For a long time it had seemed to me that life was about to begin - real life. But there was always some obstacle in the way, something to be got through first, some unfinished business, time still to be served, a debt to be paid. Then life would begin. At last it dawned on me that these obstacles were my life. -- Father Alfred D'Souza

Meetings in 2002 - Cleveland, Padua
Travel Assistance from Mercy Airlift
Defeating Depression, adapted from Milestones
Audio and Computer text
VHL in Germany, by Gerhard Alsmeier, Germany
Sigma Phi Gamma Supports VHLFA
New Alabama Chapter, by Linda D.
Triathlon -- Alice completes the Danskin Triathlon

Question: Are people with VHL candidates for transplantation? -- Yes.

Question: There is a general recommendation for a waiting period of two years. Can this be shortened? -- Yes, as long as the kidney tumors removed were small enough (under 3 cm) and well confined, to present a low risk of metastatic disease. Where there are questions about the progress of the cancer, a waiting period of two years is a wise thing to do. If there is a cancer anywhere in the body, the immune suppression required to keep the transplanted kidney will cause the cancer to spread. When in doubt, wait.

Question: If a new kidney is transplanted into someone with VHL, won’t that kidney also be at risk for VHL tumors? -- No. VHL tumors are not carried in the blood to the new kidney. VHL tumors are "neoplasias", new growths, arising from a single cell whose VHL protein is missing. The cell structure of the new kidney is that of the donor, not the recipient, and therefore is not at risk for VHL tumors.

Question: How well do people with VHL do on transplantation? -- A study published in 1997 in the Journal of Transplantation showed that people with VHL do at least as well as people with other conditions. The average age of a kidney recipient with VHL is 20 years younger than people with other conditions, and people with VHL generally lose kidney function abruptly as a result of surgery rather than going through long-term degradation of kidney function. There are continuing advances in the anti-rejection drugs used, and there are experiments with using stem cells from the blood of the donor to accustom the recipient’s body to the donor’s tissue.

Question: The wait for a cadaver donor¹ can take three to five years. How can that wait be shortened? -- The need for organs rises every year, but the number of donors has not risen significantly in the past five years. The best chance of early transplantation and the best long-term outcome is a living related donor. Next is another living donor who may not be related but has the same blood type as the recipient and a satisfactory match. A spouse or a friend can also be a donor. Unrelated living donors give essentially the same results as a related donor, and significantly better than a cadaver organ.

Question: If a healthy relative is an excellent match and is eager to donate a kidney, why all the delays and tests? -- It is essential to assure the donor that everything after the donation is going to be the same as before. The donor undergoes a very thorough medical evaluation, beginning with DNA testing to confirm that this person does not have VHL. If we find anything that poses a potential risk to that individual, even if they might be the best possible match, we can’t do it. The most important thing is to preserve not only the life, but the quality of the life of the potential living donor. The kidney that remains in the donor must be absolutely normal. We have to be able to carry out the procedure successfully with the recipient and also not compromise the donor.

Question: What does it entail for the donor? -- In some cases, the kidney can be removed laparoscopically, through a small incision, minimizing scarring for the donor. In other cases it may entail a 3-6 inch incision and open abdominal surgery. Depending on the surgical approach required, the recovery may take 2-6 weeks.

Question: If a transplanted kidney would not grow VHL tumors, shouldn’t we simply take out both kidneys and move directly to transplantation? Isn’t that a "cure" for VHL tumors in the kidney? -- No, transplantation is not a cure. It is a serious medical path under which you are a patient your entire life, taking an expensive set of medications every day to keep the organ from rejecting. If you can stay on your own kidney power, you will be healthier and have fewer side effects. Transplantation is better than dialysis, but it is almost always better to be on your own kidney power, even if you have only part of one kidney, as long as the risks of metastatic cancer can be kept sufficiently small.

Question: What is the best way to manage VHL tumors of the kidney? -- The keys to managing VHL kidney tumors are to find the tumors early, use laparoscopic cryotherapy or radio frequency ablation where possible, and delay any invasive surgery until the largest hard tumor is approaching the 3 centimeter level. At that point, the preferred treatment is usually a partial nephrectomy using techniques to "scoop out" the tumors, minimize vascular damage and leave as much functioning kidney tissue as possible. It is important to continue keeping a close watch on the kidney, and there will likely be additional tumors within 5-10 years which will also need to be followed and treated appropriately.

Question: Is there anything the patient can do to minimize or slow down tumor growth? -- We know that smoking causes kidney tumors to grow more rapidly. The most important thing is to keep the body strong and healthy so that your body’s normal self-repair mechanisms are working at peak performance. Other than that, we have not yet identified any risk factors that would be under your control.

1. A cadaver organ comes from a person who is brain dead due to an accident or stroke, and who has signed an organ donor card. Once the heart stops beating entirely, the kidney cannot be used.

Credits: Our thanks to Dr. Oscar Salvatierra, Dr. Andrew C. Novick, Loriann Marquardt, and Damon G. for their assistance in the preparation of this article.

As printed in the VHL Family Forum 9:3, September 2001. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.