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When you can't trust, the people you have to trust.

Its a pretty sad state of affairs when there is literally not a soul on earth that you can trust 100% with the safety of your child. But it is a reality that so many parents like me face day in day out.

Prior to Alice I, like most "Normals", assumed that doctors knew pretty much everything about sickness and medicine, that Specialists in their field know way more than Google, that virtually all medical issues had some sort of treatment (even if it didn't work in all cases) and that nurses were infallible. Turns out none of this is true. And finding this out is a very scary realisation when your child's life quite literally hangs in the hands of these people.

My first experience of this awful truth (and yes I know I talk about this a lot but its relevant to so much of who I have become) was the first 12 weeks of Alices life. More medical professionals than I can shake a feeding tube at, from all sectors, failed to listen to me and my concerns. People whose job it was to listen to me and to give my child the benefit of all their medical knowledge, brushed me off as paranoid. I was dismissed by doctors, nurses, anesthetists, GPs and midwifes as over protective, irrational and finally, post natally depressed. Turns out I was none of these things and Alice wasn't just a wee bit sick, she was in fact dying and not one fucker with medical training even ran a single test.

This was Shitty Life Lesson Number One.

At the point of Alices eventual diagnosis she was seizing 24/7, vomited every feed, couldn't see, swallow, hear, cry or even move her limbs voluntarily. But on the plus side we now had a culprit and a name for our nemesis. Surely this was the point where the experts would swoop in with answers and cures and plans and knowledge? Nope.

We were told briefly what Alice had and that it was really rather shitty in terms of things you don't want your baby to have (kind of up there with Ebola, terminal cancer and The Bubonic Plague).

We were also given a vague run down on what was going on, something to do with enzymes and amino acids and the fact that me and Greg had given it to her but little else. Of course my immediate response was to reel off a load of questions and to fish for reassurances, only to be told that none of the sad faced and deeply apologetic "Experts" in the room had ever heard of it or dealt with it. They did however, know someone who had, so if we could just wait an hour or two, he would come and see us and explain. How can pediatricians in a Children's Hospital not know anything about what she has? Surely they know all this stuff, about all illnesses and all conditions? Apparently not and as it turned out the person who had dealt with NKH before only knew fractionally more than the sad faced doctors.

Shitty Life Lesson Number Two was complete.

My next wake up call was after finding a support group for NKH parents and discussing drugs that actually make NKH worse. I was horrified to find out Alice was on one of them. FFS! I have a terminally ill baby, with a condition no one knows about and a quick google search and a chat on Facebook Forum had garnered me with enough information to tell me she might as well be being raised by Wolves right now.

The drug was conveniently removed just as I was about to demand its withdrawal. I didn't have the energy or fight to say anything but to this day I think Alice was medicated first and the research was done after. The people that Id naively assumed were all knowing were in fact Googling their way through this shit show just the same way I was, so I assume they found out that Phenytoin could cause devastating outcomes in NKH around the same time I did.

This was Shitty Life Lesson Number Three and with it came the distinct feeling that at just 13 weeks into Alices life and 1 week into a terminal diagnosis, that I was well and truly on my own in terms of keeping her alive.

Once this drug was removed and the other meds began to work, Alice slowly came round and by week two we were moved to a normal room. I began my training in tube feeding and how to measure and administer the huge amounts of medications we now needed to keep Alice alive. At this stage they needed to be given 4 hourly round the clock without fail and that job fell entirely on me. Initially I did them all, day and night. Not because I'm a martyr or a control freak but I wouldn't have nurses at home so I might as well get used to it right? However, I soon realised that giving a baby milk in the night every few hours is very different to measuring out 6 different medicines and injecting them into nasal tubes. Especially when the wrong amounts of these drugs can kill and tiny babies need tiny amounts of drugs.

Its not a great feeling, knowing that you get that shit wrong because you are tired and not thinking straight and, Bingo, you just O'D'd your newborn! So when a kind nurse told me I had to rest and that I needed to trust that they could do the night shift, I relented and got my first full nights sleep in weeks. But guess what? The ward got busy, someone didn't do a correct hand over and no one medicated Alice through the night. She woke up seizing and projectile vomiting.

That was the first and last time I trusted anyone to medicate Alice for several years.

At this point I can go on and on and on and list all the epic fuck ups in the last 9 years of Alices life at the hands of those I have no choice but to trust, but the above gives you the gist of what goes on. Considering all that you have just read was in her first few months and before we'd even left hospital for the first time, I think you can safely assume I don't have time to list them all.

But this most recent fuck up is the fuck up of all fuck ups and I still cant quite believe it has happened. I hadn't shared with anyone, not even my own Mum, what has been going on, because like all NKH parents, the minute we think we are seeing those signs of "the inevitable decline" in our kids, we absolutely shit ourselves and go into this strange secluded world of "I'll fix this, then tell people about it when she's better, because if I say out loud whats happening then it becomes real".

As you may know, at Christmas time Alice learnt to swallow tablets and as such we were finally able to stop using her G Tube (a kind of petrol cap type device on her stomach that allows you to feed, hydrate and medicate directly into the stomach). NKH meds are beyond disgusting and completely unpalatable so for her to be able to take them in tablet form is life changing for us all. Unfortunately she needs A LOT of meds, but my little trooper started swallowing between 15 to 20 tablets a day with no fuss whatsoever.

Then out of the blue, in March of this year, Alice had a fit, a big one. Now I say "fit" and not seizure because we know it wasn't a seizure. There are certain things that make the two distinguishable and we know what to look for. It was right after Alices evening meds and meal when she suddenly began to shake and twitch. I lay her on the bed because it was instantly apparent something was very wrong and she flayed around fiercely. She could barely speak other than to say what sounded like "Im Ok", she drooled, her back arched and her face distorted like she was having a stroke. After about 5 minutes (around the time my heart was about to give out on me) the craziness started to slow down and by 10 minutes she was back to running around the house like a lunatic.

Since then things have steadily gone to shit. Alice has had several more of these attacks. Oddly all after her meds. She has developed vocal tics, what appears to be the beginnings of Tourettes, her behavior has gone to shit, her movements in general are worse, she has started some weird autistic type traits like hitting her own head and squealing, her sleep is worse than her already non existent sleep and lots of other little shitty changes we just cant explain.

We cant run an EEG on her brain to see whats going on, as she'd never comply with all the sticky probes and physical manhandling and being required to trust scary doctors, so an MRI of her brain was booked. Neurologists looked at the videos of her fits, we had Zoom consults with the Childrens Hospital and I had meetings with her school to warn them of these scary new episodes. Meds were increased but that just made her worse and new meds were discussed. In the end we were finally left with the fact that this is simply an evolution in her NKH and maybe we needed to accept a decline was coming and this was a precursor to a big seizure.

Then last week I had a light bulb moment. As I stood with all her tablets in my hand it suddenly occurred to me that they looked different to the first few batches.

In January and February I had her meds made up a month at a time but in late March I stock piled 4 months of meds as the whole COVID Shit Storm was really taking off.

All of Alices meds are made bespoke to her, so these are not over the counter medicines. We use a compounding pharmacy who make everything from scratch, exactly to Alices requirements and medical needs and in March they kindly made me up an enormous batch so we didnt need to keep leaving the house to restock in case the world was taken over by Zombies.

The main requirement of her tablets are that they MUST be in veggie caps not gelatin. This is not because Im vegan, or a hippy or just plain awkward but its because gelatin is literally Kryptonite in NKH. At 30% glycine its quite literally poison to her. Its the very core of NKH because she cannot metabolise glycine.

Thats what NKH is, an inability to metabolise glycine. This harmless amino acid found in all sorts of places can quite literally kill her.

Im sure you've guessed it by now, but yes, some fuckwit in March, made all her meds in gel caps not veggie. The very product that the contents of those capsules are designed to remove from her body was used to encase her medicines. With every meal, I have been feeding Alice doses of the product that will ultimately kill her. The fucking substance that has destroyed her brain and so much more was being put directly into her fragile body and as those capsules dissolved in her stomach, she was filled with poison and the result was her collapsing like she had late stage Parkinsons.

As its coursed through her veins and made its way to her brain it has, we suspect, been causing all the other crazy shit we are seeing in her behaviors and movements. Then when its fully distributed, I've been giving her another 5 tablets and we start again. Since March.

Im not going to name and shame and Im not going to say anything about what we are doing or what the pharmacy is doing or how we/they think it happened but I wanted to share whats been going on.

When I worked it out I cried, I was devastated, I felt guilty, I felt responsible, Im angry it took me so long to work it out, Im pissed that once again Im reminded not only is it 100% on me to keep my daughter alive but also that yet again a Professional I have no choice but to trust has endangered her. I fed that shit to her over 15 doses a day, I failed her. Sure its not my fault and my rational mind knows that, but you try telling that to my non rational side.

Under this post I will include a video of one of the fits. If you dont want to see it, thats fine. Its not horrific or anything but it is heartbreaking, especially if you listen to her trying to tell me shes "OK" as she always did when they happened.

I hope Alice wont mind me showing it and of course I am always torn about showing stuff like this. But the whole purpose of this blog is to be real and transparent and to let parents know in similar situations to me, that you are not alone. Please see this as a reminder that whatever you see on social media, from just about everyone, is NEVER the full story. Its never the grim behind the scenes version.

Disability is not all smiles and wheel chairs or hilarious special need anecdotes. Alices fight with NKH is not all giggles, success against the odds and crazy toilet jokes. Its relentless and harsh and brutal and exhausting, but we will always smile and joke and laugh at every possible opportunity.

But we don't fucking trust anyone!

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