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
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 ....
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!!!!!
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...
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....
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......
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...
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....
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.....
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!)
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....
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!
We are trying to take Daisy to school for 2 -3 afternoons a week to coincide with her breaks from TPN - I stay with her for the session as she is still so medically fragile but as you can see from the picture she is so happy to be back, even though all she can do is sniff and lick the crisps on her plate! (you can see the line to her enteral (milk) feed pump at the back of the chair - my role at school is basically to follow her around with the backpack containing the pump - from Global Brand Manager to official bag carrier....)
Daisy amazes me at how adaptable she is, she was so excited to come home and now takes it in her stride and is not even upset when it is time to go back to the hospital (at 5.30) - she even gets excited seeing her room in the hospital again as it is also such a part of her life. I'm so relieved that Daisy has transitioned into this new stage so easily, the night before she was due to come home for the first time I lay in bed worrying - for nearly 8 months our lives have revolved around Daisy being in hospital and we have adapted our home life to fit with this. Would it feel strange having her around the house again? Of course, I needn't have worried, from the moment she arrived home the traumas and worries of the past few months melted away. Lots of people have commented how the other children look so much happier too, they love to come home and play with Daisy and we have settled into a routine of sorts. The TPN can be taken down at 12pm and goes back up again at 6pm, it's amazing how much we can fit in during these short hours. One of the real bonuses is that I have been able to take Daisy back to school for a few sessions, it's a safe environment for Daisy infection wise as there are only 2 other children in her class, she also gets to spend time with the class next door which only has around 6 children. Not suprisingly we have seen huge leaps in Daisy's development since this new phase has begun, she is more vocal, is getting stronger on her feet and her sign language is racing ahead.
One of the unexpected problems has been a massive increase in Daisy's summer allergies, having been confined to a room for so long to suddenly be released to a pollen filled outdoors has meant that her nose is constantly streaming and any effort leaves her wheezing. Hopefully this is a small price to pay for a taste of life in the real world!
The reality is however that Daisy is still attached to a pump 24 hours a day. From a few months of age she has been feed her prescription milk feed via a pump into her tummy for 20+ hours a day. In order to maintain her blood sugars while off TPN and continue to stimulate her dodgy gastrointestinal system Daisy is attached to her feed pump all the time, running at a fantastic rate of 35 mls an hour - the highest she has managed since last summer. The feed all goes into her new jejunal tube at the top of her intestines, we know that not all of it is absorbed and she still has really runny nappies but this too is a step forward. The truth is though that being attached to a pump all the time is limiting her new freedom, I would love to see her make her own way around the classroom without an adult walking behind with her backpack containing the pump. She is too small and weak yet to carry the backpack so for the timebeing she gets freedom, at a price...
The next milestone would be to have a complete fortnight off iv antibiotics without any more line/blood infections. Next week she will hopefully reach this point so fingers crossed! We are waiting for the funding panel at the hospital to approve the use of specialist line locks which we can put into Daisy's Hickman line and decrease the risk of line infections. In fact waiting for funding panels to approve various things is going to be the common theme for us now. Despite our excitement at the thought of getting a mobile TPN pump following our last GOS visit, this has been stalled a bit by the fact that in todays wonderful NHS, devolved budgets means that no-one actually wants to pay for anything as it is always someone else's responsibility... So in the meantime we are still waiting for some appropriate funding panel to approve the mobile TPN pump which would ultimately mean that Daisy could have even more time at home or school and improve her quality of life even more. Another example of this crazy situation is Daisy's enteral pump which we use to give her milk feeds. She has had a mobile enteral pump for many years but while in hospital tends to use the hospital feed pumps. As we are now out and about for a few hours we use her mobile pump reverting the the hospital pump for overnight feeds. When I tried to order a box of giving sets (the tubes which attach to Daisy's tummy) which we use with the mobile pump I ran into a situation worthy of a Monty Python sketch - as Daisy is in hospital we cannot order equipment (ie giving sets) for use at home, but the only way she can be mobile at home is to use the mobile pump -again this is all down to who owns the budget for what. Much as I love our wonderful NHS, Daisy is testing their systems to their limits!
A while ago, in the early days when Daisy was still tiny I would have got angry and thrown my toys out of the pram at this situation but the years, and particularly the last 8 months have taught me patience and perspective. We are inching towards the day when we don't have to put her back into the car at 5.30 to go back to the hospital, and when the juggling of children, activities and Daisy will be focused around the home a bit more so these bureaucrats and their inconveniences are quite low on my list of priorities - there is always a solution as long as we stay focused on the goal. And in our case the goal is to be home for the summer.
The next day she was very subdued and I had a sneaky suspicion something was not quite right, this was confirmed later that afternoon when she fell asleep, something she only does during the day if she is ill, and woke up with a 40.2 temperature. It was actually one of the few times I just felt like bursting into tears then and there - not another line infection, and only 36 hours since the new one had been placed. She also developed copious runny nappies so I was also panicking about Cdiff, which (of course) Daisy has already had! Well thankfully she does not have Cdiff although the runny nappies are still continuing and look more like her gut deciding it's not going to work (not that it actually works anyway) but she does have a line infection and her gastrostomy site is colonised with staff aureus. Now having managed against all the odds to clear her of MRSA (methycillin resistant staff aureus) I don't want these little bugs to go and mutate into it so we are trying to be really rigorous with infection control procedures - not that we weren't anyway, it's just we all know what Daisy can do. So to cut a long story short she is back on IV antibiotics . This is such a familiar patter, she had only been off antibiotics for 8 days before the new infection, we have not managed a fortnight off antibiotics since the summer, that would be a real milestone!
We are, in Daisy fashion, making progress - ie; two steps forward and one step back but at least making some progress. The exciting news is that we are now on the radar of the Nutrition Nurse Specialists at GOS. To be able to go home we have to be on their radar as they organise the home TPN package, they also insist on children being a minimum of 10 hours off TPN a day(they have moved the goalposts, I had heard it was 8 hours!). The best thing is they agreed with our need to get Daisy out of the hospital and getting some sort of life outside of her isolation room as 7 months in isolation is not conducive to neurodevelopment and probably some sort of crime against her basic human rights! So we have actually left GOS this time with a plan - both to start giving Daisy breaks from TPN so that she can get outside and to try and get a mobile pump for times when she is on TPN.
The former is probably easier to achieve than the latter. This week, we implemented the TPN weaning plan with the aim being to get her to tolerating 6 hour breaks off the TPN. She still will be permanently attached to her other pump which drips milk into her jejunostomy, but that has been a permanent fixture 24/7 since she was just a few months old (and I promise never to complain about being tied to a pump again knowing now what the alternative is!). As of today she is tolerating four hours off her TPN and her blood sugars, though on the low side are reasonably stable, I am just hoping that we can achieve 6 hours by next week as I would love to be able to take her back to school for a couple of hours each day. I am also tentatively hoping we can bring Daisy home for a few hours this weekend - I just hope nothing scuppers this plan as I mentioned the possibility to the children and they are so excited at the thought.
Getting a mobile TPN pump is, as always, a lot more complicated than it should be. The plan is to start Daisy on the home delivery service while still in hospital - this means that her TPN would be made up by a pharmacy and 2 weeks supply delivered to her at a time to be used in conjunction with a mobile pump. This will work fine when we are at home but having a child still in hospital accessing a home delivery service is proving a bit difficult for the good old NHS to get it's head around. The issue as always is who pays for it. It Daisy was at home it would be the Primary Care Trust (PCT) but she is in hospital and so they insist that the Hospital Trust should pay, as the delivery is to a hospital and not home the delivery company say that VAT is applicable on the TPN and anyway the hospital trust are saying they have their own TPN pharmacy and so will not pay for something they can provide (albeit without a mobile pump), as Daisy is not currently an inpatient at Great Ormond Street they are not going to pay so we have reached a stalemate until someone out of the three trusts sees sense. I'm just focussing on getting enough time off TPN for Daisy to have some time out, if these silly conversations are still happening by this time next month I may consider a major toys out of pram episode but you never know, maybe it will be all sorted!
The other useful tip we got from the nutrition nurse was the use of line locks to help stop infection. Basically when Daisy's hickman line is not being used we can put a drug called Taurolock into the line to kill any bugs which may be pushed into her blood stream when the line is used again. Apparantly since using this drug they have seen a massive drop in line infections. The pharmacists are on the case and I hope we can start using it soon as we cannot risk any more line infections or losing any more lines.
Anyway, inspite of all the continuing issues, it's fantastic to be home, sleeping in my own bed and spending quality time as a family. The children have been so pleased that Daisy is back close to home - both Jules and Xanthe wrote about it in their school diaries. They are definitely more relaxed now we are out of GOS as they know that the only reason we are ever in that hospital is when things are not so good. I'm just really hoping we get to bring Daisy home for a few hours this weekend and do normal things like sit on the sofa and watch TV, these are things that we all take for granted but when your family is torn apart for so long like ours it's the little things that are so important.
The good news is that she is so much better than a week ago, her additional potassium is being weaned down and she is off all antibiotics and anti-fungals - this makes me twitchy, she does not last long without needing some sort of weapon of mass destruction coursing through her veins - I will remain positive however, maybe this time the line will last....
The plan now is to take out her picc line and put in a new Hickman line early next week, theoretically then we should be good to go although we will let Miss Daisy decide on that. It has been nice to have Daisy reasonably well over the past few days as we have been able to use the fantastic play facilities at Great Ormond Street. The best thing is the other children have also been able to use the play centre too which has taken some of the pressure off entertaining them. Today Daisy decorated fairy cakes with a group of other children including a young boy on a "Berlin Heart" machine, which is an artificial heart. Great Ormond Street is probably one of the few places in the UK where a little girl being kept alive by a complex mix of chemicals dripped into a central line into her blood stream can play with a boy being kept alive with an artificial heart and no-one bats an eyelid!
I had a good chat with the gastro dietitian today. Understandibly they are very twitchy about pushing Daisy too quickly particularly given her recent experiences. For Daisy to be able to have night time only on TPN she would need to tolerate 34mls an hour of feed a day, with possibly more fluid during hot weather because of her heart and kidney problems. This currently seems a long way off so we are aiming for 2/3 time on TPN which would make us eligible to come home as the minimum that is allowed is 8 hours off TPN. At the moment she is managing 14mls/hour of feed into her tummy over 24 hours but it is difficult to push this any more at the moment as she will need to be starved again for another general anaesthetic next week when her new Hickman line is put in, and we will be back at square one as Daisy cannot go straight back onto full feeds after being starved and will need to build up ml by ml. As always, two steps forward and one step back, however I am optimistic that with the new feeding tube directly into her intestines we can try and aim for our 8 hours off TPN and get home (at some point this year!)
I have now given up on the bed in Daisy's room - basically she is going to wake up and cry whether I am there or not and I need a decent night's sleep to keep functioning, whereas the night staff can go home at the end of their shift, I cannot. For the past few nights I have left the hospital at 8.30pm and slept at home leaving for the hospital at 7.30am the next day. I am looking forward to being back at our local hospital, hopefully next week, it is only 12 minutes drive away and suddenly that life seems so much less complicated than a Northern Line commute to Great Ormond Street. It's over to you now Daisy, show us that you can turn the corner and get some time off that TPN. I'll do all the training I need and put in as many hours as required in the hospital just to get her home.
So last Monday she was scheduled to have her infected Hickman line taken out following a weekend of high temps and lethargy. Unfortunately and in a bizarre way our old nemesis of MRSA came back to haunt us. Daisy is still categorised as MRSA+ in this hospital inspite of all the clear swabs and cultures, the infection control rules here state that the child must be out of hospital for 6 months for this category to be lifted. No-one believes for one minute that Daisy is still colonised with MRSA but unfortunately while she has this against her name she has to be isolated and when it comes to surgery she has to have the last theatre slot so that the theatre can have a "level 2 clean". This situation was becoming farcical, as Daisy was last on the list , the interventional radiologists did not have time to take her line out and her condition was rapidly worsening, her peripherals were shutting down and she needed fluid rescuscitation....cue another trip to the Patient Advice and Liaison Team (Pals) for me and strongly worded emails to infection control from Daisy's medical team. Thank goodness everyone saw sense and her MRSA+ status was lifted and she was scheduled for the next day, second on the list. Not a moment too soon as she was really becoming poorly and also needed a blood transfusion as her haemoglobin had dropped. So that all happened on Tuesday - infected Hickman line whipped out, temporary picc line placed and a blood transfusion given
That should really have been the end of it, and in some ways it was, her temperature came down, but normally a blood transfusion would give Daisy more energy and colour and in this case it didn't. She looked grey and lethargic the next day and her blood gas results showed that her her potassium was dangerously low and her sodium was very high. She was started on extra fluids but we have not been able to bring her potassium levels up. Some of the meds she is on lower potassium and some of them are damaging to the kidneys which play a role in how potassium is used in the body. The past week has been a cycle of taking bloods, running a blood gas, finding her potassium is too low, putting up massive potassium infusions, checking her bloods again....... This is risky, too little potassium can cause the heart to stop, Daisy has cardiomyopathy anyway so this is extra worrying, too much potassium can kill you and we have been pumping maximum doses into her. She has been permanently wired up to an ecg monitor and the doctors have been walking around with worried looks on their faces. One of the meds which suppresses the potassium has been stopped and her immunosuppressant iv infusions have been stopped as they can interfere with the kidneys. This has given us another conundrum - stopping these meds will hopefully help Daisy rebuild her supply of potassium and hold onto it, but not having the immunosuppressants may cause the inflammation to flare up taking us right back to square one and undoing all the progress of the last few months.....
This plate spinning is too much for me, I'm leaving it to her big cheese consultant to work out. He is the expert and Andy and I have confidence that he will do the best thing for Daisy, always aware that our number one priority is to get her home at some point. At his point however we begin our third week at Great Ormond Street, today we had the first potassium level which was in normal limits, in my new found understated manner I did not jump for joy as I need to see a few of these results before being convinced that she is holding onto her potassium and turning the corner. The levels that are required for her immunosuppressants to work are now too low and so a decision needs to be made very soon about what to do next before her gut flares up. Feeds through her jejunostomy seem a long way off at this point but this is the only solution and at some point very soon we need to try again as this is the only way to reduce the reliance on TPN and the vicious cycle of infection and damage it brings...
I'm waiting for someone in the street to say to me "cheer up love, might never happen" then I can floor them with our story and add the additional icing on the cake that Andy was told on Friday that his role is redundant and he will have to apply of another role within the company or accept redundancy, still, it could be worse I suppose, we have just got through another week where Daisy put the frighteners on us and we have lived to tell the tell.
I always give up alcohol and chocolate for Lent, I am particularly proud that I have managed to see it through this year because quite frankly, come the end of lent I am going to enjoy the biggest chocolate and red wine hangover known to mankind!
Daisy has developed another bacteria infection of the blood ontop of the candida in her bloodstream and urine infection. She is now on antibiotics I have never heard of and apparantly starting to move into last resort categories as the bug she has developed here is one she has had before and seems to be resistant to some of the usual antibiotics she is on. Her hickman line clearly has to come out and this is now scheduled for Monday with insertion of a temporary picc line to give her chance to clear her infection. Regular readers will know we have not had good success with previous picc lines, they should last a few months (they are like semi- permanent lines) but Daisy's only lasted a week resulting in another blue light dash to GOS for a new line. I am wondering if they will keep her here now until the infection clears and they can put a new Hickman line in . This will really test the new Rainforest happy attitude - previously I would have heard mutterings about needing the bed space but instead the staff have been fantastic and really concerned just for Daisy's well being and the fact that things have not gone according to plan for us.
Logistically things will get difficult if we stay any later than the middle of next week. Mum goes back to Wales tomorrow and Andy and I will juggle staying at the hospital and managing the school run - probably Andy will stay here for nights and I will commute back and forth during the days with the children at after school club and at friends houses...
There has been good news though. Daisy had her upper and lower endoscopy on Thursday and the inflammation has decreased which means that the immunosuppressants and steroids are working - yippeee!! Her doctor wants to keep her on this therapy for the forseeable future so her risk of infection will still be as high. He also placed a jejenal tube for feeding Daisy at the top of her intestine, bypassing her stomach altogether. We are hoping that we can increase some of her feeds using this method and get more calories into her rather than just via her TPN. The endocrine doctors also feel that Daisy should be able maintain her blood sugars for small breaks from the TPN with just milk feeds. The plan, once Daisy is over this infection, is to try and achieve longer and longer breaks from the TPN with the aim of 8 hours a day off. She will still be attached to a continuous feed pump for milk feed but this was Daisy's life before TPN and I promise I will never complain about carrying a pump around again having seen the alternative!!!
The home word still has not been mentioned - we need to achieve these breaks from the TPN before this can happen so the reality still is June/July. Being superwoman I am already contingency planning for Daisy being in hospital during the long school holidays although it would be nice for her to have long enough breaks from TPN to be able to come home for the best part of the day and go back to the hospital at night.
Andy stayed at the hospital last night and I was able to get home overnight, fitting in a quick meal out with some friends and a chance to catch up on some gossip - these moments keep me sane and de-institutionalise me for a few hours! It was great to wash my hair, the shower on Rainforest still runs on lukewarm and you have to brace yourself to get into it so hairwashing is out of the question. The best bit was sleeping in my own bed - luxury! Did a quick Primark shop with Xanthe in the morning to pick up dungarees for Daisy as they are great at holding Hickman lines and enteral tubes in place, then we all drove up to GOS. Fortunately siblings can go to the Activity Centre here on the weekend so while mum sat with Daisy Andy and I were able to have a quick bite to eat and a coffee together, a rare treat these days. The combination of a sick child, 3 hyperactive siblings plus tired parents/grandma is not a great one so Andy has now driven everyone home and I am back to my hospital bed and prison regime!
Xanthe is singing at the Albert Hall on Monday evening and we have been promised that Daisy is first on the afternoon theatre list so if our plan works out of Daisy's respite nurse sitting with her for the evening and various children being delivered to the Albert Hall we may just get to all be able to go. As we know, the best laid plans........
I am relieved that Daisy is at GOS at the moment, if we need it paediatric intensive care is just down the corridor, hopefully she won't need it, but as always her happy smiles really don't show how ill she is. But inspite of it all we are making progress, and slowly, very slowly, we are inching towards the day when we can come home.
Of course everyone who knows Daisy well knows this pattern, we plan something, in this case, Andy going for a weekend to France with one of the children or we have another inpatient stay at Great Ormond Street booked and you can guarantee that Daisy will throw a spanner in the works. Of course this week it was a double whammy, weekend plans and inpatient stay so Daisy threw a double infection - candida in her bloodstream (you will remember from previous posts this is bad, bad, bad!) and a really bad urine infection , and for good measure streptococcus was isolated in her gastrostomy site. The bottom line is the Hickman Line (no pun intended) will have to be removed, candida sticks and you do not want to risk it creating fungal balls which settle in the brain, heart, lungs, kidneys - as I said, this one is bad.
She is smiling and gorgeous as ever which really belies her state - the infection is systemic, ie everywhere and requiring big doses of anti-fungal infusions which have really nasty side effects. On top of that she is back on antibiotics for the urine infection. These drugs together with the steroids and immunosuppressants do not make for a good combinations so her kidneys and liver are being monitored constantly. The only good thing is that we are going into GOS tomorrow so hopefully they can fit in a line change then, if the ward doctors can get their act together, I'm just keeping everything crossed that the endoscopy and jejunostomy can still go ahead as without these two procedures we will not know if we can stop the immunosuppressants and try and increase her feeds to get her time off the TPN. And time off the TPN means we can become eligible for the HPN programme - Home TPN and maybe a plan and a timeframe for coming home....
So many things keep moving and changing, many not under our control, the stress is starting to get to us all. Thank goodness Andy had a chance to get away and recharge his batteries, I do not want to be away from Daisy so knowing that at least one of us is getting a break is a comfort. I am getting fed up of lots of things about our institutionalised life however and have started to compile a list, many of which will be familiar with friends who read this blog:-
In no particular order...
- having to ask permission to do things, eg like getting Daisy's growth hormone out of the fridge
-not being able to take my cup of tea into her room
-dragging a huge IV pole with me and holding 3 lines to make sure they do not go on the floor everywhere we go
-trying to keep 3 other children entertained while spending time with Daisy after school
-not being able to spend any time with Andy, particularly on the weekends when one or other of us is at the hospital and the other is at home with the 3 children
-having to leave Daisy at night
-not being able to have her home and to be together as a family
-living our lives in a goldfish bowl with no chance of any privacy
Anyway, whinge over. I am hoping that this week we may leave GOS with a bit of a plan and way forward. The longer Daisy is in hospital the more susceptible she is going to be to infections, but she cannot come out until she is stable. We have to go at her pace but at the same time I want the doctors to remember that she is a 4 year old girl with a life to live and she cannot continue to live it confined to a cubicle in hospital. If her life is going to be short then I want it to be full of memories of fun with her family at home....
Websites close to my heart
- Shooting Star Chase (Daisy's Hospice)
- Great Ormond Street Hospital Children's Charity
- Torbay Holiday Helper's Network
- Sick Children's Trust
- Daisy's Angels - Facebook Page
- About Costello Syndrome
- Asperger Syndrome
- Caudwell Children
- Together for Short Lives (Children's Hospices in the UK)
- Costello Syndrome Support Group
- Give Kids The World
- PostPals - Putting Smiles on Sick Children's Faces
- Singing Hands (Daisy is their No1 Fan!)