The treatment of von Willebrand disease (VWD) varies depending on the type and severity of the condition. The main goal is to prevent or control bleeding episodes.

VWD is a common inherited bleeding disorder caused by a deficiency or dysfunction of von Willebrand factor (VWF), a protein that is essential for blood clotting. A person typically inherits VWD from a parent, although the severity of symptoms can vary greatly among family members.

The bleeding symptoms can range from mild to severe and include frequent nosebleeds, easy bruising, heavy menstrual bleeding, and prolonged bleeding from cuts or after surgery or dental work.

This article looks at the treatment options for people with VWD.

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The main goal of treatment for VWD is to manage bleeding symptoms and improve the quality of life for those with the condition.

Preventing bleeding episodes is particularly important for people with more severe forms of VWD or those with a history of frequent or severe bleeding. In these cases, doctors may use prophylactic treatments, such as regular infusions of clotting factor concentrates.

When bleeding does occur, the aim is to promptly and effectively control it. Prolonged or uncontrolled bleeding can lead to complications, such as joint damage or anemia. Treatment aims to minimize these risks by managing bleeding episodes effectively and maintaining healthy levels of blood clotting factors.

Desmopressin, also known as DDAVP, plays a vital role in the management of VWD, especially in treating type 1 and some subtypes of type 2 VWD.

As a synthetic version of the natural hormone vasopressin, desmopressin stimulates the body’s release of VWF and factor VIII, which are crucial for blood clotting. This temporary increase in clotting factors can effectively control bleeding episodes and is particularly useful in preparing people for surgeries or dental procedures where bleeding risk is a concern.

Healthcare professionals administer DDAVP as either a nasal spray or injection. It offers flexibility and convenience in treatment, making it a cornerstone in managing mild to moderate cases of VWD.

Replacement therapies in VWD involve the infusion of VWF concentrates. These therapies are essential for managing more severe cases of VWD, such as type 2 and type 3, where there is a significant deficiency or dysfunction of VWF.

By providing the missing VWF directly into the bloodstream, these concentrates help restore the normal clotting process.

Doctors can use regular infusions as a prophylactic measure to help prevent bleeding or on demand to treat acute bleeding episodes.

Clot-stabilizing medications, such as antifibrinolytic drugs (tranexamic acid and aminocaproic acid), complement other treatments by helping to prevent the breakdown of blood clots, thus enhancing the effectiveness of replacement therapies.

Learn more about anticoagulant therapies.

In people assigned females at birth who have VWD, oral contraceptives play a dual role. They not only provide birth control but also help in managing heavy menstrual bleeding, a common complication in VWD.

By regulating the menstrual cycle and increasing the levels of VWF and factor VIII, oral contraceptives can significantly reduce menstrual blood loss.

This effect helps not only in managing symptoms but also in preventing anemia — a frequent consequence of heavy menstrual bleeding in VWD.

Fibrin sealants are topical agents that medical professionals use during surgical procedures to enhance local blood clot formation.

By forming a fibrin clot at the site of bleeding, fibrin sealants work effectively to control local bleeding.

This is particularly beneficial in dental procedures or surgeries where local clotting is important.

Some things people can do themselves to manage their VWD include:

  • Detect bleeding early: Learn to recognize the signs of bleeding, such as prolonged nosebleeds, heavy menstrual periods, unusual bruising, or extended bleeding from cuts.
  • Respond promptly: Apply first aid measures such as pressure and ice for minor bleeding and seek medical attention for more serious or uncontrolled bleeding.
  • Choose the right exercises: Engage in safe, noncontact sports and exercises to maintain good health. Avoid activities with a high risk of injury. Use appropriate safety equipment, such as helmets and knee pads, when involved in physical activities.
  • Eat a balanced diet: Eat a nutritious diet, including iron-rich foods if there’s a history of anemia due to bleeding.
  • Stay hydrated: Proper hydration is important, especially if taking desmopressin (DDAVP), which can affect fluid balance.
  • Advise medical professionals: Telling doctors and dentists that a person has VWD, especially before surgeries, is essential. This way, they can prepare and take proper precautions.
  • Wear a medical bracelet: It is also important that people with VWD wear a medical bracelet to note that they have the condition. This alerts medical professionals in case of an emergency.

Here are some commonly asked questions about treatment for VWD.

Should a person eat or avoid any particular foods if they have von Willebrands?

There are no specific dietary restrictions or recommendations uniquely associated with VWD.

However, maintaining a balanced and healthy diet is always beneficial. Iron-rich foods can benefit someone with a history of significant bleeding leading to anemia.

Should a person avoid any medications if they have the condition?

People should generally avoid aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs), as they can exacerbate bleeding by affecting platelet function.

People must consult with healthcare professionals before taking new medications, including over-the-counter drugs and supplements, as some might increase bleeding risk.

How do doctors diagnose VWB?

Diagnosis typically involves blood tests to measure the levels and activity of VWF and sometimes clotting factor VIII. A detailed personal and family history of bleeding is also important for diagnosis.

What is the outlook for people with von Willebrand disease? Is it a life threatening condition?

VWD is usually not life threatening, especially with proper diagnosis and management. The severity and type of VWD play a significant role in determining a person’s outlook, with type 1 being the most common and mildest form.

Most people with type 1 VWD lead normal lives. Regular monitoring and medical care are important for managing symptoms and preventing complications.

Many treatments are available for people with VWD. A person’s healthcare team will determine which treatment best suits their needs.

Doctors may advise a combination of different treatments to manage VWD.