Cancer patient has her appendix, large bowel, gall bladder, spleen, womb, ovaries, fallopian tubes, cervix and most of her small bowel removed in 'mother of all surgeries' to treat 'one in a million' cancer misdiagnosed as IBS
By Kelly Strange for MailOnline
15 December 2014 http://www.dailymail.co.uk
Pam Pope, 65, from Surrey had nine organs removed by surgeons trying to save her life when they diagnosed a very rare form of cancer
A cancer patient underwent a 13-hour operation to remove all the organs she could live without as surgeons battled to remove an aggressive and rare form of cancer from invading her body.
The morning after the procedure, dubbed the 'mother of all surgeries', Pam Pope's surgeon stood by her bedside and listed all the organs his surgical team removed in order to save her life.
By the time he reached the ninth, the 65-year-old had burst into tears.
The gruelling operation carried out at The Hampshire Clinic in Basingstoke, aimed to remove all cancerous tissue in the body.
In Ms Pope's case her appendix, large bowel, most of her small bowel, gall bladder, spleen, womb, ovaries, fallopian tubes and cervix were removed, as well as most of her stomach lining and navel.
'I just wasn't expecting to be told so much of my body had gone,' she said. 'The list of organs that had been removed was so long that I actually lost count.
'I just could not fathom how I could still be alive when so much of me was missing.'
The operation, carried out by a team of six leading UK surgeons, is known in medical circles as the 'mother of all surgeries', with good reason.
It was followed by a ‘chemo bath’, where the abdominal cavity is filled with warm chemotherapy liquid - normally for 90 minutes, while the patient is still under anaesthetic.
It is one of the most invasive operations performed and the only way to treat a rare cancer thought to originate in the appendix - pseudomyxoma peritonei (PMP).
The disease quickly spreads around the abdominal cavity meaning diagnosis usually comes with a death sentence.
Now the mammoth operation to remove as many organs as possible while keeping patients alive means sufferers like Ms Pope, who would previously have been considered too sick to treat, are being offered a lifeline.
'I was already stage four when they operated because the cancer had gone undetected for so long,' she said. 'I would not be here now had it been left for a few more months.'
Though Ms Pope suspects her cancer may have developed earlier, she had been complaining of symptoms for 15 months before her final diagnosis.
'I just did not feel right,' she said. 'I had stomach pain and nausea.'
After several visits to her GP, the insurance executive from Camberwell, Surrey, was diagnosed with Irritable Bowel Syndrome (IBS).
'I felt that was not accurate,' she said. 'I had always been very fit and very healthy with a good diet but my GP did not want to consider anything else.'
Ms Pope returned to see her GP several more times over the course of the next year, each time requesting a referral to hospital which was denied.
It was only after she reported a change in bowel habits to a different doctor that alarm bells finally rang.
Doctors initially diagnosed Ms Pope with IBS, but not convinced, she sought a
second opinion. Further tests revealed the 65-year-old was suffering
pseudomyxoma peritonei (PMP) - a cancer which is thought to spread from cancer
of the appendix, causing tumours to grow on other abdominal organs
'The new GP agreed my symptoms warranted further investigation, which was a huge relief because by then I was in no doubt that it was not IBS,' she said.
In December 2012 a laparoscopy - where a fibre optic instrument is inserted through the abdominal wall to look at organs - quickly identified that abnormal cells were present.
She said she felt 'strangely relieved' to hear this news having spent so long insisting she did not have IBS.
VERY RARE CANCER AFFECTING ONE IN 500,000 PEOPLE WORLDWIDE
Pseudomyxoma peritonei (PMP) is a rare cancer, affecting around one in 500,000 people a year.
It is characterised by the presence of mucin-producing tumours in the abdominal cavity.
There are a number of possible origins of PMP, but the most common source is appendix cancer.
There is no known underlying cause of PMP but there are a number of theories around its origin.
Cells from a tumour in the appendix are thought to leak into the abdominal cavity and implant on other abdominal organs.
The tumours then replicate and produce a mucinous byproduct that then spreads throughout the abdomen.
Many patients lack clearly defined symptoms, making the disease difficult to spot until the later stages.
The most common symptoms are:
Source: PMP Research Foundation
It was like 'being proved right', she added.
But when her consultant told her she was the one in a million patient suffering with rare PMP, like many people, she had never heard of the cancer.
He explained the disease had started in her appendix but may have spread around the abdominal cavity in mucus leaked from the organ.
The microscopic cells can then attach themselves to other organs and tissue the mucus comes into contact with.
An operation to 'debulk' the body of cancerous cells, she was told, was her only hope of survival.
The surgeons had no idea how far spread the cancer was and how many organs they needed to remove until they operated.
Ms Pope was hopeful when she was wheeled into theatre in May last year that surgery would be minimal because she had not suffered the weight loss or thickening of the waist common with advanced PMP.
Just 24 hours later she was told surgeons had removed her appendix, large bowel, most of the small bowel, gall bladder, spleen, womb, ovaries, fallopian tubes, cervix as well as most of her stomach lining and navel.
'I cried because I could not believe I was still alive,' she said.
'I had thought I might lose one or two organs but not all that. I realised then why it was called the "mother of all surgeries" - everything that I could live without had gone to give me the best chance of survival.
'It was a shock but the surgeon was very supportive in offering to arrange counselling,' she said.
The radical operation had been followed by a radical procedure called a chemotherapy bath.
This is where the cavity was filled with hot chemotherapy liquid in an attempt to wash away any remaining cancer cells.
Ms Pope said: 'Despite everything that they had taken I was told five per cent still remained on my small bowel.
Six surgeons removed Ms Pope's appendix, large bowel, most of her small bowel, gall bladder, spleen, womb, ovaries, fallopian tubes, cervix as well as most of her stomach lining and navel
'It was too dangerous to remove it all so they left as little as they could which needed conventional chemotherapy and radiotherapy treatment.'
Ms Pope spent the best part of the following year in hospital recovering and undergoing more chemotherapy.
DRASTIC 42°C CHEMO BATHS KILL CANCER CELLS IN ABDOMEN
Chemotherapy baths, officially called Hyperthermic Intraperitoneal Chemotherapy, involve filling the abdominal cavity with warm chemo liquid, normally for 90 minutes.
The patient remains under anaesthetic.
Bathing the inside of the stomach with heated chemotherapy increases the chances of the cancer cells being killed.
Heat at 42°C kills cancer cells without affecting normal, healthy cells.
If the drug was given in general circulation, as with normal chemotherapy, the temperature would heat the body up and the heart would stop.
But the abdominal organs act as a barrier, absorbing the drug.
It can therefore reach a high concentration in the area where the tumours are, without reaching the blood stream and damaging tissues in the rest of the body.
The treatment was approved by the government's health watchdog, the National Institute for Health and Clinical Excellence (Nice), in February 2010.
The biggest hurdle was adjusting to the loss of so many organs. At one stage she looked unlikely to pull through and was advised 'enjoy the time you have left'.
Determined not to give up, the 65-year-old contacted cancer professor Justin Stebbing at the London Clinic of Oncology.
She said: 'I had read about his work and my surgeon agreed to refer me.
'He was a leading voice on cancers like mine and probably about the only person who wouldn't turn me away.'
Professor Stebbing found a regime Ms Pope's body could cope with.
A scan earlier this year revealed no signs of any cancer, but hydration is still an issue and Ms Pope relies on a drip every night, which she uses at home.
She struggles with tiredness and remains frail, though her health has improved enough for her to return to work part time.
'I love my job so that was a major achievement for me,' she said.
Fortnightly chemotherapy will, she hopes, keep the cancer at bay.
'This has been a fight but I need to tell my story to make people aware of this very sneaky cancer,' she added.
'I was told this disease would likely kill me but I'm still here thanks to the 'mother of all surgeries, which has lifted a death sentence.