For some this can be a perfect storm

Frequently, a correct diagnosis is just the beginning of a patient’s trauma.  A late-stage diagnosis can still save a person’s life, but it can also present new challenges that will need specific attention to maintain quality of life.  All these accumulative events and circumstances can create huge uncertainty, often with no signs of hope or a way out for the patient.  Some of these events and circumstances will be sequential, but for many, they may appear simultaneously and within a relatively short period of time. For some, it can be like a “Perfect Storm” and even those who find themselves in the seemingly tranquil ‘eye’ of the storm, may still feel trapped and uncertain of what lies ahead.  These ‘storm’ effects comprise:

• Diagnostic Difficulties – People often assume the symptoms they are experiencing are things they can treat themselves with over the counter drugs before finally seeking professional help. This could go on for some time, it’s a pretty deceiving disease. After several visits, and despite your concerns, your doctors may diagnose you with something else. In extreme cases they could become so frustrated that nothing serious is obvious, they might even start to think it’s all in your head. It’s even possible that some people will be referred to secondary care only be referred back to primary care still undiagnosed. The result of these delays is a diagnosis at a later stage and for some an additional diagnosis of an associated ‘syndrome’ in addition to cancer.  Fortunately, this doesn’t happen all the time, thanks to improvements in awareness and the work of organisations such as Neuroendocrine Cancer UK and Specialist organisations.
• The Tumour Effect – Like many cancers, it can grow in your body without you knowing, without you having any reasons to seek intervention by healthcare professionals.  It finds places to hide mainly the small intestine, appendix, lungs, stomach, pancreas, rectum and a host of other less common places.  Once it has established in the primary location(s), it will try to break out via your blood and lymphatic systems, as cancer does. In indolent cases, it may not do this very fast but as it can be quiet, it will do this over several years. This cancer wants to establish other bases in your liver, your lymph nodes, your bones and any other place it can get to. If a person is lucky, their disease may be discovered incidentally while being investigated for something else, sometimes this is at an early stage but often it happens at a later stage.  If a person is unlucky, the silence will continue until the tumours announce their presence via symptoms caused by growth, often in an emergency at a hospital and often leading to diagnosis at the later stages of cancer.
• The Syndrome Effect – As if that wasn’t bad enough, some of the sub-types of this cancer is known to be able to oversecrete hormones and peptides which can mimic routine illnesses and conditions including (but not limited to) Irritable Bowel Syndrome (IBS), asthma, general abdominal upset, diarrhoea, diabetes, rosacea, menopause and heart disease.  Depending on the type of tumour, these present as common symptoms, some of which include flushing, diarrhoea, generally feeling weak and fatigued, pain, agitation, anxiety, dizziness, nausea, acid reflux, skin irritation, anaemic, weight loss/gain, low blood sugar, high blood sugar, heart palpitations, headaches, sweating, high blood pressure. So even when it becomes ‘noisy‘ and you seek professional help, the disease is so complex that it can often fool the host and the physicians into believing these are just routine illnesses needing routine approaches. Meanwhile, the tumours continue to grow and spread, often reaching an incurable state.  There are several associated syndromes with Neuroendocrine Cancer, depending on the offending hormone(s) and in some cases the primary location(s). Many people focus on serotonin as the culprit hormone but there are other oversecreting hormones that are specifically associated with one of the Neuroendocrine Cancer syndromes and certain organs in the body, including (but not limited to) Insulin, Glucagon, Gastrin, Vasoactive Intestinal Peptide (VIP), Pancreatic Polypeptide (PP), Somatostatin, Adrenaline and Noradrenaline, Cortisol, Calcitonin, Adrenocorticotropic hormone (ATCH), Parathyroid hormone (PTH) and many more. The disease is also associated with hereditary syndromes which predispose a person to neuroendocrine tumours, these include 4 types of Multiple Endocrine Neoplasia (MEN), and familial versions of Medullary Thyroid Carcinoma, Pheochromocytoma and Paraganglioma.  Other less common hereditary connections include Von Hippel-Lindau syndrome, neurofibromatosis type 1 and tuberous sclerosis complex.
• Post diagnosis discoveries – Even at diagnosis, things can suddenly get worse as specialist scanning, blood/urine testing and biopsies are conducted to discover the grade and stage of the patient’s disease.  Further metastasis can be discovered via nuclear scans and for some, the primary location remains undiscovered. The most common syndrome associated with Neuroendocrine Cancer is currently known as ‘carcinoid syndrome’, predominantly caused by the over secretion of the hormone serotonin.  Those who remained unaware of their disease or were diagnosed late, could now potentially find two further issues caused by high levels of this hormone.  Firstly, a phenomenon known as desmoplasia which in the extreme results in a ‘fibrous mess’ in the mesentery and less commonly in the retroperitoneum.  These issues were potentially involved in pre-diagnosis pain and discomfort in the abdominal area, at worst causing obstructions and damage to major organs and blood vessels, which may have led to the diagnosis.  Secondly, a similar scenario involving serotonin can introduce fibrosis to the right side of your heart causing even more life-threatening issues to contend with on top of cancer. Other syndrome types are also capable of introducing significant disease morbidity.
• Post diagnosis side effects and long-term consequences – Diagnosis and initial treatment over, the patient’s and physician’s tasks are not over.  A late-stage diagnosis means the disease is essentially incurable. While most will be ‘incurable but treatable‘, this does introduce additional challenges to the mix. Many of those in the lower stages and lower grades may need ongoing support and surveillance for a considerable period. Moreover, the side effects of surgery and other therapy types have the potential to introduce new treatment-induced side effects for many including (but not limited to); malabsorption, vitamin and mineral deficiencies, diarrhoea, diabetes, thyroid issues and many more. It may feel like the patient never recovers from the diagnosis with the patient unable to work out what a new normal is supposed to look like.
• Coping – The sheer amount of testing and surveillance required, the uncertainly about the prognostics, the consequences of the therapy, the worry about the impact on work, finances, family, (including any genetic worries) all have the potential to lead to anxiety and more serious mental illness issues in the long term, all add to this ‘Perfect Storm‘. Quite often these peripheral issues go unnoticed, untreated and essentially ignored. Fortunately, organisations such as Neuroendocrine Cancer UK are trying to ensure these issues are brought ‘front and centre’ via their ‘Mind the Gap’ campaign. However, it’s true to say we are only seeing the tip of the iceberg, there’s much more to do in this area.