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

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Neuroendocrine Cancer Nutrition Series Part 4 – Food for Thought?

Food for thought

Nutrition is an important subject for many cancers but it is particularly important for Neuroendocrine Cancer.  In the previous parts of this series I focussed on the following:

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

I said in Article 1 that my intention is not to tell you what to eat, even though that might be a challenge for many and this theme continues. The issue with Nutrition and Diet in general, is that it’s very individual and what works for one may not work for another. Rather I’d like to focus in on why such things might have an effect – patients can then experiment and see what works for them. NET patients may have multiple problems and issues (including the effects of eating) which people may be relating to their cancer or the effects of a particular syndrome or treatment (working that out can be difficult!).  Even if I link you to an authoritative site, it will most likely only show GENERAL GUIDELINES, since patients with NET Cancer should really be assessed on a case-by-case basis.  However, I can say that from personal experience, these guidelines are a good base to start in understanding the issue.  You should always seek professional advice from a reliable ‘NETs aware’ nutritionist that can help you determine what your nutritional needs are and also can guide you in the right direction regarding food and supplement intakes.  Be wary of the internet on diet and nutrition, there is much ‘quackery’ out there and normally they want to sell something regardless of whether it’s good for you or not.  Fake healthcare news is big business unfortunately.  You may also enjoy article 2 and article 3 of this series in internet dangers.

In this article, I want to cover the ‘knotty’ problem of what is in food that might be provoking a reaction and why.  The other thing I would emphasise is that the cause of ‘provocation’ might not just be from what you have eaten, but how much. Moreover, whether the cause is syndromic, due to treatment; or from a comorbidity. For example, if you’ve had classic small intestinal NET surgery, you’re likely to be missing a few feet of small intestine and at least your ascending colon and all that goes with that (i.e. you’ve had a right hemicolectomy).  It follows that your food might transit quicker than normal on its journey from mouth to toilet.  There are no doubt other issues which might cause you to ‘move quickly’ and most of these issues will have been covered in Series Articles 1, 2 and 3.  For those with Carcinoid Syndrome, you may also find my blog on the 5 E’s useful.

A high level of serotonin is something people might be looking to avoid due to its relationship with midgut NETs and in particular those with Carcinoid Syndrome. One thing I noticed is that experienced dietitians are not saying you must totally avoid foods associated with serotonin.  I say “associated” because serotonin is not found in foods (another NET myth), it is manufactured from the amines in food.  The only time dieticians would recommend staying totally away from these foods is before and during a 5HIAA urine test (5HIAA is a by-product of serotonin) as this could skew the results. Experienced NET dieticians will also tell you that amines in foods containing the precursor to Serotonin will not affect tumour growth.  

It’s not just a serotonin problem – it is actually a much wider issue with something ‘vasoactive amines’ (or pressor amines).  They are precursors for catecholamines such as adrenaline, which trigger some NETs to secrete vasoactive substances, which cause symptoms or in extreme cases, carcinoid crisis.  Tyramine is the most active of these amines. Other strongly active vasoactive amines found in food include histamine that can cause strong dilation of capillaries, and also cause hypertensive crisis.  Reported reactions from these vasoactive amines are acute hypertension, headache, palpitations, tachycardia, flushing and unconsciousness. As a general rule, Tyramine and other pressor amines are usually only present in aged, fermented, spoiled protein products, but quite often, it’s food containing a precursor amine that is what you are looking for (for example Tryptophan is a precursor to Serotonin).

Personally I cannot think of a single food which causes me to have a ‘reaction’ other than if I eat too much or eat something with a high fat content.  Basically for someone who has had abdominal surgery, the system cannot cope for one reason or more – see Series Article 2.   It’s important to distinguish this type of reaction which is actually something caused by the consequences of cancer treatment rather than one of the ‘syndrome’ effects .  The answer might simply be to reduce or adjust food intake rather than cut foods out, particularly foods that you may need for nutrition and energy.  And of course, foods you enjoy which don’t cause issues, are related to quality of life.

What I do know from masses of experimentation and running a diary, is that large meals can give me issues. However, as hinted above, I put that down to surgery – NOT syndrome.  I also reduced consumption of fatty foods but that was mainly to combat malabsorption caused by my surgery and exacerbated by Somatostatin Analogues. Again NOT syndrome. I reduced alcohol but mainly because I was concerned about my compromised liver after surgery.

So what are the most provocative foods?  This diagram here is extremely handy BUT I must emphasise that the cause of the provocation may not have been caused by the food itself, just what people think and reported (clearly scientific intervention might prove it was caused by something else).  Everyone is different, so some people might not have any reaction to these foods.  As you can see, a large meal is top and I can almost guarantee much of this was caused by people having a shorter bowel due to surgery.

foods provoking
Graphic courtesy of The Carcinoid Cancer Foundation (CCF)

What are the foods containing high levels of these vasoactive amines?  It is here that I refer you to a site which was one of the very first things I read after my diagnosis, and I re-read it after my initial treatment when I discovered that my debulking and cytoreductive surgery came with some consequences.   This is an amazing piece of research put together by the late Monica Warner (wife of Dr Richard Warner) who herself said “It has not been an easy task to put these guidelines together“.  I don’t believe there is another source of such detailed research and guidelines on the Nutritional Concerns for the NET Patient (note the term Carcinoid is used throughout, therefore it tends to be focused on carcinoid syndrome.  Many other NET Syndromes have associated diet and nutrition constraints and problems too.

This is not an exact science and as the author said “I must emphasize at this point that these are only GENERAL GUIDELINES since patients with carcinoid (sic) may have multiple problems and must be assessed on a case-by-case basis.”. So for example eating a big meal comes out top of the survey and does not necessarily mean that is caused by carcinoid syndrome – as I said above, it’s very frequently caused by having a shorter gut, or no gallbladder, and other issues. You can eat a large meal containing very low levels of the offending amines and still run to the bathroom because your waste disposal system can’t cope with the amount – that is not a syndrome problem.  One person’s perceived ‘syndrome’ problem is another person’s cancer treatment ‘side effect’.  Working out which one is not easy but it’s worth the effort to try to understand which one might be causing the problem.

READ THE RESEARCH AND GUIDELINES BY CLICKING HERE

I hope you found my ‘food for thought’ tasty 🙂

Other useful links which have an association to this blog:

{a} Read a Gut Surgery Diet Booklet 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

[e] There is an excellent video from the NET Research Foundation (what to eat and why) – CLICK HERE

You can hear me talk about my diagnosis by clicking 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

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

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Read my Cure Magazine contributions

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

patients included

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