Von Willebrand Disease

Von Willebrand Disease – Inherited Bleeding Ailment

Von Willebrand disease is the most common inherited bleeding ailment caused by a deficiency of von Willebrand factor – VWF. People suffering from this disease either have a deficiency of a blood protein known as Von Willebrand factor or the protein does not function properly. It means that their blood cannot clot properly and are inclined to prolonged or excessive bleeding. Von Willebrand factor tends to act like glue enabling platelets which is a kind of blood cell, to stick together and plug damaged blood vessels.

They carry and protect another clotting factor known as FVII – factor 8.To know about this ailment it is essential to understand more on how blood clotting works. The platelets are circulating blood cells which functions by stamping together and plug broken blood vessels in order to stop from bleeding. VWF is accountable in helping platelets to clot together. If the VWF is absent or low in the body, the platelets are unable to stamp properly leading to prolonged bleeding. This disease is quite rare and according to the Centres for Disease Control and Prevention, it tends to affect at the most, 1% of the overall population.

Fault in the Gene – Responsible, Production of VWF

VWD is the result of a fault in the gene which is responsible for the production of Von Willebrand factor. The genetic fault could be passed on to a child through one or both of their parents. This condition can cause problem at any stage of birth though for several people the symptoms are mild and they may not notice that they could be suffering from this disease. Some of the symptoms may include –

• Bleeding from the gums, nose and the lining of the gut
• Bruise easily
• Prolonged bleeding from cuts
• Excessive bleeding after tooth extraction or surgery
• Women suffer from heavy menstruation

Types of Von Willebrand Disease 

There are four kinds of VWD and are classified according to the function and amount of the Von Willebrand factor in the blood.

• Type 1 is the mildest and the most common type and those suffering from this type, have a reduced level of VWF in their blood. The symptom could be so mild that they may never know that they have VWD. They do not generally bleed suddenly though they can bleed considerably if they tend to have a surgery, have an injury or tend to extract their tooth.
• Type 2 is divided into further four subtypes. The VWF does not functions properly and the symptoms of this type are often more severe than type 1, though milder than type 3.
• Type 3 is a rare type with very low levels of VWF in their blood or not at all causing problems which are similar to haemophilia. There is bleeding from the mouth, nose and bowels in the early age with joint and muscle bleeds which could occur after injury.
• Platelet or Pseudo type is similar to type 3 and instead of the abnormality taking place in the VWF, it takes place in the platelets.

Diagnosis of Von Willebrand disease emphasises on family and personal history of abnormal bleeding, People suffering from type 3 are easy to diagnose since these people begin their life with severe bleeding. Together with history, the physician may also use various other laboratory tests to check for low or absent VWF and measure plasma levels and the time taken for the bleeding to stop.

Due to their specialized nature, it could take two to three weeks to receive blood test results. Timely treatment tends to helps people suffering from severe symptoms to lead a normal and active life