India Blood Bank

Hemophilia is classified as an X-linked recessive disorder, which means it is passed from a mother to her son.  About 1 out of every 5,000 - 10,000 males born in the U.S. are diagnosed with Hemophilia.  A woman who is a carrier of the gene has a 50% chance of having a son with Hemophilia, or a daughter who is a carrier.  A man with hemophilia will not pass it on to his sons; however his daughters will be carriers of the gene.  In some cases there is no known history of Hemophilia in the family and gene mutations are sometimes the cause for the condition.  Approximately 80% of the people who have Hemophilia have classic Hemophilia or Hemophilia A.  This is also known as Factor VIII (8) deficiency.  The remaining have Hemophilia B, also known as Christmas Disease or Factor IX (9) deficiency.

Hemophilia is most often classified by its severity.  Although the levels can overlap, there are three basic classifications of Hemophilia.  The severity of the disease is defined by how much of the clotting factor is produced by the body, and in what situations bleeding occurs.  The three classifications are as follows:

• Mild Hemophilia:  Clotting Factor VIII (8) or Clotting Factor IX (9) is at 5% of normal levels or greater.  Mild Hemophilia may not be recognized unless there is excessive bleeding after a major injury or surgical procedure.
• Moderate Hemophilia:  Clotting Factor VIII (8) or Clotting Factor IX (9) level is 1% to 5% of normal levels.  Bleeding usually follows a fall, sprain, or strain.
• Severe Hemophilia:  Clotting Factor VIII (8) or Clotting Factor IX (9) level is less than 1% of normal levels.  Bleeding episodes often happen spontaneously (for no apparent reason).

Von Willebrand disorder

Von Willebrand disorder (vWD) is more common than Hemophilia, and is found in both men and women.  People with von Willebrand disorder lack functional von Willebrand factor in their blood.  This disorder is inherited autosomally meaning either the mother or father can pass it on to a son or daughter.  A person who is a carrier of the gene has a 50% chance of having a child with von Willebrand disorder.  There are three different types of von Willebrand disorder known as Type 1(Mild), Type 2(Medium), and Type 3(Severe).

Treatment of Hemophilia & von Willebrand disorder

Effective treatment of Hemophilia & von Willebrand disorder is available for most people.  This is achieved by replacing factor that is missing in their blood.  Very mild cases can be treated with inter nasal or IV desmopressin acetate.  In more severe cases a concentrated form of the missing factor protein is administered intravenously as soon as the person knows they are having a bleed, or to prevent a bleed from occurring.  A small percentage of people will develop inhibitors to infused factor.  This may make the treatments less effective and also may complicate the treatment process.

Medex BioCare (MBC) offers a complete line of biotech pharmaceuticals in all available brands and assays for the home maintenance of chronic conditions such as Hemophilia & von Willebrand disorder.