On lunch in a park

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After the hospital appointment last week, we stopped in at Regents Park to eat our (home packed) lunch. There isn’t a safe place to eat near the hospital, no place to be socially distant and not worry about someone walking too close without a mask.

Regents Park is huge, and was on the way home so off we went.

It was nice to be outside, to be somewhere different. But to be honest, I was so highly strung. Anytime I caught sight of a person in my peripheral vision my entire body would go into high alert.

No one was wearing a mask (apparently people don’t feel the need to outside) and people weren’t really social distancing (and if they felt they had to pass you on a footpath, they would. Instead of waiting further back or crossing the road or any of those other things that require a bit more thought or effort).

So yeah, we sat outside and it was nice but I was too highly strung and anxious about strangers to really enjoy it.

But Mikaere, oh my days the joy. He enjoyed being out, he had his feet in the grass and the wind in his hair and ginormous grins with little happy chirps.

He was happy to be out, for sure, so I’m glad we took the opportunity when we did.

Because when I looked at the gov stats this morning, I saw this giant spike of positive cases. You can try minimise it all you want (and I’ve seen people try, using smaller numbers, or reducing to x per million, or it’s not in my area or trying to say it’s because testing capability has improved) but the long and short of it is that no matter which way you cut it, THOUSANDS of people in the UK are testing positive everyday. THOUSANDS of people have covid and are contagious.

We only need one person, just one, to be near us and we’re down the rabbit hole of doom.

Looking at that spike makes me feel ill. The risk for us is real. Like we were risky to go out, and sit in a park.

And the hard thing is no one else seems worried. Schools went back this week. Pubs are still open. Friends of mine are openly travelling (!) like it’s no big thing. And I get it, people are fatigued. But if the cost was death to someone you loved, would you go to a pub? Would you go get your hair cut? Would you get on a plane?

Please stay home. Please wear a mask. Please wash your hands with soap more than you think you should. The safety of our family relies on others like you doing your part.

On a hospital visit

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 We left the apartment, first time in over 120 days (17 weeks, or 4 months), thanks to some unavoidable in-person hospital appointments. It’s been a long, stressful morning, and I’m sitting in a waiting room by myself. Mikaere’s had a seizure seconds before he went in (why is it always so hot in waiting rooms?! Pretty sure the warmth triggered it) and I can hear Mikaere breathing, in the little haematology cubby. The nurse is giving Sam instructions, and I’m holding my breath waiting for the cry that’s certain to come when the nurse tries to takes my babies blood. This never gets easier.

It’s worse, with covid. This is the first time we’ve been outa and my anxiety is sky high… why aren’t people wearing masks?! Why wear a mask if it doesn’t cover your nose?! I’ve spent all morning screaming “GET AWAY FROM MY IMMUNE VULNERABLE CHILD!” in my head. Wear a mask. Even outside. Any time you might possibly come within a two metre range of people (like on a FOOTPATH) wear a mask! It’s not hard. You could be literally saving someone else’s life. Put another way – if you don’t wear a mask, you could be killing a vulnerable person, like my baby boy. You don’t know, you could have covid. You could be putting people like Mikaere at risk. Wear a damn mask!

Leaving was made easier because we were running late. The urgency to get in the car with all our gear made stepping out the door easier, and it wasn’t until there was a guy (without a mask!) arguing about our parking spot that my deep distrust of other people came to the fore.

I hate this. I hate being out here. There are so many people, and so many people who aren’t social distancing or wearing a mask who clearly have the privilege of health, of not worrying about if they get sick, who don’t care about other people. That is not us, and I worry. This doesn’t feel like a nice jaunt out, it feels scratchy, and uncomfortable and risky. I feel panicky anytime anyone comes remotely close to us.

But we’re here because we need to be. We have appointment after appointment (metabolic and bloods and orthotics and radiology) and they all needed to be done. We’ve crammed six months of appointments into one day, one visit, to reduce the risk.

Mikaere’s squeal pierces the silent waiting room and I find it hard not to cry. I hate this. I can hear that’s he’s miserable and I’m rooted my chair, I can feel his cries through my chest. God, this is awful.

There’s a pause. They’ve fucked it up, I know it. That cry was too short. I know he’s a hard stick, and he’s so much bigger and he wriggles more. I’m not wrong, and a few minutes later there’s another scream. He’s distraught and I’m sitting a whole room away. Oh god, I hate this. I hate that he’s in pain, that we’re causing this just “for to check” and just, fuck.

I hate that we’re in a world we were don’t feel safe. Where people aren’t wearing masks, and social distancing, where my baby has unavoidable hospital appointments and has had so many needles in his arms and hands and feet that his veins make it impossible to retrieve blood.

I am not loving the world right now. It seems cold and cruel. I’m crying. I hate that this is our reality. The special needs life can suck it so hard.

Neverending Isolation

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This guy. So, I think we’re (touch wood) out of the last seizure war and into a place of relative calm (I say relative calm because he’s not seizure free, but it’s not the 20+ episodes with weird new presentations we were seeing before). We did lots to bring him out (mainly med adjustments for weight, and opened a new bottle of possibly oxidised oil), but as always its slow and takes time to kick in, and the recovery back to baseline is heartbreakingly slow. But there have been more smiles, and more awareness and more vocalisations (this kid has opinions!)

But. We’re still in this place. 25 weeks of isolation. Today is day 175. We’re still sleep deprived (getting up at 3am is not any easier 5 months later) and I still have all the anxiety about what’s safe for us.

Here’s what we know: the risk of Mikaere getting covid19 is worst case scenario (aaand we live the palliative care life, so worst case has a different, very real meaning for us). We know that covid is still rife outside our flat. Over 1k of people in the UK tested positive YESTERDAY. Last week over 9000 people died (400+ more than the week before).

Just, not fully knowing what needs to happen for us to be able to be safe outside is really doing my head in, because the honest truth is that it may never be safe. And if it’s never safe, then it becomes a balancing act between quality of life and the possible quality of death.

And the never leaving the apartment isn’t the kind of quality of life we want for Mikaere… but, the thought of taking the risk and going out, with the very real risk he might suffer unnecessarily/die is paralysing, you know?

I don’t know how to align those two things, and I’m all over the place about it. How do we provide the best possible life for Mikaere, considering the dangers? I’m struggling so hard, and I don’t have an answer.

I suspect this is even more difficult because in our little bubble we’re sleep deprived and without a wider perspective and without all our usual support (from our teams, therapists, groups, friends). It feels like we’re trying to make safety life/death decisions in the dark.

So. What would you do? Would you go out if the risk was death?

Trying to navigate blind

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I’m wary about posting this. When there are so many things out of your control, and those same things are basically what makes your heart beat, you become way more superstitious and faith going person than before.

So I’m wary about telling you that Mikaere’s seizures have lessened because the universe might hear and laugh and send more seizures our way.

His seizures haven’t stopped, but the frequency has definitely decreased.

Because the seizures were presenting differently, because they came on so suddenly, because they were relentless, it’s been a stressful few days.

Do we take him in to a&e to be reviewed? No, because all they’ll do is take bloods. They’ll swab for an infection he doesn’t have (because we’re still in isolation, day 164 today) and we won’t get the important plasma results back for two weeks, so going in is basically a risk (cause global pandemic, with little to no benefit). And at that point, it’s on *US* to figure it out.

So we do all the things. We weigh and adjust his meds based on any weight gain, and we video what the new seizures look like (is that movement really a focal seizure? Could it be dystonia? Could it be spastic movements or chorea?)

We go down a rabbit hole of research, reaching out to people we know in the community to understand what it typically looks like, how it’s treated, when it came on for other kids.

Spoiler: not dystonia or spastic movements or chorea.

We check the use by dates on all the meds, make sure the meds are what they say they are on the label, check they’ve been stored correctly and haven’t oxidised.

We try figure out what he might have come into contact with. Is he too hot? Too cold? Is he in pain? Are any of his muscles unbearably tight? Has he lost any range of movement in his limbs that might indicate pain? Are there any rashes or spots or anything that might give an indication?

Is this NKH progression? (I hate considering this, because it means there is nothing we can fix).

And so we sit. With our anxiety, asking Mikaere to please breathe while he’s having a seizure, cuddling him close after, reassuring ourselves that he’s still here, still with us.

We’ve already made the med changes, so we need to wait and see before we make any other changes.

But it’s hard to wait, and it’s hard to just watch and there’s definitely a toll on everyone (it’s hard not to feel like a failure, when you can’t help your kid when they’re suffering).

And slowly, the time between seizures stretched out and the frequency came down and the weird focal seizures went away and the tonics we’re used to seeing took precedence.

But it’s hard to trust this lull. It’s hard not to be on full alert, hard to trust that there might be less seizures today. That we might get awake time and maybe even a smile. It’s hard to relax into this deescalation and trust it will hold, while at the same time desperately wanting it to.

The special needs life, hey? Seizures can suck it.

Seizures are awful :(

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I’m holding the sedative meds, I’m ready. Waiting. Because I know it’s coming. I know that I’m going to have to sedate my child. Again. And that if I don’t, he’ll keep having seizures. Over and over and over again.

Around 4am there was a twitch. A slight lifting of the elbow and a tense shoulder. For a *moment*. I saw it, but before I could figure out if it was a seizure he’d relaxed. And then it happened again. And over the course of an hour it happened over and over, for longer and longer until they were recognisable as seizures. I need to wait for one more, as per the seizure care plan. Three in an hour, that’s the threshold. But I know it’s coming.

You’d think I’d be used to it now, but knocking your kid out isn’t an easy thing to do. Well, that’s a lie. It isn’t hard: position him so he won’t choke, pop the lid, squeeze half between his cheek and gum on one side, the half on the other side and rub his cheeks until he crashes out. Physically it’s one of the easier cares (way easier than nasal suctioning or button changing or fighting with the Lycra suit).

Emotionally? I feel like I’ve been pummelled. Emotionally I would like to never see my kid suffer seizures again. Emotionally I wonder if this is the beginning of the end (it’s not, universe. I didn’t put that out there so you could run with it).

Because it’s a never ending fight. Trying to figure out what’s causing them, trying to get them to manageable levels so he can experience some kind of quality of life, with awake time and development and (and in my head, I want to cry, because my sons quality of life comes down to him BEING AWAKE, and not, you know, every other joy a three year old might experience).

Honestly, the weight of this hand we’ve been dealt gets harder to carry. The never ending seizure war. NKH, you’re awful and I hate you.

Isolation and Backsliding

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We’re struggling. Without our giant team of therapists and nurses and all the many many people in our home who help us help Mikaere… we’re operating at a completely different level to pre-covid. The bar is much lower. Trying to get stretching and physio and therapy in…

Bluntly, it’s not happening, and I can see Mikaere’s development backslide as a result. It’s heartbreaking, and managing the guilt of we’re doing our best and knowing that our best isn’t good enough… because it can’t be. Two people can not replace the team we had. We’re trying to be everything and… we can’t. We can’t be night nurses and physios and SALTS and CCNs and OTs and vision therapists. That list doesn’t even include the fun groups, the social sensory time, or swimming or yoga or horse riding. Trying to find the mental capacity during the day to get in the basics of stretching and the physio and the equipment… it’s not happening.

We pulled out the walker today. The idea is not for him to walk, but to spend some time in a different, upright position, feeling weight through his legs and ankles. It’s been a while.

He spent a good five minutes of the fifteen he was in it not wanting to put his feet down. He’s miles away from where he was, and just… it’s hard.

Quality of life wise we’re doing our best, but how do we calculate risk/reward in this case? The risk of allowing people into our bubble? Most of the UK has normalised living with Covid. But when the risk for us is death? How do we normalise that? Is that risk, his death, worth allowing our team in to help support us support Mikaere? To give him a better quality of life? In NZ, our family is a vicarious no, because they’ve normalised life without Covid. In the UK, our friends shrug and ask what would need to happen for us to open our doors, and he’s terminal anyway, so shouldn’t we bet on quality over quantity?

I’m torn and I’m scared and I don’t have the right answers. I don’t know what the right answer is. How long can we keep living in isolation like this? It’s been months and months and months.

I can’t help but feel like there is no right answer, and either way we’re failing Mikaere. On the up side we found out it costs half a million pounds to hire a private plan to fly us to NZ. Where we could be safe. Anyone have a half mill handy they want to donate to our covid-safe migration? 🤣 I wish!

On early mornings

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Every morning with this one, when it’s quiet. The suns just coming up and London is quiet. No cars or helicopters or pedistrians. It’s cool (thank goodness), and quiet.

It’s in these early moments I wonder what kind of day we’re going to have. Will there be smiles today? Will he be awake and alert and want to play? Will there be Seizures? Vomits? Recovery naps that span hours and hours and hours?

I don’t know, yet. Right now he’s still sleeping, still cuddled in close and he’s peaceful.

Today is meant to be less hot (hopefully!), though we’ve been managing quite well with the heat (thanks for all the suggestions re: a cool mat. We were lucky, a very kind neighbour ran down to our local and picked one up. They’re magic, aren’t they?) but we’re all done being uncomfortable and sticky. We’ll see.

Another day in isolation (were Day 152 today). Onwards we go.

The correlation between heat and seizures.

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Another warm day, another day of seizures and struggle. He has both a portable ac unit and a fan pointed at him. He’s lying on the floor because it’s 1.4 degrees cooler than the couch, or a chair. I love the warmer weather, but not when it causes seizures. They’re happening every few hours. Just long enough for a recovery nap and some minor awake/aware time before another one hits. Blah.

Seizures are the thief of everything, and I hate them. They take away so much. At this point, what kind of quality of life is this? Seizing and recovering, not moving, no joy.

This is what NKH looks like. Seizures because it’s warm. The brain damage that’s happening in my little guys brain because of a tiny tiny mutation which fucks up a large chunk of his carbon folate metabolic system….

Living the super special needs life atm. We’re one of many, our story is standard, not exceptional. But that doesn’t make it any less heartbreaking 🙁

One of those days

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Today’s been a whole day. Of seizures and vomits and screaming and unhappiness and this face, this face that says so clearly he’s feeling sorry for himself? Yeah. That’s our today.

The shit thing is that this is our status quo. And I’m torn, I’m torn between pulling back the curtain and feeling vulnerable so you can see our reality, or resisting the pull to put some kind of positive slant on it or whether I should just delete this post because to be honest, today is a hard day. It’s one hard day of many hard days, because that’s just how life with NKH goes.

It’s hard for us to continuously share the hard things. It’s hard for us to then manage other people’s sadness at our hardship. It’s hard to navigate all the feels, because at the heart of it, no one wishes we weren’t living the NKH life more than we do (watching our son suffer and not being able do anything is our own special kind of living hell).

And I could, I could paint this with the positive brush. Of what we’re grateful for and focusing on the positives, but I don’t want to diminish this… this is our reality and I don’t want to positive-pretended make it palatable for others consumption.

So, honesty then. Today’s a hard today. And honestly, I’ve never hated NKH more.

#nonketotichyperglycinemia #specialneedslife #seizuressuck #nkhcansuckit #itiswhatitis #hardday #seriouslywherearethecustarddonoughts

On the The Online Charity Pub Quiz

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TLDR: Quiz was a raging success. We’re extending the quiz slightly for the next week for those who couldn’t make it – for a £5 donation, if you can correctly guess the movies and characters from the picture rounds, we’ll put you in the draw to win a surprise, silly, light hearted prize.  Photos under the post!

Donate here:  http://paypal.me/teamMikaere
Draw will be made on Friday 31st.
Rules: Open world wide. Add your answers in the note (please also untick the goods and services box, else the transaction fee will come from your donation).  Donations will be matched, so please dig deep!

Additional side notes: #5 – any of the movies he appears in is fine. It’s hard to know exactly which film this image is from!

———

WELL! I don’t even know where to start – the online pub quiz was a raging success!! Tony and Tess did a PHENOMENAL job of running our quiz. The rounds were hilarious, some questions were easy, others were more difficult (we’re rubbish at quizzes, so I was pleased to be able to answer some of them – though I found the music round to be particularly entertaining!)

There was an NKH round which, if I’m honest, I struggled with. I did a bit of a spiel and honestly, I hate doing the spiel. We do it because spreading the word about NKH and the realities of it is important, but oh. Telling people your kid has a terminal disorder, and is likely to die and lives a really grim reality is emotionally hard. I’ve been doing the spiel for over three years now, and each time I get teary. It’s important that people know, but oh. It’s so hard.

We’re grateful that people are kind, afterwards so many people sent a lot of lovely messages sending their love and support. It sounds really stupid, but making ourselves vulnerable, and opening ourselves up – even at just a quiz – it’s really really personal to us.

I had to take a mini break mid quiz after the spiel, it’s been a long time since I’ve had to say out loud, to an audience what life with NKH is like. Listing symptoms and average death ages and just – just how limited life with NKH is. We try so hard to put a positive spin on things, because how else do you live with the day to day of grim? But peeling all that back and being truly honest,  it cuts down all the defence mechanisms I’ve put in place to manage.

But, like I said, we have hope (and I hold so hard to that hope it’s ridiculous). In research, and a gene therapy cure and that one day our reality will be a thing of the past for families like ours, because life with NKH WON’T EXIST.  There will be an EFFECTIVE TREATMENT! (I’m getting shouty, because the idea that kids won’t need to suffer seizures and developmental delay and be severely disabled and die because of NKH is HUGE)

Which is why I do the spiel, even though it’s hard. Because the hope and the dream is worth it.

I’m grateful for Tony and Tess smoothing over my emotional moment with grace and kindness, acknowledging the hard and gently guiding the tone of the quiz back to fun and light while not taking away from the purpose. Honestly, they’re such pros.

And YOU GUYS! We raised £1880, which will be matched, thanks to Tony’s employer.

But we think we can raise a smidgen more (the NKH cure dream is strong, you guys). So we’re opening up the Picture Round for everyone who couldn’t make it on the night.

for a £5 donation, if you can correctly guess the movies and characters from the picture rounds, we’ll put you in the draw to win a surprise, silly, light hearted prize.

Donate here:  http://paypal.me/teamMikaere
Draw will be made on Friday 31st.
Rules: Open world wide. Add your answers in the note (please also untick the goods and services box, else the transaction fee will come from your donation).  Donations will be matched, so please dig deep!

Additional side notes: #5 – any of the movies he appears in is fine. It’s hard to know exactly which film this image is from!

Side note: if you’d like to donate and not play, thats okay too. If you’d like to donate more than £5, or less, we’re grateful for any contribution!

Please get involved, we’d love to hit the £2500 mark.

Again, thank you to everyone who came to the quiz. We had 37 teams from all over – in NZ, the US, the Netherlands, Scotland, England. It was mind blowing. To everyone who donated and sent messages and was so clearly on Team Mikaere. We love you guys. We couldn’t live this life without your support x

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How to play:

  1. Make a donation to: http://paypal.me/teamMikaere
  2. In the notes name the movie and character!
  3. (Surprise, silly and light hearted) prize drawn 31 July