Tag

Year 3

Oct 18
0

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!

 

Oct 14
0

On Fighting for Nursery

By | #teammikaere | No Comments

Well blow me over with a feather. After that initial post on nursery, I got a call from the early years teacher at Lovely School.

Here is what she said:

  • the offered package is not right for Mikaere. They can see that, and yes, it’s very clear to them that it wouldn’t be appropriate to put him in that package.
  • There aren’t very many children like Mikaere who have come in to Lovely School (I took this to mean: pallative). Focus on quality time has to be paramount to his package, without causing any harm.
  • They had an internal meeting and agreed, that they should go back to the council and advocate for Mikaere and other kids like him.

Ahhhh. I literally cried, big fat tears rolling down my cheeks. I had been HEARD! It’s so so rare that I’m heard, that what I say is given weight and considered.

I guess it helped that I piled them with paperwork and evidence before they left. With emergency care plans and seizure plans and symptom care plans and respiratory care plans. With his ambulance directive and his cheat sheets and medication lists and schedules and doctor contact lists. I saw that they were a bit shocked at the sheer amount of paperwork in our emergency kit (it’s the stuff we take everywhere with us). Apparently not all parents provide folders of paperwork at the first meeting.  But if they want a full picture of his needs, they need all of this. I’m grateful that I had documentation to support my previous concerns. Would they have listened to me if I hadn’t provided them?

Still, what matters is that I was heard. That’s huge.

Also, the relief that someone else was going to do the fighting was huge. I don’t have to gear up to go fight the immovable bureaucratic council and explain why my boy is more than a budget item.

I mean, I absolutely will if I have to, but I’m already fighting so many other services and it’s hard constantly being in conflict. I’m running on empty and it’s hard.

That someone else is going to take this on? That’s everything.

When I asked what package they were advocating for, it was more along what I had envisaged, which is a few mornings a week, with a focus on settling into the new space and forming secure relationships with staff and peers. We want him to be happy, and safe, and engaged with play. It would also mean that therapy would be done outside of school, which was a comfort to me.

You guys. The sheer relief at being heard, at not being the one to go into battle, but that Mikaere was still being advocated for was overwhelming. Tears. So many tears. Can all services be like this please? Still. We’re only at the first step. We’ve got a few more months to shift the council’s rigid perspective, Mikaere’s school package hasn’t been agreed as yet. But I’m already in love with Lovely School. They listened, took it on board and are acting accordingly with genuinely what’s right, even if it’s against the grain.

Let’s hope the council can see it too.

I’m so hopeful you guys. Hopeful that something will come together and it won’t be me having to go into battle again. Fingers crossed!

Oct 11
0

On the b:Generous Amsterdam + Paris Run

By | #teammikaere | No Comments

Every year Sam’s work does a Be Generous week, that’s focused around giving back to the community. It means charity events and volunteering and donating. Every year there is a run in Amsterdam (and this year Paris too!) for #teamMikaere and NKH Research. That this is a regular run blows my mind.

People sign up to run around Vondel Park, and people get their laps sponsored. I love that they do this. There were a few cheeky sponsors this year, who increased their sponsorship rate for higher laps. Sam flew across to Amsterdam to run, which was amazing. He ran 5 laps, with each lap being 3km. I can’t really fathom running that personally, we’re super proud of him!

Overall, there were 28 runners in Amsterdam, and 7 in Paris. They raised over €5,500 for NKH Research, which is no small act!

When I think about all those people who ran, when I think about Chris R who organised the runs, and all the people who  sponsored/donated – I get teary.

This is personal for us. This is our son’s future, one which is not promised or even expected, and here are all these people who clearly are with us. Research is the only hope we have for an effective treatment, for a better quality of life for our son. That money is the only bottleneck is frustrating.

I can’t begin to explain what a comfort it is to know that there are people fundraising on our behalf. That we’re not alone, that we’re not doing this by ourselves.

You make us feel heard, and seen. You give us hope. So thank you. We love you guys, and we’re grateful you’re on #teamMikaere

Oct 09
0

On of course we ended up in hospital

By | #teammikaere | No Comments

I wish I hadn’t said the bit about a&e in that last post. I didn’t meant to put that out into the universe.

I called the patch team (they’re our out-of-hours nurse team, who are on call in those hours when no one else is available), just for some reassurance that we were doing the right thing and she immediately suggested we call an ambulance. Despite us keeping his O2 stats up in a good place, his heart rate is too high. His respiratory rate is too high. He’s working so hard to breathe.

So here I am, blue lights flashing, in an ambulance. I can’t figure it out. He’s got a cold, but his chest is clear. We saw the gp 6 days ago, the paediatrician 4 days ago,  and now today we are being blue lighted into hospital.

Why is he working so hard to breathe? Why why why?!

And then we’re down the rabbit hole. How do I prevent this from happening again? How do I take care of my boy? How do I do this?

(And then, if we’re getting really dark together, how do I manage if it turns out I can’t do anything, that I can’t help him breathe, that I can’t help his heart beat slower, that I can’t stop him from being ill).

Kai’s just vomited all over the ambulance. Fuck.

——-

After a few hours in the a&e, I’m in a showdown with the registrar. Bloods have been taken and don’t show anything of concern. The chest X-ray is clear. We’ve taken swabs cause clearly he has cold of some kind, but it will take a few days to grow those cultures.

The registrar wants to admit Mikaere for observation. I want to take him home. We have everything they have up on the ward, we have a night nurse tonight and he’s more at risk of catching something else on the ward than he is at home. Home is safer. It’s also only an 8 minute blue light ambulance away from the hospital.

I know I’ve won the stand off and he’s coming home when I ask what they would do here that we can’t do at home, and she doesn’t have an answer.  What would they do if he got worse? No answer. Depends on what the ‘worse’ is, apparently.

The registrar has gone to speak to the consultant. We’ll see. I might be be in a showdown with the consultant next. But I genuinely believe that the best place for him is home. That it’s safer than on the ward. I get the impression there aren’t that many parents who fight the authority of the registrar, but here I am.  Let’s hope the consultant on call either see’s common sense, or is too busy to want to cross words with me.

Update: We were discharged, with strict instructions to come back if things get worse. They gave us loose definitions of what ‘worse’ looks like, but really it’s just if he doesn’t improve, if his heart rate stays “too high”(but again, didn’t give a firm definition of what “too high” looks like and he can’t hold is O2 levels, we’ll go in again.  Our community nurse will come see him tomorrow, and the day after, and every day until it’s clear he’s on the mend. We’re going home! Thank fuck.

 

Oct 07
0

On an awful morning

By | #teammikaere | No Comments

It’s just gone 5am, and Sam’s moved out into the lounge to sleep and I’m playing it fast and loose by moving Mikaere into our bed. He’s on o2, but despite it his heart rate is through the roof, his respiratory rate is ridiculous – he’s working hard to breathe.

There are tubes and wires everywhere, and Mikaere is frustrated at the mask on his face and keeps tangling his fingers in all the tubes.

I’m trying really hard to hold it together. But I hate this. I hate that my baby is struggling. I hate that we have to have monitors and alarms and tanks of o2 by our bedside. I hate that at this point I don’t know whether we’ll still be here in bed in the next hour or in a&e.

We all desperately need the sleep. Sam’s night ‘shift’ finished only three scant hours ago when he finally got to go to bed, and I’m run down and exhausted and poor kaikai, he needs the recovery time. Instead he’s thrashing about trying to get his mask off, and the stats monitor is kicking off because his heart rate is too high. (A typical heart rate for a sleeping toddler is between 80 – 120 beats per minute. The stat monitor reads 173 beats per minute right now. That’s me in the middle of a HIIT class!).

It’s just, Kai being ill is such a trial. And I hate this. I think frequently about how neurotypical parents get to go to sleep at night and not worry whether they’ll end up in the a&e before their alarm goes off. About how they’re not woken by stat monitors, heart racing as they try see if their baby is blue or not.

I hate the special needs life, you guys. It’s so royally awful, and my baby suffers and there’s no way to fix this

I read a book a while back, called ‘Was this the plan?’ about a special needs Mum and how she managed her through daughters life and again through her husbands trial with cancer. She says frequently that you can’t do anything about the cards you’re dealt, but you can decide how you play them. BS, is what I think. There’s no good way to play these cards. There is no way to accept the unacceptable. There is no way I’m going to be okay with my son living a life of pain and suffering. With my baby living a life less than what everyone else expects and enjoys.

And if I’m being honest, that entire book was a factual recount of an awful series of events, pain, grief and death. What was missing was any kind of emotional accounting. That’s what would have been helpful to me. How to manage these roiling emotions when life is one emergency after the next, when you don’t have any emotional capacity left and you’re in the dark place.

Because that’s what I want to know. How do I survive this grief, when I don’t even know whether we’re all going to get through this morning?

Oct 04
0

On Going to Nursery

By | #teammikaere | One Comment

As I write this, we’ve just come out of September. There were many back to school or first day photos in September. So so many.
We were also gearing up for a first day at nursery, possibly by the end of October. It was broached by our portage play therapist, knowing how I felt about being separated from Mikaere (which is no thank you never), and that at five, he was going to have to go to school (by law).

Knowing that it would take a long time for me to feel comfortable with Mikaere going somewhere outside my care, and into the care of strangers (especially when he’s on palliative care with a terminal disorder and every single day is precious), we went very very slowly.

Last year we visited schools. We talked about what some basic goals might look like, and what we’d be comfortable with. And then we went to the council and said, yes please. Please find us a place at this Lovely School here. That happened across months and months and months. It was very slow, and very gradual. Plenty of time for my mama heart to get accustomed to the idea of sending my baby to school.

Last week I hosted a group of professionals into my lounge where they presented an offer of placement: Five days a week, three hours a day between 12 – 3pm. His entire therapy caseload would be transferred to the school and how lovely was that?

Not lovely. Not lovely at all. I was shocked and made it clear that this package would not work for us for several reasons:

  • Mikaere is on a feed between 12pm and 2pm. That’s two hours where he is in a chair, relatively upright and still, else vomits.
    He typically sleeps from 2pm – 4pm, or has down time. He’s not typically very active in this time.
  • Five days a week is too much. He’s very easily over stimulated, and without lots of down time to compensate, he tires quickly and has seizures. Obviously we want to avoid that.
  • I’m not comfortable having his therapy transferred to school when I’m not there to oversee it. So much of what we do is lead by us, by me, because Mikaere’s well-being is so fluid. Seizures steal so much of his skill, so we’re can’t build on what he’s learnt week to week. One week he has full control of his limbs and his rolling. The next he’s floppy and can’t bend his elbow. The idea of not being there and things being missed without me to advocate for him (like I do now) is inconceivable. What’s more, if he’s meant to be having all this therapy when he’s on a feed or sleeping, he’ll never get the therapy he needs.

So no. It was not lovely, and I felt like this package had been designed by someone who did not know Mikaere, or his medical needs, and did not care. When I expressed all of this, it became clear it wasn’t the school setting it up, it was a council led, one size fits all special needs package. Have you ever heard anything so ridiculous – as if you could bundle the entire spectrum of special needs toddlers into one package. A package which does not suit Mikaere or me.

I was devastated you guys. Because right then my choices are put my son in a situation that I know will not work well and cause harm, or not have him go to nursery altogether and deprive him of lovely social interactions. That’s hard.

So I did what I always do when I’m faced with two unacceptable choices, which is accept neither and escalate. I asked for a few months leeway to review, and I wanted the name of the person at the council who works with Lovely School to arrange the packages so I could get one that works for us.

I think they were unprepared for me to do that. But you guys, I’m so tired of fighting.

I feel like I’m fighting every corner, for more for better for Mikaere. There is no part of his care that just works for him, no service that ‘just works’ like it’s meant to to meet his needs. (Just as a side note: I’m still trying to manage home visits from our gp, and his neurodisability service is in severe crisis to the point where they can’t offer prescriptions for medications he needs to keep him out of a coma. I’m fighting for the very basics of his medical care, so I’m not exaggerating when I say there isn’t a single part of his care that isn’t being fought for).

I’m so tired. The idea of gearing up for another fight against another immovable entity, another faceless group who doesn’t acknowledge my capability, my knowledge or expertise, who sees me as just another ‘hysterical mum’ – it’s exhausting and I haven’t even started yet. But I will. I will put my head down and provide paperwork and research and requirements and laws. I will escalate higher and higher up the ranks until it’s acknowledged that a one size fits all package is not appropriate for all children. That it’s not appropriate for my son, and that is unacceptable.

I will fight this fight, this extra fight in this extra corner, because I love my son, and he deserves the very best quality life I can wrangle for him.

So, off we go. Battle armour on. I can do this.

Aug 16
0

On the vomiting & the PH Study

By | #teammikaere | No Comments

Oh the vomiting. I feel like it’s one of the invisible parts of our life, but Mikaere vomits EVERY DAY. Everyday the gagging, and the vomit and the changing and washing and comforting.

We carry towels around with us (most which have ever so slightly off colour splotches). We have a change of clothes for him, and for us. Vomit and the discomfort of vomiting is our day to day, and has been since February 2018.

Can we just take a minute and talk about how awful that is? How uncomfortable for Mikaere, how sad and smelly and gross and just, awful?

How happy would you be, if you vomited everyday? Tasting the bile, feeling it rise up the back of your throat? Knowing that day after day you wouldn’t be able to keep anything down? And no choice but the vomit, not able to communicate that you feels nauseous, not able to move your body into a more comfortable position.

How is that acceptable? How is that quality of life?

It’s not. No one seems as upset about it as I am.  It’s taken a year and half for anyone to investigate. A year and half of me speaking about it with every single medical professional on our team, insisting that this is not okay, asking for help, asking what else we can do, asking for more for better.

Then our gastro follow up for the gastrostony that we had in Feb 2018 was set for April 2020. Because the gastro Service at our local hospital is chronically underfunded and there is no consultant to see us.

This week we finally, finally had a ph study. It’s the very first step in understanding how much vomiting/reflux is happening. It’s only for 24 hours and requires a prob is put down Kai’s nose (and an X-ray to confirm correct placement).

It’s frustrating. We already know he’s vomiting, we already know he has reflux, and we know this because we take detailed logs of everything Kai does. I’m not sure whether they think I’m a hysterical mama bear whose making it up, or whether my data is just inaccurate (or are they just doing their due diligence?) but regardless, a step has been taken. Something has happened, and I’m hopeful that that something results in a review which will then kick off a bigger investigation.

My fear is that there is no reason. Or the reason is something stupid like the surgeon hitched the gastrostomy too high.

We’ll see. I’m grateful something has happened though, even if that something was a redundant waste of time. If it means someone is looking at its data and recommending a review, it will be worth it.

 

Aug 04
1

On the research happening in the UK

By | #teammikaere | 2 Comments

Woo! Research update!! One of the super cool things is the number of NKH mice models that are being used at UCL, that Prof. Nick Greene and his team work with.

They now have THREE mice models (more than any other research team) which is phenomenal. They’re all in the GLDC gene, but they are all different in severity. This means they can study different aspects of NKH and see how different treatments work with the different models.

Quick science aside: In every cell in our body, the cells read part of our dna (called genes) and use that as a recipe, make a protein. That protein goes on to do a specific job within the body. In NKH, if the child has a mutation in the GLDC gene, the GLDC protein is broken and the child is unable to process glycine. 

The mouse models are designed to mimic NKH – these mice are bred to have mutations in their GLDC genes so they are unable to process glycine, just like our kids.  Prof. Nick and his team have three working mouse models:

GldcGT1

These Mice have a ‘gene-trap’ – a magic genetic switch that allows the research team to have the GLDC gene either create the GLDC protein, or to turn it off so it doesn’t. 

When it’s off, these mice have NKH and present very similar symptoms like our kids do. They have higher levels of glycine in their blood, urine and body tissues, and about half the mice live for approximately 3 months. In the liver, they have perhaps 10% functionality of the GLDC protein (someone who doesn’t have NKH would have 100%).

The bonus of being able to turn the gene trap on and off is so they can understand how the glycine cleavage system works in different places in the body. It also means that if they turn it on (so the mice a producing the GLDC protein correctly), they can simulate what effect a working treatment might have.

(Side note: don’t be intimidated by the name. GLDC is the name of the gene. GT presumably stands for genetrap, and because they have 2 and this is the first, there is a 1 on the end)

GldcGT2

This is another gene trap mouse model (where they can turn the gene on and off), but this one completely prevents all GLDC gene expression (0% functionality of the GLDC protein). Very few mice survive after birth – this is a more severe model than the previous model.

By having a model that doesn’t produce any of the GLDC protein, they can see what the biochemical and metabolic effects are. This is huge for understanding what NKH does, and how it works. 

Gldc missense mutation

The team were able to create a model that has a missense mutation – a mutation that changes one ingredient in the NKH recipe (imagine replacing sugar with salt in a cake recipe, for example. The cake can no longer do its job of being delicious. Similarly, if the recipe for the GLDC protein is wrong, it can’t do its job processing glycine).

This is very similar to the kind of mutation a lot of NKH kids have. In fact, this is a known mutation, there are children with NKH who have this exact mutation! 

This model is used to test treatment options, and to better understand NKH progression. This is also huge, because if a treatment option works well with this model, there is a chance it would also work well with our kids. 

When I spoke to Nick last he said we know more about NKH now than we ever have before, and a lot of that is because of these models.

You guys, were so lucky to have Nick and his team on our side! He’s literally so far ahead of everyone else (side note: he’s already three years into gene replacement therapy research using these mouse models. I know the Drake Rayden Foundation are fundraising right now to kick off the exact same research that Nick’s already done.)

For everyone that supports #teamMikaere and Joseph’s Goal – this is where your money goes. This is where the life changing research is happening. This is where change is happening. So thank you, thank you thank you to everyone who has ever donated, bought a book or a tshirt or made a justgiving page! You guys are the best! 

PS – The Charity Fun run is happening on the 28th of September in London. Looking for both runners and volunteers! More at www.nkhcharityrun.com 🙂 

Jun 21
0

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

 

Jun 19
0

I don’t know how to accept the unacceptable

By | #teammikaere | No Comments

I’m struggling you guys. There is a part of me that feels like I’m a broken record. Everything I’m about to say you’ve heard me before. But I can’t,  I just CAN’T wrap my head around our situation. Usually, if you’re unhappy with a facet your life you can change it. It’s not always an easy change but change is possible.

I can’t change that my son is disabled. The cognitive dissonance of trying to accept that this is our truth, that this is our life and the depths to which I wish it was not is shaking my core value system.  The cognitive dissonance is debilitating.

I struggle with the anger. When I can’t for another second listen to him scream because he’s hurting. I struggle SO MUCH with watching him suffer (I might appear calm and in control in the moment, but I promise you, under the veneer I am a sea of violent emotion). How are we meant to be okay with the pain and the seizures and the vomits?! Holding his little pudgy arms while I apologise over and over as another needle is stuck in his arm. The apologising I do as I force him through another round of physio or therapy. One more time, one more movement, one more I’m sorry as I push him through something he doesn’t want to do. More apologising as I force the button back through his stoma because the button has come out. More ‘I’m so sorry’ as I run my fingers over the cannula scars on the backs of his hands, the tiny tiny scars on his heels from the millions of heel pricks.  The millions of ‘sorry’s  because he’s uncomfortable and screaming and I can’t tell what is wrong.

My baby suffers. Not all the time. But even when he’s not suffering, when he’s smiling, he’s still disabled. He still doesn’t have the same opportunities in life that everyone else has. And that’s shit.

I’m not asking why us. I know why us. I carry gene for NKH, in the same way I carry a gene for brown eyes and wavy brown hair and skin that tans deep when exposed to sun. Sam carries an NKH gene, and a gene for his long legs and his grin and his blue eyes.

Its matter of fact. It is what it is. And it is SHIT.

But accepting that nature and science have made my son so severely disabled, and as a result this is our life is soul crushing. I love him an unfathomable amount and I can not fix this for him. I can not heal him.

And so I struggle. I’m questioning my life values, and struggling with myself because there are no answers (all the whole smiling and saying ‘I’m fine thanks, how are you?’ in social situations because this is too big for small talk, even if you’re living the special needs life).

This is one of the reasons why I took that posting break. Because it’s all very lovely to have you guys love on my baby, and the support you give us is valuable beyond all price, but when your core value system is breaking, when you’re so far away from your family and your dearest friends (WHY is New Zealand so far away and so expensive to travel between?! Sometimes you just want your Mum, but my Mum is on the literal other side of the world) it’s all kinds of shit.

I’m struggling you guys. There is no clear path. There is no emotional way forward, and I don’t know how to carry this. My son is disabled and I wish to the very depths of my soul that he was not. I don’t know how to align those two emotional opposites.