My story has lots of ups and downs, but more importantly I wanted to show what living with VHL has been like for me. It has taken me weeks to write this story, as putting it down on paper has been so upsetting.
I turned 50 a month ago and it has been a very long journey with VHL. Not only is my body tired out, I am also emotionally tired out too. I keep being reminded that life expectancy with VHL is 49-50 years old. My mum is over 70 as are her living siblings. Some have passed away as a direct result of VHL and that’s very scary. Many individuals bounce back, but many others don’t, and no-one realises the impact it has on those of us living with this condition – both physically and mentally.
I was first told I had VHL in 1993 (when aged 20 years), initially following eye problems which led to a retinal detachment, and a brain tumour which was removed. Since then, I have had multiple manifestations in my brain, kidneys, spine and eyes. I’ve had a partial nephrectomy to my kidney, 2 spinal tumour operations, 3 retinal detachments, a cataract removal, 9 brain surgeries, plus gamma knife surgery to the brain, insertion of an Omaya reservoir, VP shunt. With the VP shunt, I had to have an ICP bolt fitted first to monitor the pressure. I have also had Rapid Arc SRS radio surgery (radiotherapy).
In addition, I’ve had so many “procedures” done to my brain and eyes I’ve actually lost count – and that is on top of the 24 surgeries to date, while number 25 is scheduled for my eye in the next month or two.
I currently have a tumour in my spine, several cysts in my kidneys that seem to be growing (a few are more than 3cm) and a couple in my brain that seem stable. However, I have a further brain tumour that is causing problems – It is located at the base of the cerebellum (posterior fossa)- resection of this residual tumour is not currently a viable option, and at best carries significant risks. I had gamma knife surgery to it initially as far back as 2012. It helped and stopped its growth for 7 years before it started growing again and causing problems. I then had an Omaya Reservoir fitted in 2019 to drain the cystic component (followed by a dozen or so procedures to drain the cystic fluid), and then radiotherapy in 2020 to halt the tumour growth. It is now “stable” but how long that will last no one knows.
Any potential future surgery, if required, to remove the posterior fossa and other brain tumours may prove particularly difficult given the quantum of previous neurosurgery. Additionally, I may potentially need further kidney surgery in the next year or two. The spinal nodule I have has grown a bit but is stable I’m told, and I hope it stays that way.
Each day to me is like playing Russian Roulette, where I hope that there is no growth spurt in any of the lesions/ manifestations which would necessitate additional surgery. It is both physically and emotionally draining due to the unpredictability of VHL tumour growth and the varying symptoms.
The array of physical impairments that I have as a direct consequence of VHL is vast, and their impact on my life is immense but the impact has become the “new normal” over time, and to some extent it is like living with garish wallpaper – the homeowner does not realise the wallpaper is so awful, but a visitor sees it straight away.
– I can no longer write (I’m right handed) so my phone is my new best friend. I type with my left thumb now.
– There is nerve damage caused as a function of radiotherapy for posterior fossa tumour- this is distinctly different to constant pain in my left arm caused by spinal surgery nerve damage.
– I also have numbness now in the right side of my face after the radiotherapy.
– My short-term memory is depleted, and I have ‘brain fog’ due to the posterior fossa tumour pressing on brain tissue.
– I also have problems with balance and have graduated from walking with a frame, to very short distances on a good day with a stick and may partner at my side, andneed a wheelchair (which I hate) for anything longer or a route with turns.
– Other problems are my stamina which is non-existent, my sight loss in my left eye, occasional chest pain caused by the VP shunt tube, nausea, my 24/7 pain in my left arm, loss of appetite, tiredness, my self- esteem, fear, anxiety, depression etc…. the list goes on.
On a positive note, I have a great fiancé, family and friends. I’ve also had amazing doctors and nurses. I have a roof over my head and food to eat, so am very fortunate.
There is the hope of a new drug called ‘Welireg’ that’s available only in the US but shows such promising results. It has MHRA approval here but not NICE approval. I hope that it is approved here soon as I don’t think my body can take more surgery. It’s a real struggle daily and no one realises how much the little things in life matter as they are always taken for granted until they are snatched away from us.
I read something yesterday which I find very true- Michael J Fox was talking of Parkinson’s Disease but it is equally true of VHL ‘The message is so simple, yet it gets forgotten. The people living with the condition are the experts’
