My name is Lydia, 16 years of age and diagnosed with MEN1 when I was 4 years old. MEN1 is on my dad’s side of the family and was a 50/50 chance I would inherit it. I live in Oxfordshire, UK with Mum, Dad, younger brother (also with MEN1) and 2 dogs not too far from the Oxford University NHS Trust Hospitals who thankfully have a good understanding of MEN problems throughout various departments which I access.
How were you diagnosed and how long did it take?
Nothing much happened for the first 6 years of my diagnosis other than yearly blood tests. However in 2016 when I was 10 years old I was diagnosed with an insulinoma in my pancreas. I began having funny turns at school and home such as dizziness and shakiness in 2015. I had to be careful when I went out, eat lots of snacks and use dextrose tablets to help stop my blood sugars from falling. This was so difficult as I could no longer keep up with my friends and started to lose a lot of time in school. I had to stop doing after school clubs and hobbies I loved as I was just so tired. I wanted it all to go away so I could be just like my friends.
After many blood tests and an MRI it was found I had 3 tumours in my pancreas (1 in the head) 1 in my liver and 1 by my adrenal. The problem at this point was which tumour in my pancreas was the insulinoma. It was decided I would have an angiogram with venous sampling to try to ascertain the insulinoma to decide how extensive my surgery would be. The outcome from this was not completely definitive but the tumours in the tail of my pancreas looked most likely to be an insulinoma.
Finally in early November 2016 I had the tail of my pancreas and my spleen removed but luckily everything went well and recovery was smooth. At last with the insulinoma removed I felt much better. Following further MRI scans it was decided I would require further surgery to attempt to remove the other tumours I needed specialist liver surgery not available in paediatrics in Oxford so I was admitted to Kings College Hospital London for surgery in August 2017. The surgery lasted 10 hours as my pancreas had leaked onto my stomach wall following the previous surgery. They had to remove part of it due to necrosis, as well as removing part of my liver and a lymph node developed into a NET.
What treatment have you had and how did it affect you then and now?
For being 11 years old it was scary to go through especially being in an unknown place and surrounded by unknown people, however recovery went well and I carried on starting secondary school late at the end of October 2017. In December 2017 I went back to Kings for my first Gallium Dotatate PET-CT. In January 2018 I had the terrible news of 12 metatastic tumours in my liver and a pancreatic head NET. We decided along with doctors to take action through treatments as my liver NETS were inoperable. I started Octreotide injections which I absolutely hated whilst the team of doctors looking after me in Oxford pursued the best course of treatment.
Mark Gaze, clinical oncologist at UCLH secured funding through the NHS for 4 treatments of Lutetium Dotatate and in December 2018 I began the treatment at UCLH. I was isolated in the treatment room by myself and had four doses of treatment every eight-twelve weeks. I was luckily given a Hickman line so I wouldn’t have to suffer with needles as I had become very needle-phobic by this point in time. After each treatment dose I had a SPECT scan for the UCLH team to monitor the LuDo uptake in my NETs. This whole experience was fairly overwhelming at the age of 13.
During these cycles of treatment I also had an ovary removed in January 2019 to be cryopreserved for the future. In February 2019 I had a nasty line infection during treatment resulting in spending two weeks in UCLH, removal of the line and insertion of a new one. I also suffered a sudden rise in my calcium levels in early 2019. Following a PET-CT to locate an adenoma between my 2nd and 3rd treatment I had two parathyroids and my thymus removed. I completed my LuDo treatment in July 2019 and had a GaDot PET-CT in January 2020.
This showed a very good response to treatment and for the first time in 4 years I had positive news that all NETs were no longer visible on the PET scan. However in July 2020 following another GaDot PET-CT I had disappointing news that many tumours had returned in my liver. After consultations it was decided that repeat LuDo was my best option in an attempt to treat the NETs. With huge thanks to Mark Gaze I was able to have treatment again following his application for compassionate use, and began the 1st cycle in October 2020.
At this point I had started year 10 at school to begin my GCSEs. As well as feeling ill and tired from treatment I found it incredibly hard to study and with COVID-19 now an issue meant I was incredibly isolated. This series of treatments did not go uninterrupted either due to another line infection during Christmas 2020 requiring removal and re-insertion and then removal of my gall bladder in June 2021 unfortunately by laparotomy following problems with gallstones diagnosed some months earlier. I then completed my 8th treatment dose and I was anxious to know how the treatment had affected the new grown tumours. We already knew the response may not be so good this time due to the LuDo uptake still seen on SPECT scans following each treatment.
A GaDot PET-CT in January 2022 confirmed small NETs remaining in my liver. A further PET-CT in July 2022 remained stable with no progression or new development and so we wait for results from a planned MRI in October this year and a PET-CT in January 2023.
The waiting game and the unknown for the future is the worst but you try to go on with life as normal as you can and push the MEN and cancer to the side.
In 2019 I was diagnosed with a prolactinoma in my pituitary and now take medication to control it and my parathyroid hormone is slowly rising but I try to ignore all of this and plod on.
What impact has the diagnosis had on your personal life?
Despite losing huge amounts of time from school I have achieved the GCSEs I need to start equine care at college in September 2022. My love of horses has been there since a young age but since riding and becoming part of the Riding for the Disabled Association community has been what has kept me going through all of this. The support of charities alongside all of the medical teams has been a life saver by the advice, help, gifts and distractions they give to us throughout my treatments and surgeries.
I suffer with anxiety and depression and had to cope with losing my school and social life but as I have matured over the years with my illness I know now that everything is going to be okay and that I can take the challenges ahead straight on with the help of friends and family.
