Congenital Von Willebrand Disease - What You Need to Know Skip to main content

Congenital Von Willebrand Disease

Medically reviewed by Drugs.com. Last updated on May 6, 2024.

What is congenital von Willebrand disease (CVWD)?

CVWD is an inherited blood disorder that causes heavy bleeding or bleeding that will not stop. CVWD is present at birth.

What causes or increases my risk for CVWD?

CVWD is caused by a change in the gene that makes von Willebrand factor (VWF). VWF is a protein that helps blood clot. You may not have enough VWF, or it may not work correctly. If one of your parents has von Willebrand disease, you have a 50 percent chance of getting the mild form. If both parents carry the gene, you may get the severe form, or you may not get it at all. The chance of having the mild form is also higher if both of your parents carry the gene.

What are the signs and symptoms of CVWD?

  • Bleeding too much or too long from wounds, after surgery, or after having a tooth pulled
  • Easy bruising
  • Bleeding from the gums or nose that happens often
  • Bowel movements that are black or dark
  • Urine that is pink or red
  • Heavy monthly periods, or heavy bleeding when giving birth

How is CVWD diagnosed?

Your healthcare provider will ask about your medical history and the details of your bleeding episodes. Tell your provider if other family members have CVWD or other bleeding problems. You will have blood tests to check VWF, platelets, and other clotting factors.

How is CVWD treated?

CVWD cannot be cured. The goal of treatment is to prevent and control bleeding. You may need any of the following:

  • Fibrin glue is placed directly on a wound to stop the bleeding. It is also called fibrin sealant.
  • Desmopressin helps your body make more VWF.
  • Replacement therapy is concentrated VWF that is given through IV infusion. This therapy may be given regularly to prevent bleeding if you have severe CVWD.
  • Antifibrinolytic proteins is an IV treatment that helps stabilize blood clots after they form. This helps stop bleeding.
  • Birth control pills may be given if you are female to increase VWF levels in your blood. This may also help control heavy monthly periods.

What can I do to manage a bleeding episode?

  • Apply direct pressure to the bleeding area. Use a clean cloth. Hold pressure constantly for at least 10 to 15 minutes and until the bleeding stops. Place another cloth over the first cloth if it gets soaked with blood.
  • Pinch the end of your nose during a nosebleed. Hold pressure constantly for 10 to 15 minutes. Breathe through your mouth and lean forward to keep blood from going down your throat.
  • Rest when you have a nosebleed. Place 2 to 3 pillows behind your head when you are lying down. This will help you breathe easier. If you ever feel weak or dizzy, sit or lie down right away.
  • Use extra pads during your monthly periods. Ask your healthcare providers about other ways to help control heavy menstrual bleeding.

What can I do to prevent bleeding episodes?

  • Do not play contact sports, such as football or hockey. Contact sports increase your risk for bleeding or bruising. Ask your hematologist or healthcare provider which sports and activities are best for you.
  • Do not use aspirin or NSAIDs, such as ibuprofen. These may thin your blood or increase your risk for bleeding. Ask your hematologist or provider before you take other over-the-counter medicines. Some may worsen VWD.

Call your local emergency number (911 in the US) if:

  • You have chest pain or tightness.
  • You have trouble breathing.
  • You have a seizure or faint.

When should I seek immediate care?

  • You cannot control your bleeding episodes, even after applying pressure.
  • You have many large bruises or joint swelling.
  • You vomit blood or have bloody or black bowel movements.
  • You have blood in your urine.

When should I call my doctor?

  • You feel tired and weak.
  • You have a fever.
  • You have nausea, are vomiting, or have a severe headache.
  • You have questions or concerns about your condition or care.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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