Gut Instincts

Three weeks ago today Daisy had her ileostomy surgery and as we all know, three weeks is a long time in our world……

The surgery itself went according to plan, the anaesthetist spent over 90minutes going through everything about Daisy, checking her recent echocardiograms and preparing a plan for her pain management post surgery. Daisy, like many children with Costello Syndrome is not great with anaesthetic and this is where Great Ormond Street Hospital comes into it’s own. She was in theatre for about 3 ½ hours but spent longer than usual in recovery mainly because she developed an allergic reaction to one of the anaesthetic drugs and her face and lips were swelling up. She came back to the ward on morphine and ketamine in a pump and stayed on this for over a week.

Immediately post surgery the stoma prolapsed which means that the intestine was pushing out further than it should. So having looked a pictures of stomas on the internet to ready myself my first sight of Daisy’s stoma was not quite what I anticipated, infact it looked like a six inch sausage of intestine hanging off her abdomen.

Another trip to theatre loomed but fortunately the prolapse corrected itself and now at least I know the difference between a prolapsed stoma and a normal one. I also had not anticipated that Daisy would have a large scar from her navel to her pubic bone where the surgeons had opened her up to take out a piece of her colon. This scar infected and we have had a three week battle getting it to heal, not a great task when you consider how close it is to a stoma which is pouring out bowel contents…Being immunosuppressed slows the healing time and this has meant that the infection in the surgical site has been very slow to heal.

After a week we were told the results of the biopsies that the surgeons had taken. Dr Lindley our Gastroenterologist is a leader in his field of motility disorders and neuropathic bowel disease, however he has never seen a disorder of the nerves like those in daisy’s colon. His words were that we should have done the ileostomy a year ago and it was the best thing that we could have done. It has been bittersweet having the proof that Daisy’s colon is not formed properly and has never worked, she was born this way and all along Andy and I have said that we did not feel that she would grow out of her gut problems, our instincts have been proven right. It also confirms that she has been experiencing severe pain in her gut and we are so grateful to be under the Symptom Care team, working with them to manage Daisy’s pain. The problem now is that the ileostomy is permanent, the hope that by resting the colon it would get better and the ileostomy could be reversed has gone. Daisy’s colon will never work, we still don’t know whether it is part of her Costello syndrome that has never been seen in another child with the syndrome or whether it is something she was going to have regardless of her syndrome, the important thing now is that the ileostomy has to work – and this is now our biggest battle.

Normally an ileostomy output (of poo basically!) should be around half a litre – at the moment Daisy’s ileostomy is pouring out double and some times three times this amount and it is just watery in content (sorry but Andy and I are now becoming experts on stoma bag contents and this is our daily conversation, we’re immune to the grossness of it!!!). Daisy is still on 24 hours a day TPN and we have started a tiny bit of milk – the equivalent of 1 tsp an hour – this just pours out of the stoma looking the same as when it went in!

The doctors initially started treatment for bacterial overgrowth and have found some bacteria in her small bowel (the only bit that is left and working) that would normally only be found in her colon, however they are still scratching their heads as to why Daisy is losing so much fluid, their strategy is to wait and see and hope. Losing more fluid than goes in also makes her dehydrated very quickly and lose essential electrolytes so she has to have daily intravenous fluid top ups ontop of her TPN. Add into this the intravenous pain medication she needs daily and that means that there are four pumps she is attached to at any one time.

Because her stoma losses are so watery they were leaking from the stoma bag and reinfecting the infected surgical scar, so the wonderful stoma nurses have come up with a solution involving a urostomy bag (normally used for draining wee) hooked up to a urinary catheter – so ontop of the pumps she has a big catheter bag we have to manage. This means we are pretty much confined to her cubicle all day and all of us are slowly going slightly mad…..

Of course it is the school holidays now. Thankfully we anticipated a long hospital stay so some plans are in place for the children, most of which involve Andy or I picking up or dropping off a child on a weekend. While Daisy’s stoma refuses to work properly or absorb any feed then we are here pretty much for the long term.  We also need to be here at the moment to get her pain management regimen fine tuned – now she is on maximum gabapentin for her age, daily IV paracetomol and ketamine when she needs it. We have not seen the hoped for improvement in Daisy’s pain at night, infact if anything she needs more medication than before but at least we have a co-ordinated plan driven by our symptom care (palliative team) from both this hospital and Chase Hospice.

Family life has still gone on over the past three weeks. Xanthe said goodbye to St John Fisher School, the second of my children to progress to high school.  I vividly remember the day I took her to nursery, all pigtails and smiles and now she is growing up into a beautiful girl, with her fair share of pre-teen hormones!  She amazed us in her school production of A Midsummer Night's Dream where she wowed the audience with her performance as Bottom, clearly she has the Nimmo acting genes.  The children are all visibly more relaxed now school has finished and the holidays have begun, even if once again they are spent in different locations it is nice for them not to have the added pressure of early mornings and homeword deadlines to contend with.  I hope I will get a chance before the end of summer to do the dreaded new school shoes shopping run and get the last bits of high school uniform for Xanthe before she starts the next chapter of her life as a St Philomena's Girl.

Daisy had a brilliant report from Linden Lodge and will be progressing to Year 1 when (emphasis on when) she returns in September, she also had a great report for her piano playing, and has suprised us all with her musical ability!

So we muddle through again, and make the best of things, I am so grateful that at the outset of our journey with Daisy Andy and I had a strong marriage as we have been tested time and time over, our children are growing into lovely, intelligent , self sufficient young people and at the tender age of 7 even Jules can made a cup of tea for his mummy!
We will just have to wait and go at Daisy's pace and hope the ileostomy begins to behave and function as it should. In some children, without the complications Daisy has a small bowel transplant can be an option to get a child off TPN however, while this has not 100% been ruled out it is highly unlikely that she will be accepted for transplant or do well with the surgery so TPN and an ileostomy is our only option. With gastro children there are often no magic fixes, what works with one child does not work with another – the mantra is always the same, one day at a time, we just have to wait and see if this ileostomy decides to work and hope it makes the difference we are looking for….
Well it turned out that a weekend in Glastonbury was just what I needed to recharge my batteries and prepare for Daisy's impending surgery.  I had spent the best part of a year trying not to look forward to it incase it didn't happen so couldn't actually believe it when I did get there.  There is certainly something magical about that part of Somerset and it worked it's spell on me, I left my watch at home, switched my mobile off and moved to Glastonbury time.  Those in the know, know that Glasto is more than just the headline bands and my best memories are just of chilling listening to some amazing music and eating some lovely food (and partaking in one or two somerset ciders!).  We were blessed with the weather and the extra money I paid to camp in a location with decent loos and showers reaped dividends.  All in all it was a wonderful opportunity to just pull myself together again, relax and unwind.  I got to spend some precious mum and daughter time with Xanthe too which was a real bonus.  I just can't wait to go to Glasto again next year, it's cast a spell on me... (and I can't wait to thank the nurse on Rainforest ward who told me over a year ago that I should apply for tickets as I would love it).

So since Glastonbury my time has been filled getting ready for Daisy's impending ileostomy surgery...sorting out a complex childcare spreadsheet for the summer has been the main challenge.  It is not easy making sure that children of different ages and different genders all get to have a fun time while their parents are either in hospital or working.  I have evolved my plans on the assumption that nothing will go according to plan and Daisy will do another long term stay, it does seem to involve Andy or I spending a lot of time driving down motorways dropping various offspring at various relatives/holiday camps at different times in the summer  - just hope they appreciate how much planning has gone into them having fun!

I have also been getting everything ready for Xanthe's big transition to High School.  When Theo went up to his high school little did we know that a few weeks in Daisy would go into hospital and not come out again nearly 12 months later.  This time I want to be better prepared as it really impacted him.  Now instead of after school clubs we have a lovely after school carer who comes to the house and can turn her hand to looking after Daisy or one of the other children if needed. I have also made sure that Xanthe's new school is aware of the situation so that if things get stressful at home they are aware of it.  Having a child like Daisy has a ripple effect on the entire family, we plod along then suddenly something happens like a tube being pulled out, a line infection, a UTI and lives get turned upside down,  We all become used to this rollercoaster but over the years I have realised that we have to put some safety nets in place and not assume that we will all be able to ride the storm.

I have a realisation today also that when you have a child with a complex medical disability you become disabled as a parent/carer - or differently abled.  I have to create a new persona and take away a bit of me in order to operate in this world of disability.  I am now Daisy's mum, or just "mum" as the doctors and nurses call me (not all of them, but enough for it to jar).  I have to be nice to people in order to get things for my daughter, I have to deal with people who I don't necessarily like, I have to be diplomatic and swallow my tongue, I have to know my place.  The other day our community nurse dropped around some medicine syringes for Daisy, it was only that evening when I was drawing them all up I discovered that one of the syringes she had dropped off was the wrong type to connect to Daisy's jej tube.  I was planning to visit a friend who was in our local hospital with her daughter that day so drove up and asked a nurse if I could have some syringes - two were produced together with a telling off that I shouldn't get them from the ward but should be getting them from the community nurses.  It was only afterwards when I mulled over this I realised the situation I was in, here I was a highly educated woman who had once had a successful career, now trained to care for an TPN dependent, medically complex child, having to beg for syringes and being told off for asking the wrong person....This is the situation many of my hospital friends find themselves in - we are expected to do so much for our child but we are so isolated - I am friends with the nurses on a superficial level but I am not a nurse, I can talk to the doctors about Daisy, but I am not a doctor, I can work with social workers, occupational therapists, speech therapists and all the multitude of people who seem to have a say in my child's life but the power is with them.  I am dis-abled because I have had to become a different person in order to get my child what she needs.  So next time, please give me the sodding syringes, in fact give me ones that fit her tube so that when at the end of the long day I am drawing up one of the 20 odd meds she needs during the day I have the equipment at hand I need to do the job I need to do.....

So now as I type it is the night before the hospital admission. Tomorrow Daisy goes back onto Rainforest.  I phoned twice today to check the bed is available and so far so good although I have not packed at this point (I am an expert hospital packer now so not too worried about doing it at short notice). Tomorrow we should be talking to the surgeons, anaesthetist and pain team in anticipation of the surgery on Friday.  Daisy's pain med which had lulled us into a false sense of security a week ago has stopped working so Andy and I know that this is the only route to go down to hopefullly alleviate the night time pain Daisy experiences.  Our wonderful palliative care team at Chase have visited and there is a plan post op so now the only thing left is for us to get to the hospital and for Daisy to have her surgery.  I hope and pray it will make a difference, I am realistic enough to know that things will probably get worse but maybe we will turn the corner over time and not regret our decision....