Patients told they have an irritable bowel when it’s really a rare cancer
The symptoms Sarah Smith experienced on and off for nearly ten years were, she says, the kind of ‘everyday complaints’ most women suffer from time to time. ‘I had tummy upsets, acid indigestion, hot flushes and diarrhoea,’ she says. ‘I’d go to my GP each time something new came up — and each time, there’d be another explanation.’
Sarah, a teaching assistant, had a family history of irritable bowel syndrome (IBS), so the stomach upsets, which began in 2002 when she was 36, were easily explained.
and, as her mother had been through an early menopause at 43, Sarah put the occasional hot flush down to hormonal changes.
But in 2011, her GP thought her issue might be due to gallstones and referred her for a scan.
Finally, Sarah learned the truth: she did not have gallstones, but numerous large tumours on her liver. For almost a decade, she’d been living with an undiagnosed rare neuroendocrine cancer, which had caused her symptoms.
neuroendocrine tumours ( net s) grow predominantly in the digestive or respiratory tract, but can occur in any part of the body.
apple founder Steve Jobs died from an net on his pancreas, while the BBC’s political editor, nick robinson, was diagnosed in 2015 with an net in his lungs.
the tumours secrete large amounts of hormones, which cause symptoms such as cramps, flushing , diarrhoea, wheezing , skin problems and abdominal pain, depending on where they are.
Yet, according to the net Patient Foundation, doctors have so little clinical experience of rare cancers such as nets that patients often wait years for a diagnosis.
‘When the tumour is finally identified, it is often misdiagnosed as one of the more common cancers,’ says neil Pearce, a consultant hepatobiliary and pancreatic surgeon at university hospital Southampton.
‘then the wrong prognosis is given and incorrect treatment commenced. a well-known example is musician W ilko Johnson [of the band Dr Feelgood], who was initially diagnosed with pancreatic cancer when it was, in fact, a slow-growing neuroendocrine tumour of the pancreas. he was given weeks to live and told it was inoperable, when neither was correct.
‘there are probably several thousand patients in the uK with neuroendocrine tumours who’ve been given the wrong diagnosis, the wrong prognosis and the wrong treatment.
‘Patients are commonly misdiagnosed with benign conditions such as IBS or the menopause, and given treatments that have no effect.’
While diagnosis is often delayed, the prognosis for nets is generally good (though this depends on their location and how aggressive they are).
however, delayed diagnosis with other lesscommon cancers can have a devastating effect on mortality rates, with the 12-month survival rates for pancreatic cancer as low as 9 per cent and, for liver cancer, 12 per cent.
Indeed, while death rates for more common cancers are dropping, those for less -common cancers — defined as those outside the big four of lung , breast, bowel and prostate — have risen to a new high and are continuing to rise, according to a report from Public health england and the charity Cancer52, an alli - ance of 84 cancer support charities.
‘Because the symptoms of rarer cancers are not as well- known, many patients are diagnosed at a late stage,’ explains Jane Lyons, Cancer52’s chief executive.
these rarer cancers include brain, bladder , kidney and genital cancers, as well as cancers of the mouth, larynx, thyroid and some bones, which together made up nearly half of the 280,000 newly- diagnosed cancers in england in 2013. nor do the problems end with diagnosis. ‘If you are managed by a doctor who has never seen a case like yours before, then it is very unlikely that you will be getting access to the best level of care and support or the latest treatments,’ says Mr Pearce, who specialises in the management of a number of rare cancers.
CatherIne BouvIer, the head of the net P atient Foundation, adds that these cancers often require highly specialised treatments.
‘neuroendocrine tumours are a complex group of cancers, which often do not respond to chemo or radiotherapy,’ she says. ‘the cells in each neuroendocrine tumour behave differently, so they need individuallytailored treatment plans.’
Furthermore, the rarity of these less common cancers leads to a lack of research and trials.
‘out of £500 million spent every year on cancer research, most goes into work involving the “big four”,’ says Mr Pearce. ‘Just over £90million is spread across hundreds of rarer cancers.’
In 2015, the Cancer Drugs F und cut funding for the drug Lutathera, which has been used successfully in the treatment of net tumours for 20 years.
‘Lesser-known cancers are simply at the bottom of the pile for funding ,’ adds Catherine Bouvier.
Sarah’s symptoms began in 2002 while on holiday with her husband Mark, a Bt engineer , and their daughter Daisy, then two. She had excruciating backache and assumed she’d pulled a muscle.
‘But then, I started to get terrible indigestion,’ recalls Sarah, who lives in orpington, Kent. her GP prescribed medication for acid reflux.
When, soon afterwards, Sarah developed diarrhoea, her doctor did some blood tests and they all came back clear. three years later , Sarah developed mastitis. She was given antibiotics and referred to a breast clinic, but was told there was nothing to worry about.
then, in 2008, six years after her symptoms began, Sarah suffered a massive allergic reaction to a friend’s dog — her skin erupted in a rash and her body swelled. her GP referred her for tests, but nothing out of the ordinary showed up.
Sarah was, by now , also having constant hot flushes, even though she was still under 40. and her blood sugar could drop suddenly , leaving her weak and dizzy.
She recalls: ‘I’d start slurring my words and my movements would become uncoordinated. then I’d eat a packet of P olos and be fine again.’ this time, her GP diagnosed hypo - glycaemia. three years later , Sarah woke up one day at 4 am in agony and experiencing waves of heat so intense that she crawled out onto the paving slabs in her garden to try to cool down. an ambulance took her to hospital.
‘they gave me Buscopan, an anti - spasmodic medicine, and the pain immediately went. I felt as though I’d wasted everyone’s time. My GP said it sounded like a classic case of gall - stones — and I thought: “F antastic”, because it was an answer!’
But instead of an enlarged gall bladder, an ultrasound showed ‘shadows’ on her liver. Within 24 hours, S arah h ad a n appointment at King’s College hospital in London, which has a specialist liver centre.
her blood tests were normal apart from one, which a less specialised hospital might not have run. It measured the level of a hormone called gastrin, which stimulates the release of gastric acid and is secreted in large quantities by nets.
Sarah’S result was sky-high. her stomach was producing massive quantities of acid, which was causing her symptoms. She was given a full body scan and asked to return.
‘When I walked in and saw a nurse as well as the consultant, I knew it was cancer and my legs buckled. all I could think was: “I have a ten-year-old daughter. this can’t be happening.” ’
two months later, in a ugust 2011, Sarah underwent ten hours of surgery to remove 1.5 kg of tissue, including one- sixth of her liver , her appendix and gall bladder . But doctors have been unable to find the primary site of the cancer, and so it keeps returning.
In 2013, new growths were found on her liver and in her small intestine. She had surgery to remove one-third of her digestive tract. Last year, more tumours were found and she’s receiving injections of octreotide, a drug that helps block the production of hormones from the tumours.
‘It’s bad enough having cancer, but having a rare cancer no one has heard of is horribly isolating,’ says Sarah.
‘the worst thing is not having a prognosis because no one knows when, or if, my cancer will kill me.’