On Standing

By | #teammikaere | No Comments

This kid. The heat has been awful the last couple of days, hey? We’ve been sequestered inside in front of the aircon and fan, with the cool mat and paddling pools and spray bottles to mist. We’re all very relieved the heat has broken!!

Also, how tall is this kid now?! We don’t have a standing frame (because it’s massive and doesn’t fit in our apartment), but we do have a walker. While Mikaere doesn’t walk in it, it’s great for supportive standing. He’s not in it often enough if I’m honest (standing is one of the first things to be put aside when we’re under pressure) but we do the best we can considering. He’s pretty comfortable, if he can sleep in it, right? 🤣 I really wish Trexo Robotics were available in the UK, but hey ho! I’d love to see my boy be able to get around independently.

On the Pinkies

By | #teammikaere | No Comments

This post is tangentially related to Mikaere, so excuse the round about narrative. This here is my oldest friend. Liz. She’s literally the most beautiful person. When we were teens she put herself in harms way to stop me from fleeing from (imagined) warthogs and I ran full tilt into her. When I moved to London, she was one of the first to reach out and helped me navigate the tube (though she laughed at me when I got the lines mixed up). When she left London, I missed her terribly. She came back to be a bridesmaid at my wedding, which meant pushing back her own plans. She’s supported I think every major fundraiser we’ve ever done for NKH. She recorded a video of her reading a story to Kaikai, you know? She’s an amazing friend, is what I’m trying to say.

Liz is also a breast cancer survivor. I’m so grateful, so so so grateful that she is now (and may she always) be cancer free. She wasn’t quiet about it. It was very f*ck cancer and check your breasts, ladies! She worked with Look Good, Feel Better NZ when she was in recovery – sharing her story to support and encourage others in similar positions. They put her face on a Mazda, and honestly, it was the most amazing thing.

She also joined at dragon boating team, one for breast cancer survivors. They paddle in pink. And if you know Liz, you know how ambitious she is with her sports. How much she likes to win, and how much she’ll work, determined to put her best foot forward for her team.

There’s clearly a story to be told here, and a film was made. The Pinkies are BACK! The trailer is hilarious – these women who have lived through breast cancer are amazing, yes. But they’re also just relatable women, who make fun of themselves and each other. It’s mostly only available in NZ, EXCEPT there is a virtual screening.

And here’s the thing – while the NZ screenings support cancer charities, Liz was very clear that with the virtual screening she wanted to support us, and raise money for Joseph’s Goal. For Mikaere. For NKH Research.

It makes me teary to think about, if I’m honest. It makes me feel so seen, and it’s not a “despite her own story” type thing. I think its more we’ve both faced unexpected hardships – different as they are. And we get it. I’m so grateful for Liz, and for her friendship. For her generosity.

So, you’ll join us? For a virtual screening of what is guaranteed to be a great movie. Seriously – watch the trailer at: https://www.youtube.com/watch?v=04p8si7gAYY and tell me you didn’t laugh. At a time of a global pandemic, we could all use a bit of feel good entertainment.

October 30th 2021 – 7pm UK time. It’s USD$10 a ticket (that’s about £7.40), of which half will go to Joseph’s Goal.

Tickets available at: www.fanforcetv.com/programs/pinkies #linkInBio

Please join us – you can view from anywhere and it’s going to be a great one.

#myfriendsAreBetterThanYourFriends #ThePinkiesAreBack #pinkies #fuckCancer #nonketotichyperglycinemia #nkh #nkhawareness #ifhnkh #metabolicdisorder #glycineencephalopathy #nkhcansuckit #someoneFundACure #complexmedicalneeds

On Ophthalmology

By | #teammikaere | No Comments

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

On a bit of elastic

By | #teammikaere | No Comments
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 🙂

Isolation and Backsliding

By | #teammikaere | No Comments

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 AFO’s. 

By | #teammikaere | No Comments

Sometime last year, our physio mentioned AFO’s – Ankle Foot Orthosis. We were using trainers + leg gaiters to help with free standing (I say ‘free’ standing, but what that really means is that we hold Mikaere up, rather than him standing in a stander).

The trainers were to stop his feet over pronating (meaning: the arches of his foot rolled inward), and the gaiters to help maintain knee extension (meaning: he can’t lock out his legs, and use his knee to keep his leg straight).

But, she thought that AFO’s – like a hard sandal that has a back that goes up to the calf – might help his standing without the need to fix his knees.  Mikaere is also showing dystonic posturing of his feet into an equinovarus position (pointing down and inwards), and the idea was that AFO’s would offer him more time with his feet supported in a more neutral position.

We’re lucky that we’re part of a tertiary hospital that isn’t currently suffering from funding issues thanks to private funding, which meant that with a referral letter from our clinician and a letter from our physio, we got the thumbs up for AFO’s. To be clear: our local community hospital wasn’t going to fund them. So we’re grateful!

They’re custom made for Mikaere – his feet were covered in plaster and the AFO’s made from the mould. We use them most days during physio-like activities, and sure enough, they help keep his feet in more neutral positions and definitely make standing easier!

They’re another set of supports in our kit. At this point – it’s heart breaking knowing my baby is almost two years over the point where most kids learn to stand. It’s unlikely he’ll ever it do it by himself, without support.

I also know that if he doesn’t stand, if he doesn’t bear weight through his legs, his hip sockets won’t form and he’ll be at risk of hip sublaxation (where his hip is partly dislocated) and/or hip dysplasia (where his hip socket isn’t formed correctly, which doesn’t hold his hip joint in place).

I know that both those options can be painful, and awful. I’d like to avoid that. So, standing. As much standing as we can do. To be honest, it’s not a lot. Finding time in our days to fit standing in isn’t easy. But every minute counts. So some days it’s kneeling against me across the back of the couch, and some days its in the stander, and some days in ten minutes in the AFO’s.

We do what we can, hey? We’ve picked a fun blue camo, in the hope that it will make them more ‘fun’ to use. Sigh. Onwards we go.

On the Walker Saga

By | #teammikaere | No Comments

The thing with having a child that can’t be upright by himself means that every time Mikaere is upright, his legs are strapped down. Every time. He’s entire lower half is static. Can you even imagine?  That to upright independently, you have to have your entire body from the chest to your toes strapped down.

This means Mikaere has never been in an upright position and realised he has ankles. He’s never had the opportunity to understand what his ankles are, when he’s upright. To realise what his legs are for.  Or realised that if he locks his knees he could stand.

There was also a theory, once, that if Mikaere learnt he could move his legs, realised he had mobility and ankles and knees, he might begin to weight bear. Maybe (this is very long long shot).

So I looked around at equipment that might be supportive for his trunk (because obviously he’s going to need support) and have his legs free, and I came across gait trainers, or walkers. They’re exactly like what they sound – exactly like those little walkers you plop babies in so they can move their feet and go – except instead of fisher price it’s a full on piece of therapy equipment.

I convinced our NHS physio that it was a good idea to try, and she said no harm. So we tried a walker. We organised a rep to come out and fit it and put him in it.  You guys. Nothing in the sky aligned for us.

Mikaere refused to nap and he’d a load of seizures so already we were on the back foot. Coaxing Mikaere into the walker, sans nap, into a completely new experience was both fine and heartbreaking.

There were moments were he was happy. Holding his head up, curious about what was happening to his legs, the freedom and movement, but when he realised he couldn’t get out of it, he screamed. Big tears, very unhappy, completely helpless, frustrated that what he wanted (out) he couldn’t do himself. Proper, loud, distraught screaming. Back arching, over extension. Rage. Screaming.

I hate it when he screams. I hate it when we’re trying something and we go too fast, or it’s too much and I can’t anticipate it and he just, it’s too much. Screaming. It hits me right in the heart that something I pushed for has caused my baby to scream like that.

I pretty much tore through the straps to get him out out as quick as we could, and comforted.  We cuddled and sang songs and coaxed him back in. Fine for a few minutes, not fine after that.

Essentially I called it after the third attempt. My aim was to see if we could put him in a supportive position to teach him how to weight bear through his legs himself. Or even, teach him that his legs are helpful for weight bearing, for standing. To see if he had any inclination in propelling himself forward with his legs. To teach him he has ankles.

Turns out he wasn’t keen. We had a talk about how neurotypical babies learn, but things like a baby doorway bouncer or a baby activity ring, they don’t have enough core support for him. Our NHS physio basically said they wouldn’t fund it. He didn’t show enough forward momentum for him to show that it would be beneficial. Womp.

We talked about standing slings, to see if I could suspend him from a doorway (like a doorway bouncer without the bounce) but the hoist company wouldn’t sell the sling to a family without a hoist. Our plan was to suspend it from a pull up bar in the doorway, knowing full well that if it could hold Sam’s weight it could hold Mikaere’s. But no go. (I did, later, try suspend the upsee vest from the pull upbar, but it makes him lopsided, head forward legs back. Not what I was looking for!)

And so I mulled on it. And thought about it.  And then I mentioned to our private physio what I was thinking.  See, the difference between our private physio + our NHS physio is that she has the time, and space and freedom to try different things. She isn’t bound by KPI’s, or the weird NHS culture that our other (lovely, completely competent and wonderful) physio is.  She’s also knows Mikaere well enough to know what he’s like on a bad day, and how different he is on a good day.

So, we tried again, privately. I’d look at private funding, but first we’d get the rep out and see him again in a walker.

It was 100% a different experience. We went slow. Spending a good few minutes looking at the walker, touching the walker, going in and out of the walker. Feeling his feet, on the carpet, on the tiled floor. Feeling his weight move from the front of his foot, to his heel. Literally holding up his head in our hands, his hands exploring the conveniently placed ring for holding onto.

What a different experience. He didn’t love it, he tolerated it. But – BUT! He was using his ankles, weight was going through his feet. There was definite learning there, figure out how and when and what! If the goal is for him to be more aware of his body, to learn he has ankles and to strengthen those muscles and maybe, one day (extreme stretch goal), weight bear independently, then this session showed he could.

So we pushed go. I got funding, and we said yes please to the rep company, Jiraffe. We would like a small pacer please. In speedy purple. Could we please have a quote? It took a while for the funding come through, but it did (thank you, Elifar!) and when it had, unfortunately Jiraffe had upgraded to a new walker that wasn’t quite as appropriate for Mikaere. They only had the demo walker left.

We’re not precious, so we asked if we could purchase the demo version. There was radio silence on Jiraffe’s end, and when they came back, blow me over with a feather. Because Mikaere’s condition is terminal, they would like to GIFT the demo pacer to Mikaere! Oh my days! We were so grateful, so so so grateful and accepted.

And now Mikaere has a gait walker. It’s beautiful. He’s tolerating it for longer and longer periods (it helps that his vision has improved and he now watches TV. The walker puts him at the right height). He doesn’t throw his head back anymore, and has learnt to hold his head forward (which means we don’t need to hold his head!). He’s beginning to understand the concept of taking steps (with a lot of support, not independently!) but mostly, he’s feeling his legs and his ankles upright, free of straps. He moves his legs, moves his ankles, and that’s everything. He also smiles when he’s in it, which is everything.

So, onwards we go. Upright, with feet free. With Disco parties in the hallway. We’re doing it!


On the Lycra Suit

By | #teammikaere | No Comments

Mikaere has hypotonia, which means he has low tone, or muscle weakness. Kind of. It’s not that his muscles are weak, and that he just needs practice to build up his strength, but that his brain finds it difficult to send the signal to ‘fire’ his muscles, so they can do what they’re meant to.

This means he finds it difficult to do things we take for granted, like hold up his head, or pull himself forward, or even lift his hands to his face (something even neurotypical newborns do).

One the things we do to help him is set him up in a Lycra compression suit. One benefit is that supports his core. It helps realign his spine and pelvis, giving stability and also provides better proprioceptive feedback, which is knowing where his body is and what it’s doing as he moves himself around.

The suits are custom made, so we went and got him measured up for a one. Spider-Man colours, just for fun.

We know this helps with his core stability. Fingers crossed it’ll help groove in some pathways in his brain and he’ll eventually be more stable without the suit as he is with it.

It’s just gone back to the shop for adjustments, so we’ll wait and see!


On the ABM intensive

By | #teammikaere | No Comments

One of the new therapies we’ve tried recently is the Anat Baniel Method. It’s a lovely therapy that meets Mikaere where he is, that doesn’t force him to do anything, but builds on his current skill set.

It’s a bit like magic, to be honest. We went away with another special needs family down to the Isle of Wight where there is an amazing Anat Baniel therapist – Michelle Wheatley. She’s positively lovely. She also lives the special needs life, so she *gets it*. She also is able to interact with Kaikai with love and care and intention, which I appreciate.

So many of therapists force Mikaere through their intended programme without first taking stock of what kind of day he’s having. Michelle always starts with Mikaere. How he’s doing, what kind of day he’s having, what he’s interested in, the kind of movements he’s making. I love that. I love that it’s the kind of therapy that focuses purely on where he is right now.

I do want to say that while I trust the method, and I like Michelle a lot – I feel like a lot of ABM practitioners are about the money. I’d also even go as far and say for Anat Baniel herself, the leading lady is only about the glory and the dollar signs. That *frustrates* me so much, that there are so many practitioners who are taking advantage of special needs families. (Not all, mind. Michelle for example is affordable and a genuinely lovely person).

But we’ve seen gains with ABM. Mikaere rolling over, beginning to sit up, started tolerating tummy time, started being more intentional with a handful of movements he’d practiced with Michelle – it was pretty mind blowing .

It’s also a bit frustrating, because if we hadn’t seen gains I could have left that awful ABM culture where London appointments are £125+ for 45 minutes behind and focused on something else.

Still. We *have* seen gains. That’s what I’m holding on to. So we’ve been down a few times, driving down to the Isle of Wight for a week for an intensive. Its amazing being there. Its amazing for all of us, for our mental health and emotional wellbeing to be out of the constant therapy/appointment loop. Beach walks definitely help.

So ABM. I’d recommend it, but not at the ridiculous prices some practitioners charge. (If you’re in the UK, I would highly recommend Michelle. More information about her practice at www.brainbodysense.co.uk


On the Upsee

By | #teammikaere | No Comments

Have you heard of an upsee? It’s a harness that allows kids with special needs to walk. You strap a harness to yourself and attach your beautiful special needs baby to the harness, and together you walk.

You guys – kids who have never had the chance to walk are WALKING!

You can bet we got one for Mikaere (thanks to a grant from the amazing Eilfar Foundation) and we were pretty keen to try it out.

Well, it turns out my legs are too short, or Mikaere is too tall so it doesn’t work well when it’s the two us, but with Sam or Andy (Mikaere’s grandad) it’s magic.  For a small while, our boy walked. He rocked on his toes and did the hockey pokey and he was upright and WALKING.

I can’t being to explain how huge this was for us, my emotions just went all over the place and I wanted to cry. He NEVER gets the opportunity to do this, to walk around the kitchen, walk about the garden with his grandad. He’s always strapped in, and wheeled around. Never ever has he been in the position where he can walk, even if it is assisted.

I didn’t know this, but my boy is tall, so tall. And he’s got the skinniest little legs and the sweetest knobbly knees! The best bit is when you’re wearing him, you can feel him *want* to walk. You can feel him lift his feet in anticipation and move himself forward. It’s beautiful. So so beautiful.

I’m aware that this amazing bit of technology only works for us because Mikaere’s head holding has improved dramatically. If he can hold his head up, that opens up all sorts of interesting opportunities for him.

It’s amazing hey? We’re pretty pleased.