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Haemophilia in children

Published by Bupa's health information team, March 2008.

This factsheet is for parents of children who have haemophilia, or for people who would like information about it.

Haemophilia is an inherited bleeding disorder in which the blood doesn't clot properly. It's a life-long condition and although it can't be cured, it can usually be effectively controlled so that there is minimum disruption to your child's everyday life.

About haemophilia
Symptoms
How do you get haemophilia?
Diagnosis
Treatment
Complications
Special considerations
Living with haemophilia
Further information
Questions and answers
Related topics
Sources

About haemophilia

Around 6,000 people in the UK have haemophilia, mainly men and boys.

Haemophilia is an inherited disorder of the blood-clotting system. If your child has haemophilia, he has lower than normal levels of a clotting factor in his blood. Clotting factors make your blood turn from a liquid to a sticky solid (a blood clot) and stop or limit the bleeding from damaged blood vessels.

If your child has haemophilia, he won't bleed to death from a minor cut - but bleeding may tend to restart after initially stopping and may last for longer than in a child without the condition. Bleeding into his joints and muscles (known as "bleeds") is characteristic, usually as a result of an injury, although in more severe types, bleeds can happen spontaneously - that is, without any obvious cause. He can also be at risk of bleeds if he undergoes surgery or has a tooth extraction without any precautions being taken.

Types of haemophilia

There are two main types of haemophilia.

Haemophilia A - also known as classical haemophilia - is the most common type and is caused by a lack of clotting factor VIII (factor 8).
Haemophilia B - also known as Christmas disease - is less common and is due to a lack of clotting factor IX (factor 9).

There are three levels of haemophilia, depending on how much clotting factor is in your child's blood.

Mild - he will generally only need treatment after a severe injury or during surgery.
Moderate - he is more likely to have bleeds after a minor injury and during surgery rather than have spontaneous bleeds.
Severe - he may have bleeds spontaneously, after an injury or during surgery.

Symptoms

The signs and symptoms of haemophilia A and B are the same and include:

an ache or tingling in your child's joints, most commonly in his knees, elbows or ankles - this is the first sign that your child is having a bleed
pain and swelling in one of your child's joints - you may notice that your child is reluctant to use or move an arm or leg
large and deep bruises
prolonged bleeding after your child has a cut, tooth extraction or surgery
blood in your child's urine or faeces

Although not necessarily a result of haemophilia, if your child has these symptoms you should take him to his GP and discuss your concerns.

Babies rarely have any symptoms before they start to crawl or walk, but a baby with severe haemophilia may develop bruises after being picked up or held. It may be diagnosed if he bleeds more than usual following an injury or surgery.

How do you get haemophilia?

Haemophilia is a genetic disorder. This means that the haemophilia gene is passed down from parent to child. There is usually a family history of haemophilia, but one in three people with haemophilia have no family history of the condition and it occurs because of a spontaneous change (mutation) in the genes.

The chromosomes that determine whether a person is a male or female are called the X and Y chromosomes. Males have one X and one Y chromosome, and females have two X chromosomes. Chromosomes are structures that carry genes which contain the instructions for life and are inherited from your parents. The genes for both haemophilia A and B are on the X chromosome.

Boys only have one X chromosome, so if your child inherits the haemophilia gene, he will have haemophilia. Girls have two X chromosomes, so if only one of these has the haemophilia gene, the other one will usually make up for it and she will not have haemophilia. Although extremely rare, girls can be affected if both the mother and father carry the haemophilia gene and both pass it on to their daughter.

Girls who have the haemophilia gene on one of their X chromosomes are called carriers. The daughter of a man with haemophilia will automatically be a carrier because she will inherit the X chromosome from her father. Being a carrier means she is very unlikely to have the condition herself but could pass the gene onto her children. If the X chromosome carrying the haemophilia gene gets passed on to her child, he will have haemophilia. Their sons will have a 50 percent chance of having haemophilia and their daughters will have a 50 percent chance of being carriers.

Occasionally female carriers do have low levels of clotting factor VIII or IX and have symptoms of the condition.

Illustration to show how haemophilia can be passed from parent to child
How haemophilia can be passed from parent to child

Diagnosis

The GP will ask about your child's symptoms, examine him and will take a blood sample.

Children with severe haemophilia are usually diagnosed within the first year, but if he has moderate or mild haemophilia he may not be diagnosed until adolescence or even adulthood.

Children who have a family history of the disorder may be tested and diagnosed before they have symptoms. Girls can be tested to see if they are carriers.

Treatment

There is no cure for haemophilia but with treatment it can usually be effectively controlled.

There are a number of haemophilia centres across the UK that provide specialist care and treatment (see Further Information).

A bleed can be treated by injecting a factor concentrate of the relevant clotting factor (VIII or IX) into a vein. Bleeding stops, or is prevented, when an adequate level of the factor in the blood is reached. It's important that your child is given treatment as quickly as possible both to reduce pain and prevent long-term damage.

Factor concentrate can either be a synthetic genetically engineered concentrate, known as recombinant factor or made from human blood plasma (the fluid part of blood).

Treatment for mild haemophilia

If your child has mild haemophilia, he will usually be given clotting factor concentrate only before surgery or after a severe injury. A minor cut may not bleed excessively and can usually be treated by applying pressure and covering the cut with a plaster or bandage.

If your child has a mild form of haemophilia A, he may be prescribed a medicine called desmopressin which stimulates the body to produce more factor VIII. A medicine called tranexamic acid may also be given which can help with blood clotting.

Treatment for moderate or severe haemophilia

If your child has moderate or severe haemophilia, he may be given:

regular injections of clotting factor to prevent bleeds (known as prophylactic treatment)
an injection of clotting factor concentrate after a bleed or before and after he has surgery (known as 'on demand' treatment); these may need to be repeated for a number of days
a medicine called tranexamic acid may also be given which can help with blood clotting

You can learn how to give your child his injections and he can learn how to inject himself when he is ready. Home treatment gives you and your child greater flexibility as you won't have to see a medical professional every time he needs treatment, and also allows bleeds to be treated more quickly.

Your child may have a small device, called a port-a-cath, placed under his skin that empties directly into one of his veins and makes giving injections easier.

Complications

Untreated bleeds can be very painful and can cause longer-term damage.

A joint that has been affected once may become prone to frequent bleeds. This is known as a "target joint". Repeated bleeding into your child's joints without treatment can cause damage and lead to chronic arthritis and even disability.

If your child has a bleed in his brain, throat, gut, bladder or kidney area he will required immediate hospital assessment and treatment.

The symptoms of:

a bleed in the brain include a headache, dizziness, vomiting, unusual sleepiness and dilated or unequal pupils
a neck and throat bleed include tenderness, pain or swelling in his neck
a bleed in the gut include coughing or vomiting blood, passing black faeces and pain in his abdomen
a kidney or bladder bleed include your child's urine being reddish-brown in colour

Your child's immune system, which helps his body fight infection, may develop antibodies which stop the replacement clotting factor from working. These antibodies are known as inhibitors. Bleeds can still be treated successfully in this situation, either by being given larger doses of clotting factor to make up for the amount destroyed or being given other special blood products.

Special considerations

Medicines

Your child shouldn't take any product that contains aspirin if he has haemophilia, as it stops another element of blood clotting and can add to problems with bleeding. Always check the patient information leaflet that comes with any medicine or dietary supplements and ask your doctor or pharmacist for advice.

Living with haemophilia

Treatments may be frightening for young children. As your child recognises when he is having a bleed he may not tell you or a carer because he doesn't want to have an injection. You, or your child's carer, must therefore keep a look out for signs of a bleed such as reluctance to use one of his limbs. As your child gets older he will realise that treatments make the bleeds stop and the pain go away.

When your child goes to school he may, at times, need special consideration. The school may provide an area where your child can have his treatment.

Some people with haemophilia don't exercise because they think it may cause bleeds, but exercise can actually help prevent bleeds. Having strong muscles helps protect him from spontaneous bleeds and joint damage. Your child can take part in many sports and activities, but you and your child should think carefully about which to choose. Non-contact sports such as swimming, cycling and walking are usually safe but contact sports such as rugby, football and boxing should generally be avoided, particularly if he has severe haemophilia.

It's important that your child carries details about his condition and treatment with him at all times in case of emergencies. Usually, he will be provided with either a card or bracelet with details of his condition by his local haemophilia centre (see Further information).

Further information

The Haemophilia Society
0800 018 6068
www.haemophilia.org.uk