Congenital Von Willebrand Disease
Medically reviewed by Drugs.com. Last updated on Apr 2, 2024.
Congenital von Willebrand disease (CVWD) is an inherited blood disorder that causes heavy bleeding or bleeding that will not stop. CVWD is present at birth. You may not have enough von Willebrand factor (VWF), or it may not work correctly. VWF is a protein in the blood that helps it clot.
WHILE YOU ARE HERE:
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Activity:
At first you may need to rest in bed. You may need to breathe through your mouth or lean forward when sitting if a nosebleed is present. Use 2 to 3 pillows when lying down to help you breathe easier. Your healthcare provider will tell you when it is okay to get out of bed. Call your healthcare provider before getting up for the first time. If you ever feel weak or dizzy, sit or lie down right away.
Intake and output
may be measured. Healthcare providers will keep track of the amount of liquid you are getting. They also may need to know how much you are urinating. Ask healthcare providers if they need to measure or collect your urine.
Medicines:
- 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.
- Fibrin glue is placed directly on a wound to stop the bleeding. It is also called fibrin sealant.
Tests:
- Blood tests give healthcare providers information about how your body is working.
- A chest x-ray is a picture of your lungs and heart. Healthcare providers may use the x-ray to look for signs of infection, such as pneumonia, or to look for a collapsed lung. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
- Joint x-ray or scan pictures of the bones and tissues in your joints may be used to check for bleeding. Contrast liquid may be injected into your joint to help your joint show up better on the x-ray. Tell the healthcare provider if you have ever had an allergic reaction to contrast liquid.
Treatment:
You may need a blood plasma replacement therapy to control or prevent bleeding. This is done by injecting or giving factor VIII concentrate that is rich in VWF through an IV. Factor concentrates help the blood clot and stop bleeding following accidents, surgery, or spontaneous bleedings. In severe CVWD, this therapy may also be given regularly every few days to prevent bleeding. Ask your healthcare provider for more information on receiving blood concentrates.
You may be weighed each day.
Healthcare providers compare your weight from day to day to record how much body fluid you have. You can become dehydrated if you lose too much fluid. You can have shortness of breath or swelling in your legs if you retain (hold) too much fluid.
RISKS:
If congenital VWD is not treated, you may bleed easily and often. Heavy blood loss may be life-threatening or damage organs.
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.© Copyright Merative 2024 Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.
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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