Diagnosed with Neuroendocrine Cancer? – 10 questions to ask your doctor (and where to find a NET Specialist Worldwide)


find net specilaist and 10 qeusitons

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.

Finding experts

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

USA:

  • One US center is now the first to achieve a European NETs Center of Excellence accreditation – read more hear about University of Iowa Holden Comprehensive Cancer Centerclick here
    NANETS have listed “NET Centers” here – NANETS NET Centers and Clinics
  • The NET Research Foundation as they also have a ‘Doctor Database’ section which differs slightly from CCF below.
  • Here at Carcinoid Cancer Foundation – Find a Doctor

Australia – here: Australian NET Doctors

New Zealand – Dr Ben Lawrence, based in Aukland.

Canada (from patient knowledge):

  • Dr. Simron Singh at Sunnybrook in Toronto
  • Dr. Shereen Ezzat at Princess Margaret in Toronto (PMH)
  • Dr. McEwan, The Cross Clinic, Alberta?
  • Dr Kavan at Montreal Jewish General Hospital (Oncology)
  • Dr Buteau / Beauregard at Quebec Hotel Dieu (Radiation Oncology (PRRT, Ga68)
  • Dr Rivera at Montreal General Hospital (Endocrinology)
  • Dr Metrakos at the Montreal Royal Victoria Hospital (Surgeon) sees a lot of NET patients
  • On the French side Dr Andre Roy at the CHUM in Montreal (surgeon) also sees a lot of NET patients
  • Dr. Jamil Asselah also treats net patients. He is an oncologist….Quebec
  • Michael Sawyer at Cross Clinic in Alberta Edmonton.
  • Drs. Parkins, Card, and Paseka at the Tom Baker CC in Calgary.
  • London Ontario: Dr. David Laidley, Dr. Robert Reid in the Neuroendocrine Clinic at London Regional Cancer Program and Dr. Daryl Gray, Surgeon.

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.

AskDoctor_0

Neuroendocrine Cancer – 10 questions to ask your specialist

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.

Pre-question Check

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.

Palliative Care – it might just save your life

 

The P word

When you’ve been diagnosed with cancer at an incurable stage, certain words start to mean more. Take ‘palliative’ for example.  Before I was diagnosed I had always associated the word ‘palliative’ with someone who had a terminal disease and this type of care was to make the final days/weeks as comfortable as possible. So it was a bit of a shock to find out in 2010 that my treatment was palliative in nature. However, I’m still not dead and I’m still receiving palliative care. Go figure! The answer is simple – the cancer story is changing. What was once feared as a death sentence is now an illness that many people survive. As survival rates increase, so too will the number of people living with the legacy of cancer and its treatment.

What is palliative care?

Some people with incurable cancer will continue to receive treatment to keep the cancer at bay and that treatment is by definition, palliative.  In fact, palliative care can be given at any time during an illness. It’s not just for treatment of the cancer, it’s also to help with the effects of that treatment, i.e. the consequences of cancer.  It also encompasses things such as emotional and other practical support.

In the most general terms and while it clearly can go into some detail and long lists, palliative care can be defined as follows:

Cancer and its treatment often cause side effects. Relieving a person’s symptoms and side effects is an important part of cancer care. This approach is called symptom management, supportive care, or palliative care. Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care.

I looked at a few sites and many of them confirm the above.  However, there appears to be even more sites where it is still heavily associated and inextricably linked with end of life or hospice care where you may come into contact with the term palliative care specialist.  Whilst it’s not wrong to make that association, more work needs to be done to cater for the growing numbers of ‘incurable but treatable’ who are not ‘terminal’ and still need this type of support, in some ways like you would with a chronic condition.  I also sense a push in certain areas to emphasise the meaning of palliative care to include a much broader definition than is currently in most people’s minds.  This needs much more publicity.  I’m not saying that ‘palliative’ does not include ‘hospice care’ but I’m not intending to cover that aspect in this blog which is aimed as those with incurable but treatable cancers.

My palliative care experience

When I was diagnosed with metastatic Neuroendocrine Tumours (NETs) in 2010, I quickly accepted the fact that any treatment I would receive would not be curative.  I also quickly accepted that if I didn’t have any treatment, I would probably die.  The words used were ‘debulking’ and ‘cytoreductive’, more technical sounding but essentially meaning the same thing as palliative.  Debulking means removing as much tumour as possible in order to increase the chance that perhaps other treatments can be of some help. Cytoreductive means the same thing but generally extends the ‘debulking’ activity to other modes of treatment (e.g. chemotherapy/radiotherapy).

NETs is one of a number of cancers for which ‘debulking’ and ‘cytoreductive’ therapies can in many cases confer some survival advantage. In fact if you read ENETS or NANETS guidance for advanced NETs, you will frequently see the statement that cytoreductive surgery should be considered if greater than 90% of metastatic tumour burden can be safely resected or ablated.  NETs, particularly with distant metastases, can come with a ‘syndrome’ and some of the symptoms can be rather debilitating for many patients. These syndromes are a result of tumours secreting excess amounts of hormones and the types vary from patient to patient and from NET type to NET type.  It follows that if surgical debulking reduces the amount of tumours, then it should normally decrease the effects of the associated syndrome.  In fact, one letter from a specialist did describe my surgery in symptom palliation terms. I can confirm this is about right as my hormone marker 5HIAA remained elevated after surgery to remove my primary and local tumours, but did not return to normal until after my liver surgery.

However, there are a number of other treatments that can be considered ‘palliative’ in a metastatic or advanced environment.  Getting rid of tumours is always the optimum treatment for any cancer but just as surgical debulking can reduce the amount of cancer, other non-surgical modalities such as liver embolization or ablation can have the effect of reducing the symptoms of the cancer and therefore providing relief to the patient. Somatostatin Analogues (Octreotide/Lanreotide) are another good example of palliative care.  Although they might have an anti-tumour effect for some, they mostly work by reducing or inhibiting the secretion of excess hormones which contribute to the various NET syndromes.  ‘Symptom control’ is as defined above, palliative care.

I’m already looking forward to my next palliative care appointment.

Thanks for reading

Ronny

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



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Neuroendocrine Cancer – Incurable is not untreatable

Incurable is not untreatable

OPINION. When I was being officially told I had an advanced and incurable cancer, I did what most people seem to do on films/TV ….. I asked “how long do I have“.  The Oncologist said ” … perhaps just months“.  That must have been quite a shock because for a few moments after that, I heard nothing – my brain was clearly still trying to process those words – I wasn’t even feeling unwell! The really important bit I missed was him go on to say “…but with the right treatment, you should be able to live for a lot longer”.  Fortunately, my wife Chris heard it all and I was refocused.  “OK Doc – let’s go” I said.  Always take someone with you to take notes at important meetings with Oncologists!

I continue to see quite a few posts and articles about death and dying and I noticed some patients were using the word ‘terminal‘ to describe Neuroendocrine Cancer, despite in some cases, having been diagnosed some years ago, despite in most cases in reference to well differentiated diagnoses. This label is not just confined to use within Facebook forums, I’ve also seen this on wider social media including twitter, blogs and newspaper items. For some, this appears to be the prognosis given to them by their doctors. I find this surprising. However, I’m much less surprised to see many comments on forums from people who had been told the worst by their doctors but were still alive and kicking WAY beyond those worst case prognostic statements, including the higher grade cases.

Definitions are important so what does ‘terminal cancer’ actually mean? 

I’m conscious there are legal ramifications with the definitions (wills, life insurance, disability etc) and that these may differ on an international/federal basis.  I therefore intentionally confined my searching to a couple of ‘big hitter’ and ‘authoritative’ sites:

Cancer Research UK defines terminal as “When cancer is described as terminal it means that it cannot be cured and is likely to cause death within a limited period of time. The amount of time is difficult to predict but it could be weeks to several months”.

The American Cancer Society defines terminal as “an irreversible condition (it cannot be cured) that in the near future will result in death or a state of permanent unconsciousness from which you are unlikely to recover. In most states, a terminal illness is legally defined as one in which the patient will die shortly whether or not medical treatment is given.”

Can terminal as defined above be applied to Neuroendocrine Cancer? 

I’m sure it can, e.g. with very advanced and very aggressive disease and for any grade when taking into account the condition of the patient and other factors (secondary illnesses/comorbidities, refusal of treatment etc). Clearly, that is a terrible situation.  I’m also conscious that some people do eventually die because of this disease or its consequences and that is also terrible.

How long is a piece of string?

I think with most Neuroendocrine Cancer patients, “how long do I have” can be a tough question to answer. Thinking back to my own situation, although it was an obvious question to ask my Oncologist, I can see it might have caught him unawares.  I suspect he was erring on the side of caution as I don’t believe he had formulated my treatment plan ….. i.e. my case had not yet been looked at by a Multi-Disciplinary Team (MDT), a bit like a ‘Tumor Board’.  I had already been confirmed Grade 2 (via liver biopsy) and my CT scans were indicating widespread disease.  I was yet to have an Octreotide scan and the conventional biochemical markers (CgA and 5HIAA).  I suspect, faced with my question, he went for the worst case, based on the statistics he had access to at the time. What I now know is that, in the year of my diagnosis, the median survival was 33 months in patients with advanced Grade 1/Grade 2 NETs with distant metastasis.  These statistics are certainly better today but my Oncologist was probably on the right track.  However, at no time did he use the word ‘terminal’.

The Cancer story is changing

What I also found during my research is that as more and more people in the UK are now living with cancer (all cancer) rather than dying from it, there is a new class of patients emerging – Macmillan UK call this “treatable but not curable” and I believe this is very relevant to Neuroendocrine Cancer.  I touched on this in an awareness blog entitled “Living with Neuroendocrine Cancer – it takes guts“.  You will find some data in this blog about a major increase in the amount of people with cancer who eventually die of something else (…… basically it has doubled). For many, Cancer is no longer a death sentence.  I do accept that it can be difficult to live with certain cancers and this is also covered in my “it takes guts” blog linked above.

Survivorship and Hope

You can find numerous examples of long-term survivors of advanced Neuroendocrine Tumours on the ‘airwaves’, many with a relatively good quality of life (QoL).  I don’t normally pay much attention to prognostic data, I take my lead from the huge number of patients living a long time with Neuroendocrine Cancer.  However, I was particularly interested to read a set of USA statistics from NOLA (Boudreaux, Woltering et al) which said “Our survival of stage IV midgut NET patients that we performed surgical debulking on was published in the Journal of the American College of Surgeons in 2014. It showed our 5, 10 and 20-year survival rates were 87%, 77% & 41% respectively. It’s also worth noting the comparison with the 2004 SEER database analysis which listed the 5 & 10 year SEER survival at 54% and 30% respectively”.  Clearly, the NOLA figures are guidelines (and only for midgut) but they do seem to reflect my previous statement about seeking out positives rather than dwelling on the negatives.  The SEER 2012 figures are much better than the 2004 versions stating “Survival for all NETs has improved over time, especially for distant-stage gastrointestinal NETs and pancreatic NETs in particular, reflecting improvement in therapies.

Exciting times ahead

On the subject of therapy improvement, there has been a plethora of new treatments coming online and more entering and progressing through the approvals pipeline.  Check out my article entitled Exciting Times Ahead Also listen to a NET Expert along the same lines.  PRRT is making a real difference.

Summary

Following my diagnosis in 2010, I went on to receive really good treatment and it continues to this day with Lanreotide backed up by a rigorous surveillance regime (and this is backed up by my own advocacy!).  However, I have totally accepted the fact that I have metastatic Neuroendocrine Cancer and that it cannot be cured.  By the way, I intentionally used ‘metastatic‘ rather than Stage IV.  Mention of Stage IV can set off alarm bells and send the wrong message to the recipient. I don’t believe Stage IV has the same ‘red flag’ meaning for well-differentiated NETs as it does with more aggressive cancers of the same stage. Given what I know now, I would certainly challenge any doctor who told me I had a ‘terminal disease’ and at the same time told me I had a slow-growing well differentiated Neuroendocrine Cancer.

I now live with this disease (….and it’s consequences) and do not feel like I’m dying of it.  Moreover, I most certainly do not see myself as a ‘terminal’ cancer patient, particularly as I’ve now been living with it since 2010.

I like to focus on how I can live better with it.

Whilst we’re on this subject, please note Palliative Care is not just end of life / hospice care.  That’s another misunderstanding bordering on mythical status. Read more here.

being_there_front
Graphic courtesy of Ellie McDowell

 

Thanks for reading

Ronny

I’m also active on Facebook.  Like my page for even more news.  I’m also building up this site here: Ronny Allan

Disclaimer

My Diagnosis and Treatment History

Most Popular Posts

Sign up for my twitter newsletter

Read my Cure Magazine contributions

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

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