What is the difference between Hemophilia A and Hemophilia B?

There is very little difference between hemophilia A and hemophilia B.  Type A is ofter referred to as classical hemophila.  Type B is referred to as christmas disease.  Both A and B are casued by a low level or absense of a protein in the blood that controls bleeding.  These proteins are called factors.  Hemophilia A is caused by a deficiancy of factor VII.  Hemophilia B is caused by a deficiancy of factor IX.  The only main difference between the two types is that hemophilia B is five times less common than hemophilia A.

Are there other types of bleeding disorders?

There are several other disorders of the blood.  They are all caused by deficiancies in factors I, II, VII, VI, X, XI, XII and the von Willebrand factor.

How does a person contract Hemophilia?

Hemophilia is a genetic disorder that is inherited from parent to child.  It cannont be caught or transmitted.  However one third of all new hemophila cases is caused by a new mutation of a gene in the mother or child.  There families have no prior history of the disease.

Is hemophilia a lifelong disorder?

Yes.  Someone born with the disease will have it for life.  The levels of VIII or XI stays the same throughout the persons life.

How is hemophilia inherited?   

Hemophiila is a disorder that is passed from parent to offspring.  The genes for hemophilia A and hemophilia B are found on the X chromosome.  The X and Y chromosomes are the chomosomes that determine sex in an organism.  Organisms that have both and X and a Y chromosome are male and organisms that have two X chromosomes are female.  Since men have only one X chromosome, if they happen to have the gene for hemophilia they will automatically have the disease.  Women are often just carriers of the gene because their other X chromosome makes up for it.  If the father has the hemophila but the mother is normal, only the couples daughters will be carriers of the gene.  If the father is normal and the mother is a carrier, there is a 50% chance their son will have hemophilia and a 50% chance the daughter will have the gene.

Father with hemophilia	Mother carrying hemophilia gene

Are there any precautions you should take if you have hemophilia and are pregnant?

What is acquired hemophilia?

In rare cases, humans can develop hemophila later in life. Acquired hemophilia is caused by the development of antibodies to factor VIII, which is the factor that caused the blood to clot.  There is, however, a treatment for acquired hemophilia and usually the dieseases is resolved with this treatment.  The treatment involves a combination of steroid treatment and the drug cylophosphamide.

How rare is hemophilia?

Hemophila is actually considered a fairly rare disease.  About 1 in 10,000 people will be born with hemophilia A each year and about 1 in 50,000 people will be born with hemophila B each year.

How serious is hemophilia?

The severity of hemophila is determined by the level of clotting activity of factor VIII or factor IX in the blood.  There are three different levels of severity: mild, moderate and severe.  In severe cases, people with hemophilia bleed frequently into their muscles and joints.  They usually bleed up to two times a week.  The bleeding is uaually spontaneous which means there is no real casue that starts the bleeding, it simply happens.  People with a moderate case of hemophila bleed less frequently than those with a severe form.  They will usually bleed once a month.  The bleeding can be spontaneous but usually isn't.  People with mild hemophilia usually bleed only as a result of surgery or major injury.  The bleeding is usually never spontaneous and some may go their whole lives without a bleeding problem.

What are the signs of hemophilia?

They symptoms for hemophila A and hemophilia B are the same.  People with hemophila will discover big bruises, bleeding into muscles and/or joints, (especially into the knees, elbows and ankles), sudden internal bleeding caused by no apparent reason, (often referred to as spontaneous bleeding), bleeding for a long time (usually after a cut, ect.), bleeding into vital organs usually after severe trauma.

How is hemophilia diagnosed?

The disease is diagnosed by taking a sample of blood to measure the factor levels.

How is hemophila treated?

Hemophila is treated by replacing the missing clotting factors in the blood.  The clotting factors are injected into the vein.  The bleeding does not stop until the clotting factors reach the bleeding site.  Clotting factors are found in blood products such as factor concentrates, cryoprecipitate, plasma and whole blood. 

Factor concentrates are the preferred treatment for hemophilia.  They are made from human blood.  It is a plasma derived product.  Factor concentrates can be manufactured by using genetically engineered cells that carry the human factor gene.  These are call recombinant products.

Doctors will treat people with hemophilia A by using desmopression, or DDAVP.  This is a synthetic hormone that stimulates the release of factor VIII.

Cryoprecipitate is taken from the blood and contains a high concentration of the clotting factor VIII.  This is usually used for joint and muscle bleeds.  Cryoprecipitate is less safe from viral contammination than factor concentrates.

Fresh plasma is another way of treating people with hemophilia.  Plasma is made when the red blood cells are removed from the blood, leaving blood proteins that include clotting factors behind.  Large volumes of fresh plasma must be transfused to stop the bleeding and this in turn may cause circulatory overload.

Using whole blood as a treatment has considerable drawbacks.  The blood must be fresh because the clotting activity decreases over time.  The red blood cells must also be compatible with the recipiant.  The use of whole blood for treating hemophilia may cause the heart to fail because it takes a large volume of blood and may overload the circulatory system.

Hemophilia

Signs and Symptoms of Hemophilia Eckert 

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