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Healthy Living

What Is Hemophilia?

Hemophilia is a bleeding disorder characterized by a deficiency of selected proteins in your body's blood-clotting system. Clotting is the process by which your blood changes from a liquid to a solid state in order to stop bleeding. The clotting process involves blood particles called platelets and clot-forming proteins called clotting factors. Your blood has at least 10 clotting factors that are involved in the clotting process.

Hemophilia occurs in three types hemophilia A, hemophilia B and hemophilia C depending on which clotting factor is deficient. All three types can cause prolonged bleeding. If you have hemophilia and you have a cut, you'll bleed for a longer time than if your blood were clotting properly. Small cuts normally aren't much of a problem. The greater problem is deep internal bleeding and bleeding into joints.

Hemophilia A, B and C are inherited diseases. Because of the pattern of inheritance, hemophilia A and B occur almost always in boys. Hemophilia C can occur in both boys and girls.

About 17,000 Americans have hemophilia. Most cases are either hemophilia A or B, with hemophilia A being about seven times more common than hemophilia B. Hemophilia C is rare, with only about 200 known cases in the United States since it was identified in 1953.Hemophilia is a lifelong disease. But with medication, replacement of deficient clotting factors and proper self-care, most people with hemophilia can live an active, productive lifestyle.

Signs and Symptoms

Signs and symptoms may include:

  • Many large or deep bruises
  • Joint pain and swelling caused by internal bleeding
  • Bleeding within your muscles
  • Blood in your urine or stool
  • Prolonged bleeding from cuts or injuries, or after surgery or tooth extraction

At first, a baby with hemophilia usually won't have many problems related to hemophilia because of limited mobility. But as your baby begins to move around, falling and bumping into things, superficial bruises may occur. This bleeding into soft tissue may become more frequent the more active your child becomes. Most of the time, these bumps and bruises aren't serious and don't require medical treatment.

Emergency signs and symptoms of hemophilia may include:

  • Bleeding into your head, neck or digestive tract

  • Sudden pain, swelling, and warmth of large joints, such as knees, elbows, hips, and shoulders, and of the muscles of your arms and legs

  • Bleeding from an injury, especially if you have a severe form of hemophilia

Causes

Three categories of blood proteins play a role in blood clotting. Procoagulant proteins help form clots. Anticoagulant proteins prevent formation of clots. Fibrinolytic proteins help dissolve clots that have formed.

The clotting process involves blood particles called platelets and procoagulant plasma proteins called clotting factors. The process begins when platelets stick to a blood vessel at the site of an injury. An intricate cascade of enzyme reactions occurs to produce a weblike protein network that encircles the platelets and holds them in place (platelet phase) to form the clot (coagulation phase). In this cascade, each clotting factor is transformed, in turn, from an inactive to an active form.

The cause of hemophilia is a deficiency of one of several of your blood's clotting factors. There are three types, all inherited disorders:

  • Hemophilia A, the most common type, is caused by lack of enough clotting factor VIII.

  • Hemophilia B is caused by lack of enough clotting factor IX.

  • Hemophilia C, extremely rare in the United States, is caused by lack of clotting factor XI.

Hemophilia A and B occur almost always in boys. Generally, hemophilia A and B are passed from mother to son through one of the mother's genes. Men cannot pass along the gene that causes hemophilia to their sons. Everyone has two sex chromosomes, one from each parent. Females inherit an X chromosome from their mother and an X chromosome from their father. Males inherit an X chromosome from their mother and a Y chromosome from their father. The gene that causes hemophilia A or B is located on the X chromosome. Most women who have the defective gene that causes hemophilia are simply carriers and exhibit no signs or symptoms of hemophilia. It's also possible for hemophilia A or B to occur through spontaneous mutation of a gene.

Hemophilia C can occur in both boys and girls. The defective gene that causes hemophilia C can be passed on to children by mothers and fathers in a different inheritance pattern than occurs with hemophilia A and B.

When to Seek Medical Advice

If you're pregnant or considering a pregnancy and have a family history of hemophilia, talk to your doctor. It's possible to test your child during pregnancy to see whether he or she has inherited hemophilia. If your baby boy hasn't been circumcised and bruises easily as he becomes more mobile, see your doctor.

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