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On rolling

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We’ve hit another milestone – Mikaere rolled over today. From his back onto his left side.

Mikaere ROLLED OVER! Onto HIS SIDE! BY HIMSELF!

I can’t even begin to explain our excitement. The first time I saw it I thought it was a fluke. He’s been very wriggly recently – a little tiny worm as he wriggles about the mat, but always on his back, with shuffling his hips from side to side to get around.

This is the first time we’ve seen him roll. Intentionally. With something we haven’t explicitly spent hours and hours teaching him. My baby can roll!!

It’s the small things, hey?

On having the best of friends

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Good friends of ours (possibly some of my favourite people ever) live overseas. They’ve loved on us from a far and loved on Mikaere when they’re in London. A few weeks ago they hosted a murder mystery fundraiser for #teamMikaere. You guys – I’m jealous it wasn’t here because it looked absolutely AMAZING!

Phe and Dom hosted dinner for 10 of their (incredibly generous) friends. They did a short intro about Mikaere and why they were fundraising and holding a murder mystery event in lieu of running marathons or walking cross country like some of the other epic efforts (which made me laugh – I love they did an eating/drinking event. Phe and I once tried to run together and both of us I think decided walking was more our preferred pace).

Set in 1967 the Champagne Murders was accompanied with a very terrible fake Austin Powers DVD random clues and scripted characters. Apparently there was a lot of heated debate while they picked apart the clues and pointed fingers at each other as the potential murderer (which I also love – as a big Mafia/Secret Hilter fan I love a good Kangaroo Court!). With a lot of food and free-flowing booze their very generous friends raised a phenomenal 3,000QAR, which is roughly £633!

Isn’t that one of the most amazing things you’ve ever heard? I’m continuously overwhelmed with how our village has come together, and still does – months on, to fundraise for us and love on our family. So a big giant THANK YOU to Phe and Dom. We love you guys more than you know.

On reusables vs disposables with tube feeds

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When we finally left the hospital and Sam and I were finally able to parent, one of the first things I did was switch to reusable nappies. We used baby genius freetimes and they are AMAZING. As convenient as disposables and easier to manage (mainly because I never had to leave the house for nappies, all I had to do was a load of laundry). Overall I felt pretty smug that we were doing great things for the environment and that our nappies weren’t ending up in a landfill.

Except that now we have the gtube, we’ve changed his feed a bit (high five for the blended diet) and increased his fluids as appropriate and woah buddy.

The intake outtake has been ridiculous. I can’t keep up, not with liners, not with anything. The nappies were leaking. Leaking doesn’t even seem like the appropriate word – it was like every half hour Kai was DRENCHED, requiring a bath and full outfit change, and washing whatever he was lying on. I can’t tell you how many times a day I was stripping the crib and washing the sheets. I got to be an expert in washing the car seat cover and I started putting incontinence pads down everywhere.

So we switched back to disposables. I hate myself for saying that, but we did. The all-in-ones weren’t cutting it and I didn’t want to spend a lot of money upfront of more reusables that may not suit. I’m sad I didn’t have the capacity to change the nappies every 15 minutes and the full outfit/bath/strip the crib/wash the car seat routine several times a day, but I just couldn’t.

Disposables, I don’t love them. Here’s the other thing, even now the disposables (and we’ve tried several different brands) don’t always hold up overnight. I’m considering going up a nappy size to help with the absorbency because wtf. Knowing that nappies are likely to be in our forever with Mikaere, it’s infuriating that my kid isn’t even 2 years old and with tube feeding we’re dealing with leaks all over the place. It’s even more heartbreaking that this isn’t a temporary measure – Mikaere is likely to be in nappies for his entire life – that’s A LOT of nappies in the landfill not breaking down. Le sigh. We’re currently using Naty’s. They’re apparently an eco disposable (though I’m aware this is because they are PRODUCED according to the most environmentally friendly production methods. This however has NOTHING to do with their landfill implications. Their website says parts of their nappies are biodegradable in appropriate conditions (if that’s not snake-y marketing speak then I don’t know what is). Truth is they still take forever (400+ years) to break down in a landfill.

It’s very blah. So – special needs tubie parents I want to hear your tips. What nappies are best? How are you managing with absorbency? How do I not add several nappies a day forever to the landfill while still not having to change and wash everything?

(Image throwback to baby Mikaere in our very first few days home…)

On drama with buttons

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Ahhhh. Fuck. I’ve talked before about how Mikaere’s button is in the perfect position for him to reach when his elbows are supported on the floor (so anytime he’s lying supine). He has pulled it out numerous times, and even worse – the balloon has burst and it’s fallen out.  Womp.

As a bit of a back history: this is what a button looks like. Essentially, there’s a feeding port with a balloon that holds it within Mikaere’s stomach. You deflate the balloon when you put it in, and once in you inflate the balloon to hold it in place. These buttons cost about £400 a pop and should last 5-6 months.

 

In the last 3 weeks Mikaere has had three (to the tune of £1200. W.T.F). I literally don’t understand why they’re not lasting.  When I was trying to explain to our nurse what was going on, it was very confusing. In the end I had to write up a breakdown just to get my head around it:

– 14th – Button got pulled out in the carseat fully inflated. (Button A). Went into A&E for a replacement, which we put in (Button B)
– 21st – Mikaere pulled out his button fully inflated (Button B). I put Button B back in.
– 22nd – Picked up a replacement button (Button C) for just in case
– 1st – Mikaere pulled out his button (Button B) fully inflated. I put Button C in.
– 3rd – Button C had a leak and fell out. I sterilised Button B and put it in.
– 3rd – Button B also had a leak and fell out. I sterilised Button A and put it in, and taped it so it couldn’t come out.

Just in case it was hard to follow:

Button A – Currently in. Inserted twice.
Button B – leaked. Inserted 3 times.
Button C – leaked. Inserted once.

I’m getting really good at putting the button back in, but YOU GUYS – it’s always a moment of panic. It’s so terrible. First it’s because someone notices that Kai’s onsie is wet, and there’s a wet spot on his onsie right over his belly button (which is a weird place for a onsie to be wet). Whoever is nearest will jank up the onsie to check, and sure enough, the button is out and vomit is leaking out of his stoma.

It’s a bit of a race to get something – anything – into the stoma to prevent it from closing. We currently use leftover NG tubes. We have so many and they’re thin and easy to get in and tape. The concern is that if the stoma closes then 1) we’re back to the NG for meds and feeds (boo!) and 2) he’d need surgery again to put another button in. Considering the huge risk of general anaesthetic and the emotional upheaval of the first round, I’m not keen.

Once we have an ng in the stoma we can relax for a moment and figure out next steps. Sometimes that’s going into A&E if we don’t have a replacement button on hand. Sometimes it’s sterilising the existing button, letting it cool and putting that one back in. If we do have an unopened, sterile replacement we’ll usually use that.

But oh, putting it back in. It’s not as bad as the NG, but it’s not fun. Because typically Mikaere pulls out the button fully inflated, his stoma is sore and a bit tender. When I attempt to push a new one back in (deflated, obvs), Mikaere screams and clenches his abdominal muscles, essentially stopping me from pushing it through. I have to wait until he takes a breath in – when he takes a breath in his lungs expand and his abdominal muscles relax for a millisecond. Listening to your baby scream because of something you’re doing is horrid. It’s heartbreaking and I hate it. But it’s necessary, so necessary, so on we go. Eventually it’ll be in and we aspirate stomach contents up to make sure it’s in the right place (testing with a PH strip).

Having to do this 5 times in the past three weeks has definitely upped the daily stress factor of our lives. The most current button is physically taped to his body, so he’d need to get through several layers of hyperfix to the button out. We don’t have a spare at the minute, so I’m really really hoping he doesn’t pull it out. I don’t fancy a trip to A&E for another one.

We’ll see. Hopefully things will get better soon. Fingers crossed this one will stay in longer than a few days!

On more vomits and our magic osteos

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After my victorious post about Mikaere putting on weight… I’ve had to retract all that joy and positive forward momentum. As of this morning, Mikaere is 10.37kg. Which is to say, he’s the same weight he was three months ago. Except that now he’s longer, and he’s lost all of his beautiful baby chubb. You wouldn’t know it to look at him – he looks so well. Like a happy little boy. Except that most people don’t see him without his clothes on. You can’t see his ribcage or the way his little vertebrae stick out.  Now, just to clarify, he is underweight, but he’s not in emergency, dire situations. We’re sitting at about the 20th percentile.

Even more relevant, is that we know *why* he’s not putting on weight, and it’s because he can’t keep food down. Oh the vomiting. The vooooommmiting. It’s everywhere, all the time.

Here’s the thing. Mikaere has a not-cold. He’s not really ill, he doesn’t have a chest cold or a temperature or a touch. What he has are secretions. From teething, maybe? From life? And every time he can’t handle a secretion he vomits. His body and his stomach is so sensitive. I spent a long time talking to our osteo about the structure of the stomach.

I’ve said it before and I’ll say it again – here is research that says a lesser curvature gastrostomy reduces the incidence of postoperative gastroesophageal reflux (aka, vomiting because of the gastro – source) so for everyone due to get a gastro – you’ve been warned. Asked for a hitch with a low curvature and make your surgeon agree.

I’m positive that because Mikaere’s stomach has been hitched high to his abdominal wall, this has pulled his stomach up at an unnatural angle, changing the way food sits in his belly and puts pressure on the lower esophageal sphincter, which is that the nice bit at the bottom of your esophagus that keeps your food in. The outcome of this is that he’s a trigger-happy projectile vomiter now. All the time, everywhere. In the buggy, the car seat, the crib. Sitting up, or down or side lying. Moving, not moving. On the blended diet, formula or dioralyte.

I thought we were getting somewhere with reducing the frequency of his feeds and the rate at which we feed him, but now we’re going even slower, because fuck. The vomiting. I thought we were on the up but I was wrong. Womp.

Anyway, like I said, we see an Osteopath every week thanks to the amazing charity Osteopathic Centre For Children. Side note: Osteo is essentially concerned with the mechanical arrangements/movements in the body, particularly in terms of alignment. They do a lot of soft tissues massage and joint manipulation, though to be honest, sometimes it literally just looks like they lay their hands on Mikaere and close their eyes. We see the same therapist every week and every visit in the few weeks has included a projectile vomit. It’s so so horrid.

But this week, this week Stuart was in the clinic. He’s the director of the Foundation for Paediatric Osteopathy and is essentially one of UKs leading osteopaths. Stuart is essentially magic. He came to see Mikaere and we talked about the vomiting. Leading an additional two osteos, they did their hand laying/eyes closed trick and talked about medical terms (the sacrum, the vagus nerve etc. They later translated to say Mikaere’s insides were a bit twisted around the hitch, which caused his body to work a bit like a spring. They were trying to untwist all the things).

I’m hopeful, as always. I wasn’t convinced they were going to stop the vomiting, but Mikaere enjoys osteo, he always relaxes and they’re not doing any harm.

Oh, silly disbeliever me. After his appointment, we went FOUR DAYS without a vomit.  Considering we had normalised at to 2-3 vomits a day, four days of not changing sheets or cleaning carpets or multiple baths – what a relief. You can be sure I showed up the next week with a ‘more please’.

After every visit, we’re seeing longer and longer vomit free times. I don’t fully understand it, but I’m grateful. Mikaere is not yet back up to where he was, weight wise, but if he can keep food down it’ll start, right?

Magic Osteo’s. Definitely recommend.

On small wins

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Mikaere’s on two antiepileptics and a supplement to help with his seizures: Zonisamide, Phenobarbital and Omega 3 (with all of it’s fantastic DHA seizure support capabilities). Now, it turns out that phenobarbital is one of the most frequent drugs used to treat neonatal seizures, and considering Mikaere is palliative it seemed like a good choice when nothing else was working.
 
But here’s the thing, long-term use of Phenobarbital has enough studies to suggest it has a terrible effect on a developing nervous system, it has a detrimental developmental effect too (with developmental quotient declines (in both cognitive and motor skill) which are thought to reflect a slowed neurological growth rate) and causes an increase in the probability of a cerebral palsy outcome.
 
Whats more, side effects include a decreased level on consciousness (it’s essentially a sedative) and it’s addictive, causing withdrawal symptoms when weaning. Aaaaand it would make Mikaere vomit. If it wasn’t diluted and given really really slowly he would vomit immediately.
 
Also, it turns out phenobarbital is used in the lethal injection of death row inmates and also prescribed to terminally ill patients to allow them to end their life through physician-assisted suicide. Always nice to know we’ve been giving this drug to our baby twice a day for last 14 months.
 
A good 9 months ago now Mikaere’s seizures started becoming a bit more, dare I say it, under control? Mikaere started having a handful of seizure free days. At first, it was sporadic, one day here, one day there. It was amazing, so amazing to see. The fewer seizures he had, the more gains he was making. Eventually, he worked himself up to two or three day stretches. I was ecstatic, to say the least.
 
It was at this point we discussed weaning with our palliative care team. There wasn’t much harm, they said. As long as we went slowly. If there was an increase in seizures we could put the dose up, no problem. So we started a very very slow wean. Every three or four weeks, we’d reduce the dose by 2mg (which is about 0.2ml – a tiny tiny amount). We’d wait, and watch. We already track Mikaere’s seizures and I’d hold my breath waiting to see if there would be more seizures. Sometimes there would be if he had a cold or was having a tough time. Sometimes you wouldn’t have noticed the dose had dropped.
 
It felt tricky, weaning. We eventually got to a sub-therapeutic dose. We high fived that day. And today? Today, after months and months and months of tiny, incremental weans – we’ve managed to get Mikaere off phenobarbital.
 
It’s been amazing. He’s been more awake and aware, making more developmental gains. I have seen an increase in seizures if the Omega 3 is a bit old (and has oxidised) or his Zonisamide dose is later than it should be, but generally, as long as we’re on the ball he’s a happy little guy who is still having some seizure days.
 
I’m stoked to have Mikaere off one of the medications. Phenobarbital is still on our list of emergency meds, but I’m delighted we’re no longer giving it on a twice-daily basis.
 
Celebrating small wins, woohoo!

On cancelling

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Ahhh. Before Mikaere I couldn’t understand people who flaked. People who would agree to a time and date and event and just not show or would make excuses or whatever – I really struggled to empathise. As a person, I might be late but I prioritise getting there. If I say I’m going to be there, I am.

Today Mikaere and I were set to see a friend and her sweet 6 month old baby. It had taken us month to pin down a date and it was going to be in a lovely pub with a beer garden and the weather forecast was set to perfect. Except that just before 7am we were woken by our night nurse. Mikaere had a temperature. His chest was junky. He had a cold.

Womp. Wooooooooooooommmmppp. The offshoot of this is the more seizures he has as his body attempts to fight it off. Which has the delightful second effect of more vomits as he can’t handle the secretions when seizing. So much vomit.

I hate this. Was it because we were at the hospital yesterday? Because we had friends with kids over for dinner? Was it the small boy who made a beeline for Mikaere’s buggy and touched him before anyone could stop him? Was it someone at Sam’s work? Was it a stranger while we were on one of our walks?  Was it because I’ve been a bit more lax with the sterilising? That I just washed his spoons in soapy water and didn’t run them through the steriliser? I did say recently that it would be nice to build up Mikaere’s immune system a bit more, do less sterilising, have him hang out with more kids…

I’d forgotten how hard colds are Mikaere. So I take that all back, fuck building an immune system if it’s going to be so difficult for Mikaere to fight through.

I hate this. I hate that he’s sick. Hate that we’re constantly cancelling (I’m so, so sorry to everyone we’ve cancelled on, and I’m so grateful for your understanding and grace). I hate that my boy struggles so. Mostly I hate NKH. Positively loathe it. (So much so that I put it on a t-shirt and I wear it frequently).

Hopefully this cold will roll on by in the next day or two, but we’re battening down the hatches till it’s on it’s way. Send us immune-boasting-get-better thoughts.

On Kaleb

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I’ve been silent this last little while because this is hard. I held myself back because I’m not sure exactly how to post about this. It feels raw and hard, and unbelievable. It feels too big, too wrong.

Kaleb, beautiful beautiful Kaleb, a 4 year old with NKH died.

 

 

 

 

I’ve spent forever looking at my cursor blink. Again, there are no words. This is too big for a Facebook blog post, where I post a picture and I’m sorry and I say something blithe about wings and NKH and encourage you to donate to research. Kaleb deserves more than that. Justine, his Mum, deserves more than that.

But the truth is, I’m grieving because I felt an affinity with Justine and Kaleb. Justine sent me funny Māori videos and would send encouraging words about Mikaere and I watched Kaleb from afar, loving on him from the other side of the world because he shares the c.395 mutation with Mikaere. I’ve never met them, I’ve never held Kaleb or hugged Justine. They live in Australia. And yet my heart is broken. I feel fragile and time has stilled and I can’t grasp the enormity of how Kaleb is not here, and how that feeds into the complexity of one day Mikaere will also not be here and I just… I don’t know how to face that.

And even worse, is that I know Justine and her family are facing that. Right now; and if it’s huge and unwieldy for me, their grief must be overwhelming. I think of Charlotte, who lives near them and how her grief must be heart-stoppingly raw too.

I think about that statue, with the grief and the insides all missing. I think about how my chest is getting pieces knocked out, how I’m feeling these deaths. There have been so many, and there will be so many more. I think about how grateful I am that I can hug Kai, that he’s here and how fearful I am for the day he’s not.

I think it’s mighty hard how grief and death are not talked about more frequently. That I live in the UK with their stiff upper lip-ness and “feelings, we don’t have feelings” kind of mentality and how do I navigate that when my life is perpetual grief and death? Would this life of death after death be easier to manage if we as a society had a healthier approach to acknowledging the hard feelings around grief? If we could talk about it with someone saying they’re sorry, or ‘let me know if I can help’ or ‘I can’t even imagine’ or ‘You’re so strong, I could never do what you do’ (shut up with your platitudes, they’re terrible). I’d like to be able to talk about grief and feel the things and be uncomfortable for a second because I’m sad because I hurt for this sweet little boy. I’d like to feel this grief without someone trying to fix it, or make it better.

I think that’s another conversation for another day because right now I don’t have the right words for this, I can’t explain the depth or the unwielding nature of grief I have for Kaleb. Of the fear I have for the day it’s Mikaere. I don’t have words. There are no words.

Grief. Anger and Rage and Disbelief. Disassociation. Tears. I just, no. Those little words aren’t enough. There is no relief or respite here.

Kaleb, you sweet sweet little boy. Fly high. You are so loved and will be fiercely missed.


————-

If you’d like to help, you can contribute to Kaleb’s funeral costs, here: gofundme.com/kalebsfuneralcosts

————-

NKH children I want to remember:

July 2018 – Kaleb Donaldson (4 years old)
July 2018 – Neeraj Ks (6 and a half months old)
March 2018 – Halle-Mae Arbuckle (17 months old)
Feb 2018 – Mayanak (4 years old)

(I had to stop myself at 2018. This list is already too heartbreaking).

On The NKH Car Sticker

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Did you know the ‘Baby on Board’ sign isn’t so that other drivers will drive safer around your car? It’s so that if you’re in a car accident, the emergency team know there is a baby in the car and to adjust their priorities appropriately.

I’ve been thinking about that for a while, not sure how to help in that situation. We carry emergency medication and emergency files with us everywhere we go. But if we were in a car accident and I was unable to advocate for Mikaere… what would we do then? Mikaere is on six hourly medications – or what if he was in so much pain he started seizing? There are a bunch of medications he’s not able to have – most which are first line seizure medications.

What would happen if I weren’t able to advocate for him?  It scares me.

So I’ve been looking at medical alert seatbelt things.

Then another NHK Mum posted a car window sticker (thanks Amber!). It was pretty brilliant, so I altered it slightly and got more printed. Now on our car window there are stickers to let emergency teams know specifically about Mikaere. We haven’t got the seatbelt alert yet, but at least there’s a sign in the window to indicate something’s wrong. I’m positive that if worst came to worst, he’d be taken care of the best he could.

PS – NKH family I have extras. If you want some, £1 each + p&p. I’ll send them to you! All proceeds go to Joseph’s Goal + NKH Research.

On Bar-b-kai 2: The Meat Returns

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After the wonderfulness of the Joseph’s Goal Ball, we packed up the next morning and high tailed it back to Bedforshire. Penny and Tony were throwing the second Bar-b-Kai, a BBQ fundraiser. They threw one last year, in aid of Team Mikaere, Joseph’s Goal and NKH Research. It was our very first fundraiser and it set the tone for our attitudes towards fundraising.

I’ll forever be grateful for Penny and Tony for showing us how easy it could be, how insanely possible fundraising was. We’re so lucky, genuinely, to be surrounded by people who love Mikaere, who are willing to throw fundraisers for research. Last year, that first BBQ showed us that we could fundraise. That we could raise money for research, that we could, without a doubt, make a difference. It was possible, it was easy and we could do it.

So when they were throwing a second BBQ? We were going to be there. So we drove straight from Wigan down, about a six hour journey with comfort breaks for Mikaere and arrived just in time. YOU GUYS! There was a ridiculous amount of meat (cooked to perfection, and some kind of delicious butternut chickpea curry concotion with I inhaled thirds of), there was a super fun raffle and the wine/water game was addictive success. A special shout out to The Chocolate Deli in Worcester, who donated a beautiful chocolate hive which went up for raffle. It was very oh la!

We ate, we drank, we caught up and everyone loved on Kaikai. It was a lovely, lovely night. I’m so glad we were able to be there. In total they raised a ridiculous £1,200, which honestly – it makes me teary to think about how generous everyone was. Thank you, again, to Penny and Tony – you both have been so gracious to our little family.

Loved it. Charity BBQ’s are the best.