Hemophilia

Hemophilia is a hereditary, lifelong condition in which the blood doesn’t clot properly. People with hemophilia are deficient in a blood protein called clotting factor. The condition is found in all races and socioeconomic classes.

Normally when you bleed, your body responds by clotting the blood so that the bleeding slows down and eventually stops. A deficiency in clotting factor means it’ll take longer for the blood to clot.

There are at least 13 different clotting factors in the blood that work together when an injury occurs. Clotting proteins are usually designated with Roman numerals.

Hemophilia usually occurs in males. It’s passed along by females who carry the hemophilia gene. Though rare, females may also have hemophilia, but it’s more common for carriers to have low factor levels that are associated with bleeding problems.

Hemophilia is diagnosed by blood tests that measure the level of clotting factor. Factor levels generally remain unchanged throughout a person’s lifetime. Testing for hemophilia may be indicated based on family history or symptoms while bleeding. At present, there’s no cure, though research into gene therapy shows promise. In about a third of all cases, there’s no family history of it, meaning hemophilia may occur as a new mutation of the gene.

There are two types of hemophilia, depending on which clotting factor is missing. People who are missing or deficient in factor VIII haveType A hemophilia.This is the most common form, affecting approximately 12,000 people in the United States. Hemophilia A is also called factor VIII deficiency or classical hemophilia.

Those who are missing or deficient in factor IX haveType B hemophilia,which affects about 4,000 in the United States. It’s also referred to as factor IX deficiency or Christmas disease.

Though lacking a cure, hemophilia can be treated. The missing factor can be replaced by clotting factor concentrates. These elevate the factor levels enough to allow the body to form a clot and stop the bleeding.

Once factor is in the bloodstream, the clotting potential remains at normal values for 8 to 12 hours for factor VIII and 18 to 24 hours for factor IX.

Clotting factor concentrate comes as a freeze-dried powder that’s mixed with sterile water prior to use. It’s infused into the body through a vein or central venous access device.

There are two types of clotting factor concentrates, depending on the source.Plasma-derived factoris made from donated human blood plasma. The clotting proteins are separated from other blood components, then purified and made into a freeze-dried product. All plasma donated for concentrates is tested for viruses.

Recombinant factoris made in a laboratory and uses little or no blood plasma. Since human blood proteins are kept to a minimum, there’s little risk of contracting viruses. Recombinant factor VIII products have been available since 1992 and recombinant factor IX products since 1997.

Several regimens are used to manage hemophilia, as directed by the doctor.Episodic treatment(also called on-demand treatment or PRN) is given only in response to a bleeding episode.Prophylactic treatmentis the practice of giving factor on a regular basis, usually several times a week. The goal of prophylaxis is to raise factor levels enough to prevent bleeds.Planned preventive treatmentmeans to treat someone with factor before an event or activity that may cause bleeding, such as a medical operation.

People who have severe hemophilia often have bleeds in joints and muscles. In such instances, there’s no visible blood. Some bleeds occur for no reason and are termed spontaneous. Other bleeds are caused by injury or trauma.

Symptoms of a joint bleed include pain, tenderness, heat, swelling, limited motion, limping, or difficulty in using the affected joint. The symptoms may develop gradually as the injured joint slowly fills with blood.

Common symptoms of a muscle bleed include pain to the touch, a hard lump in the muscle, bruising, and tightness.

Some bleeds are inherently serious, including those in the head, throat, spine, and internal organs. Such bleeds require immediate attention.Hemophilia is a hereditary, lifelong condition in which the blood doesn’t clot properly. People with hemophilia are deficient in a blood protein called clotting factor. The condition is found in all races and socioeconomic classes.


Normally when you bleed, your body responds by clotting the blood so that the bleeding slows down and eventually stops. A deficiency in clotting factor means it’ll take longer for the blood to clot.


There are at least 13 different clotting factors in the blood that work together when an injury occurs. Clotting proteins are usually designated with Roman numerals.


Hemophilia usually occurs in males. It’s passed along by females who carry the hemophilia gene. Though rare, females may also have hemophilia, but it’s more common for carriers to have low factor levels that are associated with bleeding problems.


Hemophilia is diagnosed by blood tests that measure the level of clotting factor. Factor levels generally remain unchanged throughout a person’s lifetime. Testing for hemophilia may be indicated based on family history or symptoms while bleeding. At present, there’s no cure, though research into gene therapy shows promise. In about a third of all cases, there’s no family history of it, meaning hemophilia may occur as a new mutation of the gene.


There are two types of hemophilia, depending on which clotting factor is missing. People who are missing or deficient in factor VIII have Type A hemophilia. This is the most common form, affecting approximately 12,000 people in the United States. Hemophilia A is also called factor VIII deficiency or classical hemophilia.


Those who are missing or deficient in factor IX have Type B hemophilia, which affects about 4,000 in the United States. It’s also referred to as factor IX deficiency or Christmas disease.


Though lacking a cure, hemophilia can be treated. The missing factor can be replaced by clotting factor concentrates. These elevate the factor levels enough to allow the body to form a clot and stop the bleeding.


Once factor is in the bloodstream, the clotting potential remains at normal values for 8 to 12 hours for factor VIII and 18 to 24 hours for factor IX.


Clotting factor concentrate comes as a freeze-dried powder that’s mixed with sterile water prior to use. It’s infused into the body through a vein or central venous access device.


There are two types of clotting factor concentrates, depending on the source. Plasma-derived factor is made from donated human blood plasma. The clotting proteins are separated from other blood components, then purified and made into a freeze-dried product. All plasma donated for concentrates is tested for viruses.


Recombinant factor is made in a laboratory and uses little or no blood plasma. Since human blood proteins are kept to a minimum, there’s little risk of contracting viruses. Recombinant factor VIII products have been available since 1992 and recombinant factor IX products since 1997.


Several regimens are used to manage hemophilia, as directed by the doctor. Episodic treatment (also called on-demand treatment or PRN) is given only in response to a bleeding episode. Prophylactic treatment is the practice of giving factor on a regular basis, usually several times a week. The goal of prophylaxis is to raise factor levels enough to prevent bleeds. Planned preventive treatment means to treat someone with factor before an event or activity that may cause bleeding, such as a medical operation.


People who have severe hemophilia often have bleeds in joints and muscles. In such instances, there’s no visible blood. Some bleeds occur for no reason and are termed spontaneous. Other bleeds are caused by injury or trauma.


Symptoms of a joint bleed include pain, tenderness, heat, swelling, limited motion, limping, or difficulty in using the affected joint. The symptoms may develop gradually as the injured joint slowly fills with blood.


Common symptoms of a muscle bleed include pain to the touch, a hard lump in the muscle, bruising, and tightness.


Some bleeds are inherently serious, including those in the head, throat, spine, and internal organs. Such bleeds require immediate attention.

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