Has anyone heard of Von Willebrand's Disease? Or now anyone who has it?

Question

I went to the doctor yesterday because of bleeding gums, nose bleeds, tingle in the fingers and other odd symptoms. He said he wanted to take a few blood tests and on the paper he wrote von willebrands disease. Does anyone have any experience with that disease? I would love to hear about it.

I did look it up in a search- I just wanted to know if anyone has it, or know someone who has it and what the complications may be.

Answer 1

DEFINITION

Von Willebrand's disease is a congenital disorder of hemostasis characterized by defective or deficient von Willebrand factor (vWF). There are several subtypes of von Willebrand's disease. The most common type (80% of cases) is type I, which is caused by a quantitative decrease in von Willebrand factor; type IIA and type IIB are results of qualitative protein abnormalities; type III is a rare autosomal recessive disorder characterized by a near complete quantitative deficiency of vWF. Acquired von Willebrand's disease (AvWD) is a rare disorder that usually occurs in elderly patients and usually presents with mucocutaneous bleeding abnormalities and no clinically meaningful family history. It is often accompanied by a hematoproliferative or autoimmune disorder. Successful treatment of the associated illness can reverse the clinical and laboratory manifestations.

SYNONYMS: Pseudohemophilia

EPIDEMIOLOGY & DEMOGRAPHICS

• Autosomal dominant disorder
• Most common inherited bleeding disorder
• Prevalence is 1% to 2 % in general population, according to screening studies; estimates based on referral for symptoms of bleeding suggest a prevalence of 30 to 100 cases per million

PHYSICAL FINDINGS & CLINICAL PRESENTATION

• Generally normal physical examination
• Mucosal bleeding (gingival bleeding, epistaxis) and GI bleeding may occur
• Easy bruising
• Postpartum bleeding, bleeding after surgery or dental extraction, menorrhagia

ETIOLOGY

Quantitative or qualitative deficiency of vWF.

DIFFERENTIAL DIAGNOSIS

Platelet function disorders, clotting factor deficiencies

WORKUP

• Laboratory evaluation
• Initial testing includes PTT (increased), platelet count (normal), and bleeding time (prolonged)
• Subsequent tests include vWF level (decreased), factor VIII:C (decreased), and ristocetin agglutination (increased in type II B)

LABORATORY TESTS

• Normal platelet number and morphology
• Prolonged bleeding time
• Decreased factor VIII coagulant activity
• Decreased von Willebrand factor antigen or ristocetin cofactor
• Normal platelet aggregation studies
• Type II A von Willebrand can be distinguished from type I by absence of ristocetin cofactor activity and abnormal multimer
• Type IIB von Willebrand is distinguished from type I by abnormal multimer

TREATMENT

NONPHARMACOLOGIC THERAPY

• Avoidance of aspirin and other NSAIDs.
• Evaluation for likelihood of bleeding (with measurement of bleeding time) before surgical procedures. When a patient undergoes surgery or receives repeated therapeutic doses of concentrates, factor VIII activity should be assayed every 12 hr on the day a dose is administered and every 24 hr thereafter.

PHARMACOLOGIC MANAGEMENT

• The mainstay of treatment in von Willebrand's disease is the replacement of the deficient protein at the time of spontaneous bleeding, or before invasive procedures are performed.
• Desmopressin acetate (DDAVP) is useful to release stored vWF from endothelial cells. It is used to cover minor procedures and traumatic bleeding in mild type I von Willebrand's disease. Dose is 0.3 μg/kg in 100 ml of normal saline solution IV infused >20 min. DDAVP is also available as a nasal spray (dose of 150 μg spray administered to each nostril) as a preparation for minor surgery and management of minor bleeding episodes. DDAVP is not effective in type IIA von Willebrand's disease and is potentially dangerous in type IIB (increased risk of bleeding and thrombocytopenia).
• In patients with severe disease, replacement therapy in the form of cryoprecipitate is the method of choice. The standard dose is 1 bag of cryoprecipitate per 10 kg of body weight.
• Factor VIII concentrate rich in vWF (Humate-P, Armour) is useful to correct bleeding abnormalities in type IIA, IIB and type III von Willebrand's disease without alloantibodies. Alloantibodies that inactivate von Willebrand factor and form circulating immune complexes develop in 15% of patients with type III von Willebrand's disease who have received multiple transfusions. In these patients, recombinant factor VIII is preferred because autoantibodies can elicit life-threatening anaphylactic reactions because of complement activation by immune complexes.
• Life-threatening hemorrhage unresponsive to therapy with cryoprecipitate or factor VIII concentrate may require transfusion of normal platelets.

Answer 2

Von Willebrand disease is the most common hereditary bleeding disorder.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.

Blood plasma or certain factor VIII preparations may also be used to decrease bleeding.

I would wait until you get the result of your clotting screen,blood count and bleeding time before worrying further.

Answer 3

Von Willebrand's disease is a bleeding disorder of the blood much like hemophilia. Bruising and bleeding from various parts of the body is common. It is a congenital disease. Symptoms will decrease with age .

Answer 4

Von Willebrand's Disease is a type of hemophilia. Von Willebrand factor is a protein in the blood that is necessary for clotting. It's a genetically inherited disease. It can be easily diagnosed with appropriate blood tests, like PT/PTT/INR.

Answer 5

It's a mad horse disease

Answer 6

i think you misread something. von willderbrands is an animal disease


read the links this is a dog disease

http://search.yahoo.com/search?search=von+willderbrands&ei=UTF-8&ico-yahoo-search-value=http%3A%2F%2Frds.yahoo.com%2F_ylt%3DAkXV62RNJrvywFT81xIh.TjkxQt.%2FSIG%3D111gjvvgj%2F%2A-http%3A%2F%2Fsearch.yahoo.com%2Fsearch&ico-wikipedia-search-value=http%3A%2F%2Frds.yahoo.com%2F_ylt%3DAnZG4qGh1eao2MeWru0kGQfkxQt.%2FSIG%3D11ia1qo58%2F%2A%2Ahttp%253a%2F%2Fen.wikipedia.org%2Fwiki%2FSpecial%253aSearch&fr=ks-ques&p=willebrands+disease

Answer 7

http://www.mindspring.com/~mattrk/
here's a site that tells you a bit about it

Answer 8

why dont you type it up in search...and see what comes up about it...