A: If you have no symptoms and you've been told you have VHL, the diagnosis was likely made through DNA testing, or through routine clinical screening. These tests are usually performed because someone else in your family has also been diagnosed with VHL. It may be that this other person has a medical issue, and you're wondering if that same medical problem is in your future.

Nobody can predict your future, but this diagnosis is an early warning for you that your body has a "genetic risk factor," a tendency, that you need to be aware of. With care and sensible precautions you should be able to avoid the worst consequences of this tendency, but it is something you will need to pay attention to in order to stay out of trouble.

There's a tiny little flaw in one microscopic bit of information in the information in your genes, the part called the VHL gene. Everybody has two copies of this information -- one copy inherited from each parent. Each of your parents has two copies each of them inherited from their two parents, and so on. Each time a child is made, only one of those copies is given to each child. So you might have inherited from your mother the one she got from her father, etc.

The flaw in this case is that one of the two copies of your VHL gene doesn't work just right. There is some change in it that causes it not to work. But the important thing to focus in on is: you still have one copy that does work!

If both copies of the VHL gene in a cell do not function properly, a tumor may begin to grow. However, as long as one copy of the VHL gene is functioning properly, you can keep tumors from growing. So your job is to keep that second copy healthy and keep tumors from occurring.

We don't have all the answers on why the second copy sometimes stops working, but we do know some things you can do that will definitely help keep it healthy. If you do get a tumor, it's not your "fault," none of us has total control over these things. But if you follow the guidelines you will do better than if you don't.

Eat a balanced diet with lots of fruits and vegetables
Avoid alcohol, drugs, or other pollutants that may weaken or harm your body
Get a good night's sleep, give your body time to strengthen and repair itself naturally
Learn to manage stress through physical and mental exercise
Follow the preventive medical screening guidelines

You will hear the word "cancer" applied to VHL in this website. VHL is in the family of diseases known as hereditary cancer syndromes. Cancer involves the loss of control over cell division. In that sense, all tumors are in the general category of cancer, since the cells are not needed and shouldn't be there. But unlike "real" cancers, most of the tumors in VHL do not send out cells that invade other tissues in other parts of the body. This invasion is called metastasis (me-TAS-ta-sis), and is usually not a worry in VHL. There is one kind of tumor (hard kidney tumors) which can progress to cancer, and one special kind of pancreatic tumor. These tumors in particular rarely have symptoms until they are well established. Most people in the general population who experience these kinds of tumors don't find them until they are really in trouble. But you have a powerful weapon -- advance knowledge. You can use this early warning to protect yourself. That's why the screening is so very important. Knowing that your body has this tendency to form tumors, it will be essential to keep an eye on things and find them early, long before they have developed enough to be able to go off and invade other tissues. We are learning at what point it is important to get them out so they never metastasize.

We are working to find a medicine that will keep tumors from forming, or keep them small. Meanwhile you and your family will work with your medical team to watch out for problems, deal with them appropriately as necessary, and keep you healthy and strong.