Von Willebrand disease
Von Willebrand disease
Von Willebrand disease is a condition that involves extended or excessive bleeding. It's the most common inherited bleeding condition.
The cause of von Willebrand disease is a deficiency in or impairment of a protein called von Willebrand factor, an important component in your blood clotting process. In general, it takes longer for people with von Willebrand disease to form clots and stop bleeding when they're cut.
Treatment of von Willebrand disease focuses on stopping or preventing bleeding episodes, typically by using medications. With the right treatment, people with von Willebrand disease can lead normal, healthy lives.
In many people with von Willebrand disease (vWD), the signs are mild or they may be absent altogether. When signs occur, their intensity can vary from one person to another. Von Willebrand disease is often challenging to diagnose in milder cases.
Abnormal bleeding is the most common sign of von Willebrand disease, although it may be present at only moderate levels.
The abnormal bleeding associated with von Willebrand disease may occur as:
People with von Willebrand disease may also experience:
Some people may realize that they have a bleeding disorder only after a surgical procedure or serious trauma in which excessive bleeding occurs.
Signs and symptoms of von Willebrand disease in women
Signs and symptoms of an abnormally heavy period that may indicate von Willebrand disease include:
When to see a doctor
If you're scheduled to have surgery (including dental procedures), make sure your doctor or dentist knows that you have von Willebrand disease, which can increase the likelihood of post-surgical bleeding. Include the information that you have von Willebrand disease when you fill out health-screening questionnaires. Also be sure to mention if anyone in your family has a history of excessive bleeding.
Consider wearing a medical ID bracelet, noting that you have von Willebrand disease, in case you are in an accident and are taken to an emergency room. Also carry a Medi-alert card in your wallet.
The cause of von Willebrand disease is a hereditary defect in the gene that controls von Willebrand factor, a protein that plays a key role in your blood clotting process. When von Willebrand factor is scarce — or not functioning properly because of structural abnormalities — 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 uncontrolled bleeding may persist.
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.
A family history of von Willebrand disease is the leading risk factor. 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.
Von Willebrand disease affects males and females about equally and is present in up to 1 percent of the U.S. population.
Race does not appear to play a role in the disease.
Autosomal dominant inheritance pattern
In an autosomal dominant disorder, the mutated gene is a dominant gene located on one of the nonsex chromosomes (autosomes). You need only one mutated gene to be affected by this type of disorder. A ...
Autosomal recessive inheritance pattern
To have an autosomal recessive disorder, you inherit two mutated genes, one from each parent. These disorders are usually passed on by two carriers. Their health is rarely affected, but they have one ...
Complications of von Willebrand disease may include:
Preparing for your appointment
You're likely to start by seeing your family doctor if you suspect you have a bleeding problem. However, in some cases when you call to set up an appointment, you may be referred to a doctor who specializes in the diagnosis and treatment of bleeding disorders (hematologist).
If you're in the middle of a severe bleeding episode, your doctor may recommend immediate medical care.
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared. Here's some information to help you get ready, including what to expect from the doctor.
What you can do
Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out.
For von Willebrand disease, some basic questions to ask your doctor include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment any time that you don't understand something.
What to expect from your doctor
What you can do in the meantime
Steer clear of contact sports associated with a high risk of bruising or injury, such as football and hockey. If you have any medical or dental procedures scheduled, tell your doctor or dentist about your history of heavy bleeding from minor injuries. If a scheduled procedure is not urgent, reschedule it until after you've been evaluated for a possible underlying bleeding disorder.
Tests and diagnosis
Because many people with von Willebrand disease have very mild signs, the condition can be difficult to diagnose. Some people live for years with the disease before it's identified.
If you have any indication of a bleeding disorder, your doctor may refer you to a blood disorders specialist (hematologist). This doctor will perform specific blood tests to diagnose or rule out von Willebrand disease. These tests may be repeated to confirm that the diagnosis of von Willebrand disease is correct and to determine the disease type.
Classifications of the disease
Specific blood tests your doctor may order include:
The results of some of these tests may not be available for two to three weeks after blood is drawn because the tests are conducted in a specialized laboratory. When these findings are analyzed together, your doctor can make a definite diagnosis.
If von Willebrand disease is present, your doctor may recommend that family members undergo the same or similar tests to determine if this condition runs in your family.
Treatments and drugs
Even though von Willebrand disease is a lifelong condition with no cure, your doctor can treat it effectively. Treatment may vary, depending on the type and severity of the disorder, as well as your response to previous therapy and other medications you may be taking. The most commonly used treatments for von Willebrand disease include:
If your condition is mild, your doctor might recommend treatment only when you're undergoing surgery or dental extractions, or when you've experienced trauma (in an automobile accident, for example).
Lifestyle and home remedies
To reduce your risk of complications from von Willebrand disease, take these steps:
Because von Willebrand disease is 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.
Last Updated: 2011-02-05
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