HealthInfo Canterbury
Haemophilia
Haemophilia (hee-mo-fill-ee-a) is a rare condition that affects your blood's ability to clot.
For most people, when you cut yourself, clotting factors in your blood combine with blood cells called platelets to make your blood sticky. This makes the bleeding stop.
If you have haemophilia, you are missing a clotting factor in your blood. This means your blood doesn’t clot properly and you bleed for longer than usual.
The two most common types of haemophilia are:
- Haemophilia A – this is the most common type and is caused by a factor VIII deficiency. Recent studies indicate that around 87% of haemophilia cases globally are haemophilia A.
- Haemophilia B – the second most common type is caused by a factor IX deficiency (sometimes known as Christmas Disease).
Haemophilia is an inherited condition, which means it is passed on from your parents. It is rare and mainly affects males: 1 in 10,000 males born in New Zealand has haemophilia. Female carriers may have mild symptoms and, very rarely, severe symptoms.
Symptoms of haemophilia
The symptoms of haemophilia can be mild to severe, depending on the level of clotting factor you have.
The main symptom is bleeding that doesn't stop (prolonged bleeding). For many people with haemophilia, the bleeding won’t stop without an injection of the right clotting factor medicine.
Bleeding can be external or internal:
- External – on the outside of your body, where it's visible. You may notice:
- nosebleeds that take a long time to stop
- bleeding from wounds that lasts a long time
- bleeding gums.
- Internal – on the inside of your body, where it's not seen and is harder to identify. You may notice:
- skin that bruises easily
- tenderness, swelling or stiffness around your joints, such as elbows
- an ache or funny feeling in a joint or muscle.
In babies, the first signs of haemophilia are usually heavy bleeding from the mouth from bites to their gums and tongue, or severe bruising.
Diagnosing and treating haemophilia
See your doctor if:
- you or your child bruises easily and has bleeding that doesn't stop
- you or your child have symptoms of joint bleeds, such as tingling, pain or stiffness in the joint, and the joint becoming hot, swollen and tender
- you have a family history of haemophilia and you're pregnant or planning to have a baby.
You will be asked to have a blood test, which can diagnose haemophilia and find out how severe it is.
If there's no family history of haemophilia, it's usually diagnosed when a child begins to walk or crawl. Mild haemophilia may only be discovered later, usually after an injury or a dental or surgical procedure.
There's no cure for haemophilia, but there is treatment that means you can enjoy a good quality of life. With proper treatment, life expectancy for people with haemophilia is almost the same as those without haemophilia. The aim of treatment is to reduce the risk of prolonged and excessive bleeding. Bleeding can be controlled by injections of a clotting factor medicine.
There are two main approaches to treatment, depending on the severity of your condition:
- preventative therapy – regular injections of clotting factor medicine are given to help prevent episodes of bleeding and prevent joint and muscle damage
- on-demand therapy – an injection of clotting factor medicine is given in response to a bleed. You will also follow the P.R.I.C.E (Protection, Rest, Ice, Compression, Elevation) regimen.
Pain relief and physiotherapy are also part of the treatment programme.
Complications with haemophilia
Repeated bleeding in and around your joints causes damage to them. This is similar to the damage and pain caused by arthritis, but with haemophilia you are likely to experience it at an earlier age.
A more serious complication for some people with haemophilia is when you develop antibodies, called inhibitors, to the clotting factor medicines. This makes them ineffective. It means bleeding can’t be controlled quickly and there is a risk of permanent damage from bleeding into your joints or death from serious internal bleeding.
In the past, there has also been the risk of infection from blood-borne viruses. However, in New Zealand, blood-donor screening and the use of recombinant factor products have removed nearly all risk of getting viruses through blood products.
Self-care with haemophilia
Good self-care includes:
- avoiding contact sports, such as rugby
- being careful taking other medicines – some can affect your blood's ability to clot, such as aspirin and ibuprofen
- maintaining good oral hygiene and having regular trips to the dentist so you do not develop gum disease (which can cause bleeding).
HealthInfo recommends the following pages
- Haemophilia Foundation of New Zealand
Information and support for people with von Willebrand disease, haemophilia and other bleeding disorders.
On the next page: Treating haemophilia
Content shared between HealthInfo Canterbury, KidsHealth and Health Navigator NZ as part of a National Health Content Hub collaborative. Endorsed by clinical director, Haematology Department, Canterbury DHB. Page created May 2020.
Review key: HIBLD-52881
