It is World Hemophilia Day!

17th April was recognized as World Hemophilia Day by the World Federation of Hemophilia (WFH) in 1989. Hemophilia was first discovered in the 10th century, when physicians started to take a serious interest in people, especially males, who were bleeding to death after sustaining only minor injuries. At that time, it was called Abulcasis.

What is hemophilia?

Hemophilia is normally an inherited disorder. In hemophilia, the blood does not clot as it should. It happens because of a defect in one of the clotting factor genes on the X chromosome.

Females have XX sex chromosomes while males have XY sex chromosomes. Females may be carriers of hemophilia, but they are unlikely to have the disorder. For a girl to have hemophilia, she must have the abnormal gene on both of her X chromosomes, and this is very rare. Hemophilia tends to occur in males, since the gene can be passed from mother to son.

Sometimes, hemophilia is acquired because of a spontaneous genetic mutation.

As a result of this condition, Proteins called clotting factors that work with platelets to stop bleeding at the site of an injury are less likely to be produced. People with Hemophilia produce lower amounts of either Factor VIII or Factor IX than those without the condition.

Types of hemophilia

There are two major types of hemophilia, type A and type B.

In hemophilia A, there is a lack of clotting factor VIII. This accounts for about 80 percent of hemophilia cases. In hemophilia B, also known as “Christmas disease,” the person lacks clotting factor IX. Both A and B can be mild, moderate, or severe, depending on the amount of clotting factor that is in the blood.

Symptoms

The severity of symptoms depends on how low the level of clotting factors is in the blood.

• Bleeding can occur externally or internally. Any wound, cut, bite, or dental injury can lead to excessive external bleeding.

• Spontaneous nosebleeds are common.

• There may be prolonged or continued bleeding after bleeding previously ceased. Signs of excessive internal bleeding include blood in the urine or stools, and large, deep bruises.

• Bleeding can also happen within joints, like knees and elbows, causing them to become swollen, hot to the touch, and painful to move.

• A person with hemophilia may experience internal bleeding in the brain.

What Are the Treatments for Hemophilia?

There is no way to cure hemophilia, but there are ways to reduce the risk of excessive bleeding and to protect joints, include: Receiving clotting factors through replacement therapy.

Medications, such as:

• Rixubis: is a man-made protein that helps blood to clot

• Desmopressin: is a man-made hormone that helps the body to release more clotting factors

• avoiding certain medications, such as aspirin, nonsteroidal anti-inflammatory drugs, and heparin.

The treatments you need will depend on the type of hemophilia and the severity of hemophilia

References

1

2