Bayer Resources
Hemophilia Basics
Hemophilia Overview
Hemophilia is the name given to a variety of rare genetic blood clotting disorders. It affects primarily males who are missing some or all of one of the blood clotting proteins normally found in blood. Hemophilia A and B are the two most common forms of the disorder. Patients with hemophilia A (also called classical hemophilia), have either little or no clotting factor VIII. Patients with hemophilia B (also called Christmas disease), have either little or no clotting factor IX. About 85% of those with hemophilia have hemophilia A; the rest have hemophilia B.
Hemophilia is a hereditary disorder, and most people who have hemophilia A or B are born with it and have a family history of the disorder, but in approximately 30% of cases, there is no family history. It is possible for the gene mutation to occur spontaneously. Each year, approximately 400 babies in the United States are born with hemophilia.
Contrary to common belief, people with hemophilia do not bleed more profusely or faster than normal, rather they bleed for a longer period of time. How severe an individual's case of hemophilia is depends on the amount of clotting factor found in the blood. 70% of hemophilia A patients have less than one percent of the normal amount of clotting factor, and are said to have severe hemophilia. Patients who have up to five percent of normal clotting factor have mild hemophilia and rarely experience bleeding except after injuries or surgery.
The treatment of hemophilia has advanced considerably in the past several decades. The recombinant human factor VIII treatments available now, such as Kogenate® FS, utilize complex manufacturing steps that inactivate or remove viruses and prions to assure patient safety. Research is ongoing as we look into ways of improving the quality of life of patients with hemophilia, and gene therapy is being investigated as a possible cure.
Hemophilia Challenges
Treating hemophilia still poses considerable challenges. These are the most important challenges facing the hemophilia patient, health care provider, and research community today:
- Ensuring the safety of products used in the treatment of hemophilia
- Managing hemophilia, including inhibitor formation, irreversible joint damage, and life-threatening hemorrhage
- Making progress toward a cure for hemophilia
Remember, your doctor or healthcare provider is the single best source of information regarding you and your health. Please consult your doctor if you have any questions about your health, your symptoms, or your therapy.
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