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How Hemophilia is treated
Indications for treatment
Treatment should be given as soon as possible for the following: -
1. Bleeding into a joint.
2. Bleeding into a muscle, especially in the arm or leg.
3. Injury to the neck, mouth, tongue, face or eye.
4. Severe knocks to the head and unusual headache
5. Heavy or persistent bleeding from any site
6. Severe pain or swelling in any site
7. All open wounds requiring stitches
8. Following any accidents that may result in a bleed
2. Bleeding into a muscle, especially in the arm or leg.
3. Injury to the neck, mouth, tongue, face or eye.
4. Severe knocks to the head and unusual headache
5. Heavy or persistent bleeding from any site
6. Severe pain or swelling in any site
7. All open wounds requiring stitches
8. Following any accidents that may result in a bleed
How Is Hemophilia Treated?
Hemophilia is treated by replacing the missing clotting factor in the blood. This is done by injecting the needed factor into a vein. Bleeding stops when enough clotting factor reaches the bleeding site. It is very important that treatment is given as quickly as possible to prevent long-term damage. The specific factors used to treat hemophilia are:
- Factor VIII for hemophilia A
- Factor IX for hemophilia B
With an adequate quantity of treatment products and proper care, people with hemophilia can live perfectly healthy lives. Replacement therapy can be used: - To prevent bleeding (prophylactic or preventive therapy)
- To stop bleeding when it occurs, on an as-needed basis (demand therapy)
Replacement therapy can be given as needed, or on demand, to stop bleeding as soon as possible after it begins. The goal is to prevent damage to joints, muscles, or other parts of the body from bleeding. The amount of clotting factor given depends on:
- The type of hemophilia
- The bleeding site and severity of the bleeding
- The person’s weight
- Whether the person has developed an antibody that neutralizes or knocks out the activity of the clotting factor. Clotting factors are found in the following blood products in order of decreasing concentration:
- Factor concentrates
- Cryoprecipitate
- Plasma
- Whole blood
Factor concentrates are the treatment of choice for hemophilia. They can be made from human blood (called plasma-derived products) or manufactured using genetically engineered cells that carry a human factor gene (called recombinant products). Factor concentrates are made in sophisticated manufacturing facilities. All commercially prepared factor concentrates are treated to remove or inactivate blood-borne viruses.
Cryoprecipitate is derived from blood and contains a moderately high concentration of factor VIII (but not IX) clotting factor. It is effective for joint and muscle bleeds, but is less safe from viral contamination than concentrates and is harder to store and administer. Cryoprecipitate can be made at local blood collection facilities.
In fresh plasma the red cells have been removed, leaving the blood proteins including the clotting factors. It is less effective than cryoprecipitate for the treatment of hemophilia A because the factor VIII is less concentrated. Large volumes of plasma must be transfused. This can cause circulatory overload.
The life of the clotting factors is preserved by making a product called fresh frozen plasma (FFP). FFP is still the only product available for treatment of hemophilia A and B in some countries.
There are considerable drawbacks to the use of whole blood in the treatment of hemophilia. First, it must be fresh because the activity of the clotting factors in the drawn blood decreases quickly with time. Second,the red cells it contains must, of course, be compatible with those of the recipient. Third, it takes a large volume of whole blood to stop a bleed and this volume may overload the circulation and cause the heart to fail.
When bleeding occurs, it is important to get treatment as soon as possible. Delayed treatment can lead to complications. The PWH and his family must learn to recognize signs of bleeding, and make sure that it is treated quickly.
Damage due to delays in treatment
When treatment for bleeding is delayed, damage to the area affected (such as a joint) can occur. It is important for persons with hemophilia to learn to recognize signs of bleeding as soon as possible after bleeding starts and to get treatment quickly.
Other Treatments
Desmopressin
Desmopressin (DDAVP) is a synthetic hormone used to treat people with mild to moderate hemophilia A. DDAVP cannot be used to treat hemophilia B or severe hemophilia A. DDAVP stimulates the release of factor VIII and von Willebrand factor stored in blood vessels and increases the level of these proteins in the blood. Von Willebrand factor carries and binds factor VIII, which then can stay in the blood circulation longer. DDAVP usually is given by injection or in a nasal spray.
Antifibrinolytic drugs
Antifibrinolytic drugs (including tranexamic acid and aminocaproic acid) are medicines used with factor treatment. They are usually given as a pill to help keep clots from breaking down. They are most often used:
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Before dental work
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For treating bleeding from the mouth or nose
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For mild intestinal bleeding
