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How is von Willebrand disease treated?
What is von Willebrand disease?
How is von Willebrand disease (vWD) diagnosed?
Oct 26, 2021 · Even though von Willebrand disease has no cure, treatment can help prevent or stop bleeding episodes. Your treatment depends on: The type and severity of your condition
1. Is there a cure for von Willebrand disease? No, von Willebrand disease is a lifelong disorder. However, most people have a mild form that causes little or no change in their lives. Even those with type 3 von Willebrand disease can live normal, active lives once they are treated. 2. Who should know that I have von Willebrand disease?
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Apr 29, 2021 · von Willebrand disease (VWD) is an inherited bleeding disorder resulting from quantitative or qualitative deficiencies in von Willebrand factor (VWF), a plasma multimeric glycoprotein controlling platelet adhesion and aggregation. 1 Although VWD is expected to affect men and women equally, there are more women diagnosed with VWD because of gynec...
- Cécile V Denis, Sophie Susen, Peter J Lenting
- 2021
- Overview
- Goals of treatment
- Desmopressin
- Replacement therapies
- Clot-stabilizing medications
- Oral contraceptives
- Fibrin sealants
- Self-care
- Frequently asked questions
- Summary
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.
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.
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.
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.
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.
Learn about long-term use of oral contraceptives.
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.
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.
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.
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.
Von Willebrand disease (VWD) is a common bleeding disorder, affecting males and females equally, that often manifests in mucosal bleeding. VWD can be secondary to a quantitative (Type 1 and Type 3) or qualitative (Type 2) defects in Von Willebrand factor.
- Angela C. Weyand, Veronica H. Flood
- 2021/12
- 10.1016/j.hoc.2021.07.004
Nov 11, 2017 · Von Willebrand disease (VWD) is the most common inherited bleeding disorder, with a reported prevalence of approximately 1 in 1000 persons. 1 Quantitative defects include type 1 VWD, with partial deficiency of von Willebrand factor (VWF), and type 3 VWD, with virtually complete deficiency of VWF.
Von Willebrand disease (VWD) is an inherited bleeding disorder caused by an abnormal blood-clotting protein called von Willebrand factor (VWF). The VWF is important for the body to create the first steps of a blood clot to stop bleeding with injury and surgery.
