People with haemophilia are at risk of prolonged and excessive bleeding caused by a deficiency of clotting factor VIII or factor IX. The aim of treatment is to reduce the risk of prolonged and excessive bleeding. Haemophilia can be treated safely and effectively by the use of clotting factors medicines.
Clotting factor medicines are given as concentrates (called factor concentrates or factor replacements), which are infused, or injected into the bloodstream. They are very concentrated so that a small amount can control major bleeds, even in surgery. For severe bleeds it is very important that treatment is given as quickly as possible to prevent long-term damage. Clotting factor medicine can be effective for up to 3 days.
Most factor VIII or IX medicines are made using genetic engineering to create artificial products that contain no human blood components. Those that are made from donated human blood are subject to rigorous testing and viral inactivation steps to make them as safe as possible.
Approaches to treatment
There are two main approaches to treatment, depending on how severe your condition is:
- preventative therapy – where regular injections of clotting factor medicine are given to help prevent episodes of bleeding and prevent joint and muscle damage
- on-demand therapy – where an injection of clotting factor medicine is given in response to a bleed.
Most cases of haemophilia are severe and need preventative treatment (prophylaxis). This involves regular injections of clotting factor medicine.
Instead of just using on-demand therapy when a bleed occurs, many children and young adults have regular injections of clotting factor medicine to prevent bleeding. Preventative 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.
Preventative therapy gives children the best chance to reach adulthood without damage to their joints.
In mild or moderate cases, treatment for haemophilia may only be necessary as an immediate response to bleeding.
Early treatment is essential to minimise any long-term damage and to lessen any disruption to daily life. If you are treating a bleed at home or outside of the hospital you should:
- Inject the clotting factor medicine 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: protect your joint by not putting weight on it, at least for the first 48 hours, and possibly longer if it is a severe bleed. Use crutches or other supports.
- Rest: completely rest the affected area, to allow the swelling to ease and prevent further bleeding, but remember, too much rest can also be damaging, so start to move the joint gently within 1-2 days of treatment.
- Ice: applying ice helps to reduce swelling, prevent further bleeding and eases pain. Do not apply place ice directly on the skin as it can burn. Instead use a gel cold pack or make a cold pack from a bag of frozen peas or crushed ice wrapped in a cloth. Apply to the affected area for around 10-15 minutes every 2 hours – do not apply for more than 20 minutes at a time.
- Compression: apply pressure and a bandage to the affected area, to reduce swelling and pain. Your physiotherapist can provide you with an elasticated bandage and will make sure it fits properly. A bandage that fits too tightly can cause more damage. Try not to allow wrinkles in the bandage and remove it at night.
- Elevation: raise the injured area above the level of your heart. This increases blood flow away from the injured area and reduces swelling and pain. When you elevate your leg, remove the compression stocking to allow for normal circulation. Elevate for about 20 minutes at a time.
You will also need treatment to manage pain, as swelling caused by bleeds, especially in the joints, can be extremely painful. The more swollen an injury becomes, the pain you will feel. You need to avoid aspirin and most non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen as they suppress platelet function and reduce blood clotting. Talk to your doctor or pharmacist before trying any pain medication to discuss possible side effects.
Physiotherapy following a bleed helps with recovery from internal bleeding that may have damaged your joints. Your physiotherapist will provide you with a programme of suitable exercises, to get your muscles and joints working again. Physiotherapy appointments are free.