Von Willebrand Disease (VWD) also known as ANGIOHEMOPHILIA is a genetic disorder caused by the absence or defect of the Von Willebrand Factor (VWF) — A protein responsible for blood clotting. It is a bleeding disease (Hemophilia).
Von Willebrand Factor is the key clotting protein which aids in the formation of a platelet plug during the clotting process. It was named after ERIK VON WILLEBRAND, A Finnish physician who had first described it in the 1920s.
In the layman’s terms, the Von Willebrand Disease occurs when an individual lacks or has a defected Von Willebrand Factor which causes blood not to clotting properly or not clot at all leading to excessive bleeding.
- Heredity: Most people with VWD are basically born with it. It is passed from a parent or both parents to child. Although on rare occasions, it is possible for a person to get VWD without a history of the disease in their family. This occurs as a result of spontaneous mutation. That means that there’s an abnormal change in the person’s Gene. Once a person has VWD, the person passes it to their children automatically.
- Acquired Von Willebrand Disease: Rarely, a person can get VWD when the person’s immune system is triggered by a medication or disease to self destruct his or her VWF.
Many people Von Willebrand Disease are not aware of it because the signs may be mild or not present at all. The most common sign of VWD is Excessive Bleeding.
There are three main types of VWD. The amount of bleeding varies from one person to another, based on the type and the seriousness of the disease.
- Heavy or long menstrual bleeding
- Presence of blood in stool or urine
- Nose bleeding for more than ten minutes
- Getting bruises easily or lumpy bruises.
- Experiencing symptoms of anaemia, fatigue or shortness of breath during menses.
Types of von willbrand disease
- Type 1: It is the most common and mildest type of VWD occurring in about 75 percent of individuals affected with VWD. This is mostly inherited from one carrier parent.
- Type 2: This is further divided into four based on the level of severity. It can be sometimes gotten from one carrier parent or both.
- Type 3: It is the most severe and rarest type of VWD. It is usually gotten from both carrier parents.
Acquired VWD Syndrome: It is characterized by abnormal bleeding into the skin and other soft tissues. This can also occur due to autoimmune diseases like Lupus or due to medications.
Von Willebrand Disease has no cure but can be managed and treated. An essential managing factor is to reduce bleeding risk before it occurs.
Certain drugs that thin the blood should be avoided.
Avoid Aspirin and NSAIDs such as Ibuprofen (Advil, Morin) and Naproxen (Aleve). Acetaminophen (Tylenol) is a good alternative.
Type 1 only requires treatment if the patient has surgery, tooth extraction, or when an injury occurs.
A standard treatment for VWD, Desmopressin acetate (DDAVP) available as an injection or a nasal spray, is a synthetic form of hormone vasopressor. It causes the release of Von Willebrand Factor from one’s cells. Although it has a side effect, which is the retainment of water in the body; as a result, fluid intake should be limited.
For women with VWD, Their treatment may include birth control pills, which would increase the level of Von Willebrand Factor in their blood.
Those with type 3 are expected to get treated immediately in a case of bleeding or injury as failure to do so right away is very fatal.
Rarely, VWD can result in uncontrollable bleeding, which can be fatal.
Other complications include:
Anemia: This occurs in women who have heavy menstrual bleeding. They can develop Iron Deficiency Anaemia.
Swelling and severe pain: As a result of excessive or abnormal bleeding in the joints or soft tissues.
Women with Type 3 of VWD should not attempt pregnancy and childbirth, as this could be very fatal for them.
On many occasions, Von Willebrand Disease is usually Inherited, and to avoid this or reduce the chances of this occurring, Genetic counseling is to be considered by intending patients planning to have children. One can still pass it on to their offspring, even without symptoms.
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