When you hear the word cancer used in relation to you or your family, you may have any one of many normal reactions, such as fear, confusion, panic, anger and anxiety. You may feel like your life is turning upside down. Others who have not experienced this may not understand what you are going through.
It is important to find the help you need to get you through this. On a personal level, you may want to talk to a family member, friend, religious advisor, counsellor, or join a cancer support group. On a medical level, we recommend finding a doctor who will listen and who should refer you to a group of specialists in different areas (such as an oncologist, urologist, dermatologist, pulmonologist), as well as a genetic counsellor.
Because BHD syndrome is so rare, you may have trouble finding a doctor who knows about BHD-associated kidney cysts and tumours. A well-trained urologist, urologic oncologist, or oncologist may not be aware that BHD-associated kidney cancer is not necessarily treated the same way as ‘normal’, or sporadic kidney cancer. You may be the only person, or family, with BHD syndrome in your area. Even a doctor who sees many people with kidney cancer may never have met anyone with BHD syndrome. That is why it is vital that you know as much as possible, so that you can make the best decisions with your doctors as a team.
It is good to know that the BHD-associated kidney cancer in most cases is manageable and does not spread (metastasise). To date, there are only 5 reported cases of death due to BHD related kidney cancer. 93% of BHD-related kidney cancers reported are chromophobe, oncocytic, or hybrid oncocytic. The metastatic kidney cancer types that led to the BHD-related deaths were clear cell or papillary. Given these statistics, a clear cell or papillary kidney tumour diagnosis may seem especially frightening. However, regular scanning should enable your doctors to catch any cancers early and arrange an appropriate treatment.
This means that the odds for survival are very good. The key is finding the cancer early and working with a knowledgeable doctor. However, one thing is very clear: kidney cancer is a serious disease. Both tumours and cysts may continue to ‘pop up’ after earlier cysts and/or tumours have been treated and removed; it is not known how to stop the growth of tumours and cysts. Consequently, it is very important that you are regularly monitored by a team of doctors to catch any tumours early on.
