You may also enjoy my Invisible Illness ‘PoNETry’ – click here
Ronny Allan’s ‘PoNETry’ © series can be shared with poetry credit to:
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You may also enjoy my Invisible Illness ‘PoNETry’ – click here
Ronny Allan’s ‘PoNETry’ © series can be shared with poetry credit to:
Thanks for reading
On the day I was diagnosed, I hadn’t really thought about questions, the only one I actually remember asking was “how long do I have left to live” (I watch too many movies!). On the day of diagnosis and a period beyond, people tend to feel emotions of shock, denial, anger and sadness, before going on to accept their situation. Yes, I ‘googled‘ but not a great deal really – although some things I found did frighten me. I wish I had found this article way back then.
As things progressed in the weeks after ‘D-Day’, I started to work out the sort of things to ask but even then it was limited. I had been referred to an experienced NET team so I felt confident they would do whatever needed doing. In hindsight, I can now think of a quite a few questions I should have asked. That said, I suspect my team probably gave me the answers without having been asked the questions!
My blogging efforts have turned into a ‘Community’ of sorts. Consequently, I’m contacted daily from people finding me on the web. Many of these people are at the pre-diagnosis or initial phase. Many are undiagnosed. Most are looking for information and some sound like they are already at the ‘acceptance stage’; some are frightened about the future, some are angry because they think they are not being told important information and some also feel they have been messed about or ‘fobbed off’ by their doctors. Of course I’m happy to help but only after reminding them that I’m just a wee Scottish guy with the same disease!
I have to say that some people arrive on my site without a diagnosis but often seem to be very well prepared – the power of the internet I suspect. The questions I mostly get involve finding experts and then what questions to ask them.
As an extra bonus to this post, I offer you a starting point for the best places I know for finding NET expertise:
Europe – here at ENETS: European NET Centres of Excellence
UK – here at UKINETS: UK NET Centres
Australia – here: Australian NET Doctors
New Zealand – Dr Ben Lawrence, based in Aukland.
Canada (from patient knowledge):
Russia – Clinical Oncology Research Institute, N. N. Blokhin RCRC RAMS, Address: 24, Kashirskoye sh., Moscow, 115478, RF. NET specialist Alla Markovich
In my Group – ask other patients: Click here to join.
Many people ask me what sort of questions to ask and because NETs is such a diverse bunch of diseases, that leads to me ask them a series of questions to ascertain what they might consider asking. I’m not surprised to find some are unable to answer my questions and so those then become some of their questions to ask!
Also, questions don’t end at the diagnosis phase, they continue and in fact, some of the answers to the questions below, may bring up new questions in your mind. Some of these questions can be asked time and time again in the event of issues downstream.
If you’re currently confused about the essential facts of your condition, you’re not alone. In a recent study, almost half of cancer patients did not know basic stuff such as grade and stage of cancer, and after their initial treatment, whether they were free of disease or in remission.
For those entering or are recently just beyond the diagnostic phase, you may find certain questions cannot yet be answered without further test results etc. However, if the answer is not yet known for whatever reason, at least you have it on your list for follow up appointments. Consequently, I’ve constructed this list of questions that should function as a generic set. There may also be ‘specific to country’ questions such as insurance cover in addition to this suggested list. Of course, some of you may not want the answer to so certain questions. That’s perfectly understandable, so don’t ask!
1. Where is my primary tumour and what type of NET is it?
This is a fundamental question and it’s likely many will already have some inkling about location and perhaps a type. The difference between NETs and other types of cancer is the primary can be found wherever there are Neuroendocrine cells rather than a specific part of the anatomy in terms of naming the type of cancer, i.e. a NET of the pancreas is not Pancreatic Cancer.
The type of NET is key as it will drive a lot of other stuff including treatment. Location and type of NET are not always aligned, for example, you may have a NET in your Pancreas but there are several types of Pancreatic NET (or pNET) and these may depend on identification of a particular hormone (see syndrome below). Many NETs are non-functional (there is no oversecreting hormone).
For some the primary will not yet be found (i.e. cancer of unknown primary or CUP). There may also be multiple primaries.
2. What is the grade and differentiation of my tumour(s)?
Another fundamental question as this defines the aggressiveness of the disease and is absolutely key in determining overall treatment plans. Treatment plans for poorly differentiated can be very different from well differentiated. Read more here – Grading and here – Benign or Malignant
3. What is the stage of my disease?
Fundamental to understanding the nature of your disease. Stage confirms the extent of your disease, i.e. how far has it spread. Again this will drive treatment plans and long-term outlooks. Scans are really important in determining the Stage of your cancer – check out my scans post here. Read more here on Staging
4. Do I have a NET Syndrome?
Many NET patients will have been experiencing symptoms prior to diagnosis, perhaps for some time. It’s possible these symptoms form part of what is known as a ‘Syndrome’ and there are several associated with NETs. Syndromes are mostly caused by the effects of over-secretion of hormones from the tumours, a hallmark of Neuroendocrine disease. Carcinoid Syndrome is the most common but there are many more depending on the primary location. Read more here – NET Syndromes.
5. What is my treatment plan, and what are the factors that will influence my eventual treatment? When will I start treatment
This is a very complex area and will depend on many factors. Thus why your specialist may not have the answers to hand. Decisions on treatment are normally made by some form of Multi-disciplinary Team (MDT). Many people diagnosed with cancer expect to be whisked away to an operating theatre or chemotherapy treatment. However, for many this is not what actually happens. Depending on what testing has been done up to the actual diagnosis, it’s possible that even more testing needs to be done. Additionally, for those with an accompanying syndrome, this will most likely need to be brought until control before certain treatments can be administered; and even then, there may be checks to make sure the treatment will be suitable. Sometimes it’s a case of ‘Hurry up and wait’. My first treatment was 6 weeks after diagnosis and that was designed to control my syndrome ready for surgery which was undertaken 14 weeks after diagnosis. It’s also possible you will be placed on a ‘watch and wait’ regime, at least to begin with.
6. Can you comment on the potential for my type of NET to be related to any familial or genetic aspects of cancer?
A small percentage of NETs are hereditary/genetic in nature. This is mostly associated with those who have Multiple Endocrine Neoplasms (MEN) syndromes and a few other less common types of NET including Pheochomocytoma / Paraganglioma(Pheo/Para) and Medullary Thyroid Carcinoma (MTC) (the familial version of MTC is often referred to as FMTC). However, please note this does not mean that all those diagnosed with pancreatic, parathyroid, pituitary, Pheo/Para and MTC tumours, will have any hereditary or genetic conditions, many will simply be sporadic tumors.
7. Will you be able to get rid of all my disease?
This is a really difficult question for any specialist, even a Neuroendocrine expert. All published articles on NETs will say they are a heterogeneous collection of diseases (i.e. consisting of dissimilar entities) which makes this question (and others) difficult. I have read articles written by the world’s foremost NET experts and they all have the word ‘curative’ mentioned in various places. So I guess in particular scenarios with certain NETs, and if the disease is caught early enough, that possibility exists. However, for many, the disease could be incurable, particularly where there is distant metastasis. But, the disease has many treatment options for most types and for many it is possible to live as if it were a chronic condition. I call it ‘incurable but treatable’. Read more here – Incurable vs Terminal
8. What Surveillance will I be placed under?
Again, this is very individual in NETs and is mainly dependent on type of NET, grade and stage and how the patients reacts to treatment. This may not be known until you have undergone your initial treatment. For example, surveillance scans can be any period from 3 months to 3 years depending on tumour type(location) and stage/grade. Marker testing tends to average around 6 monthly but could be more or less frequently depending on what’s going on. Read more here – click here
9. Will I receive support and specialist advice after my treatment?
Let’s not be afraid of the word ‘Palliative’, it does not always mean ‘end of life’ care. Another example is nutrition. Many people with NETs, the condition in combination with the side effects of treatment may necessitate an alteration of diet and this is a very individual area. I would also emphasise that dietitians not well versed in NETs might not offer the optimum advice. Read more – My Nutrition Series.
10. How will treatment affect my daily life?
This is a question that many people miss but it’s becoming more important as we all live longer with cancer Again, this may not be possible to answer immediately but perhaps this question could be reserved once you know which treatment(s) you will be receiving. All treatment comes with side effects and can last for some time or even present with late effects after some years. The ‘consequences’ of cancer treatment need to be factored in earlier so that the necessary knowledge and support can be put in place. See also Unmet Needs for NET Patients
I suspect others will have suggestions for this list so feel free to submit these to me. I quite often refresh my posts over time.
In the last 12-24 months, there seems to have been announcement after announcement of new and/or upgraded/enhanced diagnostics and treatment types for Neuroendocrine Cancer. Scans, radionuclide therapies, combination therapies, somatostatin analogues, biological therapies, etc. Some of the announcements are just expansions of existing therapies having been approved in new (but significant) regions. Compared to some other cancers, even those which hit the headlines often, we appear to be doing not too badly. However, the pressure needs to stay on, all patients need access to the best diagnostics and treatments for them; and at the requisite time. There’s even more in the pipeline and I’m hoping to continue to bring you news of new stuff as I have been doing for the last year.
Some of these new diagnostics and treatments will benefit eligible patients who are in diagnosis/newly diagnosed and also those living with the disease. As we’re now in our awareness month, let’s recap:
Many NET Patients will undergo a nuclear scan to confirm CT results and/or to detect further neuroendocrine activity. Basically, a nuclear substance is mixed with a somatostatin analogue, injected into the patient who is then scanned using a 360-degree gamma camera. As gamma cameras are designed to show up radioactive activity; and as Neuroendocrine Tumour cells will bind to the somatostatin analogue, it follows that the pictures provided will show where Neuroendocrine tumours are located. Many people will have had an ‘Octreotide’ Scan (or more formally – Somatostatin Receptor Scintigraphy) which is still the gold standard in many areas. The latest generation of nuclear scans is based on the platform of the Gallium (Ga) 68 PET Scan. The principles of how the scan works is essentially as described above except that the more efficient radioactive/peptide mix and better scan definition, means a much better picture providing more detail (see example below). It’s important to note that positive somatostatin receptors are necessary for both scans to be effective. Europe and a few other areas have been using the Ga-68 PET scans for some time (although they are still limited in availability by sparse deployment). The latest excitement surrounding this new scan is because they are currently being rolled out in USA. Read about the US FDA approval here. You may hear this scan being labelled as ‘NETSPOT’ in USA but this is technically the name for the preparation radiopharmaceutical kit for the scan which includes a single-dose injection of the organic peptide and the radionuclide material. Take a look at a comparison of both scans here:
Similar to above, this treatment has been in use in Europe and other places for some time but is also to be formally deployed in USA if, as is expected, the US FDA approval is positive at the end of this year (Read here). In the most basic terms, this is a treatment whereby a peptide is mixed with a radionuclide and is drip fed over a number of treatments (normally up to 4 spaced out over a year). The concept of delivery of the ‘payload’ to the tumours is actually very similar to the preparation for a radionuclide scan as described above, the key difference is the dosage and length of exposure whilst the tumours are attacked. Once again, receptors are important. The NETTER series of trials showed good results and this is an excellent addition to the portfolio for those patients who are eligible for this treatment. Fingers crossed for the US FDA announcement due by the end of this year. Also fingers crossed that PRRT returns to the NHS England & Wales portfolio of available treatments next year. The Carcinoid Cancer Foundation has an excellent summary of PRRT here.
Whilst on this subject, I also want to highlight the innovative use of combo therapies in Australia where they are combining PRRT and Chemo (PRCRT). I blogged about this here:
Somatostatin analogues are a mainstay treatment for many NET Patients. These drugs target NET cell receptors which has the effect of inhibiting release of certain hormones which are responsible for some of the ‘syndromic’ effects of the disease. Again, receptors are important for the efficacy of this treatment. You can read the ‘geeky’ stuff on how they work here. These drugs mainly comprise Octreotide (provided by Novartis) and Lanreotide (provided by Ipsen). The latter has been around in Europe for 10 years and was introduced to North America earlier this year. Octreotide has been around for much longer, almost 17 years. When you consider these peptides have also been used to support nuclear scans that can detect the presence of tumours; and that studies have shown they also have an anti-tumour effect, they really are an important treatment for many NET Patients. I’ve blogged about new somatostatin analogues in the pipeline and you can read this here. This blog also contains information about new delivery systems including the use of oral capsules and nasal sprays (…….. very early days though).
For maintenance and quality of life, the release of a Telotristat Ethyl for Carcinoid Syndrome is an exciting development as is the first new treatment for Carcinoid Syndrome in 17 years. This is a drug which is taken orally and inhibits the secretion of serotonin which causes some of the symptoms of the syndrome including diarrhea. It must be emphasised it’s only for treating diarrhea caused by syndrome and might not be effective for diarrhea caused by other factors including surgery. Read about how it works and its target patient group in my blog here.
The announcement of a clinical trial for the Oncolytic Virus (an Immunotherapy treatment) specifically for Neuroendocrine Tumours is also very exciting and offers a lot of hope. Click the photo for the last progress update.
Earlier this year, AFINITOR became the first treatment approved for progressive, non-functional NETs of lung origin, and one of very few options available for progressive, non-functional GI NET, representing a shift in the treatment paradigm for these cancers. It’s been around for some time in trials (the RADIANT series) and is also used to treat breast and kidney cancer. It’s manufactured by Novartis (of Octreotide fame). It has some varying side effects but these appear to be tolerable for most and as with any cancer drug, they need to weighed against the benefits they bring.
In technical terms, AFINITOR is a type of drug known as an ‘mTOR’ inhibitor (it’s not a chemo as frequently stated on NET patient forums). Taken in tablet form, it works by blocking the mTOR protein. In doing so, AFINITOR helps to slow blood vessels from feeding oxygen and nutrients to the tumour.
………. and relax! Wow, I’ve surprised myself by collating and revising the last 12-24 months. Dr James Yao also agrees – check out his upbeat message in the attached 2 page summary. You may also like another upbeat message from Dr Jonathan Strosberg by clicking here.
Neuroendocrine Cancer – who’d have thought it? ….. a bit of a dark horse.
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When I was diagnosed with metastatic Neuroendocrine Cancer on 26 July 2010, I just wanted them to hurry up and fix my body so I could get back to normal. My expectations of speed turned out to be wildly inaccurate and in hindsight, I was also wildly naive. You see, with Neuroendocrine Cancer, particularly well-differentiated, low or medium grade tumours, it sometimes doesn’t work as fast as you would think and there are good reasons for that.
The complexity of the condition needs some consideration as the physicians work up a treatment plan. I’m quite happy and content they took their time, rather than rush into the wrong decisions. If you think about it, this is an advantage with low and medium grade NETs……you normally have some time to get the ducks in a line (and there are some pretty important ducks in that line).
Here’s a very short video discussing this during a patient video shoot: Click here.
I had a confirmed biopsy result following some incidental CT scans and other tests. However, they now needed further checks and marker tests to work out the extent of the disease. So the timeline leading up to major surgery ended up like this:
Diagnosis: 26 July 2010. Met with Oncologist. Grade 2 Small Intestine NET with distant metastasis (Stage 4)
Chromogranin A and 5HIAA: submitted 28 July. Results received 13 August – both elevated, indicating and confirming tumour bulk and function status respectively
Octreotide Scan: 17-19 August. Report issued 24 August – confirmed CT plus additional distant hotshots. Also confirmed my tumour receptors were avid to somatostatin analogues.
Daily Octreotide Injections: Started 9 September to control syndrome (derisk surgery)
Referred to NET Multi-Disciplinary Team (MDT): 15 September – they now had sufficient data to form a treatment plan.
Holiday: Late September (it was booked and I felt OK, why not!)
Further MDT assessment and consultations: 1- 7 October
Bland Liver Embolisation: 19 October
First Surgery: 9 November – to remove primary and debulk local and regional spread.
You can read the rest of my treatment background here.
So it took 75 days from diagnosis to opening me up to remove the first batch of tumours. With reasonably slow-growing tumours, that isn’t really a long time when you consider they had probably been growing inside me for several years. I’m sure others waited even longer.
Sometimes rushing straight into the operating theatre isn’t really the best option. I’m still here!
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Over the last few months, I’ve seen quite a few posts entitled “Not all Cancer is pink”. I suspect it’s a reference to the ubiquitous publicity that many women’s cancer related advocates, bloggers and organisations attract.
Those who use this phrase are perhaps concerned there is an imbalance and inherent unfairness in the distribution of support and are frustrated that their own cancer does not fare as well publicly? I share that frustration, however, I take my hat off to the battalions of advocates, bloggers and organisations who work very hard for breast and the various gyneacological cancers whether they push pink or not (and for the record, they don’t all push or even agree with the ‘pink’ thing).
I’ve even seen this term used within my own community – ‘Not all cancer is pink, some are black and white’. This is clearly an attempt to tie in the well-known ‘pink’ to the not so well-known ‘black and white’. Notwithstanding the potential for upsetting hard-working women’s cancer organisations and the fact that those in the NET community who push the pink ‘insult’, do not have a corresponding ‘Not all cancer is blue’ article, I also think we might be missing a trick.
And here’s the trick which is my alternative view on where we should be focused – Not all Cancer is black and white and nothing in cancer is ever black and white. As I don’t want to indulge in ‘Cancer Olympics’ (it can backfire), I’m clearly talking about the context of the phrase ‘black and white’ rather than the ribbon colours.
Let me explain my logic. There are two sides to most people’s experience or perception of cancer. Firstly, symptoms appear, a diagnosis is made, treatment is applied and if it works, the patient will hopefully go into remission after a period of time, normally 5 years. The other side is that sadly, some people may not survive the ordeal and that even applies to certain so-called ‘pink’ cancers (metastatic breast cancer for example). Clearly there are variations of my very simple binary explanation but these two outcomes are very common scenarios.
However, many cancers (including my own Neuroendocrine Cancer) are often silent, produce vague symptoms, are difficult to diagnose, treatment plans can be a challenge, most metastatic patients and many with other stages will never really be cured, and will need lifelong support (another challenge we need to focus on). They are extremely cunning and sneaky. Neuroendocrine Cancer has many ‘grey’ areas. Clearly there are also variations on this theme but with many scenarios and different outcomes.
If we want more attention, let’s learn from other cancer awareness activities instead of attacking their colours. Lesson No 1 – they don’t use animals as icons because people won’t take them seriously.
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