Living with the threat of kidney cancer

Kidney cancer touches people in many different ways. Patients, family and friends, those who have come through treatment and living a full life; its reach is far and wide. Carly Benson, her father, brothers and sister, along with Carly’s three children all live with the real threat of kidney cancer developing in any one of them. This is Carly’s story.

My name is Carly Benson, I’m 38, a mother of three and this is how kidney cancer casts a shadow over my world.
The day I found out I had a genetic disorder that could cause kidney cancer was a day that changed mine and my family’s lives forever; but in a positive way. Because now we know we have the condition and are lucky enough to have scans regularly, unlike some that don’t even know they have the condition.
A few years ago my family and I would not have known what ‘Hereditary leiomyomatosis and renal cell cancer (HLRCC)’ was, now we are trying to raise awareness of something that is very rare, under diagnosed and vey much part of our lives.

My younger brother Aaron (35) suffered from terrible lumps all over his arms, torso and, more rarely, face for years, probably from around the age of 18. He was always in a lot of discomfort and pain with these lumps. I really felt for him and couldn’t imagine what it was like, he hated the way they made him feel and he was very conscious about his body in public. Aaron had been back-and-forth to various different specialists who didn’t recognise what these lumps were. He desperately wanted to have them surgically removed and even thought about getting them done privately. I was very worried and begged him not to as I was sure even a private surgeon might not know what the lumps were and it could have been dangerous for him.
Eventually in 2011 Aaron agreed to go and see a consultant dermatologist at Benenden Hospital in Kent where he was a member and I had started working as a receptionist. The consultant looked at Aaron’s lumps and asked if anyone else in the family had anything similar as it could be related to either a genetic kidney disorder called Hereditary leiomyomatosis and renal cell cancer (HLRCC), which could be serious, or just a skin disorder. This condition was so rare that the consultant said he’d only seen them once before, though he said the chances of having HLRCC were low as there are believed to be around 250 families in the world that have it. He told us that HLRCC can lead to an aggressive form of kidney cancer for which there is currently no cure; it was at this point we needed to face the fact that our family could have it.
I remember Aaron coming home and telling me what the consultant had said and alarm bells started ringing , firstly because I was worried about Aaron and knew that if he had the condition he would need a scan on his kidneys. I was also worried because I had a hard, painful lump on my arm that I’d put down to being a cyst, unbelievably I’d never put two-and-two together; until now! Then, I asked our parents to see if they had any lumps and our father, Dennis, gave me the answer I didn’t want to hear that he had several on his back and like me thought they were cysts.

I went to see my GP (myself and Aaron have the same surgery) and remember feeling totally overwhelmed, in tears and just babbling. I was so worried that if we all had this condition I could have also passed it to my children; April, Charlie and Harrison aged 18,13 and 8. The thought filled me with dread and although I was maybe jumping the gun, I had a gut feeling that we all had this condition, everything pointed to it, also our siblings and extended family could be affected.
My GP was very supportive, but at the same time had no idea what the condition was and had never seen it before. I handed him over the pages and pages of research I had frantically printed off -I did a lot of research over a period of 48 hours with no sleep. I also spoke to some people in a HLRCC support group who were full of information and so helpful. Our GP referred us to Guy’s Hospital in London for genetic testing.
The day we went to London, along with our mother, was a strange one. We were all greeted by a lot of consultants who were very interested in our case because they had never seen the condition before. We were given genetic counselling and then had our bloods taken, dad was tested at a later date.
The three months waiting for the results was one of the worst things; feeling in limbo yet trying to get on with everyday life without thinking about it too much. Unfortunately, myself Aaron and our father all tested positive for HLRCC, this ruled out our mother Pauline as it usually come from *one parent.
I remember having all those thoughts ‘why us?’, ‘what did we do to deserve this?’ ‘we’re so unlucky’ then feeling guilty for thinking like that. In the beginning I remember thinking ‘how are we going to get past this and live a normal life?’, but that was the shock of finding out and adjusting. I believe this is a normal thought process, and if people acknowledge when going through such traumatic news as this it would help speed up the process of coming to terms with it.
So, once we had our results we were able to start having a yearly MRI to check there were no tumours on our kidneys. The process normally takes around 45 minutes as they scan through the kidney at around 3mm slices, using a contrast (dye) injected through a cannula. I had never had an MRI before and felt a little anxious, but it was actually fine.
Once the hospital had our gene pattern the tests were much quicker so next my two eldest children were tested and recently my youngest, also our father’s two sisters and our brother and sister Adam (25) and Jasmine (23).

We were told that there’s a 50% chance of getting HLRCC from a parent, unfortunately we were the wrong side of that 50% and everyone was positive apart from my father’s two sisters, which was a relief as they have nine children and 14 grandchildren between them!
The overwhelming feeling of guilt that I had over the next few weeks was awful. I felt as if this was my fault and that I’d given my children this horrible disease. Slowly I came to the realisation that it wasn’t my fault, it was just one of those things and we just had to get on with it. My father said he felt the same, I reassured him it wasn’t his fault.
The children reacted well to the news as I’d been very open with them and they’d had genetic counselling when they were tested. Harrison’s reaction was ‘yes! I get days off school’ and ‘ I like having an MRI, the bed moves; it’s like a ride’. It’s amazing how resilient kids are!
My children are scanned six monthly alternating between an ultrasound and an MRI, we are currently all looked after by Guy’s Hospital in London, except for Adam and Jasmine who live in Cornwall, so they’re seen there.
Aaron has had a lot of treatment to cut out and laser his tumours and is just about to start a new course of treatment which will run for about

three years, however he’ll never be free of the lumps as they quite often grow back. Another symptom of HLRCC is that women often get uterine fibroids which can grow to the size of a watermelon, with many woman having hysterectomies in their early twenties. I have these too and as such have a scan every year as, but mine are quite small at the moment.. long may that remain!
As of today, we are all fit and well and no one has presented any signs of the kidney cancer, we are all routinely checked and will continue to be checked for the rest of our lives. We are really lucky as we live quite far from the hospital so myself, Aaron and April always have our annual screening on the same day, so we can make it a day out and spend the day in London.
Although it’s scary knowing that if one of us produces the tumours it could be fatal, I am very positive about life and I believe that knowledge

is prevention, so I’m keen to spread awareness as it could save somebody’s life! I don’t think about the condition on a daily, weekly or even monthly basis. I refuse to let it take over and at the end of the day what will be will be. I may not have kidney cancer now but living with such a real threat of it appearing at any time is very daunting, I have to be positive for myself and for my family, so I am going to try not worry about something that may never happen.
I wish everyone living with kidney cancer the best of health and everything good for the future.
Carly Benson xx
*HLRCC is caused by having an alteration (mutation) in one copy of the fumarate hydratase (FH) gene. The FH gene is a section of DNA that codes for a protein called fumarase. Fumarase is an important enzyme needed for the production of energy by mitochondria, which are the tiny organelles inside our cells that produce most of our cells’ energy.