Report from the Anaheim Meeting

Members from around the country gathered in Anaheim, California, to be together and learn more about VHL.  Our focus for this meeting was on what we can do to manage our health, including two important presentations on nutrition, one at the beginning and one at the end of the day.  Videos and handouts from all presentations may be found at http://vhl.org/videos/2009_anaheim. There is also an audio report of this conference on the Powerful Patient. [See References at the end of this article.]

Nutrition

Haylie suggests that we not just look at food as a way to appease hunger, but that healthy food is the most important medicine that we need to manage our health.  It is estimated that one-third of the cancer in the United States is due to lack of key nutrients and exercise.

As one example, our bodies have T-cells whose job is essentially to function as a spell-checker, to make sure that genes are copied correctly.  If they find a cell that is not in good order, they can instruct that cell to self-destruct and make another healthy cell.  One teaspoon of refined sugar will cut T-cell production in half for a 3-hour period.  One soda has 7-14 teaspoons of sugar. The average American eats 2-3 pounds of sugar each week.  Much of this is ingested from soft drinks and prepared foods. 

“If you ever plan on eating, drinking, breathing or sleeping, you will be subjected to various kind of stress,” Haylie said.  “We don’t want to walk in fear of what can happen, but walk in strength to keep your body strong and ready for action.  We need to learn to manage stress – beginning with healthy foods and exercise.”

She told us that she found VHLFA very inspirational, and applauds us for taking a preventive perspective on the condition.  She has a number of good recipes and food advice on her website at www.hayliepomroy.com, and she offers VHLFA members a 30-minute consultation for free.

Eye Tumors

Dr. Michael Gorin of UCLA spoke with us about managing eye lesions.  He recommends treating all lesions, even very small ones, with laser in order to keep them small.  Once they grow to a larger size, the risk is greater that the treatment will affect the vision, or that the tumor may have accumulated enough fluid behind the retina to cause some detachment of the retina.  A little prevention at early stages can prevent much more involved procedures later.

The exception is the peripapillary tumors near the optic disc or adjacent to the optic nerve.  These are best left alone unless they are growing, as they are nearly impossible to treat without causing harm to the vision.  Some retinal specialists have had some success with photo-dynamic therapy (PDT) used in very small doses, repeated as necessary, but this is still considered experimental.

Research

Dr. James Gnarra of the University of Pittsburgh, Director of Research for the VHL Family Alliance, summarized for us the current state of VHL research.  He presented a series of cartoons that showed the interaction of three key proteins in the cell: VHL, HIF, and VEGF.  When a tumor is beginning to grow, these proteins collaborate to create new blood vessels, a process called angiogenesis, in order to bring oxygen to the growing tumor, to nourish it.  A group of new drugs called “angiogenesis inhibitors” attempt to foil this process and keep the new blood vessels from growing.  Without a sufficient supply of blood and oxygen, the tumor will die.  This process is important not only for VHL, but in nearly all solid cancer tumors.

A complete strategy for combatting VHL will include early detection, better treatment, and also a focus on prevention.  Prevention includes things like quitting smoking, protecting yourself from harmful rays of the sun, clean water, chemoprevention, anti-cancer vaccines, healthy diet, and exercise.  Chemoprevention includes the nutritional advice we have been hearing at this meeting, like eating cruciferous vegetables (broccoli and such), but no one thing is the whole story.  This is a complex problem and will have a complex, multi-pronged solution.

Kids’ Handbook

Melissa Kruger, the lead author of the Kids’ Handbook, introduced the four authors of the book, discussed their strategy, and presented a number of the wonderful illustrations.  Produced by a committee of parents and professionals, the book was illustrated by kids with VHL and their friends.  It is designed for children of all ages and their parents, to help them have a constructive conversation about VHL, and learn to take responsibility for managing their own health. 

The book includes a Foreword by Dr. Anna Muriel, a child psychologist, to suggest some ways that parents might use the book.  The book is in a question-answer format, posing commonly asked questions and presenting simple answers that children can understand.  It works to empower the child with things we can do to stay healthy, both physically and mentally.

Pheochromocytoma

We then viewed an episode of the Mystery Diagnosis television show, in which Malinda S. from Pennsylvania tells her story of sixteen years of symptoms with no diagnosis.  Multiple times she went to the doctor and was sent home with antidepressants or advice to relax, but no careful analysis of her symptoms and no effective advice.  For the first eight years she suffered intense headaches, heavy sweating, terrible anxiety attacks, and increasing desperation that the medical world had no answers for her.

After the first eight years, another symptom began to occur: reactions of intense anger to the tiniest upset.  At this point what had begun as a medical condition was becoming a serious problem in her marriage, and interfering with her ability to take care of herself or her children.  On one occasion, she went to see a new doctor and was sternly told that there was nothing wrong with her.  On the way out, she passed out on the floor of the waiting room.  For “nothing wrong” her symptoms were now intense.  She was down to 85 pounds, all skin and bones.  One day she felt a large lump under her ribcage.  This time the doctor was concerned, and did an MRI, which revealed a mass the size of a grapefruit on her adrenal gland.

Fortunately she found a surgeon who had worked with pheochromocytoma before and knew the importance of “blocking” or medicating her before surgery to neutralize the effects of the pheo so that her blood pressure would remain steady during the surgery.  Similarly, he knew that once he removed the pheo and the hormones it was pumping into her system, her body would need time to “withdraw” from the effects of these hormones and stabilize at its new chemical levels.  He kept her heavily sedated for nearly a week following the surgery as a safety precaution, monitoring her vital signs closely.

Now she is doing very well, is back to work, and her relationships with husband and children are back on an even keel.  All the panic and rage is gone as well as the physical symptoms.  As she says “It is good to be me again.”  Nonetheless, those years of undiagnosed pheo did damage to her heart, which she is still living with.

Her story gave us a good chance to talk about the importance of persisting, even when the doctors discount your symptoms.  If the doctor doesn’t listen, perhaps you need a new doctor.  Perhaps you need to keep a log of your symptoms so that you have a more complete story to present to the doctor, with more information to show whatever pattern there might be to the symptoms.  And if you know you have VHL, remind the doctor to please test for a pheo.  If Malinda’s pheo had been identified and treated any time within the first eight years of symptoms, she would never have gotten to the point of rage, and never have incurred the significant heart damage she is dealing with.

Treating Pheochromocytoma

Dr. Michael Yeh of the Department of Endocrinology at UCLA introduced us to his department, and to the new VHL Clinical Care Center he is heading at UCLA.  While there are a number of physicians at UCLA who have worked with people with VHL, they have always done so one specialty at a time.  Now they are embarking on a team approach, as part of the VHL Clinical Care Centers program.

Dr. Yeh explained the different kinds of tests for pheo, and how they are interpreted to determine whether a pheo is present.  Once we know chemically that a pheo is present, the next challenge is to “localize” or locate the tumor.  In VHL, 80% of pheos will be on or near the adrenal gland.  Twenty percent, however, will be somewhere else in the body.  Imaging tests that can help to find the tumor include: CT, MRI, MIBG, or F-DOPA PET.

CT, MRI, and MIBG are all explained in the VHL Handbook.  F-DOPA PET is new.  MIBG and PET scanning (positron emission tomography) do “functional imaging.”  While CT and MRI give you a map showing where a mass is, functional imaging gives you some information about the cell type.  You are injected with a radioactive tracer that seeks out cells that make adrenaline.  The scan then shows the specific areas where the tracer has concentrated.  MIBG finds about 85% of the pheos present, while F-DOPA PET finds nearly 100%.

F-DOPA PET is not yet widely available in the United States, though it is fairly readily available in Europe.  NIH and UCLA have research programs using F-DOPA PET. 

Pomegranate Juice

Dr. Allen Pantuck, also of UCLA, has been studying the effects of the polyphenols in pomegranate juice on cancer tumors.  In one study, men with early stage prostate cancer and rising PSA levels drank 8 ounces of pomegranate juice each day.  In two years, the time needed to double their PSA levels slowed from 15 months to 60 months. 

Dr. Pantuck’s team is studying the pathway, the sequence of events that occur as cancer progresses, trying to learn how to slow this process.  They have learned in the lab that the polyphenols in pomegranate juice slow the action of NFkb (NF-kappa-beta) in cancer cells. 

Simlarly, in clear cell renal cell carcinoma, the VHL gene is damaged in most kidney cancer tumors.  When the VHL protein is lost, HIF becomes overabundant and turns on NFkb (NF-kappa-beta), which has been associated with proliferation of cancer cells.  Pantuck and associates are studying the effects of the polyphenols in pomegranate juice in inhibiting NFkb and slowing the growth of many kinds of tumors including prostate, kidney, breast, and colon. 

Being Together

When we first met in Kansas City in 1994, we had people in the room who had been diagnosed usually somewhere in their teenage years, through age 50, but always because of some tumor that made itself known and needed immediate attention.  Today we have an increasing number of children diagnosed before or shortly after birth, long before any tumor activity has begun.  Early diagnosis gives us the opportunity to do more with prevention than ever before.  We look forward to learning together how to prevent tumors, or slow down tumor growth.

As always, in addition to all our wonderful speakers, the biggest benefit was simply being together, sharing stories and hugs, and knowing we are not alone.  Brian N. participated with Joyce in a Powerful Patient radio show about the experience of attending the conference.  We hope that you will join us June 19, 2010, in St. Louis, Missouri, for the the 17th Annual VHL Family Alliance Conference.

Report from the conference (audio):
http://powerfulpatient.org/archive/2009_anaheim 
Recordings of all talks and copies of the handout sets:
http://vhl.org/videos/2009_anaheim

As printed in the VHL Family Forum 17:3, October 2009. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.