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Von Willebrands Diseases

Von Willebrands Disease

Von Willebrand disease is an inherited disorder that affects the blood's ability to clot properly. When a blood vessel is cut or torn platelets (small cells that float in the blood) plug the hole in the blood vessel to stop the flow of blood. Later, Dr. von Willebrand discovered in truth that the illness was linked to a missing blood factor, which assists with the clotting of blood. Von Willebrand disease is the most common inherited bleeding disorder, affecting about 1% to 2% of people. A substance in the blood known as von Willebrand factor (vWF) helps platelets stick to damaged blood vessels. Another function of von Willebrand factor is to carry the important clotting protein, called factor VIII , in the blood and to the site of the injured blood vessel. With the help of calcium, vitamin K, and a protein called fibrinogen, the platelets create a mesh to hold the plug in place and close the wound. The disorder is not sex linked (autosomal), meaning that both men and women can have it, but some can be carriers only and not manifest any of the symptoms. Generally, vWD is divided into three types: Type I (mild), Type II (Medium) and Type III (severe). The lower the type, the less the symptoms will occur and the less strenuous they will be.

Other blood factors can be short-changed as well. Symptoms of von Willebrand's disease may include excessive bleeding after injury, frequent nosebleeds, heavy menstrual flow, easy bruising, blood in the urine or stool, and bleeding from the gums. When a blood vessel is cut or torn, platelets (small cells that float in the blood) plug the hole in the blood vessel to stop the flow of blood.In fact is 100 times more common than classic hemophilia! It was discovered by a Finnish doctor in the 1920s, who named it after himself and called it a disease. People with von Willebrand disease have a problem with the amount or quality of one or both of these blood components. Symptoms can range from abnormally long bleeding time due to a cut toenail, to hemorrhaging during minor surgery, to spontaneous nosebleeds, with a wide assortment of presentations between. von Willebrand's disease can be fatal.  There is no cure and no effective treatment.  It appears that DNA screening is the most accurate means of testing currently. Only through testing of all prospective breeding stock (in breeds known to be prone to vWD) and selective breeding is there a hope of erradicating this disorder.

Causes of Von Willebrands Disease

The common Causes of Von Willebrands Disease :

• Von Willebrand's disease affects both men and women alike (unlike hemophilia, which only leads to bleeding in men).
• Hypothyroidism
• Wilms tumor.
• Congenital heart disease.
• The condition is worsened by the use of aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs).
• Most cases are mild, and bleeding may occur after a surgical procedure or tooth extraction.
• Von Willebrand's disease is passed down through families. It is the most common bleeding disorder present at birth (congenital).
• Von Willebrand disease is caused by a deficiency of von Willebrand factor.
• In some cases, VWD is believed to result from other pathologic processes; however, because of the relatively high prevalence of VWD, its concomitant occurrence with other disease states may be coincidental.

Symptoms of Von Willebrands Disease

Some Symptoms of Von Willebrands Disease :

• Bruising .
• Frequent nose bleeds.
• Bleeding of the gums .
• Abnormal menstrual bleeding .
• Easy bruising .
• Nose bleeds .
• Skin rash.
• Bleeding into muscles and joints in more severe cases.
• Heavy or prolonged periods

Treatment of Von Willebrands Disease

• Desmopressin medication, to help increase the production of von Willebrand factor.
• Medication that helps prevent the breakdown of blood clots (antifibrinolytic agents).
• Fibrin glue is medicine placed directly on a wound to stop the bleeding.
• Hormone therapy, to control heavy menstrual periods in women.
• Medications such as desamino-8-arginine vasopressin (DDAVP) can be given to raise the levels of von Willebrand factor, which will reduce the tendency toward bleeding.
• Replacement therapy is the injection of a concentrate of von Willebrand factor and factor VIII.
• Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.
• Birth control pills-may be used to control heavy menstrual periods in women with type 1 VWD.

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