I once wrote a post about patient stories, in particular the ones I receive in my private messages. The headline was “The shock effect never wears off“. But none have been more shocking than the one I received 2 weeks ago.
This is a story about someone who is a private person but felt the need to reach out to me about their diagnostic experience. This person wanted to talk about it, but in private and I was happy to listen. I was so moved by this story, I persuaded this person to let me tell it here whilst retaining their anonymity. Hence referral going forward as ‘Patient E’. I just felt that someone somewhere might learn something very important.
The Story of ‘Patient E’
In December 2018, Patient E was told ‘Stage 4 Pancreatic Cancer’ and had 10 months to live. Chemo was to be attempted in January in an effort to extend life but in the meantime was told to spend Christmas for the last time with the family, a spouse and 3 young children. On 2nd January, Patient E was then told (with apologies) it was actually a ‘Neuroendocrine Tumour with a pancreatic primary’. The only good thing about this story so far, is that they told the children nothing over Christmas. “Why worry happy little people” was the bit of the story which brought out my tears.
Initially I was quite angry this could happen to anyone but I was then calmed by Patient E who now had fresh hope and optimism, perhaps realising that there were possibilities for many more years with the family.
So why do these things happen? Apart from the serious communications lapse by the hospital, this is another example of the problems we as a community face with the anatomical approach many doctors take with cancer, with some even describing a pancreatic Neuroendocrine Tumour as a type of Pancreatic Cancer (this happens with other parts of the anatomy too).
Patient E is not alone, I once wrote a story about rock star Wilko Johnson (of Dr Feelgood fame) who was given a very similar prognosis. However, Wilko was in the later stages of his life and decided instead of undergoing gruelling chemotherapy, he would forego any treatment and tour with his band in the final 12 months of his life and …….. make an album with Roger Daltrey of The Who. It wasn’t until someone enquired why he wasn’t dead after 12 months, that they re-checked and changed the diagnosis to Neuroendocrine Tumour with a pancreatic primary. Read the whole story here.
I wonder how many other times this has happened.
Neuroendocrine Tumours with a pancreatic primary (pNET) is a totally different cancer to Pancreatic Cancer. With Pancreatic Cancer, most people die, quite the opposite with pNETs where most people live. Read more about the main differences here.
I’m grateful to Patient E for contacting me to let me know that my blog and my other support sites have been helpful in the transition from despair to hope since finding out the correct diagnosis on 2nd January. I do hope Patient E will keep me updated.
OPINION – nothing in here should be taken as advice from the author.
On paper, surgery remains the only potentially ‘curative‘ option for Neuroendocrine Tumours (NETs) but there are stage, grade and anatomical constraints to that opinion. Many people get ‘twitchy’ about any inference of the ‘C word’ (cure) but our most eminent NET specialists use the term frequently including in the major treatment guidelines.
I use the word ‘curative’ with some reservations because for many who are diagnosed at an advanced stage, surgery will not cure but will debulk or cytoreduce as much tumour as possible in order to palliate symptoms and improve quality of life. This is a big deal because NETs is one of a small number of cancers where debulking surgery can often provide a survival advantage for metastatic cases. One of the reasons it’s a big deal is because with more aggressive cancers at an advanced stage, surgery just might not be offered. It follows that surgery is most likely adding to the fairly decent NETs survival statistics, including for those with metastatic disease at diagnosis. More on this below.
That’s a fairly simplistic explanation on behalf of surgery. However, as we all know, nothing in Neuroendocrine Cancer is simple. There are always a number of factors involved and every decision can in some way be on an individual basis. There are guidelines for treatment of most types of NETs but ……. they are just that – guidelines. NET Centres and NET Specialists are encouraged to use these guidelines, for example, a European Centre of Excellence has ENETS Guidelines. There is a North American equivalent set published by NANETS and NCCN have a decent complementary set. The UK and Ireland guys (UKINETS) also published a set although many UK centres are ENETS accredited.
Whether to cut or not to cut (or watch and wait then cut if necessary) and the sequencing of treatments is a really difficult issue for NET specialists. I quite liked watching these two video clips and they cover this issue quite nicely including some interesting abdominal challenges in surgery from known NET Specialists – these short video sessions are highly recommended:
a. Risk Stratification and Management of NETs – click here
Surgery can sometimes be a tough call (……to cut or not to cut?)
It is an area where I have some sympathy for physicians and surgeons who sometimes have tough decisions to make. Surgery is risky, particularly where people are presenting in a weak condition, perhaps with very advanced disease, secondary illness and comorbidities. I also suspect age is a factor (I was surprised to find myself considered ‘young’ at 55). Physicians and surgeons need to weigh up these risks and the consequences of the surgery against a ‘watch and wait’ or alternative non-surgical approach. This would normally be discussed via a ‘Tumor Board’ or Multi-Disciplinary Team (MDT) meeting. However, and although imaging helps, the situation is not really 100% clear until the surgeon ‘gets inside’. Remember, all physicians and surgeons are bound by the ‘Hippocratic oath’ of “Do no harm“. Sometimes with NETs, it’s a tough call not only before they go inside but whilst they’re inside.
Surgery should be a carefully considered treatment (…..think before cutting?)
I read many stories from many different parts of the world and I also hear them from people who contact me privately on a daily basis. Some of them are perplexed why they are not receiving surgery and some are not entirely happy with the surgery they received. Many are perplexed by different advice from different doctors. I find it very difficult to respond to many. My most frequent answer is “ask your doctor” but I’m normally pretty helpful with the sorts of questions to ask.
One thing which tends to surprise people is speed – or lack of it! With lower grade NETs, the extent of the tumour (stage), its metastases, histological grade and secretory profile should be determined as far as possible before planning treatment. I like to remind people that in 2010, it took from 26 July to 9 Nov before my body saw a scalpel. With Grade 1/2 well differentiated NETs, you can often get away with that gap. Sometimes when you are diagnosed with NET, it’s a case of ‘hurry up and wait’.
Back to the guidelines, of course most people will probably fit reasonably well into the relevant guidelines flow chart. A very generic example here (not for active use please, your area may have an alternative based on availability of treatments etc):
If you search long and hard, you will find articles about whether to “cut or not to cut”. Not just a dilemma for NETs but also for many cancer types. During my research, I found there’s some overlap between this conundrum and the issue of “overdiagnosis”. By “overdiagnosis”, I mean the unnecessary declaration and treatment of something which would probably not harm a person whilst they live. This is a bit of a modern phenomena as diagnostic tools and screening programmes become more sophisticated and more sensitive …..something to consider with Ga68 PET scans as they are more widely used. If you search for ‘overdiagnosis’ you will see many articles, in particular (and as an example), with many Thyroid diagnoses. In another example, I read an article about Rectal cancerwhere the author suggested a ‘wait and see’ approach might be better for most. Worth adding at this point that many autopsies show up NETs in areas such as the appendix (…..more often than you think) – check out my article “Benign vs Malignant” and The Invisible NET Patient Population. When I attended ENETS 2017 and 2018, I heard many ‘experts’ talk about conservative approaches. However, I also heard many talk about aggressive approaches. Another term I see a lot is “one surgeon’s inoperable is another’s operable”.
Timing of Surgery (……to cut now, to cut later?)
Following on from the scenario above, timing of surgery can be another factor in a ‘watch and wait’ situation. I guess this might be something in the back of the minds of more cautious doctors when faced with a rather indolent and very slow growing Neuroendocrine Tumour. For some this can be a sensible thing – ‘kicking butt’ in a surgical context is sometimes the wrong approach. The worry is that if they are not a NET specialist, they may not fully understand the vagaries of neuroendocrine tumor behaviour (i.e. they all have malignant potential – WHO 2010/2017). We’ve all heard the stories of people being told it’s not cancer, right? Please note my article Benign vs Malignant. However, you may be interested in this post from someone who is one of the most experienced NET surgeons on the planet. Dr Eric Liu talks quite candidly about the ‘timing’ of surgery suggesting a ‘watch and wait’ approach in certain scenarios.
Of course cutting now might actually be a pre-emptive measure. For example, if physicians can see a growth which is critically placed close to an important structure such as a blood vessel or the bile duct or bowel. Even if the disease cannot be cured, removing the tumour may prevent problems in the future by removing disease from key areas before the vital structure has been damaged or blocked. For example, my surgeon conducted a high risk operation on some desmoplasia (serotonin fibrosis) which had encircled my aorta and cava almost occluding the latter. There’s an excellent surgery pamphlet from NET Patient Foundation and I strongly recommend a read as it’s an experienced surgeon’s approach to surgery with NETs (actually written by my own surgeon Mr Neil Pearce!). Click here to read it.
One NET centre in USA has published very detailed surgical statistics indicating that surgical cytoreduction in NET patients has low morbidity and mortality rates and results in prolonged survival. Their conclusion went on to say “We believe that surgical cytoreduction should play a major role in the care of patients with NETs”. You can read the extract from this document by clicking here. Authors: Woltering et al.
Was Steve Jobsa smart guy who made a stupid decision when it came to his health? It might seem so, from the broad outlines of what he did in 2003 when a CT scan and other tests found a cancerous tumour in his pancreas. Doctors urged him to have an operation to remove the tumour, but Mr. Jobs put it off and instead tried a vegan diet, juices, herbs, acupuncture and other alternative remedies. Nine months later, the Neuroendocrine Tumour had grown. Only then did he agree to surgery, during which his doctors found the cancer had spread to his liver. The rest is summarised in my article Steve Jobs.
This is a difficult subject and no one size fits all. Treatment for NETs can be very individual including surgery. I guess you need to be comfortable with your team. I was lucky, in that I lived close to a NET Centre. I was referred to their surgical team once my staging and grading were complete and I was stabilised on somatostatin analogues (carcinoid syndrome under control). I realise it’s difficult for many but I always say to people who make contact, it’s best if you can be seen by a NET centre or an experienced NET specialist – at least be guided by one if not possible or practical. Personally, I think the surgeon’s experience in dealing with NETs is really important. But even experienced NET centres/specialists have to make tough calls.
You may benefit from my 10 Questions article which also has links to NET Specialists.
There’s a saying that the patient is the most underused person in healthcare and I think there’s a lot of truth in that. However, I would suggest with Neuroendocrine Cancer, it’s less true than for many other cancers. There are so many NET Cancer patients out there who know quite a lot about their cancer, and in some detail. Even the great Dr Liu once said that NET Patients frequently know more about NET Cancer than their doctors.
If you go onto Twitter, if you go onto Facebook, if you read newspaper stories, you will find cancer patient stories in abundance and they will normally be patients diagnosed with the big 4 cancers. This is not surprising as these tend to affect more people. However, the ratio of NET Cancer patient stories still does not seem to be right. I’m not ‘dissing’ breast, lung, bowel and prostate cancer patients, all credit to them for pushing their cancer awareness – respect!
I truly believe that patient stories, whether they are written, presented live or recorded for mass media, are an extremely valuable tool in spreading awareness of NET Cancer. A ‘human being’ talking is a thousand times more potent than the endless stream of ‘memes’ and cartoons that seem to pervade our community – one reason why I don’t use them on my own site. It’s also the reason why I always jump at the opportunity to tell my story, because it’s real, it’s factual and I’m sensing an increasing willingness from the medical and healthcare communities to use patients in this way. Quite right too, patients have a lot to offer.
I’ve been video’d several times in the past 12 months and one day you might actually get to see those, there are some contractual reasons why I cannot yet share them with you. It’s quite a scary thing to do and I found it mentally exhausting – but very worthwhile.
I was therefore delighted to find this recently published group of videos from Cure Connect. Within the clips, there are 2 patients stories, one Pancreatic NET (pNET) and one Carcinoid and they are interspersed and integrated by input from NET specialist Dr. Reidy-Lagunes (a very knowledgeable and enthusiastic speaker). Each clip is only around 5 minutes long so not too taxing. The pNET patient, Michael, is a great supporter of my blog and one of the first NET patients I met on twitter. I’m very thankful to him for alerting me to the videos. Dr Reidy-Lagunes is fast becoming a ‘fav’ of mine and I note she emphasises some of the things I’ve been consistently saying in my blogs; i.e. this cancer can be treated and it’s not as rare as people think.
Another bonus is the addition of Carcinoid Cancer Foundation (CCF) and my friend Grace Goldstein. CCF is the largest and most respected NET Cancer organisation on the planet and Grace works tirelessly to spread awareness and help patients including me! CCF was the first site I found and remains my go-to site today.
Well done Michael and Brenda. Thanks also to Dr. Reidy-Lagunes, Grace Goldstein/CCF and Cure Connect for once again highlighting our cancer.
Most people have perceptions of cancer in their heads, fairly fixed perceptions too. They think about all the stuff they see daily on TV, in the main press, and people they know. The big cancers set the scene.
Most doctors know about the big cancers. They also know how to treat them, many of them have a fairly fixed regime of surgery/chemotherapy/radiotherapy. Many survivors will have side effects of their treatments, e.g.perhaps temporarily losing their hair. More people are now surviving these cancers and many will be declared disease-free or placed into some sort of remission status (no evidence of disease is a common term I see).
Most NETs are not like that! Whilst it has a reputation for being a generally slow-growing type of tumour at the lower grades (but very sneaky though!), offers a good outlook/prognosis for many, has many different types of treatment modalities, it can frequently present at an advanced stage and become incurable; and/or offer some quality of life challenges. For example:
Not many doctors know a lot about it.
Many patients will have gone through extended diagnostic periods, perhaps months, years in extreme cases. In many cases it can be ‘silent‘.
Only doctors who know a lot about it, really know how to quickly diagnose it. Only they know how to properly treat it. It’s a very individual disease, there are many factors involved.
Another key difference with NET Cancer is that many people will have an associated ‘syndrome‘ and this might have been with them for some time before diagnosis. The symptoms of these syndromes can sometimes be rather debilitating, even after treatment.
Many people will never be disease free nor will be they given a status of full remission. Their surveillance (scans etc) could continue indefinitely.
Many people will live with the consequences of this cancer for a long time and this plays on their mind as well as the effect on their body.
There’s a lot of talk about something called ‘unmet needs’ and quite right too. However, there’s not appear to be enough action to deliver those unmet needs in our community. This is a highly prevalent cancer and many people live a long time with the consequences of the cancer.
Before you receive treatment, always ask what the side effects might be, how long they might last and what support you will get to treat or lessen them. Don’t be afraid to ask, you deserve to be told.
OPINION. When I was being officially told I had an advanced and incurable cancer, I did what most people seem to do on films/TV ….. I asked “how long do I have“. The Oncologist said ” … perhaps just months“. That must have been quite a shock because for a few moments after that, I heard nothing – my brain was clearly still trying to process those words – I wasn’t even feeling unwell! The really important bit I missed was him go on to say “…but with the right treatment, you should be able to live for a lot longer”. Fortunately, my wife Chris heard it all and I was refocused. “OK Doc – let’s go” I said. Always take someone with you to take notes at important meetings with Oncologists!
I continue to see quite a few posts and articles about death and dying and I noticed some patients were using the word ‘terminal‘ to describe Neuroendocrine Cancer, despite in some cases, having been diagnosed some years ago. This label is not just confined to use within Facebook forums, I’ve also seen this on wider social media including twitter, blogs and newspaper items. For some, this appears to be the prognosis given to them by their doctors. I find this surprising. However, I’m much less surprised to see many comments on forums from people who had been told the worst by their doctors but were still alive and kicking WAY beyond those worst case prognostic statements.
Definitions are important so what does ‘terminal cancer’ actually mean?
I’m conscious there are legal ramifications with the definitions (wills, life insurance, disability etc) and that these may differ on an international/federal basis. I therefore intentionally confined my searching to a couple of ‘big hitter’ and ‘authoritative’ sites:
Cancer Research UK defines terminal as “When cancer is described as terminal it means that it cannot be cured and is likely to cause death within a limited period of time. The amount of time is difficult to predict but it could be weeks to several months”.
The American Cancer Society defines terminal as “an irreversible condition (it cannot be cured) that in the near future will result in death or a state of permanent unconsciousness from which you are unlikely to recover. In most states, a terminal illness is legally defined as one in which the patient will die shortly whether or not medical treatment is given.”
Can terminal as defined above be applied to Neuroendocrine Cancer?
I’m sure it can, e.g. with very advanced and very aggressive disease and for any grade when taking into account the condition of the patient and other factors (secondary illnesses/comorbidities, refusal of treatment etc). Clearly, that is a terrible situation. I’m also conscious that some people do eventually die because of this disease or its consequences and that is also terrible.
How long is a piece of string?
I think with most Neuroendocrine Cancer patients, “how long do I have” can be a tough question to answer. Thinking back to my own situation, although it was an obvious question to ask my Oncologist, I can see it might have caught him unawares. I suspect he was erring on the side of caution as I don’t believe he had formulated my treatment plan ….. i.e. my case had not yet been looked at by a Multi-Disciplinary Team (MDT), a bit like a ‘Tumor Board’. I had already been confirmed Grade 2 (via liver biopsy) and my CT scans were indicating widespread disease. I was yet to have an Octreotide scan and the conventional biochemical markers (CgA and 5HIAA). I suspect, faced with my question, he went for the worst case, based on the statistics he had access to at the time. What I now know is that, in the year of my diagnosis, the median survival was 33 months in patients with advanced Grade 1/Grade 2 NETs with distant metastasis. These statistics are certainly better today but my Oncologist was probably on the right track. However, at no time did he use the word ‘terminal’.
The Cancer story is changing
What I also found during my research is that as more and more people in the UK are now living with cancer (all cancer) rather than dying from it, there is a new class of patients emerging – Macmillan UK call this “treatable but not curable” and I believe this is very relevant to Neuroendocrine Cancer. I touched on this in an awareness blog entitled “Living with Neuroendocrine Cancer – it takes guts“. You will find some data in this blog about a major increase in the amount of people with cancer who eventually die of something else (…… basically it has doubled). For many, Cancer is no longer a death sentence. I do accept that it can be difficult to live with certain cancers and this is also covered in my “it takes guts” blog linked above.
Survivorship and Hope
You can find numerous examples of long-term survivors of advanced Neuroendocrine Tumours on the ‘airwaves’, many with a relatively good quality of life (QoL). I don’t normally pay much attention to prognostic data, I take my lead from the huge number of patients living a long time with Neuroendocrine Cancer. However, I was particularly interested to read a set of USA statistics from NOLA (Boudreaux, Woltering et al) which said “Our survival of stage IV midgut NET patients that we performed surgical debulking on was published in the Journal of the American College of Surgeons in 2014. It showed our 5, 10 and 20-year survival rates were 87%, 77% & 41% respectively. It’s also worth noting the comparison with the 2004 SEER database analysis which listed the 5 & 10 year SEER survival at 54% and 30% respectively”. Clearly, the NOLA figures are guidelines (and only for midgut) but they do seem to reflect my previous statement about seeking out positives rather than dwelling on the negatives. The SEER 2012 figures are much better than the 2004 versions stating “Survival for all NETs has improved over time, especially for distant-stage gastrointestinal NETs and pancreatic NETs in particular, reflecting improvement in therapies.
Exciting times ahead
On the subject of therapy improvement, there has been a plethora of new treatments coming online and more entering and progressing through the approvals pipeline. Check out my article entitled Exciting Times Ahead. Also listen to a NET Expert along the same lines. PRRT is making a real difference.
Following my diagnosis in 2010, I went on to receive really good treatment and it continues to this day with Lanreotide backed up by a rigorous surveillance regime (and this is backed up by my own advocacy!). However, I have totally accepted the fact that I have metastatic Neuroendocrine Cancer and that it cannot be cured. By the way, I intentionally used ‘metastatic‘ rather than Stage IV. Mention of Stage IV can set off alarm bells and send the wrong message to the recipient. I don’t believe Stage IV has the same ‘red flag’ meaning for well-differentiated NETs as it does with more aggressive cancers of the same stage. Given what I know now, I would certainly challenge any doctor who told me I had a ‘terminal disease’ and at the same time told me I had a slow-growing well differentiated Neuroendocrine Cancer.
I now live with this disease (….and it’sconsequences) and do not feel like I’m dying of it. Moreover, I most certainly do not see myself as a ‘terminal’ cancer patient, particularly as I’ve now been living with it since 2010.
EIGHT years ago. I was diagnosed with metastatic Neuroendocrine Cancer – 26th July 2010. Until I arrived at my 5th anniversary, I hadn’t thought much about how (or if) I should mark these occasions. I never thought I would dwell on such things as ‘Cancerversaries‘ but I now totally get why many patients and survivors do.
There are various types of ‘Cancerversary‘ that for some, could trigger a mix or range of emotions including gratitude, relief and fear of cancer recurrence or growth. These milestones could be the date of a cancer diagnosis, the end of a particular type of treatment (anniversary of surgery etc) or a period since no signs or symptoms of cancer were reported. Everybody will most likely handle it their own way – and that’s perfectly understandable.
The 5 year milestone was significant, mainly I suspect, because it’s a time period very frequently used in prognostic outcome statistics. When I was researching after my diagnosis, the 5 year figure for metastatic Neuroendocrine Cancer wasn’t that great, in fact it looked less favourable than more aggressive cancers. Then I gradually worked out that the prognostic figures for Neuroendocrine Cancer were very dated (like many other things) and did not take into account improved diagnostic techniques and the introduction of a plethora of new treatments, in particular somatostatin analogues. Consequently, I no longer pay any attention to prognostic statistics – I’m actually more influenced by the large number of long-term survivors I see out there.
Check out the fantastic response to my 7th year cancerversary post. Click here to be inspired.
My cancer is treatable but not curable but I will never call it terminal. Despite some issues this year so far, I’m still here and I intend to be here next year, and for many more years after that!