What is haemophilia?

Haemophilia affects the body’s ability to clot blood and stop bleeding. In a person without haemophilia, clotting factors that circulate in the blood accumulate at the site of a cut or injury, and form a blood clot. In people with haemophilia, there isn’t enough clotting factor to form a blood clot and stop the bleed.

There are several types of clotting factor: people with haemophilia A have less factor VIII and people with haemophilia B have less factor IX than people who don’t have the condition. The less factor you have, the more severe the haemophilia is.

Proportion of factor in blood compared
to normal levels
Severity of
haemophilia
5-50% Mild
1-5% Moderate
Less than 1% Severe

It’s also important to remember that not all bleeds are visible. The most serious problems in people with haemophilia come from bleeding in joints and muscles.

However, haemophilia is a manageable condition, with the majority of sufferers living a relatively ‘normal’ life. There are plenty of sources of support as well – for sufferers and caregivers alike. It’s important to remember that you’re not alone and there is a team of haemophilia specialists to help you along the way.

If you’re looking for general information on haemophilia, visit
The Haemophilia Society’s website.

For more information on living with haemophilia, go to the
FactorFitness site.

How did I get haemophilia?

Haemophilia is usually an inherited condition – that means it’s due to an irregular gene being passed down from your parents. In about a third of people with haemophilia an irregular gene may arise spontaneously.

To find out more, head to the Genetics of Haemophilia section of this site.

Living with haemophilia

Because haemophilia is a lifelong condition, continued supervision by a specialist medical team is highly important. People with moderate/severe haemophilia must be registered with a Comprehensive Care Centre where they are regularly reviewed by the multi‑disciplinary health team (a team made up of different healthcare professionals).

There are two ways to register with a Comprehensive Care Centre: you can contact your nearest centre and make an appointment, or ask your GP to organise a referral.

Managing haemophilia

There are two main ways of taking your treatment: on‑demand and as prophylaxis.

On‑demand treatment is when the missing factor is given once the bleed has started. However, this relies on the person knowing they have a bleed, and treating it quickly – although this can be difficult if the bleed is internal. Once a bleed has occurred in a joint, the joint is more likely to experience further bleeds while recovering. It may become a ‘target joint’ and experience repeated bleeds over time, ultimately leading to long‑term joint damage.

Prophylaxis aims to prevent bleeds from happening. Patients receive infusions every other day or three times a week to maintain a certain level of factor VIII in the blood should a bleed occur. Research suggests that prophylaxis is not only very effective at preventing bleeds, but it can also help to protect joints from damage caused by bleeds that go unnoticed.

Based on the information available, the UK Haemophilia Centre Doctors Organisation (UKHCDO) recommends that people with severe haemophilia receive prophylaxis. However, your haemophilia centre will be able to suggest the best option for you.1

Taking care with haemophilia

It’s important not to underestimate the effect a healthy lifestyle and a positive attitude can have. Simply staying fit helps reduce pressure on joints whilst sport and regular exercise can increase joint mobility.

Sport & exercise

Exercise increases joint mobility and strengthens muscles – as well as being fun! Improving endurance, and maintaining a healthy body weight while relieving tension and aggression are all positives for anyone. People with haemophilia can take part in most activities – although they should generally avoid contact sports that carry a high risk of head injury.

To find out more about enjoying sports with haemophilia, visit
FactorFitness.

Diet

A balanced diet will maintain strong teeth and a healthy body. Excessive eating and increased weight will put more pressure on the joints. It’s best to stay fit and keep the pressure to a minimum.

To find out more about a healthy diet with haemophilia, visit FactorFitness.

Dental care

Haemophilia doesn’t affect teeth directly, although gums may bleed more than usual if damaged or unhealthy. It’s therefore important to maintain good dental and gum hygiene to prevent them from becoming inflamed and/or infected. Regularly brushing your teeth will also reduce the number of fillings and extractions required.

You should tell your dentist that you have haemophilia so they can take the necessary precautions.

To find out more about keeping your teeth healthy, visit FactorFitness.

Reference:

  1. Richards M et al. Br J Haematol 2010;149(4):498-507. To download full reference, click here.