Von Willebrand's Disease

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Von Willebrand's Disease

Topic Overview

Picture of red blood cells

What is von Willebrand's disease?

Von Willebrand's disease is a bleeding disorder. When you have this disease, it takes longer for your blood to form clots and you bleed for a longer time than other people.

Normally, when a person begins to bleed, small blood cells called platelets go to the site of the bleeding and clump together to help stop the bleeding. If you have von Willebrand's disease, your blood doesn't clot well because you don't have a certain protein in your blood or you have low levels of it. This protein is called the von Willebrand factor. It helps your blood to clot by helping the platelets stick together.

There are three major types of the disease.

  • In type 1, you are missing some von Willebrand factor. This can cause mild to moderate bleeding episodes, depending on how much von Willebrand factor is missing. About 3 out of 4 people who have von Willebrand's disease have type 1.1 You may not know you have the disease and may not need treatment.
  • In type 2, you have the von Willebrand factor, but it doesn't work as it should. This usually causes mild bleeding episodes, but it can cause moderate bleeding episodes. Type 2 is further divided into classes.
  • In type 3, you don't have the von Willebrand factor or you have a very small amount. This type can lead to serious bleeding episodes, but it is very rare. People who have type 3 disease can develop anemia and can have dangerous bleeding after an accident or during surgery.

Von Willebrand's disease can stay the same or get better or worse as you get older.

Tell all your doctors and other health professionals, such as your dentist, that you have this disease. Doctors need to know about it before you have any procedures, because you are at risk for dangerous bleeding.

What causes von Willebrand's disease?

Von Willebrand's disease usually is passed down through families (inherited). It is the most common bleeding disorder present at birth (congenital), but most cases are mild. Men and women are equally likely to have von Willebrand's disease.

What are the symptoms?

Bleeding a lot is the main symptom of von Willebrand's disease. How severe the bleeding is will be different for each person.

Symptoms of mild von Willebrand's disease include:

  • Frequent nosebleeds.
  • Some bleeding from the gums.
  • Heavy menstrual periods in women (menorrhagia).
  • Bruises that appear for no reason.
  • Heavy bleeding after injury or surgery.

Symptoms of more severe von Willebrand's disease include those listed above and:

  • Blood in the urine.
  • Bruising easily.
  • Black, tarry, or bloody stools.
  • Bleeding into the joints, which causes stiffness, pain, and swelling. This symptom is rare.

How is von Willebrand's disease diagnosed?

Von Willebrand's disease can be hard to diagnose. Some people with mild von Willebrand's disease bleed about the same amount as other people do. You may not notice any symptoms until you bleed a lot after an injury, dental procedure, or surgery.

Your doctor will ask you how often and how much you bleed. If your doctor thinks you may have a problem with clotting, he or she will suggest:

  • Blood tests that measure bleeding time, von Willebrand factor activity levels, or von Willebrand factor antigen.
  • Genetic testing that shows if you have a defect in your von Willebrand factor.

How is it treated?

Treatment depends on the type of von Willebrand's disease you have, how much you bleed, and your risk for heavy bleeding.

If you have a mild type of von Willebrand's disease, you may need to:

  • Avoid non-steroidal anti-inflammatory drugs (NSAIDs), such as ASA or ibuprofen (Advil or Motrin).
  • Take medicine to prevent heavy bleeding if you have an injury, are going to have surgery, or are about to give birth.
  • Avoid blood thinners, such as warfarin and heparin, and antiplatelet medicines like clopidogrel.

If you have severe von Willebrand's disease, you will need to avoid NSAIDs and blood thinners. Treatment may include:

  • Desmopressin medicine, which helps control bleeding episodes.
  • Replacement therapy, which replaces the clotting factor in your blood.
  • Antifibrinolytic medicines, to help stop blood clots from breaking down.
  • Hormone therapy for women, to control heavy menstrual periods.
  • Fibrin glue or thrombin powder, which you place on a wound to help control bleeding.

If you have severe von Willebrand's disease, you most likely will need to take extra care to treat and prevent bleeding episodes. Try to stay at a healthy weight and keep active. This helps prevent bleeding into muscles and joints. Avoid sports or activities where injury and bleeding are likely, such as football and hockey.

Frequently Asked Questions

Learning about von Willebrand's disease:

Being diagnosed:

Getting treatment:

Symptoms

Most cases of von Willebrand's disease are mild. Some people with type 1 von Willebrand's disease have no more bleeding than members of the general population. Mild von Willebrand's disease may not be noticed until you have excessive bleeding after an injury, dental procedure, or surgery. Severe cases (type 3) often are recognized early in childhood because of unusual bleeding, very heavy bleeding, or easy bruising.

Symptoms of mild von Willebrand's disease include:

  • Frequent nosebleeds.
  • Occasional bleeding from the gums.
  • Heavy menstrual periods in women (menorrhagia).
  • Unexplained bruises.
  • Bleeding that lasts a long time after a procedure such as a tooth extraction.

Symptoms of more severe cases of von Willebrand's disease include those listed above and:

  • Blood in the urine.
  • Bruising easily.
  • Black, tarry, or bloody stools.
  • Bleeding into the joints, resulting in stiffness, pain, and swelling. This symptom is rare.

The severity of symptoms of von Willebrand's disease can vary greatly, even within the same family.

A very rare form of von Willebrand's disease called acquired von Willebrand's disease, occurs in some older adults. Symptoms include nosebleeds with no previous history of bleeding abnormalities.

Examinations and Tests

Von Willebrand's disease can be hard to diagnose. If you have symptoms that suggest a blood clotting disorder, such as frequent nosebleeds, easy bruising, or heavy menstrual periods, a doctor will take a blood sample and have it tested in a lab.

Tests for von Willebrand's disease may measure:

  • Von Willebrand factor antigen.
  • Bleeding time.
  • Von Willebrand factor activity levels, using a test such as PTT (partial thromboplastin time).

Early diagnosis of mild von Willebrand's disease allows doctors to know when you may need special treatment in order to help prevent severe bleeding problems. A definite diagnosis of von Willebrand's disease depends on:

  • A finding of low levels of von Willebrand factor in your blood on more than one occasion.
  • A history of excessive bleeding.
  • Genetic testing. It can show a defect in your von Willebrand factor and what type or subtype of von Willebrand's disease you have.

If you have von Willebrand's disease, your doctor may suggest that your family members get tested for it too.

Treatment Overview

Treatment for von Willebrand's disease can vary based on the type of disease, severity of symptoms, or risk of heavy bleeding. If you have a mild type, you may only need home treatment like not taking ASA. At other times, you may need medicine to prevent heavy bleeding when you have an injury, are having surgery, or are about to give birth.

If you have a more severe type of von Willebrand's disease, you likely will need to prevent and treat bleeding episodes throughout your life.

The course of von Willebrand's disease is hard to predict because it may stay at the same level of activity or get better or worse as you get older.

Medicines that increase the von Willebrand factor in your blood and prevent the breakdown of blood clots treat all types of von Willebrand's disease. But there is no cure for this disease.

Treatment for von Willebrand's disease may consist of:

  • Desmopressin to help the body use the von Willebrand factor that the body has already made. This medicine is used to reduce blood loss during a woman's menstrual periods, to lower blood loss during a surgery, and to help control bleeding episodes. Because the effects of this medicine can vary, your doctor will prescribe it on a trial basis to see how much it increases the amount of clotting factors in your blood and to monitor side effects.
  • Therapies to replace the clotting factors in your blood. If you have type 3 von Willebrand's disease, you may be taught to inject yourself with blood-clotting factors. If you can give yourself a shot as soon as a bleeding episode starts, you can avoid losing too much blood and prevent any permanent damage that could be caused by the bleeding. Replacement therapy injections (infusions) may be used with people who:
    • Do not respond to desmopressin.
    • Cannot tolerate desmopressin.
    • Have severe type 2 or type 3 von Willebrand's disease.
    • Have severe bleeding, from an injury or an unknown cause.
  • Antifibrinolytic agents that help prevent the breakdown of blood clots. Antifibrinolytic medicines can be used alone or in combination with desmopressin medicine or replacement therapy to treat mild bleeding. These medicines are sometimes used to prevent bleeding during surgery.
  • Birth control pills (oral contraceptives) or a levonorgestrel IUD to help control heavy menstrual periods in women who have von Willebrand's disease. Birth control pills and this IUD contain hormones that help reduce blood loss during a woman's period.
  • Topical medicine to control very mild bleeding in the mouth or nose. Thrombin powder is a dry powder that you can apply directly on a wound to help it clot. It is very useful during dental procedures.
  • Avoiding medicines that can affect bleeding. These include non-steroidal anti-inflammatory drugs (NSAIDs), such as ASA.

Your doctor may combine some of these treatments, depending on how the disease affects you.

Home Treatment

You can take steps at home to prevent bleeding and stay healthy. Avoid certain non-prescription medicines that can increase the risk of bleeding in the stomach or intestines and can interfere with normal blood clotting. Medicines to avoid include:

  • Non-steroidal anti-inflammatory drugs (NSAIDs), such as:
    • ASA.
    • Ibuprofen, such as Advil or Motrin.
    • Naproxen, such as Aleve.
  • Medicines that contain the ingredient salicylate, which is closely related to ASA. Alka-Seltzer, Pepto-Bismol, and many cough medicines contain salicylates.

Acetaminophen (such as Tylenol) is a much safer pain reliever for people with bleeding disorders, because it is less likely to cause bleeding in the stomach or intestines than ASA or other NSAIDs. But acetaminophen does not reduce swelling and inflammation. Ask your doctor what to do if you have von Willebrand's disease and need to treat swelling.

Tell your dentist and your doctors that you have von Willebrand's disease.

In most cases, you will need to avoid certain prescription blood thinners, such as warfarin, heparin, and antiplatelet medicine like clopidogrel. Wearing a medical alert bracelet is also a good idea.

For type 3 (severe) von Willebrand's disease

  • Try to reach and stay at a healthy weight for your height. Being overweight puts stress on joints and can cause bleeding episodes.
  • Keep active to help keep your muscles flexible and control your weight. This can prevent damage to muscles or joints. To get the most out of exercise and daily activities and to protect your muscles and joints:
    • Warm up with a few minutes of gentle stretching before being active. If muscles are warmed up, they are less likely to bleed from being pulled or torn.
    • Try swimming, bicycling, and walking, which are safe activities because of their low impact on joints and low risk of injury.
    • Avoid football, hockey, wrestling, and weight lifting (with heavy weights), which are not safe activities because they increase your risk of injury that may lead to excessive bleeding.
  • Recognize bleeding episodes. You can often feel when you are bleeding into muscles and joints. Clotting factors should be given as soon as possible after you know that you are bleeding.
  • Learn to inject (infuse) replacement clotting factors in yourself or your children. Children also may be taught by age 10 to inject themselves with clotting factors. Being able to inject at home will help speed treatment and prevent damage caused by excessive bleeding.
  • A "bleeding disorders" resource centre exists in most large hospitals. Learn the phone number to the centre closest to you, and carry it with you.

Other Places To Get Help

Organizations

Association of Hemophilia Clinic Directors of Canada (AHCDC)
30 Bond Street (70 Bond LL)
Toronto, ON  M5B 1W8
Phone: (416) 864-5042
Fax: (416) 864-5251
Email: vogela@smh.toronto.on.ca
Web Address: www.ahcdc.ca
 

The AHCDC is dedicated to ensuring excellent care for persons with congenital bleeding disorders in Canada through clinical services, research, and education.


Canadian Hemophilia Society
625 President Kennedy Avenue
Suite 505
Montreal, QC  H3A 1K2
Phone: (514) 848-0503
1-800-668-2686
Fax: (514) 848-9661
Email: chs@hemophilia.ca
Web Address: http://www.hemophilia.ca
 

The Canadian Hemophilia Society provides information, education, and support services to people with inherited bleeding disorders. It also does research into hemophilia and other bleeding disorders with the goal of finding a cure.


KidsHealth for Parents, Children, and Teens
4600 Touchton Road East, Building 200
Suite 500
Jacksonville, FL  32246
Phone: (904) 232-4100
Fax: (904) 232-4125
Web Address: www.kidshealth.org
 

This Web site is sponsored by Nemours Foundation. It has a wide range of information about children's health, from allergies and diseases to normal growth and development (birth to adolescence). This Web site offers separate areas for kids, teens, and parents, each providing age-appropriate information that the child or parent can understand. You can sign up to get weekly e-mails about your area of interest.


National Hemophilia Foundation (NHF)
116 West 32nd Street, 11th Floor
New York, NY  10001
Phone: (212) 328-3700
Fax: (212) 328-3777
Email: HANDI@hemophilia.org
Web Address: www.hemophilia.org
 

The National Hemophilia Foundation (NHF) is dedicated to the cures of inherited bleeding disorders and the prevention and treatment of their complications through education, advocacy, and research. The NHF has chapters throughout the country and a communications network that brings health professionals and the public the latest news about bleeding disorders. NHF's Web site provides information on the nature, symptoms, and treatments of many disorders.


U.S. National Heart, Lung, and Blood Institute (NHLBI)
P.O. Box 30105
Bethesda, MD  20824-0105
Phone: (301) 592-8573
Fax: (240) 629-3246
TDD: (240) 629-3255
Email: nhlbiinfo@nhlbi.nih.gov
Web Address: www.nhlbi.nih.gov
 

The U.S. National Heart, Lung, and Blood Institute (NHLBI) information center offers information and publications about preventing and treating:

  • Diseases affecting the heart and circulation, such as heart attacks, high cholesterol, high blood pressure, peripheral artery disease, and heart problems present at birth (congenital heart diseases).
  • Diseases that affect the lungs, such as asthma, chronic obstructive pulmonary disease (COPD), emphysema, sleep apnea, and pneumonia.
  • Diseases that affect the blood, such as anemia, hemochromatosis, hemophilia, thalassemia, and von Willebrand disease.

References

Citations

  1. National Heart, Lung, and Blood Institute (2007). The Diagnosis, Evaluation, and Management of von Willebrand Disease (NIH Publication No. 08–5832). Available online: http://www.nhlbi.nih.gov/guidelines/vwd.

Other Works Consulted

  • A plasma-derived Von Willebrand Factor/Factor VIII Concentrate (Wilate) (2010). Medical Letter on Drugs and Therapeutics, 52(1340): 46–47.
  • Friedman KD, Rodgers GM (2009). Von Willebrand disease section of Inherited coagulation disorders. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 12th ed., vol. 2, pp. 1386–1395. Philadelphia: Lippincott Williams and Wilkins.
  • James AH, et al. (2009). Von Willebrand disease and other bleeding disorders in women: Consensus on diagnosis and management from an international expert panel. American Journal of Obstetrics and Gynecology, 201(12): e1–e8.
  • Kessler CM (2008). Hereditary von Willebrand's disease section of Hemorrhagic disorders: Coagulation factor deficiencies. In L Goldman, D Ausiello, eds., Cecil Textbook of Medicine, 23rd ed., pp. 1308–1311. Philadelphia: Saunders Elsevier.
  • National Heart, Lung, and Blood Institute (2007). The Diagnosis, Evaluation, and Management of von Willebrand Disease (NIH Publication No. 08–5832). Available online: http://www.nhlbi.nih.gov/guidelines/vwd.

Credits

By Healthwise Staff
Primary Medical Reviewer E. Gregory Thompson, MD - Internal Medicine
Primary Medical Reviewer Anne C. Poinier, MD - Internal Medicine
Specialist Medical Reviewer Brian Leber, MDCM, FRCPC - Hematology
Last Revised January 28, 2011

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