediately. Even if suspected bleeding or trauma occurs to the head, immediate treatment should be sought. Some of these sites of bleeding can occur without known trauma, therefore bleeding into these areas requires treatment.

Hemophilia care in the United States
The federal government organized a system of Hemophilia Treatment Centers (HTCs) in the US beginning in the mid-1970s. These 146 treatment centers have experts who provide care to families and patients affected by hemophilia. The staffs at the HTCs provide diagnostic, treatment, support, and other assistance. The core team at an HTC is usually comprised of a hematologist, a nurse coordinator, a social worker, and a physical therapist. Most centers have extended teams that include dentists, orthopedists, geneticists, and other professionals skilled in providing services to those families who seek care from these centers.

These centers also provide research opportunities for their constituents. Hemophilia research gives patients and families the option of participating in state-of-the-art research programs that test new products for treatment of hemophilia, different methods of treatment, and treatment for the complications arising from hemophilia.

For a full listing of these Hemophilia Treatment Centers, contact the National Hemophilia Foundation.

Important Safety Information
Individuals who receive infusions of blood or plasma products may develop signs and/or symptoms of some viral infections, particularly hepatitis C. It is emphasized that hepatitis B vaccination is essential for patients with hemophilia and it is recommended that this be done at birth or diagnosis. Hepatitis A vaccination is also recommended for hemophilic patients who are hepatitis A seronegative. As with all plasma-derived therapeutics, the potential to transmit infectious agents cannot be totally eliminated.

Please click here for Koāte^®-DVI full prescribing information (PDF).