Von Willebrand's Disease

Von Willebrand's disease (VWD) is an inherited blood disorder that is characterized by excessive bleeding following injury or surgery. While the condition can cause heavy superficial bleeding, von Willebrand's disease can also cause internal bleeding.

Von Willebrand's disease is caused by a lack or impairment of the von Willebrand factor, a protein that is necessary for blood clotting. While von Willebrand's disease is a serious condition, with proper medical care, a person with the disease can live a long and healthy life.

Von Willebrand's Disease: Statistics

Von Willebrand's disease affects both males and females. In fact, the sexes are affected almost equally by the disease.

Though it is the most common inherited bleeding condition, von Willebrand's disease is seen in only about 1 percent of the population in the United States.

Von Willebrand's Disease: Causes

When a person has von Willebrand's disease, he has a defect in the gene that controls the von Willebrand factor. Without properly functioning von Willebrand factor or with an insufficiency of the factor, the blood cannot clot properly after injury or surgery. Therefore, excessive bleeding can result.

In order for a child to be born with VWD, either one or both of his parents must carry the gene that causes the disease. If a parent has the gene responsible for von Willebrand's disease, his child has a 50 percent chance of having VWD.

For a person to have the most severe form of VWD, type three, both of his parents must carry the gene responsible for the condition.

Von Willebrand's Disease: Symptoms

Some people who are born with von Willebrand's disease might not know they have the condition until they are adults. Often, a VWD diagnosis won't be made until a person suffers an injury and experiences heavy bleeding.

Symptoms of von Willebrand's disease can include the following:

• bleeding from the gums
• bleeding that won't stop
• blood in the urine or stools
• bruising
• heavy menstrual bleeding
• rashes.

The severity of a person's symptoms will vary depending on the type of von Willebrand's disease from which he suffers.

Von Willebrand's Disease in Dogs

Man's best friend is susceptible to many of the same diseases as humans. Dogs can be diagnosed with such diseases as diabetes, cancer and von Willebrand's disease.

The primary symptom of VWD in dogs is excessive bleeding. Bleeding from the nose, blood in the urine or feces or excessive bleeding while the dog is in heat are symptoms that should be checked out by a veterinarian. Your vet can do a simple blood test to determine whether your dog has VWD.

Certain breeds are prone to inheriting von Willebrand's disease, including:

• Dachshunds (standard and miniature)
• German Shepherds
• Golden Retrievers
• Scottish Terriers.

Treatments for dogs with von Willebrand's disease are the same as those for humans with VWD. Intravenous infusions of von Willebrand factor will help manage the disease.

Types of Von Willebrand's Disease

VWD can occur in three types. A doctor will use blood tests to determine which of the following a person suffers from:

• Type one is the mildest form of von Willebrand's disease and is caused by a mild decline in von Willebrand factor and factor VIII, a substance that stimulates clotting. Research shows that 75 percent of people with VWD have type one VWD. People with type one VWD tend to suffer from only mild symptoms.
• Type two VWD tends to cause more intense symptoms. In people who have this type, von Willebrand factor doesn't function properly.
• Type three VWD is very rare. People with type three VWD don't have von Willebrand factor and have a deficiency of factor VIII. People with this type might experience bleeding in the muscles and joints and often suffer from severe VWD symptoms.

Type two VWD and type three VWD are rare. Patients with these types may need to seek emergency treatment to prevent life-threatening bleeding following an accident or injury.

Von Willebrand's Treatment

While there is no cure for von Willebrand's disease, there are treatments that can help people with all types of the disorder. Treatment options include the following:

• Antifibrinolytic Medications: Antifibrinolytic drugs prevent blood clots from dissolving. These can be taken before surgery or dental work to prevent excess bleeding.
• Desmopressin: VWD can be treated using a synthetic hormone called desmopressin (DDAVP). This hormone causes your body to release von Willebrand's factor into the bloodstream and, thus, improves clotting. Desmopressin is most often used to treat people with type one VWD and is used in some cases of type two VWD.
• Fibrin Sealants: Fibrin sealants can be applied directly to cuts to prevent bleeding.
• Intravenous Therapy: Intravenous therapy is available to patients for whom DDAVP is not successful and to patients who have type two and type three VWD. In this procedure, the patient receives von Willebrand's factor and factor VIII through an I.V.

These therapies can be used individually or in combination to prevent excessive bleeding. Your doctor will help you determine how to best manage your illness.

Von Willebrand Management

If you have VWD, there are steps you can take to reduce your risk of excessive bleeding:

• Avoid taking aspirin or non-steroidal anti-inflammatory (NSAID) medications. These have a thinning effect on the blood. Other prescription medications can have a blood-thinning effect, so talk to your doctor or pharmacist, especially if you are prescribed a new medication.
• Consider wearing a medical alert bracelet. If you are unable to explain your condition, the bracelet can notify medical personnel of your condition.
• If you have a child with von Willebrand's, let other caretakers in his life know about his condition so that they will know what steps to take in the event of an accident.
• Let your dentist and doctors know about your condition.
• Stay active but avoid activities that could cause bruising (wrestling, football, etc.). People with VWD can generally stay in shape through swimming, walking, hiking, yoga, etc.

Resources

Canine Inherited Disorders Database (1998). What is von Willebrand's Disease? Retrieved September 21, 2007, from the University of Prince Edward Island Web site: http://www.upei.ca/~cidd/Diseases/clinical pathology/von Willebrand's disease.htm.

Foster, Race; Marty Smith (n.d.). Von Willebrand's Disease. Retrieved September 21, 2007, from the PetEducation.com Web site: http://www.peteducation.com/article_print.cfm?articleid=488.

Matsui, William (2006). Von Willebrand Disease. Retrieved September 21, 2007, from the MedlinePlus Web site: http://www.nlm.nih.gov/medlineplus/ency/article/000544.htm.

National Institutes of Health (n.d.). What is von Willebrand Disease? Retrieved September 21, 2007, from the National Heart Lung and Blood Institute Web site: http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_All.html.