Hemophilias - Treatments and Therapies

Hemophilias are a group of related bleeding disorders that are one of the most common and severe of the inherited bleeding disorders. Hemophilia A results from a deficiency in factor VIII and hemophilia B, also known as Christmas disease, results from a deficiency in factor IX. Because 30% of hemophilia patients have new mutations and a negative family history, any infant male with significant neonatal bleeding should be screened for hemophilia. The two most common types are hemophilia A and hemophilia B. Hemophilia generally affects men. In fact, one third of 10000 men is born with hemophilia A. About 17000 Americans are born with the genetic disease that causes the disease. Globally, an estimated 500000 people are living with a form of hemophilia.

Two main varieties of hemophilia exist. Hemophilia A is responsible for eighty percent of all cases. The genetic disorders responsible for hemophilia A result in low levels or abnormal production of the clotting protein factor VIII (FVIII). Hemophilia B, the second most common form of hemophilia, affects factor IX proteins (FIX) and accounts for almost twenty percent of hemophilia cases.

Hemophilia A is responsible for eighty percent of all cases. The genetic abnormalities responsible for hemophilia Following the weakness or abnormal production of the protein coagulation factor VIII. Hemophilia B, the second most common form of hemophilia, Factor IX affects proteins and represents nearly twenty percent of cases of hemophilia. Other heredity-based hemophilia disorders may affect other blood clotting factors, but they occur rarely. The most common and major complication of hemophilia is chronic hemarthroses which can lead to joint destruction. The other most common complication is infections transmitted by therapy.

Children with hemophilia have benefited greatly from the replacement of clotting factors derived from donor blood. In some children this is done as-needed, but in other children it is done regularly to prevent bleeds. This is usually done by parents. Children with hemophilia benefit from participation in a comprehensive treatment program including psychological and family support, and monitoring and treatment of complications. Hospitalization for treatment of more serious bleeds is sometimes necessary.

In addition to the more common forms of hemophilia, there are other disorders of blood clotting that can cause problems such as strokes. There is a disorder called von Willebrand's disease which causes excessive bruising and frequent nosebleeds, and can be a problem in individuals undergoing surgery.