Von Willebrand disease Definition, Reason, Symptoms, Treatment & Prevention
Definition
Von Willebrand disease is a condition of the body with bleeding disorders caused by a deficiency of von Willebrand factor (VWF). VWF is a protein that helps the blood clotting process by clumping platelets. A deficiency of VWF causes platelets to be unable to clot or clot, causing prolonged bleeding.
There are three types of von Willebrand’s disease. The first type is the most common type, which causes lower-than-normal levels of VWF. This type allows the sufferer to live a normal life.
Type 2 sufferers have normal VWF levels but cannot work or carry out optimal activities due to abnormalities in the structure and function of these factors. The third type is the most severe disease condition. The sufferer has no VWF at all. So they often experience heavy bleeding that is difficult to stop.
Symptoms
Symptoms of von Willebrand disease depend on the type. However, all types usually indicate abnormalities in bleeding which include:
- Easy to bruise
- Excessive nosebleed
- Bleeding of the gums
- Heavy menstrual bleeding
- Type three can show symptoms of bleeding that is difficult to stop. Sufferers are also at risk of experiencing internal bleeding such as in the joints and digestive organs
Reason
The cause of von Willebrand’s disease is a genetic mutation. The type of disease a patient gets depends on the genetic map of his parents. Patients will have type three disorder if both parents pass on one of the mutated genes. If a patient has only one of the mutated genes, he or she will usually have type one or two of the disease.
Diagnosis
In the process of determining a Von Willebrand diagnosis, the doctor will ask for a history related to bleeding such as bruising and other bleeding. Family history will also be investigated.
Laboratory tests will be performed to check for abnormalities in VWF levels and function. A clotting factor VIII test will also be performed to rule out hemophilia.
Treatment
Treatment for von Willebrand disease depends on the type. Various treatment options include:
-
Therapy is not a substitute
The drug desmopressin (DDAVP) can help with types 1 and 2A. The drug stimulates the release of VWF from the body’s cells. Side effects that can occur include headaches, low blood pressure, and fast heart rate.
-
Replacement therapy
VWF replacement therapy can use Humate-P or Alphanate Solvent Detergent/Heat Treated (SD/HT). Both substitutes are proteins from biological or genetically engineered elements. This protein can replace the VWF function in the body.
This substitute protein is not identical to VWF, so sufferers should not use it interchangeably. Humate-P will be given to patients with type 2, patients who cannot tolerate DDAVP and severe cases. Side effects can occur in the form of chest tightness, rash, and swelling.
-
Topical therapy
The use of topical medications such as Thrombin-JMI and Tisseel VH can stop minor bleeding from small veins or veins. But can not stop major bleeding.
-
Drug therapy
Tranexamic acid and aminocaproic acid can help stabilize platelet clotting. This drug will be given to people who are going to have major surgery. This drug can also be used in people with type one von Willebrand disease. Side effects that can occur include nausea, vomiting, and complications with clots.
People with any type of von Willebrand disease should avoid drugs that can increase the risk of bleeding and its complications. Examples include aspirin and NSAIDs such as ibuprofen and naproxen.
Prevention
It’s impossible to prevent von Willebrand’s disease. This is because this condition is related to a genetic mutation passed down from parents.