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 is reconstituted with sterile water, which is provided with the factor. The factor VIII and factor IX concentrates used for the 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 the treatment of factor VIII and factor IX deficiency in Ireland are recombinant factor concentrates.
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 it reduces the likelihood of permanent damage to joints (target joints).
Early treatment is essential to minimise any long term damage. If you are treating a bleed at home or outside of the hospital you should:
- Administer factor replacement as soon as possible.
- Contact your haemophilia centre to arrange for an appointment to see your nurse or physiotherapist.
- Follow the RICE regimen (Rest, Ice, Compression, Elevation)
Haemophilia is treated using a comprehensive care model. Haemophilia Treatment Centres provide a comprehensive service for people with haemophilia and their family, for the treatment of haemophilia and related conditions. The comprehensive care team consists of a haematologist, nurse coordinator, physiotherapist, dentist and psychologist with specific expertise in managing the healthcare needs of persons with bleeding disorders.
There is no cure for haemophilia yet, but gene therapy remains an exciting possibility and holds out the prospect of a partial or complete cure for haemophilia. There are many technical obstacles to overcome, but it is encouraging to see that clinical trials have begun.
