Von Hippel Lindau syndrome (VHL)

Hi all. Don't know if this is the right place for this - did a search, which brought up nothing.

Diagnosed with this 8 years ago; shan't bore you with the detail, but this is truly a genetic cancer nightmare. My father died of it aged 42 and his mother at the age of 36. It is only the wonders of modern surgery, radiology and chemistry that allows me to write this at the age of 58.

Currently (starting at the top!)

Haemangioblastoma inside the cervical spinal cord. They won't touch this until they have to.

Haemangioblastomas in both eyes. These are lasered as they arise.

Neuroendocrine tumours in the head of the Pancreas; awaiting biopsy results to see what can be done, if anything.

Phaeochromocytomas in both adrenal glands; both surgically removed with their associated glands.

Kidneys - multiple bilateral cysts, pre cancerous for RCC. Awaiting surgery.

I feel that I've managed to keep The Reaper skulking in the bushes for most of the time - just starting to get worried that he's making ground.

My thoughts are with all on here, and their loved ones, who have little hope. My experience, and my message - accept nothing, challenge all their decisions, push all the way. Never tolerate silence as an answer.

Richard.

Parents
  • Thanks both. I suppose that we are all forced to adapt to our new circumstances - 'sink or swim' - and get on as best we can.

    My personal preference is to be told things as they are, if necessary over the phone rather than a face to face meeting. I've noticed a trend with doctors of late not to use the 'C' word, which I find not particularly helpful and more than a bit patronising. It's my body, not theirs, and I'm entitled to know everything that is going on! R.

  • Hi Richard,

    So agree with your first line! It took me a while (my hubby diagnosed with incurable mesothelioma just over a year ago now) but I could have sunk under the total feeling of frustration/anger at what had hit us.As it is we are a year on, still here, still coping day to day, week in week out. Hubby tires easily but does what he can when he can. He chose to be told as much as possible about his condition and his team of medical support put him in touch with a charity which specialises in his illness and the hour with their representative in our own home was extremely helpful in putting things in perspective(and helping with getting financial support as his is industry linked (asbestos) disease)).  This forum helped me so much in gaining control and understanding my feelings (particularly the selfish thoughts which are now banished).  Life is an emotional rollercoaster (even without cancer) and I  have learned to deal with it one step at a time. We still plan for the future but in little steps and rejoice when we achieve our goals albeit small ones. There is no such thing as a normal day, this is our life and we get one go at it.  My perspective of life has taken on a whole new meaning and I have probably changed a lot.  We are in our 37 yr of marriage and its hard watching my once  healthy strong engineer who travelled the world now sleeping more due to lack of energy but I am luckier than some and try to appreciate life on a daily basis. Take care of yourself. Jules

Reply
  • Hi Richard,

    So agree with your first line! It took me a while (my hubby diagnosed with incurable mesothelioma just over a year ago now) but I could have sunk under the total feeling of frustration/anger at what had hit us.As it is we are a year on, still here, still coping day to day, week in week out. Hubby tires easily but does what he can when he can. He chose to be told as much as possible about his condition and his team of medical support put him in touch with a charity which specialises in his illness and the hour with their representative in our own home was extremely helpful in putting things in perspective(and helping with getting financial support as his is industry linked (asbestos) disease)).  This forum helped me so much in gaining control and understanding my feelings (particularly the selfish thoughts which are now banished).  Life is an emotional rollercoaster (even without cancer) and I  have learned to deal with it one step at a time. We still plan for the future but in little steps and rejoice when we achieve our goals albeit small ones. There is no such thing as a normal day, this is our life and we get one go at it.  My perspective of life has taken on a whole new meaning and I have probably changed a lot.  We are in our 37 yr of marriage and its hard watching my once  healthy strong engineer who travelled the world now sleeping more due to lack of energy but I am luckier than some and try to appreciate life on a daily basis. Take care of yourself. Jules

Children
  • Thanks Jules, and I share your philosophy: a step at a time. Live each day for what it might bring. Being selfish? - well, I'm sure we're entitled to a little bit of that from time to time, but it will never solve anything and ultimately drive people away.

    Three people close to my heart died of cancer recently, and each handled it in their own way. An aunt-in-law was diagnosed with metastatic cancer of unknown origin which had settled in her stomach. It was all very quick and all very unpleasant. She even ripped off the cross she had worn round her neck all her life, so bitter was she. At the other extreme, a good friend - terminal with what seemed like metastatic everything - simply pretended there was nothing wrong with him and lived his life as normal until he took to his bed. In the middle, my ex, faced with the inevitable prognosis which she was given, declined all treatment and went on her way. She was a great believer in fate..............  they all had their own characters and beliefs and dealt with their illness in their own ways. R.