Von Willebrand disease diagnosis and treatment, factor by Ray Sahelian, M.D.
March 20 2016

Von Willebrand disease is a family of bleeding disorders caused by an abnormality of the von Willebrand factor (vWF). It is the most common hereditary bleeding disorder affecting about one percent of the population. Von Willebrand disease is caused by a deficiency of von Willebrand factor. Von Willebrand factor helps platelets to clump together and stick to the blood vessel wall, which is necessary for normal blood clotting. VonWillebrand disease affects both men and women. Most cases are mild. Bleeding may occur after surgery or when you a tooth is pulled or spontaneous nosebleed can occur. Bleeding may decrease during pregnancy. For a list of natural supplements to avoid if you have this condition, see warfarin.

What to avoid if you have von Willebrand syndrome

Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) can make von Willebrand syndrome worse. It is possible that natural supplements that have blood thinning potential, such as fish oils and ginkgo, may also influence bleeding, but few studies are available to determine whether any herbs, such as garlic or onions, or nutrients that thin the blood or reduce blood clotting make this condition worse.

Risk factor for von Willebrand disease

A family history of a bleeding disorder is the primary risk factor. In women with heavy or prolonged menstrual bleeding, Von Willebrand is more common in Caucasian women than African American women.

Diagnosis

Accurate diagnosis and classification of von Willebrand disease is crucial for clinical management. A detailed clinical history, including that of bleeding, is required. A family and drug history are also important. Genetic factors such as blood group, and environmental factors such as stress, trauma, pregnancy and inflammation should also be considered. The age, ethnic group and hormonal status could also affect the von Willebrand factor levels. No single test is robust enough to detect all variants of von Willebrand disease. In view of the heterogeneity of the disease and limitations in assays, a battery of tests should be performed before a final diagnosis can be reached. These include the screening coagulation tests, factor VIII:C assay, von Willebrand factor antigen assay, assessment of functional von Willebrand factor which includes von Willebrand factor ristocetin cofactor assays, VWF collagen binding assay, ristocetin-induced platelet aggregation and VWF multimer analysis. The newer ELISA techniques based on VWF binding with factor VIII glycoprotein (Gp) 1b and cerebrosides have also helped in determining certain unusual forms of von Willebrand disease. The advent of new systems such as platelet function analysers (PFA), thromboelastography (TEG) and clot signature analysers (CSA), which are designed to assess either the primary platelet function or as a global haemostasis screen, have facilitated and simplified the diagnosis.

Inherited platelet function disorders are of variable severity and unknown frequency and may be difficult to diagnose. Nevertheless, they are increasingly recognized as an important cause of bleeding in pediatrics, particularly in adolescent girls with menorrhagia, where they may be more common than VW.

Von Willebrand Disease Treatment
Men and women are equally affected by von Willebrand disease, which is caused by a deficiency or defect in certain plasma proteins critical to blood clotting. In most affected people, the disease is mild, and treatment usually is not required to stop bleeding. However, about 2,000 people in the U.S. each year suffer from moderate and severe forms of the disease in which bleeding can be excessive if not treated. A variety of treatment options are available at present for patients with von Willebrand disease, some of which are affordable for patients in developing countries. For most patients who have type 1 vWD, desmopressin acetate (DDAVP) is the treatment of first choice, at least for minor bleedings and for prophylaxis. It is advisable, however, to use a test dose first to note the patient's response. DDAVP is safe to use, affordable, and easy to administer. However, most patients with type 2 vWD and all patients with type 3 fail to respond to DDAVP. For these patients, other options are used. Almost all patients with vWD will benefit from fresh frozen plasma and from cryoprecipitate, and these are viable options for developing countries. Both have certain disadvantages, but they can, depending upon the circumstances and facilities, be produced in developing countries. In developed countries, factor VIII/von Willebrand factor concentrates are widely used, especially for major bleedings and for surgeries on these patients. These concentrates are safe and virus inactivated, but costly. Ancillary treatment modalities such as antifibrinolytic agents and certain hormones are usually given in conjunction with other modalities.

New von Willebrand disease treatment
Antihemophilic Factor / von Willebrand Factor Complex (Human), Alphanate is a product for patients undergoing surgery or invasive procedures in whom the hormone desmopressin is either ineffective or contraindicated. ntihemophilic Factor / von Willebrand Factor Complex is not approved for patients with severe von Willebrand disease (Type 3) who are undergoing major surgery. Alphanate is the first biologic product approved for treatment of surgical and invasive procedures in patients with von Willebrand disease. Alphanate is already approved for the prevention and control of bleeding in patients with Factor VIII deficiency due to hemophilia A or acquired Factor VIII deficiency.
Successful management of surgery or invasive procedures in mildly, moderately and severely affected individuals routinely requires correction of the bleeding defect. In the absence of correction of the bleeding defect, patients may suffer from prolonged bleeding and delayed wound healing. Alphanate is purified from pooled human plasma and contains the clotting proteins deficient or defective in von Willebrand disease, which are Factor VIII (also know as Antihemophilic factor) and von Willebrand factor. Alphanate undergoes two separate steps for viral inactivation to reduce the risk for transfusion-transmitted viruses. However, the potential risk for the transmission of blood-borne viruses, and theoretically variant CJD, while very low, cannot be totally eliminated. Alphanate is manufactured by Grifols Biologicals, Inc., Los Angeles, Calif.
 

Questions
Q. I have Von Willabrand disorder and cannot take aspirin or any blood thinners. Is galantamine safe for me?
   A. We have not seen any studies regarding the use of galantamine and von Wlllebrand disease. Galantiamine should be used with caution by anyone.

Is it true that patients with bleeding disorders, example Von Willabrand's Disease should not use supplements such as: Ginseng, Ginkgo Biloba and Vitamin E? Please let me know if there are others.
    We don't have any specific research regarding these supplements and Von Willabrand's disorder, buy it may be a good idea for the time being to avoid such blood thinning supplements. A longer list can be found with the link at the top of the page for warfarin.

I have Von Willebrand Disease Type 1 and I would like to know if it is safe to take nattokinase. On a side not I am currently not on any medications for this condition. I would also like to know if nattokinase is safe to take for persons who have anemia.
   Since this is a bleeding disorder, I suspect nattokinase would not be recommended, and I also would not feel comfortable using it in those who have anemia.

I read your post and just wondered if supplementing with vitamin K could help patients with Von Willebrand disease or its acquired version (AVWS). While Von Willebrand conditions are NOT due to a vitamin K deficiency, the question is whether supplementing with vitamin K for such a condition might still have some merit.
   A. I have not seen any research in this area and, at this time, I doubt vitamin K would be of help.