I have Costello Syndrome, Costello Syndrome does not have me

I have amended this quote from one I saw on the back of a running vest as I ran the Bupa London 10k on Sunday - the quote I saw was "my name is Jo, I have MS, MS doesn't have me".  That really inspired me.

If Daisy could talk fluently and tell you how she felt then I think she would say this because this is how she lives her life.

Every single day is a monumental challenge for Daisy and truly I do not believe even those closest to her know what a challenge it is - I guess only Andy and I as her parents have a little inkling of the mountains Daisy has to climb day in day out just to squeeze every moment out of her day and be the little girl she wants to be.


I sent an email to a friend in Australia who has a daughter battling the terrible cancer that is always the risk with this syndrome, I listed out the medications Daisy was on to see if any of them had been tried with her own daughter, writing them down I was shocked, I spend a large portion of  the day drawing up these drugs or adminstering them  all with the aim of helping Daisy get through the day.  I have never shared them on the blog but I think it's about time I'm completely up front about how much Daisy actually needs to survive:-





Daisy's Daily Meds:-

Baclofen, Amitryptiline, Cetirizine, Erithromycin, Melatonin, Oxybutnin, Diazepam, Ketamine, Paracetomol, Itraconazole, Gabapentin, Chloral Hydrate - these are all given via her jejenostomy tube at the top of her intestines


Omeprazole, Co-Amoxiclav - these are given intra-venously directly into her blood stream each evening


Mesalazine Suppositories - for colitis


Growth Hormone sub-cutaneous injections twice daily - Daisy has growth hormone deficiency which causes hypoglycaemia, the injections give her metabolic stability.

On top of this she receives a special, broken down, pre-digested milk feed via a pump directly into her jejenostomy at the equivalent rate of 3 teaspoons an hour for around 6 hours a day.

Because this feed does not get absorbed at all and is soley to keep her intestines alive,  Daisy's main nutrition comes from her TPN - Total Parenteral Nutrition.  She has 2 litres of a specially prescribed intravenous fluid direction into her blood stream running from 5pm through to 8am.  We connect her drip to her hickman line which runs towards her heart via a main vein  and in the morning disconnect, flush the line and lock it with an anti-microbial solution called taurolock.  Sometimes we have to draw back blood from the hickman line for testing to ensure that she is not becoming dehydrated and to ensure that the right balance of nutrients is added to her bag of fluids.


Daisy has had 8 central lines in her short life.  This is not good as she is running out of options for lines as it is normally not possible to put another line in where one has already been placed.  Line 9 will be placed in June as this current line has been repaired and is also colonised with the bugs that permanently sit in Daisy's body.  Daisy's hickman line is her life line, without it she would not survive so we have to ensure it is in the best condition it can be.   We live with the daily fear that one of these bugs - the primary ones are ecoli, pseudomonas and candida will take hold and we will not be able to control it with the drugs available. At the moment the cocktail of antibiotics and antifungal help keep things at a manageable level but the bugs will never go away, they sit in the bits of plastic that are in Daisy's body providing the means to give her nutrition and keeping her alive - it's a catch 22.

She has had over 50 procedures and surgeries requiring  general anaesthetic in her short life including some pretty major abdominal surgery.  Most of her large bowel has been removed, her appendix was used to form a mitrofanoff stoma below her belly button in order to catheterise her bladder, she has an ileostomy where her intestines end and wears a bag to catch the waste, she has a gastrostomy to drain the permanent bile that builds up in her tummy and her jejenostomy stoma is used to administer what medications we can before she vomits them up.  Her palliative consultant believes that she must have permanent pain, often worse at night.



When most people would give up, would lie in bed, would not want to function Daisy wants to grab life and get as much out of it as she can.  She can have the night from hell, look washed out from her chronic anaemia (she cannot store iron even though we give it intravenously so is permanently anaemic) but will still want to participate in family life, go to school, be a little girl.



So when people see this happy, vibrant little girl they do not realise what effort she has gone through to get this far. I am not sure people realise the sheer effort it takes Andy and I to keep her at home, out of hospital and to enable her to have a life. We have certainly found out who understands and who doesn't, we have the most amazing friends and people who have been so kind and generous with their time or their messages or thoughts, just to know that people are thinking of us is enough to keep us going.  Conversely, despite the wonders of technology and the internet and social networking and blogging people who we thought would be there for us, have not been.  But we know this is not an unfamiliar scenario with children who are so complex and who have such a precarious life span , people are scared, they don't want to get involved, they would prefer to keep their distance or maybe despite all my attempts at communication they are just unaware of what we go through to keep our daughter alive?

But Daisy keeps on going and she keeps us going and that is the most important thing of all.  Over the last few weeks she has scaled new heights in what she can do, things we never thought possible, things that we were told she would never do.


Here she is, very excited sitting in the Theatre watching The Lion King


And she has now started horesriding every week with her school, look at that amazing straight back - this is the girl who has a huge scar from on her stomach following a seven hour open surgery last September


Daisy loves  books, she is now learning to recognise letters and beginning to learn to read with school



My name is Daisy, I have Costello Syndrome ,
 Costello Syndrome does not have me 




6 comments:

Tania Tirraoro said...

How brave she and you and Andy are to face every day anew. I won't ask how you do it - as the parent of children with special needs, though not health needs like Daisy, I know the answer. It is: What choice is there? You do it because she is your daughter and you love her and she deserves the best. It's that simple. 
That said, it still must be exhausting and distressing and heart-wrenching. But I bet she gives you just as much joy, as well. And more. 
x

Deaner81 said...

Wow, amazing story of such a brave little girl. Thanks for sharing.

Bavarian Sojourn said...

What an amazing post... and one incredible (and very cute!) little girl... Go Daisy with the horseriding and happy reading :) 

Violetsdiary said...

Wow, what a story!  I have only just found your blog.  I think you are right.  So many people just don't realise all the little extras that parents of children with complex needs have to deal with.  Daisy is so obviously happy and enjoying life.  What we need is for people to understand, not to shy away and feel sorry.  

Jill Bennett said...

What a cute little girl Daisy is!  I am a SSC volunteer and hope you don't mind me referring to your lovely daughter when I do talks to fund raising groups and at events.

Stephanie Nimmo said...

Please do - it's the real life stories that help people understand the difference children's hospices make to families like ours x

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