Along with this December 2004 newsletter we are sending a new edition of the VHL Handbook. The online version has been updated to include this new text, and a downloadable file has been put on the internet. Additional paper copies may be purchased (click here for ordering information).

This booklet is designed for use by patients and their local doctors, helping you build your own healthcare team. It provides a practical summary of the scientific literature and clinical studies on VHL. In the back of the book is a bibliography of leading articles about VHL, to give the physicians a quick guide to the most important articles. There is also a glossary of the medical terms to help patients learn the language that their medical teams will use.

The 2005 Edition of the VHL Handbook, What You Need to Know about VHL, has been significantly updated, incorporating what has been learned about VHL since the previous update in 1999. It also includes what this community has learned — the experiences you have shared with us have significantly contributed to our collective learning! Thank you!

The information about endolymphatic sac tumors has been completely changed.

The section on Reproductive Health has been significantly updated, especially the part for women. There is still no practical way to distinguish for sure before surgery between a benign cystadenoma and a cancerous tumor, except through close observation of its behavior. We are working with the ovarian cancer research community to find a better test. Even in the general population, 90% of the tumors in this region removed from pre-menopausal women are benign. That is good news — but only after surgery! Everyone needs a better way to tell for sure whether surgery is really needed.

There is a new section on Pregnancy and VHL. We still do not have data to show whether tumor growth is spurred by pregnancy, but some of the bodily changes that occur during pregnancy can certainly make things worse, at least for the duration of the pregnancy. It is very important to have a thorough checkup before and after, so that you and your medical team have the information you need to protect you. If you are already pregnant, read this section and get a check-up right now.

Recommendations for in-depth discussion of stereotactic radiation have not changed, but have moved into the body of the text, since some people were not finding them in the back of the booklet. Anyone considering stereotactic radiation should definitely read this section and have the suggested conversation with the medical team. If you don’t, you are at significant risk that the treatment may go wrong. Once they give you the radiation, they can’t take it back, so you really MUST have this conversation BEFORE treatment.

Recommendations for treating adrenal tumors stress the preference for laparascopic partial adrenalectomy wherever this approach is available and appropriate. Even if this is the first adrenal gland affected, there is significant risk of an adrenal tumor on the other side in future, so care should be taken to conserve as much functioning cortex as possible.

We have a better understanding today of paragangliomas (pheos that occur in other parts of the body) and better ways to test for them. This is still a difficult area, and a new international consortium is being formed, scheduled to meet for the first time in fall 2005, to make better progress in standardizing the testing and interpretation of the results, and in finding the location of these tumors.

Experience with kidney tumors over the last several years has confirmed the reliance on the guideline of leaving kidney tumors until the largest tumor approaches a size of 3 cm. Radio Frequency Ablation has proven effective in treating tumors that qualify for this less invasive approach.

Suggestions for screening children have changed considerably as we have identified more young children with VHL through DNA testing, and are having more experiences with early affects. Issues do crop up in children under the age of 12, and some level of screening is advised beginning at ages 1-2, to catch and treat or monitor early issues as appropriate.

The section on Nutrition has been enriched with expertise from dietitian Altheada Johnson, Gale Lugo, and the Harvard School of Public Health. Living Well With VHL has been enriched by Dr. Gary L. Wood of Tampa, Florida, a clinical psychologist who has VHL.

We are very excited about the new Handbook, and hope that you will find it useful. Your feedback is always important in shaping the next handbook.

Special thanks to our Medical Advisory Board members, and to the many additional physicians and researchers who provided key information to enrich this booklet. Thanks too to the many VHLFA members who contributed their stories and reviewed sections to add their important perspective.

Seven volunteer translation teams are working to create local versions of the Handbook in French, Spanish, Dutch, Italian, Japanese, Chinese, and Ukrainian. Announcements will be made on the language-specific websites as soon as these versions are ready. If you are interested in helping to create another language edition, please contact editor@vhl.org Thank you!

As printed in the VHL Family Forum 12:4, December 2004. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.