Each bleeding episode must be promptly treated by an intravenous infusion of the clotting factor which is deficient. Once the bleeding stops, pain rapidly diminishes and use of the limb returns. The factor concentrate is administered as an intravenous injection. This concentrate is manufactured as a white powder which should be reconstituted with sterile water, which is provided with the factor.
The factor VIII and factor IX concentrates used for treatment of haemophilia can be manufactured from blood plasma, these are known as plasma-derived factor concentrates. Alternatively, they can be manufactured through a recombinant cell line, these are known as recombinant factor concentrates. The factor concentrates used for treatment of factor VIII and factor IX deficiency in Ireland are recombinant factor concentrates. Plasma-derived factor concentrates are used in Ireland for treatment of von Willebrand’s Disease, as there is currently no recombinant factor concentrate available.
What cannot be emphasised enough is that a person with haemophilia must have treatment as soon as a bleed starts. It prevents further bleeding, pain and, most importantly, reduces the likelihood of permanent damage to joints (target joints).