Birt-Hogg-Dube Syndrome.
Asked March 7, 2011
Hello Dr. Jaggernauth, five years ago I was diagnosed with a rare incurable cancer in both kidneys known as Birt-Hogg-Dube (BHD) syndrome. I am currently preparing to have my third surgery at the National Cancer Institute. I have asked the doctors repeatedly when will I be placed on a kidney transplant list. The reply is always the same, "it's too soon to talk about that". Well, I think it's high time we talk about it, because there is an increased probability that I will be left with only a half kidney with a 1.5 centimeter tumor in the center of it. My question to you is "Am I a candidate for a kidney transplant, and if so when can I expect to be placed on a transplant list? I forgot to mention that the type of cancer in both kidneys is chromophobe cancer. Thank you in advance for your reply.
Expert answer
Hi, I understand your frustration but you have to remember that a transplant may not be the best option for you with BHD. Although it would take care of the kidneys, you will most likely require chronic immunosupression which in itself could be associated with complications. You will need to talk to a kidney transplant specialist - and they will most likely only do so when your kidneys are near failure or require dialysis. Kidneys are not simply available as spare parts - as for the obvious reason - someone has to die or directly donate them. The NCI may also have a clinical trial available for you. There is a special pathway involved in this BHD and your cancer called the mTOR pathway (mammalian target of rapamycin) and there are 2 commercially available medications that may help you. One is Torisel and the other is Afinitor - and these may also help your BHD too. Talk to your doctor to see if they are willing to treat you with these as viable options.
Let us know how it goes!
Dr. Jag.
Answered March 8, 2011
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