What is Von Willebrand Disease (vWD) and its treatment?
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Von Willebrand disease (VWD) is a blood condition that causes blood to clot improperly. Many proteins in blood assist the blood clot when it is needed. Von Willebrand factor is one of these proteins (VWF). VWD patients either have a low amount of VWF in their blood or the VWF protein does not function properly. VWD can be hereditary, acquired, or pseudo or platelet-based. VWD type 1, VWD type 2, and VWD type 3 are the three types of hereditary VWD. Among types 2 there are various subtypes that may be present. This is an inherited condition as well.
When a person is wounded and begins to bleed, the VWF in their blood binds to platelets, which are tiny blood cells. This causes the platelets to adhere together like glue, forming a clot at the injury site and stopping the bleeding. Because the VWF does not function properly in people with Von Willebrand disease, clots may take longer to form or form improperly, and bleeding may take longer to stop. This can result in severe, difficult-to-stop bleeding. Although uncommon, the bleeding can cause injury to joints or internal organs, as well as be life-threatening.
Diagnosis
Treatment:
Although there is no cure for Von Willbrand disease, therpay can help
avoid or halt bleeding episodes. Your treatment depends on:
The type and severity of your condition
How you respond to previous therapy
Your other medications and conditions
- Desmopressin: This medicine is available as a nasal spray or as an injectable (DDAVP) (Minirin) It's a synthetic hormone that helps to reduce bleeding by encouraging your body to create more von Willebrand factor, a protein found in the inner lining of your blood vessels.
- Oral contraceptives: These can help women reduce excessive bleeding throughout their menstrual cycles. The oestrogen hormones found in birth control tablets can increase the activity of von Willebrand factor and factor VIII.
- Replacement therapies: These include infusions of concentrated blood-clotting factors containing von Willebrand factor and factor VIII.
- Clot-stabilizing medications: Aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda) are anti-fibrinolytic medicines that can help halt bleeding by delaying the disintegration of blood clots. These medications are frequently prescribed by doctors before or after a surgical operation or tooth extraction.
- Drugs applied to cuts: A fibrin sealant (Tisseel VHSD) is applied to a wound, it helps to stop the bleeding. A syringe is used to apply it like glue. Nosebleeds can also be treated with over-the-counter medications.
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