How is haemophilia treatment managed?
Thanks to the introduction of preventative treatment, improvements in safety of plasma products and the development of clotting factor made in the lab, people with haemophilia are growing up healthier and with less lasting damage from bleeds than they used to.
That’s not to say that there aren’t some demands placed on people with haemophilia that others’ don’t have. You will always need to have treatment, whether that’s regular preventative treatment (prophylaxis, pronounced prof-ill-ax-iss) or treatment for bleeds.25
Everyone with haemophilia should have their care co-ordinated through a haemophilia centre.41 You may have all your treatment managed through a comprehensive care centre (CCC), but some people live too far away to go regularly and will have their day to day treatment through a haemophilia centre (HTC) at a nearer hospital. The treatment your HTC gives you should always be overseen by the haemophilia comprehensive care centre because that is the centre of expertise. They will have specialist nurses, physios and other health professionals, not only specialist doctors.42 People who have their haemophilia managed through a comprehensive care centre do better overall than those who don’t.42
Every now and then, you will have to travel to your comprehensive care centre. A couple of times a year, you will need to see the haemophilia specialist physiotherapist. They need to check your joints for damage from any bleeds and can give you an exercise programme to strengthen your muscles and joints and make bleeds less likely.26
As a person with haemophilia you should have contact with your centre at least once a year. If you have severe haemophilia this will be at least twice a year and for very young children, it will be 3-4 times a year.43
In between check-ups, you give your treatment yourself at home. Or, for children, usually their parents give it. Being able to give your own treatment at home does make life easier. But making sure you have the absolute minimum of long term damage from bleeds means that you should always have factor and infusion kits with you.
Giving themselves treatment becomes second nature to most people and they are able to inject themselves quickly and relatively easily, keeping to good hygiene standards so that they don’t pick up an infection. People also learn to spot symptoms of bleeds so that they can treat as early as possible and minimise damage. Very early signs can be difficult to spot – people talk about tingling or bubbling, stiffness and warmth.
You should have been given a Bleeding Disorder Information Card by your haemophilia comprehensive care centre. This has your diagnosis and the number of your haemophilia comprehensive care centre. You should have this on you all the time – just in case. Many people also wear a medical alert bracelet to tell people you have haemophilia.
Aside from having the treatment, how will haemophilia affect my daily life?
However well you manage it, haemophilia will affect your daily life to some extent. You’ll always have to put a little bit of extra planning or thought into what you do and where you go than other people need to. But with preventative treatment (prophylaxis) there is less of an impact than there used to be.
There are some implications for your general health if you have haemophilia. Because of the risk of bleeding, it’s important to take very good care of your teeth to prevent decay and gum disease.44 You need to see the dentist and hygienist regularly.44
There are some over-the-counter medicines you need to avoid, including aspirin and anti-inflammatories such as ibuprofen.45 If in any doubt at all (including about herbal medicines) it’s best to check with your haemophilia centre.45 You can have vaccinations as injections under the skin (subcutaneously), but shouldn’t have injections into a muscle because of the risk of bleeding.46,47
One area that’s changed radically for people with haemophilia over the years is advice about keeping fit and taking part in sports. Being active is now encouraged and the emphasis is on what you can do, rather than what you can’t.13 There are very few sports that you can’t take part in. Individual planning for prophylaxis means that your treatment schedule can be planned around your activity so that your factor levels are at their highest when you are most active.34
Travel also needs a bit of planning ahead. You’ll need to take enough factor with you and that also means having a letter from your treatment centre explaining why you need it. Finding out where the nearest haemophilia centre is to where you’re going is also a good idea.
Aside from needing to arrange storage and access to somewhere to self-treat, life at school, college or work should be pretty much the same as for anyone else. Anti-discrimination legislation means that employers have to make reasonable adjustments to accommodate you.48 There are still some jobs you may not be able to do, such as joining the armed forces, the police or the fire service or being an airline pilot.42 But those aside, you can pretty much choose anything that suits you. There will always be the issue of how open you want to be about your condition and, particularly for children, feeling a little different to everyone else. Remember that who you tell and how much will always be up to you.