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

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

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

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?

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 🙂
 

On visiting the A&E

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It’s not the first time we’ve had to call an ambulance this year. It doesn’t get easier. The counting of the seizures, timing them, logging the presentation. After we give the second buccal, we’re meant to call an ambulance, but I don’t want to. Not really. I don’t fancy spending hours in a&e, holding down my boy for bloods. (That’s not why, though. The why is that I don’t want this to be beginning of the end, I don’t want this to be a deterioration, but I don’t let myself think those things. Instead I think about the uncomfortable chair and the boredom and the endless waiting and the child with the disorder so rare people just ‘pop in’ to introduce themselves and say hi).
So instead I call our critical care nurse, and let her convince me. She clears the way. When they arrive, the ambulance service already has Mikaere’s directive. We’re ready and waiting, and so we head right out.
Thanks to our nurse, the paeds A&E knows to expect us, which means we go right into Majors (it’s busy. The other two bays are already full with a broken ankle and a concussion. Usually they’re empty).
Triage is quick. Seizure frequency is up and drastically different from his baseline. We’ve given emergency meds twice and the seizures are still breaking through. We’re here for a review, and to rule out any acute indications that would mean a symptom stay.
The physical review is clear. No red flags. The bloods come back clear. Infection markers are low (so no infection). Everything looks fine.
At this point we’ve been in the a&e almost seven hours. The last four have been seizure free, and as there are no acute concerns, they’ll send a message to his neurodisability consultant to follow up, and they wave us out.
Grateful to be home. Hate seizures. Hate that this is happening with more regular frequency.
Hey ho. Life with NKH, hey?
  

On a bit of elastic

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Mikaere’s at risk of hip subluxation or hip dysplasia. Subluxation is where the thigh bone is partially dislocated or fully dislocated from the hip socket, and hip dysplasia is when a hip socket that doesn’t fully cover the ball portion of the upper thighbone (which in turn allows subluxation).
The reason for this is quite simple: because Mikaere can’t weight bear independently, he’s not spending nearly enough time on his feet to allow his hip sockets to form like they should. Let’s be honest, he spends most his day lying down, or in supportive seating. Now that he’s so big, standing is typically a two person job, and as he grew out of his standing frame last year and our trial appointment for the next one isn’t till July… basically, there isn’t a lot of standing going on.
When lying on his back, Mikaere has a tendency to splay his legs wide. It’s called excessive hip abduction, it’s no big deal, except that because he has low tone and it causes his hip to rotate slightly, it can hasten the journey to subluxation.
So, on the advice of our physio we introduced a bit of elastic band to our set up. Don’t get me wrong, there are lots of fancy, therapy-esque things you can get (like hip helpers, or the happy strap, both which we’ve tried and he’s outgrown) but they are all VERY expensive. We had elastic at home, and tied it together in a loop (very technical, and free) which works in the same way.
Essentially, it stops the excessive hip abduction, gives his muscles a teeny bit of a stretch in a different position and it was affordable and accessible. Woo!
I wish all disability equipment was as affordable and accessible as bit of elastic, but hey ho. I’m glad we’ve made it work in this case 🙂
  

On celebrating the wins

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This guy. Despite a whole year of no therapy bar us, not seeing any professional therapists and despite some minor backsliding in regards to his truncal tone, he’s doing SO WELL! We recently reintroduced weekly physio sessions with a new physio (our previous and much loved physio @lauragates82 moved away!) and Mikaere has risen to the challenge. Just LOOK AT THIS HEAD HOLDING!
I remember sitting in a room, once, learning about laser therapy and practically crying as I disclosed my fear that my baby would never hold up his own head. I worried that without head holding his ability to interact with the world would be limited (my well meaning but very ableist fears were ripe back then, and I’d soon learn how to adapt so he could engage with the world, head holding or no).
Regardless of my hopes and fears for him, I want to share just how hard he’s worked to develop his head holding skills. It is not all the time, but YOU GUYS! Just look! Is he not just the *most beautiful*? (Obvs, his pretty face is not related to his head holding ability, though I’m chuffed for him for that, too).
My mama heart, I love him so much!

On visits during covid times

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After getting vaccinated, isolating, and having negative covid tests, Mikaeres grandparents came down for a visit. Mikaere loves his grandparents, when they walked through the door he got very vocal, and there were smiles.

He loved the cuddles, and oh! The IN PERSON stories instead of on the iPad! So good. It’s been sixteen months since we’ve seen them last, and we’re grateful that they were able to come and see us all.

The things we’ve missed, hey?

 

On staying in, still

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Things are opening up here in the UK, and as always, I’m anxious. The world doesn’t feel safe for Mikaere. I want to trust in the vaccine (please get vaccinated if you can!) and hope that it does drastically slow transmission… but from the (ridiculous amount of) research I’ve read, it looks like the vaccine only reduces the risk of transmission (by between 25%-35%) but doesn’t STOP transmission. What it does is prevent you, a vaccinated person from experiencing severe symptoms.
Which is great, genuinely. Except that Kai wont be vaccinated (his first set of vaccinations put him on end of life care. We were living in intensive care/hospice for MONTHS). So, if the vaccine doesn’t stop transmission, Kai’s still at risk.
With the world opening back up and the CDC gettin frivolous with mask advice… the world just seems like a less safe place. I don’t know. I’m hopeful with reduced transmission rates thanks to increased vaccinated (ignoring the Indian variant) and with summer coming… perhaps it will be okay?
Perhaps in a few months positive cases would have dropped off (rather than the increase we had last week) and perhaps it’ll mutate itself into a less scary version and just… go away.  Obviously, we’re as keen as anyone to get back to normality, but the risk for our little family is so much higher than everyone else. It’s really hard, hey.
So, we’re doing what we can. Sensory foam play, with ducks. That brought a good 15 minutes of joy, I’ll take that.