I've got the von Willies

Von Willebrand's Disorder (often abbreviated as vWD) is a hereditary bleeding condition and I've lived with it since I was diagnosed when I was a few months old.

The disorder occurs when there is missing or malfunctioning von Willebrand factor in the blood system. Normally von Willebrand factor attracts platelets and helps them stick to each other to make a clot when you are bleeding. The von Willebrand factor also protects and works with another blood clotting component called factor VIII - it is a lack of this clotting factor which causes haemophilia.

Von Willebrand's disorder is the most common bleeding disorder and approximately 1% of the population inherit it in some form. Most of these cases are mild and often go undiagnosed. It is more likely to be diagnosed in women since their bleeding tendency is more detectable during menstruation.

If one parent has von Willebrand's then their children have a 50% chance of inheriting it. It is possible for parents who do not themselves have von Willebrand's disorder to pass it down through either of their bloodlines. Unlike haemophilia where women carry the condition and rarely inherit it, men and women are equally likely to have von Willebrand's disorder.

The main sympton of von Willebrand's disorder is excessive bleeding - the severity of this can vary from person to person, both within the same types of the disease, and within the same family. Over half of all women with von Willebrand's disorder have excessive menstrual bleeding. Other symptoms include frequent nosebleeds, gum bleeds, bruising and heavy bleeding after injury or surgery. More rare symptoms include severe joint or internal bleeding and heavy blood loss during childbirth.

Von Willebrand's has been classified into the following types:

• Type 1 - reduced quantity of von Willebrand factor (mild). This type of vWD can result in mild to moderate bleeding, depending on how much von Willebrand factor is missing. Between 60-80% of all cases of vWD are Type 1. Type 1 patients show levels of Von Willebrand factor 10%-45% below normal and may experience easy bruising and/or heavy periods. Most patients with Type 1 vWD lead normal lives but trouble may arise during surgery or dental procedures where bleeding could be heavier than normal.
• Type 2 - reduced quality of von Willebrand factor (usually mild). The body produces normal amounts of von Willebrand factor but it doesn't function properly, this usually leads to bleeding problems similar to that of Type 1. Approximately 20%-30% of vWD cases are type 2 which actually contains 4 subgroups (2A, 2B, 2M and 2N) classified based on the specific quality reduction.
• Type 3 - little or no von Willebrand factor (more severe). Type 3 vWD is very rare. When the body does not produce von Willebrand factor, the platelets needed to form a clot cannot work properly and the factor VIII levels are reduced - both of which can lead to severe bleeding. Patients with type 3 may experience severe mucosal bleeding and occasional joint bleeding

vWD is a lifelong condition with no cure. People with vWD will need to prevent and treat bleeding episodes throughout their lives. The treatment varies based on the type of VWD you have and the nature of the bleeding episode.

Treatment may include:

• Desmopressin medication, to help increase the production of von Willebrand factor.
• Clotting factor replacement therapies.
• Antifibrinolytic agents that prevent breakdown of blood clots.
• Hormone therapy, to control heavy menstrual periods in women.
• Topical medication (fibrin glue).

I've sourced this information from two websites, my friend Helen's MSN Group Women Who Bleed:
http://groups.msn.com/WomenwhoBleed
and a site I've just found:
http://www.vonwillebrands.com/Articles/What_is_Von_Willebrand_Disease.php
set up by a man who has a son with vWD.