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Congenital Von Willebrand Disease

Medically reviewed by Last updated on Oct 3, 2022.

What is congenital von Willebrand disease?

Congenital von Willebrand disease, also known as congenital VWD, causes heavy bleeding or bleeding that will not stop. It is an inherited blood disorder that is present at birth. Von Willebrand factor (VWF) is a protein in the blood that helps it to clot. If you have congenital VWD, you may not have enough von Willebrand factor in your blood, or it may not work correctly. This makes it is difficult for you to stop bleeding because your blood does not clot properly.

What causes congenital von Willebrand disease?

Congenital VWD is caused by an error in the gene that makes a healthy von Willebrand factor. You may inherit congenital VWD if your parents have passed the gene to you. If one of your parents has VWD, you have a 50 percent chance of inheriting a mild form of VWD. If both parents carry the gene, you may get the severe form of VWD, or you may not get it at all. The chance of having the mild form of VWD is also higher if both of your parents carry the gene.

What are the signs and symptoms of congenital von Willebrand disease?

With congenital VWD, there may be times when you bleed for no reason at all. These are called bleeding episodes. Bleeding episodes can be any of the following:

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

How is congenital von Willebrand disease diagnosed?

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

How is congenital von Willebrand disease treated?

There is no cure for VWD. The goal of treatment is to prevent and control bleeding. You may have one or more of the following:

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

What are the risks of congenital von Willebrand disease?

Treatment may cause unwanted side effects. Medicines may cause nausea, vomiting, headache, flushing, fast heartbeat, or seizures. Your body could react poorly to replacement therapy. The new blood cells may attack healthy cells and cause a serious allergic reaction. If congenital VWD is not treated, you may have frequent bleeding. This may cause you to lose too much blood, which can be life-threatening and may damage other organs. Without treatment, your health, quality of life, and ability to function may decrease.

When should I contact my healthcare provider?

  • You feel very tired and weak.
  • You have a fever.
  • You have nausea, vomiting, or a severe headache.

When should I seek immediate care or call 911?

  • You cannot control your bleeding episodes, even after applying pressure.
  • You have difficulty breathing, chest pain or tightness.
  • You have many large bruises in your body, or swelling in your joints.
  • You have difficulty breathing.
  • You have a seizure or faint.
  • You vomit blood or have blood or black stools.
  • You have blood in your urine.

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