Approximately 20,000 people in the United States have been diagnosed with Hemophilia. People with
Hemophilia lack adequate amounts of certain blood proteins that would normally help blood clot. As a result,
they have frequent bleeds or bleeding episodes that can occur spontaneously or following an injury. They may
experience excessive or heavy bleeding following dental surgery or other operations. They also may have easily
bruised skin or hematuria (blood in the urine), both indications of internal bleeding.
- 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).
The significant danger for a person with Hemophilia or other bleeding disorders is internal bleeding and joint bleeds. Bleeding episodes in the joints can cause damage to the joint reducing the range of motion in the elbows, knees, or ankles. A bleed in the head can be very serious and may cause brain damage or even death.
In most cases Hemophilia is a disorder that is inherited. Hemophilia is classified as an X-linked recessive disorder, which means it is passed from a mother to her son. Statistics show that about 1 out of every 5,000 to 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 either a son with Hemophilia or a daughter who is a carrier. A man with Hemophilia will not pass it on to his sons; however all 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.
Most people who have Hemophilia have classic Hemophilia or Hemophilia A. This is also known as Factor VIII (8) deficiency. The remaining patients 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.
Von Willebrand Disorder
Von Willebrand disorder (vWD) is a bleeding disorder similar to Hemophilia that is usually genetically passed on from parent to child. It is more much common than Hemophilia, and is found in both men and women. People with von Willebrand disorder lack functional von Willebrand factor in their blood. Von Willebrand factor (named after the Finnish doctor who discovered it in 1924, Dr. Erik von Willebrand) is what makes blood clot normally. Von Willebrand factor can be defective or present in insufficient quantity to make blood clot normally.
Von Willebrand 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 the factor that is missing in their blood. Very mild cases of Bleeding disorders 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 bleeding episode from occurring. A small percentage of people will develop inhibitors to infused factor, and this may make the treatments less effective and also may complicate the treatment process.
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and assays for the home maintenance of bleeding disorders such as Hemophilia and
von Willebrand disorder.