Question: When I went to renew my driver’s license this year I was handed an organ donor registration card. I would like to think that when I die someone else might benefit from a few of my "spare parts" -- but I’m not sure whether VHL would rule me out as a donor. I know that my kidneys would not be right for anyone, but what about corneas, skin, heart? What should I do with this card? -- Tania D., Canada

Answer: The best advice is to sign your donor card and ensure that your family members are aware of your desire to donate, and are willing to facilitate the donation when you die.

Each patient is evaluated on a case-by-case basis at the time of their death for all donation options (research and transplantation). The Organ Procurement Organization (OPO) will be called and will evaluate the decedent’s medical condition at the time of death. They will discuss all available donation options with the family (or decision maker) at the time of death.

What can be donated will vary depending on the cause and manner of death. Medical/Social history will also play a role and we would consult with the next-of-kin or designated decision maker to determine medical suitability. Tissue bank and Eye bank criteria vary depending on the bank with which the hospital is affiliated.

Remember tissue criteria is much stricter than organ donation. Tissue is life enhancing ... organ donation is life saving...

A surgeon or patient may choose to take an organ that is less than perfect if the patient’s only other option is to die. With tissue, on the other hand, we want to limit the risks, since the decision to receive a tissue transplant (cornea, bone, skin) is usually not life threatening.

-- Lori E. Brigham, Executive Director, Washington Regional Transplant Consortium,+1 (703) 641-0100 Lori@WRTC.org

Editor’s note: Remember too to register with the VHL Tissue Bank, to donate tissue needed for research. Pre-registration shortens the process of donating surgically removed tissue, or of communicating with the Tissue Bank when someone dies. Even if your tissue does not qualify for transplantation, it will be very helpful for research. Call 877-221-6374 U.S. or see http://www.vhl.org/bank to register or to make arrangements.

Mutation Numbering Scheme

Question: In newer papers there is a new numbering system, where the first amino acid of VHL (codon) is number 1. I understand that the original numbering scheme (with 505 mutations and 712 mutations) was based on the initial cloning of the VHL gene which did not precisely describe the start and stop markers of the gene. Since many families who participated in the early studies know their mutation best by the old numbers, how do we convert from the older to the newer notation? -- Joyce Graff, Mass.

Answer: It is quite simple. If you talk about amino acids (AA), you need to use the formula: old AA - 71 = new AA. If you talk about nucleotides, the formula is: old nucleotide - 213 = new nucleotide. It takes three nucleotides to make one amino acid, so there are three times as many nucleotides as amino acids.

For example, if you talk about 505 (old nomenclature) it is now nucleotide 292 corresponding to AA 98 (292/3). 712 (old nomenclature) is now nucleotide 499 or AA 167 (499/3).

I hope that my answer will help you to understand our new data.

-- Christophe Béroud, Ph.D., Genetics, Necker Hospital, Paris, France, and manager of the Universal VHL-Mutation Database on the internet at www.umd.necker.fr

As printed in the VHL Family Forum 7:4, December 1999. For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.