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Quality of Life among Women with Bleeding Disorders Von Willebrand disease (vWD): An Overview Von Willebrand disease (vWD) is a most common type of hereditary bleeding disorder which prevails in 1% of the world’s population. Majority of people suffering from vWD are not diagnosed because its symptoms are usually mild…
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Download file to see previous pages However, health care physicians play a significant role in detecting signs and symptoms of vWD and in the ensuring that proper management is given to patients suffering from the disease (Lillicrap and James 2009). Von Willebrand Disease (VWD) is named after Dr. Erik von Willebrand (1870-1949) who first described the condition in 1926. He became famous for his description of bleeding among inhabitants of the island of Foglo in the Aaland archipelago in the Baltic Sea. In 1925, he examined a 5-year old patient whose four siblings died of bleeding disorders. In his report, he presented this as an unidentified type of hemophilia. He initially called the disorder “pseudohemophilia” because of lengthened bleeding time. During the course of his study, he noted that platelets were involved so he renamed it “constitutional thrombopathy” (Owens 2007). The ability of blood to clot is usually affected by vWD and can cause excessive bleeding. In rare cases, such bleeding may harm the internal organs or may even be fatal. VWD manifests in a deficiency in protein in the blood which is called von Willebrand factor (vWF) found in the linings of the blood vessels. Bleeding occurs when blood vessels are injured. Blood cells called platelets cluster together to block the hole in the blood vessel and control the bleeding. vWF serves as adhesion to help the platelets bond together to form a blood clot. Another vital protein that helps the blood clot is factor VIII (FVIII) found in vWF. FVIII is normally inactive in hemophilia which is another clotting disorder. In comparison with hemophilia, vWD more common and less serious among inherited bleeding disorders. It affects 1 out of 100 to 1000 people. Unlike hemophilia which mostly affects males, vWD has effects in both males and females (NHLBI 2008). vWD Disease Forms or Types Just like in many health conditions, vWD is classified into different forms. Type 1 is the most prevalent and is the mildest form, wherein vWF in the blood is decreased. The symptoms manifested are mild and may not even be detected. However, use of aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs) can aggravate the condition. People with Type 1 (partial quantitative deficiency), vWD do not have spontaneous bleeding but may bleed considerably with trauma, medical and dental procedures. In Type 2 (qualitative deficiency) vWD, there is an abnormality in vWF. This type is further subdivided into Type 2A and Type 2B. In Type 2A, the multimer which are the building blocks in VWF are smaller than the normal size and rupture easily. In Type 2B, vWF glues to the platelets too well resulting to the clustering of the platelets which can cause reduced platelet number. There are 2 more subtypes (2M and 2N) but these are much rarer. In Type 3 (total deficiency) vWD, there is severe bleeding with vWF and FVIII in the blood at very low levels. Pseudo or platelet type of vWD is comparable to Type 2B but in this case, there is a deficiency in the platelets instead of vWF (Griffin 2010). Prevalence of vWD VWD is prevalent worldwide and can also manifest in animal classes including canine and hogs. Its occurrence in people differs depending on the method applied to identify medical findings. It has been reported in two extensive epidemiologic researches that symptoms and signs of vWd were evident in up to 1% of children and infant population (Werner, Broxson, Tucker, Giroux, Schults, and Abshire 1993). Type 3 vWD ...Download file to see next pagesRead More
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