VON WILLEBRAND’S DISEASE

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Von Willebrand’s disease (VWD) is a condition that disrupts your blood’s ability to clot properly. This happens because of a shortage or malfunction of a protein called von Willebrand factor (VWF), which plays a crucial role in forming clots. As a result, cuts, injuries, or even surgery can lead to excessive bleeding.

The Most Common Inherited Bleeding Disorder

Von Willebrand disease (VWD), affecting roughly 1% of the population, is the most common inherited bleeding disorder passed down from one or both parents through their genes. There are three main inherited types of VWD, with varying severity of symptoms.

• Type 1: This is the most frequent form, causing mild symptoms like frequent bruising or nosebleeds. People with Type 1 typically have low levels of VWF in their blood.
• Type 2: This type is characterized by malfunctioning VWF, leading to symptoms ranging from mild to moderate.
• Type 3: The rarest form, Type 3, causes the most severe symptoms due to a complete absence of VWD or extremely low levels of another clotting protein. People with Type 3 may experience episodes of severe bleeding without any apparent cause.

There’s also a rare, acquired form of VWD that can develop due to autoimmune diseases, medications, heart disease, or certain cancers.

Recognizing the signs of Von Willebrand’s disease (VWD)

Symptoms of VWD vary depending on the type and severity. Here’s a breakdown of what to watch out for:

• Types 1 & 2: These often present with mild to moderate symptoms, such as:
  • Frequent or prolonged nosebleeds
  • Easy bruising from minor bumps
  • Blood in the stool or urine (internal bleeding)
  • Heavy bleeding after injuries or medical procedures
  • Heavy or prolonged menstrual periods (for women)
• Type 3: In addition to the symptoms of Types 1 & 2, people with Type 3 may experience:
  • Unexplained episodes of severe bleeding
  • Pain and swelling in soft tissues and joints due to bleeding

Diagnosis

If your doctor suspects VWD, they’ll consider your family history and bleeding symptoms. Blood tests can measure your VWF levels and clotting time to confirm the diagnosis.

Management and Treatment

While there’s no cure for VWD, it can be effectively managed. Here are some key strategies:

• Reducing Bleeding Risk: Avoiding medications that thin the blood, like aspirin and NSAIDs, is crucial.
• Desmopressin (DDAVP): Desmopressin (DDAVP) helps your body release more VWF from your cells, so it’s often used before surgery or dental procedures.
• Clotting Factor Concentrates: In severe cases, concentrated clotting factors may be administered intravenously.
• Medications for Heavy Periods: Birth control pills or hormonal IUDs can help regulate periods and reduce blood loss.
• Endometrial Ablation (Severe Cases): This procedure destroys the lining of the uterus to minimize menstrual bleeding.

Living with Von Willebrand’s disease (VWD)

While VWD is a lifelong condition, effective management strategies can significantly improve your quality of life. If you think you might have VWD, talk to your doctor for a proper diagnosis and treatment. For individuals with Type 3 VWD experiencing bleeding episodes, prompt treatment is crucial to prevent potentially fatal outcomes.

Sources:

• National Hemophilia Foundation: https://www.hemophilia.org/
• National Heart, Lung, and Blood Institute: https://pubmed.ncbi.nlm.nih.gov/18315614/
• Mayo Clinic: https://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/doctors-departments/ddc-20354986