Von Willebrand disease (VWD) is a common inherited bleeding disorder. This guide provides an overview of the condition, its causes, symptoms, treatments, and how to manage living with it.
What is Von Willebrand Disease?
Von Willebrand disease is a lifelong bleeding disorder caused by a problem with a protein in your blood called von Willebrand factor (VWF). This protein helps your blood clot when you have an injury. If you have VWD, your body either doesn’t produce enough VWF or the protein doesn’t work as it should. This can lead to prolonged or excessive bleeding.
Key Facts About Von Willebrand Disease
Background
- VWD is named after Dr Erik von Willebrand, who first described the condition in 1926.
- It is the most common inherited bleeding disorder, affecting approximately 1% of the population.
Causes
- VWD is caused by genetic changes (mutations) in the VWF gene, which affects the production or function of von Willebrand factor.
- It is usually inherited from one or both parents, but in rare cases, it can occur without a family history.
Incidence and Prevalence
- Incidence refers to new cases diagnosed each year, while prevalence refers to how many people have the condition at any given time.
- VWD affects about 1 in 100 people worldwide, but only around 1 in 10,000 experience symptoms severe enough to require treatment.
Who is Affected?
- Age: Symptoms can appear at any age and are often noticed during childhood or adolescence.
- Gender: Both males and females are affected equally, but women may experience more noticeable symptoms due to heavy menstrual bleeding or complications during childbirth.
- Ethnicity: VWD affects all ethnic groups equally.
Geographic Distribution
- VWD occurs worldwide and does not vary significantly between regions or countries.
How Does Von Willebrand Disease Impact You?
Types of VWD
There are three main types of VWD:
- Type 1 (Mild): The most common form (70–80% of cases), where VWF levels are lower than normal but still present.
- Type 2 (Moderate): The VWF protein is produced but doesn’t work properly; this type has several subtypes (2A, 2B, 2M, 2N).
- Type 3 (Severe): The rarest form, where there is little to no VWF in the blood, leading to severe bleeding episodes.
Symptoms
Symptoms vary depending on the type and severity of VWD and may include:
- Frequent nosebleeds that last longer than usual.
- Easy bruising with minimal injury.
- Prolonged bleeding after surgery, dental work, or injuries.
- Heavy or long menstrual periods (in women).
- Blood in urine or stool (less common).
Living With Von Willebrand Disease
Living with VWD can be challenging but manageable with proper care:
- Avoid activities that increase your risk of injury or bleeding (e.g., contact sports).
- Inform healthcare providers about your condition before any medical procedures.
- Women should discuss menstrual management options with their doctor if periods are heavy or prolonged.
Expected Life Expectancy
With appropriate treatment and management, people with VWD typically have a normal life expectancy.
Managing and Treating Von Willebrand Disease
Available Treatments
Treatment depends on the type and severity of VWD:
- Desmopressin (DDAVP): A medication that stimulates your body to release more von Willebrand factor into your blood. It is often used for mild cases (Type 1).
- Clotting Factor Replacement Therapy: For moderate to severe cases (Type 2 or Type 3), this involves infusions of von Willebrand factor concentrate to replace the missing or defective protein in your blood.
- Antifibrinolytic Medications: These drugs help prevent clots from breaking down too quickly and are often used during dental work or minor surgeries.
- Hormonal Therapy: Women with heavy menstrual bleeding may benefit from hormonal treatments such as oral contraceptives.
- Topical Agents: Special gels or sprays may be applied directly to wounds to stop bleeding.
Ongoing Clinical Research
Research continues to improve understanding and treatment of VWD, including:
- Development of longer-lasting clotting factor therapies to reduce the frequency of infusions.
- Gene therapy approaches aimed at correcting the underlying genetic defect.
- Studies exploring new medications for better symptom control.
Support Groups and Resources
If you have been diagnosed with VWD, connecting with others who understand your experience can be helpful:
- Haemophilia Society UK(org.uk) – Provides support for individuals with bleeding disorders in the UK.
- World Federation of Hemophilia(org) – Offers global resources for patients with haemophilia and related conditions like VWD.
- Von Willebrand Disease Support Group– Many online forums provide peer-to-peer support for those living with VWD.
