Rafed English

How to Deal with Hemophilia

Hemophilia (say: hee-muh-fih-lee-uh) is a genetic disorder in which a person's blood does not clot properly.

A person who has hemophilia has a tendency to bleed excessively. Think of the last time you had a cut or scrape. When the bleeding stops on its own, you're seeing clotting in action.

A Bit About Blood

If your blood is clotting normally, your body naturally protects itself. Platelets, which are "sticky cells," go to where the bleeding occurs and cover the hole, plugging it up. This is the first step in the clotting process. When the platelets plug the hole, they release chemicals that attract more sticky platelets and also activate various clotting factors, which are proteins in the blood.

These proteins mix with the platelets to form fibers, which make the clot stronger and stop the bleeding. Our bodies have clotting factors that work together to make fibrinogen (say: fy-brih-nuh-jun), the substance that makes the fibers. Trouble begins if the body is missing one of these factors.

A person can have one of two types of hemophilia, A or B. It depends on which clotting factor they are low on. If someone produces 1% or less of the affected factor, the case of hemophilia is called severe. Someone who produces 2% to 5% has a moderate case, and someone who produces 6% to 50% of the affected factor level is considered to have a mild case of hemophilia.

Why Do Kids Get Hemophilia?

Hemophilia almost always affects boys. Why? Because the disease is an X-linked genetic disorder, passed from mother to son. Boys get an X chromosome (say: kro-muh-soam) from their mother and a Y chromosome from their father. If the mother carries the gene for hemophilia on one of her X chromosomes (girls have two X chromosomes), each of her sons will have a 50% chance of having hemophilia.

A mother who is a carrier also has a 50% chance of giving the faulty X chromosome to her daughter. That does not give the daughter the hemophilia disease, but it does result in the daughter becoming a hemophilia carrier. So it's possible one of her sons someday could have the disease.

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