A blog by patient Sally Jenkins, Cardiff, UK

Palliative versus end of life care, we all know they’re not the same, but even so, they’re often used synonymously. Nowhere is the need to distinguish between them more pressing than in the management of neuroendocrine cancers, known as NeuroEndocrine Tumours, or ‘NETs’. Arising from unusual neuroendocrine cells, these NET primaries occur commonly in the digestive system but due to their vague symptoms and often slow growth, they are rarely diagnosed until metastatic. Various effective therapies mean that the bulk of NET patients can live with their tumours for years, sometimes decades, effectively in ‘palliative’ or supportive care (as they will never be cured), but certainly not in constant ‘end of life’ modus.

I’ve had a NET for twelve years now. I have had positive and less positive experiences. Confusion arises when the correct therapies aren’t applied at the right time. And, unsurprisingly, given universally stretched NHS resources, the psychological support just isn’t adequate.

Amongst the general population and indeed some health professionals, cancer‘s reputation is of an acute ‘kill or cure’ disease. However, NETs buck this trend alongside a few other cancers. To achieve an acceptable quality of life, it is vital that patients are cared for by a specialist MDT (multidisciplinary team) composed of, wait for it,  Gastroenterology, Oncology, Endocrinology, Hepatopancreaticobiliary Surgery, Gastrointestinal Surgery, Pathology, Radiology, Interventional Radiology, Nuclear Medicine, CNSs, Cardiology, Respiratory medicine and dieticians! Well-tested and proven therapies exist to alleviate the symptoms, but one size doesn’t fit anyone at all and, as NETs do not fit a standard profile like other cancers, the ‘pathways’ can be extremely complex and tortuous. Every patient needs an individual treatment plan drawn up by a specialist MDT and physician and this needs to be reassessed regularly (usually every 4 to 12-months).

Those are the bare facts, but what do they mean in terms of lived patient experience? Without specialist care, uncontrolled NET symptoms can make life a misery. Think perpetual stomach bug with a side helping of hormonal flushing and greater or lesser abdominal pain.

What often happens when encountering an uninitiated health professional (in secondary care as well as primary care) for the first time, is that ‘metastatic’ or ‘stage 4’ is instantly homed in on with an assumption of ‘end of life’. Careful, gentle tones suited to those not long for this world are then adopted, with a willingness to smooth the road in any short term way possible.   At the other end of the spectrum, sometimes the obsolete term ‘carcinoid’ is dredged up from the mists of long ago student lectures and NETs are classified as not a ‘real’ cancer, hence not particularly serious. In either case, it can take time to establish the patient’s actual needs.

Then there’s the outpatient scenario. Imagine you are a newly diagnosed cancer patient, at your first clinic visit after diagnosis. Months, sometimes years of painful symptoms politely dismissed as likely stress-related (or Irritable Bowel Syndrome) are all of a sudden a thing of the past, your condition has finally been given a name: NET. You’ve never heard of them, until you found out you’ve got them, and Dr Google homework has just added to the confusion, making your head and stomach whirl horribly with anxiety and urgent questions. However, the six weeks waiting for a clinic appointment and today’s hour in the waiting room are quickly forgotten, because at last this agonising wait is over, and you finally have your precious opportunity to talk to someone  in the know. How, then, would you feel if the doctor you were pinning your hopes on, opened with ‘I don’t know much about your type of cancer, can you tell me what you know?’. Such is the general dearth of knowledge in this area that this scenario can be repeated at later clinics and until 3 years ago, this was a regular experience among NET cancer patients in South Wales. Yes, really. I’m not joking.

Acceptable? Of course not! And it’s no-one’s fault, it’s what happens when an uncommon cancer slips through the proverbial net into the waiting room.

Until only a few years ago, patients in South Wales lacked access to a dedicated service, with a consequence of poor patient outcomes. 58% of patients reported having an average of more than five bowel movements a day, with poor quality of life and 19% overall satisfaction rate^{1, 2}. After some vigorous awareness-raising efforts at Wales government level, funds were made available to begin setting up a brand new service, based in Cardiff and led by Dr Mohid Khan, a Gastroenterologist with expertise in NETs. In an amazingly short time, Dr Khan, working with colleagues, set up a new (award-winning) cancer service covering the whole of South Wales, crossing organizational boundaries once thought of as battlegrounds in the health service. This involved improving the MDT meetings, improved communication between referring teams, coordination across complex networks, establishing a specialist clinic dedicated for patients regardless of geography (yet still keeping some care close to home), training CNSs and dieticians, policies to access special diagnostics and therapies (like an unusual PET scan, Gallium-DOTATATE PET). The patient satisfaction rate has improved considerably (now over 95%), with elation at the patient group meetings backed up by evidence suggesting the gastrointestinal symptoms and quality of life have in fact improved³.

Regardless of the availability of specialist expertise and treatment, what about the psychological effects of living with long-term NETs? They can be devastating, and they get worse as time goes on. You never get used to it. My experience was that in the beginning, immediately after diagnosis, the overwhelming emotion was relief. Relief that these symptoms weren’t imaginary or caused by stress! This sense of elation lasted until the hard facts sank in that I now had an invisible disability, which had changed my life forever. However hard I tried, I could never go back to my old ways. That realisation was a ‘sandbag across the back of the neck’ moment, very difficult to cope with and hardly acknowledged.

Next came the acceptance phase. Yes, life had changed radically and symptoms intruded every day into ‘normal’ life but a new normality had been forged, the tumours were ‘stable’ and life still had value. This lasted a couple more years but was gradually eroded by a niggling voice in the back of the head whispering ‘OK they’re stable now, but for how long? Sometime soon that will change’, and then the dread word ‘progression’ appeared.  At this point a heart-in-the-mouth moment crept into the ritual of the 6 monthly scan results – is now the time? But on the whole, life was still OK provided that little voice could be drowned out.

Eventually, the second shoe dropped and the tumours started growing again.  Abdominal pain re-entered my life and the highly effective medication controlling it comes with the side effect of a horrible brain fuzz. Thankfully there’s the option of a year-long course of special radio-targeted therapy (Peptide Radionuclide Receptor Therapy) which will hopefully control the tumour growth but with the isolation of the COVID-19 restrictions, this has been the hardest psychological phase so far.

My story is one of many but all are quite different since NETs are diverse in terms of primary body site, hormones produced (or not), grade and the effect on the rest of the body, like the heart. Thankfully there are now over a dozen specialist NET centres in the UK. For those with access to them, they and NCUK, the largest NET dedicated charity in the UK, provide a world-class patient experience. I thank my lucky stars for the day I encountered a specialist NET consultant. My life was truly revolutionised, after so many years of misunderstanding and misinformation. It felt miraculous.

So when you have a NET patient sitting in front of you, they may look composed, but remember that there’s a lot going on underneath. NET cancer always exacts a cost. Underneath the calm exterior there’s pain, confusion and sadness. Make allowances and if you can’t provide the necessary expertise, please refer on to someone who can.

References:

1 Williams M, Reid K, Lewis J, Sunderraj L, Williams H, Rees DA, Khan MS. Exploring gastrointestinal symptoms in patients with neuroendocrine tumours. ENETS Neuroendocrinology 2016;103(S1):120 R18

2 A Amin K, Gruber MY, Shayan-Arani H, Taylor R, Powell C, Cook K, Benny A, Ng S, Christian A, Khan MS. Improving Diagnosis Times as Part of the Transformation of the South Wales NET Service Neuroendocrinology 2020;110(suppl 1):1-312(D02)

3 Khan MS et al. Neuroendocrine tumours (NETs) – improvements in service provision for Wales. British Society of Gastroenterology. 2019.