In 2000 at the age of 40 my husband Peter Hannan was diagnosed with Neuroendocrine Gastric Tumours. It would be 14 years before we met or talked to another person with Neuroendocrine tumours as it was before the Unicorn Foundation was established and as far as we knew at the time there were no other Neuroendocrine patients in our area. Due to the rarity, lack of funding for research and misconceptions at the time e.g.” Its not a real cancer”, “it’s benign”, “you can’t die from it”, “oh, but you look good”, these tumours were often misdiagnosed or ignored until they became metastatic, at stage 4 and incurable as it was to be in Peter’s case.
Peter was always very fit, played sports, balanced hard work with recreation and could not have grown up in a healthier environment. His grandparents were market gardeners and fresh food was always in abundance including fruit, vegetables, seafood and fresh dairy. He also did not smoke, drink excessively or take drugs.
Our 3 daughters were 11, 14 and 16 years at diagnosis, it was one of the hardest conversations we have had with them as we had just been told he had 12 to 18 months at best. Being the positive optimistic (or just grin and ignore and it will go away) person he is, with all evidence to the contrary, he told them “don’t worry I promise I’m not going to die”. Thankfully after 23+ years, 3 graduations,16ths,18ths,21sts, 3 weddings and 3 beautiful granddaughters later, either through good genes, good luck or good management or all three, he has managed to keep that promise.
For approximately 3yrs before the diagnosis Peter was not well, his energy and endurance were declining rapidly (which others also noticed) and he was having to take excessive amounts of over-the-counter antacids every day for what was thought to be simple reflux, in addition he often experienced sharp pains in his abdomen. Peter saw three different GPs over this time, regular bloods were taken, and physicals were performed ,they were not looking for anything specific. The tests came back normal, he was sent home with a diagnosis of reflux and the potential for gout. One day after finding Peter in the foetal position in severe abdominal pain we once again saw the doctor, a diagnosis of reflux and a pulled muscle was made. Deciding I was not satisfied with the GPs assessment I took Peter back the next day to a different doctor (that had seen Peter before) and asked him to look outside the box, a scan for gall stones was organised.
Instead of gall bladder disease the radiographer discovered multiple spots over the liver ranging in size from 1cm to 5cms, which prompted him to investigate further. The radiographer found what was to be a 12cm+ sized tumour over Peter’s appendix. After several x-rays, US, PETs, MRIs and a biopsy, a diagnosis of Gastric neuroendocrine tumours was made. On the day of the biopsy the gastroenterologist came out to the waiting room to give Peter the results and said “I hate it when young people like you come in with a diagnosis like this” the prognosis is 12 to 18 months at best.
Peter was referred to a hepatic oncologist (there were no NET specialists in Canberra at that time), where we were told “if you were not standing in front of me and I was just looking at your scans I would have said you were no longer alive, I can’t give you a prognosis because you shouldn’t be here” we were also told there was no chemotherapy for neuroendocrine tumours, radiation was not a cure or effective, but they did have a counteractive treatment called Octreotide (Sandostatin) that would help to control the excess serotonin the tumours were excreting but would not slow the growth of the tumour. The only option left was surgery but even that was considered limited for Peter. As we left the oncologists rooms I asked if there was anything we could be doing nutritionally the well-meaning Oncologist said “no go home and live it up”.
When Peter was referred to a surgeon the plan was to remove the primary over the appendix with a right-side hemicolectomy and then to go ‘Cherry picking” (as he called it) into the liver to remove some of the bigger tumours. After a six-hour operation the primary was removed but we were told the liver mets were so extensive that it was futile to remove any as it would make little difference to the progression of the disease and doing so could cause the liver to shut down completely. A liver transplant was out of the question as the rest of his body was compromised with more Mets which we found to be true as time progressed.
We discovered approximately 5yrs later that Octreotide did slow the progression of the disease in some patients and was effective in managing Carcinoid syndrome that some patients suffered with.
Peter went back to work for a time after his first surgery to remove the primary but then developed carcinoid syndrome this included flushing, profuse perspiration and severe diarrhoea bought on by physical exertion, heat, alcohol, some foods and anxiety. This all led to early retirement at 41years of age, very confronting for a workaholic that took immense pride in his work. Despite this Peter continued finding other pursuits to occupy his mind, gardening, photo framing and a small business on eBay.
Over the last 23+ years Peter has endured a right-side hemicolectomy to remove the primary, removal of his lower left mandible due to a tumour in the jaw, 2 hernia repairs caused by the hemicolectomy surgery and 13+ bowel blockages from the disease complicated by the surgical hernia. There are tumours in his skull, thyroid, spine, ribs, hip, pancreas, tumour bulk taking up more than half of his liver as well as lymph nodes in his neck and thoracic area.
The removal of the lower left mandible was due to a tumour that had eaten through the jawbone which three of his teeth were barely holding together. Initially the GP thought it was just a gum boil from a bad tooth and sent him off to the dentist. An xray found it to be a tumour, we were told it would be another type of cancer as Neuroendocrine tumours “did not go to the bone”, another misnomer as upon biopsy it did turn out to be a metastatic neuroendocrine tumour.
A ten-hour operation to remove the jaw bone was conducted and was rebuilt with titanium and the fibula bone from his left leg. Peter was in ICU for 9 days 4 of which he was put into a medical coma, this experience was hard on Peter as due to a large tracheotomy he was unable to speak and for a time he was writing in a language no one could decipher making communication difficult, this led to some misunderstandings and inappropriate treatments.
A nurse in the ICU mistakenly thought Peter was having bowel issues when he was trying to communicate that he had pain in his abdomen and gave him a laxative, extremely uncomfortable for a neuroendocrine patient that has 12+ bowel movements a day. The nurse who administered the treatment in good faith complained to me later that Peter had made a lot of work for him overnight. Peter was terribly upset, all of which could have been avoided if the nurse had had a better understanding of the disease. Probably not because of, but after this operation Peter developed chronic anxiety, which often goes in hand with this cancer, he was prescribed Valium because he cannot take SSRIs due to his carcinoid syndrome.
In our experience the lack of knowledge of Neuroendocrine Cancers and side effects are no longer about not being heard of, or considered anymore due to the hard work Neuroendocrine Cancer Australia has achieved in its extensive dissemination of information and education over the last ten years. It is more about the complexities of the disease which requires a deeper understanding for frontline staff and carers. An intern once organised an emergency late night operation for one of the many complex bowel blockages Peter has had without a proper understanding of the back history and impact of anaesthetics on Carcinoid Syndrome. Thankfully, the anaesthetist met Peter in the operating theatre and Peter was able to explain his limitations, planning and protocols before any surgery should be undertaken for a carcinoid patient. The operation was cancelled by the anaesthetist and after some Nasal gastric tube treatment the issue resolved itself.
Peter has always welcomed any requests to be interviewed when he has been in hospitals for the educational benefit of his experiences with Neuroendocrine tumours.
Fast forward to 2023 and Peter has had nearly all of the treatments available for Neuroendocrine tumours ie. 400+ Octreotide (Sandostatin) monthly injections, Radioactive Sirtec spheres, 8 rounds of Lutate (PRRT) and 24 rounds of CAPTEM oral chemotherapy. All the treatments Peter has had to date have been considered for their therapeutic benefits and symptom control, not for any realistic belief in a regression of his disease, this is due to the extent of the tumours and length of time at diagnosis. Some doctors have been hesitant to treat, as in their view “it would be like punishing him”. Peter continues to defy all odds to the bafflement and delight of all his medical professionals, family and friends and he has been lucky enough that all the treatments have had mostly positive outcomes.
The first treatment was removal of the primary over the appendix which was felt would help slow the disease, we believe it did. Approx 12 months later Octreotide injections to counteract the excess of serotonin were started. There have been ups and downs due to the unavoidable instability when preparing the drug for administration. For various reasons the mixture could flocculate and block the needle sometimes requiring replacement doses and up to 4 attempts to reinject. The impacts from this was a shortfall of the drug causing an increase in symptoms, pain from multiple needle jabs and discomfort while waiting for the next month’s injection or requiring short term rescue needles in the interim. Other than that, the injections have been very effective at controlling Peter’s symptoms, he would have been lost without this treatment.
In 2013 Radioactive SIRT spheres were injected into the liver to embolise and slow the growth of the tumours which it did do very effectively. Interestingly Peter had a radiation burn on his back from this therapy that looked like a sun burn in the shape of an Australia map, this dissipated over time and didn’t cause him much pain just a few weeks of exhaustion and nausea.
Eight rounds of Lutate (PRRT) was given over approximates 6 yrs starting in October 2014 which also had good effect slowing the progression of the disease. Unfortunately, the Lutate didn’t work as well on the last round so it was decided CAPTEM oral chemotherapy was the next step. The Oncologist told us the chemo was limited and we were looking at 6 to 9 months at best before it stopped working all together and was the last treatment available to Peter and referred us to palliative care.
After 24 rounds, Peter is handling the chemo very well. There is tiredness, lethargy, nausea, dry skin on hands and feet and brain fog. All his symptoms from the chemo are very well managed by our oncologist at the hospital we are with. The cocktail of chemo drugs is offset with antiemetics and steroids for inflammation which Peter takes at the appropriate times with food to ward off any severe reactions. He rests when he needs to, occupies his mind with reading, mind games and music. Because of the chemo and a bit of birthday-it is, photo framing and eBay have gone by the wayside but he still potters in the garden for short periods with a little help from his daughters and wife, carrying on the family tradition of cultivating the perfect tomato, spinach, beans, strawberries and so on.
Our message in this story is above all be your own advocate, trust yourself, listen to your body, investigate reputable sources, ask questions until you are satisfied and understand. Also try not to give power to a prognosis, it is self-limiting and pointless. Peter has been told on many occasions there are no more effective treatments. We stopped listening when we got to the 5 year mark and he asked me “shouldn’t I be gone by now?’’, proving the point nobody really knows. Share your story, you never know what you may learn or what a difference you can make in someone else’s day. Above all be kind to yourself.