So we are nearing the end of March and still our battles with Daisy go on. This year we have already racked up 5 hospital stays, and the fact that we can now do things like TPN and bloods at home have kept her out of hospital the rest of the time.

We are facing some big decisions and seeing Daisy deteriorating in many ways so our mantra continues to be "will it improve her quality of life".....

The ongoing issue remains infections, she is chronically unwell a lot of the time with low grade fevers and "pyrexia of unknown origin" - we know that the taurolock line locks we now use in her hickman line are helping protect the line from infection but the large doses of immunosuppressants she is on mean that she picks up every bug going and ontop of that bugs move from her not very well functioning gut into her blood stream causing her problems. This has been the main cause of her recent hospital admissions as any bug also causes her tummy to go into "pseudo-obstruction" and shut down - this is happening more and more and I have had times where I have considered only buying yellow pyjamas and bedsheets for her to match the bile that pours out of her tummy during these times..

One of our admissions was slightly more dramatic than normal and for the first time ever I called 999 for an ambulance when I discovered Daisy soaked in blood pouring from her hickman line - to this day we still do not know why it started to bleed suddenly but may be something to do with her high blood pressure issues.

We have had two meetings with Surgeons since my last post - after a couple of cancellations because Daisy was too unwell to attend the appointment we met with her Orthopaedic surgeon and have at last got the ball rolling to get her feet sorted, this can't come soon enough as Daisy has now gone from being able to walk around with her little backpack carrying her pump to not being unable to walk unaided and mainly shuffling on her knees to get around the house. The tightening of her achilles tendons is so severe that her big toe joint is now at a right angle to the rest of her foot when upright. She cannot wear shoes or her foot orthotics and has considerable pain in her hips if she spends too long on her feet. Once she has the surgery to lengthen and stretch her achilles tendons, which will be in about two months time because of the waiting list, she will need to be in casts for quite a few weeks, hopefully once everything has healed she will then need to go into hospital for a two week intensive rehabilitation stay as her muscles in her legs have wasted and essentially she will need to re-learn how to walk again. I am so frustrated by this situation as I have been saying to physiotherapists for years that tight heel cords can come on very very quickly in children with Costello Syndrome but, if we ever actually saw a physio which was not often, I was accused of worrying about things before they even happened. We have now reached a point where what would have been simple surgery has now become quite complicated with no guarantee of a complete success....

The other surgeon we have met with was one of the Paediatric Surgery Consultants at GOS, our lovely gastro consultant referred us to him to discuss surgery to form an ileostomy. This is where the bowel is brought out through the abdomen to form a stoma, bypassing the large colon. The pellet study of Daisy's intestinal transit showed that the radio-opaque pellets she had to swallow took at least 3 days to reach her intenstines and then she took a total of 19 days to pass them all.... confirming, that she has very poor intestinal motility, particularly in the lower part of her colon. The severe pan-colitis she also has, has probably affected the nerves and in a nutshell her gut, which didn't work properly from birth, has now deteriorated massively. The hope is that by performing an ileostomy we create a smaller area for the gut to work and bypass the areas we know are the most diseased and dysfunctional - possibly to allow them to heal and one day work properly... This may reduce Daisy's dependency on TPN and maybe even allow her to be fed into her stomach instead of into her jejenum...These are all the positives, the negatives are that it is major surgery and there are no guarantees except for the fact that she will get worse before she gets better and we may after all of it end up having to reverse the stoma and reconnecting the bowel if it does not make any difference...

This is a huge decision for Andy and I, we know we have to try and give Daisy the best chance but it's all the unknowns....and, if I am completely honest, with the memory of our recent 12 month stint still fresh the thought of another long marathon stay in hospital fills me with dread. The surgeon has left it to us to make the decision while booking the bed and the theatre slot anyway so that we do not have to delay if we decide to go ahead. It is such a big decision for us because Daisy is just not straightforward - she is classed as being risky for anaesthetic because of previous problems and because of her heart condition, her endocrine issues mean her blood sugar mangement will be even more tricky, her gut does not tolerate change will definitely go into shut down for a while and then there is the risk to her hickman line as she will have a hickman, gastrostomy, jejenostomy and ileostomy which are all possible sources and sites for infection....

However both Andy and I know we have to make a decision soon, Daisy's condition has deteriorated massively over the past two years and is definitely not getting better. The amount of feed she receives into her jejenum is paltry, the equivalent of 3 teaspoons an hour and most of that is made up of sugar just to maintain her blood sugars while off the TPN. She is TPN dependent and this is not conducive to a long life, her pan-colitis is poorly controlled - at the moment she has a flare up which has slowed her gut down and is causing her significant pain. The immunosuppressant therapy she is on to dampen down her body's response to the inflammation is difficult to manage as her gut does not absorb the drugs well and she cannot take them orally as she risks aspirating them. I have to take bloods weekly to measure her cyclosporin levels - too high and she risks kidney damage, too low and she will have a flare up of the inflammation. In a year she has not had a consistent run of her levels being where they should be. For all these reasons and for many more I know we must give the ileostomy a try, if I could see into the future it would be so easy, but then life with a child with Costello Syndrome was never meant to be easy....

My gut feeling (pun intended!) is that we will ask for the surgery to be pushed back to late summer to allow her to recover from the orthopaedic surgery which is essential to ensure she does not lose the ability to walk and allow us some family time over the summer. In the meantime Daisy is due a planned week long stay in GOS for a whole range of tests including another endoscopy/colonoscopy - perhaps the results will help tip the balance on how quickly we go ahead with the surgery...

Life at home still goes on - Daisy now has transport to take her to school and this has freed up so much time in my day. However she has not managed a full week at school, so much time is taken up with illness or hospital appointments. When she is there however she is at her happiest. She gets so excited when the bus arrives to collect her and her cognitive development continues to amaze everyone who works with her. The plan now is to move to "sign supported english" as she is starting to vocalise words more and more but will need the signing to get her point and meaning across. We all sign naturally with her and never have any trouble understand what she is trying to communicate.

Andy is still building his new business and work is picking up more and more, Theo is totally involved in his life at high school and Xanthe is over the moon having been offered a place at her first choice High School. Jules is busy preparing for his holy communion and we are all looking forward to that date as it is yet another excuse for a Nimmo Summer Garden Party...we continue to make plans, and tweak and change them to fit in with Daisy...this has become our life, living with uncertainty has become normality, we have learned not to fight it but to go with the flow, expecting the worst but sometimes being pleasantly suprised. Maybe it's not a textbook strategy but for us, it works....

Every day my youngest daughter's capacity to overcome the odds that are stacked against her completely amaze me. Yesterday she had a dual sensory assesment at her school to look at the support she needs to take into account her visual and hearing impairments. In fact Daisy is multi-sensory impaired, all her senses are not wired like yours or mine so every day tasks take so much longer, she has to work twice, three times as hard as any other child just to do simple things like sit at a table and play with a toy. She has to remain balanced, focus her hearing and vision, explore using touch and taste and smell, all of these things she does with a system that does not function in the way it should. What we discovered, or rather confirmed that everyone in the room already knew, is that Daisy is a bright little cookie. There is so much going on in her brain, but she needs to be given the resources to help her use it. The biggest problem continues to be speech, which is limited, however, her communication (using signs) is coming on leaps and bounds - just because she does not speak it does not mean that she does not understand or need to tell you how she feels. One of the outcomes of yesterdays session was to really work on her signing, progressing hopefully to using British Sign Language. I hope that as she begins to use signing more and more this will equip her for later life and help her assert herself and get other people to understand that lack of speech does not mean that she does not have feelings and comments to make on what is happening to her.

Today Daisy wore her new bone conductor hearing aids to school - what a transformation, the teacher told me she has been so vocal, it's like a light bulb has gone on with her ability to hear more clearly...of course we are now waiting for the proper digital ones to arrive so that she can have even better sound quality...

Daisy started the new year in Rainforest Ward - just to make her mark that hospital life would continue, despite our wonderful birthday, Christmas, new year break....The stay was for a new hickman line, two repairs to her hickman line were too risky for infection so she was prioritised for a line change, removing one from the right jugular and placing a new one into the left jugular. Fortunately this was relatively straightforward inspite of the interventional radiologist (who has worked on Daisy before) commenting, yes I remember Daisy, she has very wiggly veins....don't you just love these doctores and their casual asides...

This year will see lots more hospital stays - the likelihood of an ileostomy to bypass Daisy's non-functioning colon is looking more and more strong. Tests that were conducted over Christmas seem to confirm this although we will get the official results when we meet with Daisy's main Gastroenterologist on Monday. This takes us into yet another new territory - another bit of plumbing to contend with and what essentially is very major surgery for a little girl with a lot going on. Our hope, if this is the route we go dow, is that this will put an end the awful pain that she is experiencing, mainly at night time.

This is not the only surgery she will have this year, we are still waiting for the date for the tendon releasing surgery - she now spends more time off her feet than on her feet and if this surgery is not done soon Daisy will be unable to walk. Following this surgery will be around 6 weeks in plaster casts - fun for all involved and then a two week intensive rehabilitation stay at GOS. The only good thing with all this surgery and hospital stays is that it is all planned, hopefully we will see less emergency admissions this year and instead have more planned stays focussing on improving Daisy's quality of life.

Merry Christmas and a Happy New Year

Well, after much cajoling by family and friends, I am back to blogging.... to be honest it has been difficult to fit in the time to write now that Daisy is at home, also, sometimes I feel like I am writing the same stuff over and over and maybe people don't want to read it but based on feedback when I stopped writing for a while it is obvious that a lot of you out there quite like my ramblings.


So here, instead of cards (do you think I have time to write Christmas cards???) - is my Christmas blog...........


Lots has happened (no suprises there) since my last post. two emergency hospital stays, lots of outpatient appointments, more surgery plans and continuing battles with bureaucracy. Lets start with the good news first however...


After the October half term our local education authority saw sense at last and agreed to fund a full time one to one support for Daisy so that she could attend school full time. The difference in Daisy has been amazing, she is more confident, vocal and her communication skills (using sign language) are truly fantastic. She ended the term being awarded the Principal's prize for excellent signing and also enjoyed playing an angel in her class assembly, sitting confidently and watching all the other children, joining in with all the songs and applause. School has been the making of Daisy and we are so grateful and happy that she has been able to attend this particular school, a specialist sensory school where the teachers are skilled in working with children with sensory impairments. The only downside is that although funding has now been approved for Daisy to be taken to school with the school transport service this has not yet happened (due to bureaucracy again) meaning Andy and I have to take her back and fore to school which takes up two hours of our day and a huge amount of juggling with the other children. I'm really hoping that this is resolved in the new year as the daily battles with other school run mums across Wimbledon are taking their toll and we are worried about the words Daisy is learning as we vent our frustrations on other mad drivers!!!



Another lovely thing that happened in November was Theo's confirmation. Some of you will know that I am a practising catholic and a confirmation is a really big rite of passage for a young person. It was such a lovely chance to be together as a family with the focus on one of the other children for a change. In catholicism you take a saints name at confirmation and Theo chose "John" which was my dad's name, particularly poignant as mum was his confirmation sponsor and dad's anniversary had been the week before . I know dad would have been so proud of all our children so it was so nice for Theo to honour his memory in this way...



Of course as always there have been the obligitary hospital visits both planned and emergency. We had a few trips to A&E in November as Daisy was experiencing really acute pain and screaming out, always in the back of our minds is the increased risk of malignancy that Costello Syndrome carries. It's so difficult taking Daisy to A&E now as staff change so frequently and it can be really frustrating going over and over things with new people. Theoretically she should have notes kept in A&E for what to do in an emergency - something that was agreed with the Great Ormond Street team on discharge - but we found on our visit there that the notes had not been updatede which made things very difficult. In addition it doesn't help that Daisy is so complex, some doctors can flounder a bit, particularly when faced with such knowledgeable parents. It took four visits before Daisy was admitted with a 40 degree temperature and quite poorly. As there were no cubicles at our local any more we had to be shipped to Epsom hospital which might as well have been Manchester as we had to start all over again with staff who did not know Daisy or us. They were however fantastic and Daisy was home after a week and then went to our wonderful hospice to recuperate...



A couple of weeks later her temperature shot up again, we always have to suspect line sepsis in this scenario and we had to rush again to A&E. This time she did get a cubicle at our local hospital so at least we were closer to home. This last stay in hospital highlighted some things to us which have changed so much since the days when Daisy was smaller and much, much less complicated (although we didn't think so at the time!!!). Daisy is the only child who normally is on the ward who is totally TPN dependent and a patient of Great Ormond Street. Part of the philosophy of the Home TPN programme at GOS is that in order to minimise infection risk only the parents should set up or take down the TPN or access the line for bloods. In theory this is fine but when you have a child like Daisy who never ever sleeps through the night and has lots of complications it puts a huge pressure on Andy and I, particularly when you add into the mix Andy trying to run and manage a business and three other children who are not allowed onto the ward because of swine flu precautions. We had agreed at a meeting at GOS that we would manage Daisy's TPN when in hospital but use a hospital pump provided by GOS, normally this works in every other hospital where their home TPN patients visit as the pump that was provided is a standard hospital one. Standard except in our local children's ward (even though it was in use in the rest of the hospital). So we had to cope with the scenario of nurses unwilling to use another hospital's pump, unwilling to put our TPN through their pump, sleep deprived parents who were having to stay awake for 18+ hours while also trying to juggle childcare and in Andy's case client work. Needless to say things were not good during Daisy's last hospital stay and while she recovered well we were disappointed that, without going into detail, we were seen as a problem. In five years we have got very close to the staff at our local hospital and I guess with the demands being put on us as parents of a medically fragile and complicated child our relationship with them has had to change as we have become more knowledgeable about Daisy's needs and she has become more complicated. Ultimately we would like to avoid any unplanned hospital stays and, now that Andy and I have been trained in lots of areas like giving TPN and IV medication hopefully emergency visits will be short and sweet.

However the planned visits are unavoidable and these all take place at Great Ormond Steet Hospital. Again since having time at home we have really come to appreciate how incredibly lucky we are to be able to have all of Daisy's specialist care at GOS. As she grows bigger other issues, more common in her syndrome, are starting to manifest. The one area which has crept up really quickly is Daisy's feet and general orthopaedic issues. Her achilles tendons are now so tight she walks in a permanent tiptoe and her leg splints are now causing her severe pain. She will have surgery in the new year to release her heel cords, followed by several weeks in plaster casts. After that is all over she will then be readmitted to hospital for extensive rehabilitation physiotherapy under the care of the rheumatology team as she has been found to have benign hypermobility syndrome complicated by fluctuating muscle tone (try saying that after a few drinks!) - the bottom line is that this causes her pain and puts things out of alignment which causes her even more pain and was probably responsible for the fluid on her hips during the summer.

We met with Daisy's Gastroenterologist a few weeks back and he now feels that the extreme inflammation she experienced with her inflammatory bowel disease has damaged her nerve ending in her gut which were not functioning properly, hence her dependency on TPN for her nutrition and her inability to manage more than 15mls/hour into her jejenum. Over Christmas we are faced with the fun task of trying to get Daisy to swallow four radio-marked pellets over four days to try and work out how slowly her gut moves - obviously if on x-ray the pellets show up in her lungs it will confirm that her swallow is still unsafe!!! We are then back in clinic in January to see if there are any further options for treatment, including, surgery, which may help her.

Despite everything, and even though she has kept us on our toes during the past few weeks Daisy managed to be well and stable for her birthday, for the first time ever she spent it out of hospital and hospice and we managed a short pre Christmas break to Center Parcs in Longleat. The Gods must have been smiling on us as considering we have not been further than our hospice in Guildford in the past 18 months Daisy was fit and strong and loved every minute of our time away, we avoided all the severe weather and the snow we had just made the forest seem even more magical than ever. I can hand on heart say it was the best birthday ever and whatever happens in 2010 the memories of happy family times in the run up to Christmas will sustain us all for a long long time.

Right - I need to get back to Christmas preparations, the presents are not going to wrap themselves. We have vigil mass in a couple of hours, Xanthe is playing an angel in the church nativity, Theo is altar serving and even Andy, the arch atheist, is coming to church (mainly to hear the choir sing a not-tongue in cheek version of Johnny Mathis' "When a Child is Born" - including the spoken bit in the middle....)

Hopefully this blog update will make up for the fact that I have not sent any Christmas cards, however to everyone who sent us cards and Daisy birthday cards, thank you so much - especially post pals who brighten our days!!!!

I'm going to post some pics later but in the meantime:

HAPPY CHRISTMAS AND A HEALTHY AND WEALTHY 2010


XXXXXXXX


useful links:

www.lindenlodge.wandsworth.sch.uk
www.postpals.co.uk
www.facebook.com/people/Stephanie-Nimmo/653854349
http://www.gosh.org/x-factor/the-charity-single/your-say/stephanie-nimmo/

Apologies again for not updating more frequently - I'm sure I'm breaking the European Working time directive rules on the number of hours I work, on an unpaid basis, nursing Daisy, let alone looking after the rest of the family....

Again, so far, so good - apart from the usual dramas our lovely daughter throws in our path. This month's rare occurence which befell Daisy was a 1cm hole in her hickman line. As you can imagine a hole in a line which goes straight into her heart is not desirable so as always we had panic stations.... I noticed the problem when flushing her line on a Friday morning (it's always a Friday!), there was a strong smell of TPN in her room and when I flushed the line I saw the hole. I now know that repairing a hickman line is not straightforward, initially, in my naivety, I thought it was a case of calling the community nurses to come and carry out the repair, obviously not. There are limited people who can carry out hickman line repairs, and no-one in our local NHS trust could do this. In fact, I have since discovered that in some areas a repair would not even be attempted, instead it would be a case of removing the line and inserting a new one. As you have guessed, it was time for another trip to Great Ormond Street and a three hour stint with their fantastic nurse practitioners who, after a couple of false starts, repaired the line....

Of course it shouldn't have happened in the first place, holes in hickman lines are rare, especially in such a new line, but rare and Daisy go together! And as always her timing was impeccable - not only was it a Friday, but Andy's parents were due to arrive from Scotland having not seen the children in a year and Theo was due to return home from his Ocean Youth trust trip. Major multi-tasking and, as always, wonderful help from Chase hospice, and everything was sorted in time.....

Apart from this drama, our month has been spent trying to get back to some semblance of normality as a family again. We have all spent a year living on our nerves and separated from eachother, and the scars run deep. While superficially it looks like the children have coped well, Andy and I have realised how difficult it has been for all of us. Theo had his first year of high school with minimal support from his parents, often coming home to an empty house and cooking a meal for himself. Jules and Xanthe went to after school club or to friends houses. Andy and I were like ships that passed in the night putting all our energy into the children. Now we are all just relishing being under the same roof and enjoying the simple things....Theo is loving having his parents there when he comes home from school, Xanthe likes our pampering evenings, Jules is able to have friends back home to play. Daisy remains the centre of attention, but in a positive way. Andy and I are happy to be back home, able to catch up on TV together and share a joke....

Life is still tough but at least we are all at home. Every day I spend an hour in the morning doing the TPN and meds and getting Daisy ready for the day, the same again at night....It is physical work that requires maximum concentration. Before Daisy's deterioration she was on 24 milk feed through a feed pump and around 13 different meds a day. Now the feed pump time is reduced to 10 hours but she then has 14 hours on TPN and still the same number of meds, just different ones...ontop of this is her physical problems, her splints, without which she can no longer walk, the pain at night, the yet to come health problems.

She now goes to school for four hours a day and the benefits show on a daily basis. She is becoming more and more vocal and cognitively is really bright. The school is going to start progressing her from using makaton to British Sign Language as her brain works quicker than her ability to acticulate herself. We are really hoping that in arming Daisy with the skill she will benefit in the long term as she can really get across her feelings. Of course this will mean that Andy and I will need to learn BSL - we are really excited about this prospect.

After half term Daisy should go to school full time - paperwork and bureaucracy permitting! The next battle will be to sort out transport to get her to school and back as most children in special schools are taken there in buses and taxis . Daisy will need an escort, so this will be put to yet another committee for funding approval.....This is what drags you down as a parent of a disabled, medically fragile child - you spend your days fighting to keep them alive, fighting for the best for them and it all boils down to paperwork, panels and case conferences, none of which,you, as a parent, have a say in....

I do sometimes wonder if Andy and I were not so articulate, educated and determined to cut through all the red tape which prevents our daughter getting what is her right, how she would do......

Anyway, as I said, so far, so good, we are staying away from hospital and doing the nursing stuff at home, life is better, we are all together ....

Well, so far, so good, I am pleased to report we are still out of hospital and only visiting as an outpatient. My workload has not eased however as now is the time to fight all the other battles that have been put on hold for a while. Examples of these included sorting out a proper bed for her which will enable me to do her IVs without breaking my back and which will keep Daisy safe when she thrashes around at night in pain. We have had a few false starts with our wonderful Primary Care Trust on this one and two, totally unsuitable, beds have been provided already. The third one will arrive hopefully in around 6 weeks so I am really hoping it is third time lucky. In the meantime we are improvising, my wonderful friend's wonderful carpenter husband has adapted a second hand cot bed with higher legs and a door which opens outwards - thank goodness for friends like this as I really do not know how I would have coped with an ordinary cot in the meantime.

Another battle is to get Daisy the support she needs in school. Prior to her massive increase in medical needs (when her needs were still very significant) Daisy had a one to one learning support assistant for 4 hours of the school day. We now have to demonstrate to the education authority why she needs a full time assistant - it's patently obvious, but unfortunately there are massive bureaucratic hoops to jump through to achieve this so in the meantime she is back at school, but only for 4 hours a day.

Daisy is loving being back at school. It is a specialist school for children with visual impairments in the most wonderful location between Wimbledon Common and the All England Lawn Tennis Club. The facilities and staff are outstanding and even in just a few days back in school Daisy is coming on in leaps and bounds. The staff are so happy to have her back, everyone seems to know her there and I just hope that she can have a really long run of time back at school so that she can continue progressing.

I have my usual battles with the medical issues, we now have a date for her rheumatology appointment, this is at the end of October. I am still trying to get a date and plan for her surgery on her feet. These are getting worse and worse, she can no longer stand up without wearing her foot splints and even with these on she is unsteady and falls easily. I am so concerned that this surgery needs to happen very soon as I don't want her to lose the ability to walk. We are waiting for a date to go back to GOS for the TPN/Nutrition Clinic. Despite pushing up the volume of TPN Daisy is receiving she is continuing to lose weight and I am trying not to worry too much but you can't help wondering if there is a more sinister reason behind why this is happening...

Anyway, the next couple of days are an opportunity for me to put all of these worries behind me. Tonight I am staying at Chase Hospice with Daisy, tomorrow Andy and I are going to Wembley Stadium to see Coldplay as our wedding anniversary treat to ourselves. This will be our first night off in a year as Gemma will stay overnight in Chase to do Daisy's TPN. On Saturday, Andy and I will stay in Chase without the other children (mum is looking after them for the weekend). After we have put the TPN up we are going out for Dinner! This will be the first time we have stayed at Chase without the other children and with Daisy now TPN dependent this is the only chance we get to have a proper break. On Sunday mum will drive to Chase with the rest of the children so that we can all enjoy the famous Chase Sunday Lunch as a family.

Being at Chase is giving me a break from all of the stress I deal with on a day to day basis. This service is provided completely free to our family and is funded almost entirely by voluntary donations - without Chase I really don't know how I would cope.

I'm off to bed now, no getting up to Daisy tonight and no school run tomorrow - bliss!!!!!

We lasted 18 days at home before a return to hospital....I have been putting off updating this blog because I wanted to write that we were back home, which we are now, but also because managing the needs of four children during the school holidays and a husband who is working freelance means that there are even less minutes, let alone hours in the day to update. In not writing for a while I discovered how many people actually read this blog as so many people have contacted me to ask me to update it. It is so flattering to know people enjoy my writing and I promise to try harder to update a bit more frequently. In fact it will make my life easier to do so as so much has happened since my last post that it becomes it nighmare to condense it all into a manageable chunk of text for people to read, so in advance, apologies if this one rambles a bit but here we go.......


I looked back over my last post and the signs were all there - the early return from Chase with high temp...of course it was not just going to go away... The day after our return from Chase Daisy was due for bloods so we decided to run some blood cultures also to check that there was no line infection, I think 18 days at home had made me forget too quickly as I was caught off guard when a call came through from the ward to say that the cultures had come through as positive and could I bring Daisy in to start IV antibiotics.....





Hindsight is such a great thing isn't it - she had been looking increasingly pale as the days at home had worn on and I think I was just trying to ignore it. Things went well in hospital for a while, she responded to antibiotics and the plan was for me to take her home and continue to administer IV antibiotics at home, but of course this was never going to be a smooth journey. To cut a long story short, big sister Xanthe accidentally pulled out Daisy's jejunostomy tube (all 30cms of it, sutured into place in her intestine) while Daisy was allowed home for a few hours before discharge. I had been out at the time and came back to chaos - Daisy screaming, Theo panicking, Xanthe very tearful and Andy with a 30 cm jejunostomy tube in his hands. Normally if a child just has a gastrostomy button, which Daisy used to have, it is possible to simply reinsert it - we have done this at home previously. A jejunostomy is a different matter as it has to be put back surgically....and so our problems began.


As far as GOS was concerned Daisy was not an emergency as she had a Hickman line and could receive nutrition via TPN, the fact that we had to run her TPN over 24 hours and switch her immunosuppressants back to IV infusions lasting for 2 hours twice a day, didn't seem to matter. We were frantically trying to get her back into GOS to get her jej tube back in as she had been so close to home after what had initially been just a blip. But of course once one thing goes wrong the house of cards just falls down, the fluid came back on Daisy's hips causing her massive pain and then no suprises she spiked another temperature. Of course Daisy couldn't spike a temperature in a safe controlled environment like the hospital ward, instead she waited until we had been allowed a few hours day release at Chase Hospice.


The plan had been to leave Daisy with Gemma and Rachel at Chase while I took the three other children for lunch. We had just settled down for a lovely lunch when I got a call from Gemma - Daisy's temp was up and she had become poorly - all in the space of an hour. Cue a frantic request to our waitress for takeaway containers, our meals were poured into them and we ran through the streets of Guildford. The children were so brilliant, taking it all in their stride as always. I was doing my quick mental scenarios of what to do - if she was dangerously septic then it was a 999 call scenario, if not well enough to travel back to Queen Mary's then we would have to take her to the nearest hospital, Royal Surrey, who not suprisingly have their own set of notes on Daisy. Fortunately when I got to Chase I felt I had enough time to get her back to Queen Marys. The lovely girls from Chase dealt with the issue of what to do with the other children by taking them swimming and arranging to bring them home separately.




Daisy was started back on IV antibiotics, yet again but at least GOS had come through with a date for the surgery. The biggest issue with having Daisy in over the summer holidays was not only that the children were on holiday but also anti- Swine flu measures meant that only two named adults could be with Daisy on the ward so I was left juggling children, Andy's work schedule and Daisy on 24 hour TPN. The agreement with GOS when we had been discharged had been that if Daisy ended back up in hospital again only Andy or I would access her hickman line. That worked well for the first few days, but as the days turned into weeks and the number of times her line needed to be accessed increased with infection the practicalities of this became more and more complex. The bottom line was that I was expected to be two places at once, 24 hours a day with Daisy administering 4 hours worth of IV infusions plus various antibiotics, taking bloods and managing her TPN as well as looking after our 3 other children on their summer holiday - clearly not practical especially as all my friends were on holiday (and why should they have to look after another child during the school holidays), by this stage all the holiday clubs were booked up and my mother had swine flu and couldn't come up. At one point I even had to leave the children for over an hour in the lift lobby outside the hospital ward door playing on their nintendo's while I sorted Daisy out! A complete nightmare!



I think our experience of looking after Daisy over the summer in the hospital shows that with Daisy you can have plans - for example being strict on exactly who can touch her hickman line, but the reality of our lives means that these plans need to be fluid. As I said to the Matron of the Children's ward would she expect a member of her staff to work 24 hours a day, seven days a week with no break? I think what really brought it home for me also was when Daisy developed an infection while on the ward and I was worried about her - I wanted to be worried about her as a mum, not also worrying about managing the medical side of her. The pressure of managing a lot more of Daisy's medical needs while she was an inpatient knocked both Andy's and my confidence as we realised how much pressure there is on managing Daisy's complex medical needs. The GOS policy is to minimise the number of people accessing a TPN dependent child's central line in order to prevent infection, but the reality is this needs to be balanced with the practical and emotional needs of the family. We are going to have further conversations with all of the teams involved in Daisy's nursing care to fine tune plans based on our experiences over the summer.



Anyway, to cut a long story short, Daisy ended up back on Rainforest ward and eventually she had her jejunostomy replaced. Having spent 2 weeks with just a naso-gastric tube holding the stoma open we were not even sure if the existing stoma would be viable. This was pointed out to me by the surgeon who came out of the theatre to inform me that if they could not use the existing stoma they would have to wake Daisy up and rebook her as they did not have enough time to do the more complicated procedure of opening a new stoma. Well, thankfully (and I say thankfully for the surgeon's sake as she seemed to think it would be OK to anaesthetise my child for possibly no reason) the stoma was viable and she came back from theatre with a lovely shiny new trans-gastric jejunostomy.....and an allergic rash to the anaesthetic which required IV antihistamine...





While we were in GOS Daisy had a scheduled appointment with her orthopaedic surgeon. This has to rate as the worst meeting with a consultant I have ever had, for so many reasons. At the time Andy was at home with a bad kidney infection, running a high temperature and trying to look after 3 children, I was completely sleep deprived and emotionally raw . Orthopaedic clinics are always chaotic and this was no exception, lots of wheelchairs means limited space, ontop of that we were late and Daisy's infusion had not finished, I forgot to pick up her notes when I left the ward so that caused further delays. When we got to the surgeon he did not even acknowledge Daisy and just leapt straight into "well you want me to look at her hips" - well no actually I would like you to understand some of the complex orthopaedic issues the children with costello syndrome have, currently manifesting as severely tightened achilles tendons causing her an inability to put her feet flat on the ground or walk unaided, her risk of scoliosis, and yes, the fact that she keeps getting this fluid on her hips which is not a costello thing and is causing her a lot of pain.....that's what I wanted to say but instead I blubbed some nonsense and burst into tears, something I never do infront of health professionals. It probably helped slightly as, although he still did not interact with Daisy (I guess this is true of most surgeons, they are used to seeing their patients asleep), he said her feet needed surgery and that her hips were a rheumotology issue and the gastro team on Rainforest should involved rheumotology in her care. Bang, consultation over, except it was only later I realised there was no plan for scheduling her foot surgery and the problem with Daisy's hips was not his problem....



We waited all day on Rainforest for the rheumotology team to come and review Daisy, possibly with the plan of booking her in to inject steroids into her hips under general anaesthetic. The plan had been to transfer back to Queen Mary's that day so transport was delayed until this review, the review that never came because at 5pm a call came through to the ward that the Rheumotology registrar could not make it that day (something to do with being the Friday before the bank holiday weekend perhaps) and our local hospital should refer and sort it out - bloody great. So there we were, no ambulances available to transport Daisy back to Queen Marys and no further forward on sorting out the problem with her hips...we got back to Queen Mary's at 9.30pm and as for the Rheumotology appointment, well we are still waiting, I'm planning to see what tomorrow's post brings and then it may be toys out of the pram time again.

The rest of Daisy's time in hospital was spent weaning her TPN down from 24 hours a day back to 14 hours and building up her jejunostomy feeds ml by ml to 15mls an hours for 10 hours a day - no hopes of any breaks from some sort of pump yet as her blood sugar drops as soon as they are stopped. But the good news is that after a month in hospital - in fact adding it up it was 12 months in hospital altogether with just 18 days at home - Daisy came back home again last Tuesday!!! Yippeeee!

We are seizing the moment as we strongly expect another crisis to hit, there is no reason not to expect this, so in the meantime while she is looking the best she has for months, she is back at school for 4 hours a day. And loving every minute of it of course. We are just loving having her home - everything is so much easier when she is at home; updating the blog, doing the TPN, doing the meds, helping the other children with their homework, catching up with my friends, spending time with my husband - it's just great and I really do savour these moments to keep me going during the inevitable hospital stays that will come.

Next week brings a cardiology appointment and the worry that brings with it that her cardiomyopathy may be worse still. But it also brings the normality of family life as the children get back into the full swing of school life, after school clubs, music lessons, swimming. With Daisy at school during the morning I can start attacking the cupboards which have been stuffed full of clutter and papers over the past year - the obsessive compulsive in me is looking forward to this greatly!!!!

Till the next time...which I promise will not be as long...

Daisy has now been home for 2 weeks and, just like the pain of childbirth, memories of the endless months in hospital are slowly disappearing.... Just because Daisy is home does not mean she is better and the stress and worry of looking after her is still there, however this time we are in control and at least we are in our own environment.

The first week at home was spent getting into the swing of doing the TPN at home, clearly 10 months in hospital has institutionalised me as I have arranged Daisy's drug cupboard like a hospital cupboard and labelled all the shelves and boxes , sometimes the obsessive compulsive streak in me can be quite handy. We have now been trying to arrange and organise everything Daisy needs to have some sort of normality and for Andy and I to get a break. We have been lucky in getting our care package hours increased to get some more support during the day and we have relented and agreed to having night support which will mean having someone in the house for a few nights a week to manage Daisy's needs overnight. We have always shied away from this sort of support, preferring to rely on time spent at our hospice as overnight help, but as Daisy has moved the goalposts in her needs somewhat we have to accept more help with her. Of course this is all in the planning stage - now we need to get the right people in place to help us. When you have a child with complex needs you get used to having people come to the house all the time, over the years we have got used to a regular team of people - our friends from Chase Hospice and The Rainbow Trust, community nurses, key worker, social worker, physio. Now our team will be expanded even more.

We had a discharge videoconference with the Great Ormond Street Team and Queen Marys team a few days after coming home. Some people couldn't make the meeting but I was still amazed with how many people are involved in Daisy's medical care - and this was only to discuss the gastro elements of her life. We now have plans for managing future line infections and how to manage various other emergencies. The main plan to stay infection free however is for only Andy or I to ever touch Daisy's hickman line. This is a big emphasis with the GOSH home TPN programme, their research shows that the less people accessing the line, the less risk of infection. We also have to use anti-microbial "line locks" which have been proven to decrease infections in home TPN patients by 70%. The bottom line is to try and optimise Daisy's chances of remaining infection free we have to commit to only being the ones to put up and take down the TPN, take bloods from her line and do all the other stuff involved with managing a child with a central line on home TPN. At times over the past 10 months we were very close to not bringing Daisy home alive so this is a small price to pay for having her home.

Our second week at home has been spent at our wonderful hospice, Chase, in Guildford. Daisy stayed in her own room (complete with obligatory TV showing continuous Peppa Pig episodes) while the three other children, Andy and I stayed in an upstairs flat in the building. I did the TPN connections in the evenings and disconnections in the morning but apart from answering questions on Daisy's care my role was redundant which meant I could indulge in some retail therapy in Guildford and enjoy lovely walks along the canal near the hospice with Andy and the children. In fact tempting the children out into the fresh air for walks was really difficult as there is so much to do at Chase and the british weather even managed to shine for a couple of days so they could play in the garden. We left on Sunday, a bit worried as Daisy decided to spike a temperature and I had visions of putting the A&E plan into action. Her temp came down later in the evening and I am keeping my fingers crossed it was nothing sinister, although we did blood cultures this morning to rule out any infection. Daisy is massively at risk of infection at the moment as not only is she immunsuppressed because of the treatment for the inflammatory bowel disease, but she is also neutropenic, which means she does not have enough of the disease fighting white blood cells to cope with an infection. The GOS doctors think this may be a side effect of one of her medications, unfortunately this medication is working well to help calm down her inflammation so they don't want to take her off it, so for the timebeing she is having regular bloods done to keep a close eye on things.

We have a week at home now and I am still trying to get to the bottom of the mountain of clutter that has grown in all the cupboards since Daisy has been born. The best thing is just being able to spend family time together , Daisy's infection risk means that we can't plan too much but it's so nice all be under one roof - we regularly ignore the phone (sorry to all those that have experienced this) and the door bell because after months of having people around us all the time, it's just so good to be just us....