All Posts By

Ellly

Oct 01
0

Meet Charlie

By | #teammikaere | No Comments

I’d like to share a project we’ve been working on. Introducing Charlie.  Charlie is the protagonist of the next charity children’s book. Eva was *such* a success, it seemed like a good idea to add another book to the line up.

I’ve been working these last few months with Zoe Ëllison,

who is an illustrator – that’s right, no more will you need to suffer my amateur illustration skills. Instead, Charlie is *beautifully* illustrated by a *real illustrator* (much excitement and dancing, because Zoe is AMAZING!)

You guys, meet Charlie.

Charlie is a crow, who is super good at honking (and who doesn’t love a good honk, hey?) Except… the other crows make fun of Charlie (boo!). So Charlie goes on an adventure to find other birds who sound like him. It’s pretty cool. A book about gently accepting differences – being different is okay.

The other super cool part is that this book has been designed as a sensory book. Sure, the book up front is exactly as you’d expect, pictures and words and a story. Buuuut the back half is full of suggestions for how to experience the story with your other senses. There are AAC symbols, and cut outs so our CVI friends can isolate each bird, there is a link to audio cues so you can hear each bird call, and there suggestions for things you can touch, or smell or feel. Inclusivity for our disabled friends with sensory or learning differences – it’s important.

So here’s my ask.  Eva was translated into so many different languages by volunteers (THANK YOU!) and I’m positive that’s why Eva has been so successful and raised as much money for NKH research as she has.  Would you be willing to help translate the Charlie book into a language you speak natively? It’s approximately 1250 words all up. All languages welcome, and you’d get translation credit.

But here’s the thing – last time I put all the words into a google spreadsheet, made it public and let everyone go to town. Some rascal altered some of the translations into naughty words. We caught it before the books could go to print, but it’s meant a lot of work on our part.

If you’d like to help translate the book, or triple check a translation to make sure the meanings are accurate (and that there are no naughty words!) please fill out this form here:

https://forms.gle/Vt4V2NjeGKojWN93A 

I’ll email you back with your very own spreadsheet (if you want to translate, or a copy of a translation if you want to check). That way we know who you are, and hopefully no rascals will interfere this time.

I’m so excited for this book. As before, every single penny from the sale of these books will go to directly to Joseph’s Goal, for NKH Research.

NKH – were coming for you. We’ll fund a cure one way or another.

Sep 29
0

On the changing table

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Speaking of equipment, we got this behemoth changing table put into our bathroom. It’s great, in that the bathroom has a hoist and we can now hoist Mikaere from the changing table to the bath. That changing him is significantly easier and all in all, it’s a huge improvement over the bathroom floor.

It’s just – it’s so big. The installer didn’t quite install it all the way over, and so it’s difficult to slide in and out of the room when the table is down. We also had drama with him wanting to do the electrics – so while you can plug it in to the mains to move it up and down to an appropriate height… you have to do that with the door open. It’s so frustrating.

At this point we’ve been too busy to chase the small details – so live with it unplugged. But honestly, I wish the installer had just done what he was meant to do, and had done it properly. It’s so hard not being able to trust people do things right, you know? And now we’re in a position where we have to pay to have everything sorted out.

Still, not changing Mikaere on the floor is a win, so I guess we’re mostly in the positive?

#greatChangingTable #stupidInstaller #disability #nonketotichyperglycinemia #nkh #nkhawareness #ifhnkh #metabolicdisorder #teamMikaere #glycineencephalopathy #nkhcansuckit #someoneFundACure #cureNeeded #disabledparenting #disabled #disabledfamily #complexmedicalneeds #metabolicdisorder #nkh

Sep 27
0

On a Useless Hoist

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We have this stupid portable hoist in our living room. It’s not long enough to actually be of use (it fits over Kai’s bed, and then literally just the half metre next to it). We used it initially getting him in and out of his crib, when we had the crib, but now he’s got his bed it’s just… it’s not a good fit.

We’re still waiting for a ceiling track to be put in

I can’t wait to get rid of it, honestly. It doesn’t belong to us, it’s from the council. So I have to organise to have mediquip come and dismantle/pick it up. But to do that, they need to come in. And with cover, I’m not keen. So we have the big giant (but not giant enough) hoist was just sitting there, not being used.

SO, I’ve pulled it out and now we use it as thing to hangs toys from. It’s huge, and silly but also, kind of fun? It’s definitely not what it’s meant to be used for.  Hey ho, we’re making it work for us!

#stupidHoist #disability #nonketotichyperglycinemia #nkh #nkhawareness #ifhnkh #metabolicdisorder #teamMikaere #glycineencephalopathy #nkhcansuckit #someoneFundACure #cureNeeded #disabledparenting #disabled #disabledfamily #complexmedicalneeds #metabolicdisorder #nkh

Sep 24
0

On Ophthalmology

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As everyone here in London is just Getting On With Life as if cover wasn’t a thing, there was pressure to take Mikaere into his Ophthalmology appointment. You can’t really do ophthalmology over zoom, and it’s been two years so… yes. With much anxiety and mask wearing (even Mikaere) we went and saw his ophthalmologist.

Mikaere has CVI (cortical vision impairment). We know that. It’s not clear what he can see, what connections are getting through to his brain. There’s also an issue with the tone of the muscles around his eyes, which means one eye floats in a bit (it’s called strabismus). We also found out this time that his eyes are oval shaped – called astigmatism. That’s not NKH related, as Sam has that too. Mikaere is also long sighted. So it’s all a bit complicated, but mostly fine.

The lovely ophthalmologist looked at Mikaere’s eyes, and shone various lights and made some decisions. Mikaere thought it was generally hilarious, and was very cheeky, holding still just long enough for her to almost get her readings, and then turning away with a laugh (he thinks he’s hilarious!)

Anyway, the short of it is Mikaere’s getting glasses. Again. He actually had glasses briefly almost two years ago, but after a while he refused to wear them. The ophthalmologist then said it’s likely that his eyes changed shape and the script was likely off. He was right, I guess.

But awkwardly because of Covid, the spectacle dispensing office was closed, and so we need to wait for another appointment to have a fitting.

Still, I feel like it was good to go, and novel for Mikaere to be out and about and meet people outside our bubble (!)  I guess stay tuned for the glasses reveal?

#disability #nonketotichyperglycinemia #nkh #nkhawareness #ifhnkh #metabolicdisorder #teamMikaere #glycineencephalopathy #nkhcansuckit #someoneFundACure #cureNeeded #disabledparenting #disabled #disabledfamily #complexmedicalneeds #metabolicdisorder #nkh #spectacles #strabismus #astigmatism #eyeCare

Sep 22
0

On School Transitions

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One of the changes that happens now is Mikaere’s therapy is done at school. Where I am not, which means I can’t be there to advocate for him. This here is Mikaere in his stander at school. This is not the stander we trialled at home. It doesn’t have head support he needs, or support behind his elbows. You can see how his chin tilts up, which is problem, because then secretions pool in the back of his throat which means more gagging and vomits and breathing difficulties. I can’t see where the strap is, in regards to his tube – is it rubbing? Is it accessible? I can’t see how he’s moving in it. Is he comfortable? Is he extending against the straps? Is anything rubbing? How is his feet? Are they turned out? I can see it’s not the superman pose we set up at home (because it’s better for hip abduction). And I’m so frustrated. So frustrated that this was done without me.

Because of course it’s now all going to be done without me. He’s at school now. I’ve been told repeatedly that his independence is important, for us and for him, and that letting him have that is necessary.

The tug here is that I genuinely feel like no matter how great his team is (and they are great), they are not as great as me. They don’t know him as well, they don’t know about NKH as well as I do, they’re not able to anticipate like I do, and they are not his parents.

There is also this frustration that if his time is limited, he’s spending it with people who aren’t his family. With people who aren’t US. They are getting all his awake moments, all the beautiful interaction and fun. And we get the tired Mikaere, who is so exhausted after school that he sleeps.

I’m struggling with this transition. There are (obviously) benefits (for us and him) that he goes to school and we get daily respite, but it’s not a black and white situation. I don’t know how to advocate for him when I can’t see what is happening with him. I’m not the one making decisions for him, and I’m not the one ensuring that he’s getting the best out of his time there.

Instead my mama heart is trying to be okay with ‘good enough’. This stander is not the one that we trialled. It’s not set up the way that I would set it up, and it’s not as supportive as I would like. But. But he is standing, which is good for him. It’s good for his hips and muscles and digestion and I bet it feels really good.

I hate this. How do I advocate, and how do I settle for ‘good enough’ when for the past five years I have done everything in my power to ensure Mikaere has everything, and more that he needs?

#disability #nonketotichyperglycinemia #nkh #nkhawareness #ifhnkh #metabolicdisorder #teamMikaere #glycineencephalopathy #nkhcansuckit #someoneFundACure #cureNeeded #disabledparenting #disabled #disability #disabledfamily #complexmedicalneeds #metabolicdisorder #nkh #transitionToSchool #standingFrames #hateThis

Sep 20
0

On Eva the Adventurer

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When we first released Eva the Adventurer, this is how small Mikaere was. He was teeny tiny back in 2017 (!!!) Most of you brought a copy – you know the story. I was desperate to *do* something. To help fund a cure for NKH, to raise money any which way I could – and so, despite my lack of illustration skills I wrote and illustrated, and published Eva. So many of you volunteered to translate it into 23 (!) different languages, and just, woah. I put it up on Amazon, and then life happened. I’ve not marketed it or really mentioned it. But, the universe kind of stepped in and helped.

As of this month, we’ve sold just over 4000 copies, and raised £5,000 for NKH Research. That this little book has raised so much for NKH research is just… I couldn’t have imagined we could have raised that much with a book. I’m grateful, for everyone who has purchased a copy, who has shared photos of your kids enjoying it, who has gifted copies – you guys are AMAZING and just, thank you.

They’re available on Amazon, please buy a copy if you’re able: https://www.amazon.co.uk/s?k=eva+the+adventurer

The book is bilingual, and available in many languages (the top 10 of 2021 are: Dutch, Swedish, Welsh, Africans, Irish, Finish, Spanish, Italian, Scottish, German. English on it’s own doesn’t even get a look in!). If you don’t have kids, please consider purchasing a copy and donating it to your local library, play centre or school.

If you’ve already bought a copy, would you mind leaving a review? It turns out reviews power purchase decisions! #bazaarvoice #otod #dontStopBelieving

Thanks hey. We couldn’t have raised this much money for NKH Research without you. All the royalties are deposited directly into Joseph’s Goal’s bank account. We don’t see a single penny (hurrah for making things easy!)

#EvaTheAdventurer #nkhAwareness #nkhCanSuckIt #bilingualBooks #kidsLit #kidsbookshelf #kidsbookswelove #childrensbooks #kidsshelf #kidslit #raisingReaders #booksForKids

Jun 15
0

On Ava + Willow

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I’m just… devastated. This morning I learned two beautiful girls with NKH have gained their wings. 

I hate NKH. I hate it I hate it I hate it, I hate that it’s not malicious or intentionally awful, but that it just is what it is. Science, in the most brutal and matter fact way. That a single gene doesn’t produce quite the right protein, which means a teeny tiny amino acid can’t be broken down. That smallest of small changes means that entire lives are changed, children die before they’re meant to, they suffer seizures, aren’t able to control their bodies, can’t see like everyone else, can’t move, or play, or live independently. No running or dancing or futures like we dreamed of for our babies.

And death. All the time the death. And the grief. The grief. These two sweet girls were SO LOVED! They were both so beautiful and amazing and so so so fiercely loved. And now two families are hurting, in a way I don’t and can’t understand because it hasn’t happened to me. Yet. It hasn’t happened to me, yet.

But today isn’t about me, and my fears about our future with Mikaere. It’s about these two beautiful sweet girls who deserved more. Who are so loved, and desperately missed.

It’s for all the kids who have gone before them (Alexander and Arthur and Kaleb and Halle Mae and Cathryn, Maynak, Gregory, Siem and and and…), and all the kids who come after, who are yet to join our little community. It’s for all our NKH kids who, with a gene therapy cure that is IN THE WORKS (that’s been proven to work, that is so close I can taste it, a cure that is practically tangible except for the complete lack of funds) would be living completely different lives. It’s for a different generation of NKH bees, who one day might not have to suffer.

Today I’m donating to Joseph’s Goal for NKH Research in Willows and Ava’s name, and I’m holding my boys a bit closer, a bit tighter. I’m remembering Willow and Ava and the many others, who are so loved, still. Just like I love my boy.

Fly high, lovelies. We miss you so much already x

PS – There is a fundraiser happening for Willows family here: justgiving.com/crowdfunding/willows-fund and you can learn more about beautiful Willow on her page: facebook.com/willowwarriorprincess
 
There’s also a fundraiser for Ava’s family, here: gofundme.com/for-ava-marie

Jun 13
1

On School

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One of things that has happened in the last little while is that Mikaere has started going to school a handful of mornings a week.

This is a huge change for everyone, and I desperately wish it hadn’t happened in covid times when weighing up risk and quality of life didn’t feel so dire. But, this has been in the works for 2.5 years, delayed in part by covid (in part by a really rubbish SNAS team – the special needs assessment service, I wish they would just used the word disabled, all children have needs, you know?)

So, knowing that the school gets it, they’re full of vulnerable children with complex medical needs too (some even more so than Mikaere), and there are risk assessments and policies in place – it’s possibly the safest place, other than home, he could be right now. Plus, he loves it.

Right now we’re working on building relationships, between them and us, so they can get to know Kai and his quirks.

Let’s be clear though, my mama heart hates this. After being told NKH is terminal and our days together are limited, and precious, the idea that he’s to spend HOURS away from me, from his family with people doing their jobs and not for love is just unfathomable. (Don’t get me wrong, I believe his team should be paid and they’re great and they do care about Mikaere, but let’s be honest, if anyone of them won the lottery, looking after my son at school is probably not going to be on their todo list).

On the other hand, I can’t give him the engagement at home, the variety or the social stimulation he can get at school. I don’t have the capacity, and I hate that too. I haven’t yet had to drop off and leave him. They’re allowing me to settle him (though it’s clear now, weeks after he’s started, that he’s fine without me).

But eventually, I’ll have to leave him there. Not looking forward to that day. So, rock and a hard place. Knowing something is what it is, and wishing it wasn’t, well, that’s a familiar spot to be in.

Hey ho, onwards we go. School. It’s happening.

3

Jun 06
0

On unnecessary, ableist pressure

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I’m over reiterating this, and frustrated that it’s a conversation we have to have again and again. For some, the UK is opening up and returning to some form of normality. For people like us who are vulnerable or protecting the vulnerable, we watch with envy because the world is not safe for us. People who aren’t at risk and don’t get it don’t wear masks, they aren’t willing to get vaccinated. They broke lockdown to have raves, and they pressure our nurses to break shielding and safety rules.
It’s frustrating to have to remind ourselves and others: we don’t want to be in this position, we HAVE to be in this position. We’re strict with our bubble for good reason. If Mikaere were to catch covid, it’s likely to be FATAL for him. The last time he got a cold that turned into a chest infection we were fighting to keep him out of ICU. The resulting seizures were devastating. He suffered. The last time he went into ICU, we also ended up on END OF LIFE CARE in hospice. Mikaere catching Covid would be an unmitigated disaster for our family.
We’re not unreasonable people, you know? Everyone in our bubble lives by the same rules. We haven’t been out in the world for non medical reasons in I can’t remember how long. We haven’t seen our family, friends, the inside of stores, people on pavements in A YEAR AND A HALF. Sam desperately wants to run outside for the ultramarathon he’s training for, that’s he’s had to cancel twice. His sisters just had a baby that we can’t meet. I desperately want to see my friends but can’t. Kai is missing out on 90% of his therapies because extending our bubble is too dangerous, and has literally lost skills because of it.
We’re not draconian gatekeepers though. If someone genuinely wanted to join our bubble, it’s possible. They just need to isolate, get a negative test and agree to the same conditions we live with. A handful of close family/friends have isolated to see us, sometimes taking annual leave. We’re grateful our loved ones are understanding and happy to see us in a manner that doesn’t endanger Mikaere or put him at risk.
Going the other way though, we’re strict about keeping Mikaere safe. If a friend isn’t able to isolate we don’t see them. If we thought for a second one of our nurses was engaging in risky behaviour, putting Mikaere at risk, we’d immediately remove them from the package. That’s hard, because we rely on our nurses to get through every single day. We can’t be everything Mikaere needs and have to trust them implicitly to keep him safe.
We have to be that strict, that sure. The world is unsafe for the vulnerable like Mikaere. It’s even clear in the data: since relaxing the rules, accessibility has reduced, the daily positive test numbers are going up, and they’ve doubled in the last week. Mikaere can’t be vaccinated and the risk is high. So we stay in. We don’t go out (even though we desperately want to) but it’s WORTH IT to keep Kai safe.
Having to remind people over and over that our rules are there for a reason? That our rules are not to be “fought” against because of some minor insecurity or lack of patience? Blah. If you have never had to consider the actual death of a person you care about as a very real, very literal and likely consequence of your actions, you’re living in a world of privilege that not everyone has. And that’s not an exaggeration. That’s our life.
I mean, could you look at that beautiful wee face and decide to make his world a less safe place? I’m just so frustrated you guys. Why do people not get it?
Jun 02
0

On the days following our A&E visit

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Mikaere’s been a bit flat since our a&e visit. Many hours sleeping, but he has some active/awake time. As It’s so warm, we’re out on the balcony for portions of the day, when he’s up for it, willing the breeze to blow our way. Warm weather is always bitter sweet for us, it’s delicious but the risk of seizures increases.
So we pulled out the cool blanket, the magic mat that’s filled with gel, so it’s cool to the touch. It lasts for about 15 minutes, and then you move to a new spot. It’s worked wonders to keep Mikaere cool.
We’re still not comfortable going out into the world, and the pictures of everyone else enjoying their break at the beach or in crowds of just, out an about with a bit of normality has me feeling very green. But onwards we go, enjoying the balcony, a bit of water in a tray, paddling pool static nonsense.
Onwards we go. Managed to get some smiles, even 🙂