On Turning Two

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Recently our sweet boy turned two. TWO! Can you believe it? We welcomed the day cautiously, another birthday made. This feels different to his previous birthday – there wasn’t any fear. We were so fearful at his first birthday, that it was the only one he was going to have. That it was the only birthday we were going to celebrate (which is why we went big. Big party. Lots of people. Ridiculous cake and food and decorations and everything).

This year? This year it was quiet. A small family lunch with balloons and bunting and cake. Nothing too fancy. Here’s the thing though, Kaikai is so loved. He got a bucket load of cards (thank you all for your kindess in remembering!) and some sweet gifts from friends. We’re so grateful at the kindness others have shown. So grateful. So grateful he’s still with us, despite our doctors predictions. So grateful that he was well on his birthday. So grateful for his little smiles.

Grateful, and forever bittersweet.  But he’s well loved, and he’s pretty happy and content and right now that’s all we can ask for.

Happy birthday little man. We love you more than anything x

On our Seatbelt Medical Alerts

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I’ve talked about medical alert car stickers a while back, for alerting emergency response if there was ever an accident that Mikaere was non-mobile, non-verbal and an epileptic. It says in red Nonketotic Hyperglycinemia, so they can’t miss it. It also says Glycine Encephalopathy so they don’t confuse it with Nonketotic Hyperglycaemia, because you know someone’s in a rush that’s what they’ll read and if I’m not there to advocate for my baby you can bet they’ll test his blood sugars unnecessarily.

Anyway, what I wanted to say is that I found these medical alert seatbelt straps, for both the buggy and the car seat. They live on the straps, so we don’t need to remember them. Inside there is a little card with our details, the basics of his diagnosis AND a link to his medical care plan (essentially our cheat sheet).

It’s such a huge thing, my fear about not being able to advocate for Mikaere. About all those what if scenarios. I feel like we live in that space where all the unlikely things happen (a weird kind of luck?) and I want to give him the best shot I can.

So, seatbelt medical alerts. He’s got one on his car seat, and one on his buggy. A simple easy thing we’ve done to help stave off the worst case. Worth it, hey?

The place we got ours no longer sells them (womp!) but there are other places like here or here.


The Cheatsheet

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We have a cheatsheet for Mikaere. It goes everywhere we do, and is the first thing that’s handed over to nurses at A&E visits or admissions, doctors at appointments or any new therapist we have.

It’s literally a combination of every single question we’ve ever been asked, all the details people want to know, or didn’t know they should know. The nurses love it and the registrars love it too. Our consultants love it because it means we don’t have to go through the medical list and doses one by one. They can take the cheatsheet away, and we can use the appointment time more effectively.

It also means when someone asks something stupid, I know they haven’t taken the time to read the cheat sheet and I redirect them. It saves time. Theirs and mine and means I don’t have to fight to make myself understood, or worry that I’ve forgotten anything.

It’s also been helpful in other ways. The main being it signals that I’m a competent parent. That I know my child, I know his disorder, that I’m familiar with the medicalised setting and establishes that I’m an authority on my child.

It means that when it comes time to discuss an action plan, I’m included in the discussion as it’s taking place, not being told what’s going to happen after. It means when I disagree, I’m listened to, and my concerns are heeded. I can’t tell you how many times we’ve avoided being admitted because the cheatsheet gave me credibility when I’ve said no to admission.

The cheat sheet has just made our lives easier. For those that are interested, I’ve created a template here on google drive.

It’s the small things, hey? Cheatsheets. They make a difference.

On Stat monitors and o2 levels

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There are two little red numbers on the stat monitor, and I’m staring at them, willing them to go up. 92. 92. 92. 92.

92 is too low. I know the guidelines. He’s got to stay above 94 on 1L or less. I check the tank (is oxygen even coming out of this thing? Is there even any oxygen in there? But the needles on green, when I pull out the tube and crank it up I can hear the o2 rush out).

92. 92. Maybe he just needs a minute. I’ve put him down and he’s just fallen asleep. Asleep is when we need to pay attention, when he’s not moving about or working as hard. 92. 92. 92. 92.

Is the stat monitor probe even on correctly? I peel back the blanket to look at Kai’s chubby toes. He’s still, and the little red light is steady and exactly where I left it. It’s not the monitor. 92. 92.

I put the blanket back and reposition the mask slightly, bothering Mikaere in his sleep.

92. 92. 92.

Do I call our nurses? Not yet. I already know they’ll say if he can’t stay about 94 we have to go back into hospital. I don’t want to go onto the ward. I will, if I have to, but I don’t want to. He’ll catch something else there for sure, and I don’t fancy living in a half metre gap along side his hospital bed, sleeping on a plastic armchair that folds flat. With no sleep for anyone, gross showers and shitty food. No thanks.

92. 92….

Do I crank up the o2? Just to see if 1L isn’t enough?

Just as I reach over to the tank, the light flicks to 93. 94. 95. 96.

The relief is overwhelming. It’s not 92. He just needed a minute. I’m on edge, and have been the last few days. He’s back into the safe zone. I feel like I can breathe again.

We’re not in hospital yet, hey? Thank fuck for that.

On the isolation of the special needs life

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Over the summer my world got bigger, just for a moment. Sam had some time off, which meant that we could split caring for Kai. It meant some time for me. I worked, for a bit. Travelled some. Essentially I stepped away from the special needs life into what felt like taking back a bit of my life.

And I’m struggling to step back into the special needs world. Don’t get me wrong, I love Mikaere, with every fibre of my being and he delights me like no other. I love being his Mum.

But in the special needs life, I’m not just his Mum. I’m his nurse, and his chef and personal assistant and therapist and every moment is about monitoring and repositioning and anticipating his medical needs moment to moment. Watching the time for his meds, blends, flushes and sterilising. Repositioning him upright so he can cough, watching for those tiny moments where I can interject baby led physio, and a lot of the time not baby led Physio or OT or laser therapy. Keeping him entertained, or settling him to sleep.

It’s making myself smaller to fit into the very intense schedule of his day, managing the nurses and therapists and remembering appointments and chasing the thirty or so different things we’re currently juggling.

It was hard to leave Mikaere with Sam so I could have some time. It felt selfish. At the same time, it felt like falling into an oasis after walking months in the dessert. I got to speak to people outside of our nurses. I danced through some side hustles. People talked to me as if I was a competent adult. I didn’t have to fight anyone. I found some independence for me.

And now, making myself smaller, putting aside myself for the same same mundane, for the small moments, for the intensity of Mikaere’s day to day. I’m having a hard time with that. I find it hard to get moving, I’m leaning heavy on Sam and I’m spending too much time in the ‘what if’ daydream.

Most neurotypical Mums, they get to go work (or not, if they don’t want to). They can leave their kids with babysitters and have nights out with their partners. They can travel with their family. They can take public transport and take their kids where the crowds go. Their kids, eventually, grow to be somewhat independent. Where they can play without you sitting right there to make sure they don’t have seizures or aspirate or vomit or whatever else might go wrong.

I’m whinging, I know. The special needs life sucks so hard for everyone.

I read a book once, a fiction something a rather, where they played the champagne game. They’d open a bottle of champagne and bitch, for a moment. And then say something they were grateful for.

I’m gutted that I can’t go back to work full time, but I’m grateful we have the means so I don’t have.

I’m beyond gutted Mikaere lives a life less than the neurotypical life, but he’s here and he’s happy and content and I love him.

I’m sad that when we travel, it will always be split – Sam, Mikaere and I are unlikely to travel together internationally (boo immune vulnerabilities and planes), but I’m grateful that this summer I’ve had the chance to visit some phenomenal places, bucket list destinations.

I’m sad that our family doesn’t get to walk the neurotypical path, but I’m grateful I have a family, and we’re all well loved.

I’m sad I’m going back to my day to day with a heavy heart, but I’m grateful my day to day is still there to go back to.

Hey ho. Onwards we go.

On normalising death

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There are a few NKH kids right now who are struggling. Who are at home and ill. Who are in hospital on the ward. Who are in hospice, on what the doctors say is towards the end of their lives. It’s hard, being in a virtual community, physically so far away knowing these families are hurting, that these kids are suffering.

There are also many children who have fought their fight with NKH, who have gained their angel wings and are no longer suffering. Their families are still grieving, though. Still hurting.

And I say all these things, but I don’t have words for how deep the suffering is. How truly intense and horrific it is. I don’t have words for that kind of anguish.

I struggle. I don’t have words and I think this is all coming out wrong – how do I talk about this? How do I talk about the tears and the emotions just below the surface? How I’m constantly grappling with how I feel, with grief. Is it a relief for all the children who have passed? Will it be a relief for the children who are suffering right now, considering how much pain and hurt there has been in their lives? Is there relief for their families? Will there one day be relief for us?

Because that’s the thing, I can’t comprehend Mikaere dying. I can’t. But he will, one day, because NKH is terminal.  I don’t want death to visit us. I don’t want death to visit any of the families that are waiting for it right now. I don’t want the curtain of grief to envelop any of our NKH families. I just don’t.

And I feel powerless to help. The families who children have passed, the families that are struggling.  I don’t feel I can bring comfort to them. I don’t know how to support them. I send messages to let them know we’re thinking of them (and I am, all day every day) but it feels… inconsequential. I feel powerless to help our little family, and the endless waiting and fear for the terminal end.

There have been a few times we’ve waited for the news. A handful of times we’ve sat, anxiously waiting for that call, to hear when another child gained their angel wings. We’d send love and thoughts and cry. We’d talk about organ failure and seizures and that respiratory arrest is more likely to happen before cardiac arrest. We talk about the dignified death bill, and slower than slow breathing rates. We’re normalising the lead up to death.

I hate that this kind of conversation is normal for us now. That we as parents fight so hard to keep our children happy and healthy, and that we’re powerless in the end to stop the pain and suffering that comes with NKH.

I hate that our days with our kids are tinged with the word terminal. That sometime in our future with Mikaere we’ll be in the same position. That we’ll, one day, be watching his organs fail and be witness to his pain and his suffering. To his death. Just like other NKH families are right now.

I’m heartbroken. I’m absolutely heartbroken.

I’m talking about death and dying today because November 2nd, was NKH Remembrance Day. I’m late by a few days, but I want to pause and remember our NKH kids.

I want remember Alexander, the sweetest little sausage there was. I want to to remember Kaleb, and his cheeky grin.

Gregory, and his brother Elijah who is missing him. Halle Mae. And Cathryn. Maynak. Siem.

There are actually many more children. There’s a list. A list of 124 children who have died from NKH whose names should be remembered. There’s a slide show, even. And I started reading the names… there are so many names. Too many names.

And I’m aware that these names, they’re just the ones in our NKH group. They’re just the ones we know about. Other languages, other countries… they have their own groups with children who are dying before their time. They also probably don’t include the families whose babies died before they could even find support in the NKH groups.

Too many names. Too many children.

I don’t want them to be forgotten. I want to remember them, I want their families to know I remember them. To know their names, and their faces. They made marks, they made a difference. They were loved.

I held my little guy a bit closer today while I remember the others. And I think about how all of those children were so loved like I love my little guy. So loved and cherished. NKH can suck it.


Running 31 Days in October

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Mick sits one desk across from Mikaere’s Grandad, and today marks the end of the unbelievable challenge he’s set himself. Mick ran every single day in October, on every single one of those 31 days. And he did it for #teamMikaere and NKH Research.

There’s something incredibly powerful about someone running for our son. Knowing Mick has been out every day, convincing himself out in all weathers for us – you guys. There is something so supportive, so genuinely heartfelt when your day to day is encouraging others to do hard, challenging things, to keep on keeping on.  From a recent post, he said “While getting out and doing this every day has been a challenge, it’s been great to have such variety – and of course when we think about why I’m doing it, the challenge I’ve given myself is nothing compared to the challenges others are forced to face every day. Hats off to #teamMikaere.”

The acknowledgement of our day to day, the support and the act – not just words or platitudes, but the physicality of running every day, committing to it, following through and fundraising an amazing amount – that has made a huge difference to how connected we feel. We feel seen. We feel heard. We feel like we’re not alone. We’re here with Kaikai, and we know that without fail, every day this month Mick has been out running for us. What a guy. Plus today, on his last run, he ran in fancy dress (!)

Mick has raised over £2k for NKH Research – a phenomenal amount of money. It works out to over £70 per run (blows your mind a bit, doesn’t it?)

I know we ask, repeatedly, over and over. But if ever there was a fundraiser to donate to, please donate to this one: justigiving.com/mick-holton

Thank you Mick. What an amazing month, what an amazing effort. We are genuinely appreciative of your support, and are glad you’re on #teamMikaere.

On being in the A&E

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Mikaere’s had the once over by several people. His nurses have taken bloods, he’s had a nebuliser and is on o2. His gas and air look good (his lactates especially), his temp is holding steady and we had a visit from a PICU Nurse we know who came in to say hey.

So no red giant flags, and I have a chance between consultants to go get breakfast.

And as I’m walking down the familiar halls to M&S I think Saturdays are the best days to be here. There are less people in the hospital today. We had the bonus of coming in early, just after the shift change but before all the other kids had enough time to hurt themselves and fill up the a&e waiting room.

Is it weird that I think all this? That I know the time shift change happens? That I know when the lulls and peaks of our a&e are? That I come armed with a cheat sheet that answers 99% of the nurses/drs questions, and the two questions I’m asked (what is blood test for glycine on the system as – amino acid blood profile – and what colour is the bottle – green) – I can answer without really thinking about.

Our last a&e visit was months ago. MONTHS ago. I guess this is the winter season kicking off, right after a week of appointments and therapies after the summer break. I’m not happy we’re here, but now that we are I’m not panicking about it, too much. I’m hopefully we’ll make it home this afternoon sometime.


A few hours later and I’m worried. We’re still in the A&E majors, Mikaere’s still on o2 and sleeping, but he’s not holding his o2 levels above 92%. We need 94% or higher. We up the rate to 1L. That’s the borderline dose. It’s not a super high dose of o2… but any higher and we’re staying over.

The nurse notes say they’ve booked a bed upstairs on the ward (just in case) and I’m positive that if this was any other kid, or if I hadn’t expressed my concerns about the risks of being on the ward we’d be up there already for observation rather than down here. But down here is safer. Down here the A&E majors is filled with broken bones and a girl with ENT drama. There are no other chest infections or mystery illnesses in our room. The ward I know is probably filled with more things that pose a risk.

So we wait, we see. The idea is that the longer on o2 he is the easier it’ll be for him. His body won’t have to work so hard, he’ll have more in the tank for recovery. Or so we hope.

I’m worried though. I don’t want to go up to the ward. That’s the start of the path towards deterioration and I’m scared that each escalation will mean a step toward terminal.

But we’re not there yet. We’re not. We’re just in A&E still, just in observation. We’ll see.


The bloods are back. The chest xray clear. But Dr Erin has a game plan. She called our apartment a mini hospital. We have suction, a stat monitor and oxygen. We have all the medical equipment that Mikaere’s currently making use of on A&E.

I think that’s our saving grace. Kai’s just barely managing to keep his stats up with o2, and we’re able to wean to 0.5L when he’s awake. It’s a small wean, but it’s enough. We’re  discharged with very strict instructions of what to look for and when to come back. We have nurses calling to check in, and with a discharge note we’re waved off.

Mikaere’s not any better than he was this morning, but he’s not any worse. I was right to put him on o2 it turns out + I’m grateful for the reassurance.

Kai’s ill. He’s not holding his o2 levels up by himself. But we’re home. Thank fuck for that.

On the first hospital visit of the season

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And just like that our little man is back on oxygen, working overtime to keep his o2 levels up (and failing). It’s the first stupid cold of the season. We had a nurse overnight and when I walked in this morning she was just getting ready to wake us – Mikaere’s o2 level was sitting at 89%. Typically it’s at 99% – 100%. 89% is LOW. Lower than I’d like, lower than anyone would like. And you could see it. My baby was struggling to breathe. His breath was fast and shallow, he had a tug and an intercostal pull. He was working overtime to breathe.

We have tanks of o2 sitting in the spare room for moments like this, and I pulled one out, grabbed an o2 mask. It’s been forever since he’s needed o2, and you can tell, because the mask is too tight on his face. He’s outgrown the paediatric baby o2 masks. Regardless, it does the job. I watch with relief as his o2 climbs back up to more normal levels, settling at 96%. In the safe zone. He relaxes a little, he doesn’t have to work as hard. Poor baby has a temperature, and we give him Calpol.

I take pause. Get dressed, say goodbye to our night nurse. Sam and I discuss whether we should go into hospital. He makes coffee and I think about calling our community nurse. His levels are fine on o2, but he hasn’t need o2 in I don’t know how long. He’s clearly got a cold of some kind. If it’s a cold, we probably could manage it just fine at home. We have o2, we have stat monitors and suction machines. He sounds a little rattly, but I’m pretty sure it’s all upper airway.

If I call our community nurse looking for reassurance, she’ll ask us to go into hospital. Go into hospital. Complex needs. Difficult case. Better safe than sorry. Go into hospital. They always tell us to go into hospital ‘just to be safe’. Everyone is scared of taking the chance to say does he really need to go right now? What are the risks? Benefits? It’s all go into hospital, and go now. The risks of being wrong is too high.

But the hospital isn’t safe for us. Mikaere could very well catch something else from the hospital. Something worse. Plus, it’s loud and tiring and disrupts our routine, and I’d really like NOT to spend our Saturday in the A&E.

In saying that… he hasn’t needed o2 in forever.

I make the call anyway, knowing she’ll send us in and alert the paeds registrar for us. Also, on a Saturday morning at 8am, none of the other kids have had a chance to hurt themselves yet. Their parents will just be waking up and they’ll take longer to decide to take their sick kids into the A&E. If we left in the next 15 minutes, we’d make it in just after the shift change when the paediatric A&E is quiet. I mean, if you’re going to go in, might as well time it for non-peak time, right?

Sure enough, our community nurse said to go in.  So off we go. We’re on our way into hospital. 🙁

On feeding into the world of pretend

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I talk a lot about the ‘what if’ daydream in my head.  The ‘what if Mikaere never had NKH’ daydream.  I think goes hand in hand with grief, with loss. When you grieve something has happened that you didn’t want to, the flip side is that there was another option, another path, another something that you expected/hoped for/wanted. For us, obviously, it’s that Mikaere didn’t have NKH. That he was neurotypical. That he didn’t have two little missense mutations in one tiny gene.

Daydreaming about what it would be like if Mikaere was neurotypical is a dangerous, dangerous past time. It’s like a sink hole, so I shy away from it. At the same time, I also want to confront what Mikaere is not – I want to rip that band aid off so that it doesn’t hurt so much the next time I’m faced with the gap. So that the developmental gap that’s widening by the day isn’t so unexpectedly large that it’s crippling.

So I straddle the line between hanging out with kids that are Mikaere’s age (thanks to our wonderful NCT friends and their gorgeous babes), the grief that he’s not developmentally where they are and imagining what it would be like.

I’ve noticed recently that when I’m having a hard day, when we have back to back appointments or Mikaere is projectile vomiting everything or the seizures are uncontrollable – I have a bad habit of leaning towards the daydream. If I’m writing posts, I’ll post the photo where Mikaere looks more neurotypical. That if you weren’t here behind the camera you wouldn’t even know. I like the photos where Mikaere looks neurotypical best.

There’s one photo I love. Friends of ours have a son that was born on the same day as Mikaere and we went to visit. I lay Mikaere down on the floor where he’s most comfortable and T roamed, as a toddler who has independent mobility is want to do. But there was a moment where T lay down next to Mikaere and they both laughed.

I love that photo. I love that T lay down and was the sweetest little guy. That he wanted to do what Mikaere was doing, with zero knowledge of disability or difference or anything. I’ll forever love T for that tiny tiny moment.

But I realised I love that photo because it looks like, just for a minute, what a neurotypical life could be like with Mikaere. It feeds into the world of pretend, if you weren’t there – it looks like two boys playing together. The reality is they’re not. T had a wonderful, kind moment with Mikaere before he toddled off and Mikaere had a moment of awareness that T was there, but the truth is they didn’t play together.

It’s insane how to the very depth of all I am I wish they had been. How much I wish Mikaere was neurotypical. He’s not, I know. But I struggle with the cognitive dissonance of knowing he’s not, and the emotional intensity with which I wish it was otherwise.

It’s been almost two years of this cyclic grief. Over and over and over again I battle with this. Over and over again I’m faced with such grief that Mikaere suffers. And when he’s not suffering, when he’s happy and content and smiling at us and making small gains – that his life will never be as full or as varied as a neurotypical life.

That he will never love romantically, to know those butterfly feelings when you meet someone. He’ll never know what it’s like to travel independently, to delight in discovering a new place with new people living differently to you. He’ll never know what it’s like to work hard and be considered an expert at something, he’ll never know the satisfaction of when your peers recognise something you’ve achieved. He’ll never know what it’s like to snowboard, to pick a line, to make fresh tracks down a powder black run on a bluebird day (Its been a long time since I’ve been to the snow, but it remains one of the greatest joys I’ve ever had). He’ll never know what it’s like to kite surf, or boogie board or cook an amazing meal for the glory of it. He’ll never sit in a side restaurant in Japan eating the best oyster of his life, or jump off the back of boat into crystal clear waters in Croatia. He’ll never be able to introduce someone he loves to the things he loves. He’ll never adventure with a group of friends, getting up to mischief. He’ll never feel the satisfaction of creating something others can’t.

These are my life highlights. I’m devastated the highlights of his life will be smaller than mine. And I know that these are all just the highlights, and he’ll also never experience the downsides of a broken heart or the culture shock of being somewhere out of your depth or break a wrist taking a jump with a bad landing. But fuck, aren’t the highlights worth it?

And the hard thing, the thing I really struggle with – this grief is cyclic. It’s never ending. For as long as Mikaere lives – and past that, I suspect for as long as I live this is something I’m going to carry. This big dark grief that my child will never have the opportunities I did, that he’ll not live a live as full as others and he’ll forever be disabled.

I know for certain it’s this grief that pushes me to fundraise. That pushes me to figure out the next thing I can do to raise more money for NKH Research. That this big, dark stupid grief fires that ‘do something about it’ tick I have. And so I push and I design and I build websites and read research papers and oh god, the whole time I’m furiously wishing my son wasn’t disabled.

He is though. So onwards we go.

PS – if you want to donate, as always, we’re fundraising at justgiving.com/team-mikaere. All funds go to Prof. Nick Greene, who is researching a cure for NKH.