Von Willebrand’s disease is a hereditary condition that affects an estimated 1 percent of the population.
Typically less severe than the bleeding condition called Hemophilia that targets males, von Willebrand’s disease affects adults and children of both genders. For members of those families who are affected, bleeding from an operation or serious injury can pose a major concern.
A Lack of Awareness
Many individuals are unaware that they even have this condition, sources say.
In addition, many primary-care physicians and other front-line medical professionals are not well-versed when it comes to von Willebrand’s disease. As a result, they may tend to overlook or fail to recognize tell-tale signs of this bleeding disorder.
Though von Willebrand’s disease is potentially fatal in rare instances, 70 to 80 percent of cases are classified as mild to moderate.
Typical symptoms include:
- Frequent nose-bleeds
- Bloody gums
- Easy bruising
Because of their physiology, women tend to be affected more often by symptoms of von Willebrand’s disease. For them, the most common symptom is heavy menstrual bleeding.
Understanding the Cause
Researchers have learned a great deal about von Willebrand’s disease since its discovery more than 80 years ago. The disease is named for Finnish physician Erik von Willebrand, who first described the condition in 1925.
In recent decades three major types of von Willebrand’s Disease have been identified. These classifications are based on the quantity and quality of a key blood protein that is called, fittingly enough, von Willebrand factor.
Our bodies have developed an efficient mechanism to control bleeding after we sustain an injury. But if one of the ingredients in this complex recipe is in short supply or defective, the entire system can be thrown off kilter.
This is precisely what occurs in individuals with von Willebrand’s disease.
When a blood vessel is damaged and starts bleeding, it triggers a three-step process. First the vessel constricts, slowing the flow of blood to the injured area. Then platelets in the blood start sticking to and spreading on the walls of the damaged vessel. Finally, a clot forms on the surface created by these platelets, stopping the bleeding.
The von Willebrand factor protein plays a crucial role in forming blood clots. It acts like glue, allowing the platelets to stick to each other and to the damaged vessel wall, eventually making a plug that stops the leakage of blood.
The symptoms linked to von Willebrand’s disease stem from either a lack of von Willebrand factor or flaws that prevent this essential protein from functioning properly.
Managing the Disease
A bitter irony about von Willebrand’s Disease is that although the disorder can be challenging to diagnose, medical breakthroughs have made it a relatively easy condition to treat and manage. There is no cure, however.
Current treatment options for patients include medications that can boost the body’s supply of von Willebrand factor, lowering the risk of uncontrolled bleeding episodes.
These medicines, some of which can be delivered in the form of an easy-to-use nasal spray, often are used before a patient with von Willebrand’s disease undergoes a dental procedure or takes part in rigorous outdoor activities.
The early use of these medications should be closely monitored by the physician because of potential side effects like changes in blood-pressure and the potential for seizures.
Individuals with more severe symptoms can receive infusions laden with von Willebrand factor. This is known as replacement therapy.