AN INTRODUCTION
It is one of the most common hereditary bleeding disorders in which the blood is unable to clot properly. Von Willebrand Factor (VBF) is the protein factor that is required for the blood clotting process. The deficiency of this factor in the blood causes bleeding disorders for a lifetime. It cannot be cured completely but by maintaining a healthy lifestyle, sticking to your medication, and by doing self-care one can easily fight the battle.
A breakage in the blood vessel causes bleeding. The blood platelets aid in the clotting process by clumping together at the site of injury. These platelets clump together with the help of all the important protein factors that are responsible for clot formation. VWF is one of these factors and the absence or low amount of it can stop platelets from clotting efficiently.
CAUSE OF VON WILLEBRAND DISEASE
The prominent cause of this disease is a genetic mutation. There are different types and its type depends upon the factor of the affected gene that has been passed from one or both parents to their offspring. When the VWF is very low it disrupts the functioning of blood platelets and they fail to attach themselves. As a result, uncontrolled or excessive bleeding occurs. It has also been found that most people suffering from this disease also have a deficiency of FACTOR VIII protein – responsible for hemophilia. Hemophilia affects only males but Von Willebrand Disease affects both males and females.
COMMON SYMPTOMS
The symptoms of this disease vary with the various types. It also depends upon the severity of the condition. Some of the common symptoms of this disease are:
· Heavy bleeding during menstruation
· Bleeding nose (frequent)
· Blood in urine
· Bruising
· Bleeding gums
· Abnormal bleeding after a slight injury
· Excessive bleeding after any surgery or operation
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TYPES OF VON WILLEBRAND DISEASE
· TYPE 1 – lower than normal level of VWF is present in the body
· TYPE 2 – normal level of VWF but with structural and functional defects
· TYPE 3 – no production of VWF in the body, a very dangerous state.
DIAGNOSIS
After assessing your medical history, the health care providers will ask you to perform several blood tests.
· Platelet count test
· Bleeding Time Test
· Factor VIII level
· The specific test for VWF
· PT & PTT TEST
LINE OF TREATMENT
The treatment of this disease depends upon the type of disease you have. The doctors may prescribe DDAVP, a drug that stimulates the release of VWF in the body. Along with this replacement therapy and other drug therapies are prescribed to patients depending upon their medical history and age. Regular follow-ups with strict medication regimens become the new normal for these patients.
PATIENT SELF TESTING is the most convenient method of performing your blood tests on your own. To know your PT/INR LEVEL you can easily monitor it with the help of PT/INR METERS ( a portable and accurate device) that gives you the result in no time. Patients who are taking WARFARIN (COUMADIN) or long-term blood-thinning medications can now get their doses adjusted very easily.