Bleeding stops when enough clotting factor reaches the bleeding site. It is very important that treatment is given as quickly as possible for joint bleeds to prevent long-term damage. Factor VIII or IX concentrates are infused, or injected into the bloodstream, to prevent or control bleeding. They are very concentrated so that a small amount can control major bleeds, even in surgery. Factor replacement therapy can be effective for up to 3 days.
Although called ‘blood products’, today most factor concentrates are being manufactured using genetic engineering to create artificial and recombinant factor replacement products that contain no human blood components, making them even safer. Those that are made from donated human blood are subject to rigorous testing and viral inactivation steps to make them as safe as possible.
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.
- Start to follow the PRICE regimen (Protection, Rest, Ice, Compression, Elevation).
If you experience pins and needles in the bleed site or suspect a brain bleed or a bleed in another vital organ seek medical care immediately. For more information see What to do if you are having a bleed, a brochure produced by the Auckland DHB Haemophilia Centre.
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, and an HFNZ Outreach Worker with specific expertise in managing the healthcare needs of persons with bleeding disorders. See a list of Haemophilia Treatment Centres in New Zealand.
After a learning period at the haemophilia clinic, many people in New Zealand prefer to undertake the infusion of factor concentrates at home, which allows for quicker treatment when a bleed starts, a more normal life for the person with haemophilia, and a greater acceptance of treatment by young children. Children often learn how to infuse themselves around the age of ten, allowing for greater independence as they are able to treat bleeds anywhere, home, school or on holiday.
Instead of just using on-demand therapy when a bleed occurs, many children and young adults use prophylaxis therapy to prevent bleeding. By regularly infusing factor concentrates several times a week, prophylaxis therapy aims to keep the levels of Factor VIII or IX in the blood high enough that clots can form easily if a bleed occurs. Prophylaxis therapy gives children the best chance to reach adulthood without damage to their joints.
One important aspect of treatment is pain management, as due to the swelling, especially in the joints, bleeds can be extremely painful. It is very important that people with haemophilia avoid aspirin and most non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen as they suppress platelet function and reduce blood clotting. A doctor or pharmacist should also be consulted before trying any pain medication to discuss possible side effects.
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 for both factor VIII and IX have begun to see success.