7 Common Von Willebrand Disease Symptoms You Should Know

Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting approximately 1% of the population worldwide. This condition occurs when the blood lacks sufficient von Willebrand factor (VWF), a protein that helps blood clot properly. People with VWD may experience various bleeding symptoms ranging from mild to severe, depending on the type and severity of the deficiency.

Understanding the symptoms of Von Willebrand disease is crucial for early diagnosis and proper management. While some people may have mild symptoms that go unnoticed for years, others may experience significant bleeding episodes that affect their daily life. The following symptoms are the most common signs that may indicate the presence of this bleeding disorder.

1. Frequent and Prolonged Nosebleeds

Nosebleeds, medically known as epistaxis, are one of the most common symptoms of Von Willebrand disease. Unlike occasional nosebleeds that most people experience, those with VWD tend to have nosebleeds that:

• Occur frequently without obvious cause
• Last longer than 10 minutes
• Are difficult to stop with normal pressure
• May require medical intervention or packing
• Often happen spontaneously, especially during childhood

The nosebleeds in VWD patients can be particularly troublesome because the nasal passages contain many small blood vessels that rely on proper clotting function. When von Willebrand factor is deficient, these vessels cannot form stable clots, leading to prolonged bleeding episodes. Some patients report experiencing nosebleeds several times per week, which can be both physically draining and socially embarrassing.

2. Easy Bruising

People with Von Willebrand disease typically bruise much more easily than the average person. This symptom, known as easy bruisability, manifests in several ways:

The bruises tend to appear after minimal trauma or sometimes even without any remembered injury. They are often larger than expected for the degree of impact and may take longer to heal compared to normal bruises. Women with VWD often notice extensive bruising on their legs, arms, and other areas where they might bump into furniture or other objects during daily activities.

These bruises occur because the blood vessels under the skin leak more blood than normal when damaged, due to the inability to form proper clots quickly. The bruises may appear as purple, blue, or greenish-yellow patches on the skin and can be quite extensive even from minor bumps.

3. Heavy Menstrual Bleeding

Menorrhagia, or heavy menstrual bleeding, is one of the most significant symptoms in women with Von Willebrand disease. This symptom is often the first clue that leads to diagnosis in female patients. Heavy menstrual bleeding in VWD is characterized by:

• Periods lasting longer than 7 days
• Soaking through one or more pads or tampons every hour for several consecutive hours
• Needing to use double protection (pad and tampon together)
• Passing blood clots larger than a quarter
• Experiencing symptoms of anemia such as fatigue and shortness of breath
• Having to change sanitary products during the night

Many women with VWD have lived with heavy periods for years, assuming it was normal, only to discover later that their bleeding was excessive. This heavy bleeding can significantly impact quality of life, causing women to miss work or school and avoid social activities during their periods. The chronic blood loss often leads to iron deficiency anemia, requiring iron supplementation.

4. Prolonged Bleeding from Cuts or Injuries

When people with Von Willebrand disease get cuts, scrapes, or other minor injuries, the bleeding tends to last much longer than normal. While a small cut might stop bleeding within a few minutes for most people, someone with VWD might experience bleeding that continues for 15 minutes or longer.

This prolonged bleeding can occur with various types of injuries:

• Minor cuts from shaving
• Kitchen accidents while preparing food
• Scrapes from falls or accidents
• Small puncture wounds

The bleeding may initially stop but then restart hours later, a phenomenon called delayed bleeding. This happens because the initial clot that forms is unstable without adequate von Willebrand factor. Patients often need to apply pressure for extended periods and may require medical attention for injuries that others would consider minor.

5. Excessive Bleeding After Dental Procedures or Surgery

Surgical and dental bleeding is a hallmark symptom of Von Willebrand disease and often provides an important diagnostic clue. Patients with VWD frequently experience excessive bleeding following:

Dental procedures: Tooth extractions, dental surgery, or even routine dental cleanings can result in prolonged bleeding that may continue for hours or days. The bleeding might stop initially but resume later, requiring patients to return to the dentist for additional treatment.

Surgical procedures: Any type of surgery, from minor procedures to major operations, can cause significant bleeding complications in people with VWD. This includes tonsillectomies, which are particularly problematic as they involve a very vascular area.

The post-operative bleeding may not be immediately apparent during the procedure but can manifest hours or days later as the initial clot breaks down. This delayed bleeding pattern is characteristic of Von Willebrand disease and distinguishes it from other bleeding disorders. Healthcare providers need to be informed of any history of excessive bleeding before performing any surgical or dental procedures.

6. Blood in Urine or Stool

While less common than other symptoms, some people with Von Willebrand disease may experience bleeding in the gastrointestinal or urinary tract. This can manifest as:

Hematuria (blood in urine): The urine may appear pink, red, or cola-colored, indicating the presence of blood. This can occur spontaneously or following minor trauma to the kidneys or bladder.

Gastrointestinal bleeding: Blood in the stool may appear as bright red blood, or the stool may look black and tarry, indicating digested blood from higher in the digestive tract. Some patients experience bleeding from the gums or nose that is swallowed and later appears in the stool.

These symptoms are more common in people with more severe forms of Von Willebrand disease. The bleeding can occur from ulcers, hemorrhoids, or other gastrointestinal conditions that might cause only minor bleeding in people without bleeding disorders. Any presence of blood in urine or stool should be evaluated by a healthcare provider to rule out other serious conditions.

7. Bleeding in the Mouth and Gums

Oral bleeding is another frequent symptom of Von Willebrand disease that can significantly affect daily life. People with VWD often experience:

• Bleeding gums during tooth brushing that lasts more than a few minutes
• Spontaneous gum bleeding without provocation
• Prolonged bleeding after biting the tongue or cheek
• Bleeding from minor mouth injuries that lasts unusually long
• Blood blisters in the mouth

The mouth is particularly prone to bleeding in VWD because it contains many small blood vessels and is subject to frequent minor trauma from eating, drinking, and dental hygiene activities. Some patients notice a metallic taste in their mouth from blood and may find traces of blood on their pillow in the morning from gum bleeding during sleep.

This symptom can be particularly concerning during dental visits or orthodontic treatments. Patients may need to take extra precautions and inform their dental care providers about their condition to ensure proper management and prevention of excessive bleeding.

Main Causes of Von Willebrand Disease

Von Willebrand disease is primarily caused by genetic mutations that affect the production or function of von Willebrand factor. Understanding the causes helps explain why this condition runs in families and how it develops:

Inherited Genetic Mutations: The vast majority of VWD cases are inherited from one or both parents. The disease is caused by mutations in the VWF gene located on chromosome 12. Depending on the type of VWD, the inheritance pattern can be:

• Autosomal dominant: Type 1 and Type 2 VWD typically follow this pattern, meaning only one mutated gene from one parent is needed to cause the condition. If one parent has VWD, there is a 50% chance of passing it to each child.
• Autosomal recessive: Type 3 VWD, the most severe form, requires two mutated genes (one from each parent) to develop the disease. Parents may be carriers without showing symptoms.

Acquired Von Willebrand Syndrome: Though rare, some people develop Von Willebrand syndrome (not disease) later in life without a genetic cause. This acquired form can result from:

• Autoimmune disorders where the body produces antibodies against von Willebrand factor
• Certain cancers, particularly lymphoproliferative disorders
• Cardiovascular conditions such as aortic stenosis
• Hypothyroidism and other metabolic conditions

Types Based on Underlying Defect:

• Type 1: Partial quantitative deficiency – the body produces less von Willebrand factor than normal (most common, about 75% of cases)
• Type 2: Qualitative deficiency – normal or near-normal amounts of von Willebrand factor are produced, but it doesn’t work properly (about 20% of cases)
• Type 3: Complete quantitative deficiency – virtually no von Willebrand factor is produced (rare, about 5% of cases, most severe)

Prevention Strategies

Since Von Willebrand disease is primarily an inherited genetic condition, it cannot be prevented in the traditional sense. However, people with VWD and their families can take important steps to manage the condition and prevent bleeding complications:

Genetic Counseling: Families with a history of VWD should consider genetic counseling before having children. A genetic counselor can explain the inheritance patterns, assess the risk of passing the condition to offspring, and discuss available options. This doesn’t prevent VWD but helps families make informed decisions.

Preventing Bleeding Episodes:

• Avoid medications that interfere with blood clotting, such as aspirin and non-steroidal anti-inflammatory drugs (NSAIDs), unless specifically recommended by a doctor
• Use appropriate protective equipment during sports and physical activities
• Choose activities with lower risk of injury when possible
• Maintain good dental hygiene to prevent gum disease and reduce the need for dental procedures
• Use a soft-bristled toothbrush to minimize gum trauma

Medical Preparation:

• Always inform healthcare providers, dentists, and surgeons about the VWD diagnosis before any procedures
• Wear a medical alert bracelet or necklace identifying the bleeding disorder
• Carry a medical information card with emergency contact numbers and treatment information
• Establish care with a hematologist experienced in bleeding disorders

Lifestyle Modifications:

• Maintain adequate iron intake to prevent anemia, especially for women with heavy menstrual bleeding
• Stay well-hydrated to help maintain proper blood volume
• Avoid excessive alcohol consumption, which can affect platelet function
• Learn to recognize early signs of bleeding and know when to seek medical attention

Education and Awareness: Educating family members, teachers, coaches, and caregivers about VWD ensures that appropriate actions are taken during bleeding episodes. Children with VWD should be taught age-appropriate information about their condition and how to communicate their needs to adults.

Frequently Asked Questions

What is the difference between Von Willebrand disease and hemophilia?

Von Willebrand disease affects both males and females equally and involves a deficiency of von Willebrand factor, while hemophilia primarily affects males and involves deficiencies in clotting factors VIII or IX. VWD is generally milder than hemophilia, and bleeding patterns differ, with VWD causing more mucosal bleeding (nosebleeds, gum bleeding) while hemophilia causes more joint and muscle bleeding.

Can Von Willebrand disease be diagnosed with a single blood test?

No, diagnosing VWD typically requires multiple blood tests because von Willebrand factor levels can fluctuate based on various factors like stress, exercise, pregnancy, or hormones. Tests usually include von Willebrand factor antigen, von Willebrand factor activity (ristocetin cofactor), and factor VIII levels. Sometimes tests need to be repeated to confirm the diagnosis.

Is Von Willebrand disease life-threatening?

Most people with VWD, especially Type 1, have mild symptoms and live normal lifespans. However, severe bleeding episodes, particularly after surgery or major trauma, can be serious if not properly managed. Type 3 VWD, the most severe form, requires careful medical management. With proper diagnosis and treatment, most complications can be prevented or managed effectively.

Can women with Von Willebrand disease have children safely?

Yes, most women with VWD can have children safely with proper medical management. Von Willebrand factor levels often increase naturally during pregnancy, which can help reduce bleeding risk. However, careful monitoring by both an obstetrician and a hematologist is essential, particularly during delivery and the postpartum period when bleeding risk increases. Planning ahead and choosing an appropriate delivery facility is important.

Do symptoms of Von Willebrand disease get worse with age?

Symptoms typically remain relatively stable throughout life, though they may be more noticeable during certain periods. Women may notice symptoms more during their reproductive years due to menstruation and childbirth. Some people find that symptoms become more apparent as they age and may require procedures or surgeries. However, the underlying severity of the disease itself doesn’t typically worsen with age.

Can people with Von Willebrand disease play sports?

Yes, most people with VWD can participate in sports and physical activities. The type of sport and severity of VWD should be considered. Contact sports may carry higher risks, especially for those with moderate to severe VWD. It’s important to discuss activity choices with a hematologist, wear appropriate protective gear, and ensure coaches are aware of the condition. Many people with VWD successfully participate in various sports with proper precautions.

Will my child inherit Von Willebrand disease if I have it?

The inheritance risk depends on the type of VWD. For Type 1 and most Type 2 variants, which are autosomal dominant, each child has a 50% chance of inheriting the condition if one parent is affected. For Type 3, which is autosomal recessive, both parents must carry the mutation for a child to be affected. Genetic counseling can provide specific risk assessments based on your family history and VWD type.

Should I avoid all medications if I have Von Willebrand disease?

Not all medications need to be avoided, but certain ones that affect platelet function or blood clotting should be used with caution or avoided. These include aspirin, ibuprofen, naproxen, and other NSAIDs. Always consult with your doctor before taking any new medications, including over-the-counter drugs and supplements. Your healthcare provider can recommend safe alternatives for pain relief and other conditions. Never stop prescribed medications without consulting your doctor first.

References:

• National Heart, Lung, and Blood Institute – Von Willebrand Disease
• Mayo Clinic – Von Willebrand Disease
• National Hemophilia Foundation – Von Willebrand Disease
• Centers for Disease Control and Prevention – Von Willebrand Disease
• NHS – Von Willebrand Disease