A quick primer on the word ‘CARCINOID‘. It originates from the term ‘Carcinoma-like’. ‘CARCIN’ is a truncation of Carcinoma (by definition cancerous or malignant tumour). ‘OID’ is a suffix meaning ‘resembling’ or ‘like’. This infers that Carcinoid cannot be a truly malignant tumour – thus the confusion (….. and anger!). The most worrying connotation of the use of the word ‘Carcinoid’ is the belief that they all have benign clinical and biological behaviour. That is dangerous thinking which could end up killing people.
There is now widespread use of the term Neuroendocrine Tumours (NETs) and this is based on the latest classification scheme pushed out by the World Health Organisation (WHO) in 2010 subsequently updated by WHO 2017. However, ‘Carcinoid’ seems to prevail in many parts of social media and advocate websites. As a blogger and social media geek, I still use it as it’s a popular search phrase (although I’m always careful with context and in the correct ‘scenario’). However and unfortunately, some still use it as a synonym for all NETs which is clearly incorrect. So called Carcinoid was ever only one rype of NET albeit covering more than one location. I’m therefore baffled by the continued and frequent use of phrases such as “Carcinoid and Neuroendocrine Tumours” which misleadingly infers they are different cancers. Not helpful.
To prevent confusion, the use of the term NETs frequently needs to be expanded to distinguish between the different types. However, there does not appear to be any agreement on the naming conventions and I suspect this is probably one of the reasons why many people (including NET Specialists and advocate organisations) continue to use the term Carcinoid. I’ve seen sporadic use of the term SINET (small intestinal NET) and SB NET (small bowel NET) and we already have pNET (notice the syntax difference …..) and more recently I’ve seen ‘PanNET’. I believe use of the anatomy has potential as a way forward but we need consistency. Check out my article below entitled Carcinoid vs Neuroendocrine for advice on the correct terminology to use.
The primary NET is really important for context and understanding, thus why many patient advocate organsiation and cancer sites will still classify and list ‘Carcinoid’ as a single NET type rather than the long list of anatomical locations which can no longer be grouped under the heading of the Carcinoid type. There are also many other factors involved and no solution seems to be perfect up to this point. As for syndromes, there are several. So patients confused by the ‘instruction’ on the use of ‘Carcinoid’ will just say “I have the syndrome”. Just which one are they talking about? We also need to consider Carcinoid Heart Diseaseand Carcinoid Crisis and I have excellent suggestions for renaming both.
Another term I regularly see is ‘Noid’ – a truncation of Carcinoid. Whilst I suspect that might have been popular and convenient many years ago, clearly it is not helpful when you consider the issues above. Personally speaking, I find myself annoyed by being described as a Noid! Particularly when the ‘oid‘ part is what is causing the angst described above. And while we’re at it, I’m also annoyed by being described as a zebra, that is another thing holding up our 21st century awareness campaigns. Let’s move out of the 1980s please!
Some might say all of the above is just semantics and it’s nothing to get too excited about. However, I believe we need more coordinated awareness and more coordinated clout for Neuroendocrine disease. We should at least be consistent with the nomenclature messages (amongst other things).
Fortunately the big NET specialist organisations are slowly getting rid of the word ‘Carcinoid’ and this is long overdue. It will take a while and patients can set the example for the doctors and medical writers.
I recently wrote a blog entitled “Trust me, I’m a Doctor” which was a genuine attempt to say that we should try to work with our Doctors. However, I also covered the issues that Neuroendocrine Tumour (NET) patients face in finding someone who understands their disease and how best to treat it; and that can on occasion lead to issues with doctor-patient relationships and communication. The blog then commented on a number of tips for better doctor-patient relationship and communication. These tips were provided by a Doctor via my friends in Cancer Knowledge Network.
In the blog above, there was an underlying theme indicating certain cancer patients might need to know more about their disease than would be considered normal and that can influence the nature of the doctor-patient relationship. I certainly believe this is the case with NETs and is indeed something evident on most forums. I’ve touched on this subject a few times including a much earlier blog entitled Passive patient or active advocate?As I said in this article, whilst I have a great medical team, I also like to be my own advocate and this means understanding what medical people tell me! I have no intention of becoming a passive patient anytime soon! I realise this is not for everyone but I know some patients have others functioning in this way on their behalf. That’s fine too!
I spotted another excellent discussion article on Cancer Knowledge Network and although the context is patient advocacy at committee level, I thought it applied nicely to many scenarios including the most simple one where a patient decides to learn about their disease in order to better represent themselves at meetings with their nurses, doctors and specialists.
The author, who is both a Doctor and a Patient, describes 5 myths about the usefulness of patients that can sometimes be present within medical circles . I actually believe the patient is the most underused person in healthcare and so I found myself nodding my head to much of what she had to say. Let me know if you were nodding too 🙂
It was wet, windy and very cold and that was at the bottom of the hill I’m looking up at. It wasn’t a terribly big hill but I knew it would need considerable effort and perhaps some temporary pain. Whilst the rain splashed onto our jackets and the wind howled, Chris and I looked at each other and we almost simultaneously said “do we really want to go up that in this weather?” Oh yes ……. no pain, no gain! So we went up the hill and it hurt. Up there, it was wetter, windier and colder! However, the ‘euphoric high’ was worth it. It was medicine healing the body and mind.
Of course, the ‘hill’ is also a metaphor which might be a number of things from simply venturing outside the house to a full-blown adventurous holiday. It might be a simple bit of gardening, a walk to the shops (mall), 30 minutes on a treadmill at a leisurely pace. Or it might be something more physically demanding like a marathon. It doesn’t even need to be anything physical, for example, starting a diary, trialling a change in diet, learning about specific aspects of your condition. In general, the “hill” is a state of mind and you metaphorically climb it to fight cancer and improve quality of life.
Whatever your ‘hill’ is, two of the common elements required to ‘climb it’ are motivation and willpower. However, if you’re fighting cancer and you’re being affected by the symptoms or the side effects of treatment, it can be easy to opt to stay within your ‘comfort zone’ even though you know the ‘hill‘ might do you some good. I suspect this is a common predicament for a cancer patient, do I ‘climb that hill’ or do I walk away? Sure, I walk away from the “hill” occasionally but I try not to make a habit of it.
Sometimes you just need to go and climb that hill!
One of the most frequent posts on forums is about the Patient-Doctor relationship (or occasionally a lack of it…..). Personally, I have a lot of time and respect for all medical staff and I suspect that has been influenced by my general life experience, perhaps cemented since my diagnosis of metastatic Neuroendocrine Cancer in 2010. The vast majority of people tend to trust Doctors and I’m a bit old-fashioned in this respect. If you have metastatic Neuroendocrine Cancer, you see medical staff a lot! Relationships and communication can therefore become more important than ever.
However, people with less common conditions can perhaps be more difficult to satisfy. A ‘generalist’ doctor (i.e. a GP or PCP) is unlikely to be very knowledgeable about every single condition. Even at secondary care level, certain less common conditions still need dedicated specialists and these services may not be located at every hospital. Clearly with Neuroendocrine Cancer, the optimum scenario is to be treated at a NET specialist centre or at least be overseen by them. However, these can be thin on the ground and/or the medical system in place is not able to provide access to these experts. Geography may also be playing a part causing further anxiety and this is not helpful if you are already fighting cancer. Communications and relationships between patients and doctors can therefore be more difficult even with the right diagnosis.
I see so many issues on forums ranging from people who are simply looking for a specialist to people who still don’t think they got the right treatment from the specialist they eventually found. Emotions directed at physicians range from ‘god-like adulation’ to offers of violence! If you only looked at forums, you would believe there are only a handful of NET Cancer specialists when in fact there are many more than this. Check out the most up to date lists inside this article – click here.
I know from talking to other patients that some have not had the ideal experience with their doctor(s). Even those who found a NET specialist report the odd issue and feelings of unhappiness. I never cite these issues publicly, in particular the hospital or the doctor, because for every one of these stories, you can find dozens of good patient experiences with the same hospital and doctor.
It’s a really complex area and it can be compounded by the health system in place but many things are common across the board. One of the reasons making it complex is that it can be about relationships and communication – both ways! Thus why I was interested to read an article by a physician who listed a number of tips for patients which I think are as relevant to Neuroendocrine Cancer as they are to other conditions (……in fact some more so!). Relationships and communication will not cure or reduce your cancer; or debulk your tumours – well not directly ….. but it can help along the way. And although the article appears to be written in a post diagnosis context, some of it is also relevant to pre-diagnosis.
The top 8 tips are:
Know your own communication style and preference for informing and being informed. This is an interesting point which I hadn’t really thought about. That said, some of the response to this tip can be addressed in some of the other tips. I guess in hindsight, asking my doctors not to hide stuff and to just “hit me with it” is an indication that I had set my preferences early on. I wanted to know the real problems I was facing. Additionally, my Oncologist knows I like copies of all tests and reports and he obliges. I always take notes.
Think about how you prefer to hear important health information such as the results of a biopsy or a scan and then convey that to your doctor or nurse. I think this is partly addressed above. I see my MDT face to face every 6 months but if it is for bad news, I would certainly like some notice in order that I can be accompanied by my wife. I don’t think I’ve made that clear enough so an action for me here.
Prioritize your concerns, if you present your doctor with a very long list of questions or symptoms at the very end of the visit, it’s quite likely that you will both end up frustrated. I have experienced this issue many times but gradually I’ve learned how to improve this form of communication. It’s easy to forget your physician has other patients and only has a finite time to spend on your case. I now send my Oncologist a summary email with my top 3 or 4 concerns and I do this around 2 weeks prior to each appointment. I copy in the specialist nurse who is mostly already aware via frequent communications. This not only gives them some time to read but also prevents the scenario above. It’s starting to work better.
Make your needs known, doctors and nurses cannot read your mind. This is an absolutely key tip as far as I’m concerned. I believe the patient is the most underused person in healthcare. Patients have a part to play in their own diagnosis phase and this continues all the way through to ongoing treatment (including wary of the doctor). Patients must have a voice and patients must use this voice to describe what’s going wrong with their body and what’s troubling their mind. Doctors and nurses cannot read your mind but they must listen to your voice.
Trust the clinicians involved in your care and think of them as partners. I think all clinicians want us to trust them after all they’ve done the 10 years training and we have not! However, with less than common conditions, I suspect patients probably need to be wary and advocate more. I think of myself as a partner (part of the MDT for the period of my consultation) and so by default, I already think this way.
Beware of the common trap of thinking in terms of all or nothing or rushing to conclusions. This is an interesting one for incurable but treatable cancers. I think with incurable Neuroendocrine Cancer, you need to be prepared for a long haul and the occasional bump along the windy road. Services and inspections will need to be done and tyres will need to be changed. It’s not a perfect journey and don’t trust the SatNav!
Share the burden of not knowing how things will ultimately work out. This is a difficult one and I suspect each person will have their own concerns and their own way of dealing with it. I’m thinking this might be more important for younger patients who have young families to look after. I’m a ‘glass half full’ person so it’s an awkward one for me. I guess as I’m feeling confident I’m not leaving anytime soon, it’s something still stuck in the back of my mind.
Find ways of being at ease, even during frightening or turbulent situations. Easier said than done! Again, we all have different ways of dealing with our situations but I do believe if you have addressed all the tips above, this should make it easier. I also think that learning a lot more about your disease really helps to communicate about it better.
I’m often shocked to hear that people ‘fire’ their doctor but I guess if you are paying out of your own pocket, it can be an apt word to use! Clearly if the service you receive is not working to your expectations, then a move might be beneficial for both parties. It’s a big decision though and for those who have moved on, I sincerely hope the grass has been greener on the other side.
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 stated “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
Recently, there’s been a plethora of new treatments coming online and more entering and progressing through the approvals pipeline. Check out my blog entitled Exciting Times Ahead. Also listen to a NET Expert along the same lines.
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 therefore 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 also told me I had a slow-growing well differentiated Neuroendocrine Cancer.