VHL Handbook

What you Need to Know about VHL

The VHL Handbook

What you Need to Know about VHL

A Reference Handbook for people with von Hippel-Lindau Disease, their families, and support personnel

Click here for a downloadable file in pdf format

Copyright 1993, 1997, 1999, 2005, VHL Family Alliance
International edition 3.0, Revised, 2005
ISBN 1-929539-05-3

Table of ContentsPreface

This information has been compiled to help individuals with VHL, their families, and other interested people understand VHL. The information presented here is intended to add to conversations with physicians and other health care providers. No brochure can replace personal conversations and personal advice about questions on treatment.

One of our primary goals is to give affected individuals and their families greater confidence in the future. With early detection and appropriate treatment, there is more hope today for families with von Hippel-Lindau disease than ever before. Recent research on VHL and related diseases has led to better methods of diagnosing and treating it. Knowledge about VHL is increasing rapidly through the open sharing of information throughout the world among families, health professionals and the research community.

We acknowledge the important contributions to this booklet of our many collaborators and reviewers, both family members and physicians. Knowledge and effective treatment of VHL has been moved forward with greater speed since 1993 through international cooperation, fostered in particular by symposia:

• Freiburg, Germany, 1994, led by Dr. Hartmut Neumann
• Honolulu, Hawaii, USA, 1996, led by Drs. Y. Edward Hsia, Berton Zbar, and
  J. M. Lamiell
• Paris, France, 1998, led by Dr. Stéphane Richard
• Rochester, Minnesota, USA, 2000, led by Dr. Virginia Michels
• Padua, Italy, 2002, led by Dr. Giuseppe Opocher
• Kochi, Japan, 2004, led by Dr. Taro Shuin

and by several extensive research projects —

• in the United States under Drs. W. Marston Linehan and Edward H. Oldfield;
• in France under Dr. Stéphane Richard;
• in Germany under Dr. Hartmut Neumann;
• and in Japan under Dr. Taro Shuin.

Local language editions of this Handbook are available in French, German, Spanish, Portuguese, Dutch, Italian, Ukrainian, Japanese, Chinese and other languages will be prepared as required.

Revision 3, 2005, updates the clinical information throughout, reflecting the many advances in screening, diagnosis, treatment, and quality of life. It is clear that the best way to manage VHL is to identify issues early, monitor and treat them appropriately with minimal invasion and damage, and focus on long-term health. We look forward to working with you and your medical team.

This text is also available over the Internet, both as a Web service and for download. See www.vhl.org.

Throughout this booklet, words that may be new to readers are printed in italics. Definitions of these and other medical terms related to VHL appear at the back of this booklet. [or click words that are marked like this in the online version] A “sounds-like” spelling is also given for some words.

We will appreciate your suggestions and comments to make future editions of this booklet even better.

Joyce Wilcox Graff, Editor
January 2005

Friendship is born at the moment when one person says to another, "What, you too? I thought I was the only one." – C. S. Lewis

Membership information

Preface

1. What is VHL?

Angiomas, Hemangioblastomas, Cysts and Tumors

What is Cancer?

How Do People get VHL?

Early Detection

General Recommendations for Screening

2. Possible Manifestations of VHL

VHL in the Retina

VHL in the Brain and Spinal Cord

Considering Stereotactic Radiosurgery

Hearing Changes and VHL

VHL and your Reproductive Health

For Men
For Women

Pregnancy and VHL

VHL in the Adrenal Glands

VHL in the Kidneys

VHL in the Pancreas

3. Diagnosis, Treatment, and Research

Diagnosis and Treatment

Genetic Research and VHL

Progress toward a Cure

4. Living Well with VHL

The Healthy Eating Pyramid

Living with Knowing

Family Support

Suggested Readings

Questions to Ask the Doctor

Reminder Calendar

5. Suggested Screening Guidelines

Commonly Occurring VHL Manifestations

Common Treatment Recommendations

Preparing for Pheo Testing

6. Obtaining DNA Testing

7. Medical Terms

8. References

9. Prepared by

10. Tissue Bank: Your contribution to VHL Research

Donor Registration Form

VHL Family Alliance
Thomas D. Rodenberg, Esq., Chairman of the Board
Joyce Wilcox Graff, M.A., Executive Director

Directors:
Altheada L. Johnson, M.S., R.D.
Eric I. Lipp
Thomas A. Lusk
Robert E. Schoenhals
Joseph M. Verdi, Ph.D.
Bruce S. Weinberg, J.D.
Clenton G. Winford II, Ph.D.

Council of International Affiliates:
Gerhard Alsmeier, Germany
Gilles Bohlinger, France
Gilles Brunet, France
Kan Gong, M.D., P. R. China
M. Luisa Guerra, Italy
Vibeke & Richard Harbud, Denmark
Chris Hendrickx, Belgium
Pierre Jacomet, Chile & Latin America
Valerie & Jon Johnson, New Zealand
Jennifer Kingston, Australia
Susan Lamb, Canada
Jill Shields, Canada
M. Shinkai, Japan
Helga Süli-Vargha, Ph.D., Hungary
Hélène Sultan, France
Hanako Suzuki, Japan
Erika Trutmann, Switzerland
Paul & Gay Verco, Australia
Karina Villar, M.D., Spain
Michael Walker, Australia
Mary Weetman, M.S., United Kingdom

The VHL Handbook:
What You Need to Know About VHL
Third edition, Revised, 2005
ISBN 1-929539-05-3
Copyright 1993, 1997, 1999, 2005, VHL Family Alliance,
All rights reserved

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