We now have the first generation of people with haemophilia who are actually going to live long enough to experience diseases normally associated with ageing and the risks related to this will become clearer. Life expectancy figures for people with haemophilia from various studies vary from a median of 63 to 75 years of age and many people with haemophilia can expect to live beyond their 70th or 80th birthday. This is very reassuring and a testament to the improvement in comprehensive care and availability of factor replacement therapy for people with haemophilia over the past decades.
The risk for heart disease includes increased blood pressure, obesity, smoking and age. In some post-mortem studies, it was seen that people with haemophilia have the same risk as the normal population. Therefore, it is quite clear that having haemophilia does not give you a free pass on trying to ensure that you maintain normal blood pressure, avoid obesity and not smoke. There are, of course, further difficulties if people with haemophilia do develop heart disease which would normally necessitate treatment with Warfarin or anti-coagulant therapy.
In relation to cancer, the main risks for men are cancer of the prostate, colorectal cancer, lung cancer or bladder cancer. Studies from the Netherlands and Germany showed an increased cancer risk in people with haemophilia, whereas a study in the UK showed no increased risk and no increase in risk was also seen in other studies from the USA, Canada and Italy. The screening test for bowel cancer is to test for occult blood. In relation to renal disease, a UK study found an increased risk of renal or kidney disease in people with haemophilia – almost double the normal risk. This risk was associated with HIV, inhibitors or an increased blood pressure.
In addition to haemophilic arthropathy, increasing age increases the risk of osteoarthritis and rheumatoid arthritis. This would mean a requirement for increased physiotherapy and more work on balance and coordination. There is also a concern that with increasing age, the risk of inhibitors may increase. Haemophilic arthropathy is also a problem in the older population of people with haemophilia who did not have access to prophylaxis or even regular on-demand therapy when they were growing up. Most older people with haemophilia have multiple joint damage and often severe pain.
There may often be a need for prophylaxis in older people with haemophilia and prophylaxis can increase the quality of life dramatically. One study in Italy of 54 adults with 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 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 pattern but generally, this is because those who have self-selected to stop prophylaxis generally have a milder bleeding pattern. In relation to the additional risks from osteoarthritis and rheumatoid arthritis, it is worth noting that bone mineral density can be reduced in people with haemophilia. In a German study of 62 people with haemophilia, only one-third had normal bone density and a US study in Arizona of 30 people with haemophilia only one third had normal bone density.
We are very fortunate that for the first time we have a population of people with haemophilia in developed countries such as Ireland, who are going to have to confront the challenges of ageing. The normal challenges of ageing for men include the risk of heart disease, cancer, kidney disease and osteoarthritis. Some of these risks can be increased or exacerbated in people with haemophilia by their haemophilia and by their co-morbidities including existing arthritis, HIV and/or Hepatitis C. It is a well-recognised phenomenon that men generally do not like going to doctors and do not engage at a sufficient level with doctors in relation to preventative health. This is probably not the case in people with haemophilia who are used to visiting their haemophilia treatment centres and who have in the vast majority of cases have developed long standing relationships with healthcare professionals whom they trust.
For this reason, it is very important that the existing comprehensive care haemophilia treatment centres look at integrating into their comprehensive care services for haemophilia the screening and testing required to prevent and manage the diseases of ageing in the haemophilia population. In reality, this may well mean that there would be a requirement for Haemophilia Treatment Centres to integrate into their normal screening for people with haemophilia tests for cardiac disease, cancer, renal disease and arthritis in addition to the routine tests for factor levels, inhibitors and other specific tests relating to haemophilia. Centres may need to consider monitoring blood pressure, lipid profile including LDL and HDL cholesterol, blood glucose (for Diabetes), testing for protein in the urine (renal disease), prostate specific antigen (for prostate cancer), faecal occult blood (for bowel cancer) and dual energy X-ray absorptiometry to check bone mineral density.
A cardiologist may need to be added to the comprehensive care team as people with severe haemophilia over a certain age should have access to a comprehensive cardiovascular risk assessment. Additional complications of haemophilia on treatment and management of cardiovascular and other diseases of ageing are substantial and should be avoided where possible. It would be an excellent use of resources to prevent the development of these diseases if at all possible by screening, testing and by giving continuous lifestyle advice including management of weight, body mass index and cessation of smoking.
We are committed to taking a strong role on behalf of people with haemophilia, to ensure that the most effective pathways for monitoring, diagnosis and treatment for conditions associated with ageing are put in place. While no one wants to dwell on the degenerative effects of ageing and the problems they may bring, being prepared can go a long way towards alleviating these challenges.
Please click on this link to see the I.H.S. publication Ageing & Haemophilia. This booklet contains a broad overview of medical issues associated with ageing and haemophilia.