Hemophilia B is a rare genetic bleeding disorder that affects individuals who have low blood protein levels called factor IX. Factor IX is a clotting factor.
Clotting factors are special proteins needed for blood clotting to stop bleeding. Individuals with hemophilia B will bleed easily. This is due to the loss of protein involved in blood clotting and cannot effectively stop the flow of blood in the injured area. This is sometimes referred to as prolonged bleeding.
Hemophilia B is classified as mild, moderate or severe. In mild cases, symptoms of bleeding can occur only after surgery, major injury or tooth extraction procedures. In some moderate and most severe cases, symptoms of bleeding can occur after a small injury or sudden spontaneous bleeding without any triggering.
Causes of Hemophilia B
Hemophilia B is caused by a disorder or change in the factor IX gene. The factor IX gene is located on one of the two sex chromosomes, namely on the X chromosome. Men have one X chromosome and one Y chromosome and thus one altered copy of the factor IX gene in men is sufficient to expose him to hemophilia.
Women have two X chromosomes and must have two copies of the changes in the factor IX gene to have hemophilia. Hemophilia in women is very rare and that’s why disorders almost always affect men.
Hemophilia B is the most common type of hemophilia and is estimated to occur in 1 in 25,000 male births and affects all races. Hemophilia B is also known as factor IX deficiency or Christmas disease. This disorder was first reported in the medical literature in 1952 in a patient by the name of Stephen Christmas.
The most famous family with hemophilia B is Queen Victoria from England. Through their offspring, the disorder is inherited from the German, Spanish and Russian royal families and because of that hemophilia B is also known as ‘royal disease’.
To note, an individual is not born with the condition of hemophilia B, but in its development he eventually exposed to hemophilia B due to the production of body antibodies against factor IX protein itself. Factor IX antibodies destroy factor IX in the blood which causes bleeding symptoms called hemophilia B acquired. But this is very rare because most cases of acquired hemophilia are hemophilia A.
Symptoms of Hemophilia B
If someone has hemophilia, the blood does not clot as they should. If they are injured, contemplated, or otherwise injured, their bodies will bleed longer than anyone else. This can occur both inside and outside his body.
This is something serious. With proper treatment, and while avoiding certain risks, a hemophilia can have an active life. People with hemophilia do not have enough protein to help blood clotting.
A person with hemophilia can also:
- Nosebleeds for no apparent reason
- Lots of blood loss from small wounds
- Bleeding in the mouth from being bitten, or after tooth extraction
- Bleeding from wounds or injuries that cannot stop
- Blood in the urine or stool
- Big bruise
Bleeding can also occur in the body, such as in joints. If your child experiences muscle or joint bleeding when it hits, then the child will complain of pain especially when he moves it and usually the area of the muscle or joint is swollen and hot to the touch.
With this disease, even a small bump on the head can become serious. If that happens, watch out for signs of bleeding in the brain:
- Neck pain and stiffness
- Sudden weakness or running problems
Hemophilia B diagnosis
The diagnosis is usually made in early childhood. Only about 1 in 10 people have hemophilia, and often in boys than girls. Most people inherit it from mothers who carry defective genes. But it can also occur when a gene changes (mutates) before birth.
In the first 6 months of life, infants rarely fall or get injured, so early diagnosis is rarely done. You might start seeing problems after your baby becomes more active. When he starts crawling, the baby can bump and bruise. Bruises most often appear on the abdomen, chest, back and legs. Bruising and bleeding from small wounds can even be suspected as hemophilia.
In addition, your doctor will also ask about your family’s medical history to find out if there are problems with bleeding or blood clots. To get a diagnosis, the doctor will do a blood test to find out how long it will take for the blood to clot and to see each clotting factor
Hemophilia Treatment B
Usually the doctor will enter the clotting factor IX into the bloodstream through the syringe. In addition, doctors can also use protein replacement obtained from blood or made in the laboratory.
If your child has severe hemophilia, he may need regular treatment to prevent bleeding. If not, he may only need treatment to stop the bleeding if there is bleeding.
First aid is important when your child is injured. Clean small cuts, scratches, and blisters, and immediately press and bandage. Your doctor may recommend acetate products to treat mild bleeding. While for serious injuries, it requires more complete medical attention.
Meanwhile, some drugs that can be bought freely, such as ibuprofen, mefenamic acid (ponstan), paracetamol (panadol) can cause bleeding, so consult your doctor before giving treatment. To provide additional protection, also make sure your child wears knee pads, elbow pads, and helmets when playing.