Haemophilia - Symptoms of haemophilia

The symptoms of haemophilia vary depending on how severe the condition is, but the main sign is prolonged bleeding.

The severity of the condition is determined by the level of clotting factors in the blood:

• mild haemophilia – where someone has between 5 and 50% of the normal amount of clotting factors
• moderate haemophilia – where someone has between 1 and 5% of the normal amount of clotting factors
• severe haemophilia – where someone has less than 1% of the normal amount of clotting factors

Most cases of haemophilia are severe.

Mild haemophilia

Children born with mild haemophilia may not have any symptoms for many years. The condition usually only becomes apparent after a significant wound, surgery, or a dental procedure such as having a tooth removed. These events could cause unusually prolonged bleeding.

Moderate haemophilia

As well as the above, children born with moderate haemophilia bruise easily. They may also have symptoms of internal bleeding around their joints, particularly if they have a knock or a fall that affects their joints. This is known as a joint bleed.

The symptoms usually begin with a tingling feeling of irritation and mild pain in the affected joint, most commonly the ankle joints, knee joints and elbow joints. Less commonly, the shoulder and hip joints can also be affected.

If a joint bleed is not treated, it can lead to:

• more severe joint pain
• stiffness
• the site of the bleed becoming hot, swollen and tender

Severe haemophilia

The symptoms of severe haemophilia are similar to those of moderate haemophilia. However, joint bleeding is more frequent and severe.

Children with severe haemophilia have spontaneous bleeding. This means that they start bleeding for no apparent reason. Spontaneous bleeding can take the form of nosebleeds, bleeding gums, joint bleeds and muscle bleeding.

Without treatment, people with severe haemophilia can develop:

• joint deformity, which may require replacement surgery
• soft tissue bleeding, which could lead to further complications
• serious internal bleeding

When to seek emergency medical help

There is a small risk of bleeding inside the skull, known as an intracranial haemorrhage. It is estimated that 3% of people with moderate or severe haemophilia will have an intracranial haemorrhage. However, spontaneous bleeding inside the skull is uncommon and is usually only caused by a head injury.

Bleeding in the skull should be treated as a medical emergency.

The symptoms of an intracranial haemorrhage include:

• severe headache
• stiff neck
• vomiting
• a change in mental state, such as confusion
• speaking difficulties, such as slurred speech
• changes in vision, such as double vision
• loss of co-ordination and balance
• paralysis of some or all of the facial muscles

Call 999 for an ambulance if you think that you or your child has bleeding inside the skull.