How does blood clot ?
Bleeding causes a biological "domino effect" in which a series of steps are set in motion. Blood vessels begin to shrink to reduce blood flow. Then, platelets (blood-clotting cells) begin to collect at the injured site to form a temporary plug. In the meantime, clotting factor proteins are sequentially activated in the blood clotting cascade to form a fibrin clot at the wound site, and stopping the bleed.
What is clotting factor ?
Clotting factors are proteins found in blood that work together to make blood clot. They are designated by Roman numerals I through XIII. When the body detects bleeding, clotting factors are switched on in a specific order, each activating the next factor in the blood clotting cascade to ultimately form a fibrin clot. The fibrin acts like a net that holds the platelets together to make a firm blood clot. Factor VIII is one of the clotting factor proteins that help produce the fibrin clot. In persons with haemophilia A, whose bodies do not make sufficient Factor VIII, fibrin is not made properly so firm blood clots do not form in the wound and bleeding is not easily stopped.
What is haemophilia ?
Haemophilia is a rare disorder in which a person's blood does not clot properly because the body does not produce enough of, or is missing, the blood clotting proteins, Factor VIII or Factor IX. The body depends on these clotting factors to stop bleeding after injury and to promote healing. When a person with haemophilia gets a cut or bleeds internally, he bleeds longer than someone without haemophilia. Internal bleeding is usually into joints and muscles, but can also occur in the brain or other organs.
What are the types of haemophilia ?
Haemophilia A, also called classical haemophilia, occurs when clotting Factor VIII is either absent or not present in sufficient amounts. Haemophilia B, also called Christmas disease, occurs when clotting Factor IX is either absent or not present in sufficient amounts.
What are the levels of severity of haemophilia ?
The severity of haemophilia is dependent on the amount of clotting factor that is either missing or deficient.
• Mild haemophilia A patients have Factor VIII levels of 6% to 50% of normal.
• Moderate haemophilia A patients have Factor VIII levels of 1% to 5% of normal.
• tenderness, and
• Severe haemophilia A patients have Factor VIII levels of <1% of normal.
Can haemophilia be cured ?
There is presently no cure for haemophilia, but with proper treatment using clotting factors, everyone, even people with severe haemophilia, can enjoy a full and active life.
What are the treatment options for people with haemophilia?
Haemophilia can be treated when it causes bleeds by infusing the missing factor in a vein. For patients with haemophilia A, this is known as Factor VIII replacement therapy. This therapy works by substituting the missing clotting Factor VIII protein. When clotting factor is administered after an injury, the body begins to use it immediately to form a clot. This therapy is not a cure for haemophilia A-it is a temporary replacement therapy used to treat symptoms of haemophilia. In people without haemophilia, clotting factor is continuously used and remade by the body. There are many types of Factor VIII concentrates available for replacement therapy. A well-recognized and safe treatment for haemophilia A is an ultra-pure Factor VIII concentrate that is made using recombinant gene technology.
How much does it cost ?
Unfortunately the factor treatment is very expensive as it is produced in western countries and has to be imported. You will be registered with the haemophilia society and you may be able to get the factor for a fraction of its original cost. If the factor is not available, we will have to give you a blood product called cryoprecipitate, which can be obtained from a blood bank. This is not the preferred mode of treatment as the rise in factor level after the treatment is not reliable.
Living with Haemophilia
Preventing bleeding and staying healthy are important if you have Haemophilia. You should:
• Avoid over-the-counter medicines that can affect blood clotting, including aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs).
• Always check with your doctor before taking any medicines.
• Tell your doctor and dentist that you have haemophilia. Your dentist can talk to your haematologist about whether you need medicine before dental work to reduce bleeding
• Exercise regularly and maintain a healthy weight. Exercise helps keep muscles flexible. It also helps prevent damage to muscles and joints. Always stretch before exercising.
Some safe exercises or activities are swimming, cycling, and walking. Football, hockey, cricket, wrestling, and lifting heavy weights are not safe activities if you have bleeding problems. Always check with your doctor before starting any exercise program.
Since your parents, brothers and sisters, and children may also have haemophilia, you should consider getting them tested.
You should always carry a "haemophilia card" which will be given to you after the diagnosis.