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.
Look on any site about Neuroendocrine Tumours (NETs) and you’ll find the term IBS (Irritable Bowel Syndrome) frequently mentioned. That’s because it’s a common misdiagnosis for many before being formally diagnosed with NETs.
But what exactly is IBS, why is it such a common misdiagnosis for many NET patients and how can these misdiagnoses be prevented or reduced in future? I just spent a few hours doing an online training course on IBS and I want to pass on some stuff I found to be very useful. I have never been diagnosed with IBS but having researched the issue through some training, I can understand why it might be in the thoughts of a general practitioner for many scenarios. Much of my research was focussed on the British Society of Gastroenterology (BSG) who sponsored the online course I completed which also used material from their magazine Gut, a leading international journal in gastroenterology.
What is IBS?
Irritable bowel syndrome (IBS) is a common, long-term condition of the digestive system. It can cause bouts of stomach cramps, bloating and excessive wind, diarrhoea and/or constipation, feeling of incomplete emptying, mucus in stool; and many other symptoms (see NHS IBS site) The symptoms vary between individuals and affect some people more severely than others. They tend to come and go in periods lasting a few days to a few months at a time, often during times of stress or after eating certain foods. IBS is a heterogeneous condition with a range of treatments. There are in fact different classifications of IBS and the diagram below supports the list with some context:
IBS-D – diarrhea based
IBS-C – constipation based
IBS-M – mix of both diarrhea and constipation
You can see why someone presenting with diarrhea or IBS-D type symptoms might be automatically assumed to have IBS despite the fact that these symptoms could also apply to many other conditions including several cancers. However, what I also found is that in the UK, there is now updated guidance from NICE (National Institute for Health and Care Excellence) to aid GPs and other physicians on how to properly diagnose IBS. In fact, the online course I undertook is one of many now being offered to medical staff as part of the new guidance. That sounds like a good thing in practice (although I did notice some differences between the BSG recommendations and what is published by NICE…….. £ )
How is IBS diagnosed?
That is considerably complex as the symptoms are fairly general. However, I was encouraged to find that doctors should assess any ‘red flag’ indicators that would need referral to secondary care before any firm decision on IBS was made. These include (but not limited to), unintentional and unexplained weight loss, rectal bleeding, family history of bowel or ovarian cancer, a change in bowel habit to looser and/or more frequent stools persisting for more than 6 weeks in a person aged over 60 years, anaemia, abdominal masses, rectal masses, inflammatory markers for inflammatory bowel disease. That’s very interesting because in 2010, after mentioning some unintentional weight loss, my GP said ‘anaemia’ to me and referred me to secondary care. Perhaps I was lucky but perhaps, my GP’s team were just professional and thorough? That said, if you’re with me so far, you can see why IBS might be an easy diagnosis to make for someone presenting with either diarrhea/constipation issues with no other obvious symptoms or abnormal test results (particularly IBS-D).
Why might NETs be frequently misdiagnosed as IBS?
Using the NICE guidelines, I noted there are a range of tests to preclude other diagnoses including: full blood count (FBC), erythrocyte sedimentation rate (ESR) or plasma viscosity, c‑reactive protein (CRP), antibody testing for Coeliac disease. Whilst abnormal results of these tests might show up something to investigate further (i.e. FBC – haemoglobin worked for me), none of them include looking ‘inside’ the patient and I guess there is a resource/finance issue involved here.
In fact, the guidelines also list a number of tests that are NOT necessary to confirm a diagnosis of IBS. These include: ultrasound, rigid/flexible sigmoidoscopy, colonoscopy, barium enema, thyroid function test, faecal ova and parasite test, faecal occult blood, hydrogen breath test. You can see the issues ………..
The guidelines go on to say that a diagnosis of IBS should be considered only if the person has abdominal pain or discomfort that is either relieved by defecation or associated with altered bowel frequency or stool form. This should be accompanied by at least two of the following four symptoms:
abdominal bloating (more common in women than men), distension, tension or hardness
symptoms made worse by eating
passage of mucus.
Other features such as lethargy, nausea, backache and bladder symptoms are common in people with IBS, and may also be used to support the diagnosis
I also noted that the causes of IBS are inextricably linked with Psychological issues and the guidance also includes therapies including cognitive behavioural therapies (CBT) relaxation therapies, and hypnosis.
So if you’re one of the unlucky ones who has presented with “IBS like” symptoms and have normal test results as per above, you may not get the opportunity to get to further testing to find the true diagnosis. It’s possible that you saw a physician who has not followed guidelines for diagnosing IBS, if indeed such guidelines were available to him/her. The inclusion of psychological issues also connects with many anecdotal stories of NET patients being told they needed psychological help before eventually being diagnosed with NETs.
I can see many similarities in the descriptions of IBS symptoms and the sort of things you can read on NET forums – curiously including the effects of NET Cancer surgery and other treatment after diagnosis.
Slight digression but if this subject is of interest, you may like to comment. I once said to my Oncologist that I felt as if I had IBS since my surgery and somatostatin analogue treatment. In fact, I told him that I thought my bowel was more than irritated, it was bloody angry 🙂 During my research, I couldn’t help noticing that some of the suggestions and recommendations for IBS are similar to that offered to a post surgical NET patient. You may therefore like my blog series on Nutrition which was co-authored by a NET specialist dietician who is also IBS aware.
Preventing or Reducing a Misdiagnosis of IBS (all illnesses)
The course looked at this angle as it was clearly keen to emphasise this to medical people going through the module. The NICE guidelines read like a process which must be strictly followed but at the end of the day, they are just ‘guidance’ and should not stop doctors thinking outside the box.
A recent study suggested that as many as 1 in 6 patients (~16%) with symptoms of IBS had another disease. Approximately 7% had Crohn’s disease, 3% coeliac disease, and 2% microscopic colitis when they were formally tested. Patients with IBS-diarrhoea predominant more often had abnormalities than those with IBS-constipation predominant (interesting for NETs). The paper stresses the importance of tailored investigation of patients presenting for the first time.
One in six patients with symptoms compatible with IBS without alarm features in this selected group exhibited organic GI disease following investigation. Assessment of alarm features in a comprehensive history is vital to reduce diagnostic uncertainty that can surround IBS. You can, if you wish, read the abstract of the paper on the link below.
The issue here is that people not meeting the criteria for further checks may be precluded for scans and other tests due to lack of clinical evidence and their diagnosis of IBS will stand. As this was a study, clearly some of them might have gone on to present with sufficient clinical evidence to warrant more checks and subsequent diagnosis of something else at a more advanced stage. Clearly this sounds familiar with NETs. However, looking at the figures above, I suspect the figures for NETs IBS misdiagnoses are pretty small in comparison to those who are correctly diagnosed with IBS. To put that into context (in the UK), according to the NHS, IBS is thought to affect up to one in five people (10,000,000 in UK alone) at some point in their life, and it usually first develops when a person is between 20 and 30 years of age and around twice as many women are affected as men. Compare that with a UK NETs prevalence of around 40,000 (guesstimate), you can see that a misdiagnosis of IBS for NETs, is not that common. However, one misdiagnosis is one too many.
Having done this course and read the accompanying references (some only abstracts), I can see the scope for people with many different illnesses being misdiagnosed with IBS. However, the use of alarm symptoms and ref flag indicators should be helping to reduce this. I’m guessing that many people in first line care may not be fully aware of the IBS guidelines to be able to take heed. I’m also guessing that in the UK (at least), a 10 minute appointment with a busy GP is just not going to solve some of these symptom clashes and many visits might be required to move forward.
It’s really difficult to advise anyone going through a diagnosis of IBS as to how to approach a physician who says they have IBS and they think this is wrong. In the case of NETs, other cardinal symptoms may be of use in convincing physicians (e.g. flushing). Armed with this knowledge, I would say to anyone who suspects NETs but are faced with an IBS diagnosis, take a copy of the BSG and NICE guidelines to your doctor and tick off all the differential ‘ref flag’ and ‘alarms’ issues ensuring that each has been tested before accepting the IBS diagnosis.
References used to support compilation of this blog:
I recently blogged about a well-known BBC political reporter who has a Neuroendocrine Cancer with a Lung Primary. However, in the usual media ‘double speak’ which can sometimes pervade the coverage of such events, he is said to have Lung Cancer. As I said in that article, sometimes with Neuroendocrine Cancer – the devil is in the detail and you just need to dig to find it. Annoying, we shouldn’t need to dig as he doesn’t have Lung Cancer. I wrote about this anatomical issue here. This is exactly what happened to Steve Jobs and Aretha Franklin.
No sooner had I published the Nick Robinson article, I was alerted to the broadcasting of a film about rock star Wilko Johnson who has the most amazing story to tell. Wilko is a former member of Dr Feelgood, a famous British R&B band who were pretty popular in the 60s/70s/80s and remain so today.
In 2014, Wilko was diagnosed with Pancreatic Cancer and was told he had a year to live. One year later, a photographer friend Charlie Chan (who just happened to be a doctor) commented that he looked too well and was still doing his routine and fast-paced musical performance, that something was perhaps not right about this diagnosis. To cut a long story short, he was retested and re-diagnosed with a Neuroendocrine Cancer with a primary in the pancreas. However, it was a large tumour (7 lbs in weight and the size of a melon) and the surgery was ‘extreme’ taking 11 hours and with a lower than normal chance of survival. He seems to be doing OK so far. However, the ‘double speak’ is also being used in his case as nearly all reports and news articles state he has Pancreatic Cancer.
Until I saw the film on BBC1 a few days ago, I hadn’t realised the film was even being made. The first half of the film is really about a man who thinks he is going to die and he doesn’t really have an issue with this – he misses out all the usual emotions moving straight to acceptance. He also decided to do a ‘final’ gig teaming up with Roger Daltrey (The Who) – you can watch this from a link below.
I suspect the film sponsors were totally surprised to be continuing the film to include his re-diagnosis, his surgery and the beginning of his recuperation (I suspect Wilko was more surprised though). Having now watched the full 90 minutes, I can say I enjoyed it (particularly the second half) but I suspect it won’t be everyone’s ‘cup of tea’. However, it gives an insight into the man himself along with his journey. Some of the music clips will get your feet tapping. A little bit of me wants to get to know him more as I hadn’t realised he is a bit of a philosopher (ex-English teacher) and an astronomer in addition to being a rock star and generally down to earth ‘geezer’. There are some good quotes in the film including “if the cancer is going to kill me, I don’t want it to bore me”. This probably explains his very positive attitude when told he would die and decided his ‘new normal’ would be his usual normal!
There is not a single mention of Neuroendocrine Cancer (unfortunately). However, the film came out on DVD 11 Dec 2015. There might be limitations on playback in non-UK countries but WATCH THE TRAILER click here
I have in fact had an online chat with Wilko Johnson who said he would help with Neuroendocrine publicity (not yet seen though). Check out the conversation here:
There’s also an interesting interview with Roger Daltrey (member of The Who and friend of Wilko). There is more detail of this “other” cancer and his recuperation but again the word Neuroendocrine is not mentioned. Watch Here
Here is the gig with Roger Daltrey, which Wilko thought this would be his last. Watch Here
Having watched the film, I now have more sympathy with Wilko’s position and there’s a bit of me thinking we might hear some more about his condition downstream ………..
23 May 2016 – two new clips to add to the story:
1. Newspaper interview 21 May 2016. Click Here.
2. Interview with Victoria Derbyshire on 23 May 2016. Click Here.
OPINION. Sometimes when I’m searching for cancer information, I’m presented with a ‘pick-list’ of types which mostly tend to be anatomy based. I do find it annoying when I cannot find my own cancer on the list …..some respectable organisations are just not as up to date as they should be! I can now totally understand why so many Neuroendocrine Tumour (NET) patients have become their own advocates and why they have to shout quite loud for recognition and understanding.
One of the key facets of NETs is that it is not tied to a particular part of the human anatomy. Unlike (say) lung cancer, where the primary is in the lung, or breast cancer where the primary can be found in the breast, neuroendocrine tumours arise from a cell type which can be present more or less anywhere in the body. Ignorance of this fact can at best lead to misinformation and confusion about Neuroendocrine Cancer – at worst, misdiagnosis and unnecessary treatment for something else (including a different type of cancer in the same location – see below for an example). Take my own diagnosis phase. When I look at the radiology reports produced prior to diagnosis, there were mentions of ‘peri-aortic lymphadenopathy’, ‘mass in the small bowel mesentery’, ‘multiple liver lesions’, ‘retro-peritoneal fibrosis’, ‘extensive lymphadenopathy consistent with lymphoma or metastatic adenocarcinoma’. You can see from the mostly generalised wording, there was some scope for confusion given that 3 potential cancers were mentioned in one paragraph. However the biopsy confirmed NETs. That is what is now documented, that is what I tell people I have and that is what I’m treated for.
The point I’m making here is that certain cancers can appear almost anywhere in the body. Neuroendocrine Cancer is one of those. For example, a Neuroendocrine Tumour which originates in the intestines isn’t Bowel or Colon cancer. Similarly one which originates in the (say) Pancreas or Lung should not be confused with ‘core’ Pancreatic or Lung cancers. These are all histopathologically different cancers to NETs, they arise from different cells and the presentation, testing, treatment (curative or palliative) and prognosis can be very different. At worst, the wrong treatment will shorten the patients life. This is another key point as Neuroendocrine Cancers really need NET specialist medical teams (although there are certain types which I suspect on occasion may require external experts in conjunction with NET specialists).
Take the quite recent case in the news about Wilko Johnson, a well known R&B musician who was told he had Pancreatic Cancer and would die within 10 months. But a friend (a doctor) became curious as to why he wasn’t dead after 10 months and why he wasn’t even feeling ill! It was then discovered he had a NET, i.e. he had a Neuroendocrine rather than exocrine based cancer of the pancreas. So he went from dying to living (albeit living with the consequences of the cancer). Of course the newspapers even today continue to report he has “Pancreatic Cancer”. Read his amazing story by clicking here.
Dave Thomas the founder of Wendy’s Hamburger Chain had a Neuroendocrine Tumour but many newspaper reports said he died of liver cancer. Whilst they got the detail of the cancer correct, the ‘headline’ location is technically wrong as the liver was a metastasis (a secondary location). This robs us of vital awareness messages due to the ‘headline reading only population’.
They are not alone, the most famous NET patient is the late Steve Jobs (the founder of Apple). To this day, it is frequently reported he had “Pancreatic Cancer” when in fact he had a Neuroendocrine Cancer with a primary in the Pancreas. I see this error repeated weekly in my news alerts plus with many other diagnosed patients. Read a very details Steve Jobs story by clicking here.
However, on 16th August 2018, some might say a person more famous than Jobs was diagnosed with Neuroendocrine Tumors (pancreatic primary). Aretha Franklin was initially diagnosed in 2010, other than pancreatic primary, other details are scant as she wanted to keep her condition private. However, the media exploded with claims she died of Pancreatic Cancer, although several outlets did mention it was the ‘Neuroendocrine type’ and many left that bit out. Although this left a little door open for Neuroendocrine awareness, the community faces a very difficult task in regaining the high ground and it is looking like ‘Steve Jobs’ all over again as the news went viral. That said, it appears her death certificate does confirm Neuroendocrine Cancer. Read more by clicking here.
On 13 Jan 2017, it was announced that Siri Co-Founder Dag Kittlaus has Pancreatic Cancer. Although the detail said Pancreatic Neuroendocrine Tumor, it is still a misleading statement and once again, the headline reading population receive only the Pancreatic Cancer message.
There are huge differences between Pancreatic Cancer and Neuroendocrine Cancer with a pancreatic primary – click here to read more.
I’ve also lost count of the number of times I’ve read regional and national patient stories where the headlines mentioned various parts of the anatomy only to find it was a Neuroendocrine Tumour in the detail. Frustratingly, many of these articles are also fundraising for the wrong type of cancer in addition to the misdirected awareness messages.
The most well-known Neuroendocrine Cancer patients are so famous, thousand of articles were written about them when they died and continue to this day. These articles are ingrained in the bowels of the web and in books – many people will use them as research to reference in their own articles. This issue will continue for many years.
The same thing is now happening with UK celebrities Nick Robinson and Wilko Johnson to a certain extent (although Neuroendocrine is starting to creep into their vocabulary). I have in fact had an online chat with Wilko Johnson who said he would help with Neuroendocrine publicity (not yet seen though). Check out the conversation here:
Let me add that this is not an attempt to bash Aretha Franklin, Steve Jobs, Nick Robinson, Wilko Johnson, Dag Kittlaus or any patient, or any patient advocate organisation who have been recipients of cash raised for a different cancer. I believe patients mostly only say what their doctors say to them in terms of cancer type.
The power of social media will help to dilute the incorrect publishing of celebrities with the wrong cancer types, a particularly disadvantage for Neuroendocrine Cancer. The more stories and articles like this one, the more we can do to counter the onslaught of incorrect articles which are denying our cancer the publicity we deserve. The share button is below.
Whenever I need to know anything nowadays, I mostly just look on the internet and sometimes I ask my virtual PA ‘Alexa’ to look for me! However, you need to be very careful in acceptance of what is credible information and what isn’t.
As a relatively experienced health blogger and activist, I like to think of myself as ‘internet savvy’, so I occasionally find myself using ‘Dr Google’ to diagnose my aches, pains and unusual feelings (and I confess to using it to help others). I mostly find there are no real or definitive answers online for patient issues. Although I seem to learn something on each piece of research, I also find some really worrying stuff. Some symptoms can have dozens of reasons and I often realise how difficult it can often be for a doctor faced with unusual, vague and nonsensical symptoms!
On a recent online symptom check for lower left abdominal spasms, I discovered I was pregnant with an alien baby!
The internet is really powerful but also really dangerous. For example if you look up “best treatment for cancer”, you have an astonishing 300 million offerings. Right there with rigorous, evidence-based sites, there are those offering fermented foods and DIY cancer cure kits (e.g. fake healthcare news and cancer myths). Worried patients sometimes need help to distinguish between sensible advice and fanciful claims/ miracle cures.
When I combine my own experience with what I read on patient forums, I can see that internet searching is not for the faint of heart. Some people are already in a state of anxiety before they started searching Dr Google’s archives, and what they find has probably made their anxiety worse. In fact, the rise of the internet has created a new term for those who worry themselves sick and continually misdiagnose symptoms on the internet – ‘Cyberchondriac’.
Even when we know ‘googling’ our symptoms won’t end well, we don’t seem to care, we just need answers! Searching authoritative sites is therefore really important and the availability of proper medical information online is actually putting more power in the hands of patients. It’s how we as patients exploit it that is really important. Just as you can find examples of ‘cyberchondria’ online, you can also find examples of patient power in a doctor’s office. Worryingly, you can also find examples of ‘Dr Google’ being right after being dismissed by real doctors, sometimes resulting in patient illness or even death.
The medical community need to accept that searching for more information is a natural patient instinct, not a slight against one’s doctor. The profession will have to get better at educating the next generation of doctors now that Dr Google is here to stay and, I think, to help. That said, I don’t believe the internet will ever replace the profound human dimension of the doctor-patient relationship.
Tips for online searching:
1. Don’t actually use internet search engines if you can help it, go to a reputable site and then search that. For NETs try RonnyAllan.NET
2. Try to be specific as possible because vague search terms will result in frightening answers, and in practice any symptom can be read as a sign for nearly every single horrible illness, or a worsening or recurrence of an existing condition.
3. Less common conditions are less common, and minor symptoms often resolve themselves in time. If you have more worrying symptoms, or if your symptoms are changing or progressing, then go ‘offline’ i.e. visit your GP or primary care facility. If you’re sure of your facts, be assertive until you’re convinced otherwise. However, accept that the internet may be wrong when you seek medical help.
5. If you’re someone with an already diagnosed serious illness, theworry that goes with that is quite understandable – check out my 8 tips article. However, the same tips apply although you may now have established your own specific sources of advice in addition the general health areas.
6. Charities and associations for specific conditions are also a good information source but just note they may not have the best or up to date simply because they have been granted a ‘charity’ or equivalent status, so be careful, I’ve been some complete rubbish on these sites. Patient forums can be ‘frighteningly good’ but they can also be ‘good at frightening’. Personally, I try not to compare myself to strangers on the internet.
OK, the lead graphic is slightly ‘tongue in cheek’ but for those who are very anxious, it’s a reality. I can see from my own group that many Neuroendocrine Cancer patients have become very adept at searching online – useful because many still need a lot of help.
Be careful out there it’s dangerous. I have a private group for patients and caregivers where I like to ‘keep it real’. Check it out here.