In | Health Update

September 2017


I cannot even begin to explain how strange writing this post is. A Year - it has been one whole year since we announced that Ivy had finally had her fontan open heart surgery. It's felt like a single minute and an entire lifetime all at once and in so many ways I could never have believed what was going to happen in those 365 days to get us to where we are now.

I never know how much to share in these posts - I am fully aware that as she get's older, Ivy deserves a right to her own privacy but I am also aware of the amount of people in her real life, and in an online one, that love and care about her. Selfishly, as her parent, I do find it easier to get information out on this blog because 1) I like to write things down so I can try and explain the complications as clearly as I can with links to relevant information and 2) it is much less stressful for me to let you all know all at once so I don't have to repeat myself all too often. I don't want my openness about Ivy's life to be confused with enjoyment of talking about it - goodness knows I'd give anything to not have to.

Over the summer, I had various messages about how jealous people were of my life, because I met Jools Oliver. However much I know these comments were in no way malicious, I can't help but take them to heart. My great bits are, of course, great but the bad ones are worse than you could possibly ever imagine - please don't ever be envious of the life I lead and if you are, I'd challenge you to be envious of the whole package. Beyond the summer's holiday snaps and fancy 'celeb' parties we have been going through a hell. I have mentioned previously that Ivy has been diagnosed with a condition called Protein Losing Enteropathy and it is not until quite recently that we have truly begun to realise just how serious this is for her. It is quite rare and there is incredibly little research on the disease itself so we have had to seek out other parents (mainly in the USA) and sift through pretty hefty medical papers ourselves. It doesn't help that in the majority of instances that this does happen to children that have had a Fontan operation (please read here) it usually does not develop until the patient's teens, not at 2 years old.

We have struggled to manage her symptoms medicinally which has resulted in continuous blood tests, switches and trial and error dosing that has taken it's toll on our day to day life but we have also undergone tests to try and determine the cause of this in the first place only to find that there doesn't seem to be a reason at all. We have had a sit down discussion with her main consultant and although I won't go into details on here, I do feel I need to share the basics:

- Over the summer, Ivy has undergone numerous invasive tests and dietary changes in a last ditch attempt to try and stabalise her. Many of these have been under general anaesthetic.

- She has, however, come off a horrendous steroid which we had trialled but to no avail. However upsetting it is to see a drug that has worked for numerous other sufferers do nothing for us, it has been a sheer joy to see my little girl's real face again these last couple of weeks between PLE flare ups.

- Ivy was meant to start nursery on the 4th September. She has spent the entire summer excitedly counting down to starting only to have a huge flare up of symptons mid-august (caused by a simple tummy bug) but a surgery which was due 'before christmas' was pulled right forward to her exact starting date.

- They were hoping that placing a fenestration (a small hole in her circuit) would put her PLE into remission. There is not much medical evidence to support this but it has been known to work. The possible benefits of the surgery outweighed any of the risks in doing it so we had nothing to lose by giving it a go but unfortunately it has made no difference. Once again Ivy has stunned consultants by acting in a way that they have not seen before in their long and very experienced careers. We are naturally disappointed but sadly, we are not surprised by the outcome.

- Currently, we have been allowed home with further in-depth investigations planned in the near future. Due to the unique nature of Ivy's case, there are many people involved in each decision of her care and we have to patiently accept that this can sometimes take some time. Whilst we wait, it has been advised that she does not start nursery due to her compromised immune system and for me, this has been one of the most devastating outcomes of them all.

- The only known 'cure' for this terrible disease is a heart transplant. There are many positives and negatives to taking the transplant road and I am finding myself already asking questions that I was hoping I would not have to do until she was much older. I am still wishing harder than ever for a miracle that can put this off for as long as we possibly can but with each failed attempt for answers I am slowly beginning to accept that this is becoming quite real.

We as a family are certainly not looking for sympathy and to all intents and purposes I don't even want to use it as an excuse for being a shit friend (but I did broadcast an apology for that also). There are so many people we have been so keen to meet up with since her fontan in september and if that hasn't quite happened yet, it's not because I haven't wanted to. It has been an incredibly long and rocky road in the lead up to her surgery last September and even worse following it and sadly it doesn't look like that's going to end anytime soon. It has been a lot for us to deal with and only now do we finally we have a clearer picture of what's to come in the immediate future (something we have very much lacked this past year), I felt it was time to be honest with all those that have invested their interest in my incredibly brave little girl.

She has been absolutely amazing in all of this - she can name all her medicines, she refers to her nurses as her friends and despite screaming the place down when it's time for a blood test (understandable) she just takes it all in her stride and never ever stops smiling. It is so overwhelming for me to see, as a parent, how strong and resilient she is to this horrific side of her life she leads as I'd do anything to take it away from her. I know I say it repeatedly but I just cannot put into words how proud I am of her.

For this reason, I don't want anyone to ask me how I am (thank you though). I have felt everything and nothing all at once in this last year and as I have always done, I just want to take each day as it comes, try and lead as normal life as possible in between the extravagant treats (that she rightfully deserves) and the downright depressing amount of hours we spend in a hospital of some sort. I will remain strong for her, and for us, because I have to - just as any single one of you would for your own child or your own family. This is my life and I accepted that a long time ago and all I want from friends, families, followers and the like is to just enjoy my little girl alongside me, just as much as I do. With just that, I know we will all be more than fine.

You can all posts about Ivy's heart here