I am a 41 year old wife and Mum with VHL. Last year was the worst year of my life thanks to my VHL. In June ’22, I had one of the most grueling general surgeries you can have for a VHL induced pancreas tumor, called the Whipple’s procedure, which involved the removal of half my pancreas, gall bladder, some upper intestines and a complete ‘re-plumb’ of my digestive system which, apart from a very hard, long recovery, left me needing to take medication every time I eat, for the rest of my life. If it had not been removed it could have soon become metastatic, un-treatable and ultimately killed me. Some months later, I had to undergo my second brain surgery and the combination of both these surgeries in the same year had a huge impact on me and my family’s physical, financial, and mental well-being. We are back on our feet now but are left with some ongoing challenges.
Prior to last year, I have had laser eye treatments (lost count how many, maybe 50?), one eye removed, brain, spine and kidney surgeries. My father has VHL and is still alive, my children do not have it, as we used Pre Genetic Diagnosis (PGD) to ensure I did not pass it on. We also needed the help of a friend & surrogate, to avoid repeating the VHL progression that I experienced while carrying my first.
Earlier this year, after some delays, the NICE appraisal process for a new drug called belzutifan (Welireg) began. This drug has already been available to patients in the USA since FDA approval in August ’21. Since then, and during the years of trials prior, it has seen incredible success. Many patients have seen VHL tumors stabilise, reduce in size and even disappear! In the UK, it is not accessible for most patients currently. The only way patients will be able to access it is if the NICE committee recommend it for use on the NHS and as it is very expensive, it is critical that during this process we are able to convince the committee that whist many of us living with VHL don’t let us stop us from living our lives, it can be devastating and have a huge impact on quality of life for both patients and their carers. Indeed, it effects every aspect of our lives from education, careers, relationships, finances, travel and Mental Health.
From a personal point of view, sadly, I have multiple further tumors, currently dormant that could grow at any time. It terrifies me that one day, one of them may be either inoperable or just one surgery too much for my body, which has already endured so much. I can’t put into words what it would mean knowing, I could try this incredible medication should I need it. I never thought I’s see a treatment like this in my lifetime, it really could be a game changer for many of us VHL patients and our carers.
Kat x
