Daisy is Home!!!!!!!

Daisy is Home!!!!!!!!!!!

Obviously, given the time which has elapsed between this and the last post you can guess there were a few hitches along the way! We had been working towards coming home on Thursday 16th July and Andy and I had completed our TPN training and had done our first "live" connections and disconnections on Daisy. Very daunting as it really goes against your instinct as a parent to connect your child to a bag of fluids which while keeping her alive could also kill her if you get it wrong. Still, we did it and were all set to come home when out of the blue on Tuesday night she lost the ability to move her legs and was screaming in agony. Now I know that this blog is called "is this in the plan" but recently I have seriously thought about renaming it " well, we didn't see that one coming...."

There are times when I am so grateful that Daisy is already in Great Ormond Street Hospital, and last Tuesday night was certainly one of them as we had top doctors and nurse practitioners gathered around her cot scratching their heads and wondering what had happened to suddenly cause this acute episode. An ultrasound of Daisy's hips revealed she had fluid on both of them, so instead of going home we made yet another trip to theatre and the MRI suite to have her hips looked at and the fluid drained from them. Over the course of the weekend it was apparent that the only thing now keeping us in hospital was the 4 times a day doses of two different antibiotics to treat the infection in her hips....Andy and I were clearly competent in connecting and disconnecting TPN, taking blood, changing dressings, adding spec sets and changing smart sites, drawing up jejunal and gastrostomy meds and connecting enteral giving sets...we were more than happy to take her home on iv antibiotics. Cue visits from a few more hospital teams - infectious diseases, the unfortunate name of the team that look at children that have lots of infections (like Daisy)..Irony of ironies, the doctors from the infectious diseases team was the locum doctor who treated her in February when she nearly died of a line infection, the very man who phoned me at midnight and was treated to the spectacle of me in my Primark Leisure wear! He of course remembered Daisy, as anyone who ever meets her never forgets her, and ordered quite a few immunology tests. Although all of Daisy's recent infections have been from her hickman line, in common with many gastro children she also has a problem of translocation where the bacteria which normally sit in the gut move into the bloodstream, and being immunosuppressed to control her inflammatory bowel disease this is a major problem for Daisy.

We also met the lovely Dr from the Orthopaedic team (Xanthe insists he looks like Gary Lineker but in my myopic state I am reminded of a young George Clooney on ER). Daisy has been under the Orthopaedic Team at our local hospital for a long while now but this new change in events coupled with Daisy's need for Ankle supports and extra tight achilles tendons means that she is well and truly on the radar of this team now and will definitely need botox injections into her achilles tendons, probably surgery to lengthen her tendons and constant monitoring of her hips to make sure the fluid and inflammation does not come back.

Anyway, to cut a long story short, the fluid was drained from Daisy's hips, the physio's got her walking again, we were able to stop the IV antibiotics and around 3pm on Thursday 23rd July, almost exactly 10 months since she first went into hospital our daughter, Daisy Rose came home and our family became complete again......

I could blog about how wonderful the past 24 hours has been doing normal things like pottering around the house, going to the supermarket, cuddling up on the sofa, but the pictures speak for themselves. Needless to say it is difficult, even before this change our lives were diffficult as Daisy had considerable medical needs. These have now gone through the roof in terms of what we have to do and how we have had to adapt our lives but it means that our daughter is home with her brothers and sister and our family at long last is complete again!!!!!

I cannot finish this post without thanking and acknowledging the family and friends who have helped us on the rollercoaster - the secret gardeners who transformed our front drive, the ironing mother and mother-in-law, the friends who took children in at the very last minute, the people who texted, sent messages, called, sent cards, the nurses, doctors, health professionals.... - you all know who you are and we thank you from the bottom of our hearts, we could not have got through these times without you! Anyway, as Andy says, onwards and upwards, here's to the next chapter in our lives with our beautiful children......don't worry, I'll keep blogging, just want to smell the roses for a while......

Yes - we're still in Great Ormond Street but hopefully the end of our hospital life is in sight as we have another possible date for release (prison analogies are so relevant) but I'm not naming it as I just don't want to jinx anything!

Our lives were brightened up by the recent visit of the the lovely Johnny Depp - I still can't believe it was really him (it definitely was) - and also a visit by Dr Linden Cullen from Holby City (or rather the actor who plays him!). Daisy was also well and fit enough to really enjoy some of the benefits of the hospital school including a cookery lesson with a West End Chef where she made delicious blueberry muffins and a strawberry tart, she also had a private music lesson with two musicians form the City of London Symphonia and has been a regular at the truly fantastic Singing Hands sessions. Doesn't this all sound like a holiday camp! Well believe me it's not, these little interludes just help us get through the rubbish bits which recently have included taking a good 24 hours to recover properly following a "short acting" anaesthetic for an upper and lower endoscopy, the trauma of having to have a canula to decrease the risk of her hickman line being infected during the endoscopy, being stuck on 24 hour fluids while trying once again to build up her milk feeds, constant "BM" checks to make sure her blood sugar is stable and vomiting meds from her jejunostomy (this shouldn't happen because this is not even her stomach).

Anyway - that is all behind us, Daisy appears to be relatively stable now, everyone is happy that she will only have 15 mls an hour feed going into her tummy and this is only to maintain her blood sugar, not to provide any useful nutrition, added to this a special carbohydrate powder giving her 20% carbohydrates in this 15mls or 3 teaspoons, specifically to prevent the hypoglycaemia. The growth hormone therapy, while not achieving the metabolic stability we had hoped for (growth hormone has a role to play in helping regulate blood sugar) has at least enabled Daisy to grow to the point that she can now wear a small backpack and carry her feedpump around with her for the 10 hours a day when she is not on TPN - this is absolutely fantastic as it means she is independent for the first time since she learned to walk and can now make decisions about where she wants to go. Today she ran up to Andy and gave him a hug when he walked into the room - this is the first time she has been able to do this without having to wait for someone to move her pump. Daisy has also been getting some physiotherapy at last and now wears splints on her legs and feet to attempt to stretch her achilles tendons which are now so tight that without them she does a fair impression of Darcy Bussell on pointes. The likelihood is that the next stage will be botox injections and casting to try and stretch her achilles so that she can walk with flat feet without her splints and longer term possibly tendon lengthening surgery. All this is fairly common in Costello Syndrome, but as her consultant says having the Costello Syndrome on its own or the Gastro problem on its own would be bad enough, but both together is a real double whammy as she just has to battle with so many issues. Which in true Daisy form she does with a huge smile on her face and massive determination.

TPN training had been put on hold with all of the setbacks but now it is completely back on track and the big day for Andy and I is Monday when we will be watched by all three Nutrition nurses doing a dummy run through of a connection and disconnection of TPN in preparation for doing a "live" connection on Daisy. Just to add to the pressure one of the nurses popped her head around the door this afternoon to remind me that if either of us did anything wrong we would not be able to do a live connection and Daisy's discharge would be delayed. This is worse than any test or exam either of us have ever had to do as so much is at stake - essentially our daughter's life. If we make a mistake when putting up the TPN we could cause a fatal line infection or air embolism which could kill her - the fact that Daisy has already had so many touch and go line infections makes us fully appreciate what a major deal going home on TPN is, you cannot cut corners and you cannot have any lapses in concentration when doing it as it really is a matter of life and death.........

After 10 months going home is really daunting, we have been so used to having nurses and doctors around if we have any concerns, now we will have to dial 999 if we have any worries - the days of me piling Daisy into the car to go to the hospital while Andy stays at home with the children are gone, it's a good thing Daisy is used to ambulances as this will be the emergency transport if she spikes a temperature as it probably is a line infection and we cannot risk her deteriorating on a car journey...

So now the end is in sight the next thing we are having to contend with is the worry of Swine Flu - blimey there's always something isn't there? We can't wrap her up in cotton wool, so we just need to be aware, the plan is that if she has know contact with Swine Flu we have to start Tamiflu immediately, hopefully this will nip it in the bud as the last place she will need to be is hospital - apparantly Queen Mary's is full of Swine Flu cases at the moment so I don't think we will be making any social visits when we get out of hospital!

My plan for the weekend is to swot up on my TPN drill , I know from talking to other home TPN parents here that it will become second nature once we get her home but at the moment, like learning to drive, I am having to think through each move. I also want to go to Patisserie Valerie and order a massive cake with "Welcome Home Daisy" to take home with us, there's a bottle of Bollinger in the fridge waiting at home although I doubt if Andy and will be drinking it, we'll be tossing a coin for who does the first home TPN connection......

When Johnny met Daisy

an unexpected visit from Johnny Depp dressed as Captain Jack Sparrow.....he has strong links with the hospital after they saved his daughter's life a couple of years ago. Obviously he was very taken with the lovely Daisy, particularly when she blew kisses at him although when he walked through the door she signed "where's Peppa Pig?"...
More blog news to follow tomorrow - home for a few hours to unpack the delivery of all the equipment we need to manage TPN at home...

Same old, same old - Daisy has infection, Daisy recovers from infection, gut becomes inflamed again, feeds not tolerated - and on and on and on we go.....next month it will be 10 months since we first were admitted to hospital........

Daisy is clear of the last line infection but her gut is clearly still inflamed so she will definitely have an endoscopy next week and I'm hoping they will be able to get some more biopsies this time to give us some more answers. She is still on IV antibiotics as they are all so twitchy about taking her off them and opening the floodgates for another infection. Her consultant came around today and he did refer to Daisy as a miracle child given all that she has been through, he said the plan is to get her stable enough to get home and then try and work towards - if at all possible- a cure.....yes he said cure so maybe he has something in mind??? One of the last resort treatments for Daisy's gut condition is a Bone Marrow Transplant, maybe he was thinking of that??? The problem is with us parents of chronically sick children, we hang on every word our consultants say looking for a little source of hope, they do not realise how much we churn over the conversations we have had with them, pinning our hopes on maybe just one word...

As always in the plate spinning world that is Daisy there is a problem, and this week it's back to her old problem of blood sugar regulation. One of the reasons she is on 24 hour iv fluids is that she cannot tolerate any time without sugar and we cannot get enough into her tummy to keep her blood sugar up. In fact if there is any delay in her IV being changed her blood sugars drop and she goes "hypo". Fortunately Daisy's endocrinologist also covers this ward so he will try and work out what we can do to try and maintain her blood sugars without this dependency on a constant supply of glucose into her bloodstream. The reason he had originally diagnosed for her problems was insufficient growth hormone, as this plays a role in blood sugar regulation. However Daisy has been having Growth Hormone injections for quite a while now and while she has grown to nearly 90cms the sugar regulation problem remains.

In herself she is really happy and enjoying the benefits of staying at GOS as it is so well geared up for long stay patients - she has a visit every day from a teacher who is actually able to use sign language, and she also goes to Singing Hands sessions and Music Sessions in the hospital school as well as spending time in the activity centre playing with other children. Then there are the visitors to the ward - neither Daisy or I are very enamoured with the Clown Doctors finding them deeply unfunny but we did enjoy a recent visit from the living legend that is Sir Terry Wogan.

So on we plod - Daisy has had a few tests this week, a video fluoroscopy which had to be abandoned as she refluxed up all the contrast fluid used to show up her gut, the outcome of which confirmed what we already knew, her tummy is now redundant and cannot be used at all. She also had a DMSA scan of her kidneys today to check for scarring following lots of urine infections as this may be the cause of her elevated blood pressure. We restarted feeds into her jejunostomy today, slowly, slowly as always at a rate of 5mls an hour, so it will be a while before she is on enough volume to take her off daytime iv fluids. Of course all of this delays us getting home, TPN training has been delayed and now it is getting really close to my deadline of the long school holidays to be home. I am reaching the end of my tether - I don't mind hospital when Daisy is acutely unwell, she is safe there, but as her condition is chronic, Andy and I can manage her at home. I hate that she is referred to as a patient and that we are so institutionalised, I don't enjoy my goldfish bowl existence or this complete lack of privacy, I crave the chance to sit on the sofa, cook food in anything other than a microwave and shut the door to the rest of the world. Most of all I am fed up of our family not being together - I want to be able to help Theo with his homework face to face, not via the phone, I want to make sure that Xanthe is remembering her Irish Dancing shoes on a thursday, not texting Andy to remind her and I don't want to miss any more of Jules growing up....

Snakes & Ladders

A friend of mine in a similar situation once described this life as like a game of snakes and ladders - you climb a ladder and make some progress towards your goal then when you least expect it you hit one of those pesky snakes and back you go again.... Over the past few days we have been climbing some big ladders, Daisy was managing 10 hours off TPN, she was enrolled in the hospital school and loving it, Andy and I were enrolled in TPN training school and feeling one step closer to bringing her home, we had even discussed a date of 24th June to discharge Daisy back home for good - I use the past tense as when we least expected it we hit that great big snake you get just before the finish line, the one that sends you all the way to the bottom of the board again....

Daisy started vomiting on Thursday evening and by Friday evening it was clear that her gut had gone on strike again (technically called pseudo obstruction) just as it had when she was first admitted to hospital all those months ago, her temperature also shot up and blood cultures show that once again she has a line infection. The bottom line is that she has had another flare up of her gut inflammation which has caused her to gut to stop working again, this has meant that we have had to stop anything going into her gastrostomy or jejunostomy. She is back on 24 hour IV fluids/TPN plus IV antibiotic infusions and her immunosuppressants have also been switched to IVs. Her consultant came to see her today and confirmed what we already knew, Daisy will be on TPN for the rest of her life. The plan now is to try and manage the inflammation again and then she will have another scope (where a camera looks at her gut to see what is going on), she may have to go onto even stronger immunosuppressants which would definitely only be IV infusions and at this stage we really don't know if getting back onto feeds into her jejenum (top of her intestine) is a possibility. All we can do is hope that in the same way Daisy has suprised us with the sudden turn of events she will suprise us by picking up and getting back to where we were before.

As always we hope to save her hickman line but the infection is pretty bad as it is also around the entry site in her skin as well as in the line. It's just a waiting game to see what the next few weeks bring. Andy and I are continuing our TPN training and all the equipment we need is being delivered to the house so at least we will have everything in place although it is looking likely that the hospital will have to bend their own rules and allow Daisy home with minimum breaks from the TPN.

The rest of the children are really disappointed - I was supposed to have brought Daisy home for the day on Saturday to have a belated birthday party for me (it was my birthday on the 10th) - instead I came home on my own and then spent most of the day catching up on sleep as I had spent the previous night up with Daisy.

I guess I was a bit taken aback when the hospital were talking about a discharge date of the 24th - always in my mind I had imagined that Daisy would get home to coincide with the rest of the children breaking up for the end of term so perhaps if I keep that in mind then I won't be disappointed and we really will get home in time for the summer holidays.....

So we got Daisy over her latest line infection and managed to save the line and life was ticking along, with the big focus on her next admission to Great Ormond Street. I had a call early last week saying that we were booked into a 5 day bed for her to have two procedures on two separate days under general anaesthetic. In complete frustration at the slow progress in moving Daisy from 6 hours off TPN to a regime where she would be able to come home I phoned the nutrition nurses at Great Ormond Street and basically said that Daisy, all of us, needed a life and we need to find a way to get her out of hospital and home.

Cue a second phone call from Great Ormond Street late last week, can we bring Daisy in that day, in fact asap, as a long stay bed had become available. Sometimes you have to be careful with what you wish for – one minute I was plodding along enjoying the few hours freedom in the day with Daisy and gearing up for a short term stay at GOS , leaving hopefully with a date to start TPN training and bringing her home, next minute I am frantically rushing around, phoning friends to pick up the children, ironing clothes and sorting out the million and one things that need to be sorted out when you have a family of 6 so that Daisy and I could transfer from Queen Mary’s to GOS quickly enough not to lose the bed. We arrived last Thursday at 8pm, not really knowing why we were there…

The first thing that needed to be sorted was another blood transfusion. Daisy has been chronically anaemic for a long time now and while she copes well with a haemoglobin level well below target levels every now and then this drops very low and she needs a transfusion. The levels of immunosuppressants are currently low in her body and the doses are needing constant tweaking and changing. The most exciting thing is that following my call to the nutrition nurses Daisy became the priority for home tpn training and set up and we arrived her to find there was actually a plan to get her home!

For the past few days we have been weaning down the hours Daisy spends on TPN – it’s early days yet but so far so good as she needs to be 8-10 hours a day off TPN to be allowed to go home on TPN. The dietitian has been keen to try and increase the amount of feed that is going into Daisy’s jejunostomy, however sometimes an increase of even a couple of mls and hour can upset the applecart with Daisy and for the past two nights she has vomited so we may not be able to push these feeds too much. However she is now allowed a two hours break from her milk feed pump which means for two whole hours a day she is completely free of tubes and wires and skips around like a little spring lamb – it’s the best sight in the world!!!!

The plan for the week coming is for Andy and I to start TPN training, fitting this around the two general anaesthetics that Daisy needs this week plus any thing else she throws our way (another transfusion is a possibility). Given that this is the first week of Andy leaving his old company on a redundancy package and starting up his new business this is a huge commitment on our part as he has had to completely rejuggle everything to fit this in. We have also had to completely rejuggle the children’s schedules to take into account Andy and I both spending all day at the hospital. But to get Daisy home this has got to be worth it.

So as I said at the beginning, be careful what you wish for.........I am so excited at the prospect of having all my family under one roof, but at the same so daunted, not by having to do the tpn, we will do whatever it takes to give Daisy quality of life and both Andy and I are dab hands at changing gastrostomy buttons, checking blood sugars, placing ng tubes....It's more the fact that for the past 9+ months we have been cocooned in this world of hospitals, desparate to get out and reclaim our role as parents, and reclaim our daughter. Now this is about to happen we realise how much we have depended on nurses and doctors being round us all the time, providing us with a security blanket, now it's over to us. I guess the thing I am dreading more than anything is night-time. Children with Costello Syndrome are notoriously bad sleepers, add into that reflux, tummy pains, beeping and leaking pumps, itchiness from excema, oversensitivity to noise and you get the recipe for broken nights. Theoretically we are entitled to some sort of night respite package but whether that will be forthcoming in the short term is another matter. And then there's the twitchiness about line infections and illness - we have seen Daisy go dangerously downhill very very quickly, this makes you very overprotective as parents and it will take me a while to feel safe without the comfort of a resus trolley outside her door and a picu down the corridor. The children will have to adjust too, they will get a full time sister back and parents who need to focus on her needs while in the house. The time we need to put TPN up is the worst time of the evening for parents - 6pm -7pm, right in the middle of tea time, activities, homework, bath time - our already very self sufficient children will have to become even more so. It will be so good to get back to being a full time mum to all of them, we are realistic in knowing that we will still get stays in hospital but nothing should ever be as long as this stay, who knows, maybe next year I can even get back to work!!!!!! (those who know me well will know this is only half a joke, watch out, maybe I will sign up for med school!)

I guess I blogged too soon......at 6am this morning I had a call from Chase to say that Daisy's temp had spiked to 41.5 and they had called an ambulance to transfer her to the nearest hospital. I woke the children and delivered them to my wonderful friend Maria (thank you so much Maria!!!) and bombed down the A3 to the Royal Surrey Hospital in Guildford.

In true Daisy style she was still smiling, however her blood pressure was unstable and she needed one of her favourite IV fluid boluses to stabilise her. Blood cultures have been taken but we are working on the assumption she once again has bacterial sepsis caused by a line infection. Antibiotics have been started (2 weeks and 1 day since the last lot were finished - a record!) and by late afternoon she was stable enough to be transferred back to our wonderful Queen Mary's Hospital.

As always it's two steps forward and one step back and at least this time she did not need to go into Paediatric Intensive Care....and she did get to go to Chase at last, though leaving by blue light ambulance was not quite in the plan....

We achieved another milestone yesterday. Since Christmas every time we have tried to get another break at our hospice our plans have been scuppered at the last minute, mainly by Daisy getting an infection and becoming very poorly. Well we managed it at last, and I took Daisy to Christophers, the Chase Hospice in Guildford, yesterday afternoon. I did not want to jinx anything so did not pack for her until the very last minute and there was a little glitch when she developed four strange new spots on her face (which the doctors think are some sort of allergy reaction).

She is there for two nights until Thursday lunchtime - theoretically this should give me a break but the reality is I have had a day of taking rubbish to the dump, sorting out the children's clothes, tidying cupboards and taking bags to the charity shop. I know to some people this will not be the ideal day off but for me it is a chance to start gaining some control back, over the past few months (and years) we have lived off our nerves, ultimately Daisy's condition is incurable and the uncertainty of what will be around the corner is always going to be there but at least we have learned to go with the flow of it now. This way of life is the norm for us now so rather than putting off sorting out the house until things get better I have decided to deal with things now. Things have not got worse and that's the best way to look with things. so rather than putting off all those house tidying projects I'm going to tackle them now....I have black bags at the ready and I'm braced to sort out the children's wardrobes once and for all!

I did speak to the nurses looking after Daisy this evening and she has a low grade fever so I'm keeping everything crossed this does not materialise into something more sinister. As always I sleep with my mobile and the home phone next to my bed so will not be suprised if I do get a call in the early hours of the morning. It will be a bit of logistical nightmare if the hospice do call to say that Daisy needs to be back in hospital however as Andy is in Manchester tonight so I am on my own with the three older children. We are blessed with the most wonderful friends and I know I can phone any number of good friends in the early hours if needs be to sit with the children if I do need to drive to Guildford.

Andy is in Manchester for a couple of business development meetings for his new business. He officially leaves his company on a redundancy package next week and we both agree that this is the best thing that could happen to us. He was looking increasingly tired and stressed and could not have continued at the pace that he was working, it is so nice to see him looking relaxed and even losing a few pounds in weight as a result of being made redundant! The one thing Daisy has taught us is to really put things in perspective - the most important thing is family time and creating happy memories. On Friday Andy will be able to attend one of Xanthe's school assemblies for the first time in years, that is worth all the bonuses in the world!