In addition to all the age-related health risks described above, many people with haemophilia have the added challenge of long-term damage to joints caused by repeated bleeds, especially before the introduction of effective prophylaxis with factor concentrates and non-factor replacement therapy.
Bleeding into a joint causes the lining of the joint (synovium) to be inflamed. Swollen strands of the synovium may extend into the joint between the bones where they can be pinched, resulting in further bleeding. Blood within the joint gradually destroys the smooth gliding cartilage surface of the bone resulting in pain and decreased range of motion.
The importance of prophylaxis
Use of prophylactic factor replacement has been very effective at decreasing joint bleeds and delaying the onset of chronic joint damage. Removal of the swollen synovium (synovectomy) can decrease recurrent bleeding into a target joint.
Prophylaxis with factor concentrates or non-factor replacement therapy has been shown, if started early enough, to reduce the long-term joint damage caused by repeated effects of haemarthroses, called haemophilic arthropathy. However, many adults with severe haemophilia did not have the benefits of prophylaxis as children and therefore have significant joint disease associated with repeated joint bleeds such as deformity, loss of flexion and extension, muscle weakness, and impaired balance. These can cause difficulty with mobility and result in pain on a regular basis. Also, the combination of joint disease and advancing age can leave people with haemophilia at an increased risk of falls and social isolation due to reduced mobility. To keep joints in the best possible shape as we get older requires a coordinated approach.
Prophylaxis may be needed in older people with haemophilia even when they thought it was unnecessary in their younger years. Prophylaxis can increase the quality of life dramatically. There is some evidence that bleeds can increase after the age of 50 and prophylaxis will reduce the likelihood of this happening. One study in Italy of 54 adults with a mean age of 30 (admittedly not very old) showed that the number of bleeds per annum decreased on average from 33 to 4 bleeds per annum, when one went from on-demand to prophylactic therapy and the days lost at work or college decreased from 35 to 4 per year. In other countries such as Denmark and the Netherlands, where young men have stopped prophylaxis at an average age of around 20-21 years, they have not seen a dramatic impact in relation to their bleeding patterns, at least in the short term, but this may be because those who have chosen to stop prophylaxis generally have a milder bleeding pattern.
Joint bleeding accounts for more than 90% of all serious bleeding events in persons with severe haemophilia. This risk of bleeding persists throughout life, and the benefits of prophylaxis in some form will be experienced by all age groups. The use of prophylaxis in adulthood has increased in recent years. It may be recommended to continue, restart, or even start prophylaxis on an ongoing basis or a short-term basis for a number of reasons. This will help to:
- Prevent bleeds.
- Preserve joint function or slow down the progression of joint damage.
- Relieve pain associated with bleeding and/or synovitis.
- Allow for gentle exercise and reconditioning.