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.

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 .

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).

This clotting factor concentrate is packaged in a freeze-dried formulation that requires reconstitution with sterile water 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 (see below) 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.

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.

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

Important Safety Information
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.

Allergic-type reactions may result from the administration of Antihemophilic Factor (Human) preparations. Reactions include tingling in the arm, ear, and face, blurred vision, headache, nausea, stomach ache, and jittery feeling.

Koāte-DVI is made from human plasma. As with all plasma-derived therapeutics, the potential to transmit infectious agents, such as viruses and theoretically, the Creutzfeldt-Jakob (CJD) agent that can cause disease, cannot be totally eliminated. There is also the possibility that unknown infectious agents may be present in such products.

Hepatitis B vaccination is essential for patients with hemophilia A; vaccination is recommended at birth or at the time of diagnosis. Hepatitis A vaccination is also recommended for hemophilia who are hepatitis A seronegative.

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

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.