We had such a wonderful Birthday celebration and Christmas with Daisy! She was soooo excited just to be in the ambulance (that's how weeks in isolation gets to you, I guess prisoners must feel the same when they have a court appearance!) and her little face when she realised when she was at Chase.... it was a picture. The picture above is Daisy getting ready to leave the ward with our wonderful Chase friends Gemma and Rachel. If it wasn't for Gemma and Rachel we would not have got to Chase for her birthday or Christmas, they organised everything - training all the relevant staff on how to do Daisy's TPN and infusions, liaising with the ward, sorting out presents for the children and Daisy's birthday party and even booking a babysitter for Andy and I to go out for an evening during our stay.

This is Daisy arriving at Chase and realising where she was!

Getting stuck into the "In the Night Garden" DVD box set....

Opening presents with big sister Xanthe.

I will post more pics but I need to download the memory card from Andy's camera, and as I said, at the moment there are just not enough hours in the day!

We just had the best time at Chase - Margaret the chef cooked wonderful veggie food for us as well as Daisy's birthday cake (which Xanthe decorated), the nursing staff took care of the TPN, the boys alternated between the Xbox and the computer room and Xanthe created a production line of Christmas presents and cards in the art room. I caught up on some sleep and Andy caught up on some TV! Daisy was in her element, she was able to sit in the high chair with us for mealtimes - something we have so missed at home, she loved the sensory room and joined Xanthe's craft sessions in the art room. Seeing her at Chase made us remember what a bright little girl she is, she just loved all the social interaction and attention. All the activity meant that she depleted her blood sugars a bit quicker than usual and we got through a couple of tubes of hypostop when her sugar levels dropped. It's something we are going to have to remember when she is back on her feet.

Christmas day was magical - Santa had left snowy footprints down the corridor and there were big sacks of presents outside all the children's rooms and even our room! Daisy has got the hang of present unwrapping and joined in with all the general mayhem with the children. After breakfast two fire engines arrrived and Santa got out of one of them together with more presents! Jules ran a mile - inspite of his gun obsession and violent tendencies he is a wuss at heart and is terrified of Father Christmas! Daisy loved the fire engines and the firefighters were very taken with her. They sprayed the gardens with white foam and turned the outside into a winter wonderland, it looked fantastic.

We returned to the hospital on boxing day and everyone commented on how well we all looked - I definitely benefitted from some sleep. Daisy has been in fantastic form since Christmas and the best news of all was that her blood cultures have come back clear which means that (for now) we have beaten the bugs in her bloodstream. She still needs to be on her antibiotic infusions (including a dose of Vancomycin which would normally be prescribed for a 70kg adult according to the pharmacist!) until her port is removed. This is the sticking point - our friends at Great Ormond Street offered us a date of 23rd December to take the port out and insert a picc line temporarily. As I had turned this down Daisy now has to go onto the waiting list, even though I told them not to offer us dates around Christmas week. It would have been so good if they could have taken her in this week as Andy is off work and childcare is easier but that is not looking likely. The doctors at the local hospital are liaising with Great Ormond Street to try and get things happening, until she has the port removed she cannot start on the new immunosuppressant therapy that her Gastroenterologist wants to try and after the port removal she needs a series of other procedures which are all being delayed.

Our focus for the new year is to keep pushing to get things in place for home TPN for Daisy. I have seen a lovely adapted special needs cot which would be perfect for Daisy as she needs a hospital cot at home now but this one doesn't look too institutionalised, we have applied to a charity to fund it. We are also looking into installing some sensory lighting into her room as she loves the sensory room at Chase and at her school so much. We will get her room decorated in the new year so that at least we are all ready to go from our end.

Anyway, it's new year's eve and we are actually going out tonight. Our friends are having a child friendly party, so Andy will come home from the hospital at 7.30pm and take the boys and I will go up to the hospital for the late shift and go to the party at around 10pm. We'll toss a coin to see who does the early shift tomorrow morning!

I know 2008 has thrown us a few unexpected suprises but it has also given us many things to be happy about - Theo started at the London Oratory and is loving it, even the tube journey does not phase him. Xanthe is doing brilliantly at school, she is involved in so many activities both during school and is doing well with her Flute and recorder playing, Jules seems to have clicked with school now and his reading is coming on so well. Daisy was such a hit at her school and we can't wait to get her back there. Inspite of everything going on Andy and I are so lucky to have four such wonderful, unique and happy children, it's something we will never take for granted.

Thanks to all our family and friends for their cards, messages and presents over Christmas and all our best wishes for a happy, healthy and successful 2009!

We made it!!!!! We were transferred to our hospice this morning and Daisy got to spend her birthday with the family! Everyone is tired and happy just to be together under one roof at long last. Will post photos tomorrow, I'm off to round up the older 3 children who have gone hyperactive with all the sugar they have consumed.............
It's been one of those weeks, we have been thrown challenge after challenge - not all of them Daisy related.

We are trying to keep focused on getting to Chase Hospice for Daisy's birthday on Monday and to spend Christmas together as a family. When we met with her GOS consultant last week he agreed to keep this week free and try and get a date asap to get her back in to have her infected line removed and a temporary picc line placed until we could go back in for a long term stay for all her other procedures. However despite his best efforts the only date that was available was 23rd December - the day after her birthday and just before Christmas. As Daisy has previously had problems with anaesthetic there was a danger that she could spend Christmas day in intensive care, meaning that Christmas would effectively be cancelled for the rest of the children who are counting the days until we go to Chase.

Our moral dilemma was made worse as Daisy started to spike temperatures again - we were running the risk that if the MRSA was still in her bloodstream we could find ourselves in an emergency situation with a potentially fatal blood infection. The doctors took blood cultures and I kept everything crossed that the temperature spikes were the same virus which is currently laying Andy low and nothing more serious. We had a call from the microbiologists to say that the bloods were growing something meaning that she had a bacterial infection of the bloodstream - with Andy feeling so rough I had to try and make the decision, did we cancel Christmas and go to GOS or did we take the risk with the line and go to Chase. I was talking this through with Daisy's consultant when the attending consultant popped her head around the door of the cubicle to let me know that although the cultures were growing one bug it was not MRSA and not only that for the first time in 15 months her skin swabs around her gastrostomy site were negative for MRSA. This was amazing news, there is a strong possibility that we may have rid Daisy of her nemesis!!!! This made the decision to turn down the pre-Christmas GOS admission slightly easier - only slightly, the bug she does have in her bloodstream is still nasty and left unchecked could cause heart problems (not great with an already problematic heart) however on a scale of nasty bugs to have it is slightly less nasty than the virulent strain of MRSA that Daisy had been infected with.

Of course things are never ever straightforward with our Daisy -the microbiologists would still like her line removed and this needs to be organised for as soon as possible after Christmas. Given that she also needs several other procedure at Great Ormond Street in the new year we will probably transfer up for a longer stay.

While we allowed ourselves to breathe a little sigh of relief over the MRSA, another issue crept up to suprise us. Daisy is severely anaemic - at first it was thought that blood samples may have been contaminated, but repeat samples were lower. As always with Daisy the doctors are a bit stumped as to why haemoglobin levels are suddenly so low. The assumption is that she is anaemic because of her chronic gut issues but the haemotologists have ordered lots of tests to try to get to the bottom of things. In the meantime, Daisy is currently having a blood transfusion, this is not as straightforward as it would be in any other child, Daisy has a problem maintaining her blood sugars as a result of her very minimal levels of growth hormone (this plays a role in regulating blood sugars as well as growth). Her endocrinoligist has taken her off growth hormone therapy while she is so ill so her sugars can drop quickly. While she is being transfused she cannot be on TPN so we are having to monitor her blood sugars incase she develops hypoglycaemia. I have a tube of hypstop at the ready to rub into her gums to bring her sugars up...nothing is straightforward with this girl!

Not only were we dealing with Daisy's issues this week, it was also the end of term for the children with the usual school plays, parties and trying to get end of term presents sorted for the teachers. With the tiredness and emotion of everything I walked into a door and narrowly avoided breaking my nose - as I held my face with blood pouring out of my nose I just couldn't believe what I had done, at the same time Andy was lying in bed with a temperature and the children were overexcited about going to Chase for Christmas, completely unaware of how close it had come to being cancelled. The icing on the cake then turned out to be having to take our elderly, senile old cat to be put down - the one who had mistaken my old laptop for a litter tray had now taken to using the dining table or kitchen worksurface instead. The time had come for an old friend to go in a dignified way...

Needless to say Andy and I are living on reserves now - thank goodness my mum is staying to hold the fort! We are counting the hours until Monday when Daisy will (hopefully) be transferred to Chase to celebrate her 4th birthday. The team from Chase have pulled out all the stops to make this happen, nurses have visited to be trained on how to do Daisy's TPN and they are happy to do all her various drug infusions. Having Chase hospice as part of our lives has become more important than ever now - just having a few days off the physical effort of trying to be in several places at once and keep smiling will just be so fabulous. We are due back in the hospital on Boxing day and the following week we will probably be transferred back up to Great Ormond Street but at least we will have recharged our batteries and we will have had the opportunity to spend some quality time together as a family.

I just hope my next post will be from Chase - I promise I will post some pics of Daisy in her party dress!

It's not the hand you're dealt with, it's how you play it

"It's not the hand you're dealt, it's how you play it" - is going to be my motto from now on. Last week I went through every extreme of emotion, having to deal with the reality that things are not that great with Daisy. Her GOS consultant came to the hospital on Thursday and spent a lot of time both with her doctors and with us. Now we have a far clearer picture of what is going on with Daisy's gut - in some way it is a relief to know that our instincts all along were right and that unlike the other children with Costello Syndrome, Daisy was not going to grow out of her gastro-intestinal issues. She has a diagnosis of pan colitis, basically her entire gastrointestinal tract is inflamed and ulcerated, caused by her immune system going haywire and attacking her gut. If the ulceration was localised to her colon then that section could be removed and if needs be she could have an ileostomy. She is not a candidate for an intestinal transplant as she has too many medical issues making her odds of survival zero. The possibility of a bone marrow transplant to attempt to "reset" her immune system has not been ruled out but this is further down the line. At this stage everyone agrees that the current treatment with steroids does not seem to have worked and so she will be started on new, stronger immunsuppressant therapy (and all its scary side effects).

Of course ontop of all of this is the small matter of the MRSA. It looks like the very aggressive and toxic antibiotic therapy is holding it in check but in all likelihood her central line is now colonised - meaning that the bugs are now clinging to it. So it has to come out, and this can only be done at Great Ormond Street. I found out today that her local consultant and GOS consultant have been in contact all weekend trying to get things in place for a new line before Christmas. It has to be done be an interventional radiologist, and the man who everyone wants to do the job is trying to move his list to accomodate Daisy. She has a habit of getting people to run around after her, it amazes me that we have got to the point that she is well and truly a signed up member of Great Ormond Streets special patients!

Not only are her clinical consultants rearranging diaries for her and juggling appointments but several microbiologists are working on the best drug treatments, pharmacists are tweaking tpn preparations and geneticists literally all over the world are testing her DNA and trying to get to the bottom of what has caused these massive changes to her immune system.

Andy and I find it ironic that as the goalposts are moved by Daisy's condition, previous possible diagnoses look more acceptable. I remember when I was pregnant with her we were told there was a one in four chance she would have Down's - I was devastated at the time but if I knew then what I know now......And now it looks like Daisy has something going on beyond her Costello Syndrome diagnosis. When Daisy was in the neonatal unit Daisy was tested for everything possible and we were told that although at the time there was no test for CS, hopefully she would not have that. Of course when a test was developed she tested positive and we got on with things, embracing her uniqueness both as a child and as a child with CS. Now we are facing the probability that she has another mutation or genetic issue alongside the CS and we find ourselves wishing that she "just" had CS - oh the irony!

TPN looks like it will be very much part of our lives from now on in. In the new year she will have another upper and lower endoscopy to see if there have been any changes in her gut following the drug therapy. She will also have a jejunostomy - so that we can try feeding her via the very top of her intestine missing out her tummy altogether. Giving her senstivities to volume when she is enterally fed nobody believes we will be able to get adequate nutrition into her via this route to do away with the TPN altogether, although it may enable us to limit TPN feeding to night-time only, and have jejunal feeding during the day meaning she could go home for the day and return to the hospital at night. In the long term we need to get set up to manage TPN at home - this is a minefield in itself although I know others who have managed it so I know it is not impossible, just even more life changing! However if this is the only way we can get Daisy home then that's just how it will have to be - as I said, it's not the cards you're dealt, it's how you play them!
I know my recent posts sound upbeat but they really disguise the severity of the situation. Daisy has MRSA in her bloodstream, she is on massive amounts of toxic antibiotics, her bowel is diseased and inflamed and we cannot feed her via her gastrostomy so we have to use her main line to give her TPN. It feels like we are trying to put our lots of fires at once, every time we sort one another one starts. Clinically she looks well but her bloods tell a different story. MRSA has become our nemesis - she was colonised with it on a hospital stay 14 months ago (not Queen Mary's, you work it out) and it stuck in her gastrostomy - the perfect breeding ground for bugs. While it stayed in her gastrostomy it was fine - lots of people are colonised with MRSA and don't even know it, on it's own it's not something to worry about. However if it gets into the bloodstream it can cause a problem - not that great a problem if your immune system is working OK and you are well, but if, like Daisy you also have a permanent line into a main vein and are on massive amounts of immunosuppressants and steroids this is a bad combination.

So ever one to move the goalposts, Daisy has now ensured that the priority is to sort out the MRSA - there is a big meeting on Monday of all the microbiologists and various doctors who may be able to work out a solution. She is on huge amounts of toxic antibiotics to try and contain the infection and clinically she is doing really well. Apart from the high temps last Thursday she has stayed well and bright. We know that children with Costello Syndrome metabolise meds differently and respond diffently to problems (and sometimes in a positive unexpected way as my friend Lisa reminded me in a recent email!) - so Andy and I are just hoping that Daisy will suprise us.

Then the next priority is to get us all to Chase Hospice for her Christmas and Birthday and then we have to get back up to GOS to find out if her gut will ever work or if we are facing long term TPN - all of which will radically shorten Daisy's prognosis.

I have had a lot of wobbles over the past few days and for the first time ever burst into tears infront of her consultant and dietitian, both of whom as mums themselves have become close friends over the years. Christmas is so important, this may be the last one we have with Daisy and everyone is doing everything to make this happen.

We have had lots of scares with Daisy before - when she was born and when it was thought she had neuroblastoma, but there are too many things going on at once and we are playing a very fine balancing act. However, Daisy has suprised us before and we are relying on her to suprise us again and defy the odds.

We were lifted yesterday afternoon however by the news that her Consultant from Great Ormond Street is coming today to meet with the team here and discuss Daisy's case. This is fantastic news - she has an amazing team looking after her and clinically is stable. We all just have to stay positive (and this goes for people reading this blog!) and be determined that Daisy will be OK!!!!!
Daisy has not been having the best of times over the past couple of days. The infection I had hoped was just the general lurgy turned out to be a bit more serious. She woke up on Thursday morning and vomited bile everywhere, her tummy was distended and the blood cultures showed she had a bacterial infection in her bloodstream. Thank goodness an xray showed that the distention was not the feared toxic megacolon which we dread. She had an awful night but after a few infusions of vancomycin and other antibiotics she turned the corner. Thank goodness the decision was made to use vancomycin which is normally saved as a last resort as the bugs in her bloodstream mutated to an active MRSA infection. We have hopefully got things under control now but the issue is whether it was her line which was infected or bacteria leaking from her gut (or both). She has turned the corner but the microbiologists are keeping a close eye on her blood cultures to make sure we have zapped all the bugs...

I tend to have my clearest thoughts and ideas at around 3am as I am normally awake at that time dealing with Daisy - it occurred to me at 3am on Friday morning that if we turned up for our outpatient appointment at Great Ormond Street in a week's time her gastroenterologist might not be completely up to speed with what has happened since she was transferred back here (yes I know you woud assume that the docs would keep them informed but I have learned to never assume anything in this game). I rattled off an email to his secretary and got a reply pretty quickly - the good news was that instead of us traipsing up to GOS for outpatients with all the hassle of ambulances, nurse escorts and taking enough TPN, he is prepared to come to Queen Mary's to see us. Sort of like a state visit! The bad news is he wants Daisy transferred back to GOS as clearly the treatment with steroids is not working. I am just hoping that they cannot free up a bed until after Christmas - we have to go to Chase at Christmas, it's the only way we will all be together as a family. Ironically the active MRSA infection will probably provide a suitable delay so that they will decide it is easier to wait until after Christmas.

Daisy was brighter today and she had a lovely day playing with Andy and Jules while Xanthe and I met up with friends to make Christmas wreaths. Poor Theo was left at home with his latin homework! I'm going to to home to sleep tonight as I need to build up my sleep credits again again. I'm waiting for her pain meds to kick in then I can get going as I'll be back here before I know it/
Daisy has not been very well today. She was really grumpy this morning and over the course of the day her temperature was getting higher and higher - unfortunately she can only have calpol and an occassional dose of codeine but in between we had to have the windows wide open to keep her cool. This was not great considering it was below freezing outside! She's off her milk again as she had lots of diarrhea - I think this is going to be the pattern now until Great Ormond Street decide what they are going to do.

To cap it all she snapped a line again - we really don't know how that happens. This meant that her port needed to be reaccessed again which involves changing the needle to it. This was going to be done anyway on Friday so we had to bring it forward. We're waiting for her blood results to come back - I'm just hoping she's picked up the general lurgy that half the population seems to have at the moment and nothing more ominous. I'm not anticipating much sleep tonight, it seems over the years I have managed on less and less sleep, I just grab it when I can!

I nipped home for a few hours this evening as I had promised the children that we would put the tree up. It was lovely to see the house looking all Christmassy, that's the best thing about having 3 other children, with Daisy not doing too well it would be very easy just to not make the effort with Christmas, but the other 3 would not forgive me if we didn't go completely over the top with Christmas as we have done every other year.

We have packed our Operation Christmas Child shoe boxes ready to be collected from the school - we love to pack these boxes and imagine a child somewhere in the world opening presents from us on Christmas day. I have been reminding the children recently that even though they think things aren't great at the moment they are in fact so much luckier than many children. Even Daisy has done well in the lottery of life - I know I can complain at times but we have a fantastic, free, healthcare system, we live in an affluent part of the world, have a lovely home and she has two parents who are articulate and intelligent and hell bent on getting the best for her. People often Andy and I how we cope and they can't believe that we are always so positive about things - our answer is always the same - we cope because we have no choice and we are positive because believe it or not, things could be worse!
We have been trying to regrade Daisy's feeding - this apparantly is the medical term to get someone off TPN (feeding through the veins) and back onto enteral feeding (through the tummy). As Daisy is very volume intolerant and ontop of her inflammation has lots of other issues with her digestive system it was decided to start her very slowly - on 1ml per hour of her feed over 20 hours . Yes, that's right! 1 ml per hour - not even enough to put through her enteral pump, we had to syringe it into her gastrostomy! Well - the reality is we are only going through the motions of trying to regrade her as no-one really believes she will be back up to anywhere near a sensible volume for a long, long time. Daisy reacted by having lots of mucousy diarrhea ( a sign that her gut is still inflammed) and then her tummy bloated up like she was pregnant - a sign that her gut was on strike again! So needless to say we stopped the feeds and have tentatively restarted them again today although she is still looking bloated and is uncomfortable tonight. The whole idea more than anything is to keep the gut stimulated ready for feeding at some point.

Everyone here is waiting for Daisy's next appointment at Great Ormond Street Hospital in just under 2 weeks. We hope that Daisy's gastroenterologist will give us a date for her next upper and lower endoscopy and biopsies. We also need him to confirm formally that Daisy is on TPN for the long term so that we can get the ball rolling to get TPN at home and eventually get her home. Getting home is a long way off for lots of reasons - no-one really believes that the steroids have worked and we may need to start some other treatment protocol and ontop of that going home on TPN is very complicated and when we do we will be the only family in our area on it. The pharmacist told me today that it can take a minimum of 2 months to sort out and given our experience in waiting 12 months to fund and set up Daisy's growth hormone injections we are not holding our breath. I'm now starting to think that Easter could be a good time to aim for.....

It's so frustrating as our lives are really in everyone elses hands, not least Daisy's . All these plans are made on the assumption that she will be well enough to come home however, she does have a habit of throwing some not inconsiderable spanners into the works. Andy and I managed to catch a rare 5 minutes face to face this evening and we both agreed that there's no point in beating our chests about the situation, it's crap but it could be so much worse and we are just going to have to get on with it, we've got this far, we'll keep going.

We did have some good news today. Up until about 6pm this evening our Christmas together at Chase Hospice was looking in jeopardy - there was the issue with taking the hospital TPN pump with us and then we were told that there was no guarantee that the ward could hold Daisy's cubicle for us while she was out of it. The reality that we were facing was to risk leaving the hospital with no guarantee of a bed on boxing day, we would have had to go to A&E and be shipped to wherever there was a bed. I had visions of all six of us crammed into Daisy's isolation cubicle on Christmas day trying to make the most of it and suggested to the hospital managers that they might want to explain the real reason for not being at Chase to the children. Anyway, to cut a long story short the powers that be (whoever they are) decided that in the season of goodwill not only could we take Daisy's TPN pump out of the hospital but they would hold her bed, not just for Christmas night but from her birthday on the 22nd through to Boxing day. This means we will have a whole four nights together in Chase Hospice before having to return to hospital - barring any of Daisy's infamous spanners of course.....!
It is inevitable that we will be in hospital for a long while now. I half jokingly mentioned being home by February half term to Daisy's dietitian today but she said I shouldn't think about dates anymore so that I'm not disappointed. We know that another stay in GOS is on the cards, probably in the New Year, for a full thickness biopsy and endoscopy, that way we will know definitively if Daisy's intestinal system will ever work properly and if not then at least we can prepare ourselves for long term TPN.

I'm trying to get things in place to support Daisy's developmental needs while in hospital. She currently has funding in place to attend school full time, even though she is still only in the Foundation stage. Even if she did not have this statement she would be able to access early years support. While in hospital however she is receiving nothing. The ward teacher here is really only equipped to teach older, more able children. Daisy's needs are so specialised as she is partially blind - hence the reason we worked so hard to get her to the school of our choice which specialises in teaching children with sensory impairments. She was coming on so well and we don't want her to miss out just because she is in hospital. The other problem is being in isolation. As she is MRSA positive she cannot leave her room, also her immunsuppressed status means that she is very susceptible to infection. Ontop of all of this she is tied 24 hours a day to her TPN pump. At school she was getting physio and all of the other therapies a child with complex needs should have, this has all been put on hold while she is in hospital. This is going to be my mission over the next couple of weeks to get a longer term care plan in place for her so that she continues to move forward.

This laptop has been very helpful in keeping Daisy entertained, we spent ages on the Something Special website today and used the webcam so that she could see herself on screen - she signed "me" and "mummy" when she saw us on screen. I just wish there were more people involved with her on a daily basis who are able to use sign language, she understands so much but only Andy and I really know what she is trying to say.

Our other project is getting things in place for us all to be able to spend Christmas at our hospice. It appears there now may be a problem with Daisy using the ward TPN pump, something to do with insurance, and as she is the only child in the PCT area on TPN there is no mobile pump in the community for her to use and it is too short notice to order one. Daisy's consultant is on the case, she is a mum of 3 young children herself and there is no way she will allow us to miss Christmas together as a family because of a bit of paperwork!
Today is my lovely husband's birthday - however I am here in hospital with Daisy and he is at home watching the footy. We have friends staying over on Saturday night to look after the children so hopefully we can actually get some time together this weekend, even if it is in Daisy's hospital room.

Yesterday was another busy day. The hospice had arranged for a group of mums to have a trip on the London Eye followed by lunch at the Harvey Nicks restaurant in the Oxo Tower. I had the night from hell with Daisy the night before, so was feeling very tired in the morning and not in the mood to put my glad rags on. I was also trying to catch up with Daisy's doctors to discuss plans and next steps before running for the tube to get to Waterloo in time. I did at one point find myself in the surreal situation of standing in the ward equipment room surrounded by IV poles and cardiac monitors trying to tame my wild hair into something slightly more presentable while the ward housekeeper stood guard outside!

It's always good to catch up with other mums of special children, it's the best way to swap tips and get advice. It was so strange being out in the real world however and I was torn with wanting to be back with Daisy, who was quite happy in the care of one of the chase nurses.

I stayed in the centre of London after our Oxo Tower lunch as Andy and I had been invited to Fortnum and Masons to tell Daisy's story to a group of invited guests at an event for the Association of Children's Hospices. I had written out Daisy's story and Andy had put together a slideshow of pics of Daisy and the children, I nearly lost it at one point however when I talked about the fact that Daisy is life limited and looked up to see grown men in front of me in tears. The event went very well and the Association of Children's Hospices hoped to have a few more corporate sponsors as a result.

We travelled home on the Northern Line and Andy dropped me off at the hospital while he went home to sort out the rest of the children. Once again Daisy had a very unsettled night despite regular pain relief. We are going to try some antispasmodic drugs tonight and see if they help. I think the bottom line is we are not convinced that her gut is healing as quickly as hoped. Our consultant here is going to write the the Consultants at GOS asking again for them to do a full thickness biopsy of her gut. This is the only way we will know if there is hope of it getting completely better or whether it's best to just persevere with the TPN and a tiny bit of enteral (via the tummy) feeding.

We did start the enteral feeding today - although you can hardly call it feeding - she is receiving 1ml an hour of her normal prescription milk but less concentrated, over a 20 hour period. ie: 20mls a day of milk or five teaspoons!!!!! We are going really slowly with the feeding, the doctors here know Daisy so well and they know that if we push her too quickly then she will suffer a setback. No one is completely convinced that she will ever be back to her regular regimen of 70mls/hour of concentrated feed. At the moment the best Andy and I are hoping for is a combination of TPN supplemented with some enteral feeds, this would be enough to get us home at some point.

We started the conversations today with the staff here about logistical arrangements for Christmas and our two night break at the hospice and all that will entail in terms of equipment, emergency supplies, drugs, TPN, transport etc. At the same time I thought it would be useful to get the community nursing team thinking about Daisy's needs for going home, although this is a long way off, as there is a lot involved in terms of supporting a TPN dependent child at home and Daisy will be the only child in area on home TPN. We thought we would do our bit and get her room redecorated and ready for when she does come home - I'm thinking an "In the Night Garden " theme although she might be so excited at pictures of Upsy Daisy and Iggle Piggle on the wall she might never sleep...............

I'm staying late at the hospital tonight then going home as Andy has to leave early to get to the Reading office so will not be able to drop the children off. Xanthe came home from school with a sore throat and croupy cough so I am already anticipating that tomorrow will be a bit of a nightmare . She probably will not be able to go to school and she can't sit in Daisy's cubicle because of the infection risk, she can't go to anyone elses house if she is feeling poorly so I'm going to have to juggle big time tomorrow. I have a nurse sitting with Daisy from 3 till 7 tomorrow evening so at least that time is covered - roll on the weekend!
Never content to do things by halves Daisy decided to brighten up our Friday by tying herself up in knots with her lines until the one into her main vein snapped (which it should not do but is clearly not Daisy proof) and she poured blood out straight from her line all over her cot, at the same time she thought she would whip her nappy off and wave it around in some sort of infection party invitation! This is the nightmare with Daisy and her lines - she throws herself around when asleep as this is when she gets most of her pain and gets herself completely tangled up. I wasn't too bad when it was just her gastrostomy feed as there is less danger in pulling that out and I can replace a gastrostomy button at home but main lines into veins are a different matter......

Of course all of this happened when I had just nipped home to sort out the various musical/scouting activities that our children are involved in. As Daisy's vein access is via a portacath it could be reaccessed on the ward. Normally fairly straightforward but Daisy being Daisy it took 3 attempts! Fortunately she is currently on antibiotics and this so far has kept any infection at bay.

Her antibiotics are currently being reviewed, she has a regular prophylactic dose of one sort to prevent the recurrent UTIs caused by her bowel disease, however the bugs are now resistant to this one so we are going to have to start her on something else, always a bit worrying as her bugs can become resistant to that and our options decrease. She is already colonised with MRSA so we have to keep the big hitter antibiotics in reserve for use incase she develops an active mrsa infection.

I am full up with cold and Andy is brewing one too, so we are worried that Daisy will pick it up. The ward is full of children with bronchiolitis and while she is in isolation, she is so susceptible to infection particularly given the immunosuppressants and steroids she is currently on. In fact I have now nicknamed the bit of the ward we are currently resident on "bronchi alley" as all the cubicles here are full of little babies on oxygen - I'm just keeping the door shut and staying out of the parents' room!

We had a busy day today - a trip back to GOS to see her Oncology consultant. Daisy has had a named oncologist since she was a baby when she was suspected to have neuroblastoma. Since that time a lot more is now known about the causes of Daisy's syndrome and the associated malignancy risks - between 15 - 17%. Her oncologist was not aware of this and agreed that these risks were bigger than he had previously known and now we know she also has such extensive inflammation in her GI tract this adds to her risks. He is going to manage her screening protocols at GOS now as she is very difficult to scan normally as she has megacolon and the bowel disease makes ultrasounds quite uncomfortable for her. She had a scan today, always a worry as we do know of other costello families who have had tumours diagnosed during these routine scans. She will have them every 3 months now and will have another review with the oncologist in February.

We had to wait forever for an ambulance to transport Daisy back to Queen Mary's, it was getting very hairy as her TPN was beginning to run low and we had to reduce the run rate. Fortunately the ambulance crew had previously transported Daisy and remembered her going hypoglycaemic on them before so they turned on the blue lights to get us back before the TPN ran out, although I did need to use some hypostop on her towards the end as she was showing signs of low blood sugars. We're due back at GOS for another outpatients appointment in 3 weeks so we'll take some emergency fluids in case we end up staying there longer than anticpated. Life was so much easier when all I had to remember was a dummy and some nappies, it becomes a fairly exclusive club when your main means of transport is ambulance and you carry around spare bags of saline with 10% dextrose and tubes of hypostop.

Anyway, as Daisy is actually asleep for a change I'm going to turn in, she has had paracetamol and codeine together tonight so this should kick the pain into touch for a while...fingers crossed for a quiet night

Photo Time

Photo time!

Four little telly addicts enjoying some normally banned, innaporpriate american cartoon on Daisy's TV in Great Ormond Street

Hooked up to the ecg

Off for a little walk around the hospital corridors, TPN pump in tow. Jules insisted on wearing his Beaver Scout uniform all weekend & Daisy had to keep hold of her favourite Upsy Daisy pyjamas...
Coming back to the local hospital was such a relief but at the same time reality has hit us a bit. Last week we kept going with the adrenalin of dealing with all of Daisy's issues and the medical emergencies which always seem to happen when we are at Great Ormond Street (not to mention the battles with bureaucracy). Now we are back to a slower pace and trying to juggle school runs, afterschool activities and childcare with minimal sleep. Andy and I are taking turns to spend evenings in the hospital, leaving at about 10.30pm and I return at about 8am after dropping the children off at a friend's house and Andy at the station, what sleep we are getting is not great as we are torn between needing to be with the 3 older children and being with Daisy. The reality is though there was no way I could continue to spend every night with Daisy in hospital, the nurse handed over this morning that she had had a very restless night, awake and crying and needed top up pain relief overnight. At least the night nurses can go home and catch up on sleep, that's impossible for me as Princess Daisy does not sleep at all during the day - in fact both of us are in serious need of some undereye concealer.

She is still on 24 hour TPN and IV steroids as well as iv antibiotics for a UTI. We hope to transfer her to steroids through the gastrostomy tomorrow but will not think about gastrostomy feeding until next week. I just have a feeling that we will be going home on TPN, even if it is night-time only with some gastrostomy feeds during the day.

As we are going to be in for a while, stuck as always in isolation because of her immuosuppressed status and the lovely MRSA colonising her gastrostomy, we have been trying to put some better support in place for her. Daisy has lost a lot of muscle strength since being in hospital so the physiotherapists are going to see her at least a couple of times a week, they brought a floor mat up to her room and it was great to just sit on the floor with her and play row row the boat. The ward teacher has also been in touch with her school so that we can still work on all the things we had started at school. I'm really hoping that they will send someone in even just once a week so that we can continue with her signing - it was coming on so well and she really needs specialist input to help her communication skills. Daisy's receptive skills are great, she understands loads, she has problems communicating however, although I have found she is pretty good and making her feelings felt, most of the ward staff know the sign for "finished" and "no".

The pace of things has slowed down a bit for us for now, but for one of my friends things have picked up. She and her son have been in isolation in another London hospital waiting for a bowel transplant. The call came today and as I write he is halfway through his operation. We met through our hospice and at the time Daisy was possibly going onto TPN, her son has been on TPN for a long time, so she has been a source of info for me, together with our shared adoration of the luvverly Lee Mead (Joseph & his technicolor dreamcoat). She was actually interviewed on the news earlier this week as the issue of organ donation has been very much in the news. We now know two families whose children have benefitted from a donor organ. These children have been given a second chance of life when there was no other hope because a parent in their time of grief gave permission for their child's organs to be donated to help another child live......
It feels like forever since I have blogged, mainly because the rollercoaster ride of life with Daisy was going a bit too fast to take time out and write it all down. I am now the proud owner of a shiny new laptop with wireless access however thanks to a lovely gift from my mum and Andy (combined Christmas & birthday!) so I can try and write things down as they happen.

Daisy's gut problems are extreme, in some areas the inflammation and damage it has caused is aggressive. Unfortunately it is not caused by a virus as we had hoped as this would have meant it could be cured but is autoimmune in origin. Basically her body's immune response overreacts and causes it to attack itself. The treatment for this acute phase is massive doses of steroids and immunosuppressants, weaning down to maintenance doses to try and keep the immune system in check. She is now on 24 hour TPN and the hope is that at some point we can try and reintroduce enteral feeds through her gastrostomy but at this point we don't know if she will ever be off the TPN. Her consultant was very suprised at how severe Daisy's inflammatory bowel disease is, particularly as it is rare to see it in a child so young. I bumped into the lovely Prof Hennekam, her geneticist, while on Rainforest ward, and he was very taken aback, there are no reported cases of children with Costello Syndrome developing this condition and as far as we know the only CS children who have required TPN were undergoing chemotherapy at the time.

We ended up staying at GOS for 2 weeks - possibly the worst 2 weeks of our time with Daisy and not just for clinical reasons. Sadly it really felt to Andy and I that Daisy's clinical needs were being superceded by the need to free up a bed space, they were happy to let our local hospital worry about any side effects with treatment and setting up TPN. As it was we stood our ground, supported by our wonderful team at the local hospital who refused to accept her back until treatment had been started, this turned out to be very fortunate as Daisy threw yet another of her spanners into the works.......

After a couple of days of TPN running through the port Daisy developed some strange heart rhythms, the cardiologist came to see her and arranged for her to have a 24 hour ecg done (she has these done regularly to check that her mild cardiomyopathy has not progressed). Five days after having her port placed someone had the bright idea to do a chest xray to check it was correctly placed. Well, turns out it was not correctly placed, the line was too far down and interfering with her heart function, needless to say she was back on the emergency list for a port revision asap! We felt very vindicated in standing our ground not to have Daisy transferrred prior to starting treatment, particularly given the risks of an incorrectly placed port!

Determined to keep us all on our toes, Daisy then decided to have another UTI, this time e.coli - thanks Daisy! So back onto iv antibiotics.....

There was so much more going on during Daisy's stay on Rainforest and I don't want to give too many details in this blog - Andy and I are in the middle of a letter giving feedback to the Matron of the ward. Great Ormond Street is a fantastic hospital and we are so lucky that Daisy is able to be treated there, many of the people we met were wonderful and with Daisy's anaesthetic risks we would not entrust her to any other hospital. Sadly, however, some areas of her care were so poor, samples going missing, drugs being prescribed without informing us, poor communication between doctors to the point that I was being asked what the plan was for some of her meds, we frequently found that it would take at least half a dozen times of asking for things to be done, such as removing an old canula that no longer worked, meds being administered late....I could go on. I think the worst thing for us above anything else however was that bed management seems to be the key driver over and above the clinical care and needs of the child and family. On the main ward board children were listed by their hospital number (easy to tie up number to patient a their names were written on the door of the cubicle) and next to it an estimated date of departure, it this date was exceeded it was circled in red! We felt that inspite of Daisy's problems and the fact that we had actually been given some really bad news, news that essentially worsened Daisy's prognosis, we were just overstaying our welcome. Seems that with all the pressure on beds some basic humanity was missing. We know from our experience in GOS that this is not the case in all wards, unfortunately senior staff on this ward had clearly made this their priority.

Anyway, enough of the rant, our lovely team back at Queen Mary's did everything possible to get her back and she was eventually transferred back on Sunday morning. It is such a relief to be back "home" everyone has been popping in to say hello to her. We are so much happier she is here, the doctors are in regular phone contact with GOS in terms of her management but we are so fortunate that the nursing team here are so experienced and capable and can manage her TPN needs, I heard from other families in GOS that the main reason they were far from home was that their local hospital could not manage TPN. The reality now is that Daisy will be here for a long, long time. We don't know if the treatment is working, GOS want to give it a good 6 weeks, and we have no idea if she will get off TPN completely. I'm hoping that we may eventually be in a situation where she has TPN overnight and maintenance fluids through her gastrostomy in the day to keep her blood sugars up. With so many other issues going on Daisy's endocrinologist has taken her off Growth Hormone Therapy for the time being, the main reason she was on it was to regulate her blood sugars which are liable to dip low if she is not being fed continuously. We spent a year persuading the exceptional circumstances panel at our primary care trust to fund the therapy, so I really hope she can resume it at some time (although I don't miss giving her the injections).

As Daisy is clearly going to be in long term I can't stay with her every night, but at least we are only 10 minutes drive away. I think I will give her a couple more nights to settle in back here - the pain management team are also going to come out at some point to try and find a better solution to managing the discomfort she experiences at night time when her bowel goes into spasm, if we can get that under control I will be happy leaving her overnight, especially as the staff here know her so well and are very experienced in settling her.

Theo told me yesterday that his only wish for Christmas was to get Daisy home, sadly this is not going to happen, however we were able to tell the children that our hospice, Chase, has arranged for us all to stay there at Christmas - they were over the moon, for them that is even better than Daisy coming home for Christmas. Daisy's birthday is 3 days before Christmas and again with the help of the nurses at Chase we hope to get her out for a few hours. We are so grateful for the support of two fantastic charities; Chase Hospice care for Children and The Rainbow Trust. Both support families with life limited children and get little or no government funding, yet without them Andy and I would not get time out from sitting with Daisy in hospital, I have had a couple of opportunities to take some time out when a carer from one of these charities have come to sit with Daisy, this gave me to do some normal stuff and regroup ready for the next challenge. My best day had to be meeting up with a couple of my friends for lunch in Wimbledon village followed by a free makeover at Space.NK - that was better than a full nights sleep, I caught up on gossip, ate something healthy that was not microwaved and had makeup applied that got rid of the dark circles under my eyes........priceless!

Preparing for the long haul

Andy is doing the night shift at GOS tonight and I really should be doing domestic goddess things like putting the washing away but instead I have snuck into Theo's room while he is asleep with a big glass of wine to check my emails and update my blog on his computer.

So here is the latest:-

Daisy woke up on Monday morning with a 40 degree temperature, not great when you are transferring to GOS for a procedure - anyway we loaded her up with nurofen and calpol and got into the ambulance. Interested to note through the ambulance window that there is another branch of Chariots Roman Spa in Streatham High road, we always look for it as a landmark at Vauxhall Cross on our many trips back and fore to GOS - not sure that conventional spa services are offered given the number of rainbow flags flying from the roof. Anyway, I digress...
We got to Rainforest ward and Daisy went into overdrive which is her usual for a visit to GOS - she lost a canula and then had massive bouts of bloody diarrhea. As with all of my children she does like to draw attention to herself. Daisy losing a canula precipitated something which Ihave never had to do before, have a stand up row with a doctor on a ward. As Daisy has tricky vein access the nurse in charge called the Clinical Response Nurses to come and canulate her, they have been trained to deal with children just like Daisy. So in strolled these two 6 foot, handsome male nurses singing songs from In the Night Garden - both D and I were weak at the knees. Very carefully and considerately they tried to get access to her veins but without success, they wanted to move to plan b, which was to call an anaesthetist. These are the people who can get a line into a vein where there is no vein.... However our dear ward doctor thought he could do better, he swaggered in and without even acknowledging Daisy began to pick up her hands and feet looking for access, Daisy started to scream and the hunky nurses disappeared, outranked by the egotistical doctor. The bottom line was I got assertive (while inwardly quivering) and he then told me that in 19 years in medicine he had never had a failed cannulation - I got the last laugh however, I told him that I did not want a junior doctor canulating my child, turns out he's not a junior, someone somewhere promoted him to registrar, god help us all.................

Joking aside, it's not been easy being back at GOS and persuading the team, most of whom were meeting Daisy for the first time, to make her more of a priority than they currently were. Her test results changed this. After a few delays on the Tuesday Daisy eventually went to theatre for her upper and lower endoscopy. She has had a lot of problems with anaesthetic previously and this had been our biggest concern while taking her to theatre, we just did not think about test results, so many have been inconclusive. Not this one. The consultant who had scoped her was waiting for us in the recovery room to show us the pictures he had taken of Daisy's gastrointestinal tract. Basically from top to bottom she has severe inflammation, ulceration and scarring - the pictures made us wince, her gut is raw...... He took lots of biopsies and blood and currently they are looking at whether this is caused by an increase in her existing gut inflammation, an autoimmune problem where the body attacks itself or the results of a viral infection. The problem is if it is the first two the treatment is to up her dosages of immunosuppressants and steroids, the side effects of which are cancer,something her pre-existing costello syndrome gives her a 17% increased risk of, if it is a virus they need to look at treating it.

These results now meant that Daisy could be prioritised to have a permanent line placed into her veins as there was no way she was going to be able to continue to take nutrition in her stomach. So at 4pm today she went to theatre again to have a portacath placed. This is a permanent line into her jugular vein (or dracula vein as the surgeon so tastefully called it).

All the Gastro consultants and registrars will discuss Daisy's case tomorrow and meet with us midday to discuss plans, we have asked that an oncologist be involved in these conversations given her increased cancer risk and the possible treatment options. We do know that now we have permanent vein access we can start Total Parenteral Nutrition (TPN) which is giving her nutrients through her veins and bypassing the stomach altogether. This will probably happen in the next 24 hours.

Our big hope is that there will be a treatment plan and Daisy will be moved back to our lovely Queen Mary's Hospital for Children for her TPN and treatment.

The evening after Daisy had her scope Andy and I sat in Daisy's cubicle with a big bag of crisps and tried to take it all in. The past four years have been really tough, I was in hospital for 3 weeks before Daisy was born and from the moment she was born we were looking for answers.... time after time tests have come back as inconclusive or within normal limits. Now we have a clear idea of what we are dealing with and the stakes have suddenly gone up.....

I came home tonight to be with the other children - they are so upset that Daisy is still in hospital but Andy and I have always tried to be completely honest with them. Realistically we will not be home from hospital for a long time, the bonus would be to get her closer to home, she may be in for her birthday and christmas.... I tried to get this across to the children and they were particularly amused by my descriptions of Daisy growling at anyone who came near her tonight. She is a fighter, our Daisy Rose..............

Back to Blog...

I did blog for a little while and life (and technology probs) took over, but I feel the need to get back to writing and with so much happening in my life it's going to be the only way for friends to keep up with what's going on chez Nimmo and especially with Daisy.

So, those of you that don't have the latest update here it is in a nutshell - she started school (yippee!!!) and then went back into hospital (boo!!) but came out quickly (yippee again!!!) but went back in v quickly (OK enough of the pantomime!) and has been in hospital ever since. As always it's her gut, technically known as complex GI issues! Where do I begin... basically her whole gastro-intestinal system does not work properly - from her swallow to her bottom. Any infection sends it into shutdown and because it does not work properly this can cause infections (especially wee infections, in medi speak UTI's). It was a severe UTI that caused her whole gut to shut down as never before and spend a week pouring out bile from her gastrostomy, we attempted tiny amounts of feed after a week to challenge the gut but only got to 60mls an hour before she started vomiting again, this has been the pattern for the last month which she has spent in our local hospital - she is currently on about 28mls/hour of diluted feed supplemented by IV fluids. Getting the IVs into her has been fun and she is now on her 16th cannula as a result of very limited venous access.

So this is the plan (and based on previous experience this is where it all goes even more pear shaped):-

On Monday Daisy transfers from our lovely second home at Queen Marys where she is known and loved and cherished but where there is no intensive care and where she cannot be anaesthetised, moving to Great Ormond Street..... don't get me wrong, fabulous hospital, amazing intensive care but Daisy will be an interesting case there and we will have to start from the beginning as they like to do things there way and it always takes a few of her blips to convince the staff that really they should listen to me and her local doctors, we actually do know what we are talking about. It does not help that her main GI consultant is out of the country and will not be back to Great Ormond street (GOSH) until 10 November or that the registrars he has left in charge think Daisy is only going to be with them for 3 days then shipped back to the local hospital. Daisy never does less than a week at GOSH and always needs intensive care after a general anaesthetic so I think that they will be revisiting their GI bed availability come day 3.

Things we need GOSH to do :-

Upper and lower endoscopy
Place a permanent line (eg Portacath/Hickman)
Decide once and for all if she needs Total Parenteral Nutrition or even just paretenteral nutrition - this is where the feed is given directly into the vein (not without a lot of risks but given her gut does not work this may be the ony option)
Possible perform a full thickness biopsy to look at what the constitution of Daisy's gut it
Review all her meds - I think she is on 15 different meds through the course of the day - all I ever do is draw up meds
Give us a plan

The children are getting used to Daisy being away and we are adjusting to another long hospital stint, but really all we want is to be back together again. However Andy and I are happy to have in hospital while there is hope that there could be a plan and a clearer diagnosis - while she has a diagnosis of a syndrome it does not explain her extreme GI issues and before her admission we were tearing our hair out trying to manage things, perhaps it's taken things to come to a head for us to have some answers.......

Now to blogging practicalities - we have had a technology year from hell. Virgin media continues to underwhelm us with their services, the senile cat peed on our very good laptop, our very old but serviceable laptop shuffled off this mortal coil and I am currently reduced to begging Theo for time on the only working computer in the house which happens to be in his bedroom. Andy has treated himself to a very fancy new netbook but we are not allowed a look in, he claims its for work but I saw him playing poker on it the other night... I'm trying to work on a solution so be patient if I don't update as often as I could