Von Willebrand disease is often called a bleeding disease. Most people have blood that clots appropriately after an injury or cut occurs, but in this condition, the platelets, which aid in clotting, don’t work normally. This can lead to longer bleeding time, and in very extreme cases, it may cause death by bleeding out. However there are three types, and several subtypes of von Willebrand disease, and some people won’t experience excessive bleeding with some of the types. There are also treatments and preventatives that don’t cure the disease, but allow many people with it to live a fairly normal life.
As mentioned, several types of this disease exist, and there are also different ways in which people can develop the disease. It is typically inherited in an autosomal recessive manner. Two parents who don’t 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 when the condition can be acquired. People with hypothyroidism, aortic stenosis or other conditions might lose the ability to produce von Willebrand factor, though 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 much treatment. Type 2 may require some treatment and its many subtypes make treatment variable. Type 3 is extremely serious, but may still be successfully addressed with medication.
The variance in types means that symptoms of von Willebrand disease also vary. Even with the mildest forms, women might have heavier and longer periods. Surgeries, large cuts and significant contusions do run a risk of bleeding longer. Type II may have these symptoms and add risk of bleeding in the joints, nosebleeds, bleeding in urine or in stool, and great risk when injuries occur. Any kind of injury in Type II or Type III that causes bruising or bleeding is a medical emergency.
There are many ways to treat this disease. Some medications are used to help slow down the body’s tendency to eliminate blood clots. Nasal sprays or injections of desmopressin are also important, especially in people with the first two types, as this medication stimulates release of von Willebrand factor. Some people might need infused blood clotting factor and women typically need oral birth control pills to reduce heavy periods.
Care must also be taken when wounds occur, and many doctors opt to use special skin glues to quickly stop bleeding. It’s also important to prepare for any surgeries specially, including dental procedures, when people have von Willebrand disease. This doesn’t make it impossible to do a surgery on a person with the disease, it just means doctors must follow a different protocol and watch for signs of excessive bleeding. Women who know they have the disease and would like to become pregnant should consult their doctors for advice, which can differ, depending on type.
Certain things should be avoided when people have more severe forms of the disease. Some medications need to be avoided including drugs like aspirin and non-steroidal anti-inflammatory drugs (NSAIDS). Contact sports might be dangerous. This doesn’t mean a person with this disease should avoid all activities; it’s simply important to stay active by choosing those activities that are less likely to cause serious injury.