Von Willebrand disease is a condition that causes excessive bleeding. Since it is caused by a genetic mutation, it is usually hereditary. However, Von Willebrand can be caused by other factors as well, such as autoimmune disorders or problems related to the thyroid (a gland in the neck that secretes hormones).
People who have Von Willebrand cannot clot their blood because they don’t have the protein needed for blood platelets to stick to each other. If you can’t clot your blood, you can’t form scabs in the wound healing process. This means that people with Von Willebrand have excessive bleeding.
There are three types of hereditary von Willebrand disease: Type 1, Type 2, and Type 3. Each type corresponds to how much von Willebrand factor is present.
Type 1 Von Willebrand disease
60-80% of all vWD cases are Type 1. Type 1 vWD is a result of heterozygous alleles, or having one good copy and one bad copy for the defective gene. This means that the vWD factor is present, just not as much as you would see in a normal patient. Most patients with Type 1 vWD end up leading a nearly normal life, with minimal impaired clotting. Some problems they may experience include bleeding following surgery, easy bruising, or menorrhagia (heavy menstrual periods). About 20-50% of the normal amount of von Willebrand Factor is detected in patients with Type 1 vWD.
Type 2 Von Willebrand disease
Type 2 makes up 15-30% of vWD cases. In this case, there are normal amounts of vWF proteins present in the blood, but they are abnormal or nonfunctional. For example, one type of abnormality is that the proteins may no longer efficiently bind to platelets.
Type 3 Von Willebrand disease
5-10% of patients suffer from Type 3 vWD, the most severe form. In this type, patients have homozygous alleles, or both bad copies, for the defective gene. Patients may have severe bleeding, no detectable vWF antigen, and bleeding in joints and muscles.
General symptoms of vWD include:
- bruising
- nosebleeds
- bleeding gums
- heavy menstrual periods
- blood loss during pregnancy
- joint/internal bleeding
- blood in stool or urine
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Often, especially in the Type 1 case, symptoms can be mild or overlooked because of their common nature. However, if bleeding persists and continues to become extended or severe, you should immediately contact a doctor. Some patients wear a medical ID bracelet, noting that they have vWD, in case they are in an accident.
Because patients often only complain of very mild symptoms, vWD is quite difficult to diagnose. Usually, specific blood tests are required to confirm the diagnosis. These tests include:
- vWF antigen – the amount of vWF present can help determine whether a patient has vWD, and also to classify which type the patient has
- Ristocetin cofactor activity – Ristocetin is an antibiotic that can react with blood and indicate vWD
- Factor VIII clotting activity – shows whether patients have low levels of factor VIII, which is carried by the vWF
- vWF proteins – examines the structure of vWF and identifies the type of vWD
- Platelet function
- Bleeding time – indicates the time needed for the bleeding from a small cut to stop
Von Willebrand Disease is a lifelong condition. Patients with vWD should be careful not to take aspirin, ibuprofen, naproxen, and other non-steroidal, anti-inflammatory drugs; these can increase complications with bleeding. Depending on the type the patient has, he or she may be able to live an active, healthy, and normal life.
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