Haemophilia
& Treatment
Prophylaxis
What is Prophylaxis and its advantages
What is Prophylaxis?
Regular treatment to prevent bleeds is called prophylaxis. Prophylactic treatment for someone with haemophilia (usually three times a week) helps the blood to clot and minimises the likelihood of long-term joint damage.
Prophylaxis is a very important development in the lives of people with haemophilia. If a person has severe haemophilia and experiences frequent bleeds into joints, long-term damage such as arthritis can occur and that joint can become what is known as a target joint. However, this does not mean that every person with haemophilia will get a prophylactic regimen, as this depends on a number of factors such as severity of haemophilia and frequency of bleeds.
In addition, if you have an inhibitor your treatment will need to be managed differently to someone that does not.
What are the Advantages?
Prophylaxis in haemophilia reduces the risk of ‘spontaneous' bleeds. Each time someone with haemophilia has a bleed into a joint there is the potential for damage to the cartilage. This can cause permanent damage to joints and may require surgery to repair it. In addition to the pain and restricted movement caused by a badly damaged target joint, if it is left untreated, it may put increased strain on other joints, causing problems in them as well.
As well as helping the joints, preventative injections can mean that your life is less disrupted by haemophilia, enabling you to study or work with less worry. For instance, it means that you can have a more normally active lifestyle, particularly if you build the treatment regimen around your sporting activities. This ensures that you have high enough factor levels to participate in a range of sports and activities that might have been off limits without the clotting factor boost a treatment can give. It may be recommended however, that excessive sporting activities are avoided and should be discussed with your Haemophilia Centre before participating in them. Prophylactic treatment will also mean fewer visits to a Haemophilia Centre for treatment.
Starting on Prophylaxis and sticking to a routine
When should you Start?
Ideally, for someone with haemophilia preventative treatment should start before a target joint occurs, preferably in early childhood. Exactly when you start prophylaxis will depend on your treatment centre, with some centres opting to initiate prophylaxis immediately after a baby's first major bleed and other centres choosing to monitor a bleed history to determine the best time to start. Current UK guidelines state that prophylaxis should be given if there are three joint bleeds in a year or two bleeds into the same joint within a year.
Prophylaxis aims to maintain factor levels at the same level as someone with moderate haemophilia and makes it much less likely that ‘spontaneous' muscle or joint bleeds will occur.
Different Routines
The decision to try prophylaxis may depend on the severity of a patient's haemophilia and their tendency to bleed. A patient with severe haemophilia may start as soon as possible; one with mild haemophilia may be better suited to on-demand treatment. Prophylaxis aims to reduce the risk of ‘spontaneous' joint or muscle bleeds and to enable patients to lead a more normal life.
Preventative treatment is a complex process, designed specifically for each individual, as the amount of factor and number of injections you require will change. Your Haemophilia Centre will monitor your response to prophylaxis and make adjustments to suit. Your doctor may also perform half-life studies (click here for more information) to assess how long the factor stays in your body. This may take time and require a number of visits to the Haemophilia Centre but once the treatment programme is set up it will make life a lot easier.
There are generally two different types of prophylactic regime that you can be put on by your haemophilia team. These are called primary prophylaxis and secondary prophylaxis.
In primary prophylactic regimes you start regular infusions of factor VIII treatment, either before any bleeds have occurred or after a single joint bleed.
In secondary prophylactic regimes you can either have continuous prophylaxis as in the case of the primary regimes but started after two joint bleeds, alternatively, regular infusions of treatment can be undertaken periodically for short periods, for example seasonally or before and after an operation.
Sticking with it
When medication has been prescribed, it is important to continue taking it regularly as this will reduce the risk of ‘spontaneous' bleeds. Prophylactic treatment enables patients to get on with their lives more easily.
For those with haemophilia, prophylaxis can prevent bleeds and joint damage, which can be painful and debilitating. By protecting joints the likelihood of requiring orthopaedic surgery later in life can also be reduced.
