Along with the June issue, subscribers to the newsletter will have received the latest issue of the VHL Handbook, What you Need to Know about VHL, basic information to families and medical teams to help people manage their health. The complete text of the Handbook is available on this website as well.

The Handbook is our highest priority project. Before the Handbook, it was nearly impossible for patients to find out the whole story about VHL — and nearly as hard for doctors! Thanks to a wealth of new research and publication about VHL in the past four years, there is a great deal more medical information now available, but the Handbook remains a key starting point for medical teams as well as families.

We are very grateful to the many physicians and medical research teams who have contributed to our learning over these last four years, with special thanks to our Medical Advisory Board, and to all the good questions we have received from members.

In the Handbook you will find screening guidelines for checking people at risk for VHL who do not yet have symptoms, and checking throughout the body for people who may have one or two manifestations of VHL. We have tried to be sensitive to the economics of testing. Nonetheless, there are very real reasons why MRI’s are sometimes called for. The availability of imaging testing gives us the ability to see problems before there are symptoms, and in many cases this is the key to avoiding much more serious problems. If you can see and deal with kidney cancer tumors before they spread to other parts of the body, if you can see the growth rates of brain tumors and locate one that is beginning to grow at a faster rate, you have the opportunity to deal with it before there is higher risk to functions.

There are new sections on pancreas, epididymal cysts, hearing, and pregnancy. There is an expanded treatment of DNA testing, how to go about it, and where to find it. And of course there is a glossary of the terms your medical team will likely use in discussions with you, to help you understand their jargon.

Each individual will probably experience only a few of the issues described, but there is still no way to predict which ones those will be, so it is important to be watchful in all areas. Within a family, a larger number of these issues will arise at some time. Often people find that some issue which they or their doctors had attributed to stress turns up in the handbook. Understanding that it has a physiologic cause, there is more of a chance to fix it, or to keep it from worsening, and at least there is the comfort of knowing that it’s not all in your mind.

Very often people call or write to say that even though it’s hard to hear of all the things that could occur, it’s so very much better to know than not to know! It’s a very great relief to know that somebody understands what’s going on, and that there is progress in finding the best treatment.

It’s a little like playing a video game, where the character wanders around in the twisty little passages of the dungeon, going from room to room, collecting treasure. Then he steps into a dark room. Eery green eyes shine in the darkness, swords flash out of nowhere, and your character dies. But if you had known to pick up a fireball before you entered that room, if you had a map, or if you had known to keep close to the west wall, you could have survived that room and won the treasure.

That’s not unlike having a rare disease like VHL. The Handbook is your map, and we share experiences to help you know, before you go through an experience, how best to survive it and maintain your health.

The Handbook is the beginning, but it’s not the end! We are learning more all the time. Feel free to call or write for additional information, or for the name of some specialists in the area of concern who can consult on your particular case, review the scans, and provide some additional information to your local medical team. Having a more experienced eye looking at the scans, or getting the perspective of a physician who has experience with more VHL patients over a number of years, can make a world of difference in planning successful management of VHL.

As published in the VHL Family Forum, newsletter of the VHL Family Alliance, June 1997