Elly Rarg

On this day 31.10.2016. Kai was 20 days old here. Because he was feeding so well, Kai’s NG tube was removed. He was TUBE FREE. I was elated, and spent the entire day staring at his sweet, tube free face. I felt like our boy was going from strength to strength. This is what is called the ‘honeymoon period’ – it usually lasts a few weeks, and is where your NKH baby is the most neurotypical, behaving and developing like their peers.

It’s short, but looking back – this was a wonderful wonderful day. Being able to see his sweet face, to cuddle him without tubes (only the sats monitor, which I quietly just… turned off and disconnected). I was still living at home, and our boy was still in NICU (which is awful). This time of good felt fragile and new, and it was hard to trust it.

Looking back – I’m glad there were good days. I’m glad I got to cuddle him and love him and just… I hated leaving him. I still hate leaving him, walking out of his glade is still hard. I still miss him so very much, I cry still. Every day (in silence, in the quiet moments where people can’t see or hear or intrude with their need to make you feel better for something that will never feel better).

Kai died. He died. The visceral horror that is this experience, where I live when my boy doesn’t is soul crushing. Affecting everything I do, and it makes me feel emotionally fragile and taut with wrongness. The earth is still spinning and we’re just… pulled through. What even is time? What does anything even matter anymore?

Living has never been so hard. If I could, I’d step back into these moments. Just… I’d go back, if it was a thing I could do. To cuddle him, to hold him and love him and not… be here in this reality without him. I imagine this is a thing people in my situation all do – wish for the going back. It hurts to know there is no going back, and only the dreary forward in which he does not exist here, with us.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit � from Instagram: https://instagr.am/p/DByM3OMxJqE/

On this day 30.10.2016… We could NOT get enough of his beautiful face. There are literally hundreds of photos of every angle of his face from this day. He was so awake, and looking at us and interacting with us. It was literally the most beautiful thing I’d ever seen, my beautiful baby looking back at me. Kai was a completely different baby than he was a few days back.

Even better, he started breastfeeding on this day. Briefly, but he figured it out and I was ELATED that breastfeeding was something we’d be able to experience together. I’d spent so long pumping (and hated it) that breastfeeding by comparison felt like such a win. Something long fought for (on both sides) and I’m pretty sure I cried when he latched (I mean, I was also a very tired NICU Mama who was still coming to terms with the idea that her baby wasn’t going to die imminently, so tears when anything happened, good or bad, weren’t unheard of). Still. We did that. Together.

I’m very immersed in this early days at the moment, but I came across a photo of me and Kai around 3 years old, all cuddled up together and I cried because I miss him so very much. I just do. And I wish he was here to scoop up and it hurts so very much that he is not.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit from Instagram: https://instagr.am/p/DBvoE1wPoNf/

On this day, 29.10.2016. So many good things happened today. Kai smiled for the first time, at 18 days old. In his sleep, so not even close to a true social smile (and it would even be too early for a neurotypical social smile) BUT it was a relief to know he could, knowing that many NKH kiddos don’t meet that milestone. Kai also cried – big loud cries which were SO WONDERFUL to hear (because ITU is silent…really sick babies don’t cry). He also got his cannula removed, meaning he only had the NG and the sats monitor (which felt like nothing compared the tubes of previous days), and was downgraded from ITU to SCUBU. Oh the joy to be OUT of ITU! So many cuddles happened today, a proper little snuggler – I don’t think he spent a single minute in his incubator while we were there.

Kai always loved cuddles. Oh, I miss him so terribly. I hate that its been MONTHS since I last cuddled him (how how how how…) and that I will never again cuddle him in my lifetime feels impossible. How is that even a thing? Learning how to love him without him involves some kind of trickery which I can’t get my head or heart around. Every day. Every day I miss him, and I feel the space and the loss. Knowing that this is forever is genuinely devastating.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit �#fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DBtDPx4BrAa/

On this day 28.10.2016. Looking back at these photos feels easier, less traumatic. We were still in NICU, but each day brought back a whole bunch of positive firsts. Looking back, I can feel the joy thrumming through my chest, the love and the hope and the ‘thank all the powers that be’.

We tried him on a bottle on this day. It took a minute, but he worked out his suck reflex and took half a bottle safely (honestly, the idea that we did this without a SALT present gives me genuine anxiety right now, but I didn’t know anything back then). Reading back on old posts, I wrote “He’s doing so many more beautiful baby things. We love him so much. Sam laughs when our baby farts. And plays with Mikaere’s face. And our baby cries, with noise. Wrinkling his whole face up to show his rage.”

These were the first few days Kai got to be in his body where it wasn’t shutting down. Where it wasn’t broken, it was just perfectly imperfect. These few days were the first days of more love than pain. (Also, now that he wasn’t attached to tube and just sats wires, you know he spent most of the day in our arms, and not in the incubator). This was our second good day with him. He was 17 days old. I don’t for a second take for granted how lucky we were to have more than one good day with our boy.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit �#fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DBqedQTNkF2/

On this day, 27.10.2016. That day was a big day. When we came in, he was off the vent. Which is a surprise, I didn’t know they were planning to try, to see how he’d do. It wasn’t discussed with us before hand, we came in and he’d been downgraded to CPAP. We were elated, obviously, but Kai hated it. CPAP required a mask and it’s loud and theres a hat (which meant that he couldn’t have the CFM pins in, but also… he had scabs all of his head from them, so poor bub). And then, when we got back from dinner, they’d removed the CPAP. He was just on air. He opened his eyes, and was moving around more and just… this felt like a magical day. A day of hope.

Going slowly through each day of his life, intentionally looking at each photo we have (of the literal millions) and remembering. This day was eight years ago, and I still remember it. The surprise at the vent being out, then off CPAP, the tentative joy and hope and trying, for the first time, to wonder if we had a few more days together, maybe even weeks (I couldn’t, even then, hope for years). Going through each photo has been something to hold tight too, in the vacuum he’s left behind. He was here. He existed. And I love him (and thats why it hurts so much, because he is still, so very loved).

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit �#fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DBn5qxeq9ge/

On this day, 26.10.2016. Okay, we’re edging away from doom and gloom and I am so ready for more of the joy. Two wonderful things happened on this day:

1. Kai started holding our fingers. If you put your finger in his palm he would HOLD ON. This grasp reflex meant that his reflexes were returning (!!) which considering where he was a few days ago (no reflexes, unconscious coma, imminently dying) this felt absolutely huge. Plus, it was nice to feel that connection, that he wanted to hold on to us just as much as we wanted to hold on to him. He was beginning to move a little more, wriggle a bit. It was hard to tell if it was intentional movement or seizure related, which is why the grasp reflex returning felt like such a definitive moment.

2. Kai opened his eyes. He OPENED HIS EYES. He was 15 days old. Considering the last time we saw his open eyes was at birth, we were ecstatic. This was one of the first milestones that showed, beyond a doubt, that he was moving away from imminently dying.

Oh my beautiful boy, I wish I could see your baby blues now. I wish you were here to laugh at us, to laugh at your sister and to be here, with us and your nurses and just. Here. I wish you were still here and I miss you. So very much. I can’t describe how very much, there are no words, really. Another day without you (another day carrying you in my heart).

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

On this day eight years ago, 25.10.2016. Oh this day. This day they wheeled my baby down to the OR and took a chunk out of his leg (read: skin and muscle biopsy to see if it would help explain the previously unexplained hypotonia, his floppiness). They didn’t really need to do this, because at this point they had a good idea (though unconfirmed by genetics) that Kai had NKH.

While I’m sad that it happened unnecessarily (Kai had a scar his entire life) I’m not sad that it was, many years later, given to UCL at our request so they could build iPS cells (Induced pluripotent stem cells), which were then used to grow other cell models in NKH research, helping move research forward to an effective treatment.

It brings me comfort somewhat to think that potentially, his cells live on, continuously generated in a lab for cell modelling, even if he doesn’t.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit �#fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DBipOcovv7W/

On this day, 24th Oct 2016. Here is what I remember about this day – sheer profound relief that I got to hold Kai in my arms. For almost a whole hour. He was showing signs of waking up, now that he was on sodium benzoate and dextrometorphan. He was beginning to get upset when he was in pain, and was beginning to breathe over the vent. We also were also becoming pro’s at changing his nappies, finally being allowed to do so.

I also remember that when he was in my arms, that was when I relaxed. I fell asleep (only momentarily, because you’re not actually allowed to sleep in ITU/NICU, let alone with a baby on your chest who is on a vent, which is taped to your shoulder…) but the point is here, that I was comfortable enough to do so. I wasn’t sleeping well, at home, without my babe. That I could drift off was such a ‘woah’ moment.

I held onto him as long as I possibly could that day, because it meant those awful CFM pins were not his head. And he settled I think, on my chest.

Looking back at these photos today feels like a balm. He was here, and he existed and he mattered. Even then I loved him with fierceness I still feel today. I also think these photos are getting easier, as I know that over the next few days things improve for him. And we got through a phase of joy and newborn normality (or as much as you can, when you’re basically living at NICU). Over the next few weeks was the most “neurotypical” time we ever had with Kai. He was just a teeny tiny baby, doing what teeny tiny babies do. I’m looking forward to sifting through those weeks.

Anyway. I’m getting ahead of myself. On this day, eight years ago, I was delighted that we were a little wee family, and I had my babe in my arms and that’s all that mattered right then.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit �#fundraising #teamMikaere #theMikaereFoundation #NICU from Instagram: https://instagr.am/p/DBgEb8Qtf7f/

On this day, 23.10.2016.

There’s something about enduring this – living without our boy. I personally still like my heart is anchored back in August, and that everything else (seasons, time, life) is just passing me by. I remember feeling similar eight years ago too, that my life is in NICU with my bub, and that anything that happened outside NICU was irrelevant and just, happened without my notice or knowledge. My life then was in this tiny incubator. My life now is a bit… lost. My heart is tethered to my son, who isn’t here anymore. I feel like I’m still standing here, with what’s left of my raggedy, broken teether in my hands, tears streaming down my face, and life is just… happening to other people. It’s happening to others. Me? I’m still back in August, to when I last held my beautiful boy.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #grief from Instagram: https://instagr.am/p/DBdfnONM0yP/

On this day 22.10.2016. This was diagnosis day. I didn’t know it, in this picture. In this picture I was just happy to have a chance at a cuddle. This was the first cuddle in literally days, and it was only 30 minutes. They taped the vent to my shoulder and I had sit very very still. It was heartbreaking, knowing that I desperately wanted to cuddle my baby, that I didn’t know the next time I’d get to, but equally, that if I moved, he would stop breathing.

I think almost every parent I know who has a medically complex child remembers their diagnosis day (except, of course, the parents of children who didn’t get a diagnosis 💔). It’s earth shattering. To finally know what’s going on, but not able to do anything about it. To worry about your beautiful babies future, to be told ‘global developmental delay’ and ‘life limiting’ and ‘profoundly disabled’. Seizures, dystonia, pain. I couldn’t imagine what raising a disabled child would look like, or what it would all mean, in reality. Here is what I would have told myself: it’s mostly just love. The tube feeding and the not walking and the seizures – love got us through. (It’s also love that’s decimating my heart right now, when he’s not here, but you know. I’d take every single moment over again, if I could).

He came so far, you know? In his eight years. Looking at this photo, and knowing that we had so many of these beautiful cuddles together. God, it hurts to know I won’t be able to cuddle him again.

NKH is just the most brutal, and I hate what it has stolen from us. This is why we fundraise though. To stop it from stealing life and joy from others, even if it’s too late for us.

#nonketoticHyperglycinemia #glycineencephalopathy #raredisease #nkh #ifhnkh #metabolicDisorder #inheritedMetabolicDisorder #cureNeeded #complexmedicalneeds #nkhawareness #nkhcansuckit �#fundraising #teamMikaere #theMikaereFoundation from Instagram: https://instagr.am/p/DBa60XoBFdy/