I once wrote a post about patient stories, in particular the ones I receive in my private messages. The headline was “The shock effect never wears off“. But none have been more shocking than the one I received early in 2019. (edit: After posting this article, I heard of a few similar cases).
This is a story about someone who is a private person but felt the need to reach out to me about their diagnostic experience. This person wanted to talk about it, but in private and I was happy to listen. I was so moved by this story, I persuaded this person to let me tell it here whilst retaining their anonymity. Hence referral going forward as ‘Patient E’. I just felt that someone somewhere might learn something very important.
The Story of ‘Patient E’
In December 2018, Patient E was told ‘Stage 4 Pancreatic Cancer’ and had 10 months to live. Chemo was to be attempted in January in an effort to extend life but in the meantime was told to spend Christmas for the last time with the family, a spouse and 3 young children. On 2nd January, Patient E was then told (with apologies) it was actually a ‘Neuroendocrine Tumour with a pancreatic primary’. The only good thing about this story so far, is that they told the children nothing over Christmas. “Why worry happy little people” was the bit of the story which brought out my tears.
Initially I was quite angry this could happen to anyone but I was then calmed by Patient E who now had fresh hope and optimism, perhaps realising that there were possibilities for many more years with the family.
The Story of ‘Patient F’
In March of 2017 I was having bad indigestion and since I already had two cardiac stents went to a local hospital to make sure I wasn’t having cardiac issues. They found a mass on the pancreas and metastasis to the liver. I saw a surgeon a couple of days later and he told me it was inoperable, adding that I should pretty much get my affairs in order. A biopsy was then carried out confirming it was neuroendocrine and we were thrilled. I then received a call a couple of days later, saying they had it wrong, it was not neuroendocrine and they wanted to start chemo ASAP! I then received a further call a few days later, saying they were not sure and needed to do more tests. We then went a different hospital where two different surgeons said that it was inoperable. One oncologist said she did not know; and another thought it might be a combination of the two. At this point. they sent my slides to a NET specialist hospital for their opinion, coincidentally we had already scheduled an appointment there. When we walked into this appointment, the NET Specialist greeted us with a big smile and hug, saying that that both she and the surgeon had reviewed my slides; and that it was definitely neuroendocrine – they could operate. Two weeks later the surgeon removed the mass from my pancreas and my spleen. He said that it was probably there for years and looked like it was ready to burst. I was unbelievably lucky.
So why do these things happen? Apart from the serious communications lapse by the hospitals, this is another example of the problems we as a community face with the anatomical approach many doctors take with cancer, with some even describing a pancreatic Neuroendocrine Tumour as a type of Pancreatic Cancer (this happens with other parts of the anatomy too).
Patients E and F are not alone, I once wrote a story about rock star Wilko Johnson (of Dr Feelgood fame) who was given a very similar prognosis. However, Wilko was in the later stages of his life and decided instead of undergoing gruelling chemotherapy, he would forego any treatment and tour with his band in the final 12 months of his life and …….. make an album with Roger Daltrey of The Who. It wasn’t until someone enquired why he wasn’t dead after 12 months, that they re-checked and changed the diagnosis to Neuroendocrine Tumour with a pancreatic primary. Read the whole story here.
I wonder how many other times this has happened.
Neuroendocrine Tumours with a pancreatic primary (pNET) is a totally different cancer to Pancreatic Cancer. With Pancreatic Cancer, most people die, quite the opposite with pNETs where most people live. Read more about the main differences here.
I’m grateful to Patients E and F for contacting me to let me know that my blog and my other support sites have been helpful in the transition from despair to hope since finding out the correct diagnosis. I do hope Patients E and F will keep me updated.
Judy Golz is a retired registered nurse — she’s also a neuroendocrine cancer survivor. Like many nurses who get cancer, their experience in working in the healthcare industry possibly helps get a quicker diagnosis, possibly because they can recognise symptoms and likely differential diagnoses and it possibly helps knowing how the healthcare system works.
But with uncommon and complex diseases, it’s not always that straightforward, even for a nurse or any other healthcare professional. But Judy makes an excellent point about the quantity and quality of medical information now out there, including for Neuroendocrine Cancer and suggested she would be in a better place for self-diagnosis today.
Two things spring to mind about this story. Neuroendocrine Cancer is such a complex disease, it can often be hard for healthcare professionals to diagnose themselves. Secondly, since my diagnosis in 2010, there is no doubt a lot more information out there and I’d like to think I’ve played my part in that.
I’ve always been one to keep an eye out for the postman (the postie as we say here). Even as a heavy user of computers, I still get excited about receiving ‘paper’ mail. Other than birthday cards, I personally don’t tend to see many handwritten letters nowadays. In today’s internet connected world, handwritten letters are always exciting, always special. However, the one I received in the first week of February 2019 was extra special, it was postmarked from North Carolina USA.
Now ….. for those around the same age as me, you might have been attracted by the article header and have remembered the famous radio show entitled “Letter from America”. This was a weekly fifteen minute speech radio series broadcast on BBC Radio 4 and across the world through the BBC World Service. Throughout its history, it was presented by Alistair Cooke, who would speak of a topical issue in the US, tying together different strands of observation and anecdote and often ending on a humorous or poignant note. The series ran from 24 March 1946 to 20 February 2004, making it the longest-running speech radio programme hosted by one individual in history! The BBC has archived many episodes on their website and you can actually now listen to these – click here if you fancy a trip back in time. For me, the memories of the programme were around the deep bond of friendship and shared values that the two great countries share, thus why the radio programme was so special and so relevant to listeners this side of the pond.
Some of you possibly thought about the Scottish duo composed of twin brothers Charlie and Craig Reid – ‘The Proclaimers’. I guess many people in North America and the Southern Hemisphere will be aware of this group who are probably more famous for their anthem song ‘I’m gonna be (500 miles)’ but their song ‘Letter from America’ was actually their most successful. I have a theory that when people from America tell me they like my quaint British accent, what they actually mean is my Scottish ‘twang’, just like ‘The Proclaimers’ who were born and raised not far from my home town of Dundee. Their singing style is with a Scottish ‘twang’ – always worth a listen (click here).
Anyway, back to the actual letter. The person who sent me the letter placed their name and address on the front of the envelope but it was not a name I recognised. However, he left a clue inside indicating a mutual connection (WEGO Health). What a boost this letter gave me!
It should also be a boost for all those who read my stuff and follow me on social media because ‘Josh’ is not a Neuroendocrine Cancer patient but he made some really awesome comments which I wanted to share with you. I also wanted you to know first hand that others are being made aware of Neuroendocrine Cancer and our fight for more awareness.
In case you can’t read the handwritten words or wanted to translate, I’ve extracted below:
“Dear Ronny, I just wanted to send a quick “Hello” and “thank you” for everything you do. I’ve become familiar with your advocacy work over the past couple of years and it is hard to find the quality of content and mix of science, lifestyle and hope that you consistently show the world. It is refreshing and inspiring to say the least. I hope you realize the positive impact you have on real people. Thank you again and I look forward to your future work. Sincerely……… Josh”
Thanks for listening
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Proton pump inhibitors (PPIs) reduce the production of acid by blocking the enzyme in the wall of the stomach that produces acid. Acid is necessary for the formation of most ulcers in the oesophagus, stomach, and duodenum, and the reduction of acid with PPIs prevents ulcers and allows any ulcers that exist in the oesophagus, stomach, and duodenum to heal. PPIs are prescribed to treat acid related conditions such as:
Esophageal duodenal and stomach ulcers
Gastroesophageal reflux disease (GERD)
Zollinger-Ellison Syndrome – ZES (note this is a syndrome associated with a functioning duodenal or pancreatic NET known as a Gastrinoma)
They also are used in combination with antibiotics for eradicating Helicobacter pylori, a bacterium that together with acid causes ulcers of the stomach and duodenum for eradicating H. pylori, a bacterium that together with acid causes ulcers of the stomach and duodenum.
Although this should not be considered a full list applicable to all countries, the drugs tend to be prescribed or purchased under the following names:
Aspirin and Omeprazole (Yosprala)
Dexlansoprazole (Dexilent, Dexilent Solutab)
Esomeprazole (Nexium, Nexium IV, Nexium 24 HR)
Esomeprazole magnesium/naproxen (Vimovo)
Lansoprazole (Prevacid, Prevacid IV, Prevacid 24-Hour, Zoton FasTab)
Omeprazole and sodium bicarbonate (Zegerid, Zegerid OTC)
Pantoprazole (Protonix, Pantoloc Control)
Rabeprazole (Aciphex, Aciphex Sprinkle, Pariet)
PPIs have revolutionized the management of acid-related diseases and there is evidence supporting their superior efficacy and overall safety profile. Unfortunately, it would appear this has possibly led to their overuse and inappropriate use. When used appropriately, the overall benefits significantly outweigh the potential risks in most patients.
One US pharmacist magazine has stated that almost half of all patients taking a PPI do not have a clear indication. It follows that PPIs may not be the appropriate treatment for many people. The American Gastro Journal nicely covers this issue – click here.
What is the connection with NETs?
Millions of people will have been prescribed these drugs for the various reasons listed above and as I said above quoting from a reputable US Pharmacist magazine, perhaps many do not have a clear indication for their use. So this issue is much wider than NETs.
Above, you can see a direct link to duodenal/pancreatic NET syndrome – ZES. However, there is also a known link between the use of PPIs and the effect on the Chromogranin A blood test, the most common tumour marker used in the diagnosis and surveillance of many types of NET. Several studies have concluded that PPIs falsely elevate Chromogranin A but there is another option – read more here.
Any other risks of using PPIs?
There are several well-known risks of using PPIs in the long-term. However, many drugs have side effects, often the risks of not taking a particular drug can be outweighed by taking it. I will not comment further but leave you with some references to read yourself:
1. From the UK National Health Service (NHS). They took a balanced view adding the risk element I described above. Importantly they stated that PPIs are not usually intended to be taken long-term. Read more here. The British Medical Journal (BMJ) published the study referred to by the NHS here.
2. The NHS also published an article based on the results of a US study. Again, they indicated the study had similar limitations to the one above. Read more here (links to the study contained within).
3. There are literally dozens of similar articles but most seem to point to these two studies. However, it should also be noted that the US FDA has issued safety warnings about long-term use of PPIs. This is covered in the aforementioned US Pharmacist magazine article here.
Are there alternatives to PPIs?
Firstly, you should NEVER stop taking PPIs without speaking to the doctor who prescribed them.
There’s a class of drugs known as Histamine H2 Receptor Antagonists (H2RA) that reduce the amount of acid produced by the cells in the lining of the stomach. They are also commonly called H2 blockers. They include Cimetidine (Tagamet, Tagamet HB), Famotidine (Pepcid, Pepcid AC), Nizatidine (Axid) and Ranitidine (Zantac). Brand names may differ from country to country. From what I read, they are not as powerful as PPIs but for some people they may prove adequate. Read more about H2 blockers here.
So I can just stop taking PPIs and start taking H2 blockers?
NO. As I said above, you should never discontinue a prescription for PPI without talking to your doctor. However …. it’s not common knowledge that suddenly stopping PPIs is not a good idea – you must gradually reduce (i.e. taper off).
Why taper? PPIs block the production of acid in your stomach which can help with the symptoms but that also turns on the release of gastrin. This is not ideal for two reasons according to NOLANETS:
When you try to get off of PPI, the gastrin stimulates acid production and stays elevated, potentially for several months (depending on how long you were on the PPIs). This makes your reflux worse than before and makes getting off of this medication very difficult. Gastrin also stimulates Chromogranin A thus why this can be elevated in patients who have been taking PPIs.
Gastrin also acts like a growth factor and stimulates the growth of ECL cells (enterochromaffin like cells). Clearly this does not happen to everyone on PPIs. However, and as per the NHS advice above, PPIs should not be considered a long-term solution except for conditions for which they are clinically indicated (e.g. Barrett’s oesophagus, Gastrinoma (Zollinger Ellison Syndrome).
What are NET Specialists saying about this?
The best source of information on this seems to be in two main areas:
1. One is NOLANETS (Dr Eugene Woltering et al) who appear to be leading the way on identifying those who may have a clinical indication for use of H2 blockers rather than PPI and this NET Specialist organisation has produced a sheet showing how to taper people off the drug and onto the less risky H2 blockers. Read the NOLANETS “Get off PPIs” Sheet by clicking here. They state that PPI use increases circulating gastrin which in turn increases the amount of acid in the stomach. The increase in gastrin also stimulates the enterochromaffin like cells (ECL) of the stomach to produce Chromogranin A and this explains why it can be elevated in PPI users. The US Pharmacy magazine quoted above, appears to confirm this thinking.
2. The European NET Society (ENETS) discusses the issue in their guidelines but only in relation to Zollinger-Ellison Syndrome (ZES). This is a direct quote from ENETS 2016 Guidance – “The widespread use of PPIs is a major problem for the diagnosis of ZES because these drugs have an extended duration of action (up to one week), they cause hypergastrinemia in 80-100% of all normal subjects, and can confound the diagnosis. Furthermore, if PPIs are abruptly stopped in a true ZES patient, anti-peptic complications can rapidly develop, and therefore some expert groups have recently recommended that the diagnosis of ZES should be established without stopping the PPIs or by attempting to taper the dose. Unfortunately, as suggested in a number of recent papers, in most patients, the diagnosis cannot be easily established without an interruption of the PPIs. Furthermore, a secretin test cannot be used while a patient is taking PPIs because it can result in a false positive test. Other tumour markers such as serum chromogranin A were found to be not reliable for the diagnosis of ZES patients, as up to 30% have normal plasma chromogranin A levels. PPIs also lead to increased chromogranin A levels on their own. It is therefore recommended that if the diagnosis is unclear, the patient should be referred to a centre of excellence and if this is not possible, PPI withdrawal should be cautiously performed (in an asymptomatic patients with no active acid-peptic disease or damage) and with adequate cover by H2 blockers and careful patient monitoring”.
PPIs and PERT
I have anecdotal evidence that people are being prescribed PPIs alongside Pancreatic Enzymes Replacement Therapy (e.g. Creon, Nutrizym etc). While most types of PERT are gastro-resistant, a high acid environment may impair their efficacy. The rationale behind using PPI (or H2 blocker) is to decrease the acid level and allow the PERT to work better. Given the research behind this article, I would certainly challenge the use of PPI alongside long-term use of PERT.
The aim of this article is not to scare anyone, I’ve been careful with the sources, quotes and facts. Like anything in life (including the medical world), there are risks and knowing about them allows us to manage these risks in conjunction with our doctors and healthcare specialists. If you are concerned about anything you find inside this article, I suggest you speak directly to your doctor/specialist for advice.
Personally speaking, I would like to see more from the NET Specialist community on this issue.