Overview

Von Willebrand disease is a lifelong bleeding disorder in which your blood doesn't clot well. Most people with the disease are born with it, though its warning signs may not show up for years. Some people may suspect they have a bleeding disorder when they have heavy bleeding after a dental procedure or, for women, during a menstrual period.

Most people with this condition inherited it from a parent. They have a faulty gene that causes problems with a protein important to the blood-clotting process.

Von Willebrand disease can't be cured. But with good treatment and self-care, most people with this disease can lead active lives.

Source: http://www.mayoclinic.com

Symptoms

Many people with von Willebrand disease don't know it because the signs are mild or absent. The most common sign of the condition is abnormal bleeding. The severity of the bleeding varies from one person to another.

If you have von Willebrand disease, you might experience:

• Excessive bleeding from an injury or after surgery or dental work
• Nosebleeds that don't stop within 10 minutes
• Heavy or long menstrual bleeding
• Blood in your urine or stool
• Easy bruising or lumpy bruises

Signs and symptoms of von Willebrand disease in women

Signs and symptoms of a heavy period that may indicate von Willebrand disease include:

• The presence in your menstrual flow of blood clots greater than 1 inch (2.5 centimeters) in diameter
• The need to change your menstrual pad or tampon more often than hourly
• The need to use double sanitary protection to control menstrual flow
• Symptoms of anemia, including tiredness, fatigue or shortness of breath

When to see a doctor

Contact your doctor if you're experiencing long or hard-to-stop bleeding.

Source: http://www.mayoclinic.com

Causes

The usual cause of von Willebrand disease is an inherited abnormal gene that controls von Willebrand factor, a protein that plays a key role in blood-clotting. When you have low levels of this protein or it doesn't work as it should, small blood cells called platelets cannot stick together properly, nor can they attach themselves normally to the blood vessel walls when an injury has occurred. The result is interference with the clotting process, and, sometimes, uncontrolled bleeding.

Von Willebrand factor carries an additional substance, called factor VIII, that helps stimulate clotting. Many people with von Willebrand disease also have low levels of factor VIII. This is also one of the substances involved in another inherited clotting disorder called hemophilia. But unlike hemophilia, which mainly affects males, von Willebrand disease affects males and females and is usually milder.

Rarely, von Willebrand disease can develop later in life in people who didn't inherit an abnormal gene from a parent. This is known as acquired von Willebrand disease, and it's likely caused by another medical condition.

Von Willebrand disease has several types:

• Type 1. In this most common form of von Willebrand disease, levels of von Willebrand factor are low. In some people, levels of factor VIII also are low. Signs and symptoms are usually mild.
• Type 2. In this type, which has several subtypes, the von Willebrand factor you do have doesn't function properly. Signs and symptoms tend to be more significant.
• Type 3. In this rare type, von Willebrand factor is absent and levels of factor VIII are low. Signs and symptoms may be severe, such as bleeding into the joints and muscles.
• Acquired von Willebrand disease. This type isn't inherited from your parents. It develops later in life.

Source: http://www.mayoclinic.com

Diagnosis

Because many people with von Willebrand disease have mild signs and symptoms, the condition can be difficult to diagnose. If you have any indication of a bleeding disorder, your doctor may refer you to a blood disorders specialist (hematologist).

To evaluate you for von Willebrand disease, your doctor will likely ask you detailed questions about your medical history and check for bruises or other signs of recent bleeding.

Your doctor will also likely recommend the following blood tests:

• Von Willebrand factor antigen. This test determines the level of von Willebrand factor in your blood by measuring a particular protein.
• Ristocetin cofactor activity. This test measures how well the von Willebrand factor works in your clotting process. Ristocetin, which is an antibiotic, is used in this laboratory testing.
• Factor VIII clotting activity. This test shows whether you have abnormally low levels and activity of factor VIII.
• Von Willebrand factor multimers. This test evaluates the specific structure of von Willebrand factor in your blood, its protein complexes (multimers) and how its molecules break down. This information helps identify the type of von Willebrand disease you have.

The results of these tests can fluctuate in the same person over time due to factors such as stress, exercise, infection, pregnancy and medications. So you may need to repeat some tests.

If you have von Willebrand disease, your doctor may recommend that family members undergo the same or similar tests to determine if this condition runs in your family.

Source: http://www.mayoclinic.com

Complications

Complications of von Willebrand disease may include:

• Anemia. Women who experience heavy menstrual bleeding can develop iron deficiency anemia.
• Swelling and pain. If abnormal bleeding occurs in the joints or soft tissue, swelling and severe pain can result.
• Death from bleeding. Rarely, someone with von Willebrand disease may experience uncontrolled bleeding that can be life-threatening and needs emergency medical attention.

Source: http://www.mayoclinic.com

Prevention

Because von Willebrand disease is usually an inherited disorder, consider having genetic counseling if you have a family history of this condition and you're planning to have children. If you carry the defective gene for von Willebrand disease, you can pass it on to your offspring, even if you don't have symptoms.

Lifestyle and home remedies

These self-care tips can help you manage your condition:

• Switch pain relievers. To help prevent bleeding episodes, talk with your doctor before taking blood-thinning medications — such as aspirin, ibuprofen (Advil, Motrin IB, others) or naproxen (Aleve, Anaprox). Your doctor may instead recommend pain and fever relievers such as acetaminophen (Tylenol, others).
• Tell your doctors and dentist. Let your doctors or dentist know that you have von Willebrand disease before you have any type of surgery, start a new medication or give birth. Also mention whether anyone in your family has a history of excessive bleeding.
• Consider wearing a medical ID bracelet. Make sure it notes that you have von Willebrand disease, which will be helpful to medical personnel if you're ever in an accident or taken to an emergency room. Also carry a medical alert card in your wallet.
• Be active and safe. Exercise as part of attaining or maintaining a healthy weight. Avoid activities that could cause bruising, such as football, wrestling and hockey.

Source: http://www.mayoclinic.com

Risk factors

The main risk factor for von Willebrand disease is having a family history of it. A parent can pass the abnormal gene for the disease to his or her child.

Most cases are "autosomal dominant inherited" disorders, which means you only need an abnormal gene from one parent to be affected. If you have the gene for von Willebrand disease, you have a 50 percent chance of transmitting this gene to your offspring.

The most severe form of the condition (type 3) is "autosomal recessive," which means both of your parents have to pass an abnormal gene to you.

Source: http://www.mayoclinic.com