If you want to strike up a friendly conversion with a Brit, ask him or her about the weather – we’re really famous for our weather conversations and they normally focus on rain or clouds! However, despite the famous British ‘reserve’ and ‘stiff upper lip’, they also frequently talk about being ‘under the weather’, a phrase meaning slightly unwell or in low spirits.
I find myself smiling at some of the conversations I hear in medical establishment waiting rooms, particularly the potentially long wait for blood tests. Here, conversations bypass the weather and focus on being under the weather! I thought I was a regular when I started to recognise people in the queue (line!) and their pill conversations. Statements such as “Yes, I just started a ‘blue chap’ ” (medical names are sometimes hard to pronounce). Normally followed by “I’m on that one too and I take it along with my yellow and white chaps“. Some people seem to be taking a veritable rainbow of ‘chaps’. Strangely, some people appear to be quite proud of how many ‘chaps’ they take. I tend to maintain the traditional British reserve and a stiff upper lip in waiting rooms, so I keep quiet (actually I’m just happy to be inside away from the weather!).
I might join in one day and I wonder if they would be impressed with my tally of chaps? I have a funny feeling my tally of drugs is nothing compared to some of you guys and hope you will comment to prove me right! I don’t think I’m proud to give you my list but here’s my ‘chaps’, some prescription, some over the counter:
Apixaban (Eliquis). To prevent a recurrence of pulmonary emboli (PE). Unfortunately, I had PE after my big surgery in 2010. 2 per day.
Pancreatic Enzyme Replacement Therapy (Creon). Recently added, anything between 6 and 12 per day depending on what I eat. Check out this article on PERT. Check out this article on Malabsorption with references to NET dietitians.
Multi-Vitamin (50+ age). I’ve actually been taking these since a few years before diagnosis in 2010. NET patients can be at risk of vitamin and mineral deficiencies. Check out this article on the issues and with references to NET dietitians.
Vitamin B Complex. This was added in 2013 to mainly tackle low B12 (despite my multi-vit containing 400% RDA) and it seemed to help with fatigue. Read more here.
Vitamin D3. This was also added in 2013 to tackle low Vit D levels (again, despite my multi-vit containing 200% RDA). 10µg (400iu). D3 is normally the recommended form of Vitamin D to take, easiest to absorb and more natural. Vitamin D3 is also known as cholecalciferol. Many people who do not live in sunny countries are probably deficient or borderline already.
Probiotic. This was also added in 2013 to try to offset some of the abdominal issues that many NET patients seem to have. I take a 5 billion dose and it seems to help. Check out this article with references to NET dietitians.
Omega 3. This is also something I had been taking since before my diagnosis. I think I took it for a couple of reasons, my diet did not really include foodstuffs containing Omega 3 and I was experiencing some joint pain in my hands. I just never stopped taking it. Dose size 1000mg.
Lanreotide (Somatuline Autogel). An injection rather than a pill/capsule. Quite a big chap! You can read all about my relationship with Lanreotide by clicking here.
Levothyroxine. One 50mcg tablet each morning. My blood tests are indicating hypothyroidism – check out my whole thyroid story by clicking here. All NET patients need to keep an eye on thyroid levels. Read why here.
Seretide and Ventolin. These are asthma drugs, a preventer and a reliever respectively. I hardly ever take the latter nowadays. I had mild asthma as a child, it went at 16 and came back at 35. I take 2 puffs of Seretide night and day. Seems to help. Ventolin seems to be only required if I have a cold or flu thing going on.
Of course, most people have lots of other stuff in the ‘medicine box’ ready for ad hoc issues as they arise (pain killers, imodium, cough mixture, anti-histamines, indigestion, etc etc). I could go on forever.
Please always consult your specialists or dietitian about the requirements for drugs and supplements. You may not actually need them. I only take my supplements after very careful consideration, in reaction to low blood vitamin/mineral tests and listening to what ‘NET aware’ dietitians say (you’ll find references in some of the articles above).
Warning: You should always think carefully about over the counter stuff (including online) as there’s a lot of ‘scammers’ out there selling counterfeit supplements. Always buy from a reputable source. With supplements, remember in most countries they are not regulated in the same way as medicines so it’s worthwhile checking they are compliant with regional food supplements directives. The supplements provider I use is actually approved by the Medicines and Healthcare Products Regulatory Agency (MHRA) covering UK. I’m sure there will be similar approval organisations where you live. Also be careful of some claims about the miracle cure of certain food supplements. There are plenty sites with fake health news online (check out my article on this – click here).
You should be clear why you take supplements and try to consult with a specialist or dietitian for advice.
Finally, don’t forget to take your chaps, they should help you keep well!
I quite like the Facebook memory thing. This morning I got a reminder of a post I made from 7 years ago whilst I was in hospital recovering from my 9 Nov surgery. It had taken 12 days for me to feel strong enough to venture onto social media with a simple message “I’m feeling perkier”. For those not familiar with English localisms, it just means lively, spirited, bright, sunny, cheerful, animated, upbeat, buoyant, bubbly, cheery, bouncy, genial, jaunty, chirpy, sprightly, vivacious, in fine fettle, full of beans, bright-eyed and bushy-tailed. I guess I met some of these descriptors most of the time! I had gotten through the worst and the light at the end of the tunnel was now a faint glimmer.
I’ve recently had a ton of ‘7 years ago cancerversaries’ and there’s still a few to go! I’m currently being reminded of an issue that started just after my initial treatment and by coincidence (perhaps?) the commencement of my Lanreotide (Somatuline Autogel). Itching! However, for me, it’s mainly the right leg below the knee (go figure!). Much less frequently on my arms and sides. I know many people have the same issue but no-one ever seems to find out why – I guess it’s that Neuroendocrine jigsaw thing again?
Initially, I put the issue down to Lanreotide, as this is mentioned in the side effect list on the drug instructions. The initial connection was made because it seemed to be happening immediately after my monthly ‘dart’. A really annoying itch mostly around my ankles and which had to be scratched! An application of a general emollient cream for a few days seemed to do the trick and after a week it was gone (until the next injection …..). However, after a few years, I sensed the issue was drifting away from the injection cycle and adopting a different and more random pattern. I’m also suspicious of a nutritional connection and checking my article Nutrition for NETs -Vitamins and Mineral Challenges, I can see Vit B3 (Niacin) and Vit E are mentioned in regards skin issues. I’d be confused if this was an issue today as I now take plenty supplements to offset GI malabsorption. However, I probably wasn’t taking sufficient between surgery and 2013 as I lacked the knowledge to do so at the time. So nutritional deficiency remains a possibility or at least an added complication. The most recent outbreak has unusually gone on for the last 4 weeks.
I also seem to have had an eczema type issue in my right ear and mild rosacea for more than 7 years (pre diagnosis). As you can imagine my ‘inner detective’ is working overtime! One thing is clear – this itchy leg issue has plagued me for 7 years.
I know that many people have real issues with rashes and skin itching, I’ve seen this so many times with some people describing it as severe. Clearly when this is the case, a doctor’s intervention is generally required. I’ve seen the following connections to NETs and skin issues:
Glucagonoma – a type of functioning pNET can often come with dermatological issues.
Of course there is a Neuroendocrine Carcinoma of the skin known as Merkel Cell Carcinoma – more of a skin lesion effect than regular dermatological issues.
Edit: 2019. Winter in UK has made my itching seem worse, perhaps the cold weather plays a factor. Maybe I just currently have what many people have – dry flaky skin and the onset of winter probably isn’t helping?
In my neck of the woods, “did you hear the one about the ………” is normally a precursor to a witty comment, or a joke. However, constipation for NET patients is not actually funny – read on.
Certain types of Neuroendocrine Cancer are very heavily associated with diarrhea, either as a symptom of one of the NET Syndromes (yes there is more than one …..); or as a result of surgery or certain other treatments. Occasionally, these symptoms and side effects can all combine to make it quite a nasty and worrying side effect.
I must admit to being surprised to find myself with feelings of constipation from around 4-5 years after my treatment and I set about trying to find out why that might be. To understand why I got to this stage, I assessed the history of my treatment and what I changed in an attempt to improve my Quality of Life (QoL) – I feel there is a strong connection.
When I underwent my primary surgery (Nov 2010), my surgeon said it would take months for my ‘digestive system’ to return to some form of normality. I soon found out what he meant, I seemed to be permanently affixed to a toilet seat (plenty of reading opportunities though ….. every cloud!). I suddenly realised that I needed to start looking seriously at my diet. I did find some improvements by trying to eat things that would bulk up my stools vs trying to avoid things that might increase frequency (i.e. I wanted a reduction in frequency combined with a bulkier stool). Eventually, I settled on a regime for the first couple of years and to be honest, I didn’t need to change my diet in any radical sense. I was also determined not to take any medication (I was taking enough) and wanted this to work as naturally as possible.
Things were still not ideal and in 2013, I even remember saying to my Oncologist that although I was never misdiagnosed with IBS, I felt like I now had it. I decided to attack this issue following professional advice from one of the eminent experts in the NET specialist dietitian world – Tara Whyand. My regime was now based on science (although it isn’t really an exact type!), that is checking the ‘at risk’ nutrient levels were OK (particularly ADEK and B12), taking supplements where necessary to help with deficiencies, and tackling things such as malabsorptionand diet.
The patient has a big part to play in any improvement strategy, so in 2013/14 I experimented more and completely changed my breakfast and lunch regime to oatmeal/porridge and toast which made a significant difference. I started to avoid eating large meals and I reduced fat consumption generally. I started taking probiotics to counter the effect of any bacterial imbalance as a result of my surgery (i.e. to combat SIBO). To keep track of everything, I set up and maintained a detailed diary to help identify things making it worse, tinkering as I went along. For those who are contemplating this sort of strategy, let me tell you – it takes time, effort and patience!
I seemed to make excellent progress with ‘frequency’, which is down to once or twice per day – i.e. I felt like a normal bloke 🙂 Quality was not consistently good but I’m of the opinion, this may be something I need to live with. Stomach cramps are reduced, as is gas and bloating reduced (I’m fairly confident that is mainly down to probiotics). Happy days, my strategy has worked. I reduced my average daily ‘visits’ by 400% without any medicine.
However …. (have you noticed, there’s always a ‘however’ with NET cancer?).
Although I’m generally well, I did start to think in 2016 that the balance was not quite right. My ‘visits’ were starting to last longer due to a consistent feeling of incomplete emptying – i.e. movement is OK but is followed by what seems like constipation. Additionally, I’ve had several episodes of constipation and pain with no ‘movement’ for 24-36 hours. This happened in May, September and December 2016. Had 3 more episodes in 2017 and 2 so far in 2018. My diary now has numerous ‘zero’ entries in the daily bowel movements column, something I never thought I would see again in my lifetime!
When you’ve had small intestinal surgery, as many midgut NET patients have, this sort of thing can be extremely worrying. A bowel obstruction can be dangerous and I’d like to avoid additional surgery at this stage. The second occurrence was particularly severe and the pain lasted for 1-2 weeks. Fortunately, the issues eventually settled and appear to have been a result of a sluggish system, although my regular scans check to see if any issues in that area might have been contributing. (Note – lactulose (oral) is awful, will never touch it again!). I seem to remember a few years ago thinking constipation would be a luxury. I can assure you it isn’t – things need to keep moving, the opposite is much worse!
So … am I a victim of my own dietary regime success? Possibly. The GP who assessed my constipation and pain in September 2016 told me to stop taking a Calcium supplement which was prescribed by the same practice at the beginning of that year – Calcium can slow your system down apparently (…..the calcium is a long story but it was a counter to an osteoporosis risk that I have due to long-term use of blood thinners). I already get enough calcium (and vitamin D) through the normal channels plus supplements, so it was a low risk action. I tinkered with my diet again, reducing my fibre intake and then built up again slowly. Additionally, I could probably do with more water! Perhaps my Lanreotide is having some effect too? In 2018, I changed my bread to one with less fibre as a test, nothing to report so far.
Is it just me with constipation issues? No….. I carried out some covert searches on forums and found this issue has been mentioned numerous times.
I suspect we need science and some specialist NET research in this area, not sure the over the counter prescription is the optimum solution. I was therefore delighted to see a patient survey produced by NET Patient Foundation in conjunction with the Royal Free Hospital presented right in front of me in Barcelona at ENETS 2018. In this survey (which I remember completing), they found that the most self reported side effect of somatostatin analogues was in actual fact constipation (shock horror!).
As you can see from the picture, the survey results came along with some pertinent advice which you will already find in some of my articles co-authored by Tara Whyand who was involved in the survey results analysis. Interestingly, Tara commented on the constipation figure pointing out that the constipated feeling may in fact be confused with ‘incomplete emptying’ as I indicated I was experiencing above. I think she’s right.
I’m always skeptical about patient surveys as they tend to be gathered from a very small percentage of the actual patient population and tend to be sourced from those with the worst issues (something I call ‘situating the appreciation’). There’s a little skepticism in me about this particular survey, mainly because the results were not scientifically investigated i.e. were these self-reported side effects actually caused by somatostatin analogues or something else?
However, many of the things reported in this patient survey are issues that I know patients tend to talk about anecdotally in patient forums. Some of them are already listed on patient information leaflets (often without patients knowing I might add) so this is further confirmation of the official trial results. Wide variances or new unlisted issues probably need looking at though.
Despite some of these side effects being listed, I believe doctors need to provide more support for patients who experience these issues. So, even if constipation (or incomplete emptying) is not totally caused by somatostatin analogues, at least this survey should start up a dialogue.
p.s. I recently started taking Pancreatic Enzyme Replacement Therapy to combat some of the well known side effects of somatostatin analogues but not yet evaluated their overall impact with the above story. Read about this and a Q & A session with Tara Whyand in this article – click here
Diarrhea can be a symptom of many conditions but it is particularly key in Neuroendocrine Tumour (NET) Syndromesand types, in particular, Carcinoid Syndrome but also in those associated with various other NET types such as VIPoma, PPoma, Gastrinoma, Somatostatinoma, Medullary Thyroid Carcinoma.
Secondly, it can be a key consequence (side effect) of the treatment for Neuroendocrine Tumours and Carcinomas, in particular following surgery where various bits of the gastrointestinal tract are excised to remove and/or debulk tumour load.
There are other reasons that might be causing or contributing, including (but not limited to) endocrine problems such as hyperthryoidism, mastocytosis or Addison’s disease (which may be secondary illnesses in those with NETs). It’s also possible that ‘non-sydromic’ issues such as stress and diet are contributing. It could be caused by other things such as Irritable Bowel Syndrome (IBS). Yes, believe it or not, NET Patients can get normal diarrhea causing diseases too!
I want to give a general definition of diarrhea as there are many variants out there. In general, they all tend to agree that diarrhea is having more frequent, loose and watery stools. Three or more stools per day seems to be the generally accepted threshold, although some sites don’t put a figure on it. It’s not pleasant and just about everyone on the planet will suffer it at some point in their life, perhaps with repeated episodes. Normally it’s related to some kind of bug, or something you’ve eaten and will only last a few days before it settles (acute diarrhea). Diarrhea lasting more than a couple of weeks is considered chronic and some people will require medical care to treat it. It can also be caused by anxiety, a food allergy/intolerance or as a side effect of medicine. Pharmacists and GPs will be seeing many patients with this common ailment every single day of business.
Diarrhea induced by a Syndrome
When you consider the explanation above, it’s not really surprising that diarrhea related symptoms can delay a diagnosis of Neuroendocrine Cancer (and most likely other cancers too, e.g. pancreatic cancer, bowel cancer). For example, diarrhea is the second most common symptom of Carcinoid Syndrome (Flushing is actually the most common) and is caused mainly by the oversecretion of the hormone Serotonin from the tumours. Please note diarrhea in other types of syndromes or NETs may be caused by other hormones, for example it may also be caused by excess calcitonin in the case of Medullary Thyroid Carcinoma or VIP in the case of a functional pNET known as VIPoma. I’ve heard stories of people being told they have IBS or something similar for years before they received what is now a late diagnosis and at an advanced cancer stage. This is only one of the reasons why NETs is not an easy condition to diagnose, although it is possible that some people actually had IBS and it was masking the NET. Even after treatment to remove or reduce tumours, many people will remain syndromic and need assistance and treatment to combat diarrhea induced by a NET syndrome (see below).
Diarrhea as a Consequence (Side effect) of Treatment for Neuroendocrine Cancer and Other Conditions
All cancer treatments can have consequences and Neuroendocrine Cancer is definitely no exception here. For example, if they chop out several feet of small intestine, a chunk of your large intestine, chunks (or all) of your stomach or your pancreas, your gallbladder and bits of your liver, this is going to have an effect on the efficiency of your ‘waste disposal system’. One effect is that it will now work faster! Another is that the less effective ‘plumbing’ may not be as efficient as it was before. There are also knock-on effects which may create additional issues with the digestive system including but not limited to; Malabsorption and SIBO. I recommend you read my posts on Malabsorption and SIBO.
Surgery can often be the root cause of diarrhea. A shorter gut for example, means shorter transit times presenting as increased frequency of bowel movements. Another example is the lack of terminal ileum can induce Bile Acids Malabsorption (BAM) (sometimes known as Bile Salts Malabsorption) in degrees of severity based on size of resection. Lack of a gallbladder (common with NETs) can also complicate. Bile Acids are produced in the liver and have major roles in the absorption of lipids in the small intestine. Following a terminal ileum resection which includes a right hemicolectomy, there is a risk that excess Bile Acids will leak into the large intestine (colon) via the anastomosis (the new joint between small and large intestines). This leakage can lead to increased motility, shortening the colonic transit time, and so producing watery diarrhea (or exacerbating an existing condition). Although this condition can be treated using bile acid sequestrants (i.e. Questran), it can be difficult to pinpoint it as the cause.
Surgery of the pancreas can also produce effects such as exocrine pancreatic insufficiency which can lead to a malabsorption condition known as steatorrhea which may be confused with diarrhea (although some texts call it a type of diarrhea). It isn’t really diarrhea but it may look like it given the presentation of the faeces and patients may suffer both diarrhea and steatorrhea concurrently. Patients will recognise it in their stools which may be floating, foul-smelling, greasy (oily) and frothy looking. Treatment options will mainly include the use of Pancreatic Enzyme Replacement Therapy or PERT for short (Creon etc).
Many non-surgical treatments can also cause diarrhea, including but not limited to; somatostatin analogues (see below), chemotherapy, biological targeted therapy (e.g. Everolimus, Sunitinib), radiotherapy.
Somatostatin analogues are an interesting one as they are designed to inhibit secretion of particular hormones and peptides by binding to the receptors found on Neuroendocrine tumour cells. This has the knock-on effect of inhibiting digestive/pancreatic enzymes which are necessary to break down the fat in our foods leading to Malabsorption of important nutrients. This may worsen the steatorrhea in pancreatic NET patients but also lead to steatorrhea in others with non-pancreatic locations who have been prescribed these drugs.
Other conditions may actually be the cause of the diarrhea or the treatment for those conditions. For example, it is possible that people actually do have Irritable Bowel Syndrome (IBS). Treatment therapy for common conditions may also be contributing, for example the use of Proton Pump Inhibitors for acid reflux.
Treatment for Syndrome Induced Diarrhea
Like many other NET patients, I’m on a 28 day injection of somatostatin analogues (in my case Lanreotide). Both Octreotide and Lanreotide are designed to reduce the effects of NET syndromes and therefore can often make a difference to syndrome induced diarrhea. These drugs also have anti-tumour effect and so even if you are not syndromic or they do not halt or adequately control syndrome induced diarrhea, they are still a valuable contribution to NET treatment.
Some syndromic patients find they still have diarrhea despite somatostatin analogues and they end up having ‘rescue shots’ or pumps for relief (both of these methods tend to be Octreotide based). (Hopefully they are not getting confused between diarrhea caused by the non-syndrome effects – see above). Some have more frequent injections of the long acting versions of somatostatin analogues which has the effect of increasing the dosage. There’s a new drug available for those whose carcinoid syndrome induced diarrhea is not adequately controlled or perhaps they are unable to have somatostatin analogues as a treatment. Telotristat Ethylworks by inhibiting tryptophan hydroxylase (TPH), a chemical reactor involved in the manufacture of serotonin, which is the main cause of syndrome induced diarrhea. It was approved by the US FDA in February 2017, EU areas in September 2017, and is on the way to being approved elsewhere. Read about this drug here.
Sorting out the symptoms – post diagnosis
I like to describe this as the Neuroendocrine Cancer jigsaw. It’s a really difficult one and sometimes you cannot find a piece, or the pieces won’t fit. However, metaphorically speaking, the missing piece might be a NET specialist presentation, a comment, statement or view from another patient, a link to an article from a reputable source, or even something you do to improve your lot – there might even be trial and error involved. It might even be this blog post!
How do you work out whether diarrhea is caused by a hormone producing tumour or by the side effects of treatments? There’s no easy answer to this as both might be contributing. One crude but logical way is to just accept that if you have normal hormonemarkers, for example 5HIAA (there could be more for other tumour/syndrome types), and you’re not really experiencing any of the other classic symptoms, then your syndrome might be under control due to your treatment (e.g. debulking surgery and/or somatostatin analogues, or another drug). My Oncologist labels me as ‘non-syndromic’ – something which I agree with. I’m 99.999999% sure my issues are as a result of the treatment I’ve had and am receiving.
This disease is so individual and there are many factors involved including the type of syndrome/NET, patient comorbidities and secondary illnesses, consequences of the surgery or treatments performed, side effects of drugs – all of which is intermingled with suspicion and coincidence – it’s that jigsaw again! I always like to look in more detail to understand why certain things might be better than others, I always challenge the ‘status quo’ looking to find a better ‘normal’. I really do think there are different strategies for syndrome induced diarrhea and that which is a result of treatment or a side effect of treatment. There’s also different prices, with inhibitors costing thousands, whilst classic anti-diarrhea treatments are just a few pennies. Adjustments to diets are free!
When I was discharged from hospital after the removal of my small intestinal primary, I was in the toilet A LOT (I was actually in the toilet a lot before I was discharged – check out my primary surgery blogs here) . My surgeon did say it would take months to get back to ‘normal’ – he was right and it did eventually settle – although my new ‘toilet normal’ was soft and loose and several times daily. My previously elevated CgA and 5HIAA were eventually back to normal and my flushing had disappeared. I didn’t have too many issues with diarrhea before diagnosis. Deduction: my issues are most likely not syndrome induced.
I read that many people find basic ‘Loperamide’ (Imodium) helps and I tend to agree with that if you are non syndromic and just need that little bit of help. I decided long time ago I would not become ‘hooked’ and only really take it for two purposes: 1) if I have a bad patch and 2) if I’m going on a long journey (i.e. on a plane perhaps). I estimate I’ve used 4 packets in as many years. Loperamide decreases the activity which causes intestinal motility (peristalsis). This has the effect of increasing the time material stays in the intestine therefore allowing more water to be absorbed from the fecal matter. Ideal for those with a shorter bowel due to surgery and advice from a medical professional is always advisable. To reduce the risk of malabsorption induced diarrhea and steatorrhoea, both of which can lead to loss of valuable nutrients, the use of Pancreatic Enzyme Replacement Therapy (PERT) might need to be introduced as required by your NET specialist.
Have a look at Enterade – the results from trials look good.
Clearly, I cannot offer any professional medical advice on coping with diarrhea, I can only discuss my own situation and what I found worked for me. Don’t forget, like many diseases, what works for one, might not work for another. However, I did tackle my problems following the advice of an experienced dietitian who specialises in NET Cancer. That said, I was ‘sleep walking’ for over 2 years thinking my issues were just part of the way things were after my treatment. I was wrong about that!
As for my own strategy, here’s things that helped me:
made some changes to diet(they were not huge changes),
maintained a diary to help with monitoring progress or setbacks,
hydration is also important (….still working on that one).
started taking PERT (Creon) on 23 Dec 2017 (changed to Nutrizym Feb 2019) but looks reasonably positive so far.
With no fancy and expensive drugs, I’ve gone from 6-8 visits to 1-2 visits (as a daily average, it’s actually 1.5). This didn’t happen overnight though, it took a lot of time and patience. All of this doesn’t mean to say I don’t have issues from time to time …… because I do!
In summary, I think it’s important that people be sure what is actually causing their diarrhea after diagnosis so that the right advice and the optimum treatment can be given.
Listen to Dr Wolin talking about this particular jigsaw puzzle – click here
Also see a nice article that come out of NANETS 2017 – click here
Of course, some people sometimes have the opposite effect but that’s in another blog here – Constipation
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.
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
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.
A couple of years ago, I received a request from a reader asking if I would write an article about all the symptoms experienced by a Neuroendocrine Cancer patient and how to sort out what is and what isn’t associated with NETs.
Although I chuckled and raised eyebrows at the request, inside I was genuinely humbled that someone thought I was capable of achieving this herculean task. I actually gave it quite a bit of thought to the point of compiling a matrix of types of NET, main symptoms, cross-referenced with the symptoms of the most common reported comorbidities. After it started to look like it might be bigger than the Empire State Building, I came to the conclusion that it’s an almost impossible task for a wee Scottish guy with less common disease 🙂 I also started to suspect that even the world’s top NET experts had not accomplished it either.
Here’s a picture of my work to date:
I have, however, dabbled in attempts to work out my own problems over the past few years. NETs can present with a ‘syndrome’ – a bunch of symptoms normally caused by excessivehormone secretion, some of which are particularly vague and can sometimes continue to cause issues after treatment and beyond – it’s a real witch’s brew of symptoms. They can also cause non-syndromic issues pertaining to treatment side effects and it must also be noted that even NET patients get regular illnesses which adds to the issues healthcare professionals and patients face in monitoring NETs.
In my article “Neuroendocrine Cancer Syndromes – early signs of a late diagnosis”, I focused on the key symptoms experienced pre-diagnosis and then discussed how you might go about sorting out the symptoms from main side effects post treatment (another regular conundrum for most). On a similar subject, you might want to check out my 5 E’s blog for carcinoid syndrome. I also compiled an article about the source of flushing and diarrhea given there were many differential diagnoses and not just syndromes.
NETs vs Other Illnesses
Adding another jigsaw piece to the issues with cancer and side effects – common comorbidities (many of an endocrine nature) can arise simultaneously. Is it connected with NETs are just another illness to manage alongside? All of these factors can make it really difficult to determine the source of the symptoms. I’m always conscious that the majority of NET patients are in their 5th decade onward and at an age where things start to go wrong quite naturally due to ‘time’ and ‘wear and tear’.
Here’s one classic example of this problem, I can see many people on forums also have diabetes (an endocrine disease). In the United States alone, nearly 7 million people have undiagnosed diabetes, according to the American Diabetes Association. I can also see from the news in UK, that this is becoming a much bigger deal too – a report published in Feb 2018 claims that diagnoses have doubled in 20 years. I’ve used the diabetes link as an example, there will be many other very common factors at play, e.g. hypothyroidism an age and gender relation issue. It is certainly possible that many of the problems people face might just be an as yet undiagnosed/underlying condition, unconnected with NETs. To quote the great Dr Eric Liu, “even NET Patients get regular illnesses”. Working it out is rather difficult though. Sometimes pragmatism is required.
Syndromes vs Side Effects of Treatment
On forums where most people have a diagnosis and are undergoing treatment, there is regular discussion and Q&As about the source of symptoms, i.e. are they a result of a functioning syndrome (i.e. a consequence of the cancer) or something else? For example, some people complain they still have (so-called) carcinoidsyndrome diarrhea after bowel surgery………that needs some careful thought and understanding before coming to what might just be the wrong conclusion, particularly if all tumour markers are normal. I have lost count of the number of times someone has asked about a symptom on a forum and got 50 different answers. One of the reasons why forums can be good at frightening rather than frighteningly good. Personally, I never compare myself to strangers on the internet. I just hope most people are using the forums as ‘sounding boards’ and are simultaneously addressing these very complex issues with their doctors when they are genuinely concerned.
I really feel for anyone who is going through a difficult diagnosisor has been diagnosed and then continues to have numerous problems after initial treatment. I also have a little bit of sympathy for primary care medical staff on the basis this is just one of over 200 types of cancer, many of which have wide age groupings adding to the complexity and difficulty. Moreover, many of the symptoms experienced by NET patients on analysis look very similar to everyday illnesses and other ailments. And if that wasn’t demanding enough for doctors, many patients present with already established and diagnosed comorbidities (other illnesses) which add another level of complexity. These difficulties can then continue throughout treatment. It can be a real challenge and I’m sure even Doctors can be totally flummoxed on occasion by patient presentations.
It is extremely difficult to “sort out the symptoms” when faced with multiple locations/tumour sub-types, multiple treatments causing multiple side effects, multiple side effects causing multiple symptoms, multiple comorbidities with symptoms similar to cancer syndromes and treatment side effects (and vice versa). This disease can be very individual and what happens to one might not happen to another. Although we hope doctors generally take a holistic view when treating NET patients, I have a view that sometimes focussing in on a particular symptom might occasionally be a more effective route (the bottom-up approach – pun not intended!). When eating an elephant, take one bite at a time! It’s useful to know about the range of tumor markers and hormone markers – read more here.
One thing I have learned ……educate yourself to the best of your abilities. This will help you to better advocate for yourself. Improvements are possible.
Neuroendocrine Cancer is a very difficult jigsaw and you sometimes need to look very hard for the missing piece! The ‘missing piece’ can be variable and very individual, i.e. a NET specialist, access to a particular treatment or even just more support or access to support information that works.
One of the curious things about Neuroendocrine Cancer (NETs going forward) is that it can very often exhibit one or more vague symptoms collectively known as a ‘syndrome’. Syndrome is an apt word to describe these complications as the most general meaning in medical terms is a group of symptoms that together are characteristic of a specific disorder or disease”. Having a syndrome can often be the difference between having a ‘functional’ condition or a non-functional’ condition – see more below.
This frequently makes Neuroendocrine Cancer very difficult to diagnose quickly. It’s a very devious disease.
It’s not all about Carcinoid Syndrome!
Most people think of Carcinoid Syndrome when they discuss NETs. Anyone suggesting that all NET patients get carcinoid syndrome or that all symptoms of NETs are caused by carcinoid syndrome, is WAY off the mark. Firstly, not everyone will have a ‘syndrome’ in addition to their tumours – the percentage is actually well below 50%. Secondly, there are in actual fact, several associated syndromes depending on the anatomical location and type of NET. As an example of one syndrome, statistics vary from source to source but it is estimated that around a 30-45% of all ‘midgut’ patients will present with metastatic disease and around a third of those (∼10-15% of all midgut) will exhibit Carcinoid Syndrome indicating their tumours are ‘functional’ (secreting excess hormones, particularly serotonin). It follows that Carcinoid Syndrome itself is not that common and it could be the same with other types of NET (even though it can appear more prevalent on forums).
Functional / Non-Functional
These tumours and associated syndromes are treatable for most but the difficult part can be arriving at a diagnosis. Moreover, without a syndrome, some of these tumours can be silently growing and as they grow slowly, the ‘silence’ can go on for some years. Even with a syndrome, the root cause can remain disguised as the symptoms are similar to many day-to-day illnesses, again the reason for the title of this blog. Curiously, the lack of a syndrome can sometimes lead to an even later presentation and the consequences that arise (i.e. no signs to aid a diagnosis). In fact a large proportion of Pancreatic NETs are non-functional at diagnosis. There can be the odd exception but in general terms, NETs are either functional (with a syndrome) or non-functional (no syndrome). It’s also possible that patients can move from one state to another.
It’s useful to know about the range of tumor markers and hormone markers – read more here
Syndrome and Tumors – ‘Chicken or Egg’ ?
I’m always confused when someone says they have been diagnosed with a Syndrome rather than a NET type. You normally need a tumor to produce the symptoms of a syndrome.
The exception might be hereditary syndromes e.g. MEN. MEN syndromes are genetic conditions. This means that the cancer risk and other features of MEN can be passed from generation to generation in a family. A mutation (alteration) in the various MEN genes gives a person an increased risk of developing endocrine/neuroendocrine tumors and other symptoms of MEN. It’s also possible that the tumors will be discovered first. It’s complex!
Major NET Syndromes
(information mainly taken from the ISI Book on NETs with a cross-reference from ENETS and UKINETS Guidelines)
TheISI Book on Neuroendocrine Tumors 2016(Woltering et al) confirms there are a number of syndromes associated directly and indirectly with NETs and are described as individual syndromes according to their secretory hormones and peptides. The reference publication expands on this list to aid diagnoses by including common presentations, associated tumour types and locations and the offending secreting hormones. You can see why Neuroendocrine Cancer is a diagnostic challenge!
Carcinoid – a syndrome connected with (mainly) serotonin secreting tumours in certain locations (mainly small intestine, lung, stomach, appendix, rectum). The key symptoms include diarrhoea, flushing of the skin (particularly the face), stomach cramping, heart problems such as palpitations, and wheezing. The syndrome is actually caused by the release of a number of hormones, in particular Serotonin, Bradykinin, Tachykinin (Substance P), Histamine, and Prostaglandins.
(there’s also a very rare instance of pancreatic based tumours producing carcinoid syndrome effects – according to ENETs less than 1% of all tumours associated with carcinoid syndrome)
Whipple’s Triad – Whipple’s Triad is the classic description of insulinoma which includes symptoms of hypoglycemia with a low blood glucose concentration relieved by the ingestion of glucose. These tumours can be located anywhere within the pancreas in the cells that make insulin. Insulin is a hormone that controls the amount of glucose (sugar) in the blood. It moves glucose into the cells, where it can be used by the body for energy. Insulinomas are usually slow-growing tumors that rarely spread. Some of these tumours will be associated with MEN1 syndrome.
Zollinger-Ellinson Syndrome. A tumour that forms in cells that make gastrin and can be known as a Gastrinoma. Gastrin is a hormone that causes the stomach to release an acid that helps digest food. Both gastrin and stomach acid are increased by gastrinomas. This is a condition in which one or more tumours form in the pancreas, the upper part of the duodenum or the stomach (these organs are very close and tightly packed together). These tumours secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid can lead to peptic ulcers, in addition to diarrhea and other symptoms. Associated with Gastrinoma (pNET) and Gastric NETs. Some of these tumours may be associated with MEN1 syndrome.
Werner-Morrison Syndrome. Vasoactive Intestinal Peptide (VIP) is secreted thus the pNET term – VIPoma – Sometimes the syndrome is referred as WDHA – Watery Diarrhea, Hypokalemia (potassium deficiency), and Achlorhydria (absence of hydrochloric acid in gastric secretions). Sometimes known as Pancreatic Cholera. Some of these tumours may be associated with MEN1 syndrome
Glucagonoma. A tumour that forms in cells that make make glucagon. Glucagon is a hormone that increases the amount of glucose in the blood. It causes the liver to break down glycogen. Too much glucagon causes hyperglycemia (high blood sugar) rendering most patients diabetic. A glucagonoma usually forms in the tail of the pancreas. Some of these tumours may be associated with MEN1 syndrome. See also Sweet’s Syndrome below. Sometimes known as the 4D syndrome – Dermatological, Diabetes, DVT, Depression.
Somatostatinomais a very rare type of NET, with an incidence of one in 40 million persons. These tumours produce excess somatostatin arise from the delta cells in the pancreas, although these cells can also be present in duodenal/jejunum tissue where around 44% of these tumours occur. Somatostatin is a naturally occurring peptide that inhibits the function of almost all gut hormones (author’s note – this fact should give you an appreciation of how somatostatin analogues tackle associated syndromes whilst giving you certain side effects as a result!)
Pancreatic Polypeptide (PP) – PPoma. A complicated one and not too much information (even in the ISI book or ENETS Guidelines). However, it’s the third most common type of islet cell tumour (i.e. pNET). The function of pancreatic polypeptide is not completely understood. Patients present with weight loss, jaundice, and abdominal pain. The diagnosis is confirmed by pancreatic polypeptide levels > 300 pg/ml. Some of these tumours may be associated with MEN1 syndrome.
Cushing’s – also known as hypercortisolism. A collection of symptoms caused by very high levels of a hormone called cortisol in the body. In Cushing’s disease, oversecretion of pituitary ACTH induces bilateral adrenal hyperplasia. This results in excess production of cortisol, adrenal androgens, and 11-deoxycorticosterone. Cushing’s disease, a subset of Cushing’s syndrome, is due to a pituitary corticotroph adenoma and results in a partial resistance to the suppression of ACTH by cortisol so that secretion is unrestrained. In contrast, causes of Cushing’s syndrome may include the following:
• Adrenal adenoma or carcinoma arise spontaneously. ACTH levels are undetectable.
• Non-pituitary (ectopic) tumours produce ACTH. They most frequently originate in the thorax and are highly aggressive small cell carcinomas of the lung or slow- growing bronchial or thymic carcinoid tumours. Some produce corticotropin- releasing hormone (CRH) instead, which stimulates pituitary ACTH secretion and can therefore mimic a pituitary tumour.
• Other causes include NETs of the gastric, pancreatic, and intestinal organs; Pheochromocytomas, and MCT.
The hallmark of Cushing’s syndrome is that ACTH levels are partially resistant to suppression with dexamethasone, even at very high doses. Some MEN patients with pituitary tumours may have Cushing’s Syndrome. AdrenoCorticoTropic Hormone (ACTH) releasing tumours are somerimes known as ACTHoma.
Sweet’s – Dermatitis/rash associated with Glucagonomas. Not to be confused with Pellagra (B3 deficiency)
Neuroendocrine / Endocrine tumors can be seen in several inherited familial syndromes, including but not limited to:
MEN1 – Mainly involved the 3 Ps, Pituitary, Pancreas and Parathyroid. The pituitary tumours are primarily Prolactinomas, the pancreatic tumours are mainly PPomas, Gastrinomas and Insulinoma. Many also have association with Zollinger-Ellinson syndrome (ZES). Sometimes known as Wermer Syndrome. Associated with the MEN1 gene.
MEN2A– associated with the RET gene, can result in Medullary Thyroid Carcinoma, Pheochromocytoma, and overactive parathyroid glands characterised by a high calcium level.
MEN2B. An inherited disorder characterised by the certain development of Medullary Thyroid Carcinoma, plus the possible development of pheochromocytomas and characteristic tumours (mucosal neuromas) of the lips, tongue and bowels. Parathyroid disease is extremely rare in MEN2B. Also connected with the RET gene.
MEN4. A relatively new MEN variant and related to the CDKN1B gene. Similar to MEN1 but normally only 2 of the 3 Ps, parathyroid and pituitary; and potentially other places.
Succinate dehydrogenase (SDH) is an enzyme which is important for the metabolic function of mitochondria. Patients with mutations of these genes have increased risk of pheochromocytomas, paragangliomas, stomach tumors and kidney tumors.
SDHx mutations (SDHA, SDHB, SDHC, and SDHD) can present as Pheochromocytomas/Paragangliomas and other non-NET conditions. If this interests you see site http://www.SDHcancer.org
Von Hippel-Lindau (VHL) – not an exclusively NET syndrome. VHL is a rare disorder caused by a faulty gene. It is named after the two doctors who first described the disease, and affects about one in 35,000 people. Tumours develop in one or more parts of the body. Many of these tumours involve the abnormal growth of blood vessels in parts of the body which are particularly rich in blood vessels. Areas most frequently affected are the eyes, the back of the brain (cerebellum), the spinal cord, the kidneys, the adrenal glands and the pancreas. People are affected differently, even within the same family. The only VHL tumour which tends to run in families affects the adrenal glands (Pheochromocytoma). Different VHL features tend to develop at different ages. The eye angiomas often develop in childhood. Others, including tumours found in the cerebellum, spinal cord or adrenal glands (Haemangioblastomas and Pheochromocytomas) can develop from late childhood onwards. The kidney tumours are usually the last things that develop, from the mid-twenties onwards. Most VHL related tumours are benign.
As for my own experience of syndromes, I did once show symptoms of the most common NET syndrome (currently known as Carcinoid syndrome) where the key symptoms include diarrhoea, flushing of the skin (particularly the face), stomach cramping, heart problems such as palpitations, and wheezing. You can see why those symptoms are frequently and easily confused with other conditions. If you have a similar diagnosis, you may benefit from looking at something known as The 5 E’swhich is a useful list of things to be wary of.
I did have issues for a year or two in 2010 leading up to diagnosis and until my treatment was underway. I was experiencing flushing and infrequent bouts of diarrhea but I totally ignored it (hear me talk about this). However, it ended up being instrumental in my diagnosis albeit some good luck was involved in getting to that point. My twist of fate which involved a low hemoglobin score led me to a scan and ‘bingo’. I had a ‘gastrointestinal blip’ some 18 months previously but that proved colonoscopy negative. Despite my distant and metastatic tumour disposition and seemingly late diagnosis, I’m current non-syndromic due to “early” intervention and good treatment. However, my ongoing treatment continues to play its part.
For many,the vague and routine symptoms generated by a syndrome contribute to the fact that NET Cancer is frequently misdiagnosed with some people suffering from the side effects for many years before a correct diagnosis is made.
There are many other less known syndromes that appear to be directly or indirectly connected with Neuroendocrine Tumours and I may update this post if I discover they are more prevalent than I think. Please let me know if you’ve been told you have a NET related syndrome not listed.
This is the second article in the Neuroendocrine Cancer Nutrition series. In the first article, I focused on Vitamin and Mineral deficiency risks for patients and there is a big overlap with the subject of Gastrointestinal Malabsorption. Those who remember the content will have spotted the risks pertaining to the inability to absorb particular vitamins and minerals. This comes under the general heading of Malabsorption and in Neuroendocrine Cancer patients, this can be caused or exacerbated by one or more of a number of factors relating to their condition. It’s also worth pointing out that malabsorption issues can be caused by other reasons unrelated to NETs. Additionally, malabsorption and nutrient deficiency issues can form part of the presenting symptoms which eventually lead to a diagnosis of Neuroendocrine Cancer; e.g. in my own case, I was initially diagnosed with Iron Deficiency Anemia in association with some weight loss. Even after diagnosis, these issues still need to be carefully monitored as they can manifest as part of the consequences of having cancer and cancer treatment.
Malabsorption will present via several symptoms which may be similar to other issues (i.e. they could masquerade as, or appear to worsen the effect of a NET Syndrome). These symptoms may include (but are not limited to) tiredness/fatigue/lethargy, stomach cramps, diarrhea, steatorrhea (see below), weight loss. Some of these symptoms could be a direct result of nutrient deficiencies caused by the malabsorption. Some patients (and perhaps physicians?) could mistake these for symptoms of Neuroendocrine disease including certain syndromes, perhaps leading to prescribing expensive and unnecessary drugs when a different (and cheaper) strategy might be better.
Crash Course……. We eat food, but our digestive system doesn’t absorb food, it absorbs nutrients. Food has to be broken down from things like steak and broccoli into its nutrient pieces: amino acids (from proteins), fatty acids and cholesterol (from fats), and simple sugars (from carbohydrates), as well as vitamins, minerals, and a variety of other plant and animal compounds. Digestive enzymes, primarily produced in the pancreas and small intestine (they’re also made in saliva glands and the stomach), break down our food into nutrients so that our bodies can absorb them. If we don’t have enough digestive enzymes, we can’t break down our food—which means even though we’re eating well, we aren’t absorbing all that good nutrition.
What is malabsorption?
The malabsorption associated with Neuroendocrine Cancer is most prevalent with the inability to digest fat properly which can lead to steatorrhea. Patients will recognise this in their stools. They may be floating, foul-smelling and greasy (oily) and frothy looking. Many patients confuse steatorrhea with diarrhea but technically it’s a different issue although both issues may present concurrently. Whilst we all need some fat in our diets (e.g. for energy), if a patient is not absorbing fat, it ends up being wasted in their stools and in addition to the steatorrhea, it can also potentially lead to (unwanted) weight loss and micronutrient deficiencies of the fat-soluble vitamins A, D, E and K. Certain water-soluble vitamins, particularly B3 and B12, are also at risk. Many NET Patients are prescribed a supplement of pancreatic enzymes to combat these issues – see Article 5 in this series – Pancreatic Enzyme Replacement Therapy (PERT).
What causes it with NET Patients?
Structural Changes (i.e. Surgery)
This can play a very big part in malabsorption issues. For example, if a patient has undergone Pancreatic surgery, this will most likely effect the availability of pancreatic (digestive) enzymes needed to break down food. Many Small Intestine NET (SI NET) patients will suffer due to the removal of sections of their ileum, an area where absorption of water-soluble vitamins and other nutrients take place. In fact, the terminal ileum is really the only place where B12 is efficiently absorbed. Low B12 is known to cause fatigue. Some patients with Gastric tumours succumb to pernicious anemia with the most common cause being the loss of stomach cells that make intrinsic factor. Intrinsic factor helps the body absorb vitamin B12 in the intestine. Although a less common tumour location, jejunum surgery could result in loss of nutrients as this section of the small intestine is active in digestive processes. Malabsorption issues for SI NETs are an added complication to the issues caused by a shorter bowel (e.g. faster transit time), something which is regularly assumed to be the effects of one of theNET Syndromes (particularly diarrhea and fatigue), when in actual fact, it’s a simple consequence of cancer treatment and may need a different treatment regime.
Evidence of the problems being caused by the effects of small intestinal surgery can be found in a recently published Swedish study which you can read here: Click here. This particular study recommends supplementation of B12 and D3 for those affected. If you’re having trouble getting your physician to monitor your vitamin levels, show them these studies. I get these vitamins checked annually.
The Gallbladder and Liver
The Gallbladder plays an important part in the digestive system – particularly in fat breakdown. The liver continually manufactures bile, which travels to the gallbladder where it is stored and concentrated. Bile helps to digest fat and the gallbladder automatically secretes a lot of bile into the small intestine after a fatty meal. However, when the gallbladder is removed, the storage of bile is no longer possible and to a certain extent, neither is the ‘on demand automation’. This results in the bile being constantly delivered/trickled into the small intestine making the digestion of fat less efficient. One of the key side effects of Somatostatin Analogues (Octreotide and Lanreotide) is the formation of gall stones and many Neuroendocrine Cancer patients have their gallbladder removed to offset the risk of succumbing to these issues downstream. However, the removal of the gallbladder increases the risk of Bile Acid Malabsorption (BAM) as described below. Any issues with Bile Ducts can also have a similar effect.
The Liver has multiple functions including the production of bile as stated above. However, one of its key functions within the digestive system is to process the nutrients absorbed from the small intestine. If this process is affected by disease, it can potentially worsen the issues outlined above.
Bile Acids Malabsorption
Another risk created by the lack of terminal ileum is Bile Acids Malabsorption (BAM) (sometimes known as Bile Salts Malabsorption and some texts described the resultant diarrhea as ‘Bile Acid Diarrhea”). Bile Acids are produced in the liver and have major roles in the absorption of lipids in the small intestine. Following a terminal ileum resection which includes a right hemicolectomy, there is a risk that excess Bile Acids will leak into the large intestine (colon) via the anastomosis (the new joint between small and large intestines). This leakage can lead to increased motility, shortening the colonic transit time, and so producing watery diarrhea (or exacerbating an existing condition).
Somatostatin Analogues can also impact (or worsen) the ability to digest fat as they inhibit the production of pancreatic digestive enzymes (amongst other things). This is a well-known side effect of both Octreotide and Lanreotide. The levels of the fat-soluble vitamins (ADEK) and B vitamins such as B12, need to be monitored through testing and/or in reaction to symptoms of malabsorption. If necessary these issues need to be offset with the use of supplements as directed by your dietician or doctor. Supplements are less affected by malabsorption of nutrients but their efficiency can be impacted by fast gut transit times (thus why testing is important). The evidence and recommendations for malabsorption caused by somatostatin analogues is here: Click Here.
Deficiencies of these vitamins and certain minerals can lead to other conditions/comorbidities, some more serious than others. For a list of the vitamins and minerals most at risk for Neuroendocrine Cancer patients, have a read of my article which was co-authored by Tara Whyand – Vitamin and Mineral deficiency risks.
There is a third article in this series discussing a related issue with Neuroendocrine Cancer, particularly where gut surgery has been performed. You can link directly to this article here – “Gut Health” – (Gut Health, Probiotics and Small Intestinal Bacterial Overgrowth (SIBO)).
The fourth article looks at Amines and why they can cause food reactions or exacerbate syndromes.
Many people also confuse steatorrhea with diarrhea (although these issues can appear simultaneously), again leading to wrong conclusions about the causes and effects, and worryingly, the treatment required. Check out my diarrhea article – click here.
A common problem in patients and from what I see, many just assume this is part of their various syndromes leading to the wrong therapy or no therapy as it’s simply ignored. Again, I remain very grateful to Tara Whyandfor some assistance.
This is a big and complex subject and I only intended to cover the basics. Everyone is different and nothing in here should be accepted as medical advice for you or anyone you know. If you need professional advice, you should speak to your doctor or registered dietitian.
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:
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:
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.
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 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 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.
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 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
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.
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!
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.
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.
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 B12deficiency. In addition, patients with insufficient pancreatic enzymes are also at risk of vitamin B12deficiency 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!
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.
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.
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.
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.
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.
Withincurable 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 lesionbeing 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.
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-doctorcommunicationis 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 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.