Our Pal - Daisy N

Story written 2009

Daisy was born 3 days before Christmas in 2004 and was 2 months early. She spent 8 weeks in the neonatal unit and was diagnosed with Costello Syndrome. She spent most of her first year in hospital, both locally and in Great Ormond Street, and was diagnosed with a number of issues including a heart defect, visual impairment, and severe gastrointestinal problems. Children with Costello Syndrome are at a 17% increase risk of developing malignant tumours and she is screened for these every 3 months.

In spite of her diagnosis of Costello Syndrome, Daisy’s clinical issues have not always been typical of a Costello Child, and her geneticists are now testing her blood to see if she also has another syndrome running in parallel and making her completely unique.

Her struggles with her gastrointestinal system came to a crisis earlier this year- she has always been tube fed and normally spends around 20 hours a day attached to a feed pump. However, in September, her symptoms worsened and her Doctors at Great Ormond Street discovered she has aggressive pan-enteric colitis. This is where the entire gastrointestinal system is scarred and ulcerated. It has been caused by her immune system going haywire and attacking her gut.

Since this diagnosis, Daisy has been dependent on Total Parenteral Nutrition (nutrition through a vein) for all her nutritional needs. The initial treatment to manage her condition with massive amounts of steroids has not worked and she is now on large amounts of immunosuppressants. As her immune system is not functioning properly, she is at high risk of infection and is currently on large doses of highly toxic antibiotics to manage a blood infection.

She has been in hospital for the past 6 months and is likely to remain here (with regular visits to Great Ormond Street) at least until early summer.

The diagnosis of Costello Syndrome meant Daisy had a reduced life expectancy because of the associated risks and problems. Her severe gastrointestinal disease and autoimmune issues have reduced that prognosis further.

Every day with Daisy is a blessing, she is a much loved and treasured daughter and younger sister to her 3 other siblings.

She faces each challenge with positivity and a fighting spirit and her strength of character and happy nature keep us going through the tough times.

Update March 2013

Thank you to everyone who has sent amazing post and gifts to Daisy recently – she is truly a very lucky girl!

However, Daisy has decided to take a little break from Post Pals so that other children can share in the smiles – she asks that you send post to those pals currently in hospital or on chemo and also the memorial siblings. Her special friends are Poppy H, Lydia M and Nico R (brother of Alicia R memorial Pal) – so if you were planning post for Daisy, please maybe think about these friends. Thank you xxxx

Update 20th October 2012

Sadly as I write this Daisy is facing the prospect of yet another major surgery, she has been having more and more problems with her bladder over the summer and while she was in hospital this summer having her hickman line changed (as the result of a break and infection), her urology surgeon had a good look at her mitrofanoff stoma which we use to catheterise her bladder. It has prolapsed but that in itself does not explain all the pain so he has decided to operate to have a better look and repair the prolapse. Daisy also had another admission this summer for endoscopies – these show continuing inflammation in what remains of her small bowel. The small segment of large bowel that remains, her rectum, is severely inflamed and bleeds almost daily – as a result her surgeons have decided to perform a rectal excision.

She will therefore return to Great Ormond Street on November 9th for a major 5 hour surgery to take down and repair her mitrofanoff, remove her rectum and deal with any other issues they find – as always Daisy’s recovery time will be up to her and it may be weeks or months, but we will do our utmost to get her home as soon as she is off any intravenous pain relief. She really does not want to go into hospital again but her quality of life has deteriorated quite considerably over the past few weeks and she is becoming very exhausted and finding it difficult to manage the pain in school, often becoming angry and cross with her classmates which is so unusual for her, she also spends much of the weekend catching up on sleep and in her bed or wheelchair. The palliative team have pushed up her pain management regimen to tide her over until surgery.

If this wasn’t enough, we also begin long overdue investigations on the very real possibility that Daisy has occult spina bifida and a tethered spinal cord. If this does prove to be the case (it is known that with Costello Syndrome that this can occur) she will also need surgery on her spine to prevent further damage to her nerves. Sadly any damage that has occurred is now irreversible. Life is stressful and our other children find it very difficult to see Daisy in so much pain, particularly her big brother, Theo, who has a diagnosis of Asperger’s syndrome and is in his very crucial GCSE year in school.

Despite everything though we have had a good year so far, maximising time away from hospital by managing as much of her care at home as possible. We had a fantastic time at the Olympics and Paralympics and thanks to a new dry suit, generously funded by a charity, Daisy has been able to go swimming both at our hospice and at school without the risk of her stoma bag leaking, catheter coming out, or water getting into her hickman line.

Her regular post from Pals makes her day, especially our good friends Glad, Kim, Jenny & Kate and surprises like balloons and cards from Viks at Post Pals really do make Daisy’s day – she loves to get post and takes great pleasure in opening it up and looking at pictures on cards. We have had a lot of problems with our post office recently and despite arranging redeliveries these have on occasion not happened and parcels have been sent back, also sometimes post is delivered to the wrong house and it can be weeks before it gets to us – apologies if you are one of those people who has sent Daisy post which was returned, it’s out of our hands at the moment but we are working on a different c/o address which is a bit more reliable! In the meantime, to each and every one of you who writes to Daisy, thank you, our lives are very very stressful and busy with 2 children with additional needs and two others who also need our very limited time, we wish we could write back to everyone but please know that every piece of post is treasured as it makes our girl, and us, so happy to know that people are thinking of her and care. I will update when we know which ward we will be on in Great Ormond Street as Daisy will need her post more than ever then.

Update 6th May 2012

So many apologies for not updating, a lot has happened since our last update. Daisy had her colectomy surgery and her appendix was used to form a mitrofanoff channel to her bladder to catheterise her. She stayed in hospital for a couple of months but fortunately we were home in time to spend a lovely Christmas break at our Hospice. We have struggled and struggled with Daisy’s pain and infections and now I administer two Intravenous drugs to her nightly as well as her TPN together with a cocktail of medication which keep her bugs at an acceptable level and help manage the pain. We have had to resort to a couple of hospital admissions when her pain has been uncontrollable at home but now with the support of all of Daisy’s team we have agreed that all care is to be managed at home and we are all working to make sure she is kept out of hospital. There are no more procedures or surgeries that Daisy can now have that will offer any hope of cure or treatment, every thing is aimed at managing her symptoms and maximising her quality of life.

Saying this, she is having amazing quality of life at the moment, being at home for the longest period in her life has made her so happy, for the first time ever she managed a complete term at school and she is progressing so well, learning to read and has now started weekly horse riding lessons too, something we thought we would never see. It’s hard work having Daisy at home and having to manage all her medical care, some of which is so specialised many trained nurses are unable to undertake it! It is so brilliant to be a family together again though; we even managed a short break to France at Easter, amazing!

I have to say the biggest, thank yous to two amazing Pals – Glad Shrubshall and Kim Lawrence – the regular post and gifts from them make Daisy so happy, it’s just so lovely that we are very much part of their lives, they are part of ours as we always recognise the handwriting on cards and Daisy loves to hear all about Baby Stevie, Kim’s little boy! I hope we get to meet Glad and Kim one day, they are such special people! We are also looking forward to meeting lots of Pals and Viks and Kate plus hopefully Jen M at the Summer Post Pals Party. We have so much to look forward to this year, we are living life to the full and living for the day and trying not to worry about the future – as I often remind myself “Yesterday is History, tomorrow is a mystery, today is a gift, that’s why it’s called the present”. You can read more about Daisy on my blog too.

Update 29th August 2011

Daisy is back in hospital at the moment. We have basically been in and out of hospital since February and have not had more than 2 weeks at home in a row or more than 2 weeks off IVs.

We made the decision for Daisy to have the colectomy surgery and this is planned for 9th September as a joint procedure with a Mitrofanoff procedure to her bladder. These are both major surgeries and we anticipate she will go to PICU post surgery and remain in GOS for around 3 months to recover. She will be going onto Squirrel Ward and will be admitted on 8th September.

We have such lovely people writing to the family, it gives us such comfort to know that people are thinking of us and we are not forgotten about – Daisy just loves getting post and gets so excited opening parcels or seeing her cards. Jenny, Glad, Rosie, Wendy & Kim are regular writers and we so appreciate hearing from them and all our Pals – we know you all have busy lives and it’s so kind that you give some of your time to help our little girl smile so much.

The children had a family discussion about Post Pals recently and they all decided that they love receiving Post Pals mail but they know that as a family of four there are more than the average number of children to buy for. The boys have asked if they could not have presents bought for them but they do enjoy the cards and letters. Xanthe loves receiving craft/art sets so that she can do them with Daisy but they unanimously said that the greatest pleasure is seeing Daisy receive presents and they would rather she gets gifts than them (unless they are things that they can do with Daisy, as in Xanthe’s case).

Update 9th June 2011

After 15 long and stressful weeks which included 8 anesthetics, Daisy has transferred to her beloved Chase Hospice for some respite for a couple of weeks. She has been left battered and bruised and mentally and physically traumatized by this stay. Sadly she will come home on even more pain killers and drugs and needing more interventions than before and this will be the story from now on. The doctors are now discussing her next major surgery which will be a full proctocolectomy and whether this is even safe to perform on her. Our only hope of this surgery is that it will remove the colitis which is the reason she is on immunosuppressants and steroids which greatly increase her risk of infection. We hope that we will get our longed for summer at home together as a family so that we can fill our days with happy family times and memories.

Daisy’s determination, strength and courage are inspirational. Thank you to everyone who writes to her – believe me, the cards and gifts really do make her day.

Update 31st May 2011

We had hoped to transfer to the hospice today but unfortunately Daisy’s hickman line has migrated and she will have emergency surgery tomorrow to remove it and repair the damage to her vein.

Update 25th May 2011

We’re still here in GOS. We are hoping to be home for the summer and then returning in the Autumn for the biggest surgery ever. Daisy loves to get post and Kate D saw how excited she gets when she visited us! It can really really brighten a long, stressful day.

Update 21st May 2011

Daisy is quite poorly at the mo and starting her 14th week in Great Ormond Street. She has had high temps for over a week despite being on several different IV antibiotics, her line cultures are negative but the docs are presuming this is the source of the infection as she is septic. The line is coming out on Monday and we are all keeping everything crossed she turns the corner then. She then needs a new catheter under GA and a new line, both done as separate procedures so 3 more anaesthetics to look forward to.

Update 26th April 2011

Thanks to all our pals for the lovely Easter gifts and cards – they really really brightened the children’s holidays and Daisy’s hospital wall.

We are waiting for a date for Daisy’s bladder surgery and to reduce TPN to a more manageable level. Still hoping to transfer to our hospice for a respite break in the summer half term then home – fingers and toes crossed!!

Update 15th April 2011

It has been two steps forward and one step back, as always with Daisy. This week we restarted feeds at 5mls an hour but it caused her bowel to prolapse and as it was losing blood supply and turning black, we were in danger of losing a 5cm segment. Cue the emergency bleep to surgeons who had to act immediately to save the bowel.

Good news is that pain meds through the jejenum have been restarted successfully and next week we hope to wean her off the IV ketamine. Bad news is that a suprapubic catheter is looking likely, so that will be 5 stomas/pieces of plastic tubing she needs to keep going. All milk feeds have been stopped but we are making progress, albeit in baby steps.

Update 7th April 2011

Hi all, Daisy has been in GOS for 7 weeks now, 6 of them on 24 hour TPN and IV ketamine. She’s currently in theatre having a surgical jejenostomy place so that we can have better access for painkillers which can be given when she is at home. The surgery involves creating another stoma which we know will set her back for a while as she does not respond well to anaesthetic or surgery, but we can begin to move forward once she has recovered from it and hopefully be home for the summer!!

Update 30th March 2011

Tomorrow we will start week 6 in hospital. We have been told that Daisy will be here for at least a couple more months and we will then go to the hospice for a stay before coming home.

It has been decided that the proctocolectomy surgery would be too dangerous for Daisy at the moment, so the doctors are trying to treat her with iv steroids. She has been on 24 hour tpn for the past month and the plan is hopefully for her to have surgery on her small bowel, to form a surgical jejenostomy so that she no longer needs a transgastric jej, and if it comes out again I can be trained to replace it.

We are waiting for a slot to have an MRI under General Anaesthetic to screen for tumours as Daisy has a very increased risk of tumour formation and the doctors are concerned this is the cause of her pain. The likelihood is that when we do get home it will be on intravenous pain relief; she has been on continuous ketamine for the past month supplemented with tramadol. She had a bad reaction to morphine so this is no longer an option.

On top of everything else she has already had two infections and IV antibiotics since being in hospital. Because she is permanently attached to drips and pumps we have not been able to leave her room and this means that family visits with the three other children can be very trying! We are finding this stay particularly hard as we just don’t seem to have had a break from the relentless hospitalisations and bad news for years. It was 3 months since her last admission and that was the longest we had spent at home in a continuous period since 2008. Everyone is working hard at getting Daisy home but it has to be at her pace and in a way that means she can stay at home for as long as possible and we can be together as a family.

Jenny Ault has been such a star at making sure none of us are forgotten and thanks too to Jane B, Kate D, Kim L, Viks & the Post Pals gang. Big thanks to the person whose name I have forgotten who sent the little Monkey tree house playset to Daisy. She has played with it constantly and I am on the lookout for similar sets as she has had such fun with it!!!

Update 3rd March 2011

Sorry for not updating for ages, as always life has been full on here. We had a lovely Christmas and on the whole things have been reasonably stable apart from the continued high stoma output and Daisy’s worsening night-time pain which is requiring stronger and stronger drugs. However we have not needed to make any middle of the night hospital visits which is the best thing.

We are now at the point that total removal of Daisy’s large bowel is looking like the best option. She is due to go back into Great Ormond Street for a week’s stay, during which she will have two procedures under two separate General Anaesthetics to look at her bowel function and also to replace her trans-gastric jejenostomy tube which came out again this week. We hope to meet with her surgeon to discuss the colectomy surgery and probable placement of a surgical jejenostomy tube which I could be trained to replace doing away with the need for an anaesthetic. We will also be discussing small bowel transplantation – although Daisy’s large bowel is completely dysfunctional and will be removed, it also looks like her small bowel is also dysfunctional in that it works too quickly – her options are either a lifetime on TPN with all the risks involved or small bowel transplant. At this point we don’t know if she would be a candidate, no child with Costello Syndrome has ever received a transplanted organ (or even had complete intestinal failure) so the risks may outweigh the benefits, but for Daisy’s sake we have to leave no stone unturned even if it is a route we do not pursue, we owe it to her to have tried…

At the same time as this has all been happening we have been coming to terms with Theo’s diagnosis of Aspergers Syndrome, something we had suspected for a long while. The turmoil of living with a child like Daisy is intensified when you have Aspergers Syndrome and crave routine and order and this has caused Theo considerable anxiety. We are currently going through the process of putting in place the support he needs and supporting him with his needs. I don’t think an easy path was mapped out for our family but we keep rising to the challenges!

Big thank you’s as always to all our Pals, our children love getting post and being remembered – Christmas was lovely with all their gifts from elves and thanks also to Jenny for taking our children under her wing – Jules loves her letters, especially the ones about all the scary creepy crawlies and spiders and snakes!! Daisy loves cards – especially the ones made by Kim’s Grandmother, they are works of art! Thank you for all you do, I feel guilty that I just no longer have time to thank everyone individually but please know that your cards and gifts really do make a difference and make our children smile!

Update 17th November 2010

A million apologies for not updating sooner, life continues to be hectic.

Daisy left hospital in early September and returned to her beloved school. She managed to have a whole 6 weeks at home, the longest she has been out of hospital in 2 years, which was wonderful, however by week 6 she had yet another infection and ended up back at our local hospital on IV antibiotics. The course of this infection was not as straightforward as before, her stoma poured out making her very dehydrated and needing a lot more iv fluid, she also seems to have become resistant to one of the IV antibiotics we normally use for her so these have all had to be changed. We transferred to our hospice for respite from hospital and she came home needing even more TPN than before to make up for her very low sodium and potassium levels and massive fluid output. Great Ormond Street would like her to come back in for another inpatient stay soon and we have negotiated this for the New Year as we really don’t want any hospital stays before Christmas if possible. They are hoping to see if there is anything that can be done to slow down her small bowel which is in overdrive, conversely, her now defunctioned large colon does not work and still causes her pain, especially as she continues to lose mucous and blood from her bottom even though she has an ileostomy. Andy and I have also decided that following this next in patient stay we will start seeking second opinions on Daisy’s management and treatment. At the moment all care is palliative and we have been told that a small bowel transplant is out of the question; however we do not want to leave any stone unturned in ensuring that Daisy has the best and longest quality of life possible.

Medical issues aside, we have had the most amazing experience this month – the whole family were taken away for a holiday of a lifetime to Florida, staying in a village specifically for life limited children and visiting the Florida Theme Parks – it was like something out of a dream for us all, we were part of a group of 22 families, supported by volunteer helpers and medics and were treated like VIPs everywhere we went. Daisy had the most wonderful time, the week before we were due to fly she was still in hospital, but in true Daisy style she made it and was fantastic for the whole week. It has given us all a boost, and the happiest memories we will treasure forever. We are now looking forward to celebrating Daisy’s 6th birthday on the 22nd December, there have been times over the past few years we did not think we would get this far, but Daisy is the most determined little girl ever and she embraces life to the full. She is wonderful and we are so happy that we have her!

Update 13th August 2010

Hi everyone – for those who don’t see my facebook updates, I have not had chance to blog for a while as Daisy remains in hospital following her ileostomy surgery. Following problems with post surgery infections, she then spiked temps last week as a result of her chronic bacterial overgrowth and the inflammation in her bowel coming back, then just as she was picking up she contracted parainfluenza!

On top of all of this her stoma output is massive, meaning she is on TPN for 20 hours a day plus fluid boluses and piggyback fluids and is still getting dehydrated – until this is more manageable she cannot go home and because of her problems with bacterial overgrowth and the flu, has been stuck in her room bar a couple of visits out for the entire stay. We hope to be able to get her to about 16 hours TPN and top up fluids and at this point we will be allowed (hopefully) to go home with regular bloods to make sure she is not becoming dehydrated.