It is the most common genetic bleeding disorder, people with this disease have a problem with a blood protein called von Willebrand factor that helps control bleeding.
When a blood vessel is injured, and bleeding occurs, this element allows platelets to clump together and form a clot to stop the bleeding. People with this disorder do not have enough, or it does not work correctly.
The disorder hardly affects the lives of most people, except when a severe injury occurs, or surgery is required. According to a study, 1% of the population suffers from this disorder.
- VWD Type 1: is the most common form. People with type 1 have lower levels of VWF to normal.
- VWD type 2: is characterized by a defect in the structure of VWF. The protein does not function properly, causing lower than normal activity.
- VWD type 3: It is usually the most severe. People with Type 3 VWD may have to bleed in joints and muscles, without being afflicted by an injury.
Like hemophilia, von Willebrand’s disease is a genetic disorder that gets passed from parents to children.
The son of a man or a woman with von Willebrand’s disease has a 50% chance of inheriting the disease gene.
In types 1 and 2, the child has inherited the disease gene from one parent. In class 3, the son has inherited disease genes from both parents.
Many people suffering from this disease have almost no symptoms or are mild or moderate. Symptoms usually begin in childhood and continue throughout life.
But when they do occur, the severity varies from person to person. Many only notice the symptoms when they take aspirin or similar drugs that interfere with clotting.
Common symptoms include:
- Easy bruising.
- Frequent or prolonged nosebleeds.
- Prolonged bleeding gums and minor cuts.
- Heavy or prolonged bleeding during menstruation.
- Bleeding in the digestive system.
- Prolonged bleeding after injury, surgery, or invasive dental procedures.
The doctor will proceed with a physical examination, he asked about their symptoms and medical history. Von Willebrand’s disease can be challenging to diagnose.
Low levels of von Willebrand factor and bleeding do not always mean that the person has the condition.
Probably because it is a bleeding disorder, blood tests focusing on the following criteria are a need:
- Bleeding time – in this disease is prolonged, particularly after taking an aspirin.
- Factor VIII antigen – which indirectly measures levels of VWF in their blood; Thanks to this disease, it gets reduced.
- Willebrand factor multimers – examines the different structural types of VWF in the blood; due to this condition, it gets reduced.
Most affected people do not need surgery and if required will depend on the type VW suffering and its severity. It is only necessary if you have a dental or surgical procedure that may cause bleeding.
The aim of the treatment focuses on correcting the prolonged bleeding time and if the blood clotting disorder is present. To achieve this, both von Willebrand factor and factor VIII activity procoagulant should get raised to normal levels in the plasma.
You should get an average concentration of von Willebrand factor that is functionally normal in patients with qualitative abnormalities of the protein.
There are two trends in the treatment of this disease:
The replacement therapy. In some patient’s replacement therapy can induce the formation of inhibitory antibodies that can complicate the treatment of bleeding episodes making it impossible to correct the abnormality when the titer inhibitors are very high.
Induce the release of compound tissue stores using Desamino-8-Arginine Vasopressin (DDAVP.)
But some types of von Willebrand disease do not respond to DDAVP, so tests should be done to determine the specific type of von Willebrand factor of a patient before trauma or surgery.
The Alphanate (antihemophilic factor) is approved drug to reduce bleeding in patients with the disease have to undergo surgery or other invasive procedure.
Also, blood plasma or specific factor VIII preparations can be used to alleviate suffering.
It is essential to avoid factors that worsen the ability of blood clotting, so contraindicated the use of aspirin and other nonsteroidal anti-inflammatory drugs that affect platelet function.
There are no guidelines for preventing disease. It is advised to consult a genetic counselor to help review detailed family history and discuss risks and tests available for von Willebrand disease.