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Hemophilia

Blood is carried throughout the body in blood vessels. When a vessel gets injured, blood clotting (also called coagulation) is an important part of the healing process. Cells and proteins in the blood work together to form a clot over the injury so that the vessel doesn’t bleed excessively.

When a child has hemophilia, his blood doesn’t clot normally. While he won’t necessarily bleed more than other children, he may bleed for longer. He may also have internal bleeding, where he bleeds inside of his body.

Hemophilia can range from mild to severe. In severe cases, the excessive bleeding can damage his tissues and organs, which can be life-threatening.  

About 400 babies are born with hemophilia each year, making it a very rare condition. It is more common in boys than girls.

 

Types

A clotting factor is a protein that’s needed for blood to clot normally, and a child with hemophilia doesn’t have enough of one particular factor. The specific factor that’s missing or deficient determines his type of hemophilia.

The three types are hemophilia A, hemophilia B, and hemophilia C.

  • Hemophilia A: This occurs when the body doesn’t make enough of factor VIII (eight). It is also called “classic hemophilia” because it’s the most common type—four times more common than hemophilia B.
  • Hemophilia B: This occurs when the body doesn’t make enough of factor IX (nine). It’s sometimes referred to as “Christmas disease” or the “royal disease”—a reference to the first patient diagnosed with hemophilia B (Stephen Christmas) and the most famous family with hemophilia B (Queen Victoria’s).  
  • Hemophilia C: This occurs when the body doesn’t make enough of factor XI (eleven).

 

Symptoms

Hemophilia can cause many symptoms, including:

  • Pain or swelling in affected joints
  • Constant nosebleeds
  • Bleeding from minor cuts (e.g., paper cuts) that doesn’t stop
  • Blood in the urine or stool
  • Excessive bleeding after dental work or getting a tooth pulled
  • Large or unexplained bruises

 

A girl who has started her menstrual cycle might also notice heavy periods or periods that last longer than seven days.

 

Causes

Most of the time, a child with hemophilia was born with the condition. It’s usually hereditary, meaning it’s passed on from the parents to the child. However, it’s possible for hemophilia to be sporadic—it occurs on its own and without any family history of the disorder.

It is possible for hemophilia to be acquired (developed later in life) but this is very rare. When it does happen, it’s usually due to a pregnancy, illness, or medication.   

Diagnosis

Hemophilia is often diagnosed soon after a baby is born. A physician may test for hemophilia right away if there is a family history of the condition, or if the child is showing immediate signs, like excessive bleeding after circumcision or blood draws.

Diagnosis usually includes screenings tests, where a child’s blood is tested to see if it’s clotting properly. He will also get a clotting factor test so the physician can understand which factor is deficient.

Treatment

Replacement therapy is the main treatment for hemophilia. The clotting factor that’s deficient, or missing, is injected into a child’s vein to help his blood clot normally. Replacement therapy can be done on a regular basis to prevent bleeding, or it can be used “on-demand,” to stop bleeding when it occurs.

Depending on the type of hemophilia, a physician may recommend other treatment options. For example, a mild case of hemophilia A can be treated with Desmopressin, a man-made hormone that’s given by an injection or a nasal spray.

Sometimes, children with hemophilia can start bleeding into their joints. This can happen after an injury (like a fall on the knee), but sometimes there is no obvious trigger. The blood eventually fills the joint cavity, putting pressure on the bones and sometimes causing damage.

If hemophilia has started to take a toll on a child’s joints, his physician may prescribe physical therapy, pain medication, or steroids.

Prevention

Currently, there is no known way to prevent hemophilia.

 

The OIC Hemophilia Team

OHTC pioneered the concept of providing a comprehensive, multidisciplinary team approach to the management of children and adults with bleeding disorders, and as a result the Center was designated an International Hemophilia Training Center in 1970 – one of the first four in the world — by the World Federation of Hemophilia.

Today the physicians at OHTC are highly trained specialists who are considered leaders in their field. Our physicians are at the forefront of their field and actively involved in hemophilia–based research and treatment, with several occupying leadership positions in national and international hemophilia organizations, as well as world-renowned speakers on the subject.