Continuing to Live with Haemophilia

If you have haemophilia, there is absolutely no reason why you cannot lead a healthy normal life. At an older age though, there are still things you should consider, just because you’re at an adult age doesn’t mean your condition should go untreated.

Continuing to Live with Haemophilia

I lead a busy lifestyle. How important is it that I keep to my prophylactic treatment routine?

When you embark on prophylactic factor VIII treatment for your haemophilia, it is important that you continue to treat yourself as prescribed. By sticking to your treatment routine, you can ensure that you get the most out of your prophylactic treatment. In severe haemophilia, the greatest advantage of receiving prophylaxis is that you are much less likely to have long-term joint damage than if you receive on-demand therapy only.1 You are also less likely to experience bleeds, and therefore less likely to end up in hospital with severe bleeds. It also means that you can take part in sports with less likelihood of experiencing a bleed and are protected from the everyday traumas that happen in life.

For information on comprehensive care centres and the Haemophilia society you may wish to visit the support section.

It is important that you discuss things with your doctor and haemophilia specialist

What can I do to keep my Haemophilia under control?

Sport has many benefits for everybody, but particularly for people with haemophilia. Haemophilia bleeds can affect your joints and muscles. By getting fit and keeping your joints and muscles strong, you can lessen the risk of bleeds and reduce the need for clotting factor replacement. It could also reduce the risk of arthritis or the need for surgery when you get older.

Click here to find out which sports we recommend for you

Joint Problems & Haemophilia

There are three types of joints in the body:

Fibrous joints

Connect the bones of the skull. In these joints, the bones are joined together tightly by fibres of tissues, so they hardly move at all. As a result, bleeding is extremely rare in these joints.

Cartilaginous joints

Are found, for example, between individual bones in the spine. The bones can move and are prevented from rubbing together by a piece of cartilage between them (the discs in the back).

Synovial joints

Include the shoulders, hips, elbows, knees, wrists, ankles, hands and feet. In synovial joints, the two surfaces of bone that move are covered with cartilages, so they slide smoothly over one another. The whole of the joint is enclosed in a tough capsule, like a bag, which is made of ligaments. The inside of the capsule is covered in a layer of cells called the synovial membrane, which produces a fluid called synovial fluid. The synovial fluid fills the joint space, cushioning the ends of the bones and allowing them to slide more easily – it ‘lubricates’ the joint.

Bleeding into joints and muscles

Excluding inhibitors, bleedings into joints and muscles are by far the most important problems for people with severe haemophilia and will account for the majority of their bleeding problems.

Bleeding is very unusual in young babies. Characteristically children with severe haemophilia have their first bleed when they first start to crawl or walk from around nine months onwards. Before that, they tend to bruise easily and the bruises are often ‘lumpy’.

The joints affected in haemophilia are the synovial joints, and most commonly the knees, elbows and ankles.

Recognising a joint bleed

Recognising a bleed becomes easier with experience, and people with haemophilia and their relatives come to recognise the tell-tale symptoms and signs when there is a problem Patients can recognise early tingling or bubbling sensation as an early sign. It is advisable that patients or their relatives ring their haemophilia centre if they suspect a bleed. The pattern of bleeding in haemophilia is very unpredictable and irregular. A person with haemophilia may go for several weeks – or even months – without any particular problem only to then experience three or four bleeds within a short space of time. No one really knows why spontaneous bleeds occur at a particular time.

There are some tell-tale signs which characterise joint bleeds:

  • You may find it painful to use the affected joint
  • The joint is hot
  • The affected joint is usually swollen
  • Tingling or bubbling sensation

What happens in a joint bleed?

The synovial membranes have many blood vessels within them. If one of these is damaged, blood leaks into the joint space. If the blood vessel is not repaired (by blood clotting) blood will carry on flowing into the joint until the pressure of liquid within the joint space is the same as the pressure of the blood vessel. By this time, the joint space and capsule have become swollen and the joint will have become extremely painful. Once blood is in the joint, some of the cells in the blood break down and cells from the synovial membrane will begin to clear up the debris. This has two effects. Firstly, the chemicals that are normally contained within the blood cells are released into the joint space and cause intense inflammation in the joint. Secondly, the remnants of blood cells can erode the smooth surface of the bones and cartilage making them rough and abnormal.

If the signs of a joint bleed are picked up at an early stage and the bleed is treated with factor VIII or IX, the bleeding will stop and only a small amount of blood will leak into the joint. After a larger bleed, however, or if a smaller bleed is left untreated, it can take up to several weeks for all the blood to be absorbed, thereby increasing the likelihood of joint damage. Once bleeding has occurred into a joint, there is an increased chance of another bleed occurring in the same joint and it is all too easy for a vicious circle to develop, in which bleeding causes joint damage and the damaged joint is then more likely to bleed again. These joints that bleed frequently are called ‘target joints’, and need special attention.

What can I do to prevent joint damage?

Sport or exercise has many benefits for everybody, but particularly for people with haemophilia. Because bleeds affect joints and muscles, keeping fit keeps joints strong and muscles flexible, which can lessen the risk of bleeds.2 Exercise and fitness can also reduce the risk of arthritis or the need for surgery in the future.

A physiotherapist will regularly assess the joints and muscles of a person with haemophilia, and can suggest a range of exercises to protect and strengthen joints and muscles.

Click here to find out which sports we recommend for you

Prophylaxis and sports
Click here to find out more

What can I do to manage joint damage?

Pain Management

Joint damage can cause long-term pain and the loss of mobility, which needs to be managed in order to maintain independence. Depending on the severity of pain, and joint damage, this can either be managed by painkillers and/or pain control, as described below, or through the use of surgery.

In any case, your haemophilia healthcare professionals should be able to offer you some advice on the next steps that you need to take. Always consult your Haemophilia centre before taking any medication.

Medication

When you're buying any type of medicine in a pharmacy or supermarket, make sure you always read the information leaflet in the packet. Always consult your Haemophilia Centre before taking analgesic or anti-inflammatory drugs such as aspirin or ibuprofen, as they can affect blood clotting, and make bleeding worse.

Before we review the types of pain medicines (analgesics) available to you, we'll summarise the do's and don’ts of pain control.

DO

  • Treat the underlying cause effectively and quickly
  • Tell healthcare professionals if your medication seems to stop working, or you experience any side effects
  • Keep medicine in a safe place where others can't reach it
 

DON’T

  • Offer your medication to anyone else
  • Drink alcohol without first checking with your pharmacist or doctor if it is safe

Paracetamol

Tablets and capsules

If you are looking for mild pain relief try paracetamol. Paracetamol doesn't affect bleeding and is relatively free of side effects. You should never exceed the recommended dose. Do not take paracetamol with large quantities of alcohol, and/or if you have any conditions affecting your liver. In this case, it's recommended that you speak to a healthcare professional before using this analgesic.

Also, it's worth noting here that many remedies for colds, congested sinuses and upset stomachs also contain either paracetamol or aspirin, so check the packaging of these products to make sure that you don't take those containing aspirin or ibuprofen, or that you combine medications which contain these.

Like all drugs, paracetamol must be stored out of reach of children, and the recommended dose must not be exceeded. If in any doubt, seek advice from your local Haemophilia Centre.

References

  1. Manco-Johnson MJ et al. N Engl J Med 2007;357:535-544.
  2. Khair K et al. Haemophilia 2012;18(6):898-905.
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