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Hemophilia Treatment Glossary
Hemophilia: Hemophilia is a hereditary bleeding disorder where the clotting ability of blood is impaired, resulting in uncontrolled bleeding. Small wounds are usually not a problem but when the bleeding is internal, pain, swelling, permanent damage, especially to joints and muscle, and in some severe cases, death, may occur.
Symptoms: Symptoms of hemophilia include prolonged bleeding from cuts, tooth extraction or surgery, easy bruising, spontaneous bleeding into joints with associated pain and swelling, and other types of internal bleeding.
Cause: Hemophilia is usually caused by an inherited defective gene with a sex-linked recessive trait located on the X chromosome. Females can be carriers of the trait while 50% of their male offspring can have the disease. Some cases of hemophilia have no family history and develop because of a spontaneous mutation in a gene.
Types: Severity of the symptoms varies from mild to severe. People with mild hemophilia (6-49% of normal clotting factor) usually bleed only after serious injuries, trauma or surgery. Moderate hemophiliacs (1-5% of normal clotting factor) may bleed from injuries, trauma or surgery and sometimes experience bleeding episodes with no apparent cause. People with severe hemophilia (less than 1% of normal clotting factor) may experience recurring episodes of bleeding of no apparent cause in addition to bleeding from injuries, trauma or surgery.
Treatment: Abrasions and small cuts may be treated first by applying basic first-aid: clean wound, apply pressure and band-aid. Big or deep cuts and internal bleeding are treated by replacing the missing clotting factor with an infusion of clotting factor concentrate to bring the clotting factor to a level close to normal so that clots can form and bleeding stops.
Other Bleeding Disorders:Besides Hemophilia, there are other bleeding disorders such as von Willebrand (low levels of a protein that helps the blood clot and/or the protein does not work as it should), Thrombocytopenia (low platelet count), Idiopathic Thrombocytopenic Purpura (blood does not clot as it should due to a low number of platelet), and Hypercoagulation (tendency for the blood to clot too much).
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Antihemophilic Factor (factor or clotting factor): Antihemophilic factor is a protein that is naturally produced by the body to aid in the clotting of blood to stop bleeding. In patients with hemophilia, the body does not make enough factor. If you sustain an injury that results in a bleed, your blood does not clot properly and you may continue to bleed into your muscles and joints.
The most common way to prevent or stop this is to provide your body with factor that is produced either naturally or artificially. Factor produced naturally comes from human or pig blood and factor made artificially comes from man-made processes. Factor from human blood has been extensively treated and is not likely to contain harmful viruses such as human immunodeficiency virus (HIV) or hepatitis B or C viruses. Factor produced from pig blood and by artificial means do not contain these viruses.
Inhibitor Management: Inhibitors are antibodies (proteins) that block the activity of clotting factor, which may occur after an infusion of factor. This occurs in a small percentage of patients with hemophilia. Inhibitors may be sustained with infusions of large doses of factor or by infusions of bypass agents (other clotting factors that do not contain the factor the antibodies are fighting against) to clot the blood by another pathway. Our medical team is experienced in treating patients with inhibitors and can help with the management of it.
Self-infusion: Self-infusion is treating yourself with factor. Learning to self-infuse factor is a great way to become more responsible in your health and self-care and allows you to have more freedom because you don’t have to go to your hemophilia treatment center for an infusion. By self-infusing, you will be able to inject your body with factor intravenously with a needle and syringe whenever you need to, whether you are at home, work or vacation. Our experienced nursing staff will demonstrate the safe and essential techniques for a successful self-infusion of factor product.
Radiosynovectomy: A procedure to elevate pain and bleeding at a joint site caused by inflammation and damage of the synovium, a thin layer of tissue that lines the joint space. A small amount of radioactive substance is directly injected to the inside of the joint to destroy the synovium. There is little risk of radiation damage to other parts of the body as the radioactive particles travel over very short distances.
Joint Reconstruction or Joint Replacement: This is a surgery to replace all or part of a joint, for example, the knee or the hip. Los Angeles Orthopaedic Medical Center is the home of world-renew orthopedic surgeons who specialize in replace joints in patients with hemophilia. These surgeons also specialize in other types of orthopedic surgeries to help elevate pain from joint and bone disorders that may have result from hemophilia complications.
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Infectious Disease: In the past, some plasma factor products carried the risk of transmitting infectious diseases such as the hepatitis and HIV viruses. Some factor concentrate in the early 1980’s was manufactured using blood that was contaminated with the HIV virus and many Hemophilia patients contracted HIV. Today, the factor products are manufactured under tight screening and processing methods, thereby eliminating the risk of contracting HIV through factor. In addition, genetically-engineered recombinant factor further reduces that risk.
HIV: HIV stands for human immunodeficiency virus, a virus that damages or destroys the cells of your immune system. HIV can be acquired by contact with blood contaminated with the HIV virus, through unprotected sex with an infected person, through sharing needles with an infected person or from an infected mother to her baby during childbirth. HIV interferes with your body’s natural ability to fight off bacteria, fungus and other viruses. Therefore you become more susceptible to certain types of infections or diseases that your body would normally overcome without any problems. The later stages of HIV infection is known as AIDS (acquired immunodeficiency syndrome).
Factor from human blood was not previously screened for HIV. However it was discovered that some factor produced from blood contaminated with HIV was inadvertently passed on to patients with hemophilia when they infused themselves with the tainted factor product. Since then, all hemophilic factor produced by human blood are vigorously screened for HIV.
Hepatitis B/Hepatitis C: Hepatitis B and hepatitis C are viruses that cause liver inflammation that may cause the liver to swell up and stop working efficiently. Hepatitis B or hepatitis C can be acquired by contact with blood contaminated with the virus, through unprotected sex with an infected person, through sharing needles with an infected person or from an infected mother to her baby during childbirth.
Before 1987, factor from human blood was not vigorously screened for hepatitis B or hepatitis C. However it was discovered that some factor produced from blood contaminated with hepatitis B or hepatitis C was inadvertently passed on to patients with hemophilia when they infused themselves with the tainted factor product. Since then, all hemophilic factor produced by human blood are also carefully screened for hepatitis B and hepatitis C.
Hemophilia and Physical Therapy: Physical therapy plays an important part in reducing joint problems caused by repeated bleeding into muscle and joint areas. Physical therapy is also vital in helping patients recover properly from joint surgery or other orthopedic procedures. The physical therapist may work with you on exercises such as stretching, movement exercises, resistance training (for example, weight lifting, which puts tension on muscles) or hydrotherapy (water therapy). Other therapies include splints, ice, heat, ultrasound or nerve stimulation to the affected area.
Prophylaxis: Prevention of and stopping a bleed quickly is an important part of staying healthy. Some patients may benefit from periodic infusion (prophylaxis) as part of their hemophilia treatment plan. Prophylaxis works to keep the factor level in the body high enough so that bleeding may be avoided altogether. However there are disadvantages to this approach, some of, which includes the frequency of infusions, continual access to veins by catheter and the high cost of factor which may exceed medical insurance caps for some people. The decision to begin a prophylaxis regimen should be discussed in details with your doctor.
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