Haemophilia A

Haemophilia A

Haemophilia A is caused by a deficiency of a protein in the blood; Factor VIII. The level of factor VIII in the blood determines the severity of haemophilia. Factor levels of less than 1-2% are found in people with severe Haemophilia A, they experience spontaneous bleeds into joints or muscles. Moderate haemophilia is found in people with Factor levels of 2-5% and they may experience spontaneous bleeds. Or those with mild haemophilia, who have factor levels of between 5-50%, usually only experience bleeds following trauma or surgery.
Children with severe Haemophilia A often begin to show increased bruising around the age of one year. It may present earlier, if the child under goes surgery, or after minor trauma, including immunisations.
The major problem in severe Haemophilia A is painful bleeding into joints, mainly the knees, ankles and elbows. These bleeds may occur spontaneously. If not treated treated promptly the bleeds may result in permanent arthritis and disability.
Most children and adolescents with severe Haemophilia A receive preventative treatment (prophylaxis) with an intravenous infusion of Factor VIII, 2-3 times a week. This is to prevent spontaneous joint and muscle bleeds.
Most person with Factor VIII deficiency requiring treatments will have a synthetic type of Factor VIII called Recobinant Factor VIII. A plasma derived Factor VIII will be available if required. Those with mild Haemophilia may be treated with DDAVP.
With appropriate management and factor replacement children and adults with inherited bleeding disorders can lead active and normal lives.