A Gastro-Intestinal Stromal Tumour is rare, but you are not alone!

GARRY'S GIST JOURNEY

Garry's story illustrates how very important it is to have regular follow-up CT scans. after surgery. If a surgeon says to you, "Don't worry - I have got it all out and cured you", insist on seeing an oncologist who sees lots of GIST patients. One can't help wondering how Garry's story would have been different if that had been done.

Sadly Garry lost his fight against GIST and died on 9th December 2008. Before he died Garry had expressed his gratitude to GIST Support UK for the the support and friendship he had received.

Judith

Phase 1. Discovery, surgery and remission

GIST hit me as a major surprise. It was September 14th 2001 (just 3 days after the momentous events of 9/11 and 10 days before my 46th birthday). I had an early start to work as I was driving with two colleagues from Leicester to a research meeting with our collaborators at the Veterinary Laboratories Agency in Weybridge (Surrey). I am a Professor of Biology at Leicester University and, amongst other things, we have been working for the last few years on the development of sensitive live animal tests for scrapie (in sheep) and BSE (in cattle). Anyway, the meeting was underway and we were discussing new data over a cup of coffee. Suddenly I felt very nauseous and this quickly developed into a severe abdominal pain. The local first aiders decided there was something serious going on and so I was helped into a car and driven to the A&E department of the nearest hospital (Chertsey). I recall spending several very uncomfortable hours lying on a trolley and undergoing some X-rays (very painful to get into the correct position). I gave my car keys to my concerned colleagues who had stayed with me throughout this period. I was given fluids and eventually morphine. The morphine helped and I was taken to one of the wards and fell asleep. I was woken later that night at about 11.00 pm to be told that the plan was to perform a laparotomy to check for diverticulitis (the best guess of the doctors).

When I woke from the surgery it was two days later and I was in a different hospital! I opened my eyes to see my wife, Lindy, who explained that I was in Intensive Care at the West Middlesex hospital. The laparotomy had revealed that my descending colon had ruptured due to the presence of a tumour about the size of a grapefruit. This had caused faecal peritonitis with a real risk of septicaemia. I was receiving antibiotics via a Hickman line in the neck. The surgeon at Chertsey had performed a left hemicolectomy and hence a colostomy (Hartmann's procedure). He was apparently not optimistic about my chances and so Lindy had been told to expect the worst. Lindy stayed with me for two days, before returning home to take care of the children. Whilst in Intensive Care I was extremely well cared for with one-to-one nursing. I remember the physioterrorists (sorry, physiotherapists) coming to get me out of bed and go for a walk! I managed this and after another couple of days was transferred to a regular surgical ward. I received a visit from the stoma nurse who told me a little about bowel cancer (which is what she thought we were dealing with) and taught me stoma care. The physiotherapists also made their regular visits and I was soon able to go good distances, once disconnected from many of the tubes in me. It was a couple of days before I saw the Consultant surgeon. I asked about being transferred to Leicester. His view was that this would be unlikely as I would need to find a surgeon in Leicester who would take me onto his/her list. I figured that this would not be a problem because the Dean of my Faculty at the University is also Dean of the Leicester Medical School and the three main hospitals in Leicester are all teaching hospitals. The problem I had was getting access to a working telephone! The patients' telephone on the ward had been vandalised and the nurses informed me that they could not bring me a telephone. Lindy was back in Leicestershire, so I was rather isolated. Then, there in the ward was a long standing friend, Terry, who was now working at the University of East London. He had heard about my plight on the academic grapevine and popped in for a visit. I gave him a complex message to give to Lindy and he went off to deliver the message. A couple of days later, a Saturday morning, I was stretchered into the back of a transport ambulance and driven to Glenfield Hospital in Leicester. Waiting to meet me was Adam Scott, the consultant surgeon who had added me to his list. We chatted and got along really well. I was now more able to relax since I was only a few miles from home. It was great to see Lindy and the children again.

Over the next few days my strength improved and I was visited by Sue, the stoma nurse at Leicester, who gave me excellent instruction in stoma care. I spent my birthday on the ward. One of the junior doctors brought me a copy of the pathologist's report that had been sent up from Chertsey. I read with great interest. The tumour was fully resected with good clear margins. The tumour cells were positive for the cell marker CD117 (also known as c-KIT). This would be something to follow up when I could next get on line.

A few days later I was sent home, but my freedom from hospital was short-lived. I suffered a pulmonary embolism (PE), presumed to be the result of a peri-operative DVT, and was re-admitted. The PE was extremely painful but things settled after a few days and I began a few months of warfarin treatment. Over the next few months I received CT scans, an MRI scan and a PET scan at Guy's Hospital. I also had an appointment with an oncologist at Leicester Royal Infirmary. By the time of that appointment I had done my homework and was well up to speed on GIST and its treatment. I had read the research papers on the cell biology of GIST and the role of Receptor Tyrosine Kinases (c-KIT and PDGFR?) and was aware of the development and use of STI571 (imatinib mesylate or Glivec). The oncologist explained that the resection looked clean and was the front line treatment for primary GIST. There was no evidence of metastasis from any of the scans. We talked about imatinib and he explained that it was not yet licensed in the UK and would not be prescribed for adjuvant therapy anyway. I was given another oncology appointment in one year's time. A few months later I returned for planned surgery - to have the colostomy reversed. This was all pretty straightforward and I recovered quickly. Within a few weeks I was back at work and back to playing golf.

Several months passed and it was back to the oncology clinic. A different doctor saw me and I had a very brief examination. I asked about the next scan and was informed that there was no need. My next appointment was in a year's time. By now my life was essentially back to normal. My next oncology appointment followed the same pattern as the previous one; a brief exam, no need for scans and another appointment for next year.

 

Phase 2. Recurrence and Glivec.

This pattern continued until the end of 2005 (over 4 years since the tumour was discovered). At that oncology clinic it was decided that an ultrasound scan would be a good idea. I received an appointment for the end of January, with a follow-up at oncology for the following July. However, over the Christmas period I began to experience abdominal pain. My GP suggested a scan - which I was already waiting for. By the time of the scan the abdominal pain was getting worse and it was obvious from the sonographer's demeanour that something was amiss. I received word that I would need a CT scan. This happened in the next few days followed by a hastily-arranged oncology clinic. It was a recurrence at multiple sites throughout the peritoneum! With the benefit of hindsight, I should have been more insistent about CT scans.

I asked to see the head of oncology, a colleague at the University - Prof Will Steward, whose research interests included sarcoma. He prescribed Glivec at 400 mg per day. I began taking the drug that day. I experienced a wide range of side effects to Glivec therapy (including stomatitis, rashes, nausea, diarrhoea, sensitivity to the cold and headaches). Within a few weeks my abdomen began to enlarge and it was speculated that this could indicate a response to Glivec. Unfortunately, my abdomen continued to enlarge enormously and after a few weeks I suffered a complete blockage of the bowel (such that I was vomiting faeces!). This was clearly a serious situation and I was admitted to the oncology ward at Leicester Royal Infirmary. The surgeons there were not keen to operate, despite the severity of my condition. Fortunately, Adam Scott said he would be prepared to have a go at de-bulking some of the tumour masses and so I was transferred to Glenfield Hospital. The surgery (April 2006) lasted eleven hours and I was in bad shape. It turned out that the tumours had responded to Glivec with their centres becoming jelly-like, hence the enlargement and abdominal swelling etc. I recovered from the surgical ordeal and was soon back at home, then eventually back to work and back to golf. At this stage I was put on a regime of CT scans every three months or so.

Phase 3. Glivec-resistance, more surgery and a trial drug.

All went reasonably well until early 2007 when a routine scan revealed tumour growth and new lesions. I was prescribed an increased dose of Glivec (800 mg per day), but there seemed to be little or no response and the tumours continued to grow rapidly causing another complete blockage of the bowel. After much debate, Adam Scott agreed to have another go at de-bulking the tumours. This surgery (March 2007) was extremely difficult because of the highly vascularised nature of the tumours, and was spread over two days. I woke on the third day in ITU. This was an especially difficult time for Lindy as she was given very little information about my condition or the prognosis. However, I did eventually recover from the surgery and the very next scan confirmed that Glivec was not working. In June 2007 the registrar in oncology told me that Glivec was to be stopped and I was offered only palliative care. The registrar was not aware of any other options or trials, so I contacted Will Steward about Sutent and some of the trials that were recruiting. At that time my PCT were not funding Sutent at all, so I asked Will if he would refer me to Ian Judson and his team at the Marsden. Will was happy to do this. At this stage I was quite poorly, I had lost a huge amount of weight (ca. 70 lb!) and was severely anorexic and experiencing early satiety because of two peri-gastric tumours (i.e. around the stomach). Adam Scott was contemplating inserting a feeding tube when I received word from the Marsden. I met with Ian Judson and we talked about research and about a trial of AZD2171, a multi-receptor tyrosine kinase inhibitor from Astra Zeneca with an activity profile similar to sunitinib (Sutent). I agreed to go on the trial, subject to a pre-assessment. This was fine and the trial began at 45 mg of AZD each day. The trial protocol meant very frequent visits to the Marsden (both the Fulham Road site and the Sutton site) and a regime of biochemical tests as well as PET/CT scans. My GIST was genotyped; I am wild type at both c-KIT and PDGFR? (so a rare form of GIST). The AZD side effects that I suffered are very much as described by some of those taking Sutent (persistent diarrhoea, fatigue and myalgia). The most troublesome was hypertension, which required me to take a range of drugs to control blood pressure. Over time I developed hypothyroidism (another side-effect) and had a few breaks from the drug and eventually a reduced dose (30 mg). I began the trial in September 2007 and for about six months the disease was stable.

 

Phase 4. The trial drug fails and Sutent starts.

Then in March 2008 a CT scan showed tumour growth and new lesions (including liver lesions). So, AZD had failed and I was signed off the trial. The Marsden team were great, very open and helpful and they often called me at home to check how I was doing. I particularly appreciated the role played by the research nurses Manuel and Louisa and the consultant Michelle Scurr (who had been mentored by Will Steward in her early career).

For a couple of months I received only palliative medication. We applied to the PCT for funding for sunitinib (Sutent). The essence of our application is that this is an exceptional case, and would not establish a precedent. Ian Judson also explored the possibility of obtaining Nilotinib from Novartis on compassionate grounds, but we subsequently learned that Nilotinib would not be an option due to changes in compassionate provision arrangements. At the end of May, and over two months since treatment ceased, I heard that I would receive Sutent for three cycles followed by an assessment of clinical benefit. I began taking Sutent on June 4th 2008. Compared with AZD2171 the side effects of Sutent were initially somewhat less severe (including, significant fatigue and loss of appetite) but my blood results were not great and I suffered from internal (most probably tumour) bleeding.

Towards the end of the first 28 day Sutent cycle I developed massive pulmonary emboli (PE) and some associated myocardial damage on the right side of the heart. There was very little warning, just some breathlessness prior to intense lung pain and a real difficulty with breathing. I was admitted into hospital the next day and a CT-PA (CT Pulmonary Angiogram) confirmed the PE and identified the heart damage. I began treatment with low molecular heparin (daltaparin) and was allowed home the following day. Things began to settle, but then I suffered from three "cardiac events" in quick succession and was admitted to Loros, the local hospice. After some adjustments to my medication and a week or so in Loros I was allowed home. I have been extremely weakened by these events and now need a wheelchair (as well as many adjustments to the home). I subsequently needed a second spell in Loros (10 days) to sort out more breathing issues. PE is reported to be a serious, but rare, side effect of Sutent, so I have been waiting to see whether I will continue Sutent treatment. At the time of writing I have been off Sutent for several weeks and my blood results and general feeling of wellness have improved significantly. A CT scan showed that despite being off medications, most of the tumours, especially the larger ones, are fairly stable. Together, with my consultant we have decided to wait 3 months, and have another CT scan before deciding whether to continue with Sutent. I am very concerned about further PE and the possibility of further heart damage.

It is now seven years since I collapsed with a perforated colon and underwent an emergency hemicolectomy that led to diagnosis of GIST. I am still off Sutent and managing reasonably well. I am fatigued almost all of the time with very heavy legs and I get breathless very easily, presumably the after effects of the PE and heart damage. I still need to use a wheelchair to get about and have needed two blood transfusions in the last four weeks. Each of these helps with my breathing, but the effects are not very long lasting. I need significant help with everyday tasks and so I am extremely grateful to my and family who are very effective carers.

Garry Whitelam

 

GSUK's Chair Nic Puntis discusses the BBC's report on the Cancer Drugs Fund on BBC Wiltshire Radio - NEWS>>>.  

Michael Sayers...news

Update on GIST clinical trials that are recruiting and in the pipeline....

Forthcoming Patient Meetings

  • Friday 6th October in Cardiff
  • Thursday 7 December in Edinburgh
  • Friday 9th March 2018 in London
  • Friday 5th October 2018 in Manchester

More information will appear on this website in due course.

We have launched our first GIST patient survey and hope to gather a better understanding of the landscape for GIST patient treatment in the UK. The survey closes on 10th September 2016 so if you would like to participate please complete the survey by clicking this link

'ALT' GIST Trial to open in the UK soon! We have been alerted to a new trial that will be opening in the UK in the near future for metastatic GIST patients. Read more on our Trials & Research page 

PAWS-GIST Clinic 7 - Newsletter June 2016

GSUK attends Cancer52 parliamentary reception to raise awareness of rare and less common cancers.

GSUK Research Update 2016 - Read all about it here

NHS England's Sarcoma Service Specification consultation is open until Weds 20th April 2016.       Please register your views by responding directly via this link.                             GSUK's response can be viewed here

Cycle for GSUK! We have four allocated charity places on the RideLondon event for this year and also for 2017 and 2018. Go to our News page to find out more!

The Masitinib Trial is now open.  For further details of this trial, and to find out which centres and clinicians are involved, please go to our Research & Trials page. 

Regorafenib has remained on the NHS England Cancer Drug Fund List for GIST patients! Read more!

Can you help Professor Eamonn Maher with his research Study? If you have had, or have, two or more primary GISTs, or a GIST and another tumour, find out more at the bottom of our research page

PRESS RELEASE - UKTV announces GIST Support UK as its Charity of the Year 2015

TUMOUR BANK UPDATE: "We have been granted approval from the NHS ethics committee for our National GIST Tissue Bank which will store these valuable GIST tumour samples for research. If you are about to have an operation and would like to donate your tissue for research please email  with your contact details. Visit our Retention of tissue page for regular updates.

Scottish Sarcoma Network: Interested in attending their Sarcoma education days or finding out about the services on offer ? Read our News page

Use of human tissue
Do go to our new "Retention of Tissue" page and please sign a consent form!

GSUK funds research!
GSUK provides £20,000 from donated funds to help start a research project at the Royal Marsden. Visit our News page for further information and picture.

Funds being raised for UK PAWS GIST clinic
See our News Page for more information.

NICE approves improved treatment for ascites
The PleurX system allows the patient to drain fluid from the abdominal cavity without having to pay repeated visits to hospital. More information on our News Page.

European Commission approves three years' adjuvant use of Glivec
Visit our News page for more details.

Masitinib
A press release in February claiming the effectiveness of this drug as a replacement for Sunitinib appeared to be premature at the time. However subsequent news may be more encouraging! See our News page for more details.