Congenital Von Willebrand Disease
WHAT YOU SHOULD KNOW:
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.
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 caregivers to decide what care you want to receive. You always have the right to refuse treatment.
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.
WHILE YOU ARE HERE:
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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 caregiver will tell you when it is OK to get out of bed. Call your caregiver before getting up for the first time. If you ever feel weak or dizzy, sit or lie down right away.
Intake and output:
Caregivers will keep track of the amount of liquid you are getting. They also may need to know how much you are urinating. Ask how much liquid you should drink each day. Ask caregivers if they need to measure or collect your urine.
- Antifibrinolytic proteins: These are also known as local clotting agents. These medicines help protect clots and hold them in place. Clotting agents may be used to control heavy bleeding during your monthly period. These may also stop bleeding in the mouth, nose, or gastrointestinal tract.
- Birth control pills: Estrogens, a type of hormone among women, may increase VWF levels in your blood. These pills may be useful for women with heavy menstrual bleeding.
- Desmopressin: This medicine boosts the amount of VWF and factor VIII in your blood. Ask your caregiver for more information about desmopressin if you need to use this medicine.
- Fibrin glues: Fibrin glues are also called fibrin sealants. These are tissue adhesive that are made from human and animal blood products. A stable clot made of a blood protein, called fibrin, forms when applied to a bleeding vessel.
- Blood tests: You may need blood taken to give caregivers information about how your body is working. The blood may be taken from your hand, arm, or IV.
- Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.
- Joint x-ray or scan: This is a picture of the bones and tissues in your joints, and may be done to check for bleeding. Joints are the places in your body where two bones meet. You may be given dye as a shot into your joint before the x-ray or scan. This dye will help your joint show up better on the x-ray.
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 congenital VWD, this therapy may also be given regularly every few days to prevent bleeding. If you refuse a blood replacement, your condition may get worse. Ask your caregivers for more information on receiving blood concentrates.
You may be weighed each day. Caregivers compare your weight from day to day to record how much body fluid you have. You can become dehydrated if you lose too much. You can have shortness of breath or swelling in your legs if you retain too much.
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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.