Tracy’s Jelly Belly Tracy was suffering from a lingering cough and abdominal bloating in November 2000. It turns out her abdomen is filling with gelatinous goop (mucin) in a condition called "jelly belly" (pseudomyxoma peritonei syndrome) which is a rare tumour (1 per 1,000,000 per year, so about 60 cases per year in the UK) of the peritoneal tissue. This was probably caused by an appendix tumour (appendiceal mucinous adenoma) that was mistakenly diagnosed as appendicitis while Tracy and her mother were on holiday in Ireland, back in summer 1998, where it was removed via keyhole surgery. Details can be found on the web. Have a look at http://www.surgicaloncology.com/default.htm if you’re interested in the knitty gritty, or search for "pseudomyxoma peritonei". The cough was deceptive. It was the pressure of the mucin pushing up against her lungs that caused it and not chest infection, whooping cough, etc. as was originally thought. This unfortunately delayed diagnosis. She was operated on Good Friday 2001 and relieved of 12 litres of mucin, a football sized cyst and the right ovary that it was sat on. The removal of such a large quantity of fluid caused a huge drop in blood pressure causing her heart to stop for 1:30 minutes and finally restarted by a defibrillator. After 2 days in intensive care she was stable enough to bring round and then on Easter Sunday night could go back to a normal ward. The left ovary, which had a small cyst on it, uterus and other stuff would need removing in due course. We were referred to North Hampshire Hospital (Basingstoke) as it specialises in the treatment of this condition (pseudomyxoma peritonei syndrome) in this country. Miss Fynn, the consultant in Good Hope Hospital, Sutton Coldfield, was not committing herself and rather letting the specialists at Basingstoke interpret her condition and recommend the best course of action. Tracy had her consultancy appointment on Wednesday the 25th July 2001. Met the consultant, Mr Brendan Moran. Nice, slow and methodical kind of guy. Her condition is almost exactly as we thought. Got to look at the CT scans and we could clearly see the PMP masses. Mr. Moran described it as a text book case, so we were looking forward to a text book solution. The operation (laparotomy) was done on the 25 September 2001. Well, Tracy went in at 10:30am on the 23rd for 2 days of tests but the operation was actually done on the 25th starting at 7:30. It finished 14 hours later at 9:30 She had another 12 litres of mucin and a rugby ball sized cyst initially removed. They then went on to remove the ovary, uterus (total abdominal hysterectomy), omentum (omentectomy – internal support system for the organs), gall bladder, spleen (splenectomy), pelvic peritonectomy (strip the lining of the abdomen by the pelvis). There was no need to remove any section of bowel, of which we are all very pleased. She was in intensive care for 4 days recovering in a highly monitored environment and getting intra-abdominal chemotherapy (5-florourcil) and was then scheduled to be in the ward a further 28 days (big operation – big recovery). The intra-abdominal chemotherapy after the operation is to zap any little bits that got away. Tracy went back to the ward on Saturday 29/9/2001 following completion of her final chemotherapy and recovering very well. The first few days she was a bit woozy but was back to her old self in no time. Tracy had an epidural, which is excellent, for a week and then some morphine like drug until the major pain is gone. She continued to recover well despite catching MRSA, the hospital bug, in the epidural site. We only found out recently how bad this bug potentially is but Tracy survived it OK. She came out of hospital 3 weeks to the minute from when she went in. The huge scar healed well and she has got no open wounds. Some of the internal stitches worked their way to the surface and escaped, but no real problems with infection or anything like that. Tracy is scheduled for a follow up scan in about a years time. 4/11/2002 – Tracy has her 1 year check up scan. Tracy is a nightmare when it comes to injections. Her veins collapse at the site of a needle and they always struggle with injections. They were unable to administer the iodine to show up the organs better, but otherwise she came back all clear. 20/1/2003 – Tracy has started coughing again so getting her checked out again. As per usual the doctor prescribes antibiotics a few times, to no avail. 24/2/2003 – Tracy has had her scan at Good Hope Hospital. The results were sent directly to Mr. Moran rather than Miss Fynn. Tracy is coughing whenever she bends over or changes position. She can’t lye on her back at all. She is also starting to look a little bigger round the waist - maybe. 8/5/2003 – The consultation with Mr. Moran about the results. There was a little PMP located behind the liver but looked inactive (at present) to Mr Moran. He isn’t highlighting it as an area of concern at the moment anyway. There was some fluid in the right lung. The symptoms seemed more severe than the amount of fluid would normally cause/indicate. Mr. Moran would refer us to an ENT and chest specialist to check out the lung problem. Mr. Moran suggests it is possible the lack of omentum (which normally supports the internal organs) can cause the stomach to sag down, pulling on the oesophagus and therefore the tight seal is relaxed allowing the entry of stomach acids into the lungs. We all decided that Miss Fynn would be best to arrange the ENT and chest specialists locally at Good Hope, rather than go through the loop with the local doctor (Dr. Wall). 12/6/2003 - Tracy had a meeting with Dr. Wall. Miss Fynn referred the request for ENT and chest specialists back to Dr. Wall, so yet another delay. Dr. Wall had a letter from Mr Moran requesting a new scan. We don’t know why. 2/7/2003 - Tracy got fresh scan. Due to her poor vein structure the senior radiologist was brought in. She was very nice and helped Tracy out as she can’t remain on her back for very long without coughing. The radiologist noted a great deal of fluid in the right lung and said that if she were an in-patient, she would have been placed on oxygen, it was so bad. She has scheduled an emergency appointment with Miss Fynn for the next day. About time she got some speedy care. 3/7/2003 – It appears from the scan that the right lung has been infected with PMP cells and is more aggressive this time. There are also some in the pelvic region, liver and maybe round the stomach areas (unsure about this bit). Miss Fynn has spoken to Mr. Moran and he would be unable to do more than remove the lung as he does not specialise in this area. Therefore, Tracy’s case is being handled by Good Hope Hospital in Sutton. Next Monday (7/7/2003) there will be a meeting about her condition and we should know more by the second meeting the following Monday (14/7/2003). They only meet on Mondays. There is a possibility that the Royal Marsdon Hospital in London may be recommended to handle the chemotherapy. Tracy will obviously have to go through full Chemo this time and probably more radical surgery. I’m afraid details are sketchy at the moment. I’ll let you know more as we know it. Apart from the cough and some abdominal pain, Tracy is OK and about as usual. 14/7/2003 – Sent e-mail with questions to Mr. Cecil (Mr. Moran’s No. 1) about the implications of PMP in the lung – Reply received 19/7/2003 the points of note are : The CEA and CA19-9 markers are elevated. The scan done on 24 February 2003 at Good Hope was reported by our radiologist, Dr Hilary O'Neill, saying there was a little bit of fluid in the chest with some mucin in the abdomen. There does appear to be some pseudomyxoma in the lung. In answer to your question about the lung adhesion molecules, pseudomyxoma is a rare disease and we do not understand it in its entirety. It certainly does not have a propensity to metastasise like carcinoma in which cells can easily bypass the defence mechanisms of the body. In time however pseudomyxoma can certainly turn more aggressive although this happens at a much slower rate than with carcinoma. On the CT scan that we have from February there appears to be some disease in the right lung and over the right side of the liver. We have limited experience of re-operation on people with pseudomyxoma. This would be a technically very difficult area to approach from the abdomen. The feeling from reading the notes is that any further surgery would be very risky and really would probably only be sensible to try and improve symptoms. 16/7/2003 – Spoke to Ms. Fynn’s secretary requesting information as we’ve heard nothing as yet. She’s passed the request to Jane Grove (oncology nurse that assists Ms. Fynn on cases). Waiting to hear. – The hospital rang Tracy to inform her of a 14:30 appointment on 21/7/2003 with Dr. Tim Fletcher at Good Hope. 21/7/2003 - Meeting with Dr. Tim Fletcher of Good Hope hospital in Sutton Coldfield who is co-ordinating the treatment of Tracy's chest. He explained that, from the scans, Tracy has accumulated fluid between the pleura and the lung. For those of you unfamiliar with the anatomy of the lung; the pleura is like a sac that surrounds the lungs. It includes the diaphragm and other muscles. The lungs themselves do not have muscles and do not expand on their own account. The diaphragm and pleura are normally stuck directly onto the lungs. It is they that do the expanding and contracting, pulling the lungs (stuck to them) open and closed. Getting back to Tracy - she has gained some PMP cells somewhere in there and they are producing mucin, which is filling the space between the pleura and right lung. As she breaths in, the pleura and diaphragm is pulling down and apart as normal, but the right lung is no longer stuck to it so fails to inflate properly. Tracy is having a needle put in (thoracentisis) on Wednesday (23/7/2003) in an attempt to draw a small amount off to check the fluid really is mucin rather than anything else. We also have an appointment on Friday (25/7/2003) with the surgeon from Heartland’s Hospital. He will be going through the procedure to remove the mucin from between the pleura and lung. From what we understand, either via keyhole or a small 3 inch incision (more likely due to the thick nature of the mucin), the gel will be physically drawn out and the area between the lung and pleura cleaned and, bizarrely, dusted with talc to aid re-adhesion of the pleura/lung for full re-expansion of the lung. It is thought that this will happen in 2-3 weeks time. It should be a relatively minor operation (compared to the last one) with either 1 or 2 days in hospital. No section of lung will be removed and there is no sign of the PMP cells directly invading the parenchyma (lung itself). There is no evidence of any invasion of the left lung. This operation is naturally only symptom relief, and not a cure. Tracy will need to undergo chemotherapy in an attempt to kill the mucin producing PMP cells. It is thought that 5FU will be used again, but this time it will be intravenously rather than intra-abdominally applied. We think the chemo will be performed by the Royal Marsden, but have yet to have this confirmed. 5-FU is used, as it is the proven chemo drug for these appendix-derived cancers. We'll no doubt be having a chat with a specialist chemo person in the near future. Dr. Fletcher thinks there has never been a case in this country of PMP cells migrating from the abdomen cavities to the lung cavities. From our chat with Gabriella (founder of PMPPALS) there was one case in the states. This obviously means that Tracy has a rare complication on a rare type of cancer and the treatment is therefore on a trial and error basis. The doctors are working together to come up with solutions that can be applied to Tracy in the hope that, together, they can quell this progressive disease. It could be a long road. 23/7/2003 – Tracy had the thoracentisis and no fluid could be drawn up the needle indicating it is likely to be mucin. This is because it is too thick to traverse the needle. 25/7/2003 – Tracy, Ross and I attended a meeting with Mr. JFK Marzouk (01214242561). A very nice man. He announced his burning the midnight oil searching for information on a similar case that he had helped his tutor with, while in training back in the 80’s. He found his old diaries and had phoned his tutor. His tutor has worked on just 3 cases like this throughout his entire career and is one of the countries leading chest surgeon/specialists. Both indicated the same approach of removing the mucin build up and otherwise, leaving it well alone. He let slip he personally new Sugarbaker in the states, so we asked if he could contact him for any advice; which he will do. The scans clearly showed a mass of stuff on the one side and a nice clear lung on the other. The lung is fully collapsed and probably has been for several months. The surgery will involve cutting a 6 inch whole about 6 inches below the arm pit. The ribs will be stretched out the way (so may give a bruised rib type of pain later) and then the mucin can be accessed and removed. The lung has not been collapsed for too long, so he is expecting it to re-inflate without too much trouble. There could be a day or so in the HDU, but recovery should be fairly quick; about 6-8 weeks before she can lift things, etc. It will be performed in Heartland’s Hospital in Birmingham. The operation will occur in about 3-4 weeks time. The operation is repeatable, so if it reoccurs, it’s no problem redoing the procedure. Apart from problems sleeping, some pain and the obligatory cough, Tracy is fine and going about as normal. 12/8/2003 – Tracy has received an appointment for the operation on the 19/8/2003, provided a bed is available. She is to call Heartland’s Hospital on Monday 18/8/2003 to secure a bed and check in that evening. Pre op checks will be done 14/8/2003. The chemotherapy will not be performed until after recovery from the operation. It will be performed at Good Hope Hospital, Birmingham. The regime will be developed by all those concerned, utilising consultants from the Royal Marsden, based on pathology from the operation. 14/8/2003 – The operation is postponed for a few weeks, as Tracy’s blood pressure is very high 180/128. She is taking tablets to reduce this. No new date has been supplied. 19/10/2003 – Tracy went into hospital for a few days bed rest prior to her operation on Wednesday 22/10/2003. 22/10/2003 - Tracy went for her operation at 13:45 and was in the recovery room by 16:30. The operation went very well. Mr. Marzouk said that he managed to remove 95% of the tumourous material (PMP cells) and 2 pints of mucin. The lung inflated without any problems. The 5% left behind was in too dangerous an area to be removed (inferomedial recess containing phrenic nerve, inferior vena cava & oesophagus). Tracy spent 2.5 days in the High Dependency Unit (HDU) before returning to Ward 4 on Saturday morning. Her blood pressure was too low (ironically) for several days following the operation, but came back gradually. The drains came out Monday and Tracy left the hospital Tuesday at 13:30 ahead of schedule. There was some talk of chemotherapy via the drain to wash the lung area in an attempt to kill any remaining tumourous cells. Dr. Thompson, the senior oncologist, attempted to contact Brendon Moran at Basingstoke on Friday and succeeded on Monday 20/10/2003. He advised that it would not be successful and could be both painful and dangerous and was therefore abandoned. I had a lengthy chat with Dr. Thompson who advised contacting Dr. Tim Fletcher as soon as we exit the hospital to start the ball running as far as the Chemo goes. She mentioned that Dr. Ali from Good Hope Hospital has been in contact with the Royal Marsden, so need to find out what they recommend. Dr. Thompson also mentioned that the Royal Marsden were conducting COX II inhibitor trials that may help with this type of tumour; so may be worth pursuing. Dr. Thompson advised we get an oncologist from Good Hope on board ASAP to help with the co-ordination of Tracy’s treatment. Tracy is recovering well and is initially up in Sutton Coldfield where her mother can take care of her. x/12/2003 – Tracy has started coughing again. The doctor at the local surgery suspects a lung infection so has prescribed antibiotics. We’re worried. The antibiotics do not seem to be helping so a letter has been sent by us, directly, to Dr. Tim Fletcher and Mr. Marzouk notifying them of her coughing, the actions of the local doctor and our concerns over the lack of follow-up consultation(s)/examination(s). Subsequently a endoscopic examination has been scheduled for 23/1/2004. 13/1/2004 – Tracy saw her local doctor about small vein thrombosis that may have been caused by our New years flight to the Caribbean. He also checked the lung and noticed the lung has some fluid/non-expansion present. He’s asked Tracy to go for an X-ray at Good Hope tomorrow to aid diagnosis. 23/1/2004 – Tracy was asked by Mr. Marzouk to come for a bronchoscopy at Heartland’s Hospital. Tracy’s lung appeared fine, but Mr. Marzouk suspected the coughing was caused by the diaphragm being raised on the right hand side. Presumably this is pushing against the lung and causing irritation and therefore coughing. He requested an x-ray to check the lung (as he didn’t have immediate access to the one taken 10 days earlier at Good Hope hospital) and has requested a CT scan to further confirm his findings. That may well be a month or more away, but there appears to be no immediate risk and it fits nicely with his post-operative check-up. 22/1/2004 – CT Scan was a debacle. The Staff were completely uncaring and processed Tracy like a piece of meat. The radiologist didn’t even introduce themselves. Tracy was brought in early before the liquid was completely drunk and the nurse said it didn’t matter. There was no further liquid used. 12/3/2004 – Visited Mr. Marzouk to find out the results of the CT scan and ask him loads of questions. No sign of the CT scan itself. Good hope must still have it. Mr. Marzouk will chase it up, but doesn’t expect it to tell us anything new. The meeting confirmed what we new all along, but was a bit scary when they put the x-ray up. The diaphragm on the right hand side is raised about half way up the lung reducing capacity. The diaphragm and pericardium are part paralysed due to the operation having to cut through the nerves making them work. The remaining tumour (5%) is in too dangerous a location for removal, as it can lead to catastrophic bleeding. Tracy will have a permanent cough, though it’s not too bad. In the meantime, Tracy’s abdomen is starting to look bloated so we’ll have to start pursuing that again. 16/4/2004 – Tracy’s cough is very bad. She suspects a chest infection, but the stomach is looking so bloated I think it may equally be due to the internal abdomen pressure pushing the liver and attached diaphragm even further into the lung, and causing the cough. A week or two of antibiotics should reveal the truth of the matter. Mr Moran in Basingstoke is waiting on the senior radiologist (Dr. Hilary O’Neil) to come off Easter holiday before commenting on the CT scan taken in Good Hope. They requested it after we contacted them informing them of Tracy’s increased girth and pains in the abdomen. Tracy is barely eating and sleeps < 4 hours per night due to the persistent and breath sapping cough. It’s a full hack with whoop. Tracy complains of not being hungry at all. 23/4/2004 – After an angry letter to Dr. Tim Fletcher, we suddenly had an appointment with Dr. Chris Poole, oncologist at City Hospital in Birmingham. This meeting was originally mentioned in Dr. Fletches letter of 9/2/2004, so needed the prompt. Chris started off by informing us that he usually side-steps patients with PMP. He’s treated 2 and they started off OK but deteriorated eventually though at least one is still alive. We viewed the CT scan taken 18 Feb. (don’t know why the dates do not match with her appointment date) and could plainly see the liver pushed up into the right lung (raised right hemi-diaphragm) and pseudomyxoma/mucin everywhere. Much of the right lung is paralysed due to damage of the phrenic nerve, reducing lung capacity. The CT scan failed to show contrast in the lower bowl due to the problems mentioned on the 22/1/2004. Dr. Poole pointed out that the radical debulking Tracy underwent in Basingstoke was very much experimental and no satisfactory evidence exists of it doing any good. He attended a presentation by Sugarbaker and was sceptical. In Dr. Poole’s opinion the most beneficial operation performed was on Tracy’s lung. With PMP all that can be done is removal of mucin and overt tumour periodically. Repeat mucin evacuation operations eventually destroy the stomach wall causing numerous hernias under the pressure of subsequent mucin build-up. Bowl/stomach involvement and blockages are common. Eventually operation intervals drop from one every 18 months to one every 6 months or less. We went though some drug regimes that can be tried. COX-2 inhibitors can’t be used as Tracy is allergic to NSAIDs like aspirin & ibuprofen. The remaining drugs Tracy can try (Taxol, Xeloda, Carboplatin) can have severe side effects dependent on the individual. In essence Dr. Poole recommended we wait till Tracy can no longer stomach the repeated operations and then give it a try. He has recommended some methamphetamine linctus to help with the cough. 24/4/2004 – We received a letter from Mr. Moran of the PMP centre in North Hampshire hospital stating “There is really nothing that would be easy or sensible to do from a surgical point of view on Tracy ….. I think it best to try and treat her with medical drugs at this point in time”. Impasse. I think that this does not mean that Tracy cannot undergo the mucin evacuation operations, but that there would be nothing the specialist centre could do over and above any other hospital to make her more comfortable. I will be writing to them for clarification and letting then know about our disappointment and feelings of abandonment, when they should be providing us with specialists care and support. Highlights from the pathology report from Dr. Hilary O’Neil, based on the 18 Feb. scan. Pelvic detail not included Mucin surrounds the peritoneal surfaces of liver extending into porta-hepatis Stomach has moved into left upper quadrant where spleen used to be Stomach encased in thick rind of mucin Encasement of both small bowl and it’s mesentery Mild variability in small bowl diameter Raised right hemi-diaphragm indicative of phrenic palsy Localised thickening along antero-lateral margin of right lung extending into the right oblique fissure Comparison with CT scan 24/11/03 shows moderate disease progression and significant increase in volume of peritoneal disease. The steroids Tracy is taking help with the severe coughing she has had of late. Despite all this, Tracy is up and about as normal, and in good spirits.