Elly Rarg

On this day, 04 Dec 2016. Oh, look it us. Family Christmas Jumpers. I remember this day. We had our community nurse come by to check on Kai, and I took a photo of Sam and Kai, and she asked if we wanted her to take a photo of all of us. So there we are. The three of us in Christmas Jumpers.

Today… I feel like the grief of this is bottomless. I don’t know what else I’d expect, really. This gentle, every day asking of how do I feel? As if it’s one day going to switch into something tolerable, manageable, something other than this soul sucking everyday horror.

It hurts because I love. I miss him. I miss him I miss him I miss him. Honestly – the most soul sucking thing is that it didn’t have to be like this. There is a cure, it exists. It’s at that stage just before clinical trial. And that stings. That if money wasn’t a thing, a cure could have happened when he was a baby, and he could have been saved, and lived a whole life without NKH.

One day, that will be true for other babies, and it will be because of the relentless push and the hope of parents like me.

(It was Giving Tuesday yesterday. Please donate £10, so that one day, other kids with NKH will get to live: mikaerefoundation.org/christmas)

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit #fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DDKm0-UoIUf/

On this day 03 December 2016. Okay. We were new parents. We just were – over enthusiastic parents just pleased to not be in NICU, with our baby alive and mostly well and just – we were happy.

It’s impossible to happy, in the days of today. We have as status quo and we fill our days with distractions and jobs and focusing on the two small children we have to care for (but honestly, I don’t feel like we’re doing this well. The grief is too much, too heavy. This desolate grey heaviness that touches EVERYTHING.

We laugh and we have dance parties, but a large part of me is just that its all a clown mask. A façade of what it used to be like, in the before. In the after, nothing is okay, really. We’re navigating this time the best we can but honestly, who cares? What does it even matter when one of us has died? (This is clearly the grief talking, because I can remember when I cared. When life mattered. The face of that girl in that photo, she cared).

Anyway. We made it through another day, today. The thought of continuing on, with a forever number of days without our boy is unbearable.

PS Today is Giving Tuesday. Please watch the video and donate, if you’re able: mikaerefoundation.org/christmas

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit #fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DDIKa_VRqPS/

On this day 02 December 2016. Oh baby boy. I don’t know what we did this day. There are barely any photos of what we did (weirdly there are a million of Kai sleeping on Sam).

Today, right this very minute? Our youngest is sprawled out on Sam just like this. She’s 15 months right now. The difference between parenting an NKH kid and a neurotypical kid is stark. Having two other kids doesn’t replace Kai, our eldest. He made us a family, he held us together. Our sweet boy.

Our other two – their paths are different. They’re going to grow old and independent and, one day, navigate their lives away from ours. But we always knew that Kai’s live would be intertwined with ours. That his path would only veer away from ours in death.

I hate that we’re here. I miss him so very very much.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DDFT8msx6vc/

On the 1 Dec 2016. I look at this photo and I think how full my heart was, despite the fear of NKH. The early morning cuddles, baby Kai’s weight when he falls asleep on you all warm and heavy. The sleepy just waking up faces that made me laugh. I remember bath time being a thing – it was mostly Sam’s job, but he’d proper squawks when getting undressed, be calm as a cucumber in the water, and then squawk again when you took him out.

Just… moments upon moments that made up the day. This sweet boy. This was the absolute best time in his life. The time with the least pain, when his body worked the best. These days, hey?

Today… today I’m reliving his death over and over again. Struggling with the decisions we made. We tried so hard to do what we thought was best for him, but honestly, I spent hours every day second guessing myself, wondering if I should have done something else, something different, advocated for more extreme measures, advocated or for less or for… I don’t know. A different outcome.

But then, I’m also grappling with the idea that had he lived, it would have been in pain. It’s definitely better that he lived, rather than didn’t (look at his beautiful, wonderful face) but is pain free in death better than pain in life? Does the balance of joy and pain even out? Would there have been more joy? Or would it have been more pain?

My brain goes around and around and around, and I know it will, forever, because at the heart of it – I can’t be okay that my baby has died. It might have been what was best for him, but for my heart? I will carry this every single day. Every single minute. My boy is in every moment. I live in this moment, and every moment moving forward in his absence, and god I feel it. The space. The loss.

Onwards has never felt like such torment before.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DDFSdN9xYSP/

On this day, 30 Nov 2016. I am nervous. I know what is coming, because it already happened eight years ago. We’re coming to the end of the honeymoon period. That time when it possible that it was all a mistake, when he was the best he ever was. Focusing, following, eating, vocalising, being on point with all his baby milestones.

I’m not sure how I feel about this project, now that I’ve committed to it. I thought that I’d just try, and see how I felt. Is this helpful? I don’t know. The gentle examining of Kai’s every day of his life… of examining what I remember of then, of what I feel now.

Mostly, there are a few things that are true of then, and true of now. I love my boy. I hate NKH. I’m working towards an NKH cure. He was here. He is loved. And he matters.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DC_csulsSM8/

On this day 29 Nov 2016. We met up with our NCT friends today eight years ago, a nice moment of normality. I was so jealous of their meet ups when I was in NICU, and so experiencing it for myself with my own beautiful babe felt like such a luxury. But there was also that anxious scanning comparison between their babies and mine. What was different? Could you tell he had this awful disorder?

I remember the not knowing being so hard. Not knowing what our future held, the terrifying idea that he could die at any moment (today? Tomorrow? Next week, month, year?) and trying to anticipate that genuinely did my head in.

There’s something about being on the other side of NOT holding that fear, because the worst has already happened. He’s already died. I wouldn’t call this feeling relief, but there was a definite loosening, of trading one emotion (the fear of it happening) with another (the agony of grief).

This side is not better. The other thing that has happened is that my relationship with death has changed. No longer is it this thing to be avoided at all costs. I have teethers, in my two other sweet babes but honestly, if the universe was to decide that today is my day, I’d be like, okay cool. I don’t know what happens on the otherside, but Kai’s over there. The idea of going where he is is not awful. There’s a teether from my heart to his, a taut balance between his siblings here and him there.

I don’t know what the next forever holds, but it can’t be worse than this, here and now, without him. And I know – that this seems all very melodramatic, and the me of before would be rolling my eyes so very hard, but I genuinely can’t explain the depth of what I’m feeling. The grief of having your child die, the excruciating agony of it, all the time. My every thought is him. Every day, all day. Kai kai kai kai kai kai.

A friend whose daughter died told me it does get better. I genuinely don’t believe him. There is nothing to change – Kai’s already died. How could it ever get better?

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DC9nuTvxQSN/

On this day, 28 Nov 2016. This day was the first taste of what parenting a disabled child was like. It was navigating appointment after appointment, feeds and meds on the go, big fears (was that seizure? is that neurotypical baby movements or is that NKH? What was that?) and trying to find joy (read: often deranged optimism) in the things he could do (look! He wriggled himself almost off the couch! He’s moving independently and they said he’d never do that!)

I remember feeling overwhelmed about today. Pleased when we both got to the end intact, and everything was okay still.

Looking back through todays photos, there’s a series (not shared) that to my experienced eye now is clearly a seizure. It’s so in line with Kai’s seizure pattern, but if you didn’t know what his seizures looked like you’d miss it. I missed it, back then. It was subtle, a series of tightening, slightly odd movements. And yet… I didn’t think he’d had seizures back then. But he was. He was, and I wasn’t familiar enough with them to know.

Poor baby. Seizures at a few weeks old. He wasn’t even two months old, here. What a time.

Looking back, I’m desperate to hold every detail in my mind. To remember as much as I can. To fill my Mama heart with what I want and can’t have (my son, alive and well, and even though even here he wasn’t well, he was well enough to cry, to see and look at me, to sleep and to wriggle and to love. And be loved. So many photos not shared are us and friends loving on him so hard).

And I think that’s what matters, hey? That he was so loved. I miss this time. These days were long, but often it was just me and him. What a precious time.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DC7RI97xmwU/

On this day 27 Nov 2016. Oh, teeny tiny bub. So many photos from these days are just him sleeping, but you know – he slept like a newborn his whole life. Literally hours and hours sleeping every day. Kai had good awake moments, but the bulk of each day was him sleeping. I know that was the medication, and NKH doing what NKH does. It feels awful to know that the bulk of his life he was sedated.

There were so many things I was trying to line up for him, with the support of his team. The Keto diet. A reduction of DXM and potentially SB. Was I too slow? Would it have made a difference? The second guessing and wondering is like a squeeze to my heart with every thought.

We constantly were trying our best, but honestly – I feel like my best wasn’t couldn’t enough. I was a great Mama, and I advocated and I read all the things and asked all the questions, I fought for care and chased down every last little thing I had capacity to do. And yet… I still feel like there should have been more, that extra something that I was incapable of delivering. (Please don’t comment and tell me that I shouldn’t feel that way – I don’t want to hear it. I know that everything thinks I was a stellar Mama and I was great and ad finitum.) The truth us I DO feel this way. I do feel like he deserved more and better.

To that one person who wanted to tell met that ‘screening and prevention’ would be better and used “in your words – he deserved more” as an insult with an intention to harm, I still think him living with NKH is better than him not living at all, and I still think that yes, every kid in a vulnerable situation deserves more. Kai deserved more joy and less pain. That’s not what he got, but it’s what he deserved.

One day, kid with NKH WILL have more. They will have a life without seizures and dystonia and be able to communicate with words, and not just their facial expressions. Honestly? One day a diagnosis will be no big thing, it will eradicate the need for screening because having a diagnosis like that will be irrelevant (I dream I dream I dream. One day).

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh # #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DC3uToDi0Bh/

On this day, 26 Nov 2016. Oh bub. So many of his days were like this – so much pain from the benzoate, and sleeping because of the glycine.

The realities of going through each day, of witnessing tiny segments of his life slowly and in detail have been both a balm, but also hard. These days were mundane, and I can feel it when I look back at these. I’m also terrified, because there will come a day where I didn’t take a photo of him and then what will I do on that day? Even worse, we’ll come to a point where there are no more photos ever. I hate that. I hate that so much.

He looks so little here. He’s just over a month old, 46 days. This baby. He is so very loved. I wish there was more time with him. I’m so sad there is not.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit #fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DC1JfwyvMWo/

On this day, 26 Nov 2016. I was a keen Mama, clearly. I wanted so dearly to do right by my boy. I wish I’d known that it was too early for tummy time for him. That someone, anyone, had said – please stop. Wait for a physio. You’ll tire him out, and it’s not the kind of muscle building he needs. He’s not a neurotypical baby.

But there was no one. It was too early for physio, for that kind of support. We had nurses who were just beginning to drop by, but otherwise, we were just trying to figure it out. Now I can see that it would be way too early, and that there were other ways. I guess the position change might have been good for him, but his poor sweet body couldn’t lift his head.

He would, eventually. I was so scared he’d never gain head control (many kids can’t) but he did, in his way. I wish I’d known then what I do know. That it would come, but if it doesn’t, that’s okay. That neurotypical progress (head holding, sitting, walking) is not the goal, here. Happiness, enjoyment, maintenance of gains he made at his own pace, would be better. No need to push and push and push.

Just enjoy him, love him. Make more time for him, find the things that work for him.

That is what I would have said. 2016 me would have brushed that off, because it’s not an easy thing to hear. Accept your child is disabled, meet him where he is, and building in moments of happiness and joy for him.

In today, my heart hurts. I miss him. Those words also feel like nothing words (because obviously). But the DEPTH to which my heart hurts and how much I miss him. You could blow a hole right though the earth and measure through the middle to the otherside of the solar system and my heart would say oh yeah, that tracks. Today is hard.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit #fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DCyktVcyhQi/