Hemophilia is a bleeding disorder. It is genetic in nature and is relatively rare in occurrence. This condition affects the clotting mechanism of the body where the blood fails to clot.
Since hemophilia is x linked recessive disorder, this condition affects males more commonly than females.
Types of hemophilia’s:
- · hemophilia A: this is a condition in which there is a deficiency of factor VIII of the body’s clotting factors.
- · hemophilia B: in this condition there is a deficiency of factor IX of the body’s clotting factors.
- · hemophilia C: this is not completely a recessive disorder. This type of hemophilia occurs due to the deficiency of factor XI.
The most common type of hemophilia is the hemophilia A.
Hemophilia occurs mainly because of the lowered levels of clotting factors in the body. In this case when there is an injury, due to the lowered levels of clotting factors in the body the fibrin formation fails to take place. To maintain the blood clot the fibrin formation is necessary. However the temporary scab formation does take place, but this fails to stop the bleeding.
When an individual is suffering from the severe form of hemophilia, he can bleed for several days to week sometimes even after a minor injury.
Signs and symptoms:
Symptoms of hemophilia can be seen in children in the form of large bruises after a fall. In mild cases of hemophilia, noticeable symptoms may not be seen.
In severe hemophilia patients there might be severe cases of internal bleeding after an injury which can be fatal at times.
Severe cases of hemophilia are not that common. Only about 1% of hemophilia patients suffer from the severe form of hemophilia.
Bleeding can occur sometimes in the joint spaces, the muscles and the subcutaneous tissues which may need treatment.
Management oh hemophilia:
Managing a patient suffering from hemophilia correctly is very important. No cure for hemophilia has been found yet. But there are various measures by which the bleeding can be controlled and the condition of the person can be improved drastically.
The aim of the treatment is to restore the missing clotting factor. This can be done by replacing the missing factor from human blood serum or recombinant. Sometimes the combination of the two may be needed.
Concentrates of factor VIII or IX are given to the patient intravenously depending on the type of hemophilia the individual is suffering from.
Desmopressin treatment can be given for people suffering from mild forms of hemophilia A. this can be given in the form of an injection or nasal spray.
Medicines which prevent the breakage of a blood clot can be given. These are the antifibrinolytics. These medicines are used to control any form of bleeding from the mouth. So used mostly by the dentists before carrying out any operative procedures on hemophilia patients
Complications of the replacement therapy:
- · Development of antibodies which act against the replaced clotting factors.
- · When human clotting factors are given, sometimes viral infections are developed from them.
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