I was 15 when I was diagnosed. I went back and forth to the doctor about eight times. I had nausea, headaches, tunnel vision and similar problems. Doctors continued to consider it a viral infection.
My parents took me to an optometrist because they couldn’t do anything with the doctors and wanted to see if needing glasses could mean anything to me. It was the optometrist who told me: “You should go to Addenbrooke’s.” [Hospital in Cambridge] just to check my eyes” because they saw a tumor in the back of my eye but didn’t know what caused it. Then everything turned upside down.
I went to the urgent care eye clinic in Addenbrooke’s where I was sent for an MRI. After the scan I was kept in hospital and a few days later I had surgery as I could see that the brain tumor had caused swelling. Because I had a brain tumor when I was young, I had a blood test done to determine if I had Hippel-Lindau disease. [a hereditary condition associated with tumours arising in multiple organs] which confirmed that I had it.
I didn’t think it would affect me like that at all. I was very young and innocent; nothing like this had ever happened in our family. This was all very new. Apparently my family has since recognized these symptoms and I am now 34 years old and recognize them myself. However, it took me a while to think about it myself and the implications of it all: having a job, a mortgage and a family. Luckily, no one in my family has VHL as everyone got tested after I tested positive.
I didn’t go to school for several months. I was doing mock GCSEs at the time, so that had an impact. Then for three years I had nothing, but at the age of 18 I developed a second brain tumor. However, I graduated from high school and went to university to study fashion.
I then had a nine-year break before being diagnosed with a third brain tumor. Looking back, it seems like everything happened at the same time, but in fact there was a time when I could enjoy my studies and start working, nothing happened.
When I was a teenager, I really didn’t know the impact VHL could and would have on my life with multiple tumors that could come back.
I have a “you only live once” attitude towards life, because if you want to do something – within reason – you just have to do it. My husband has been unwell in the past so I think this is helpful because he understands what this illness can mean for you when I’m tired or I just don’t have enough energy.
Someone recently told me that he was inspired by how you can overcome one obstacle at a time and not necessarily think too much about what will happen in the future. Take each day as it comes. Do one thing at a time.
In total, I had four brain tumors removed. I have about five or six cysts/tumors on my brain, but only one is being closely monitored. At this stage, surgery is not required. It’s about watching and waiting.
I only have one adrenal gland and I suspect pheochromocytoma in it, and I also suspect RCC (renal cell carcinoma) in both kidneys. Everything is controlled. Because of my kidneys, I need to have my stomach checked for six months and then have an MRI of my brain every year.
Due to illness, I had to undergo a full Whipple surgery in 2017. [the removal of half of the pancreas, gall bladder, some upper intestines and a complete “re-plumb” of the digestive system]. This was definitely the hardest thing to recover from. It’s a bit bittersweet because I was really struggling with recovery, but it brought me into contact with the charity VHL UK/Ireland. And this was a woman from a charity who had gone through the procedure herself and was very supportive.
When you see someone who has been through this and see how well he or she is doing in life, you know that you will be fine in the future, but at the moment it is a real struggle. Your digestive system has to start all over again if you get full in any other way.
It didn’t take long, about a year, before I had my left adrenal gland removed via keyhole surgery, which was like a walk in the park compared to the Whipple procedure.
I was very sick during my first three brain surgeries and immediately after surgery I felt much better. During my fourth surgery I had some symptoms for the first time since then, but they were definitely better than before. For example, my right hand is still shaking a little. So this was my first experience with brain surgery while I was still symptomatic.
I work in the F&F clothing department at Tesco where I have worked since leaving university and they have always been very supportive. In that regard, I’m lucky that I don’t have that stress.
Everything I went through was very hard on my family and friends. Nobody ever shows it to me. They are still very strong and have given me a lot of support, but having to go through all these surgeries with me has to take its toll on me. Personally, I know that if something happens to me, I can handle it, but when something happens to my family or friends, it is always difficult for me. So I can only imagine what it must have been like for her to see me go through that.
There is nothing I can do to stop the tumors from spreading. Just wait. Previously, surgery was the only option I had. I am monitoring my health in the hope that it will help.
[On Monday, a drug called belzutifan was approved for use in Scotland [an oral treatment that could potentially reduce the need for risky, life-changing surgeries and help prevent patients developing tumour-related complications. Belzutifan is currently in the Nice review process and so for patients in England currently, the only treatment option is surgery.]
I don’t know much about Belzutifan other than it sounds great. There is a global Facebook group. [for VHL] and I saw people posting results about how successful it was in America. I live in Biggleswade [in Bedfordshire] Therefore, I hope that it will be approved here as well.
Preventing the need for surgery would be huge. I’m not the only one involved in the operation. Since my son is 18 months old, I have to make sure he is taken care of and there is a recovery period afterwards – it’s not just one day where you have to have surgery. Belzutifan has been shown to prevent tumor growth.
At the moment I am lucky that the kidney tumor is not growing. They got a little worse when I was pregnant, but have since calmed down and stopped. Taking the medicine gives you more confidence that the tumors will not grow. My MRI is next month. I’m already wondering what the scan results will show and whether this means further operations.
I had IVF and we had pregenetic testing (PGD) done to make sure my son didn’t inherit VHL. I’ve been through a lot, and I know not everyone in the VHL has gone through it, but my husband and I just didn’t want anyone else to have to go through it if we didn’t have to. In an ideal world we would like to have more children, but this will influence our decision whether we will have more children because of course there is a 50% chance of passing on the VHL gene.
I am now a Trustee of VHL UK/Ireland, focusing primarily on fundraising and seeing first hand the impact it has had on other people. I try not to look too far into the future and concentrate on the present. I almost don’t want to think too much about how this will affect me later in life.
Awareness of this disease has now increased somewhat, but much more can be done to further raise awareness. That’s why I’m doing everything I can to raise awareness and just want to reach people who don’t necessarily know a lot about VHL.
For more information about von Hippel-Lindau disease visit: https://vhl-uk-ireland.org/
Source: I News
I’m Raymond Molina, a professional writer and journalist with over 5 years of experience in the media industry. I currently work for 24 News Reporters, where I write for the health section of their news website. In my role, I am responsible for researching and writing stories on current health trends and issues. My articles are often seen as thought-provoking pieces that provide valuable insight into the state of society’s wellbeing.