The conference in Kochi was wonderful. The city of Kochi is very beautiful, and our hosts treated us to
the best of Japanese hospitality.
Thursday was devoted to basic science, and the researchers shared some remarkable advances in understanding what happens in the cell. VHL is at the cross-roads of all the angiogenic processes in all cancers, and is now implicated also in colon cancer in the general population. People with VHL are not at higher risk for colon cancer, but when a colon cancer starts in anyone, the tumor shows that there has been damage to the VHL gene that unleashes the growth of the tumor. It's good for us in that lots of pharmaceutical companies are focused on VHL research, trying to learn how to control the levels of VHL, or to normalize the consequences of too little VHL protein in the cell.
Friday we heard reports on new learning from genotype/phenotype alignment and much about the amazing new computer-assisted imaging and surgical techniques. Much of the technology comes from Japan, and there were wonderful demonstrations of laparoscopic techniques and microsurgery facilities to improve the outcomes of brain and spinal surgery.
Dr. Lonser from NIH presented the work his team has done on endolymphatic sac tumors, understanding better how they grow and at what point one should move to surgery, to prevent hearing loss. Publication is in the works, and I expect to report on it in the August/September newsletter.
There is also mounting evidence from many parts of the world that it is very important to monitor the central nervous system before symptoms occur, and make sure to operate on a tumor before it becomes too advanced. Dr. Lonser said that most of the tumors he operates on these days are under 3 cm.
Saturday was focused on the families, most of whom were not able to get off work until then. We had translation services from the university, and were able to have very good interaction with them. The families here have not had the benefit of the kinds of meetings we have run in the U.S., so they do not yet understand how best to manage their health. I prepared for them the following summary of the meeting.
Meeting Summary
What happens in the cell?
What does the VHL protein do?
What happens when it is missing?
Some effects are worse when oxygen is low
We will hear more about VEGF and HIF
What does this mean for us?
Beathing exercises are good for managing stress
And also for getting oxygen to your cells
Vitamin C may also be helpful. Too early to recommend, but will not hurt.
VHL and Hearing Issues
There is a tumor that can occur in the inner ear in 10-20% of people with VHL-ELST
Untreated, it can bleed and cause hearing loss
It is important to test your hearing yearly
If changes, test for ELST. It takes a special MRI (not a normal brain scan, they have to look for it)
Treating Kidney Tumors
Goal is to avoid metastatic cancer, AND stay on your own kidney power
Watch and wait, until tumors are near 3 cm.
Freeze or cook tumors to disable them, or remove them.
Laparoscopic surgery
RFA and cryo
Pheochromocytoma
Can occur anywhere from groin to neck
Most often in or near one or both adrenal glands
MIBG or DOPA PET to find them
Remove the tumor but keep the gland
Laparoscopic partial adrenalectomy preferred
How to Get the Best Outcome
Keep your Immune System Strong
Manage your Stress
Go for your scans regularly
Watch for symptoms: hiccups, coughing, tingling in hands or feet
Take action when symptoms are mild, Don't wait for deficits
The keys to staying healthy are early detection and appropriate treatment
We had at least two dozen Japanese doctors in the room through the family question and answer session. There was a panel of eight physicians from all over the world responding to questions, and others listening carefully, learning what the Japanese families are thinking and worrying about. Due to a technical problem, the university translator could only hear a few of the panel members and not the families. One of the family members, B.K., did an outstanding job of on-the-spot translation to and from Japanese to facilitate the conversation.
After the meeting some families had to go quickly to meet their buses or trains, but 16 of us stayed together and had dinner at a Japanese restaurant, sitting on cushions on tatami mats at a low table, served by women in kimonos, with delicious food on large platters along the table. Three of the people there were able to help with the language, and we had a lively and delightful conversation.
People are people, all over the world. The concerns they expressed are the same concerns I have heard all over the world. We were two from Germany, one from Australia, one from U.S., and 12 Japanese. There are some cultural differences, due to the different health care system and medical culture, and to the societal pressures in this country, but with those flavor
variations it was a conversation that might have occurred in any other country.
We felt that we made some strong bonds with the local families, and will continue to share ideas with them. They learned the benefits of being together. People are always shy in the beginning to attend meetings, not knowing what the experience will be like. This was a great experience for all. We all have the same goal: to live well with VHL and manage our health. We look forward to learning from them and with them as their local organization grows.
If you have questions you would like to ask the Japanese families, please send them along and I will prepare an article for September.
One of the goals we had set when we first scheduled this meeting in Japan was to make better connections throughout Asia. We were able to do some very good networking, and now have at least one good connection with Korea and with China. I am going on a vacation trip into China following the Kochi meeting, and will be meeting with some doctors and families in Guangzhou and Beijing. More later!
