Von Willebrand disease affects blood clotting and can lead to excessive bleeding, but there are different types and treatments available. It can be inherited or acquired, and symptoms vary. Medications, skin glues, and special protocols for surgeries can help manage the disease. Certain activities and drugs should be avoided.
Von Willebrand disease is often called hemorrhagic disease. Most people have blood that clots properly after an injury or cut, but in this condition the platelets, which help clot, don’t function normally. This can lead to longer bleeding times and, in very extreme cases, can lead to death from bleeding. However there are three types and several subtypes of von Willebrand disease, and some people will not experience excessive bleeding with some of the types. There are also treatments and preventives that do not cure the disease, but allow many people affected to live a fairly normal life.
As mentioned, there are different types of this disease, and there are also different ways people can develop the disease. It is typically inherited in an autosomal recessive manner. Two parents who do not have the disease but are carriers of the gene have a 25% chance of passing on two defective genes to each child. To make matters confusing, there are certain circumstances under which the condition can be acquired. People with hypothyroidism, aortic stenosis, or other conditions may lose the ability to produce von Willebrand factor, although they don’t have the genetic predisposition to do so. In most cases the acquired forms are milder.
Type I von Willebrand disease is the mildest, and some people may never notice it or require a lot of treatment. Type 2 may require treatment, and its many subtypes make treatment variable. Type 3 is extremely serious, but can still be successfully addressed with medication.
The variance in types means that the symptoms of von Willebrand disease also vary. Even with the mildest forms, women may have heavier and longer periods. Surgery, large cuts, and significant bruises all run the risk of longer bleeding. Type II may have these symptoms and add the risk of joint bleeding, nosebleeds, bleeding in urine or stool, and a great risk if injured. Any type II or type III injury that causes bruising or bleeding is a medical emergency.
There are many ways to cure this disease. Some medications are used to help slow the body’s tendency to clear blood clots. Nasal sprays or injections of desmopressin are also important, especially in people with the first two types, as this drug stimulates the release of von Willebrand factor. Some people may need an infused blood clotting factor, and women typically need oral birth control pills to lessen heavy periods.
Care must also be taken when wounds occur, and many doctors choose to use special skin glues to quickly stop the bleeding. It’s also important to specially prepare for any surgeries, including dental procedures, when people have von Willebrand disease. That doesn’t make it impossible to perform surgery on a person with the disease, it just means doctors need to follow a different protocol and watch out for signs of excessive bleeding. Women who know they have the disease and want to become pregnant should see their doctor for advice, which can vary by type.
Certain things should be avoided when people have more severe forms of the disease. Certain drugs must be avoided, including drugs such as aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs). Contact sports could be dangerous. This doesn’t mean that a person with this disease should avoid all activities; it’s simply important to stay active by choosing those activities that are least likely to cause serious injury.
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