What is von Willebrand Disease: Guide to Common Questions

What is von Willebrand Disease: List of common questions and answers

Q: What is von Willebrand disease (VWD)?

A: VWD is the most common type of bleeding disorder. People with VWD have decreased or defective von Willebrand factor, a protein in the blood that is important for clot formation. When a blood vessel is injured and bleeding occurs in people with VWD, it takes longer for their blood to clot and for the bleeding to stop. Visit BeyondTheBleed.com to learn more about VWD.

References

What is von Willebrand Disease, vWD? Hemophilia Federation of America website. www.hemophiliafed.org/bleeding-disorders/vwd/. Accessed January 26, 2017.

Von Willebrand Disease. National Organization for Rare Disorders (NORD) website. http://rarediseases.org/rarediseases/von-willebrand-disease. Accessed January 26, 2017.

What is von Willebrand Disease? National Heart, Lung, and Blood Institute website. https://www.nhlbi.nih.gov/health/health-topics/topics/vwd. Accessed January 26, 2017.

A: Von Willebrand factor (VWF) helps blood clot in 2 ways:

  1. VWF functions like glue to help platelets (blood-clotting cells) stick together and form a platelet plug at the site of the wound.
  2. VWF carries factor VIII, another protein important for clot formation, to the site of injury. VWF prevents factor VIII from being broken down in the blood stream.

Reference

What is von Willebrand Disease (vWD)? Hemophilia Federation of America website. www.hemophiliafed.org/bleeding-disorders/vwd/. Accessed January 26, 2017.

A: Von Willebrand disease (VWD) and hemophilia are both bleeding disorders that prevent the blood from clotting. The difference between VWD and hemophilia is that VWD is caused by decreased or defective von Willebrand factor and hemophilia is caused by decreased or defective clotting factor VIII or IX (clotting factor VIII in hemophilia A and clotting factor IX in hemophilia B). Both disorders are usually inherited, but are inherited differently. Hemophilia is an X-linked disorder and is seen more often in males. Disorders that are X-linked are caused by a mutation or change in genes located on the X chromosome. VWD affects males and females equally and is caused by a genetic change on chromosome 12.

References

What is von Willebrand Disease, vWD? Hemophilia Federation of America website. www.hemophiliafed.org/bleeding-disorders/vwd/. Accessed January 26, 2017.

What is Hemophilia? Hemophilia Federation of America website. http://www.hemophiliafed.org/bleeding-disorders/hemophilia/. Accessed January 26, 2017.

Bleeding Disorders. American Society of Hematology website. http://www.hematology.org/Patients/Bleeding.aspx. Accessed January 26, 2017.

VWF Gene. Genetics Home Reference website. https://ghr.nlm.nih.gov/gene/VWF. Accessed January 26, 2017.

A: Von Willebrand disease is the most common type of bleeding disorder. Men and women are equally affected and it is estimated to affect 1% of the world’s population.

Reference

What is von Willebrand Disease (vWD)? Hemophilia Federation of America website. www.hemophiliafed.org/bleeding-disorders/vwd/. Accessed January 26, 2017.

A: Von Willebrand disease (VWD) is usually passed down (inherited) from a parent to a child. In rare instances, a child can have VWD even without a family history of the disease. This can happen as a result of a spontaneous mutation (change) in the gene. The child can later pass the disease on to his or her children.

It is rare but possible for someone to acquire VWD later in life. If VWD is acquired, not inherited from a parent or a result of a gene mutation, it cannot be passed along to any children. VWD can be acquired when a person’s own immune system destroys his or her von Willebrand factor, often as a result of another disease or as a result of the use of a medication.

References

How von Willebrand Disease is Inherited. Centers for Disease Control and Prevention website. http://www.cdc.gov/ncbddd/vwd/inherited.html. Accessed January 26, 2017.

Facts About von Willebrand Disease. Centers for Disease Control and Prevention website. http://www.cdc.gov/ncbddd/vwd/facts.html. Accessed January 26, 2017.

A: There are 3 inherited types of von Willebrand disease (VWD).

Type 1 – Type 1 is the most common form of VWD. People with type 1 have low levels of von Willebrand factor (VWF) and may have low levels of factor VIII.

Type 2 – Type 2 is divided into subtypes (2A, 2B, 2M, and 2N) based on the mutation that causes it. These mutations cause the VWF protein not to work properly.

Type 3 – Type 3 is rare and is the most severe form of VWD. People who have type 3 usually have little to no VWF and low levels of factor VIII.

The type of VWD a person has does not necessarily determine how severe a bleed will be, or where or when it will occur.

References

What is von Willebrand Disease? National Institutes of Health website. www.nhlbi.nih.gov/health/health-topics/topics/vwd. Accessed January 26, 2017.

Facts About von Willebrand Disease. Centers for Disease Control and Prevention website. www.cdc.gov/ncbddd/vwd/facts.html. Accessed January 26, 2017.

A: The signs and symptoms of von Willebrand disease (VWD) can depend on the type and severity of the disorder. Some people may have such mild symptoms that they are unaware that they may have VWD.

Some of the more common signs of VWD are:

  • Frequent or hard-to-stop nosebleeds
  • Easy bruising
  • Heavy menstrual bleeding
  • Longer than normal bleeding after injury, surgery, childbirth, or dental work
  • Blood in the stool (feces) from bleeding into the stomach or intestines
  • Blood in the urine from bleeding into the kidneys or bladder
  • Bleeding into joints or internal organs in severe cases

References

Facts About von Willebrand Disease. Centers for Disease Control and Prevention website. www.cdc.gov/ncbddd/vwd/facts.html. Accessed January 26, 2017.

What are the Signs and Symptoms of von Willebrand Disease? National Heart, Lung, and Blood Institute website. www.nhlbi.nih.gov/health/health-topics/topics/vwd/signs. Accessed January 26, 2017.

A: To find out if a person has von Willebrand disease, a doctor will likely ask questions about personal and family histories of bleeding. The doctor will also perform a physical exam to look for unusual bruising or other signs of recent bleeding. Blood tests can be ordered to measure how the blood clots. The amount of clotting proteins present in the blood and whether or not they are working properly can be determined by the blood tests.

Reference

Facts About von Willebrand Disease. Centers for Disease Control and Prevention website. www.cdc.gov/ncbddd/vwd/facts.html. Accessed January 26, 2017.

A: There is presently no cure for von Willebrand disease (VWD), but with proper treatment and care, bleeding caused by VWD can be managed.

References

Von Willebrand Disease. Mayo Clinic website. http://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/home/ovc-20261643. Accessed January 26, 2017.

In Brief: Your Guide to von Willebrand Disease. National Heart, Blood and Lung Institute website. http://www.nhlbi.nih.gov/health/resources/blood/vwd-in-brief-html. Accessed January 26, 2017.

A: Treatment for von Willebrand disease (VWD) depends upon several factors: the type and severity of the disease, and how a patient has responded to previous therapies. One or more treatment options may be used to manage bleeding.

The most common treatments are:

Anti-fibrinolytic (clot-stabilizing) medications–These medications may be prescribed before or after surgical procedures and help slow the breakdown of blood clots.

Vasopressin Analogues–This injection or nasal spray is usually used to treat type 1 and some subtypes of type 2 VWD. This medication stimulates the release of von Willebrand factor stored in the lining of the blood vessels.

Contraceptives–Estrogen hormones found in contraceptives can boost the levels of von Willebrand factor (VWF) and factor VIII activity. These may be prescribed for women to help reduce heavy menstrual bleeding.

Factor Replacement Therapy– This treatment contains von Willebrand factor (VWF) and factor VIII. It is used to treat people with more severe forms of VWD or some people with milder forms of VWD. Factor replacement therapy is infused (injected into the bloodstream) to replace the missing VWF.

Reference

Treatment of VWD. Hemophilia Federation of America website. www.hemophiliafed.org/bleeding-disorders/vwd/treatment-of-vwd/. Accessed January 26, 2017.

A: Medical identification–People with bleeding disorders should carry medical information on them in case of an accident or a medical emergency. Wearing a necklace or bracelet with medical information will inform medical staff of your bleeding disorder and what treatment you require.

Emergency go-bag–It is recommended that every person with a bleeding disorder have a go-bag with supplies ready at all times.

Important information–Keep important telephone numbers (hemophilia treatment center, homecare company, physicians, insurance, and emergency room) in multiple locations; for example, on the refrigerator, in your wallet, on your child’s car seat, in school or work bags, with your car registration papers, and in your emergency go-bag.

Pregnancy–With the appropriate care, women with von Willebrand disease (VWD) can have a successful pregnancy and deliver a healthy child. Women with VWD should talk to their doctor before becoming pregnant and be monitored closely throughout their pregnancy in order to plan for a safe delivery.

Talking to Your Provider–People with VWD and other bleeding disorders face many challenges that affect their quality of life. Have ongoing conversations with your healthcare provider about issues such as pain, medication, and daily activities.

For even more tips about living with VWD, visit BeyondTheBleed.com.

References

Communicating With Your Provider About Pain. National Hemophilia Foundation website. https://www.hemophilia.org/Events-Educational-Programs/Online-Education/Collaborating-in-Care-Webcasts/Communicating-with-Your-Provider-About-Pain. Accessed January 26, 2017.

Information for Women. Centers for Disease Control and Prevention website. www.cdc.gov/ncbddd/vwd/women.html. Accessed January 26, 2017.

Emergency Preparedness. Hemophilia Federation of America website. www.hemophiliafed.org/bleeding-disorders/emergency-preparedeness/. Accessed January 26, 2017.

Family Emergency Kit Checklist. Centers for Disease Control and Prevention website. www.cdc.gov/ncbddd/hemophilia/documents/FamilyEmergencyKitChecklist.pdf. Accessed January 26, 2017.

What is von Willebrand Disease: Guide to Common Questions

Von Willebrand disease is the most common type of bleeding disorder. Learn more about the types, signs and symptoms.