Tag

Year2

Jul 06
0

On The Joseph’s Goal Ball

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Joseph’s Goal – it’s the NKH charity that we’ve got behind. If you follow us at all, you know this. We talk about them all the time.

Part of the reason is because we’ve spent so much time with Emma, Paul and Joe. We spent an entire morning with them last year, when we were just getting into the world of NKH, and they were so kind. I helped with the conference website last year, getting another speaker on the line up. We spent time together in Boston, the only two UK families who came over, we definitely stuck together. We had meals and I took every opportunity to love on baby Tom. Sam and I got into belly laughs when he was telling me about a guy that fell asleep with a burger in his hand (I love that that’s where his humour is at right now, it’s the best!)

We trust Emma and Paul and their trustees to make the best possible decisions with the funds we raise. We know their values are line with ours, which is why we’re two feet all in with Joseph’s Goal. Joe’s goal is our goal.

So when we found out they were doing the annual JG ball, we drove up to Wigan (near Manchester) to go. Woah. Buddy. Not only was traffic the craziest, but with Mikaere in his not quite perfect car seat and the vomiting (oh my days the vomiting) – it was a mission.

As an example: we arrived at the hotel the same time the ball started (we’d planned to arrive three – four hours beforehand…fail). We checked in, in a room that was three floors up and down a warren of hallways that wasn’t super accessible. We luckily had a very helpful bellboy help us up with our gear. We got Mikaere out of his buggy, and no joke, he was down two seconds on the beautifully white bedspread before he projectile vomited. There was vomit everywhere.

We rang down to reception, and they offered us an easier to access room. So Sam bathed Mikaere, our wonderful bellboy came back to help me move our gear and when we were finally ensconced in the new room, we were only and hour and a half late.

We dressed at lightening speed, fed Mikaere a reduced feed, put him in a shirt and ear defenders, and showed up two hours late to the ball.

I have never been more triumphant about getting somewhere in my life.

There is nothing like being in a giant room surrounded by people who have so much love for NKH kids, and who are throwing THOUSANDS of pounds at research. It was a giant room of love.

It was great to see Emma and Joe again, and also Nick Greene and his family were there too (the Greene family are an absolute delight by the way). I was surprised that bar Joe, Mikaere was the only other NKH kid there (knowing how supportive the community is).  Emma said that predominantly, for the ball it’s the local community that as rallied behind them (which is super amazing). And they weren’t wrong – there were a lot of famous footballers there, there were a lot of local businesses who had donated prizes and were bidding on all sorts.

I quite enjoyed bidding – I learnt quick to bid early because I was always, without fail going to be bidded over. My budget didn’t stretch nearly as far as some others. Sam did better than me, and got into a bidding war in the silent auction for a signed boxing glove.

It was a phenomenal night. Once there we had a great time. We were glad to finally get to sleep and breakfast at the hotel was a real treat. Side note: it really bothered me the breakfast room wasn’t accessible. We had to carry Kai’s buggy down the stairs. What would we have done if he was too heavy to lift?! Boo to inaccessible rooms as standard.

All in all it was a great night. I’m glad we went. It makes such a difference to know we’re not alone in this life, and there are others pushing towards funding for a cure too.

          

 

Jul 04
1

On getting a new carseat

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Mikaere has outgrown his baby carseat. His torso is too long and his shoulders are too wide. But because he’s so floppy there are few things we need: it needs to recline, it needs lateral support. We’d also ideally like a rear facing, swivel seat that fits in the front seat.

We went up to Milton Keynes and met the special needs lady at the In Centre Car Safety place. There were exactly two seats that might work for Mikaere, and only one was in stock. Womp. We wanted to wait for the second to come in before we committed. So we waited and waited, and Mikaere grew longer and longer and eventually his shoulders just plain wouldn’t fit.

A few weeks after, we happened to be at our friends place, who also has a special needs child. They offered us their old car seat which Max has grown out of. A Britax Dualfix Römer. It swivelled, it was rear facing and it reclined. We knew the (crash free) history. Amazing. We were grateful for their generosity (because we’d at this point be getting a charity grant for a new seat that may or may not work. Either that or we’d very gratefully fall on the generosity of our family).

So we fitted it and off we went. Now, it’s not perfect. Because of the angle of our car seats (boo racer seats) Mikaere is uncomfortably sat almost leaning forward when rear facing, even when the seat is reclined (when it’s not reclined he topples forward). So we swivel it around to forward facing, which works better (but I cringe on the inside, I know how much worse it is to be in an accident with a forward facing car seat. I’ve seen that gif and I hate it).

Also, there’s no lateral support. There’s room to grow, for sure, but in the meantime it means he kind of crinkles in on himself. It means we use Ellie Ears to support his sides and a trunki to support his head, and it doesn’t work well with the helmet so we pull that off every time he goes into the car. It’s a bit of a faff. You can see how rubbish Mikaere’s posture is when he’s in it:

Don’t get me wrong, it’s a hundred times better than being in a too small car seat, and a thousand times better than some of the other car seats we’ve tried. We’re grateful (beyond grateful) to even have the seat we do. It’s just not the ideal carseat. It’s about 80% awesome.

When we’re in the world of trying to provide enough postural support to fend off the future risk of scoliosis, I have the fear. 80% awesome is about 15% less than I’d like.

The last option we’re looking at is the 2018 Kilppan Kiss 2 – the car seat we’re STILL waiting to come in.  It’s been hailed as the all supportive, all singing and dancing chair that might be appropriate for Mikaere. But we don’t want to buy it outright (at £450 a pop) over the internet in case it isn’t inappropriate. So, we’re waiting for it to be in stock at the In Car Safety Centre for us to try (they’re the UK stockists).  Except we’ve been waiting almost 9 months now. So frustrating. Another 6-8 weeks, they told us. It could be 6-8 weeks. It’s more likely to be another 6 months.

Do you know what else? It blows my mind that there isn’t a car seat service, or place that caters specifically for car seats for special needs children who require extra support. It blows my mind. Frustrating af.  Even if there was just in insert that would bump up the supportiveness of our current car seat, that would be excellent. Blah that that service doesn’t exist.

In the mean time we try to make it work, we do the best we can. I try not to get down on the idea that this something that neurotypical parents don’t have to worry about it – but I can’t help put feel they have an easier job it in regards to car seats. Hey ho. I’m trying really hard not to be too bitter. I guess I should practice grace? We have carseat that swivels. Its safe for Mikaere, and we were so lucky to have such generous friends….

It is all those things, and I am grateful for all those things, but I still feel very blah about carseats that aren’t fully supportive. Womp. The special needs life is already difficult, so things like this which require lots of money, some puzzling and lots of time calling places and talking to people add’s difficulty to an already complex life. Blaaaah is how I feel about that added difficulty. Still, onwards we go.

Ps – our OT did some investigating and there is a charity that will do an hour long assessment to help you fit a car seat… for the heavily discounted cost of £150.  Wooomp. And that’s just for the assessment! That doesn’t include I’m sure any travel times, fitting costs or extra whats-its that we’re going to need. Did I mention that we’re a one salary family now because I had to give up my job to care for Mikaere?! How are are meant to afford all this extra stuff?

Jul 02
1

On all the amazing fundraising goodness

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Hey you guys, we’ve been a bit absent lately. I’m not going to apologise for that, really. Life happened. The short of it is that I tried to do too much in one go and now I’m paying the price, but that’s okay because the outcome is that we hit the Van Hove Appeal goal! We raised $10,394 and absolutely SMASHED that $10k goal!

It was a pretty ambitious goal, to be honest. $10k is a huge, huge amount.  So thank you for everyone who donated in the name of #teamMikaere – we saw so many familiar names come through with donations, so many people we know and love – so thank you. You guys were a strong catalyst for pushing us forward. I’m not even kidding, 53 of the blue squares (each with a donation of $50 or more) were bought by #teamMikaere supporters. That’s a whopping 29% of all the squares, and while we were facilitating the campaign, this success honestly belongs to you and to the NKH community which rallied beautifully to the cause.

Now, I know that $10k doesn’t seem like much, but it makes all the difference. Unfortunately this amount won’t be matched by the University of Colorado like we had originally hoped. We tried working with them, but they’re a state funded school and can’t release the funds for Van Hove.

But we spent a lot of time working out a plan with Kristin from the NKH Crusaders and Emma from Josephs Goal, and we have a plan:

Van Hove needs $120k to keep his lab open from July 2018 – July 2019:

– NKH Crusaders has committed $25k upfront now, and a $15k grant early in 2019, with the second payment in partnership with the Nora Jane Almany Foundation.
– Joseph’s Goal has also committed $25k upfront, with a second payment of $15k in early 2019.
– The community has raised $10k with the Van Hove Appeal
– Les Petits Bourdons are sending $12k
– Hope for NKH is sending an additional $5k.
– Prof. Van Hove has gained the support of a fellow who will send $15k.

That’s $92k now with $30k to come. A whopping $122k all up. We’ve done it. We’ve raised enough money to keep Prof. Van Hove in NKH Research. That’s INCREDIBLY amazing. He’s the nicest of guys, literally one of the most knowledgable on NKH – and he is responsive to parents. That is HUGE. We’re grateful he’s still in research, we’re grateful he’s got funding for the next year.

In terms of more general fundraising, we’ve done really well, recently.

With Josie, Margot + Chris running their marathons (in Istanbul, London + Edinburgh respectively), Adam riding from London to Paris, with the corporate support from Infinis, the charity Art Exhibition… you guys, we’ve hit in £69,575.88 raised in the last year and a half. I’m blown away by the support and love people have shown towards Team Mikaere because you GUYS – that is a ridiculous number. Side note: the VH appeal is not even included in that number!)

Here is what I know about that number – that huge giant number was made predominantly of tiny tiny donations. Small little donations add up, and that number? We’re well on our way to our £100k goal. That’s a year worth of research right there.

With that in mind – we’re having another wine tasting! Are you in London and free on the afternoon of 22nd of July? We’re doing another wine tasting – this time with a twist! Less sitting down, and more interactive goodness, moving from table to table. Tasting wine, with pairings and wine facts and riddles and games. All proceeds go to Joseph’s Goal and NKH Research (obvs).  It’s in Putney (SW15 1SZ), tickets are £20 and available from here: https://bit.ly/wineforkai2

Please come drink wine with us. It’s going to be amazing.

Jun 11
1

On the Van Hove Appeal

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I’m not sure how to write about this. Prof. Van Hove’s research lab is set to close at the end of June due to funding issues.

This man has spent more than 15 years in NKH research. It’s his research that laid the foundation for the other NKH teams. He discovered, defined and proved the difference between variant NKH + classic NKH, solving the mystery of why some kids present so so differently and don’t have mutation in the NKH genes. He proved that taking DXM + SB earlier in life has a positive effect on outcome in his sibling study. He’s set to prove that NKH causes growth issues in the brain (as opposed to formation issues) which is hugely exciting (and the opposite to what most metabolic consultants thing).

He’s the doctor our metabolic consultants ask for help. He’s the person the NKH Research teams want to pick the brain of. Notre Dame worked with him in the beginning of their research group, inviting him to speak to their students. Prof. Nick Greene from UCL has said openly, several times that there is no person with more NKH knowledge than Prof. Van Hove.

He has also, over the years, amassed a metabolic network that is second to none. When speaking to Nick, he said that every time he makes a new contact in the metabolic community about NKH, they already know and are fond of Prof. Van Hove.

He’s worked hard, a lifetimes worth of work, with his sole focus being NKH. Which is odd, considering neither the Notre Dame team or the UCL team are focused on NKH (Notre Dame’s main focus is Malaria. UCL is at least closer with Neural Tube Defects, which share mutations in the GLDC gene).

I can’t believe he’s closing, and when I heard, my initial reaction was ‘what can I do?’

It turns out, quite a lot. Not alone, of course. Both NKH Crusaders and Joseph’s Goal were already moving to help – they’ve both set aside a small grant for Van Hove. It’s not enough to cover his costs till the next grant comes in (due Feb 2019). But, if they can get it matched from University of Colorado, that would put Van Hove short only $20k. If we were also able to get matching, then we’d need to supply $10k.

$10,000. That feels like a lot of zeros.

But here’s the thing – if we can’t raise the full amount to keep Van Hove going till the next grant comes in, there is no point. We’ll end up in the same situation again a few months down the road. If Van Hove closes before the grant comes in, then any money we send is essentially wasted.

That’s the fear of NKH Crusaders and Joseph’s Goal. If we don’t get enough to cover the entire year, if the University of Colorado is not able to match what we raise, then those extra funds will go to researchers not on the brink of closing.

So – it’s all or nothing. If we, as a community, can raise $10k, we can save Van Hove’s lab from closing. That’s it. $10,000 (well, actually only $9550 now, because we donated the entirety of our allocated donation savings to get things going). If I rationalise it into more manageable chunks, 191 families would need to donate $50 each to make that much. Now, there are almost 500 members of the NKH Q&A Community, so I’d need approximately 40% of them to donate.

Seems doable, right? So, we’re asking that each NKH family donate $50. Not to share on facebook, not to ask their friends or family (who have no doubt been asked time and time again for money) but us. This is our community. This is our researcher.

Now, not everyone has $50 to spare – I know. It’s a big ask to pull $50 out of your pocket. But you know what? As a fundraising goal, it’s really low.  Keep any eye out for a few more posts on how to fundraise later in the week.

Here is what I want to say – I want to keep the most knowledgable NKH man in NKH research. I don’t want Van Hove to close his lab. So, will you help? Will you donate $50?

US folk: https://www.facebook.com/donate/312802479252342/

UK/EU folk: https://mydonate.bt.com/fundraisers/vanhoveappeal

Help me turn the grid blue. For more information: http://fundnkhcure.com/

Jun 06
1

On meeting Arthur

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There are a few NKH family support groups. A worldwide one, a UK one and one where you can ask questions and the community will answer. Depending on how Mikaere is and how much time I have dictates how active I am in them. But I’m active enough to know the regulars. These NKH community groups have been a lifeline to share my fears and delights and to help answer questions in terms of research or care or medication or how to deal with feeds or our medical team. They are an unparalleled line of support.  I think it’s one of the benefits of having a diagnosis, is that you can find others who live the same life you do. Who have the same fears and have had to make the same choices.

There is one family we’ve met who are just a delight. We’ve been talking for months and months and a few weeks ago we happened to be in their neck of the woods. So we went by for a visit. It makes such a difference meeting people who get it, who live the same life you do.

Arthur is the sweetest little guy you could ever meet. It’s hard to tell from the photos/videos on Facebook, but he’s well more aware, active and more beautiful in person than I realised. I found it really difficult not to stare, he was just so amazing. And I know, NKH kids all present differently thanks to the hundreds of different mutations that cause it, but I couldn’t get over how amazing he was.

His Mum, Charlene was also a delight. We’ve been talking for just over a year – and honestly, meeting felt like were just continuing an ongoing conversation. It was so nice, so nice to talk to people who got it, to talk research and nurses and support. It was also so nice to do meds and feeds with someone who was also doing meds and feeds at the same time. And oh Ellie, totally fell in love when we met her. She was shy to start, but Sam won her over first and before we knew it we were bff’s (she loves Totoro more than I do, which is saying something).

I was sad to say goodbye. I was sad that we live so far from each other, but you guys! It’s just like finding family. I’m glad we’ve got people in our corner who understand. I’m hopeful we’ll see each other again soon! Fingers crossed.

PS, if you’d like to follow Arthur on facebook, you can do so over at Arthurs Adventures with NKH

Jun 04
1

On Visiting Tenbury Wells

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Last year when Mikaere was well, we took him to Tenbury Wells to introduce him to his Great Grandad. Since a whole year has gone by (!) and we were enjoying another relatively calm period, we went up again this year. It was amazing – I adore his Great Grandad (he’s great – he even tried to teach me to read music this year) and it was nice to feel like we could take Mikaere on a road trip. That we could leave home for a bit of a break and not be on edge the whole time, waiting for the untoward.

I just want to take a minute and say how huge travelling is for us. Before Mikaere both Sam and I were super into seeing the world. We had huge aspirations for travelling with our little guy and that was all put on hold when we found out about NKH. Taking him home to NZ is completely out of the question. Something like taking a road trip four hours north of London felt like a huge giant mission. We went last year I prepped like anything. We had all the emergency kit, I knew which hospital was closest to where we were staying, I knew who to talk to in the paediatric emergency department and I knew who to call if there was non-emergency blip. I did the research. We had the emergency gear and I was constantly waiting for the emergency to happen. Long story short, it didn’t.

This year, because Mikaere’s been so well I didn’t prep nearly as much. Sure, we had all the kit and our car was packed to the brim like a tetris puzzle (SO MUCH GEAR) but it was a much more relaxed trip. Mikaere’s bigger and I guess we’ve got a whole year of experience under our belts.

Even better was that the trip was delightfully uneventful. There were walks and piano playing and lingering meals and a lot of fantastic family time. Our little guy is so loved, it’s really reassuring to see so many people love him so openly. I also feel like four generations is a pretty special thing, so I’m glad we went. It was also really nice to have the extra arms, which meant that both Sam and I got a chance to sit down together (!) and drink hot beverages when they’re still hot (!) and generally relax a little bit.

I often wonder how much of the complexity and stress of travelling is just down to my fears about the worst case scenario, and I wonder how much of that I’ve been primed for by our medical team and labels like ‘terminal’ and ‘acute deterioration.’ True, I’d rather have all the kit and plans and not need them rather than the opposite, but I think my mindset around travelling and being away from the safety of home has been sculpted by the fears of our medical team.

The truth is going to Tenbury Wells was positively delightful. I’m glad we went. I’m hoping that the more practiced we are at leaving home, that one day we’ll eventually be able to holiday somewhere that is outside the safety of family.

May 25
1

On Getting a Second Crib for Mikaere

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We have nurses who stay up with Mikaere twice a week. Mikaere sleeps in the lounge instead of with us, and the nurses care for him throughout the night. This worked well when Mikaere was small enough to sleep on the couch, with a foam tube under a sheet to keep him away from the couch edge. Except then Mikaere’s legs grew to the point where you couldn’t sit on the couch and not get kicked.  It wasn’t until I walked in one morning to see one our elderly nurses sitting on the floor next to Mikaere that I realised this wasn’t a tenable solution.

But… I’m a bit fussy. We have a crib for Mikaere in our bedroom. A Silver Cross Ashby crib in white. It’s beautiful, well made and made in the UK. I love it. Buuut I assembled it in the bedroom and it was too big to move back and forth from the bedroom to the lounge 3-4 times a week.

So I went to go purchase a new one, except the Ashby has been discontinued. Great one, Silver Cross. The idea of having two different cribs irked the designer in me who enjoys symmetry and things that match. Very very blah.

So instead I scoured the internet, and found a lady who was an hour drive away selling hers. It wasn’t in perfect condition (seriously though, with a teething toddler what will be?) but it was close enough to be manageable. So off I went to collect it.

It was pretty solid (Silver Cross is always well made) and I was pretty happy with it. After a quick trip to B&Q, I got some non-toxic filler putty for the teeth marks and had a tin of (also non-toxic) paint colour matched and over the course of a weekend filled, sanded and painted the second crib.

And now I have two matching cribs for Mikaere. I’m pretty pleased with myself, to be honest. The things you do, hey?

It’s worked well for us, the second crib. He naps in it during the day, it’s a convenient safe place to put him down if we need to and still have him in my eyeline. And our nurses are no longer sitting on the floor.

 

 

May 23
2

On Reviewing the End of Life Care Plans

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There’s an appointment I always dread and it happened last week. We met with our palliative care consultant at Hospice. She’s really lovely, has a keen sense of humour and is generally very sympathetic. She’s also incredibly practical and straightforward which I enjoy.

It wasn’t our palliative care consultant that made me dread this appointment, though. It’s that every six to nine months (acute events or slow declines not withstanding) we review Mikaere’s end of life care plan. Which is to say we go through, step by step and say, if the worst case scenario happened – at what point do we say enough is enough.

Blah. Just BLAH. My son is beautiful and right this minute chirpy and smiley and very happy kicking the crap out of some shredded paper. I hate having emotionally to go to that dark place. To remember the time he turned blue, that time he was in a seizure coma and then really think about his quality of life and the quality of death we’d wish for him. I hate that bit too. Thinking about his death. I mostly block out that it’s a very real and likely possibility. It just seems so unlikely because he’s so beautiful and alive and aware and happy. But mostly because I don’t want it to happen. It seems unlikely because I don’t want it to happen.

But then I think about to Halle Mae who gained her wings only a few months ago. I think about all the NKH babies who have passed and I know for certain it’s a possibility.  And if it’s a possibility, then having a plan when we’re not in the world of grief, having something to fall back on and not have to make those decisions when we’re emotionally struggling is a good idea.

But to have a plan we have to consider the worst case scenario and I hate the worst case scenario.  Even worse, this time it was just me. Sam had to work, so there I was. Our palliative care consultant, a palliative care nurse, our carer and a lady shadowing palliative care in hospice (because the more the merrier, apparently). A whole panel of people.

And we went down the list.

Our first scenario is an acute deterioration, which is to say “in the event of a sudden collapse with respiratory and/or cardiac arrest, where breathing and/or heart stops, we agree to”:

(it starts from the least invasive treatments)

  • Airway positioning. Yes. Do this. Reposition his airway, for sure.
  • Suctioning. Also yes. If we can get him breathing again with suction, it would be silly not to.
  • Oxygen. Yes. Yes to O2 through a nasal cannula.

These ones are all easy ones. They don’t hurt, they’re temporary and won’t cause any long term damage. The next one is:

  • Mouth to Mouth/Bag and Mask Ventilation for _____ minutes until parents are present or ambulance has arrived.

We said yes. For 5-10 minutes. And we debated every single one of those minutes, and the whys and whats and all the scenarios. It was hard not to say you do mouth to mouth for as long as you need to. It was hard to find the point to let go and that 5-10 minutes is our point. I even called Sam, interrupting his work day to help talk it out.

Because the next one?

We said no to External Cardiac Compressions.

We said no to CPR. I hate that we said no, but thinking about whats best for Mikaere rather than whats best for my emotional state… it’s a no.  With a child, cardiac arrest is typically caused by respiratory arrest (the opposite is true in adults). Which is to say, if Mikaere has stopped breathing for long enough, it’s likely to also stop his heart from breathing.

If his breathing had stilled for long enough that his heart stops beating despite our interventions that’s a big big problem, cardiac compressions are likely to break his ribs and he’s still unlikely to survive. Quality of death. That’s what I keep telling myself. Quality of death is just as important as quality of life.

The rest? They were easy to say no to.

  • No to Endotracheal intubation and technical ventilation.
  • No to advanced life support with drugs and intravenous access.

No thank you. We’ve been there before, with both those scenarios. And considering the quality of life following those scenarios… we genuinely feel it would be better for Mikaere to pass. Quality of death, quality of life.

This scenario was by far the worst one. The acute deterioration, where it happens swiftly and unexpectedly. The other scenario, a slow deterioration instead of an acute event we’d be able to see it coming. It would be increased seizures, reduced consciousness, and Mikaere would increasingly be struggling to maintain his airway.

In this scenario, we’d do everything we could, up until intravenous access (with still no to mechanical ventilation). But with a slow deterioration, we’ll have the time to consider what’s best. We’ll have time to think on whether each intervention is right. The main point here is that we’d be able to transfer to hospice on end of life care if need be. There isn’t a point where we’d say we want to go to hospice. If it was anything like last time, we’ll all know it’s time. We’ll have the support we need to decide what’s best for Mikaere.

To be fair, we can change our minds at anytime about anything on the plan. We’re not locked in, but I think if it happens it’s best to have a plan. I know how crazy it gets, how fast everything moves and how you can get into a knee-jerk reaction only mode. How you can be so overwhelmed with all your feelings that thinking logically isn’t possible. That we might be still processing our fear and our grief to clearly think about whats best for for Mikaere.

Still, I walked out of the room heavy. So so heavy. It was beautiful day and Mikaere was happy, but it was a really really tough day. I’m glad we have a plan to which we agree on, but I hate we even need a plan. I hate NKH, I hate that we’re planning for his death. It’s horrific.

So, a cure, hey? That’s what we need. I’m thinking on more fundraising opportunities – because that’s what gets me through, and that’s what gives me hope. Wine tasting later in the summer – what do you think? Anyone keen?

May 16
1

On getting fitted for a wheelchair

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When you think of a wheelchair, typically you think of a seat with giant wheels, where you self propel yourself forward. We see them on tv, in movies, we see them in hospitals, we see them on the street. That’s what I thought of when we first got assessed for special seating. A lovely lady named Mary came to make sure Mikaere’s buggy was supporting his body enough. Turns out Kai is very very tall and is swiftly outgrowing his buggy. Kai’s not even two yet and when he’s all strapped in his head brushes the top of the buggy. That’s not the main problem. The main problem is he’s very long and there’s no lateral support for my low tone baby. You can tell when he’s not strapped in, because he slumps forward, and kind of rolls to one side and his behind is on the bar.  Womp.

So. We took our very long baby and made a visit to the Wheelchair Adaptive Seating services at our local hospital.

I’m not ready. I’m not ready for a wheelchair. I feel like we only just got that magical moment in supermarkets where everyone peers in and delights at how beautiful Mikaere is and THEY DON’T KNOW Kai has this terminal and rubbish metabolic disorder. For a few moments in the supermarket, I get to feel like a ‘typical’ parent, rather than a special needs one. I enjoy the ignorance of strangers – it’s all so refreshing. No odd stares, no comments, no pity or platitudes. It’s beautiful. Strangers love on my baby and tell me he’s beautiful. It’s the only moment of what I imagine neurotypical normality to feel like.

We’ll lose that with a wheelchair. And I know,  I know the wheelchair designers try. They take a pram base and put some supportive seating in, instead of the pram seat. They give it a giant cute canopy covered in dots to try disguise it, but then they stick some medical paraphernalia on the frame. Here’s a structure for the oxygen tank, the suction machine goes here, and if he needs a vent then that would bolt on here. What happened at the end was less pram and more medicalised everything.

Just blaaaaaaaah. I’m not ready for a wheelchair. I’m just NOT.

There’s also the small matter that our current buggy (the Bob Revolution Pro) is an epic epic buggy. It’s the buggies of all buggies. I spent weeks researching when I was pregnant and we were so lucky to get it. It’s designed for running and it has suspension and it’s got three wheels. It’s perfect for all sorts of walks. Walking along the river in the dirt tracks, or mud if it’s muddy. Country walks when we’re out visiting. Across fields. Running when I want to go for a run. The wheels come off if we’ve packed the boot too full. It’s just the best buggy. It’s the best of all the buggies.  By comparison the pram base of the wheelchair is good for inside and pavements. It’s no Bob. Not even close.

While I know we’ll have to switch at some point, I know the second we do our freedom for where we can take Mikaere will be halved. Just. Blah. Blah to this. Blah to more medical crap and less freedom. But it’s not the jobs of the wheelchair engineer ladies to deal with my emotions around “upgrading” my baby from a typical buggy to a wheelchair, so I plastered a smile on my face and let them adjust the chair around Mikaere.

The chair pictured isn’t the right size for Mikaere, it was used for measurement purposes. We haven’t ordered his one yet. I’m putting it off. There is still half an inch of space between Kai’s head and the top of the buggy and while he still fits I’m going to cling to the pram we have. Hey ho. I’ll keep you posted on the new wheelchair as things develop.

May 14
1

On meeting Prof. Nick Greene

By | #teammikaere | No Comments

We’re really lucky to live in London. One of the best cities in the world, for sure. One of the benefits is that it’s in this city you’ll find UCL, one of the main centres of research for gene therapy (a large chunk of the research done in the gene therapy community comes out of UCL). Conveniently it’s the centre Prof. Nick Greene is based at while he’s working on an NKH treatment. This means he’s in the best possible place in terms of the knowledge available to his team – they benefit from the knowledge the other teams are working on. Very exciting.

Even better for us, UCL is attached to our specialist hospital. So when we went in for Mikaere’s metabolic check, we met Nick for a coffee.

You guys – there is so much going on in the world of NKH research. In terms of understanding the development of NKH in unborn children, understanding the wider implications of NKH and the knock on effects into the one carbon folate system, making cell models, looking at treatments (like the components that make up cinnamon!) into bigger cures, like gene replacement therapy in mice. There is lots happening, a lot of moving parts. Very very exciting.

It’s inspiring, meeting Nick (who is the nicest guy, for sure) literally made me want to throw more money at research. Because that’s it, right? The more money the more research.

I talk a lot about fundraising. I ask over and over for donations, constantly putting my hand out (and I’m so grateful for the love and grace shown to me, you guys are the kindest and most generous friends we could have) – but what it really comes down to is I have the deepest hope that in Mikaere’s lifetime NKH will be a thing of the past. That there will be an effective treatment and future babies and families won’t have it like our kids do now. That our babies won’t die.

Now, that’s a lot of pressure to put on people like Nick, but to talk to him and have him discuss where they are right now and where they are going? It gives me hope.

When we met it was a beautiful day in London. Even better, Mikaere was on good form. He was sitting and talking and very awake and aware and interested in the world around him. I know the researchers don’t always get to see the day to day life of kids with the condition they’re researching (at least not in NKH. In NKH they’re still in mouse model stage) so it was a nice moment for Nick to meet Mikaere again. To see how well he’s doing, the development he’s making.

It was such a nice afternoon. I love that I live in a city where I can meet the guy trying to cure my sons terminal condition for a coffee. What a world, hey?