Replacement therapy with factor concentrates
Hemophilia Treatment is carried out by substitutionmissing clotting factor contained in a preparation that is manufactured from plasma or blood, or a synthetically in accordance with genetic engineering techniques. The factor can be administered into a vein nurse in a clinic under the supervision of a doctor, your family doctor or from the center of hemophilia at home parents, specially trained, or are themselves hemophiliacs, specially prepared and confident that they can do it.
Although the dried and frozen (powdered)clotting factor concentrate is the most comfortable to hold and use, less expensive locally made blood products such as cryoprecipitate or fresh frozen plasma are also effective and can be used outside the clinic and for home treatment, if there is a corresponding fridge freezer.
The problem is the content of virusesblood preparations. However, currently provided formulations are produced in a manner that reduces the risk of contamination with viruses such as different types of hepatitis viruses and human immunodeficiency virus (HIV) which causes AIDS. Donors usually fill a questionnaire about their health, and blood, which they pass, checked for these diseases. The risk of infection through the latest blood products is very small, especially by those developed synthetically.
Infection is transmitted through blood
The most severe infection, transmitted throughtransfusion of blood and blood products is the HIV, causes AIDS. Although there is very little evidence that HIV can be transmitted in the home, it should be with great care to handle needles, blood products and blood-spattered. You must use gloves to avoid injury from needles and protect cuts and scrapes on their hands to anyone who enters the medication to the patient with hemophilia. Needles, syringes and vials of a drug must be disposed of in an environmentally way, usually through the clinic. Different people should never use the same syringe or needle. If any part of the equipment for injection is intended for multiple use, it should be thoroughly sterilized every time.
HIV can also be transmitted through sexual contact, andand from an infected mother to her child during gestation. The use of condoms significantly reduces the risk of transmission of sexually transmitted infections.
Desmopressin and analogs
Desmopressin is a chemical drug, notrelating to blood. It acts by activating the reserves of factor VIII. It gives a positive result in the case of medium and mild deficiency of factor VIII (hemophilia A) or von Willebrand's disease. It does not help with severe hemophilia A when factor VIII is almost absent and ineffective for any degree of severity of the factor IX deficiency.
Types of treatment and complications
Treatment should begin as soon as possible,after bleeding began. Pain in this case takes place in a matter of minutes. In some cases, the required dose is required to re-enter, for example, if the pain and swelling in the joint or the muscles do not go if there is a wound in the mouth, where the bleeding is stopped and the first few hours renews unless stitches or if there was a head injury.
Antifibrinolytic drugs (Tsiklokapron,Amicar) may also be used. They slow the natural breakdown of blood clots and help especially for the treatment of wounds in the mouth or tooth extraction site. Blood products may also be used for prophylaxis of bleeding, as well as to the procedures of physical therapy, exercise and sports.
In many countries, it is widely practiced programtreatment of hemophilia organizations at home. Treatment is carried out under the supervision of a doctor or nurse from the nearest hemophilia center or clinic. Parents or patients themselves learn administered blood products themselves. Thus, it is possible to provide initiation of treatment for injuries, as soon as possible, and provide the patient with a degree of independence, since it reduces the number of visits to the center, or hospital that take a lot of time and go to the financial, emotional and social burden on the patient with hemophilia and his family, and also on society.
Provide rest the affected body parts
In severe hemorrhages in joints or musclesto some extent necessary to provide rest (splint) affected body part to avoid further deformation. There are various kinds of finished tires, and some can easily be manufactured independently. People need to learn how to apply them and to take into account the duration of the tire overlay. Over the duration of use of the tires can lead to malnutrition (weakening) of muscle or joint instability.
Physiotherapy should be carried out tostrengthen muscles and accelerate the process of restoring the fullness of motion of the affected joints and muscles after heavy bleeding. Physiotherapy should be started immediately once the acute pain stops. Strong active muscles protects joints.
Surgery may be necessary forcorrecting limb deformation, tooth extraction, or for other reasons. The day before surgery and for several days after the patient is given large doses of blood clotting factors required to maintain a system of blood clotting at almost normal levels, allowing completion of the process of wound healing. Thus, surgery can be carried out on the background of moderate bleeding. However, the required number of factors, ensuring a sufficient level of blood clotting significantly in volume and is very expensive. In this regard, the surgical procedure hemophiliacs must be performed only in a clinical physicians specializing in hemophilia if there are adequate for this surveillance equipment during operation.
Aspirin. be careful
The major component of all commonly usedanalgesic drugs is aspirin, which affect the normal blood clotting and increase the tendency to bleeding. Aspirin also irritate the stomach. If you have hemophilia, do not use pain relief medications containing aspirin.
Consult your physician or atexperts in pharmacy about which contain analgesics aspirin. An alternative would be in the prescribed dose of paracetamol. Remember that all medications, including acetaminophen, as well as home care products are hazardous to health and must be kept out of the reach of small children.