Age Related health risks common to everybody
The risks for heart disease include increased blood pressure, being overweight, smoking and age. In some post-mortem studies, it was seen that people with haemophilia have the same risk of developing heart disease as the normal population. Therefore, it is quite clear that having haemophilia (and a lower tendency to clot) does not give you a free pass. Maintaining normal blood pressure, not being overweight and not smoking are just as critical in people with haemophilia. Staying fit and maintaining healthy body weight may be more challenging in those with joint damage that limits options for exercise. People with haemophilia face additional difficulties if they do develop heart disease as common anti-coagulant therapies to reduce the risk of stroke and heart attack may not be recommended as they may worsen bleeding tendencies.
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, nor was there an increase in risk in other studies from the USA, Canada and Italy. 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 increased blood pressure.
Haemophilic arthropathy (damaged joints) is a greater 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 chronic pain. In addition to haemophilic arthropathy, ageing increases the risk of osteoarthritis and rheumatoid arthritis. Increased physiotherapy and exercises for balance and coordination are required in such cases. It is also 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 in a US study in Arizona of 30 people with haemophilia only one third had normal bone density.
Inhibitors
There is some evidence that risk of inhibitors may increase with age, even in those with no history of inhibitors. Fortunately, excellent coagulation therapies (e.g. emicizumab) now exist even for people with inhibitors.
Comprehensive Care
Given the complexity of care for a person ageing with haemophilia, it is very important that the existing comprehensive care haemophilia treatment centres look at integrating into their services 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 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 possible by screening, testing and by giving continuous lifestyle advice including management of weight, body mass index and cessation of smoking.