The Invisible NET Patient Population 

The Invisible NET Patinet Population

OPINION

 

I found some of the quotes from the recent NET SEER Database study (Dasari et al) very interesting.  The National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) Program is a comprehensive source of population-based information initiated in 1973 that is updated annually. Although the study is US-based, it represents the largest study of Neuroendocrine Tumors (NETs) ever recorded and is therefore a good guide to what might be found beyond USA. In fact, other national declarations of incidence and prevalence of NETs seem to bear these statistics out, i.e incidence rates of 7-8/100,000 …… almost 7 times the rate recorded in the 1970s. If you want to understand the factors behind this massive increase, I covered this extensively in my post “Neuroendocrine Tumors – not as rare as you think“.  In this article, I looked at USA and beyond. Those who are regular readers of my articles will already know I’ve been ‘banging on’ about this for a few years. Other organisations and individuals (including NET specialists) are now indicating these tumors are not rare, some vindication for my aforementioned ‘banging on’.  This is now a serious disease with some serious statistics behind it and we need a new way of doing things.

 There are two further quotes which I’d like to focus on in this article:

1. From the NET SEER Database study published 2017:

…… many cases of NETs may not have been reported to cancer registries unless considered malignant…… it is likely that we have underestimated their true incidence and prevalence” – i.e. the slide here:

SEER 2012 Underestimated

2. From Dana Farber (Kulke, Chan):

“Estimated more than 200,000 undiagnosed cases in the US” – this slide here:

dana-farber-200000

…. But what do these quotes actually mean?  Here’s my take:

Underestimating the true incidence and prevalence of NETs

I studied the latest SEER NET study, formally titled “Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States” (authored by Arvind Dasari, MD, MS; Chan Shen, PhD; Daniel Halperin, MD; et al). From this document, I can see the authors were aware of the well-known faults in cancer registries worldwide and the effect this has on the true incidence and prevalence of Neuroendocrine Cancer.  These issues, which are a worldwide problem, include the incorrect registration of Neuroendocrine Cancer as other types based on the anatomical location of the primary tumor.  At this point, you may wish to check out my post “The Human Anatomy of Neuroendocrine Cancer” which provides some real life examples of the confusion between primary Neuroendocrine location and other cancers. That said, things are definitely improving because the latest SEER data shows a marked increase in the incidence of High Grade Neuroendocrine Carcinomas (NEC), an area where this issue is prevalent. A similar increase in NEC was also illustrated in the UK’s figures from Public Health England (PHE) in 2016 (click here) indicating that cancer registries are getting better and not before time, although it has to be said this only came about due to a major intervention by NET Patient Foundation and others. Through this work, it’s becoming clear that the incidence of all NETs in UK is around 8 to 9 per 100.000 (rare threshold <=5).

But there’s another issue impacting whether a diagnosis is actually entered on a cancer registry or not.  Unfortunately, there are members of the medical community who still see well differentiated NETs as benign tumors, ‘not a proper cancer’ and still use ancient terminology ………  ‘Carcinoid’.  The WHO 2010 classification for NETs was based on the concept that all NETs have malignant potential. Here’s a quote from the UKINETS Guidelines in 2011 (Ramage, Caplin, Meyer, Grossman, et al).

Tumours should be classified according to the WHO 2010 classification (Bosman FT, Carneiro F, Hruban RH, et al. WHO Classification of Tumours of the Digestive System. Lyon: IARC, 2010). This classification is fundamentally different from the WHO 2000 classification scheme, as it no longer combines stage related information with the two-tiered system of well and poorly differentiated NETs. The WHO 2010 classification is based on the concept that all NETs have malignant potential, and has therefore abandoned the division into benign and malignant NETs and tumours of uncertain malignant potential.

The guidance in WHO 2017 for Endocrine Organs reinforces this statement.

The undiagnosed NET patient population

From above, you can see why the incidence (and therefore the prevalence) of our disease has almost definitely been underestimated.  However, that’s not the end of my story……..

A number of statements are clear about Neuroendocrine Tumors:

  • Low/Intermediate grade well differentiated tumors are known to have been growing slowly over a number of years before discovery or accurate diagnosis occurs,
  • They can be difficult to diagnose,
  • They are not that well-known amongst the general medical population,
  • Many people are initially misdiagnosed with another condition, with some this will result in late presentation with metastatic disease.
  • Many NETs are found during autopsies.

The living undiagnosed. It’s worth pointing out that one of the conclusions made by the recent SEER NET study is that the increase in incidence and prevalence can be attributed to a number of factors including earlier diagnosis.  This is of course excellent news.  Also worth noting that another conclusion of the study is that we are all living longer, reflecting improvements in therapies.  This is also great news and is a factor in increased prevalence figures. However, it seems obvious that there are hundreds of thousands of people out there still be diagnosed who have tumors silently growing inside them and who are in a loop of referrals between primary and secondary care awaiting a proper diagnosis. See the Dana Farber slide above.  Please help these people by sharing this article (you never know who it will reach – Diagnosing the Undiagnosed.

The dead undiagnosed? The true incidence of NETs may be much higher owing to the lack of diagnosis until after death.  For example:

  • In USA, a respected NET specialist stated that the autopsy find for (excuse the outdated terminology…….) ‘carcinoid‘ is 4 times the recorded diagnosis rate (based on the known incidence rate at the time, this is 8 per 100,000).
  • In Australia, one study claimed that 0.05% of all autopsies found a Pheochromocytoma or Paraganglioma. “
  • The Mayo Clinic experience shows that in up to 50% of cases of pheochromocytoma, the correct diagnosis is made at autopsy (ergo the incidence rate could be double what is published).
  • Here is an article claiming that former US President Dwight D Eisenhower had a biopsy confirming he had a Pheochromocytoma.  Click here.
  • A Hong Kong study indicated that 1% of all autopsies discovered an ‘Islet Cell’ tumour (i.e. a Pancreatic NET or pNET).
  • In one series, (excuse the outdated terminology…….) ‘carcinoid’ tumors were found in 1.22% of 16,294 autopsies in Malmö, Sweden, 90% of which were incidental findings.

It’s possible that many of these people showed no NET symptoms during their life but …… it’s equally possible that many of these people had NET symptoms but just put up with it and/or had been diagnosed with something else, and then died without a correct diagnosis.  There is no evidence that any investigation follow ups were done so this possibility remains.

The potential for even more undiagnosed. To add to the underdiagnoses of NETs issue, is this most amazing piece of research published in 2018 – Pan-cancer molecular classes transcending tumor lineage across 32 cancer types, multiple data platforms, and over 10,000 cases.  It was published in the American Association of Cancer Research (AACR) journal ‘Clinical Cancer Research and authored by Chad Creighton et al. D.  This was a pan-cancer piece of research which indicated that Neuroendocrine disease may be more prevalent than anyone has ever thought.  There’s a summary article here which I suggest you read fully.  The rather explosive extract is as follows:

We expected that about 1 percent of

Are you undiagnosed but suspect NETs?

Check out my advice by clicking here.

Summary

I suspect there’s an invisible patient population for many conditions but the slow-growing and relatively quiet nature of Neuroendocrine Cancer means there could be a significant undiagnosed burden walking around, looking for a diagnosis, putting up with symptoms and being treated for other conditions. I see people on forums looking for clues, social media can sometimes be helpful here. That said, I do get the feeling some do not have NETs, regardless of the symptoms they associate with the disease, but I guess many of them will go on to be formally diagnosed with something. I’m contacted by many ‘undiagnosed’ people on my own blog and supporting Facebook sites (mostly privately) and I can tell you that’s a tough gig.  I only hope I’ve given them some useful ideas about where to look or what to ask/suggest.

I feel earlier diagnosis reported in the SEER study is partly due to increased awareness, particularly in the medical world. I would also suggest that it has improved in the general population due to the explosion of social media information dissemination. It’s also accurate to say that improvements in diagnostic capabilities is also playing its part in pushing up incidence rates, just as improved therapies have pushed up prevalence rates, something emphasised by Dasari (et al) in the most recent study.  Things are improving but there is so much more to do.

The issues caused by inefficient registries together with ‘the undiagnosed’, combine to suggest there is a large invisible NET patient population out there ……. we just need to find them!  

Thanks to NET Patient Foundation for featuring this article here.

NET Patient Foundation logo

Road ahead closed – Bowel Obstructions

test npf

OK – we’ve gone through diagnosis, we’ve gone through treatment and now we need to live with the consequences of cancer and it’s treatment.  Not a day goes by when I don’t feel some twinge or some minor pain and I think ‘what was that?‘.  Fortunately, many things can just be day-to-day niggles. It’s the cancer …. easy to say, sometimes not easy to prove.

However, for Neuroendocrine Tumour (NET) patients who have had surgery, anything that seems like a bowel obstruction is quite a scary thought (I suspect this is also an issue for other cancer types).  In fact, even before diagnosis, a bowel obstruction rears its head as it can be how the condition is diagnosed in the first place, i.e. pain leads to more pain and that can sometimes result in a visit to the ER/A&E which can very often lead to a scan and an incidental diagnosis of NETs (and I suspect some other cancers).

I guess this isn’t just a threat for those who’ve had intestinal NETs but others in the vicinity of the intestines could also have this issue – the abdominal cavity is full of organs all very closely packed together! Both the small intestine and the large intestine can become blocked and if it can’t be unblocked by non-surgical means, it can become a bit of a drama for the patient. Blockages can be full or partial so it can often be a tough call for the medical team due to the effects of the patient’s existing surgery including but not limited to previous surgical scarring (adhesions), mesentery or retroperitoneal fibrosis complications (read about that by clicking here). Clearing the blockage by non-surgical means is the optimum solution. The presentational symptoms and scans can give immediate clues.  Although there are slightly different symptoms for large and small intestine (bowel) obstructions, the key symptoms of a blockage would appear to be:

Feeling bloated and full

Severe abdominal pain

Feeling sick

Vomiting large amounts

Constipation

Looking at some authoritative sites, the logical (and fairly obvious) decision steps seem to be:

Is there an obstruction or is the problem something else?

If an obstruction, where exactly is it?

What is causing the obstruction?

Are there any complications such as adhesions, twisted loops or hernias

Optimum treatment

In 2016, I had 3 bouts of constipation and I confess that a potential blockage did cross my mind on all 3 occasions. However, I was comforted by the fact that I had no nausea and/or vomiting which I suspect is one of the key symptoms indicating a blockage rather than just a sluggish system. Fortunately, on all 3 occasions, the matter settled following a few days of right-sided pain (RLQ). One occasion required lactulose but all three required patience sprinkled with a pinch of endurance!  I have to say the lactulose experience was not a good one – fatigue, brain fog and general malaise …..but much better than surgery.  If you have issues with ‘fear’ living with cancer, check out my 7 tips article by clicking here.

I’m once again making some adjustments to try to find the magic spot between stool frequency and bulk….. it’s really difficult and not an exact science.  I’m suspecting diverticular disease might be playing some part as I was diagnosed with a mild version in 2008 spotted during a colonoscopy (a common problem when you’re over 50). Although that tends to be a left-sided problem, I remain conscious that my ‘new plumbing’ may not be the best representation of a conventional layout!

NET Patient Foundation are really good at producing cards and there’s one for this too!  Here’s the back of it here:

NPF Bowel Obstruction Card Back

Thanks for reading

Check out my other posts with NPF cards:

Carcinoid Crisisclick here

The Diarrhea Jigsawclick here

Ronny

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

 

Neuroendocrine Cancer: To cut or not to cut?


surgery

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

b.  Surgical Considerations for 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):

algorithm-ukinets-page-2-gutjnl-2012-january-61-1-6-f2-large
Very generic treatment algorithm UKINETS – Ramage JK, Ahmed A, Ardill J, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine tumours (NETs) Gut 2012;61:6-32.  For example purposes only please.

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 Jobs a 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.

Summary

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.

Thanks for reading

Ronny

I’m also active on Facebook.  Like my page for even more news.  I’m also building up this site here: Ronny Allan

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

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Neuroendocrine Cancer – don’t let it be a Crisis

crisis

The word ‘crisis’ has a wide range of meanings and it’s well used in the media to catch the reader’s attention. Lately, the terms ‘political crisis’, financial ‘crisis’ and ‘constitutional crisis’ appear almost daily in media headlines. In a previous life, the term ‘crisis management’ was used daily in the work I was undertaking as I went from problem to problem, dampening or putting out fires (….. that’s a metaphor!).  Thinking back, my adrenaline (epinephrine), norepinephrine, and cortisol must have been very busy! 

However, in the world of Neuroendocrine Tumours (NETs), ‘crisis’ has a very significant meaning and its very mention will make ears prick up.  The word ‘crisis’ is normally spoken or written using the term ‘Carcinoid Crisis’ given this is the type of NET with which it has been mostly associated. However, I’ve studied and researched and it would appear that some form of ‘crisis’ might apply to other types of NETs. Perhaps this is another knock-on effect caused by the historical use of the word ‘Carcinoid’ to incorrectly refer to all NETs. In terms of ‘crisis’, maybe there should be a more generic NETs wide term?  Of course there should, once again ‘carcinoid’ is causing confusion.

What is (so called) ‘Carcinoid Crisis’?

In the simplest of terms, it is a dangerous change in blood pressure, heart rate, and breathing (technical term – cardiopulmonary hemodynamic instability).  On an operating table under anaesthetics or an invasive procedure such as liver embolization, this can actually be life threatening.  Incidentally, this happens with many other types of conditions (hormones and peptides do exist in other illnesses). However, with a patient already oversecreting these hormones and peptides, it could be a life or death situation.

What is the difference between carcinoid crisis and carcinoid syndrome?

Carcinoid crisis is said to be a situation where nearly all of the possible symptoms of carcinoid syndrome come at the same time and in some severity. Carcinoid crisis is a serious and life-threatening complication of carcinoid syndrome, and is generally found in people who already have carcinoid syndrome. The crisis may occur suddenly, or it can be associated with stress, a reaction to treatment, but it is mainly as a result of the use of anaesthesia. There is a thin line between a very severe bout of carcinoid syndrome and carcinoid crisis but generally it can be characterized by an abrupt flushing of face and sometimes upper body, usually severe falls in blood pressure and even bronchospasm with wheezing can infrequently occur. The attack may look like a severe allergic reaction.

It is said by one very well-known NET expert to “not to be something which happens randomly to all patients, it is usually linked to a medical procedure of some sort when you are having anaesthesia”.  Dr Eric Liu also said “Luckily it is relatively uncommon”.

Why does it happen to some NET Patients?

NETs can release a variety of ‘vasoactive peptides’ (hormones) in excess (e.g. serotonin, catecholamines, histamine).  Under normal circumstances, these would just present as routine syndromes which may need to be controlled in most cases with somatostatin analogue treatment (Octreotide/Lanreotide).

Excess amounts of these vasoactive substances can cause both hypertension and hypotension (high and low blood pressure respectively). In extreme cases this can lead to what is known as a crisis situation.

How is the risk managed?

Most people are effectively managed on monthly injections of Octreotide/Lanreotide but some people still need ‘rescue shots’ (top ups) where they are experiencing breakthrough symptoms.  When I was symptomatic (syndromic), I would regularly flush in stressful situations but that was definitely syndrome rather than crisis. Check out my video explaining how I felt.  It’s worth reading something called the 5 E’s of Carcinoid Syndrome, probably useful to other types of NETs as I’m sure there is some overlap.

If you research this plus perhaps from your own experience, you will know there are different ideas and ‘protocols’.  However, they all mostly involve some pre-procedure infusion of a somatostatin analogue (normally Octreotide) – although I’d love to hear from anyone who has had Lanreotide as an alternative.  Some doctors or hospitals are known to have their own ‘protocols’ and I’ve uploaded the one from the ISI NET book page 215 (Wang, Boudreaux, O’Dorisio, Vinik, Woltering, et al). Click here.  Please note this is an example rather than a recommendation as this is something the NOLA team have developed for their own centre.

In all the big procedures I’ve had done in my local NET Centre, I have always been admitted the day before to receive what they describe as an ‘Octreotide Soak’.  The link below is an example of the UK standard for pre and peri-operative protection (please note your NET team may be working to a slightly different protocol based on their own version of best practice, just to emphasise that this is an example and not advice).

Useful guidance from UKINETS – click here

Patients are always asking about the risk and requirements for smaller procedures such as an Endoscopy.  There does not seem to common guidance on this but Dr Woltering who is always forthcoming with advice suggests 200 micrograms of Octreotide before the procedure commences.

Dental visits involving anaesthetics can also be an issue and you can see Dr Woltering’s advice in my blog about the 5 Es of Carcinoid Syndrome.  Additionally there is advice for users of ‘Epi Pens’. You also need to derisk those situations.

What about other types of NETs

The ISI Book Link above (here for convenience), does stateregardless of tumor type, all NETs should be pre-treated with Octreotide for protection against crisis.  I know that NET patients other than those with ‘Carcinoid Tumours’ are also treated with somatostatin analogues, as they too can be subject to the effects of excess secretion of certain vasoactive peptides.

Why is the issue relative to Pheochromocytoma/Paraganglioma? 

Pheochromocytomas and paragangliomas are catecholamine-producing neoplasms that can cause life-threatening hemodynamic instability, particularly intraoperatively, when the tumor is manipulated.  In some ways their version of ‘crisis’ is more complex and dangerous than in the issues with carcinoid crisis above.  There needs to be significant pre-operative preparation in addition to peri-operative measures, in fact with this type of tumour, post surgical treatment and monitoring is also required.

I recently read an article about a person with a Pheochromocytoma. The person had what was described as an ‘Intraoperative Hypertensive Crisis‘ that appeared to be caused by her tumour type rather than the sort of incident that might occur in a standard surgery.  Hypertension (high blood pressure) can be a symptom of Pheochromocytoma so you can see the problem with surgery and other procedures. An interesting issue with this type of NET is that after surgery, the patient is at risk for hypotension (low blood pressure) from venous dilation caused by the sudden withdrawal of catecholamines. Read more here.

Summary

I highly suspect there are many examples from the NET world beyond the ‘carcinoid’ subtype of NETs and I’ve already given you one above.  I’ll update this blog as I discover other examples.  In the meantime, make sure you ask your medical team about ‘crisis protection’ if you are to undergo any surgical or invasive medical procedure. Minor procedures should also be assessed. 

Do we need to rename the term Carcinoid Crisis to Neuroendocrine Crisis?  Probably …… let’s give it a red card!

Thanks for reading

Ronny

I’m also active on Facebook.  Like my page for even more news.  I’m also building up this site here: Ronny Allan

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

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One every 2 hours

 

Neuroendocrine Cancer Incidence Rate - EnglandI’ve made no secret of the fact that I don’t believe Neuroendocrine Cancer is rare and you can read why in some detail in my article Neuroendocrine Cancer – not as rare as you think.  Better diagnostic technology, greater awareness and better recording of the correct disease in national cancer registries.

The latest figures for Public Health England (covering ~90% of UK), indicate there are now 4800 diagnoses of NETs every year, i.e. more people than ever are being diagnosed, It is calculated from an incidence rate of 9/100,000 (using the 2011 census for England of 53,000,000) The new figures do not include Lung Neuroendocrine Carcinomas (LCNEC and SCLC) – so it is understated. This would appear to debunk the myth that the condition is rare given that the incidence rate has now gone beyond the threshold to be considered rare in Europe (5/100,000).

You can read the Public Health England (PHE) paper by visiting the NET Patient Foundation site here.

To put this diagnostic data into perspective:

4800 newly diagnosed NETs a year in England alone

= 400 a month

= 92 a week

= 13 a day

= 1 every 1.84 hrs

And in USA …

The UK is not alone in recording major increases taking the incidence and prevalence beyond the threshold of rare disease categorisation.  The very latest SEER figures for USA confirmed the disease is no longer rare in 2017, particularly as the annual incidence rate is now 23,000 in that country (circa 5 every 2 hours).
Neuroendocrine Cancer Incidence Rate - USA

Please let’s stop perpetuating the myth.

Thanks for reading

Ronny

I’m also active on Facebook. Like my page for even more news. I’m also building up this site here: Ronny Allan

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!



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Neuroendocrine Cancer – If you can see it, you can detect it!

octreo-vs-g68
Octreoscan vs Ga68 PET

Scanning is a key diagnostic support and surveillance tool for any cancer.  Even though you have elevated bloods or urine (….or not), a picture of your insides is really like a thousand words…. and each picture has a story behind it.  Scanning can be a game changer in the hunt for tumours and although scans do not normally confirm the cancer type and grade, they certainly help with that piece of detective work and are key in the staging of the cancer.

When I read stories of people in a difficult diagnosis, I always find myself saying ‘a scan might resolve this’ and I always suggest people should try to get one.  Even in the case of a story about late diagnosis or a misdiagnosis, I find myself thinking ‘if only they had done a scan earlier’.  Despite what you read on NET forums, a CT scan will be able to find some evidence of tumour activity in 90-95% of cases.  However, some are cunningly small or hiding and it can be like trying to find a needle in a haystack.

However, scans are not an exact science…..not yet!   Apart from human error, sometimes tumours are too small to see and/or there are issues with ‘pickup’ (i.e. with NETs, nuclear scans need efficient somatostatin receptors).  The differences between scan types are more quality (sensitivity) related as new technologies are introduced.

As for my own experience, I was very lucky.  I managed to get a referral to a specialist early on in my diagnosis phase. He looked at the referral notes and said “what are you doing this afternoon“. I replied “whatever you want me to do“.  He didn’t know I had cancer but his instincts led him to believe he needed to see inside my body, he wanted to scan me.  The scan results were pretty clear – I had a metastatic Cancer and further checks were now needed to ascertain exactly what it was. So I took my seat on the roller coaster.  Medicine is not an exact science (not yet anyway) but here’s something I believe is a very common occurrence in all cancers – If your doctors don’t suspect something, they won’t detect anything.

There’s frequent discussion about the best types of scans for different types of NETs and which is best for different parts of the anatomy.  There’s also different views on the subject (including in the medical community),  However, a few well known facts can be gleaned from authoritative NET sources:

Conventional Imaging

Computed Topography (CT)

CT scans are often the initial imaging study for a patient presenting with signs or symptoms suggestive of many cancers including NET. These studies are most useful for disease staging and surgical planning as they provide excellent anatomic detail of the tumors themselves and surrounding structures. Primary NETs (GI and lung NETs) and their metastases are generally hyperenhancing with IV contrast and are best seen in the arterial phase of a triple phase CT scan.

In primary NETs, the average sensitivity of a CT scan is 73%. CT scans have even better sensitivity in detecting NET metastases, as they demonstrate 80% sensitivity for liver metastases (but see MRI below) and 75% sensitivity for other metastases (non-liver). This modality is also useful when the primary tumor site is unknown. In one single-institution retrospective study, it was the most common study ordered to look for an unknown primary tumor site and was able to uncover the primary in 95% of cases.

Magnetic resonance imaging (MRI)

MRI is the best conventional study to detail liver metastases in NETs. It is not as useful as CT for the detection of primary small bowel lesions or their associated lymphadenopathy, but is good for the detection of primary pancreatic NETs. A study comparing MRI, CT and standard somatostatin receptor-based imaging (OctreoScan) reported 95.2% sensitivity for MRI, 78.5% sensitivity for CT and 49.3% sensitivity for the OctreoScan in detecting hepatic metastases. MRI also detected significantly more liver lesions than the other two modalities.

You may see something called Magnetic Resonance Cholangiopancreatography (MRCP).  Magnetic resonance cholangiopancreatography (MRCP) is a special type of magnetic resonance imaging (MRI) exam that produces detailed images of the hepatobiliary and pancreatic systems, including the liver, gallbladder, bile ducts, pancreas and pancreatic duct.

Ultrasound (US)

Liver_Metastases_Ultrasound

The primary role of conventional ultrasound in neuroendocrine disease is detection of liver metastases and estimation of total liver tumor burden. This technique has the advantages of near-universal availability, intraoperative utility, minimal expense and lack of radiation. Most examinations are performed without contrast, which limits their sensitivity (compared with CT and MRI).  I know in my own situation, US was used as a quick check following identification of multiple liver metastasis during a CT scan. I’ve also had US used to monitor distant lymph nodes in the neck area but always in conjunction with the most recent CT scan output.

Endoscopic Ultrasound (EUS)

EUS

With increased access to endoscopy, NETs in the stomach, duodenum, and rectum are increasingly incidentally detected on upper endoscopy and colonoscopy. Patients are frequently asymptomatic without any symptoms referable to the a NET (i.e. non-functional).  EUS has also been used to survey patients at increased risk of developing pancreatic NETs. For example, patients with multiple endocrine neoplasia (MEN).  They are also frequently used in conjunction with biopsies using fine needle aspiration (FNA) guided by EUS.

Somatostatin receptor-based imaging techniques

owl ga68
Graphic courtesy of Advanced Accelerator Applications

Somatostatin is an endogenous peptide that is secreted by neuroendocrine cells, activated immune cells and inflammatory cells. It affects its antiproliferative and antisecretory functions by binding to one of five types of somatostatin receptors (SSTR1- SSTR5). These are G-protein coupled receptors and are normally distributed in the brain, pituitary, pancreas, thyroid, spleen, kidney, gastrointestinal tract, vasculature, peripheral nervous system and on immune cells. Expression of SSTRs is highest on well-differentiated NETs. Somatostatin receptor type 2 is the most highly expressed subtype, followed by SSTRs 1 and 5, SSTR3 and SSTR4.

It must be noted that even the most modern scans are not an exact science.  Radionuclide scans are like conventional imaging, they can be subject to physiological uptake or false positives, i.e. they can indicate suspicious looking ‘glows’ which mimic tumours.  This article explains it better than I can – click here.

The ubiquity of SSTRs on NET cell surfaces makes them ideal targets for treatment (e.g. Somatostatin Analogues (Octreotide/Lanreotide) and PRRT), but also for imaging. There are two primary types of somatostatin receptor-based imaging available:

Octreoscan – In111 based

The most common (currently) is the OctreoScan or Somatostatin Receptor Scintigraphy (SRS), which uses the ligand 111In-DPTA-D-Phe-1-octreotide and binds primarily to SSTR2 and SSTR5. In its original form, it provided a planar, full body image. In modern practice, this image is fused with single photon emission computed tomography (SPECT) and CT. This takes advantage of the specificity of the OctreoScan and the anatomic detail provided by SPECT/CT, improving OctreoScan’s diagnostic accuracy. These improvements have been shown to alter the management in approximately 15% of cases, compared with just OctreoScan images. In primary tumors, the OctreoScan’s sensitivity ranges from 35 to 80%, with its performance for unknown primary tumors dipping beneath the lower end of that range (24%). Its ability to detect the primary is limited by the size but not SSTR2 expression, as tumors less than 2 cm are significantly more likely not to localize but do not have significantly different SSTR2 expression than their larger counterparts.

Octreoscan – Tc99m based

In one study, it was shown that sensitivity and negative predictive
values of Tc-99m-Octreotide scan is significantly higher than that of CT
and MRI. Using Tc-99m instead of In-111 had several advantages that
include better availability, cheaper and higher quality images. In
addition, to less radiation exposure to both patients and nuclear
medicine personnel.  In the absence of Ga68 PET, this could prove a reliable alternative.  Please note this scan is completed in a single day vs In111 Octreotide time of 2-3 days.

Ga68 PET (or SSTR PET in general)

The newest somatostatin receptor-based imaging modality, although it has been around for some time, particularly in Europe. The most common of these labeled analogs are 68Ga-DOTATOC, 68Ga-DOTANOC and 68Ga-DOTATATE. They may be known collectively as ‘SSTR-PET’.  Additionally, the DOTATATE version may often be referred to as NETSPOT in USA but technically that is just the commercial name for the radionuclide mix.

Read more about Ga68 PET scans by clicking here

These peptides are easier and cheaper to synthesize than standard octreotide-analog based ligands, boast single time point image acquisition compared to 2 or 3 days with Octreoscan. Its superior spatial resolution derives from the fact that it measures the radiation from two photons coincidentally. SPECT, in comparison, measures the gamma radiation emitted from one photon directly. This results in different limitations of detection – millimeters for 68Ga-PET compared with 1 cm or more for SPECT. There are a few choices of ligands with this type of imaging, but the differences lie primarily in their SSTR affinities – all of the ligands bind with great affinity to SSTR2 and SSTR5. 68Ga-DOTANOC also binds to SSTR3. Despite these differences, no single 68Ga ligand has stood out as the clear choice for use in NETs. As with standard somatostatin receptor-based imaging, these 68Ga-PET studies are fused with CT to improve anatomic localization.

Comparison studies between 68Ga-PET and standard imaging techniques (CT, OctreoScan) have universally demonstrated the superiority of 68Ga-PET in detection of NET primary tumors and metastases. Two early studies compared 68Ga-DOTATOC to standard somatostatin imaging (SRS)-SPECT and CT. Buchmann et al. reported that 68Ga-DOTATOC detected more than 279 NET lesions in 27 patients with histologically proven NETs, whereas SRS-SPECT detected only 157. The greatest number of lesions were detected in the liver. 68Ga-DOTATOC found more than 152 hepatic lesions, while SRS-SPECT found only 105, resulting in a 66% concordance rate between the two modalities. The concordance for abdominal lymph nodes was worse at 40.1%.  Cleary these advantages are going to impact on treatment plans, some needing to be altered.  In addition, 68Ga-DOTA PET imaging can be used to determine which patients might benefit from use of Somatostatin Analogues (Octreotide/Lanreotide) and PRRT – you can read more about this integrated and potentially personalised treatment in my article on ‘Theranostics‘ – click here.

It’s worth pointing out that SSTR PET is replacing previous types of radionuclide scans, mainly Octreoscan (Indium 111) and is not replacing conventional imaging (CI) such as CT and MRI etc.  Whilst SSTR-PET has demonstrated better sensitivity and specificity than CI and In-111, there are specific instances in which SSTR-PET is clearly preferred: at initial diagnosis, when selecting patients for PRRT, and for localization of unknown primaries. For patients in which the tumor is readily seen on CI, SSTR-PET is not needed for routine monitoring.  The Journal of Nuclear Medicine has just published “Appropriate Use Criteria for Somatostatin Receptor PET Imaging in Neuroendocrine Tumors” which gives guidance on it’s use – issued by the Society of Nuclear Medicine and Molecular Imaging (SNMMI).

Other PET Scans

18FDG PET

18-Fluoro-Deoxy-Glucose PET (FDG PET) is used to detect malignancy for a variety of tumor types. Unfortunately, its utility has not been borne out in NETs, as the majority of NETs tend to be relatively metabolically inactive and fail to take up the tracer well. However, high-grade NETs are more likely to demonstrate avid uptake of 18FDG, giving these scans utility in identifying tumors likely to display more aggressive behavior.

18F-FDOPA PET

The use of Fluoro-18-L-Dihydroxyphenylalanine (18F-FDOPA) in PET was developed in the 80’s for the visualisation of the dopaminergic system in patients with degenerative disorders, such as Parkinson’s Disease and related disorders. The first publication on the use of 18F-FDOPA PET for brain imaging was in 1983, which was followed by many others on the use of 18F-FDOPA PET for the diagnosis of Parkinson’s disease. Years later, in 1999 the first publication on the use of 18F-FDOPA PET for imaging of neuroendocrine tumour appeared. The value of 18F-FDOPA PET has now been proven for the diagnosis and staging of many neuroendocrine tumours, brain tumours and congenital hyperinsulinaemia of infants.

18F-FDOPA is accurate for studying well differentiated tumours. However the difficult and expensive synthesis have limited its clinical employment. It currently can be successfully used for imaging tumours with variable to low expression of somatostatin receptors (SSTR) such as Medullary Thyroid Carcinoma, Neuroblastoma, Pheochromocytoma), and others that cannot be accurately studied with Somatostatin SSTR scans such as the OctreoScan (Somatostatin Receptor Scintigraphy (SRS)), which uses the ligand 111In-DPTA-D-Phe-1-octreotide or the newer 68Ga DOTA-peptides.

I-MIBG

Radioiodinated (123I) metaiodobenzylguanidine (MIBG) is an analog of norepinephrine that is used to image catecholamine-secreting NETs such as pheochromocytomas, paragangliomas and glomus tumors. It can also be used to look for Neuroblastoma in children. In patients with functional pheochromocytomas or paragangliomas, this modality has a sensitivity of 90% and positive predictive value of 100%. However, it has limited use in Gastrointestinal (GI) NETs, as this modality was positive in only 49.1% of patients. In the same cohort of patients, OctreoScan was positive in 91.2%. As an imaging tool, this study is best used to confirm a diagnosis of pheochromocytoma or paraganglioma and define the extent of metastatic disease in these tumors. (Note – the Ga68 PET is rising in prominence though). Its most practical use in GI NETs may be to determine whether patients with metastases may benefit from treatment with 131I-MIBG (a form of radiotherapy).

Miscellaneous Scans

Parathyroid Scan – Sestamibi

Sestamibi scanning is the preferred way in which to localize diseased parathyroid glands prior to an operation. This parathyroid scan was invented in the early 1990’s and now is widely available. Sestamibi is a small protein which is labeled with the radio-pharmaceutical technetium99 (Tc99m). This very mild and safe radioactive agent is injected into the veins of a patient with hyperparathyroidism (parathyroid disease) and is absorbed by the overactive parathyroid gland. Since normal parathyroid glands are inactive when there is high calcium in the bloodstream, they do not take up the radioactive particles. When a gamma camera is placed over the patient’s neck an accurate picture will show the overactive gland.  Only the overactive parathyroid gland shows up…a very accurate test.

The Sestsestamibiamibi scan will display the hyperactive gland which is causing hyperparathyroidism in about 90 percent (90% sensitivity) of all patients. If the Sestamibi does show the hyperactive gland it is almost always correct (98-100% specificity). It takes approximately two hours to perform the Sestamibi scan after it has been injected. Pictures of the neck and chest are usually taken immediately after the injection and again in 1.75 to 2.0 hours (shown above). Newer techniques allow for more complete two and three dimensional images to be obtained of a patient’s neck. This technique is called SPECT scanning (Single Proton Emission Computerized Tomography) but it is usually not necessary.

Skeletal Scintigraphy (bone scan)

Quite often, bone metastases in NETs will be found via conventional imaging or special to NET nuclear scans such as Ga68 PET or MIBG.  However, a bone scan can often find them or confirm findings of scans looking for NETs.

Skeletal scintigraphy is a special type of nuclear medicine procedure that uses small amounts of radioactive material to diagnose and assess the severity of a variety of bone diseases and conditions, including fractures, infection, and cancer.

Nuclear medicine imaging procedures are non-invasive and — with the exception of intravenous injections — usually painless medical tests that help physicians diagnose and evaluate medical conditions. These imaging scans use radioactive materials called radiopharmaceuticals or radiotracers. Radioactive energy emitted from the radiotracer is detected by a special camera or imaging device that produces pictures of the bones called scintigrams. Abnormalities are indicated by areas of abnormal bone that take up more or less of the radiopharmaceutical which appear brighter or darker than normal bone on the scintigram.

Because nuclear medicine procedures are able to image the functions of the body at the molecular level, they offer the potential to identify disease in its earliest stages as well as a patient’s response to therapeutic interventions. In fact, a bone scan can often find bone abnormalities much earlier than a regular x-ray exam.

Taking the camera inside and directly to the Tumour

Of course there are other ways to “see it” via several types of Endoscopy procedures – taking the camera to the tumour.  Read my article about this by clicking here

A look to the future of PET Scans

explorer pet scan

Just imagine something which is 40 times better than current PET scan technology?  That’s what the scientists are working on now.  Here’s an example called “EXPLORER“.  Clearly there are more answers required in order to see if this is suitable for use with NETs (i.e. will it work with our radionuclide tracers etc) but it is very exciting and like something out of Star Trek.  A little bit of me is worried about ‘overdiagnosis’ so interpretation of something that detailed will be very important to avoid unnecessary worry. Read more here and there is a later update here.  Check out this cool video of the 3D images:

Summary

If you can see it, you can detect it.

Sources:

1. Imaging in neuroendocrine tumors: an update for the clinician, Maxwell, Howe,

2. Appropriate use Criteria for Somatostatin Receptor PET Imaging in Neuroendocrine Tumors,

3. Radiology for Patients,

4.  Useful video from NET Research Foundation about which scans to use for which job.  CLICK HERE to watch.

5.  Useful video summary from the NET Patient Foundation describing the different scans for NET Cancer and what to expect.  Worth a look.  CLICK HERE for the scan video

Sooner we can ALL get access to the latest radionuclide scans the better – this is currently an unmet need in many countries.

If you are any doubt about which type of scan is best for you and their availability, please consult your specialist.

Scanning is a key diagnostic and surveillance tool for any cancer.  Even though you have elevated bloods or urine (….or not), a picture of your insides is really like a thousand words…. and each picture has a story behind it.  Scanning can be a game changer in the hunt for tumours and although scans can’t (yet) confirm the cancer type and grade, they certainly help with that piece of detective work and are key in the staging of the cancer.

When I read stories of people in a difficult diagnosis, I always find myself saying ‘a scan might resolve this’ and I always suggest people should try to get one.  Even in the case of a story about late diagnosis or a misdiagnosis, I find myself thinking ‘if only they had done a scan earlier’.  Despite what you read on NET forums, a CT scan will normally find some evidence of most tumour activity.

However, scans are not an exact science…..not yet!   Apart from human error, sometimes tumours are too small to see and/or there are issues with ‘pickup’ (i.e. with NETs, nuclear scans need efficient somatostatin receptors).  However, technology is improving all the time and you can read about this in my blog Neuroendocrine Cancer – Exciting times Ahead.

As for my own experience, I was very lucky.  I managed to get a referral to a specialist early on in my diagnosis phase. He looked at the referral notes and said “what are you doing this afternoon”. I replied “whatever you want me to do”.  He wanted to scan me.  He didn’t know I had cancer but his instincts led him to believe he needed to see inside my body. The scan results were pretty clear – I had a metastatic Cancer and further checks were now needed to ascertain exactly what it was. So I took my seat on the rollercoaster.  Here’s something I always say I believe is so much better than the  impractical early diagnosis messages that seem to pervade our community:  If your doctors don’t suspect something, they won’t detect anything and I believe this is a very frequent outcome of many diagnoses for many cancers (not just NETs).

There’s frequent discussion about the best types of scans for different types of NETs and even for different parts of the anatomy.  This is correct and there’s also different views on the subject (including in the medical community),  However, a few well known facts that can be gleaned from authortative NET sources. I found this useful video summary from the NET Patient Foundation describing the different scans for NET Cancer and what to expect.  Worth a look.

Sooner we can all get access to the latest radionuclide scans the better!

CLICK HERE for the scan video

Thanks for reading

Ronny

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The 5 E’s (of Carcinoid Syndrome)

Guidance and Risk Management
Guidance and Risk Management

Since my diagnosis, I seem to have been in a perpetual learning phase!  What not to do, what not to eat, what not to read!  However, a couple of years ago, I came across a list of ‘E’ words (5 of them) which is a handy reminder for Carcinoid Syndrome patients, particularly those whose symptoms are not under control.  When I say “carcinoid syndrome” in this article, I only mean the syndrome that is caused by what was once called “Carcinoid Tumors”, i.e. mainly serotonin secreting types but include tumours which are well differentiated found in the small intestine, appendiceal, rectal, lung, and one or two other less common places.

There are many variations of this list but this is my take!  I suspect some of this also applies to other types of NETs and other NET Syndromes.

On analysis of this list, it struck me that I was aware of the issues and their potential effects and I’m certain there is science to substantiate the content. These E’s are apparently the most common ‘triggers’ for Carcinoid Syndrome.  Clearly, they are not going to have the same effect on every patient e.g. I have the occasional drink of ‘Ethanol’ and I always enjoy it, I go for long exhausting walks and I always feel great after.  I had dental treatment without any precautions before I was aware of the risks …….. nothing happened!  Before I was treated, stressful meetings at work would make me flush though!  As for eating – well that’s another couple of blog’s worth!   (see the Diarrhea Jigsaw and Nutrition Blog 4 – Food for Thought)

The 5 Es are, however, very important, as a severe attack of Carcinoid Syndrome symptoms could be debilitating and life-threatening and I’m fairly certain the list was compiled with this in mind.  Some people are more affected by Carcinoid Syndrome and this is not necessarily related to the extent or aggressiveness of their disease.  Some people just react differently.  An extremely severe attack of Carcinoid Syndrome can also be known as a ‘Carcinoid Crisis’ which is very dangerous on the operating table due to the effects of anaesthetics  – thus why many NET patients may be infused with somatostatin analogues (usually Octreotide) prior to and during surgery or other medical procedures.  There’s a lot of excitement generated around the term ‘Carcinoid Crisis’ but it is generally uncommon.

I’m not saying the 5Es should be ignored but NET Cancer is complex and most things need to be read in the correct context. What works for some may not work for others. There can also be confusion surrounding the source of symptoms, i.e. are they syndrome or something else?  This is why I believe NET patients need to answer some key questions when considering the risks associated with the 5 E’s:

  • Are you currently syndromic?   If you are, then the 5 ‘E’ list is probably very good advice but interpreting the advice in the correct context remains important.
  • Are your syndrome related biochemistry results normal (e.g. 5HIAA)? Normal readings (in range) tend to mean the syndrome is under control and many people who were diagnosed with a syndrome may actually be non-syndromic following treatment.
  • Have you had treatment or are having treatment likely to produce side effects which might be confused with Carcinoid syndrome? For example, surgery can be the long term cause of diarrhea and other issues. Despite the role of somatostatin analogues, these could also be the root cause of certain reactions.
  • Do you have any other illnesses?  If yes, do these other illnesses produce effects similar to carcinoid syndrome? e.g. asthma, diabetes, rosacea, thyroid disorders, vitamin & mineral deficiencies, malabsorption, gut bacterial imbalance.  Sorting out the symptoms can be a jigsaw with a missing piece sometimes.

The vagaries of this disease will no doubt throw up some exceptions and additions. There will be patients who have no syndrome but have elevated biochemistry and vice versa!  Additionally, there will be patients who have had surgery and/or are being treated with somatostatin analogues but will still be syndromic in varying degrees of severity.

The so-called ‘5 Es’ are as follows:

Epinephrine: This was a new piece of information for me and I only discovered this as a potential problem when I started monitoring some of the USA Facebook forums.  This does not appear to be that well-known in UK. Epinephrine (commonly known as adrenaline) is often used in dentistry mixed with a local anaesthetic. I won’t risk this, so I’ve instructed my Dentist to place a note on my record asking for epinephrine not be used (and clearly I’ll remind them each visit!). According to NET guru Dr Woltering, plain novocaine, carbocaine or plain marcaine are preferred.  You should also check that your anaesthetist for any procedure you may be undergoing is aware of your carcinoid syndrome. However, the danger is not just with dentistry work.  Any anaesthesia is risky.  Check out my post ‘carcinoid crisis’.

For those who have standby ‘Epi Pens’, I did read the following statement on the Carcinoid Cancer Foundation website:  “ …….. one exception is the administration of epinephrine in the case of an allergic anaphylactic reaction (i.e. a bee sting), so it cannot be avoided in this case, just make sure that Octreotide (Sandostatin) is also available“.  This advice is also extremely relevant to Pheochromocytoma and Paraganglioma patients who may be a high risk of intraoperative hypertensive crisis.

Eating: This is very individual.  Certain foods or large meals can be difficult, particularly if you have had any gastrointestinal surgeries. I keep a personal diary trying to identify things that upset my system. I try to find some balance between what I know is good for me and also what I know I enjoy. For example, I found that very large meals do not agree with my ‘new plumbing’. If I eat a lot of sweets, I’ll also suffer …..so I just eat a little – check out my  blog post Chocolate – The NET Effect.

Personally speaking, I’m fairly certain the vast majority of my issues are related to my treatment (past and present) rather than being provoked by Carcinoid Syndrome, i.e. if I rush to the toilet after a meal, it’s not syndrome, it’s a reaction of my compromised digestive system. So with this in mind, I try to reduce those things but additionally strike a balance between quality of life and excessive and rigid adherence to some of the guidance out there (see below) – as I said above, interpretation and context is important. My compromised system cannot deal with big meals so I ‘graze’ most of the day and then eat a small to medium-sized meal in the evening. I’ve been doing this for 3 years and reduced my visits by 300% without any special or expensive medication.

In my blog Nutrition Blog 4 – Food for Thought, I’ve linked to authoritative sources on potential diet triggers.  I’m not suggesting you cut out all of the foods on these lists (you won’t last long!). Some can indulge in those foods and some cannot. For example, chocolate and caffeine (tea/coffee) are on the lists but I eat/drink those frequently (in moderation) and have no problem. It’s a case of testing things out.  I like to describe my eating as ‘The Risk Management of my Quality of Life’. By the way, no-one is suggesting that a NET patient with carcinoid syndrome (and don’t forget this is only one syndrome of many with NETs) should stop eating foods high in the offending amines or are precursors to serotonin (e.g. tryptophan).  They do not make tumours grow (a myth) but just make sure you adhere to the dietary restrictions for any 5HIAA test.

Emotions:  Stressful situations can cause symptoms to flare up. While it is difficult to avoid all stress (work, home, commuting, etc), it is helpful if you can manage or reduce it. Like eating, this is a very individual area. From personal experience, I know stress can exacerbate carcinoid syndrome. Before I started my treatment, I was regularly flushing in meetings at work (….. think boxing matches!). After my treatment, stress was definitely a factor causing increased bowel motility.  I’ve removed a lot of stress from my life and it helps. You may need to be ruthless in managing this aspect of your illness.

Exercise:  Exercise is extremely important for overall health and well-being and I know quite a lot of NET Cancer patients who exercise regularly without issues. It can, however, trigger carcinoid syndrome if you overdo it – it is, however, like eating, a very individual thing. I take the view that ‘zero’ exercise might potentially be an even higher risk. Even a walk around the garden or gardening is exercise. When I was at work, I would walk to see people rather than phone them. Sometimes I walk to town rather than drive, it all adds up! I have evidence from my own exercising regime proving in my case that exercise can reduce the knock-on effects of some of the other E’s (emotions and eating) and/or the side effects of treatment – check out my blog entitled Exercise is Medicine.  Those who are syndromic and/or have other conditions to manage are probably best to take medical advice on how much exercise they need to do.

Ethanol (alcohol, liquor): Many NET patients have difficulty tolerating wine, beer and spirits (hard liquor). I was never a big drinker so for me it was easy to go almost teetotal. I do have the occasional beer but very infrequently and normally on holiday – I personally don’t get any issues with the odd beer but again this is trial and error.  I really enjoy my beer when I celebrate my Cancerversaries. Also check out my blog Alcohol – the NET Effect

Summary

I’m sure there could be a 5 A’s to 5 Z’s list of things to avoid but as I said above, this needs to be balanced with what the actual risks for you are and if you’re like me, quality of life. If you read most Facebook closed group or forums, you will always find at least one person is affected by something which affects no-one else. Please note this article is just my own appreciation of these issues and I emphasise once again that everyone has different experiences. I do, however, think it’s important to consider any secondary illnesses, effects of surgery and biochemistry results (or indeed a combination of one or more of these factors). Everything in life involves some kind of risk management and if you are totally risk averse, then you are unlikely to have much of a life (or a diet!).

It’s not easy but my daily diary helps me assess trends and work out what things upset me more than others – I can then reduce or eliminate. You need to tailor your own advice perhaps with the help of a doctor and/or dietician versed in NET Cancer.  I also have some related posts on the subject of vitamin and mineral deficiencies, malabsorption and probiotics – check them out as the problems associated with these subjects could potentially look like a worsening of carcinoid syndrome and lead to unnecessary worry and unnecessary treatment.

For most, Carcinoid Syndrome can normally be controlled by the use of debulking surgery and/or somatostatin analogues (Octreotide/Lanreotide).  However, there is a new drug called ‘Teloristat Ethyl’ (XERMELO) which looks like it may provide supplementary treatment for patients whose carcinoid syndrome diarrhea is not adequately controlled by somatostatin analogues. It’s an expensive drug and comes with side effects so you need to be sure it’s your syndrome causing the problem before you commit to a prescription.

Thanks for reading

Ronny

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NET Cancer Blog – 2015 in review

The WordPress.com stats team have prepared a 2015 annual report for my blog.  Special thanks to those who got a mention! Why not review my posts which received a 2015 Mention in Despatches ?

Here’s an excerpt:

Madison Square Garden can seat 20,000 people for a concert. This blog was viewed about 62,000 times in 2015. If it were a concert at Madison Square Garden, it would take about 3 sold-out performances for that many people to see it.

Click here to see the complete report.

Neuroendocrine Cancer Surgery – a patient experience (part 1)

Neil
Chris and I with our friend and hero Mr Neil Pearce – my surgeon.

First Surgery – 8th – 26th November 2010

Memories of my 18 day stay in hospital from 8 – 26 Nov 2010, are not only reminding me of how important that particular treatment was to be, but also how surreal it felt at the time. Some of it is still a blur, particularly the early days where the morphine was in control.  For many NET patients, surgery can be a mainstay treatment, even for those with metastatic disease.  In fact, I now know from my own research that NET Cancer is one of a small number of cancers for which surgical debulking can in many cases confer some survival advantage in a metastatic scenario. However, the nature of Neuroendocrine Cancer means that treatment and surveillance will need to continue for many patients.

Prior to being diagnosed with Cancer, my experience with hospitals was very limited and I had always been a tad squeamish when it came to routine injections. So having major surgery with a projected 10-14 day stay in hospital was a massive challenge. However, I remember being fairly relaxed leading up to this event.  I suspect I had accepted my situation; and that a combination of pragmatism and trust in my surgical team had conquered any fear.

The surgery, which lasted 9 hours, was really in two parts, firstly to get rid of my primary in the small intestine plus any accessible locoregional stuff.  Basically the surgeon removed 3 feet of my small intestine, carried out a right hemicolectomy, and also removed ‘oodles’ of lymph nodes in the mesenteric region, with careful blood vessel reconstruction required. The second part comprised a careful removal of retroperitoneal fibrosis which was threatening major vessels.  This is an issue which has returned in 2018 and you can find an update by clicking here.

Spookily, I woke up from the anaesthesia just after midnight on 10th November, I woke up on NET Cancer Day.

I’ve not spoken too much about the first week in the hospital after surgery – mainly because some of the details remain scant. However, there are four stories I remember in some detail and they even make me smile, although I wasn’t laughing at the time!

Physio (a synonym for pain!)

One of the key things I remember was the extraordinary amount of tubes and other things connected to my body. Had I drawn a picture, it would have looked like the map of the London Underground. Some of them were taking things out of my body (temporary ‘toilet’, wound drains, etc) and others were for putting things in (drugs, pain killers, nutrition, etc). My legs had ‘circulating leg wraps’ to prevent the formation of blood clots. They were very uncomfortable and sweaty but important.  I appeared to be pinned very tightly to the hospital bed – any thoughts of escape were quickly subdued by the sheer weight of equipment.  It was, therefore, a total surprise to me how soon the Physiotherapists arrived to administer torture 🙂  The difficulty of arranging all the tubes in order that I could just even sit up led me to believe it would not be possible.  However, they persevered and I had of course forgotten, this is what they do for a living! Putting the tube issue to one side, the very act of sitting up and putting your feet on the floor with a 12″ north to south abdominal wound still repairing is one of the most difficult and painful things I’ve ever done (even after activating my Pain Controlled Analgesia (PCA) – more drugs!). However, and I say this in hindsight, this is a very important part of the healing process and patients need to be compliant!  I eventually got used to it and starting off with a walk around my bed, a walk to the nurse desk, a walk up and down the ward….. this eventually led to a walk around the hospital once some of the tubes were removed.  However, I was feeling so bad one day, I refused physio which resulted in a lecture from my surgeon (see photo above) later that night – the discussion ended with the words “You are a winner”. It helped as I sprinted up and down the ward corridor next day!  Isn’t it amazing how a kick in the ‘ass’ can also function as medicine?

Pain Control

The Pain Controlled Analgesia (PCA) button was never far from my hand.  After surgery, it isn’t completely painless, but the PCA does help.  It normally contains morphine which helps kill the pain but comes with other side effects including sleepiness (handy), foggy brain, inability to focus, strange dreams and on occasion mild hallucinations (I swear the people on the wall picture opposite my bed were moving!).  After a few days, this was replaced by drip fed paracetamol (I think).

Re-establishing the food trail

I hadn’t given this too much thought prior to the surgery but when they remove sections of your intestines (in my case the terminal ileum and the ascending colon), there has to be a new join (anastomosis) and this needs time to heal. This means a gradual and gentle return to normal eating.  One of the most annoying tubes was the nasogastric tube (NG tube).  I woke up with this tube already inserted but around day 3 it was removed whilst I was awake (a little bit scary).  However, I was sick a few times (quite scary), so it was re-inserted (a little more scarier than removal).  However, once it came out for the second time (still a little bit scary), they gave me a rather tasteless drink called ‘Fortisip’ which apparently had the proteins and nutrients I needed whilst I waited to move onto normal food. My first proper food after a few days was ‘heaven’ – chicken soup followed by ice cream and jelly (for North Americans, please note jelly is not jam!)

Re-establishing the ‘poop’ trail

Technically, this is just an extension of the ‘food trail’ info above.  However, a story that I have hardly ever recounted follows.  I think this was around day 15/16 Nov or thereabouts.  My surgeon kept quizzing me on ‘gut feelings’ i.e. burping, hiccups, wind etc.  I hadn’t realised he was working out when to offer some help re-establishing this element of my recovery.  I think I was late so some milk of magnesia was given one morning. That evening, nothing happened and so the night shift nurses were primed to offer me a ‘special’ suppository which I was assured would be a great help in moving things in the right direction.  I declined their very kind offer to carry out the ‘insertion’ instead opting for some dignity retention – there wasn’t much left at this point but I was determined to hang onto it!  As I was laying there, I quickly scanned the remaining tubes (by this stage, I was down to 5 or 6), I reached round and it very quickly dawned on me that this was ‘mission impossible’.  I rang the bell as a signal that sometimes practicality overrules dignity.  Like the physio thing above, I had forgotten that Nurses do this all the time.  Ten minutes later, the bell again summoned the nurses who helped me and my tubes to the toilet. The toilet/bathroom was to become a familiar place over the coming weeks.

Following the surgery and when I was mobile, I weighed myself and found had lost a complete stone (14 lbs).  Weight loss led up to my diagnosis and continues to be an issue today – read more here.

Part two covers the second period of my 18 day stay and can be read by CLICKING HERE

Almost 8 years later, please check out my new challenge – click here.

wego-blog-2018-winner

patients included

 

 

PRRT and the NHS England Cancer Drugs Fund

cost cutting vs life cutting?
cost cutting vs life cutting?

As of 4 Nov 15, PRRT was delisted from the NHS England Cancer Drugs Fund. Appeals were made but were rejected, despite the glowing results from the NETTER-1 trial.  Although a replacement system is now in place, PRRT remains barred from routine NHS use.

Please see the following post for the very latest on PRRT worldwide – CLICK HERE

I was extremely disappointed to learn of the decision to remove PRRT (Lutetium or Yttrium) from the Cancer Drugs Fund (CDF) as reported by the NET Patient Foundation. You can read the detail of the decision here: CDF Statement.  PRRT has regularly been described by NET specialists and patients as the “magic bullet” due to its potential to shrink or kill tumours.

This is the second Neuroendocrine Cancer treatment to be withdrawn this year, after the earlier decision on Everolimus (Afinitor) in April . In fact, the recent cuts to the CDF were described in the media as a “massacre” as the list was reduced by two-thirds.  You can see the current CDF list by clicking here.

The timing of these cuts is extraordinary and when you look at the output from recent trial reports presented at the Europetwo-thirdsCongress (ECC) for both Neuroendocrine Cancer related drugs recently cut:

Everolimus

The RADIANT-4 trial said that Everolimus had a significant effect in non-functional NETs which are very difficult to treat.  This is particularly important for Lung NETs as no treatment currently exists.  The RADIANT-2 trial had already proven the efficacy of the drug for advanced carcinoid (in conjunction with Octreotide) and the RADIANT-3 trial proved good data for treatment with advanced functional pNETs.  Read the report here.

PRRT – 177Lu-DOTATATE

The ECC also reported a significant finding from the NETTER-1 trial.  Treatment with the novel peptide receptor radionuclide therapy (PRRT) Lutathera significantly increased progression-free survival (PFS) over Octreotide LAR (Sandostatin) in patients with advanced midgut NETs.  It shows a PFS that has never been shown before in this type of cancer adding that this was significant because these patients have a real unmet medical need.

Lutathera is a 177Lu-DOTATATE PRRT that targets somatostatin receptors, which are overexpressed in about 80% of NETs, to deliver cytotoxic radiation directly to the tumor – See more by clicking here.

To fully understand the background to the problem, you need to understand both PRRT and the Cancer Drugs Fund and a quick primer on both follows.

What is PRRT?

For those who are not entirely sure what PRRT is, here’s a quick primer from The Society of Nuclear Medicine and Molecular Imaging:

Peptide receptor radionuclide therapy (PRRT) is a molecular therapy (also called radioisotope therapy) used to treat a specific type of cancer called neuroendocrine carcinoma or NETs (neuroendocrine tumors). PRRT is also currently being investigated as a treatment for prostate and pancreatic tumors.

In PRRT, a cell-targeting protein (or peptide) called octreotide is combined with a small amount of radioactive material, or radionuclide, creating a special type of radiopharmaceutical called a radiopeptide. When injected into the patient’s bloodstream, this radiopeptide travels to and binds to neuroendocrine tumor cells, delivering a high dose of radiation to the cancer.

The cells in most neuroendocrine tumors have an abundance (called an overexpression) of a specific type of surface receptor—a protein that extends from the cell’s surface—that binds to a hormone in the body called somatostatin. Octreotide is a laboratory-made version of this hormone that binds to somatostatin receptors on neuroendocrine tumors. In PRRT, octreotide is combined with a therapeutic dose of the radionuclides. Yttrium 90 (Y-90) and Lutetium 177 (Lu-177) are the most commonly used radionuclides.  

What conditions are treated with PRRT?

PRRT may be used to treat NETs, including carcinoids, islet cell carcinoma of the pancreas, small cell carcinoma of the lung, pheochromocytoma (a rare tumor that forms in the adrenal glands), gastro-enteropancreatic (stomach, intestines and pancreas) neuroendocrine tumors, and rare thyroid cancers that are unresponsive to treatment with radioiodine.

PRRT is an option for patients:
• who have advanced and/or progressive neuroendocrine tumours
• who are not candidates for surgery
• whose symptoms do not respond to other medical therapies.

The main goals of PRRT are to provide symptom relief, to stop or slow tumor progression and to improve overall survival.

These video’s on Nuclear Medicine are by Professor Val Lewington – the UK’s most experienced person on PRRT.  I was at this presentation and she is absolutely amazing. It’s slightly dated but still very current.  This presentation also covers Octreotide and Gallium 68 scans under the heading of Nuclear Medicine – if you are still unsure about PRRT or Nuclear Medicine in general, these videos are definitely worth a watch.

The Role of Nuclear Medicine in NETs

Q&A Sessions

This is also a great source of information maintained by NET Patients in the USA.  Click here

What was the Cancer Drugs Fund?

The Cancer Drugs Fund was money the UK Government has set aside to pay for cancer drugs that haven’t been approved by the National Institute for Health and Care Excellence (NICE) and aren’t available within the NHS in England. This may be because the drugs haven’t been looked at yet. Or it may be because NICE have said that they don’t work well enough or are not cost-effective. This was introduced as a ‘political statement’ by the then Conservative/Liberal Democrat coalition government in 2010/11.  The aim of the fund is to make it easier for people to get as much treatment as possible.

The Cancer Drugs Fund was for people who live in England. The governments of Scotland, Wales and Northern Ireland decide on how they spend money on health and so far haven’t decided to have a similar programme.

Worth noting that on 1 April 2013, NHS England took on responsibility for the operational management of the Cancer Drugs Fund (CDF). The NHS spends approximately £1.3 billion annually on the provision of cancer drugs within routine commissioning. The CDF was established as an additional funding source to this.

There was a national list of drugs available through the fund – you may have heard this called the priority list. If you met the conditions for a drug that was on the list, you should have been able to have it on the NHS if you live in England. The Fund would also have considered applications on behalf of individual patients for other drugs that are not on the list.  However, under the new system, Individual funding requests (IFRs) relating to cancer drugs will no longer be considered via the CDF process.  All IFRs relating to cancer drugs will now be considered using NHS England’s single, national IFR system, which was updated in January 2016.

The new system came info force on 29 July 2016 and you can read more if you click this link

Summary

Although the decision is shocking to most, it was not totally unexpected as the Government and NHS have been hinting for sometime that the costs of the fund need to be reined in.  In any case if was only ever a temporary arrangement until a another model could be put into place.  There is a political element as the fund was set up by David Cameron with healthcare experts suggesting that it made no sense as a response to rising drug prices.  Moreover, by topping up the fund, the same experts claimed this was making the manufacturers the real beneficiaries of the fund as they have been able to sell their drugs to the NHS at prices that are unaffordable (and therefore unsustainable) for the NHS.

UK NET patients who have advanced and/or progressive neuroendocrine tumours which cannot be removed by surgery and whose symptoms do not respond to other medical therapies, still need help.

Ironically, the UK seems to be intent on cutting provision of the treatment (at least for NHS patients) as the US is trying very hard to formally introduce it.  This is a disgraceful situation and advanced Neuroendocrine Cancer patients and those who may need this treatment in the future are being terribly let down.

I will keep this blog ‘live’ in order to add information as things progress.

Thanks for reading

Ronny
Disclaimer
My Diagnosis and Treatment History

Neuroendocrine Cancer Nutrition Series – Article 3 – Gut Health

OPINION.  Nutritional issues are one of the biggest challenges affecting most Neuroendocrine Cancer patients.  It is also a key factor in maintaining a decent quality of life and for most countries without adequate NET Specialist Dietitian support, it remains an unmet need. In this article, I’m discussing the use of probiotics to combat the potential issue of small intestine bacterial overgrowth (SIBO) in Neuroendocrine Tumours.  

When I first indicated this nutrition series was under construction, a few people got quite excited anticipating me to produce advice on what to eat.  However, that was never my intention. What people should or should not eat is such a varied problem (or solution?) that anything I said would only really be of help to those for whom it worked – this area is not an exact science. I’ve seen several ‘what to or not to eat’ publications/articles out there aimed at NET patients; some more up to date than others – all I would say is to interpret them carefully.

What my nutrition series actually covers is what causes the nutritional related issues and to a certain extent, try to work out how to tell if these issues are caused by either treatment or an associated syndrome, leaving fellow patients to make up their own minds about what to eat; or arm themselves with the necessary knowledge whether this applies to them or not.

The first two articles in the series were Article 1 – Vitamin and Mineral Challenges and Article 2 – Malabsorption. These remain popular and have a constants stream of views – no surprises as these are well known side effects of many types of NETs…… or at least they should be well known.

This particular article “Gut Health” is not as ‘clear cut’ or simple as the first two and I suggest you read Articles 1 and 2 first if you are not familiar with the issues.  Again I’m grateful to Tara Whyand (NET Specialist Dietician and researcher from Royal Free London) for some of the input below. Although I marked this with ‘Opinion’, some of it has references but I still decided to use ‘Opinion’ as the science is not yet 100%.

What is the “Gut” ?

When I first met my surgeon, I found one of his favourite words was ‘Gut‘.  Like me before diagnosis, many of you will have heard or used the word but in an intentionally non-medical context, e.g.  guts (bravery), ‘gut feeling’ or ‘gut instinct’ (intuition). I’ll return to that theme later but when you look at these contextual uses of the word, it’s no surprise why some scientists refer to our gut as a ‘second brain’.

I always thought the gut referred to just the ‘belly’ area but in medical parlance, the gut has a much bigger geography.  It is sometimes used interchangeably with the term Gastrointestinal (GI) Tract and stretches from the throat to the anus and is responsible (in the most general terms) for food intake, digestion/absorption,  waste processing and finally waste ejection.  NET patients should be familiar with the terms ‘foregut’, ‘midgut’ and ‘hindgut’ which are sometimes used to define the embryological origin and grouping of Neuroendocrine primary tumours, although the boundaries and constituent parts can vary from site to site.  The inclusion of certain anatomical locations as a sub-section of the gut is clearly for convenience rather than anatomical accuracy (e.g. Lung).

This is a massive subject but I wanted to ‘cut to the chase’ in this article and focus on the use of probiotics to combat the potential issue of small intestine bacterial overgrowth (SIBO) in Neuroendocrine Tumours.  The symptoms and signs of SIBO can be similar to they symptoms and side effects of treatment that many patients report anecdotally on patient forums.  I also found the science is complex and not really 100% tied down.

Probiotics

One of the first pieces of advice I was given after my initial surgery was to take probiotics – to keep up my stocks of ‘good’ bacteria.  I didn’t really understand why, I just complied. I started with the liquid drinks you can buy in most supermarkets and supplemented this by eating bioactive yoghurt.  I didn’t really notice any difference from either but the yoghurt was nice to eat!

Tara Whyand then confirmed this advice when I first met her in 2012 at a NET Patient conference.  In 2013 when I started looking for a new normal, I realised that the supermarket drinks and yoghurts were simply not enough good bacteria for my ‘new plumbing’, and decided to take a high-grade daily capsule containing 5 billion friendly bacteria multiple strains (Tara does recommend at least 2 billion and multiple strain).  Within weeks I was noticing a difference in bowel motility although I confess to changing other elements of my lifestyle at the same time given that I was embarking on finding my new normal.  Nonetheless, I sense probiotics are helping and I won’t be reducing or stopping them any time soon.  If you look at several NET specific dietician/nutrition presentations, most appear to promote the use of probiotics for NET patients.

Bacteria

One of the terms you find in this complex area is the ‘human gut microbiota‘, sometimes known as ‘gut flora‘. Our ‘gut’ harbours a complex community of over 100 trillion microbial cells, approx 3% of our body mass! The human gut microbiota is known to have an influence on every part of our body (including the brain…..) and disruption of this ‘community’ has been linked with several gastrointestinal conditions such as Inflammatory Bowel Disease (IBD) and obesity.

Probiotics are said to help keep the balance and mix of bacteria stable within the gut which can be affected by many different factors, including the use of antibiotics, aging, illnesses (such as IBD), following infective gastroenteritis and (of interest to NET patients) after cancer treatment or gastrointestinal surgery. {1}  Incidentally, the reference here is authored by Tara Whyand and Professor Martyn Caplin (a Neuroendocrine Tumour expert who also happens to be a Gastroenterologist). Useful reading if you have any of the conditions in the report or have had gut surgery (or like me you are a total geek!).  They are also frequently used in Irritable Bowel Syndrome (IBS).

Small Intestine Bacterial Overgrowth (SIBO)

Another interesting area of research into something called Small Intestinal Bacterial Overgrowth (SIBO), a condition where the small intestine is populated by an abnormal amount and/or types of bad bacteria. It follows that probiotics (good bacteria) may be useful in combatting this by helping to maintain balance.

So how does SIBO potentially and specifically affect NET patients?

  • It can be caused or exacerbated by abdominal surgery to stomach, duodenum, pancreas or via whipples, small & large intestine,
  • poorly controlled diabetes,
  • the long-term use of Proton Pump Inhibitors (PPI) (e.g. omeprazole and lansoprazole, etc). Several studies link to these drugs including this one,
  • possibly long term use of antibiotics which can kill good bacteria.Some evidence of surgical involvement can be found here – this link – particularly the bit about the prevalence of patients who have had an “abdominal surgery” or an “Ileocaecal valve resection”.  I guess that would include many NET patients?  (this is a big article so just focus on table 1 near the beginning).

Symptoms vary for everyone from watery diarrhoea suddenly starting 20 times a day to just bloating and wind in both directions, to nothing at all.  These symptoms are regularly reported by patients so working out the root cause might need some professional help.

Is there any testing for SIBO?

There is a test to check for SIBO is called the Hydrogen breath test. This test uses lactulose ingestion to measure the hydrogen in the breath. If SIBO is diagnosed, treatment is normally via antibiotics. However, advice is to leave a 2 hour gap between taking probiotics and antibiotics and a high dose multi-strain probiotic should be applied.  Our friend Tara has done some work on this alongside Professor Martyn Caplin which was featured at ENETS 2017.

ENETS Research – Assessment of Small Intestinal Bacterial Overgrowth (SIBO) in NET Patients Abstract #1698

Introduction: SIBO is not uncommon in NETs. Hydrogen Breath testing (HBT) using glucose may be more sensitive to proximal SIBO as glucose rarely reaches the colon. Many NET patients are likely to have distal SIBO however, as factors such as ileocecal valve removal apparently increase distal SIBO risk. Thus glucose BT alone may limit sensitivity for detecting SIBO in some NET diagnoses.

Aim(s): Assess likely risk factors for SIBO. Assess sensitivity of additional lactulose HBT and CH4 BT.

Materials and methods: Retrospective data (n=55) of NET patients undergoing HBT was examined. Twelve patients (12/55) who tested negative for glucose HBT but continued to have diarrhoea +/- wind had repeat BT using lactulose. These patients had both H2 & CH4 BT.

Results:
Midgut NET diagnoses were most frequently referred for BT (n=43, 78%). Twenty four (24/55, 44 %) had prior right hemicolectomy. Ten (10/24 ,42%) of those were SIBO positive. Ten patients were positive for HBT prior to being given the glucose substrate, they all had abdominal surgery in the past. Twelve patients who tested negative for glucose HBT had repeat testing using lactulose and measured both H2 and CH4 production. This led to an additional 3 (25%) positive results.

Conclusion:
Abdominal surgery, especially right hemicolectomy increases the likelihood of a positive glucose HBT. Glucose may still be sensitive in those with risk factors for distal SIBO. Additional lactulose use with H2 and CH4 measurement increases the sensitivity in diagnosing SIBO.

Conference:
14th Annual ENETS conference (2017)
Presenting Author: Tara Whyand

Keywords: nets, sibo, dysbiosis

My own Experience

I personally take a 5 billion dosage and am happy to recommend the source offline. However, in addition to obtaining from a reputable provider (i.e. in UK, MHRA approved supplier), there is evidence to suggest as long as it has some or all of the following strains that are widely available, they should provide benefit: Lactobaccilus plantarum, Lactobaccilus acidophilus, Lactobaccilus brevis, Bifidobacterium lactis and Bifidobacterium longum.

This article could have been 10 x longer!  I didn’t even get to the bit about the relationship between the gut and the brain – perhaps another day?

None of this should be considered medical advice.

Article 1 – Vitamin and Mineral Challenges.   This was co-authored by Tara Whyand, UK’s most experienced NET Specialist Dietician.  This blog provides a list of vitamins and minerals which NET Cancer patients are at risk for deficiencies, together with some of the symptoms which might be displayed in a deficiency scenario.

Article 2 – Malabsorption.  Overlapping slightly into Part 1, this covers the main side effects of certain NET surgical procedures and other mainstream treatments. Input from Tara Whyand.

Article 3 – ‘Gut Health’.  This followed on from the first two blogs looking specifically at the issues caused by small intestine bacterial overgrowth (SIBO) as a consequence of cancer treatment. Also discusses probiotics.  Input from Tara Whyand.

Article 4 – Food for Thought.  This is a blog about why certain types of foods or particular foodstuffs can cause issues.

Article 5 – ‘Pancreatic Enzyme Replacement Therapy’. The role of PERT (Creon etc) in helping NET Patients. Input from Tara Whyand.

You may also appreciate these articles where there is overlap:

The Diarrhea Jigsaw – different things can cause diarrhea, it’s not all about syndromes.

The Constipated NET Patient – yes they exist!

Very grateful to Tara for the input.

Other useful links which have an association to this blog:

{a} Read a Nutrition Booklet co-authored by Tara – CLICK HERE

{b} Follow Tara on Twitter – CLICK HERE

{c} Watch a video of Tara presenting to a group of NET Patients – CLICK HERE

{d} Now Watch Tara answering the Q&A from patients – I enjoyed this – NET patients are very inquisitive! CLICK HERE

Thanks for listening

Neuroendocrine Cancer Nutrition Series Article 1 – Vitamin and Mineral Challenges

Vitamins & minerals
Vitamins & minerals – the biggest QoL challenge for NET Patients?

Despite learning early on in my journey that nutrition was going to be a challenge, I sensed the initial focus of my treatment was on getting rid of as much tumour bulk as possible and then controlling (stabilising) the disease through monitoring and surveillance. Clearly I’m happy about that! However, it eventually became clear that the impact of this constant treatment/controlling, meant that some of the less obvious signs of nutrient deficiency were potentially being missed.

This is one of the key reasons I believe there is a gap in specialist follow on support for Neuroendocrine Cancer patients – at least in the UK. As I said in my article ‘I may be stable but I still need support, Neuroendocrine Cancer patients need specialist dietary and nutritional advice covering a much wider spectrum than most cancer patients. In this post, I also suggested that there does not appear to be enough research or support into these issues leaving many patients working out their own strategies post diagnosis and treatment.  However, I was delighted to see a study published in 2016 indicating a recognition of this problem.  The paper (click here), which was sponsored by ENETS Centres of Excellence (CoE) in UK, concluded that “Given the frequency of patients identified at malnutrition risk using MUST (malnutrition universal screening tool) in our relatively large and diverse GEP-NET cohort and the clinical implications of detecting malnutrition early, we recommend routine use of malnutrition screening in all patients with GEP-NET, and particularly in patients who are treated with long-acting somatostatin analogues“.  This amplifies the advice Tara has given many NET Patients in UK that regular blood checks of key vitamins at risk, particularly B12 and the fat-soluble ADEK (see more on this below).  Even those patients with very healthy diets can still succumb to these issues. Looking at the vast number of forum posts on this subject, perhaps this is also a problem outside of UK?

This is not just about what foods to avoid or eat in moderation, this is also about:

a. receipt of post surgical/treatment advice,

b. early knowledge and countermeasures for the side effects of ongoing and long-term treatment,

c. the intelligent use of supplements where they are applicable,

d. how to combat, treat or offset malabsorption and nutrient deficiencies caused by the complexities of their cancer and any treatment given.  Check out Blog 2 in this series which specfically looks at Malabsorption.  

e. how to deconflict these side effects with those of the various syndromes which can sometimes accompany Neuroendocrine Cancer.

In early 2011, shortly after my first major surgery and commencement of my monthly somatostatin analogue – Lanreotide (Somatuline), I started to notice a number of issues developing. I carefully searched for clues and I could see that some of my issues pointed to side effects from treatment (both from surgery and somatostatin analogues) and potentially some vitamin and mineral deficiencies. I had already been taking an ‘over 50‘ multivitamin tablet for some time before I was diagnosed and assumed I was already covered. Having analysed the issues I was experiencing at the time, I was specifically targeting B12 and my initial test score was just in range (i.e borderline). Surprisingly my multivitamin B12 content was 400% RDA – yet my blood test was borderline. That might explain some of the fatigue!

I later attended a fantastic patient day where I was introduced to the UK’s solitary Neuroendocrine Cancer specialist dietician (this was in 2012, things are improving in 2019). This subject was a revelation for me and I was alerted to the possibility that other vitamins and minerals could be at risk due to a combination of surgery and/or treatment, in particular the fat soluble vitamins A, D, E, K. Following a hastily arranged series of blood tests, I found my Vitamin D was insufficient and this has now been resolved through additional supplementation and more effort to absorb it through conventional means (i.e. the sun!).

I’m now on top of this issue through learning, understanding and basically becoming my own advocate. Please note this is a massive subject and the amount of information on the internet can be overwhelming.  Additionally, it is not an exact science and not everything will apply to every person.  Personally I would stick to sites where the advice is given by a nutritionist/dietician who is also experienced with Neuroendocrine Cancer.

I’m thankful to Tara Whyand who is an Oncology Dietician specialising in Neuroendocrine Cancer at the Royal Free Hospital.  Her research, advice and raising of these issues at patient meetings has been invaluable. As the only specialist in the UK (that I know of), she gets a lot of queries!  If you’re on twitter, you can follow Tara here:

https://twitter.com/LadyNourish

Even though I’ve had to limit this post to vitamin and mineral issues, it’s still much larger than what I normally produce.  Consequently, I’m planning further blogs on associated and overlapping subjects.

In the meantime, I’m very grateful to Tara for the input below:

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NET Patients are at Risk of Deficiencies

Over the past few years I have become more aware of vitamin and mineral deficiencies in NET patients, and the impact these can have on health and quality of life. When the focus of NET treatment is on eradicating and controlling the disease, the impact on nutrition, apart from obvious weight loss, means less obvious signs of micronutrient deficiency can be missed. Below is a list of nutrients which are those most at risk of becoming low enough to cause problems. It is important that the treatment of these deficiencies is discussed with your NET team so they can prescribe suitable doses.

Minerals

Magnesium

Magnesium blood tests are an unreliable measurement and there is no way of accurately measuring body stores.

Magnesium is a vital mineral required for the function structure of the human body. Prevalence of low blood magnesium levels varies from 7% to 11% in hospital patients and clinical magnesium deficiency is frequently observed in conditions causing steatorrhoea or severe chronic diarrhoea, and the degree of magnesium depletion correlates with the severity of diarrhoea and stool fat content. Signs of deficiency include low energy, fatigue, weakness, PMS, menstrual cramps, hormonal imbalance, insomnia, bone mineral density loss, muscle tension, spasms, cramps, cardiac arrhythmia, headaches, anxiousness, nervousness and irritability. If you think you could be deficient you must ensure you consume enough magnesium (375mg per day).

Zinc

Zinc levels are best measured using a combination of blood serum and urinary excretion levels.

Zinc affects the human body through a large number of channels affecting not only cell division, protein synthesis and growth, but also gene expression and a variety of reproductive and immunologic functions. Zinc deficiency is common in undernourished patients. The absence of sufficient levels of zinc in the human body is associated with a large number of adverse health outcomes, including lower immunity, alopecia, tiredness and impaired wound healing. If you are at risk of deficiency make sure you consume enough zinc (10mg per day). If you are clinically deficient your diet must be supplemented.

Iron

Iron deficiency (hypoferremia) and clinical iron deficiency anaemia is easily measured with a simple blood test.

Iron is essential for the formation of haemoglobin in red blood cells which binds oxygen and transports it around the body. Iron is also an essential component in many enzyme reactions and has an important role in the immune system. In addition, it is required for normal energy metabolism and for the metabolism of drugs and foreign substances that need to be removed from the body. Lower iron levels are common in NET patients and there may be several causes of this. Poor iron intake, dietary iron absorption-regulating factors (e.g., vitamin C and copper) or iron distribution factors (e.g. vitamin A), are believed to be causes. Patients may also lose iron due to blood loss from the bowel in intestinal or rectal NETs or after surgery. It may also be possible that diarrhoea in NETs causes malabsorption of iron in the intestine too. Symptoms include tiredness, paleness, thinning hair, impaired immunity and feeling breathless. If you are at risk of having lower than normal iron levels you must consume enough iron (14mg per day). If you are clinically deficient your diet must be supplemented.

Copper

Diagnosis of copper deficiency is based on low serum levels of copper and ceruloplasmin, although these tests are not always reliable.

Copper is an essential trace mineral that is required for human health. This micronutrient is necessary for the proper growth, development, and maintenance of bone, connective tissue, brain, heart, and many other body organs. Copper is involved in the formation of red blood cells, the absorption and utilization of iron and the synthesis and release of life-sustaining proteins and enzymes. These enzymes in turn produce cellular energy and regulate nerve transmission, blood clotting, and oxygen transport. Copper stimulates the immune system to fight infections, to repair injured tissues, and to promote healing. Copper also has an antioxidant effect against oxidative stress.  Gastrointestinal surgery can lead to malabsorption of copper and other micronutrients. Long term malabsorption of food from the gastrointestinal tract can also lead to copper deficiency which puts many more NET patients at risk.  Symptoms of deficiency include neutropenia, impaired bone calcification, myelopathy, neuropathy, and hypochromic anemia not responsive to iron supplements. If you are at risk of lower than normal levels of copper you must consume enough (1mg per day). If you are clinically deficient your diet must be supplemented.

Selenium

Selenium levels are measured using plasma selenium blood tests.

Selenium is an essential micronutrient in humans and functions in many biochemical pathways. Proposed antioxidant pathways of selenium, include the repair and prevention of oxidative damage, alteration of metabolism of carcinogenic agents, regulation of immune response and repair of DNA damage. It works alongside vitamin E and selenium levels are often low during cancer and in patients on long-term intravenous nutrition.  Symptoms of deficiency include muscle pain and tenderness.  Everyone is required to have 55 µg a day and if you are clinically deficient your diet will need to be supplemented.

Water Soluble Vitamins

B1-Thiamine

Thiamine is not usually tested as diagnosis is based on symptoms and a trial of thiamine supplementation. If a doctor is unsure, they will measure erythrocyte transketolase activity and run a 24-hour urinary thiamine excretion.

Vitamin B1, or thiamine is an essential B vitamin which is required for the breakdown of sugars and amino acids. Absorption of thiamine is greatest in the jejunum and ileum, but it is it is inhibited by alcohol consumption and by folic acid deficiency. The most common cause of deficiency is alcoholism, although states causing malabsorption such as gastrointestinal surgery are also a factor. It may also be possible that diarrhoea causing malabsorption of nutrients from the intestines could put a patient at NET patient at risk of deficiency. Symptoms initially include fatigue, irritability, poor memory, sleep disturbances, anorexia, and abdominal discomfort. When more severe it involves hospitalisation due the effects on the nervous system and heart.

Patients who are at risk of deficiency must consume enough thiamine (1.1mg thiamine per day). Patients who are deficient must have their diet supplemented.

B3-Niacin

Niacin is not usually tested but may be useful to confirm diagnosis using urinary excretion of N 1 -methylnicotinamide (NMN).

Niacin also refers to both nicotinamide and nicotinic acid and is required as part of the way energy is produced by the body.  When carcinoid tumours produce hormones such as serotonin, these patients suffer from carcinoid syndrome. These are symptoms such as flushing, diarrhoea, wheezing and damage to heart valves (carcinoid heart disease). When the tumours make large amounts of serotonin, the amino acid, tryptophan, gets used up. When tryptophan stores are low it cannot be converted into the vitamin niacin, which may then cause deficiency. In a NET study, 28 per cent of patients with gastroenteropancreatic /carcinoid tumours and carcinoid syndrome were niacin deficient. Patients without carcinoid syndrome did not have niacin deficiency.  Niacin deficiency can also be caused by cirrhosis and diarrhea. Niacin deficiency leads to pellagra, the typical symptoms of which are diarrhea, dermatitis and dementia. All patients with carcinoid syndrome must take a nicotinamide containing supplement to treat and prevent this deficiency and it is a good idea to get enough niacin if you are at risk of deficiency for other reasons (approximately 40mg nicotinamide a day). Niacin or niacinamide may cause flushing!

B6-Pyridoxine

Vitamin levels are not usually tested but measurement of serum pyridoxal phosphate is most commonly used.

Vitamin B6 comprises 3 forms: pyridoxine, pyridoxal and pyridoxamine, and has a central role in the metabolism of amino acids. It is involved in the breaking down of glycogen into glucose. In addition, vitamin B6 plays a key role in metabolism of neurotransmitters, such as dopamine and serotonin, and it ensures efficient functioning of the immune system and making of red blood cells. The symptoms of vitamin B6 deficiency are local inflammation of the skin and dysfunction of the nervous system. Some NET patients may be at risk of deficiency due to malabsorption in the intestines and undernutrition.  If you are worried you may have lower levels make sure you consume enough (1.4mg per day). If you are deficient you diet must be supplemented.

B9-Folate

Serum folate reflects folate status unless intake has recently increased or decreased.

Folic acid is the synthetic form of folate. It is used in supplements and for food fortification. Folate functions together with vitamin B12 to form healthy red blood cells. It is also required for normal cell division and the normal structure of the nervous system.  It is possible to become deficient in folate due to malabsorption of nutrients in the intestine through diarrhoea and other malabsorption states such as surgery. If you are worried you may be at risk of deficiency ensure you get enough folate/folic acid (200 µg per day). If you are deficient your diet will need to be supplemented.

B12-Cobalamin

Vitamin B 12 must be measured alongside complete blood count and folate levels.

Cobalamin plays a role in DNA synthesis and regenerates methionine for protein synthesis. Low vitamin B12 levels have been observed in NET patients receiving somatostatin analogues and therefore monitoring of vitamin B12 levels is important during long-term therapy. Vitamin B12 deficiency has also been found to be common in type 1 gastric carcinoid NETs after Antrectomy and/or Gastrectomy. Patients with diseased or surgically removed ileums (end of the small bowel) and those who have bacterial overgrowth in the area are also at risk of Vitamin B12 deficiency. In addition, patients with insufficient pancreatic enzymes are also at risk of vitamin B12 deficiency as they play a key role in the steps before absorption occurs. If you are worried your levels may be low you must consume 2.5µg a day. If you are clinically deficient your diet must be supplemented, usually with regular injections.

Fat Soluble Vitamins

A, D, E and K

Somatostatin Analogues (Octreotide and Lanreotide) based injection treatments for a variety of NETs may cause deficiencies in some vitamins. This is because they may alter absorption of dietary fats which contain vitamins. Enzymes are usually released from the pancreas to break down nutrients such as fat, but pancreatic enzyme release can be reduced when somatostatin analogue medications are given.  When fat is not broken down properly, stools become pale/yellow, loose, greasy, foul-smelling or frothy and floating –‘steatorrhoea’. Your precious vitamins therefore end up in your toilet instead. One study followed 54 patients, who mostly had carcinoid tumours and were on somatostatin analogues for at least 18 months. It found that only one fifth of patients had visible steatorrhoea, but 6% were deficient in vitamin A, 28% deficient in D, 58% in E and 63% in K1. This shows that even if you don’t have visible signs of steatorrhoea, you may still be deficient in one or more vitamin!

A-Retinol

Serum retinol blood tests are the means of measuring vitamin A in the body.

Vitamin A is a fat soluble vitamin absorbed through the small intestine either as retinol or carotene, and then converted to retinyl palmitate which is stored in the liver. Normally the liver contains a 2 year store of vitamin A. Vitamin A deficiency has a wide range of ocular manifestations including conjunctival and corneal xerosis, keratomalacia, retinopathy, visual loss, and nyctalopia, or night blindness, which is the earliest and most common symptom. If you are worried about having low levels make sure you consume enough (800 µg per day). If you are deficient your diet will need to be supplemented.

D 3 –cholecalciferol

Your 25(OH)D levels can be measured with a simple blood test.

Cholecalciferol is a nutrient and hormone. Recent evidence for the non-skeletal effects (those apart from bone mineralisation) of vitamin D, coupled with recognition that vitamin D deficiency is common, has revived interest in this vitamin. Low vitamin D levels are linked to higher rates of several other cancers. Vitamin D is produced by skin exposed to ultraviolet B radiation and obtained from dietary sources, including supplements. Persons commonly at risk for vitamin D deficiency include those with inadequate sun exposure, limited oral intake, or impaired intestinal absorption from the diet (as above). The most recent evidence actually points out that the sun is not to be relied on as a source of vitamin D and oral intake is important. If you are worried you may have low levels you must speak with your doctor to arrange supplementation with or without a test.

E- α-tocopherol

Vitamin E can be tested by looking at the α-tocopherol level or ratio of serum α-tocopherol to serum lipids.

Vitamin E is a powerful antioxidant that can be regenerated by vitamin C after oxidation in the human body. It prevents damage of polyunsaturated fatty acids in cellular membranes. Signs of deficiency include dry skin and neurological symptoms. If you think you may have low levels make sure you consume enough (12mg per day). If you are deficient your diet will have to be supplemented.

K- Phylloquinone

Vitamin K deficiency can be measured by looking at the prothrombin time.

Phylloquinone is required for blood clotting and deficiency results in bleeding. Since this deficiency is common in patients with fat malabsorption due to severe liver disease and somatostatin analogue treatment it is important that you consume enough (75 µg per day). If you are clinically deficient you will need to receive supplementation.

Summary

Of course these are only the nutrients which are at risk of deficiency, there are many other nutrients and botanical extracts which may help patients with NET’s. It is vital that nutrition is considered for every patient with a NET and we hope one day each NET unit will have NET Specialist Dietitian to make this possible.

It is vital that nutrition is considered


Links to the other nutrition blogs:

Article 2 – Gastrointestinal Malabsorption.  Overlapping slightly into Article 1, this covers the main side effects of certain NET surgical procedures and other mainstream treatments. Input from Tara Whyand.

Article 3 – Gut Health.  This followed on from the first two blogs looking specifically at the issues caused by small intestine bacterial overgrowth (SIBO) as a consequence of cancer treatment. Also discussed probiotics.  Input from Tara Whyand.

Article 4 – Food for Thought.  This is a blog about why certain types of foods or particular foodstuffs can cause issues.

Article 5 – ‘Pancreatic Enzyme Replacement Therapy’. The role of PERT (Creon etc) in helping NET Patients.

You may also appreciate these articles where there is overlap:

The Diarrhea Jigsaw – different things can cause diarrhea, it’s not all about syndromes.

The Constipated NET Patient – yes they exist!

Very grateful to Tara for the input.

Other useful links which have an association to this blog:

{a} Read a Nutrition Booklet co-authored by Tara – CLICK HERE

{b} Follow Tara on Twitter – CLICK HERE

{c} Watch a video of Tara presenting to a group of NET Patients – CLICK HERE

{d} Now Watch Tara answering the Q&A from patients – I enjoyed this – NET patients are very inquisitive! CLICK HERE

Thanks for listening

Ronny

I’m also active on Facebook.  Like my page for even more news. Please also support my other site – click here and ‘Like’

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

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I may be stable (..ish) but I still need support and surveillance


cancer-patient-support

With incurable but treatable cancers such as metastatic Neuroendocrine Cancer, ‘Stable‘ is normally not the end of the matter, for many there is still a long road ahead and that road may not be straight or flat. The long road may be considered an advantage by some given that with very aggressive cancers, incurable can frequently mean terminal.  The surveillance must continue in case of a recurrence.

It’s important to understand that ‘Stable‘ simply means the disease is “under control” with tests and scans showing the cancer hasn’t changed over time.

One of the disadvantages of ‘incurable but treatable‘ is that Quality of Life (QoL) can in many cases be compromised due to the consequences of cancer and /or treatment. However, if specialist treatment, surveillance and support are all in place, things can gradually be adjusted to a new and hopefully tolerable ‘normal’. 

I also believe patient expectations need to be managed although improvements are still possible.  In my own experience, however, this does not happen overnight. Patients must be willing to accept a new normal or status quo on the basis that things are never likely to be the same again. Many patients with chronic conditions will have minor irritants and Neuroendocrine Cancer patients are no exception in this regard. 

HOWEVER …….. The specialist view of ‘stable’ will be looking at tumour and hormone makers.  The patient is likely to have a much wider view of ‘stable’ and it will include ‘quality of life’ markers. 

So ….what is stable for me?

Looking at my medical documents, I was not really considered ‘stable’ by specialists until 2 years after diagnosis. The measure of that is in scans and markers.  Nothing has grown since 2012 although I have a thyroid lesion being tracked on watch and wait.  My key NET markers have been solidly in range since 2012.  Today, my on-going monthly treatments are well organised, I’m in touch with my specialists and undergo several surveillance checks beforehand every 6 months currently. I get regular/normal illnesses and those are logged in my diary to look for any clues or associations with anything else. In between consultations, I can call in for urgent help if need be. Irregularities of concern to my ‘stability’ are checked, referred to other specialists if necessary and treated.  I feel well, I look well (but you should see my insides ….). I think I’m on top of things.

I think the UK (for example) is very well serviced with district NET Centres across the country each with specialists in Neuroendocrine Cancer and most include a dedicated NET Specialist Nurse – some areas are better served than others. In my opinion, NET Nurses can prove invaluable in on-going care scenarios. In fact, I was very pleased to see a NET Nurse attending and taking a greater role in my most recent MDT meetings.  I’m fairly certain other countries have similar setups.  Some countries may not be so fortunate and are struggling to get the right resources – I can see this on one or two ‘corporate’ Facebook and Twitter sites. Specialist NET Nurses are an extremely valuable commodity – they do brilliant work and we probably need more!  The same could be said for NET Specialist Dietitians who are key to providing quality of life improvements. In fact, I was delighted to see this recommendation at ENETS 2018 in Barcelona. 

recommend dietitians
More dietitians for NETs?

OK … I may be stable (ish) but I still need support!

However ……. my stability does NOT mean I’m complacent.  For minor issues, it’s always useful to talk to a medical professional, even on the telephone. I think of my GP (PCP) as a ‘virtual’ member of my Multi-Disciplinary Team (MDT) and I copy them into any important correspondence between myself and my Oncologist.  They are normally copied in coming the other way (if not I make sure they are). This is starting to return dividends. Whilst my GP is positioned to deal with most of my ‘irritants’, I still believe specialist assistance is required for many NET Cancer problems or any problem where there is potentially an overlap or risk of a connection. Being your own advocate is useful in these scenarios.  Patient-doctor communication is vital and I find it best to drive this myself. I’m lucky to have direct ‘as and when’ contact a specialist NET Nurse.  All NET patients should have the same.

The best advocate for you is YOU (or someone very close to you)

Although I still need constant surveillance, being stable allows me to focus on QoL and in particular trying to improve on my ‘normal’.  Whilst we are on that subject, did you hear the one about the constipated NET patient?  This article contains a summary of my attempts to gain a decent quality of life.

Although I read patient forums, I don’t necessarily rely on them a lot for my own issues. On sporadic one-off forum questions (…..and not forgetting that hundreds of symptom questions are related to ‘the gut’), the discussions can end up with many different and confusing answers. Plus there are so many patients who are at varying stages of their disease, use different types of healthcare systems, have had different treatments and have different types of NET, have other issues going on, it can end up as a tangled mess as people try to compare apples with pears.  To help with this issue, I created my own private Facebook group and I try to emphasise these issues through moderation. 

I will not compare myself to strangers on the internet
remember all patients are different

I like to do my own research as I want to be in control of my own QoL.  One of the most troublesome QoL issues for patients is diet and the digestive system generally (i.e. managing the gut). For many NET patients, particularly those who have had surgery and/or persisting syndrome, diet and nutrition is a  huge challenge as it can very often mimic other problems which can present with a wide range of ‘syndrome like’ symptoms such as fatigue, weight issues and even anxiety. More somatostatin analogues and other drugs might just be the wrong response in certain scenarios. I feel there is a huge gap in the follow-up treatment for people who suffer this as a consequence of their cancer. For example, and to the best of my knowledge, there is only a few dedicated and practicing Neuroendocrine specialist dietician in the whole of the UK (…..I’m willing to be corrected here). Some of you might be thinking that any dietician should be able to help? Although you would be correct to a certain extent, I personally do not believe this is the best or optimum solution. There are very specific issues with NET Cancer patients that are bespoke and complex to the point that conventional cancer diet practices may not fully apply. It’s not just about what you eat………..

NET Cancer patients need specialist dietary advice covering the whole spectrum from diet itself to the use of supplements where required, post-surgical advice, managing the long-term side effects of treatment, combatting and treating malabsorption and nutrient deficiencies caused by the complexities of their cancer or the consequences of their treatment. Personally, I think more resources and research in this area would be useful.

This gap is one of the reasons why I asked Tara Whyand (a dietician with specialist Neuroendocrine Cancer knowledge) to help me co-author a series of blogs to focus in on a few key areas.  I didn’t want to say what someone should or should not do, I wanted to say why this is an area to watch.  The ‘why‘ is important as it helps you in your efforts to distinguish the effects of a syndrome or a co-morbidity from the effects of your treatment (if applicable).  I find this knowledge helps me to think ‘outside the box’ rather than just accepting ‘it’s the syndrome.  I personally feel I’ve been able to harness this knowledge to improve my QoL.

Article 1 – Vitamin and Mineral Challenges

Article 2 – Malabsorption

Article 3 – Gut Health

Article 4 – Food for Thought

Article 5 – Pancreatic Enzyme Replacement Therapy (PERT) (includes a Q & A session with Tara Whyand)

The following blogs may complement this nutrition series:

The Diarrhea Jigsaw

The Constipated NET patient

Serotonin

I now take food with my pills

Thanks for reading

Ronny

I’m also active on Facebook. Like my page for even more news. Help me build up my new site here – click here and ‘Like’

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!


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Things are not always how they seem

9540384-large
Things are not always how they seem

In 2014, Chris and I walked 84 miles along Hadrian’s Wall on the English/Scottish border.  It was a fantastic experience and we met some really interesting people on our 6 day journey.  On the 4th night, I encountered a lady who was pretty rude. I wanted to say something but I was with Chris and other people were also present, so I kept quiet.  I later discovered this lady was autistic and I was therefore relieved I hadn’t responded to her initial rudeness. However, It got me thinking about the number of times I had perhaps been too hasty to judge people in the past without thinking about what’s going on inside their heads and bodies.

Visible Illness can have awareness benefits

Conversely in 2018, I was absolutely humbled when I met a Parkinson’s disease patient. I had ‘noticed’ Matt prior to meeting him, mainly because he had difficulty walking. When he was talking to me I had to really concentrate because his head, arms and legs were constantly going into spasm. His speech was also affected. Despite his very clear VISIBLE illness, I can say he is a fantastic advocate for Parkinson’s. He told us that he has no issues with people staring or looking at him as he makes his way around and that is his key marketing point – himself. He uses the fact that people notice/look/stare as opportunities to get talking to them and he is a living breathing advert for Parkinson’s. I had no idea Parkinson’s had these effects, I thought it was just the shaking hand thing that you often see on TV programmes.

So things are sometimes not what they seem with VISIBLE illnesses just as they are with INVISIBLE.

NETs can be invisible before and after diagnosis

I know from reading and participating in Neuroendocrine Cancer (NETs) forums that many patients with my own condition frequently encounter people who clearly do not understand much about the effects of NETs on someone’s body (and mind) and day-to-day living which for many can be described as a struggle.  I read one story about a lady who was accused by a co-worker of faking her cancer because she looked so well! How many of my NET patient readers have been told they look really well?  This is something frequently said to me and I now respond with the customary “Yes, but you should see my insides“.

Not many NET patients are subjected to the rigours of chemotherapy and I for one am thankful for that. However, many NET patients have had some ‘bad ass’ surgeries and will be treated for the remainder of their life with (at least) large anti tumour and hormone inhibiting injections and perhaps other side effect inducing drugs.  There is no 5 year or indeed any remission for many NET patients.  What is incurable has to be endured!  Moreover, they will be tested at regular intervals to ensure remnant tumours are ‘at bay’ and that no new ones have appeared. So the potential for a new or re-diagnosis is there at every single meeting with their specialist.  All of that comes with a price in terms of quality of life.

I’m not trying to compete with other cancers or chronic illnesses, I’m just saying that a NET patient who looks well, may not be well inside – body and mind!  Nor am I asking for pity – I am, however, asking for understanding.

toilet-sign-wall-of-china
Seriously!

When I read about some of the issues others deal with, I suspect I’m one of the luckier patients. I’m in reasonable condition and put up with a number of minor irritants which I suspect are due to the consequences of my cancer treatment rather than from Carcinoid Syndrome.  However, one thing that does scare me from time to time is stomach cramps. Hopefully I’m not tempting fate as they seem to be reduced this year.  However, when I suffer these, it does worry me, not just because they can sometimes be very painful and debilitating, but I know that I must go to the toilet ASAP. Handy if I’m in the house, not so handy if I’m on a plane, down town or anywhere where toilets are not in abundance.

NPF Toilet Card Back
NET Patient Foundation Card – on the back

I try not to let this problem stop me leading as normal a lifestyle as possible and as I said previously, it doesn’t really happen that frequently.  Long flights are one of the few times I take Loperamide (Imodium) and for long drives and trips down town, I’m simply reliant on toilet availability. Sometimes I find only the disabled toilet is available and when it’s urgent I have no qualms about using it. Some of them are locked and you have to go get a key and again I have no qualms about asking for access despite my outwardly healthy look – nobody has argued yet.  If necessary (sufficiently urgent), I’ll even ask to use the staff toilets in shops etc.  I do have a card in my wallet which I obtained from my friends in the NET Patient Foundation but I’ve not yet had to use it ‘in anger’.

Are you nodding your head at these issues?   I also suspect quite a few of you will therefore enjoy reading an article which has given me the inspiration and motivation to update this blog post.  It’s about a lady who has major abdominal issues through surgery and illness (inflammatory bowel disease (IBD)) and to me this sounds like a worse condition than many NET patients endure.  She too looks outwardly healthy but this illness is clearly a major disability. I’d like to think this type of incident is not that common but her response to it was magnificent, well written and apparently it went viral. What great publicity that must have been for IBD.

Read the post here, it’s brilliant:  CLICK HERE TO READ
 (p.s. ‘Loo’ is British slang for toilet)

Thanks for reading

You may also enjoy these similarly related articles:

Shame on you! – click here

I look well but you should see my insides – click here

Not every illness is visible – click here

You must be doing OK, you’ve not had Chemotherapy – click here

Not the stereotypical picture of sick – click here

An Ode to Invisible Illness – click here

Poker Face or Cancer Card – click here

Thanks for reading

Ronny

I’m also active on Facebook.  Like my page for even more news.  I’m also building up this site here: Ronny Allan

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!

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Every Day is NET Cancer Day!

Every day is NET Cancer Day

Opinion.  In 2014, I experienced NET Cancer Day (10 Nov) on a major scale for the first time since its inception. Prior to that, it didn’t really do that much for me.  Spookily I even woke up on 10 Nov 2010 after major surgery.  Read about this here – I even woke up on November 10th after major surgery.

The build up to these events normally doesn’t start in earnest until around 3 months prior to 10 Nov. On or around this day, people meet up, patient conferences and support meetings are held, thousands of tweets and Facebook posts are published, people make and eat cakes, and money is raised. I suspect awareness of NETs benefits but these things can quickly be forgotten outside the rather small world of NET Cancer patients, specialists, supporters and advocates.

If ‘N’ is equal to the amount of awareness you can physically do, then ‘N + 1’ is the amount of awareness you need. You can never have enough awareness.  For me, one day doesn’t cut it.  Some cancers have a whole month but they tend to be the big most common ones.

I’m in awe of those advocate organisations who organise these annual events and the patients who gladly join in to help by giving up their time (including NET Cancer Day and all its affiliate organisations).  There’s a lot of time and effort required.  It’s rather easy for me as I sit in my chair doing my bit – but I am doing it every day. A big advantage I have is that we now live in a connected world and there is an almost unlimited reach to a broad spectrum of people ranging from politicians to the worried well looking for a diagnosis. They all have something in common though …. they’re all connected to the internet and looking for information, looking for a feed.  Social media is really powerful but the message needs to be compelling to persuade someone to read my feed again and again.  I guess when you are marketing something on a face to face basis, it’s a different ball game but the principles of persuading someone to ‘read your feed’ are the same.

Having analysed 10th November activity and the week leading up to it, I think it was pretty much like last year, i.e. the same old tired old clichés and icons, together with out of date and inaccurate information which patients and patient advocate organisations share between each other.   I want new audiences and ones who will stick with NETs instead of just liking a tweet on November 10th.  This is what the NET Community needs too. I’m afraid cartoon animals in the most ridiculous scenarios are not going to attract long term support from outside the community. This is not a criticism of any person working for or fund raising for a NET patient organisation, I know they work very hard.  This is about the out of date and incoherent strategy.

Ronny Allan (right) meeting the Rt Hon Desmond Swayne TD MP in the UK Parliament
Ronny Allan (right) meeting the Rt Hon Desmond Swayne TD (now Sir Desmond Swayne) MP in the UK Parliament

Although I woke up on November 10th after my surgery in 2010, I only really woke up to NET Cancer Day (the event) in 2014 where I and others met and lobbied our respective  Members of Parliament at a NET Patient Foundation sponsored event.  I was also honoured to lobby side by side with my surgeon (Neil Pearce) who is also one of the Medical Trustees for the Foundation.  I felt that activity made a real difference and I was so enthused by this event, I decided to step up my own campaigning activity using my blog to push and push for more recognition of our disease. Attracting the notice of politicians is a good awareness tactic as long as the foot remains on gas peddle.  In regards interactions with politicians, as another example I’m always happy to see the annual state declarations of support in USA.

When I consider the PR campaigns of other cancer types, I admit to being a tad envious. For example in the UK, breast, lung, bowel and prostate cancers probably have more awareness ‘value’ in a single week, than NETs get in a single year. However, these are the ‘big 4’ cancers and as a consequence attract a lot of support (and therefore resources) and are backed by government public health campaigns (e.g. in the UK, the ‘be clear on cancer’ campaign covers most of these cancers).  OK, they have a lot of resources but one thing I see across the board in these campaigns is the lack of icon adulation you see in NET awareness – rather they focus on firmly on PEOPLE and I  believe that is part of their success.  

When I suggest to ditch the animal analogies, people say to me “what icon would replace it”. I simply say “why you even need to replace it” as we’re talking about adopting a coherent strategy. By the way, name another successful cancer strategy using an animal as their ‘cover page’.  Spoiler alert, there isn’t one. 

Because NETs is a less common disease, the necessary ‘clout’ needs to be as wide as possible and this means international efforts to supplement national campaigns, particularly for awareness and recognition.  But the strategy needs to be coherent, effective and up to date. Of course, we need to get patients on board because patient stories are vital, particularly (and accurately ….) in the national news and TV. Resources (people and cash) are always going to be an issue and some high-profile patients or ambassadors would be extremely useful but they tend not to want to get involved.  Read my Human Anatomy blog to understand more about the effects of this issue.

I strongly believe we need new audiences – nationally and internationally.  To be more attractive to the ‘outside’ and new audiences, we also need a convincing and compelling ‘line’.  By ‘line’ I don’t just mean an icon or a phrase, I mean a whole ‘PR’ package. It’s very difficult for rare and less common cancers to get high-profile and continuous publicity (sometimes, to be rare or less common is to be ignored).  Therefore, this ‘line’ needs to be something that captures people’s imaginations and persuades them to be associated with the cause. It also needs to avoid being too ‘introvert’ by using oblique, confusing, outdated, single issue icons conveyed by what are essentially memes and which are only liked and shared by patients.  It also needs to be accurate.

New audiences means new thinking ….. different thinking.  One of my methods is to increase the audience reach by forming relationships with non-NET organisations including physicians.  Some of this is extremely hard work. For example, the 2016 WEGO Health Awards took a considerable amount of personal effort and time and ditto for 2017 and 2018. However, there’s a lot of new audiences out there now hearing about NETs that had never heard of the disease until I was able to use the platform of these awards.  It’s worth it.  Here’s a statement from the CEO of WEGO Health:

Jack WEGO NETs

My animal free blog site will hit one million views next year and I’m a relative newbie.  So perhaps there is another way?

When I set my blog up on 29 Apr 2014, I never imagined for one second it would be anything other than an obscure and niche site getting a couple of hits per day. I’m therefore really grateful to those who are supporting me including my most recent followers. It’s your support that inspires me to write the posts and then offer them up as awareness messages or simply words to help patients. Now, not a day goes by where I have not tweeted or posted something about Neuroendocrine Cancer. Although 10 Nov is approaching once again, for me……..

EVERY SINGLE DAY OF THE YEAR IS NET CANCER DAY

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Thanks for reading

Ronny

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Remember ….. in the war on Neuroendocrine Cancer, let’s not forget to win the battle for better quality of life!