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#teammikaere

Mar 06
0

On the Research

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After that Deepmind post, lots of people reached out for an update on what *was* happening in the world of NKH Research in London, and I thought I’d share. It’s important to know that the money we’re raising is having a huge effect on the research done here in the UK.  While I can’t speak for the other researchers (Dr Van Hove or Dr Katsuri Halder specifically), I do feel it’s important to note THERE IS RESEARCH happening (despite what that recent article by the Clemson World Magazine would have you believe).

There is A LOT of research happening, and it’s my genuine belief that Prof. Nick is the closest to meaningful clinical trial, with a treatment that will effect the most kids.  But I’m getting ahead of myself.

Essentially, the team under Prof. Nick Greene is trying to do two things:
1 – understand more about how NKH works, and the effects it has
2 – discover potential treatments for NKH

 

Goal 1: Understand more about how NKH works, and the effects it has

There’s so much we don’t know – the carbon folate system (within which the glycine cleavage system lives) is really really complex. There are hundreds of amino acids – glycine included – which are split and joined and run through so many different processes. Super complex. Have you seen what that looks like? Here it is:

Source: Prof. Nick Greene, UCL

Truth: we don’t fully understand the how the whole system works, or all the roles glycine plays. This means it’s even harder to understand the knock on effects in a child with NKH. In the last year or so, Prof. Nick and his team have been using mass spectrometry (a really fancy science-y way to measure amino acids as they are processed) to follow glycine, and around 900 other different molecules in the brain and liver at different stages of NKH. They’ve been doing this in two different models – mice models, and in little cell samples grown from patients with NKH.

They’ve followed literally thousands of metabolic pathways. I had no idea that glycine went on to do so many things. It even joins up with other amino acids, like hexanoylglycine or propionylglycine or N-octagoylglycine (there are so, so many more). With excess levels of glycine, you can bet that all these other amino acids also have excess levels, which causes a knock on effect.

This small project alone – identifying all the metabolic pathways of glycine – costs £50-100k per year, but here’s the thing – they know more than they ever have before about NKH, giving insight into what’s happening with our kids. This is huge! It also speeds up the process, because it gives the research team clues about where in the process a treatment might work best. Knowing that means a faster route to clinical trials.

Which is everything – obviously we all want a safe and effective treatment for NKH. Which brings us to the treatment section.

 

Goal 2: Discover potential treatments for NKH

To get the treatment from research to people with NKH is a bit of a process.

First, they need to show ‘proof of concept’ in a mouse model. Nick has three mouse models.

Two are gene trap models, which means they can turn the GLDC gene on and off (with magic science). One model completely prevents GLDC gene expression, and the other allows approximately 10% of the GLDC gene expression, which allows the range of symptoms we see in NKH. The third is a missense model, which has the exact same mutation as a child living with NKH. This isn’t a gene they can turn on and off, these mice have NKH in the same way as our kids do.

Prof. Nick has two treatment projects at this ‘proof of concept’ stage. It’s very very exciting. They’re both in gene therapy – what this means is he’d like to place a working gene into a cell that is currently has a broken gene (thanks to an NKH mutation).  What that means is the cell will be able to produce a stable protein, which brings the whole glycine cleavage system up online. Hallelujah! Also, to clarify, this is an effective treatment for EVERY child with a mutation in that gene. Whether it’s a missense mutation or a deletion.

The tricky bit is getting the gene into the cell. They use fancy technology called vectors, which are actually viruses with the bad stuff taken out (it’s kind of like hijacking our immune system. Viruses attach to our cells, depositing the bad stuff that makes us sick. Our body fights them off, and writes a little reminder of how to avoid that particular virus in our genes). Instead of depositing bad stuff, the virus will deposit a working gene!

None of this is new, by the way. Nick’s team are working on it, and have been working on it for the last three years – which is so important for people to know. There’s a charity that’s fundraising millions to REPEAT this work that’s already been done. If you’re in the community you’ll know who, and I suspect the reason they’re doing it is so they can direct the clinical trial specifically to their kids, but for me, it feels like a waste of money, repeating existing research.

I don’t want to fundraise (which is really really hard work, btw) to have existing research replicated. I want to fundraise for the team that’s going to get to clinical trial the fastest, which bluntly, is Prof. Nick’s team. It’s why we support Joseph’s Goal, as the only charity that supports Prof. Nick.

Now, Prof. Nick’s team don’t have gene replacement therapy down yet. They’re exploring with two different vectors – AAV vectors to target the brain and lentiviral vectors to target the liver (AAV + Lentiveral are just different kinds of viruses. Like say the man flu + regular flu!). There are many questions – which is the best vector to use? Is one enough, just the brain or just the liver? Do they need to do both at the same time? Will it work? Will it be safe?

This is why they need to a do a proof of concept. They have the mouse model ready to go (a huge undertaking in itself) but it’s going to cost roughly ~£2.5 million to get through to clinical trials, and around 2-3 years, if they’re funded, and if the safety and ethical regulators who patrol this type of work are kind.

Speaking of, the second step in getting treatment to clinical trial is the safety step. The team needs to show that the treatment is able to be ‘produced’ at a scale appropriate for patients the world over (meaning, safe for humans. It’s what they call ‘clinical grade’). It requires plenty of safety studies to demonstrate this.

Prof. Nick’s team have another project to do small molecule work that is almost at this point. ‘Small molecule work’ sounds very high level science – but the idea is that there will be a pill (or powder?) of some sort that might be more effective, or might have less side effects (we hope!) than the existing treatments (aka, Sodium Benzoate). The study they’ve done in the mice has been very very positive, and everyone is cautiously optimistic.

Nick is currently talking to regulators about what kind safety studies they would need to do to get to this point. He’ll need a study to confirm it’s effective (costing ~£35K), and another study to check for long-term safety/possible side effects (costing another ~£30k) which might take 12-18 months. He’ll also need to find a clinical grade (read: safe!) version of the medication to test in the trials.

Then, the third step is to go to a clinical trial. May it happen sooner rather than later.

So, in summary Prof Nicks team are:

  • Using mass spectrometry (and other tests) to follow glycine around the body and understand what happens when there is too much glycine
  • Trialling two different vectors (one of the liver, one for the brain) in gene replacement therapy as a treatment option
  • Trialling small molecule work as a possible alternative to sodium benzoate.

It seems crazy to me that a proper, effective treatment (gene replacement therapy) is less than £3 million. That all that’s standing between us and a significantly improved quality of life is MONEY. It’s also not even that far out of reach, is it? If every person in  London donated a £1, we’d have the funds three times over.

So. We fundraise. You know we’re fundraising. We’re cutting our hair. We’re eating chicken nuggets. We’re doing an NKH Race Night. We might raise between £5k – £6k between those three fundraisers (which awkwardly all happen on the same two days).

That’s approximately 2% of whats needed, but I genuinely feel like at least I’m making the effort. I’m trying. With all the desperation of a special needs mama who wants a future with her son. I’m two feet all in, desperately trying to fundraise. This past week we managed to raise almost £8k with our fundraisers that happened across the weekend. A mere drop in the ocean. Hey ho, onwards we go.

Mar 04
0

On the Chicken Nugget Challenge!

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Well. That was A LOT harder than most people thought!! Originally we set it up because we wanted to do a challenge that was a bit more inclusive. Our last one was running, which isn’t everyones bag. It’s also London based, which isn’t ideal.  The chicken nugget challenge seemed like such a good idea!!

And it was, until I hit the 30th nugget and wasn’t interested in eating anymore. You guys – 30 nuggets is A LOT of nuggets. That we’d even asked people to eat 50 just seemed ridiculous. (Having looked into in the run up, I saw that people had eaten 50, but didn’t take into account how difficult it was).

We had 23 people join in, 9 teams, across two different countries in 5 different cities/towns. We collectively raised over £2500 for NKH Research!

Well done us! Proof:

If you want to donate, you can at: https://www.justgiving.com/team/nkhchickennuggetchallenge

Again, thank you to everyone who took in the challenge. THANK YOU to everyone who donated and sent kind words. You guys are the best, and we’re grateful!!

——

A bit about NKH and where all our funds go:

We’re raising money for NKH Research. Nonketotic Hyperglycinemea (NKH) is a rare and terminal metabolic disorder that affects children. It means they can’t process glycine, which is a neurotransmitter. Toxic levels of glycine cause the neurones to overfire and die, causing brain damage.

This is shown as seizures, severe global developmental delay (delays in physical, intellectual and social development), low tone, causes children to be severely disabled.

Joseph’s Goal supports Prof Nick Greene at UCL in London, who is working on gene replacement therapy – a treatment which would radically improve the quality of life of kids with NKH.

Because NKH is so rare and is funded by families, every single penny helps. Every single pound raised has a tangible effect on what can be done. The funds raised by families last year sped up research by 18 months – this is significant, when 80% of children with NKH don’t see their first birthday.

We support Joseph’s Goal specifically because they are the only NKH charity supporting Prof Nick Greene, who we genuinely believe is the closest to a clinical trial, and therefore an effective treatment for Mikaere.

Feb 13
0

On meeting Deepmind

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Earlier this month I was invited to speak at Deepmind. It all came about in a roundabout way, because I was having coffee with a friend. She’s the best kind of friend. I don’t see her very often, but she’s that kind of person who can see the depth, acknowledge that things are hard and my heart hurts, but also move on and talk about lighter things, and things that are happening in her life. She doesn’t shy away from the hard and doesn’t let the hard weigh down everything else and I appreciate that.

She was talking about her work, and how for some, it’s hard to understand the real world effect of what they’re doing, which makes it hard to understand their purpose. Their why.

They’re all up in the code and the science, she said. When I asked what it was they were working on, she said ‘protein-folding’

I took a moment, just a beat, before I pointed at Mikaere. His entire disorder is because of protein folding.

Truth: it’s actually about protein misfolding.

———

Science sidenote: Your genes create little recipes, and your body, following these recipes creates protein chains of ingredients (amino acids). This protein chain folds in a particular way, and off it goes into your body to be used.

But, Mikaere has two little missense mutations. Which means that in his recipe, there are two wrong ingredients. These wrong ingredients go into the protein chain and cause the way it folds to be different – it misfolds – meaning it can’t be used.

Kind of like, if you have a recipe for cake, and instead of sugar you put in salt. The cake can’t do its job of being delicious.

Mikaere’s mutations mean in his little GLDC protein recipe, seriene is substituted with leucine and glutamine is substituted with lysine. So, when his body builds out this recipe chain of amino acids, the protein misfolds, and the protein can’t do it’s job in the glycine cleavage system.

Protein misfolding. It’s a thing.

——-

When I said it, I could see something had clicked with my friend. Suddenly her day to day of high level theoretical science had a very real world meaning. AlphaFold – an AI system used to predict protein structure and how they fold – meant so much more with what it might be able to do in the future.

Which is how I found myself earlier this week at Deepmind. I’d organised to have Prof. Nick Greene (from UCL, he heads up one of the very few NKH Research teams) be there too, because I was positive there would be some intense science related interest I wouldn’t be able to talk to. (I was right).

Deepmind, they are so lovely. I was in a room with some very very smart people, and while our story was more the reality of NKH and protein misfolding, when Prof. Nick spoke about the Science (capital s, lots of big words), you could see the cogs turning. There were a lot of interesting science questions (which were well over my head) but that so many people were thinking about the multiple aspects of glycine related metabolism, that was exciting for me.

Even more exciting was hearing about the interesting things Prof. Nick is working on. YOU GUYS, the research that we’re fundraising has such a huge effect, and while I’m not privy to share (Prof. Nick is writing the paper now, so hopefully it won’t be too long before it’s out) I was really really excited about the real world applications, and what that might mean for kids with NKH in the future. Things are HAPPENING, Prof. Nick is learning so much about NKH and glycine and just – I have so so much hope. So much hope!

I was grateful to share our story with Deepmind. I’d gone in with the intention to share our story (because we want Mikaere to be known) but I hope I touched a chord. I’m hopeful that one day AlphaFold will be used to aid scientific discovery in rare disorders like NKH.

Also, sometimes it’s just a privilege to be able to share with people who have influence over things like this. I think AlphaFold is going to be huge for the rare disease science community.

So yes. Thank you Deepmind for having us!

 

Feb 07
0

On the NKH Community

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The thing with NKH being so rare is that we don’t get to hang out that much. There was a group hang out in the UK up north towards the end of last year, which was beautiful and so emotionally out there. It was a delight to see everyone (I even got cuddles with some of the other kids, which was such a privilege! Antonia, my heart!) but also – I felt the gaps. The kids I missed and were still grieving.

Still, we chatted and caught up and cheered the milestones and discussed meds and therapy and equipment. We talked about what people were struggling with and had some quiet moments with tears.  There was also the unexpected delight that I was in a whole room of people who knew how to look after Mikaere. Do you know how rare that is? Being able to pop to the loo, or have a conversation without being hyper vigilant, because there are at least three other people also hanging out nearby?

There was also a video group chat last month, organised by Kristen (from NKH Crusaders).  I was the only one from the UK who called in, but honestly – it was so nice to chat and talk and just – be with people who understand.

In both cases, the call + the meet up, it such an emotional balm. Because they were a safe places,  I could ask those questions I’m wrangling with.

Like – seriously. How do you deal with grief and loss? How are we meant to SURVIVE this?! There are parents who have 7, 10, 15 YEARS of living with NKH under their belts – HOW ARE THEY DOING THAT?! Because honestly, that our current life might last that long seems overwhelming and impossible. That Mikaere might die, and we won’t reach that long is downright inconceivable.

The notes I took after both are disjointed and all over the place: “Focus on the good, focus on the day to day.” and “The stress doesn’t go away. The same old same old, the story doesn’t change, but when our day to day is still a challenge, that’s hard. “ or “It’s hard, there’s always loss. Grief is devastating. “ and  “We still have the thoughts, the negative fears, and then the hope. The hope lives everyday.”

And the thing is – all those notes are high level, it-is-what-it-is type statements. They’re living the same life we are, with the hard and the grief. What I’m struggling with is that I don’t think there is anything I can do to relieve the pressure of palliative, special needs life.  I think we feel the pain, and onwards we go because there is no other choice. There is no way out but through it. We love our children, and our children have NKH and it is what it is.

Maybe that’s my problem. I’m looking for a fix, I’m looking for a way to relieve this discomfort, this phase of life. Because thats what we’re taught, right? If you are unhappy with a facet of your life, it’s up to you to make change. It’s up to you to learn more, do more, make change to better yourself. Pull yourself up by your bootstraps, work your way upwards. Do the work: be in a better position. We reward that kind of ‘go-get-em’ stereotype.

But what if there isn’t a way to ‘fix’ the discomfort? What if the end goal isn’t change and growth, but to endure?

Even typing that makes me uncomfortable. It makes me squirm, because I don’t want to live in perpetually in grief. I don’t want to endure the stress of the special needs life, or the stress of my baby dying. Would it feel less awful if I stopped trying to fix the unfixable?

But, in the same vein, I don’t know how to accept the unacceptable.

It was easier to manage when the conversation moved on to research (apparently mice in sterile areas are getting infections they shouldn’t?) and then onto fundraising. I feel like I was talking to the pros of NKH fundraising. We do what we can (you know we’re branching out a bit more now we’re doing things like the hair shaving + the NKH Chicken Nugget Challenge).

To be honest, with each event I feel if we can raise £100, £500, £1000 pounds, every single bits helps. Every single pound makes me feel like doing I’m something, helping change our lot towards something positive. It’s not the $20k fundraisers some of the others are doing (how do they do that?!), but we’re doing the best we can. The thing is, fundraising makes me feel like we’re moving forward towards something. We’re making a difference. If I can raise £5 – that’s everything.

So yes, here’s to wonderful company with our tribe. Oh NKH community – we love you, and we’re so grateful for you. It’s such a comfort to know we’re not alone. That we’re not the only ones thinking the hard thoughts and feeling the fears. <3

Left to right:
Daisy’s Dairy with NKH, Team Mikaere, Joesph’s Goal, Jack’s Journey + the amazing Doms!
This isn’t everyone who came, but only the segment where some of us managed to line up.

Feb 05
0

On ANOTHER nursery update

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The complex needs panel. Woah. Buddy.  Where do I even start? The local authority is it’s own special maze. After chasing when the panel was (which felt like a mission. Our educational psychologist, the person who is the family contact through this process left. We were allocated a new one, but weren’t told who they were. We weren’t even told the old one had left, and heard through the therapy grape vine. Ironically that’s also how we found out about our new person, who had mentioned someone else about an aspect of our case!)

Anyway, we had a date. Finally. I showed up early, and nervous.

I walked into the panel room with Mikaere. It was a meeting room, with a big square table. There were ten people sitting across the three sides, leaving one side for the two of us. Intimidating af.

They introduced themselves to one, one at a time. There was the panel chair, that days health rep and social rep. Three people from Lovely School (the head teacher, the early years teacher and the head of therapy), and then a mix of other people on the panel (a parent rep, our educational psychologist and a few more people whose names and titles and what they did flew over my head).

It was intimidating, but I’m lucky in that there was a friendly face I knew there, so that was positive.  After a lot of talk about what the current package was, how it wasn’t appropriate for Mikaere was debated (and agreed) there was more discussion about what kind of package *would* be appropriate for us.

Here is what it comes down to:

  • They’re giving us 15 hours (which is split across three hours, five days a week). Knowing that there is no way he’d be able to manage, we’re fine to use, say only 9 hours if we want to. The idea is that we should be able to build up to 15 hours.
  • If he’s not there full time, we shouldn’t rely on the therapy through school, and it should stay with the community team until he is at 15 hours a week.
  • That means that he needs extra funding to cover equipment.
  • Also, they want someone to work with him 1-1. A nurse to cover seizure care + the blended diet.

That was all fine.

What was not fine is that because none of this was sorted before the school year, there is now no space at Lovely School that is suitable for Mikaere.

So we have to wait till NEXT September.

Lovely School has exactly eight spaces in their early years class. If you double that, for the afternoon/morning sessions, that’s sixteen. Total. Sixteen special needs toddlers is all they can accommodate.

Considering there is only one other special needs school in our borough, with a similar allocation, that means there is only 32 special needs toddlers between the ages of 3-5 who get to go to nursery a year in my borough.

WTF.

I spoke the the Head Teacher, who was due to open a second early years class for this academic year, and it was scrapped due to funding and lack of teachers. Ahhhhhhhhh.

This. Our system is so underfunded in the world of the vulnerable and special needs. I don’t know what to do about it. I emailed the director of education standards and inclusion in my borough, who sent back a vague but noncommittal email. I emailed our MP, and our local newspaper, both of which went silent. What else do I do? Where do I take this? Do I go lobby and advocate and make a generally nuisance of myself until someone listens? Till they fund a second class so my son can go to nursery?

The thought makes me tired (and guilty. Like I should be doing everything and more).

It bothers me, because after taking on the fight for getting a package that is SAFE for Mikaere, there is no resource. If he was neurotypical, this wouldn’t be a problem. There are nursery’s left, right and centre offering 15 hour places. But that there are only 32 places in Wandsworth for special needs toddlers… what am I meant to do?

So. I guess we wait till next September, and work with our special needs playgroups until late 2020. I’m not 100% sure how I feel about that.

Feb 02
0

On the Respiratory Care Plan

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One of the things we’ve added to our Keep-Mikaere-well line up is a daily nebuliser. It helps loosen any secretions in his chest and with some chest percussions and suction we’re able to remove some of what he isn’t able to do by himself.

This is one of those my-kid-has-low-tone things. Because he’s not upright, because he’s not moving himself about, his body doesn’t manage secretions and mucus the same way. The fight we went through to get the neb + the respiratory care plan was ridiculous. Requesting a referral to the respiratory team was a nightmare. We already have oxygen at home – which came from palliative, not respiratory – but if you have a kid who needs oxygen at home, SURELY it makes sense to be under a respiratory service?! There was months of asking and waiting and justifying and emailing and chasing up.  Honestly, the underfunded NHS has a lot to answer for.

But eventually, months and months after requesting we FINALLY got an appointment. We went, and we talked to a big fancy consultant who was very blasé because Mikaere has a metabolic based disorder, and not a respiratory based one. There is nothing wrong with his lungs, per say. Just his tone.

But, with his tone, he’s not always able to cough. And if he can’t cough, and mucus blocks his airway, his body overreacts, he gags and then vomits. This is clear problem (I talk about the daily vomits all the time, so you know this is a problem for us). So, we it talk through with the consultant, and then we’re palmed off to the nursing team.  I’m not sad about this, because anyone who has spent time in a hospital setting knows that it’s ALWAYS run by the nurses. The respiratory nurses were amazing and smart and in half the time of the consult we had a plan and things to try.

Thus, this daily nebuliser.  It’s not a short, easy add, though. It takes 15 minutes of entertainment in a chair, battling his little fingers as he tries to pull the mask off. Chest percussion is awful, he hates it (firmly ‘clapping’ your child while he cries is not fun for anyone. And before anyone gets on our grill, we have respiratory physios and nurses overseeing this particular bit of care). And then suction. We literally put a thin tube up Mikaere’s nose and down the back of his throat to suck out the secretions there, and then I hope that might cause him to might cough and we’ll catch the mucus and suck it out of his mouth. Sometimes we’re successful and his breathing sounds clear afterwords. Sometimes it’s less so and he sounds like he’s snoring because there is a stubborn mucus plug in his airway that I can’t get to.

But I have to say, I think the added care has helped. It’s not always an easy thing to fit in, but on days that he’s poorly and has a snotty nose – it makes the WORLD of difference. I think it’s just another thing we’re doing to help keep Mikaere as well as we possibly can.

Nebulisers and suctioning mucus plugs. Oh special needs life.

Jan 31
3

Quality vs Quantity

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There is a particularly grim aspect of palliative care that I’ve been mulling over recently. I genuinely believe that the quality of life Mikaere enjoys is awful.  While he’s often comfortable (daily seizures + vomiting aside…), his quality of life is not even close to what we ourselves enjoy.  When you don’t put the seizures + vomiting aside – his quality of life is downright awful. Today Mikaere screamed after the seizure he had. The kind of scream that really hit you in the chest, that let you know what he was experiencing was painful, and he was desperate for it to stop.

Truth: I think that Mikaere would suffer less if his life was shorter.

Side note: I don’t plan on doing anything to make his life shorter, in anyway shape or form. I am his Mama and my Mama heart can barely comprehend that his life is limited, let alone consider shortening what little precious time we have with him.

But that’s the thing. If he had less days to suffer through, there would be less suffering. I genuinely feel like the longer his life, the more suffering he will experience. While I will never ever wish for his life to end,  I also don’t wish him to suffer, or to live a life of seizures and less. Less everything, less control of his body, less awareness, less freedom and independence and joy.

On the other side, by doing my very best to give Mikaere the best possible quality of life available to him, am I prolonging his life and therefore his suffering?

By doing my very best, am I prolonging his life, dooming him to more suffering?

I feel like I’m between a rock and a hard place. I’ll always want to do the very best I can for him, to comfort and care for him. But I worry by pushing him through all the extra therapy, fighting for more drugs, closer monitoring, fending off every potential downfall, that in trying to alleviate his discomfort the best I can, I’m unintentionally extending his life which is filled with so much suffering and pain.

I don’t think there is an answer here. My mama heart wants to ensure my babies life is as comfortable and happy as I can make it, but logically, asking myself what I’m prolonging, to try look at the macro as well as the micro picture…

The palliative special needs life sucks so hard.

And then I think – neurotypical parents don’t do this. They don’t need to wrangle with the idea that their baby is definitely, 100% going to die. Whether their best parenting is going to cause their child to suffer more seizures, and vomits and painful everythings. That their child is going to suffer because of them.

Again, palliative special needs life sucks so hard.

Update: Here’s where I’ve landed with this – I don’t have an answer, but my Mama heart says I should do everything I can to help him live a better, comfortable and happy life. Sometimes that’s going to mean therapy he screams through, in the hope that long term it pays off (and I know, I’m gambling that there will be a long term). It’s going to mean to more awful drugs that make him vomit and suction catheters down his nose and an untold number of needles and blood tests. But I can’t do anything other than my very best to comfort him, and give him his best shot at life. I think I’m always going to wonder and second guess whether I’ve caused more harm, but hopefully, I’ll also know that I gave my best so he would know he was deeply loved.

Jan 29
0

On please stfu

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This is a rant post, so be warned. I know that other people have problems. I know that their problems are their problems and to them, their problems are huge and big and unwieldy. I know this, and if we are friends and I love you I want to hear about your life and listen and help you (if this what you want) navigate whatever it is you are navigating.

But if we are not friends and you have your problems and you’re passive aggressively complaining because I didn’t immediately bow down to your problems, let’s be clear: there needs to be some perspective for me to relate and empathise to your problems.

Here are the problems I live with everyday: watching my son stop breathing while he seizes, praying he comes out of it and doesn’t change colour and lose all his developmental progress. Fighting to see our neurologist to help with meds to help his seizures, who can’t see us because the nhs is chronically underfunded and the neurodisability service at our hospital is in crisis and doesn’t have any available appointments at all during the next six months. Trying to keep my son from vomiting, so he can keep his meds down and have less seizures (and the washing and comforting and changing that comes with the multiple times a day vomiting). How to manage my son so he can be part of this world (when he can’t see, speak, move or eat independently). Managing our chronic grief because my son has a terminal disorder, is on palliative and hospice care, because his disorder is terminal with no cure or effective treatment.

Problems which I don’t think are problems I, specifically, need to manage or allocate effort to: your phobia of dogs.

Not because I have a fear of dogs (I do not, I love dogs) but because the severity of what we live with day to day is so chronically at the other end of the scale, up there with literally life and death decision making and kids that literally die and suffer, that a phobia, something which has a solid treatment option (even when its hard and requires work and effort) and options to avoid the trigger (again, still hard and requires work and effort), is not even a blip on the scale. Truth, if I could swap our life with NKH and it’s seizures and death and grief for a dog phobia, I would in a heartbeat.

In short I’m in a not-conflict. Because the person who has a phobia of dogs doesn’t know our story (and also doesn’t want to know). And sure, I could probably be more tolerant and kind, but also, catering to strangers not-problems is emotionally draining and my emotional plate is full of already draining things.

Also, just so you know: service/therapy dogs that are certified and trained appropriately aren’t pets.

Also: We don’t even have a dog. I wish we did, though.

Jan 27
1

The Inaugural #TeamMikaere Run

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I’m super, super late getting this post up, considering it was last September. But, The Run! Capital T, Capital R.  You must have seen the many posts leading up to it, I was all over instagram + facebook, trying to get convince people to run, to volunteer, to donate. It was the first time we’d organised an event that felt this big, that required a proper team of volunteers and tickets and several days prep and it’s own fancy website – and I was so anxious that it go well!

But here’s the thing. We have asked every single person we know for a donation. We saturated our friends and families asking for donations. You know this, if you read our blog posts, because we ask you ALL THE TIME! We know there are only so many times we can go to our loved ones with our hands out. Because you’ve all already donated (and we love you for it) and the way we keep asking, with our desperation close in to our hearts, it’s disparaging.

So now we do events, because it’s more than just putting your hand out and asking. Because events mean our friends can bring their friends (which is reaching people outside our social circle). And because events feel like we’re *doing* something, something more than just posting another fundraising link to our social channels and crossing our fingers. But I’ve never organised a run before. Did you know that runs need Race Directors? And water stops (I literally didn’t, until the morning of our run, until our newly acquired Race Director very gently let me know that I should get *a lot* more water).

But, oh my days, we had 30 runners sign up. 30 people, who had committed to raising £100 each for NKH Research, to join us around the 12km course.  We had a dozen or so volunteers, from manning the water stops, to cheering us on, to follow us around the course and man the bag drop and put out arrows.

You guys…. 12km is not a short distance. I struggled. And in case it’s not clear what I mean by that: I was the very last one across the finish line, and I almost bawled my eyes out as I crossed it.  My son is never going to run. Not a single step, not 12km. NKH has robbed him of that opportunity.

Which meant that every step felt personal. It helped that Sam had pushed him around the course before me, and that one of my nearest and dearest, Breege (who lives the grief and special needs life too) ran alongside with me, letting me feel my feels as I went. This run, this fundraising, it’s personal. This was not ‘just’a another fundraising event (are they ever?)… This run genuinely was about hope. My hope. The hope that one day kids with severe NKH will run. That they’ll take steps independent of support equipment. That they’ll find the joy in independent movement.

I can’t tell you how much I want that for my son. The depths to which I hope and wish, and the overwhelming and shattering grief when the reality is unlikely for him. (Grief… it’s real and hard and we live with it everyday. You know how it is. I’m always bringing my grief to the internet). So, this run was personal for us. We were joined by two other NKH families, which made this run special. It’s always heartening to know we’re not alone. That there are people in our corner who share our life, our grief and our love.

Thank you to everyone who donated. Thank you to everyone who fundraised. Who ran, and volunteered (hi Lucy!) and who showed up to give NKH the finger. We love you, and we’re grateful. So so so grateful.

With matching, raised £12,741.00 – isn’t that a phenomenal amount?! It makes me quite teary. This means so much, so so so much to the world of NKH Research. I’m grateful for the love and persistence with which our runners met their fundraising pledges.

It’s important to me that everyone knows that our valiant fundraising efforts do not disappear into the ether. The funds were donated to Joseph’s Goal (run by Emma + Paul Kendrick, whose son Joe has NKH) and from there (in full) to Prof. Nick Greene at UCL, who is currently the world leader in NKH Research.

Funds like this make a tangible difference in the research that can be done by Nick.  When we met him earlier this year, he said donations like ours have sped up his research by approximately 18 months. That is life changing, when most children with NKH won’t see their first birthday. His team are making progress, which give us hope, something to hang to to.

Through him you give us hope that children with NKH might have a different future.

I also want to take a moment and thank Lou and Sam. Because without their love, and determination (and that cheeky lunchtime pint at our local) this run wouldn’t have happened. We love you guys, for offering help and for saying ‘ yes we can’ to something which felt impossible. We did it!

We’ll be back again next year. Do you fancy joining us?

If running is not your thing, is eating chicken nuggets? Fancy signing up the chicken nugget challenge – it goes down at the end of Feb!  www.teammikaere.com/chicken-nugget-challenge

 

 

Jan 24
1

On a nursery update

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I just got a call from the early years head teacher with an update. Internally, they’ve discussed Mikaere’s case, have gone to the local authority to say the package isn’t right for him and they’ve suggested we appeal to the Complex Needs Panel.

Okay, this blew my mind, because we’re talking about a special needs school, in an early years class of special needs toddlers who have very similar physical and cognitive abilities as Mikaere. What kind of complex needs does my son have that these kids don’t that requires he goes to a special panel?

And then it clicked. He’s on palliative care. That’s why. My son has a terminal disorder, and that’s why we’re jumping through extra hoops. There is no one else in the class who is on palliative. Just us.

So now we’re going to an extra panel, to ask for one to one care in case Mikaere has a seizure at nursery. I worry about this, because the first panel, without us, allocated a package that wasn’t suitable.

Truth: I’m intimidated by a room full of strangers that I don’t know, making decisions about our life. I fear they’re going to say no. That the answer will be no and our Lovely School will feel like they can’t safely provide care for him and we won’t have the opportunity.

I’m scared that in advocating for him I’ve ruined his chance to go to nursery. I wonder if I hadn’t made a fuss, if he would have been alright going five days a week (I don’t think so, but I do wonder). I wonder if I’ve done him a disservice, by fighting for more, for better. For safe care.

Sigh. I don’t know. We’ll see. We wait to hear from the complex needs panel. Another group of people we don’t know, making decisions that will have huge impact on our lives.

Fingers crossed for positive news.

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PS – If you’re thinking of signing up to the Chicken Nugget Challenge, get involved!  We’re looking for more team members! www.teammikaere.com/chicken-nugget-challenge