Haemophilia is a rare genetic bleeding disorder that affects the ability of the blood to clot properly. It is more common in boys than girls and is usually inherited from the mother.
In haemophilia, the blood lacks certain clotting factors which can lead to prolonged bleeding and difficult-to-stop bleeding after an injury or surgery. This can be particularly dangerous in children, especially if the bleeding is internal, as it can cause damage to organs and tissues and even be life-threatening.
Causes of Haemophilia
Haemophilia is a genetic condition that is passed down through families. It is caused by a faulty gene on the X chromosome. Females have two X chromosomes and males have one X and one Y chromosome.
Females with one copy of the faulty gene are carriers of haemophilia and can pass it on to their children. Males with one copy of the faulty gene will have haemophilia.
There are two main types of haemophilia:.
Haemophilia A
Haemophilia A is caused by a deficiency in clotting factor VIII, the protein that helps the blood to clot. It is the more common type of haemophilia, affecting approximately 1 in 5,000 males.
Haemophilia B
Haemophilia B is caused by a deficiency in clotting factor IX. It is less common than haemophilia A, affecting approximately 1 in 20,000 males.
Signs and Symptoms of Haemophilia in Children
The severity of haemophilia can vary depending on the level of clotting factor that is present in the blood.
Some children with mild haemophilia may never have any symptoms, while others with severe haemophilia can have frequent and severe bleeding episodes.
The signs and symptoms of haemophilia in children can include:.
- Bruising easily
- Nosebleeds that are difficult to stop
- Excessive bleeding from cuts or injuries
- Pain and swelling in joints, particularly the knees, elbows and ankles
- Unexplained or excessive bleeding during or after surgery or dental procedures
- Blood in the urine or stools
- Headaches and vision problems
- Abdominal pain
Treatment for Haemophilia
There is currently no cure for haemophilia, but there are treatments available that can help to manage the condition. The main treatments for haemophilia are clotting factor replacement therapy and prophylaxis.
Clotting factor replacement therapy
Clotting factor replacement therapy involves giving the child the clotting factor that they are deficient in, either on a regular basis or as required. This is usually done through an injection into a vein and can be done at home or in hospital.
Prophylaxis
Prophylaxis involves giving the child clotting factor on a regular basis to prevent bleeding episodes. This is usually done every few days and can help to reduce the risk of joint damage and other complications associated with haemophilia.
Complications of Haemophilia
If left untreated or poorly managed, haemophilia can lead to a number of complications, including:.
- Joint damage and arthritis
- Internal bleeding, which can be life-threatening
- Spontaneous bleeding into muscles or organs
- Developmental delays due to prolonged bleeding in the brain
- Chronic pain
Lifestyle and Prevention
There are several lifestyle changes that can help to prevent bleeding episodes and manage haemophilia in children:.
- Avoiding contact sports and rough play
- Wearing protective gear such as helmets and knee pads
- Taking care when using sharp objects such as scissors and knives
- Avoiding medications that can increase the risk of bleeding such as aspirin and ibuprofen
It is also important to ensure that the child receives appropriate medical care and treatment as soon as possible if they do have a bleeding episode.
Conclusion
Haemophilia is a rare genetic bleeding disorder that affects the ability of the blood to clot properly. It is more common in boys than girls and is usually inherited from the mother.
The severity of haemophilia can vary, but it is important to manage the condition carefully to prevent complications such as joint damage, internal bleeding, and developmental delays. If your child has haemophilia, it is important to work closely with your healthcare team to ensure that they receive appropriate treatment and care.