Von Willebrand's Disease
This is an inherited disorder characterized by abnormally slow coagulation of the blood and spontaneous epistaxis and gingival bleeding caused by a deficiency of a component of factor VIII.
Excessive bleeding is common postpartum, during menstruation, and after injury or surgery.
It is also called angiohemophilia.
Von Willebrand's disease is the most common inherited blood coagulation disorder, caused by a deficiency in a blood clotting factor known as Von Willebrand's factor.
This factor promotes clotting in two ways: first, it is an essential component in the mechanism that causes platelets to gather and adhere to one another at the site of an injury; second, it acts as a carrier for Factor VIII (also known as antihemophilic factor), a crucial protein in the process of clot formation.
A deficiency in von Willebrand's factor thus results in uncontrolled bleeding due to inadequate platelet activity and decreased levels of active Factor VIII.
Von Willebrand's disease affects both men and women alike (unlike hemophilia, which only leads to bleeding in men).
The severity of symptoms varies widely - most cases are mild, with episodes of excessive bleeding presenting a risk only after surgery. Unlike hemophilia, most people with von Willebrand's disease need not limit their level of physical activity.
The severity of the bleeding tendency is described as very mild, mild, moderate, or severe.
Very mild means that the patient has only one or two unclear symptoms.
Mild indicates one or two obvious symptoms like nosebleeds, profuse menstruation, or frequent hematomas that usually do not require treatment.
Moderate means that the patient has two or more symptoms and has needed a transfusion because of abnormal bleeding after an operation or after a tooth extraction.
Characteristics of a severe bleeding tendency include hemarthrosis (blood in one or more of the joints)and bleeding in the muscles.
Diagnosis is established through collecting a clinical history from the patient and family and through a physical examination.
Blood tests are also used to measure clotting time and blood levels of von Willebrand's factor and Factor VIII.
Until recently there were only two direct treatments for the disorder: administration of blood products, which carry the risk of introducing a hidden viral infection, and the much safer intravenous injection of a synthetic hormone, desmopressin acetate, which mimics the action of the natural antidiuretic hormone vasopressin.
This drug is now available as a nasal spray - Stimate Nasal Spray - a prescription product that patients take at the onset of menses, after a minor injury or before undergoing dental work or any minor procedure that involves bleeding.
In patients who can produce von Willebrand's factor that is structurally and functionally normal, desmopressin acetate sets off its release from storage sites in blood vessels. Within two hours, blood levels of the factor become normal and can stem excessive bleeding.
A second or third dose of the factor, given a day or so apart, may sometimes be needed. Then, the body needs time to produce more factor before another dose can be effective.
What is the cause of the excessive bleeding?
Is the disorder mild, moderate, or severe?
What treatment do you recommend?
Do you recommend taking desmopressin?
How is this taken?
How can the bleeding be controlled?