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Ellly

Nov 13
0

Kai 101: The medications

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This is a crazy one. Everyone knows Kai takes medication. It makes sense, he’s a kid with special needs and in the medical world it means fix what we can with drugs. That’s how the medical industry works, mostly. I’m not getting down on it, drugs means we can reduce Kai’s glycine levels in his blood. It means we can give his brain a break from the seizures if we have to. It means we can, somewhat, help him.

Kai is on a six hourly schedule – every six hours he gets 6-7 different medications (not including the 2:30pm stint where we give the one medication as far away from the other meds as we can).

His entire routine is set around these medications. He can’t tolerate some of the meds on an empty stomach, some require an empty stomach. so we do some meds, we feed him, and we wait 15 minutes and do the rest. Because of this Kai is eating at least every six hours, we typically do five meals a day. No snacks, just meals with additional water flushes.

Different to your typical toddler.

It’s also tricky getting the meds sometimes. Two come from the hospital (where one is ordered in from the states for us and the other is way off license use). Ten meds we can order through our gp/pharmacist, and one vitamin I order through Amazon. I do a stocktake once a month and request all the things when we’re getting low. I’m also the one in charge of asking for dose reviews. Kai’s weighed every three weeks or so by our health visitor, which dictates some of the dosages.

If I think Kai needs to go up or down (it’s pretty easy to work out, once you know the mg/kg/day ratio) I email our consultant, and cc in our community nurses. They’ll let the gp know so the next time I order through everything is on the correct dosage.

In the beginning it was overwhelming – leaving the house to go anywhere that wasn’t a five minute walk away because a logistical nightmare. I’m glad we’ve adjusted – I’m glad it’s not overwhelming anymore … it’s become a routine now. Typically I’ll do the 6am meds, and Sam will do the midnight meds if it’s just us. If we have overnight help it’ll be the nurses. During the day it’s typically me, or Sam if he’s home or the nurse if we have help. We have a little medication station where all the meds are, with some made up earlier in the day and some in the fridge. It’s pretty much overtaken our counter space, and our cutlery drawer is now shares the space with syringes. We have stuck giant whiteboard paper up, and written Kai’s med schedule where everyone can see it.

We’re making it work, because this now is our normal. A crazy amount of medication and the never ending syringe sterilising and 6 hourly meds – this is our normal as part of the special needs path.

The things we do, hey?

 

Side notes:

 

Nov 10
4

Kai’s One Year Photos

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Way back when Kai was born, we didn’t have a newborn shoot. I wanted one – I had scouted out a few photographers whose style I loved – but Kai and NKH cut all those plans short. A newborn shoot was the last thing on my mind once we were in NICU with all the life limiting, terminal conversations we were having.

When we finally made it out of NICU, we’d been put forward for a shoot with an amazing charity, called Remember My Baby. It was lovely, but so awkward because our baby was very much alive. I still treasure those photos, but at the same time there’s the expectation of terminal coming soon hovering about. Fair call, because not even three weeks later we were back in hospital on palliative care.

Now? A whole year later? I wanted to capture Kai at a year. I wanted some not-waiting-for-the-end photos. We invited Kami (who has followed our story from the beginning when I first messaged her about a newborn shoot) to come spend some time with us, and she is awesome. More than awesome.

She was funny, and loved on Kai and did a great job of setting us at ease. And her photos are beautiful. So so so beautiful. I can’t even explain how beautiful they are. We got so many amazing photos of our day. There were some posed ones of the three of us but what really surprised me was the amazing ones of just, our everyday normal. Feeds, meds, changing the tape on Kai’s tube, nap fighting, cuddles and chats and playtime.

 

 


 

We’re really grateful Kami was able to document our time with Kai. Love the photos. Love love love them.  If you’re looking for a photographer, we can’t recommend Kami enough. She’s fab!

 

Nov 08
0

Fundraising & Team Mikaere Bracelets

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You guys! We’ve raised over 30% (!!!!) of our fundraising goal! I can’t even believe it. You guys have been SO generous. It’s such a tricky thing, because we keep asking for help. And every time we do, *every time* I have to put my pride aside. It’s humbling to ask for help, but the support and generosity so many have shown us – you GUYS. It’s mindblowing. We love you. So thank you – to everyone who has donated.

We’ve actually raised enough to kick off Neurofeedback therapy (!!!) so expect to see more on that soon. We have our first appointment next week sometime. We’re so pleased (and humbled, really) that with your support (and knowing that we can only afford to do so because of your support) we’re able to help Kai.

So thank you, genuinely, to everyone who has donated. You guys are amazing.

Also – I wanted to share some cool swag with you. If you’ve been to one of our fundraisers, you might have seen the #teammikaere bracelets we’re selling. They’re handlettered (by yours truly – which means they’re all unique, and they all have their own character). They’re super hardwearing (I wear mine everyday, and I’m pretty sure Kai’s grandma Wendy wears hers too) and beautiful and comfortable.

They’re £25 a pop. If you make a donation, I’ll cover shipping and send one out for you.  Mention you’d like one in your donation message and email me (elly[dot]gedye@gmail.com) with your address and I’ll send it the post the same day (crazy days of therapy, appointments and seizures aside).

Help us reach our fundraising target for Kai? Go on, they’re so pretty. Worth it.

Please help – make a donation here. 

Nov 06
2

On the newly diagnosed

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Oh, the newly diagnosed.

Sometimes I struggle with the fear others have when their baby has been diagnosed with NKH. I didn’t really have the fear for NKH in the beginning. The name of whatever it was didn’t matter, because I’d been primed for immediate, any day now death. When they had a diagnosis and were talking about developmental delay, all I heard was that my baby was going to live. My baby was not going to die as immediately as I thought he was.

Hallelujah!

Buuuuut that’s not how other parents take it. There recently has been a slew of newly diagnosed parents. The community is pretty small, so once a family reaches out everyone is aware and responds with love and hope.

Or sometimes just a quick message, because their fear is overwhelming and difficult to align with my current life.

There was one set of parents who asked loudly and repeatedly for prayers that the NKH diagnosis was wrong. That they didn’t want a life of seizures, or developmental delay, or low tone or medical anything. That NKH was horrific and painful and difficult and horrid. Please, God, make it something else.

And yes, okay, NKH is all those terrible things. But as I read those ‘pray that the doctors are wrong’ requests, I had Kai on my lap happily gurgling away, being quite delighted with a fistful of wrapping paper. He was content and beautiful and happy.  I found it difficult to align the horror of the newly diagnosed parents fear with the happy baby on my lap.

NKH is not fear 24/7.

There are happy moments, and joy and love. It’s a mixed bag. I want to tell these parents that, but it’s hard to get through the ‘God is good and your prayers will be answered and your child will be healed’ barrage. (Also, if God has set you on a path where your kid has NKH, I’d stop praying that the doctors have got it wrong. They clearly haven’t. No one accidentally diagnoses NKH on purpose, and it’s hard to say the glycine labs are wrong when they line up with the MRI’s and all the symptoms… Better to pray that you can get your shit together to manage the new path you’re on with grace).

That doesn’t mean I’d willing choose the NKH life for Kai, never. But I didn’t have the choice and were on it.  I’ve managed to settle in best I can (Faith & Fortitude, that’s what we say). We started the Mikaere Foundation to raise funds research into NKH and a gene therapy cure. We share our knowledge and the research we’ve come across. We do the best we can with the life we’re living.

But a year down the road, NKH doesn’t hold any fear for me because we’ve faced everything but the inevitable final goodbye. Until we’re at the very end of the path, we’ve faced the worst we can face already. The hospital holds no fear. We’re friends with the a&e reg and nurses. We’ve got a fab group of therapists and I’m Facebook friends with an excellent group of NKH parents, so many of who I absolutely adore.

NKH, right now, (dare I say it?) is manageable. And we’re managing fine, the best we can. Being at home with Kai, who is mostly doing just fine, is the best and happiest place we can be. Right now, things are very good. My baby is happy and well loved and home. Sure, we still have all the appointments and medications and seizures and global delays, but compared to those weeks in NICU? I’ll take this. I’ll take this happy time.

So, newly diagnosed parent: please be strong. You can do this. Whatever happens, whatever NKH brings you, you’ll have moments of both joy and fear. And that’s important to know – it’s not all grief. There is plenty of love and smiles and happy in there too. More than you’re able to imagine right now.

When you’re ready, there is a large, loving group of people in your corner already. We’re already living the NKH life. You’re not alone in this. 

Nov 03
1

On the Fluoroscopy

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We were late. So so so late. Here’s the thing though, even if you’re late, if you rush and apologise profusely, even if they’ve closed down the clinic (whoops) they’ll typically try fit you in. That is, you know, if you have a complex needs child with all the medical requirements that Kai does.

Thank you, powers that be for the kindness shown by the paediatric X-ray team on the day we were late. The opened up the clinic for us, and in we went. After removing Kai’s comfy bottoms (they had metal poppers), it was a small matter of wearing a lead gown and putting the colourless, tasteless, odourless dye down Kai’s tube and doing the X-ray.

It was very clever, I had no idea how quickly things moved! You could see right away when the dye lined Kai’s stomach. From there we rolled him into his side, and it dripped into his small bowel. From there, his muscles did all the work. You could see the dye swishing from one side of his body to the next, outlining the shape of his small bowel.

Pretty fascinating. It was a fairly quick appointment to be fair. The whole routine was done in fifteen minutes, and by the time we were done, Kai had fallen asleep on the table, so I dressed him and off we went. 

The wonderful Dr Williams gave us a thumbs up – Kai’s stomach looks good, the small bowel looks good, everything apparently is working as it’s meant too.

Woohoo! 

We’ll need to wait for the next clinic and from there will be able to book in the surgery! 

There is a small part of me that is very very nervous about this. About how Kai will respond to the anesthetic and how we’ll managing fast but needing a full belly for meds and the pain (more seizures?). About whether it’s the right thing to do, about whether it will cause us more complications or whether it will just fine, making things easier for all of us. 

But then I look at his beautiful, scratched up face, and I know how much he hates the tube down his nose. 

So, we wait for the next appointment. Gastrostomy, we’re making progress towards it! 

Nov 01
1

Kai 101: Purées and Eating 

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One of the things we were never sure Kai would be able to do is eat. He lost his suck. Twice. Well, it would be more honest to say that when he was born he never *had* a suck, and had to relearn once he was out of the glycine coma. Then when the two month curse hit, he lost it. It took a long long time to regain, and even now we have regular visits with our Speech and Language Therapist to make sure Kai’s swallow is safe.

We’ve had to do all sorts of things to make sure feeding is safe – smaller (and much more frustrating) teats, external pacing (frustrating for Kai) and all sorts of oral stimulation. It’s worked out pretty good though, so far. Because now we’ve progressed onto purée’s and porridge. Which essentially means anything that I can blend with our little hand held blender and run through a sieve is fair game.

Just kidding! What special needs mama has time to blend at this point? I don’t blend. I buy Piccolo and Ella’s and let *them* do all the blending. I do the heating up and the feeding. Maybe later down the line I’ll get into cooking and blending, but not right this minute. Till then, it’s a pretty fun time right now. It means that Kai is trying all sorts of new foods and what’s even *more* fun is that he’s able to very clearly show what he likes and does not like.

Guess which one this is?

Turns out lentils are in! Alright… what about these ones?

Lamb stew is definitely *not* in.

So, feeding. It’s happening. Sometimes we feed with a spoon. That makes things tricky, because it places quite a lot of food right at the front of his mouth and he has to work harder to get it down the back of his mouth. Sometimes we feed with a syringe, squirting it into his cheeks. Apparently it’s good exercise for his tongue, and is one of those steps he needs to be able to do before we get to chewing (could you imagine if my little guy, who apparently wouldn’t be able to orally eat, could CHEW?)

I’m glad it’s happening. We offer food orally pretty much every meal, unless he’s post ictal. Which is to say, post seizure-y and completely konked out. It wouldn’t be safe to feed him when he’s that lethargic and groggy. We offer him as much as he wants – he’s quite cute at letting us know when he’s tired and had enough. I say cute, but what it really means is that he’ll point blank refuse to open his mouth, his lips all pursed together. Looks like this:

And at that point, we’re done. I’ll never force him to eat orally. We have the tube, so the rest of his meal goes down it. Easiest thing in the world. Very not neurotypical though. A friend of mine was telling me about how her 1.5 year old sometimes just refuses to eat and so doesn’t until the next meal, when he’s hungry. My brain kept saying ‘just put it down the tube’ but of course, her little one doesn’t have a tube. I don’t know how the neurotypical manage, I’m glad we don’t have that stress of what to do if Kai can’t/won’t eat.

Still, I’m pretty pleased. Eating. High fives all round!

Oct 30
0

On Car Seat Fittings

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Kai is quickly growing out of his newborn car seat. We’ve got maybe a month before it’s unsafe, and we’ll need to upgrade.  However, Kai can’t support his head, so going down to Mothercare to pick out a new one wasn’t an option for us.

So, we went out to the In Car Safety Centre in Milton Keynes. They have a million different car seats, all various shapes and colours, with different features and support. It was *impressive*. Mothercare’s car seat range has nothing on this place. 


For what we needed (head support, reclining and ideally rare facing, with a swivel function if possible) there were only a handful of options that were suitable for us.

I was delighted that the one that suited us best was teal (anyone who knows me knows teal is my thing). Also, look how grown up he is! Sitting in a big boy car seat! Apparently it’s comfy enough to go sleep in, too.

We tried two, hauling them out into the car park to try them in our car. Turns out our car has fancy racer-esque seats in the back. Meaning they’re not flat, they’re on a slight angle and a little bit bucketed. Ideal if you’re sitting on your bum, not if the fancy new car seat plugs into the isofix things imbedded in the seat structure.

Blah. There’s another magic seat that’s apparently amazing that they’re hoping to stock in a month or two. Kai still has some room in his newborn car seat so we have a month or two.

So we thought we’d leave it and come back to it when the other seat is in.

Will keep you posted!

 

Oct 27
0

On an accidental trial of not having a tube

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We have a community team of nurses who help with the general small things. Taking bloods, organising hospital prescriptions, giving the heads up to the on-call paediatric registrar when we have to go into A&E, and providing the NG tubes. Unfortunately, our community team is also short staffed. And they have to deal with the NHS budget, which is not as well funded as we would like it to be.

Kai pulled his tube out last Thursday, and we didn’t have any spare tubes. We were meant to see our community team on the Friday, but they cancelled. They knew we were without a tube, so I waited. I called on Saturday. And Sunday. But they were busy and I know that translates to: there are other children having a much harder time than Kai, so I waited a bit more.

Here’s the thing though – without a tube Kai had to take all his feeds and meds ORALLY.He tolerated them well, mostly.

We’d never done that before. Meds are 100% down the tube, because 1) they taste horrific and 2) because then we know exactly how much he’s getting. No need to worry about dribble, or whatever is ending up on his shirt. He tolerated them well, mostly. There was one med that burnt the skin around his face (I hate that we need to give it him at all… if it’s burning his face I hate to think it what’s doing to the inside of his belly).

There were a few projectile vomits will we were figuring things out… like when we gave meds too early in the feed (fail) or didn’t burp him as long as we should have (usually we vent via the tube) but once we got everything sorted, Kai did really really well. I enjoyed his beautiful face, free of tubes and tapes. The rash that comes with the tapes was almost healed, and he was the sweetest little guy. I love his little face and it was SO NICE to see it tube free. Seriously. Look at this face:

Except for one thing. By Monday Kai had had a good morning, and had a nap after lunch. Just like always. 

Except, then I couldn’t wake him for his afternoon feed.

And I tried, hey. I spent over an hour and a half trying to wake Kai for a feed. Everything from tickling to stripping him off and dancing (which usually almost ALWAYS works). I even put him in cold shower, just for a moment. Kai didn’t wake. He did respond, in a very lethargic, leave me alone kind of shrug way. But wakefulness. No crying. Just very lethargic sleeping.

Any NKH parent would recognise this as a big, giant red neon sign. With multiple exclamation marks. Misted with a nice cloud of fear.

I got a second opinion (it’s always best to get a second opinion when you have The Fear).  I took Kai down to see one of our neighbours, who is a paediatrician (I love our neighbours, we really lucked out). She’s lovely, and did a lot to calm my nerves.

Kai’s temp was good, his O2 stats were fine, his heartbeat was okay. He didn’t have a rash, there wasn’t anything really to be concerned about. Apart from the not waking up, thing. She did the sternum rub – which is a pain test for unconsciousness. As far as a test goes, it does have a few edge cases that mean it’s not the most accurate test, but usually, in pinch, like this, it was enough to raise concern.

Kai didn’t really respond to the painful stimulus. He kind of wriggled his eyebrows in an almost frown. I was hoping he’d wake, but no such luck. So, we were advised to go visit the A&E.

Surprisingly, that didn’t freak me out. Once I knew what we were doing, and we had a plan, I could get on board with that. I know what happens in the A&E, I have things I’m meant to do for an A&E visit, so suddenly I’d gone from the world of not knowing, into the slightly more comforting/familiar world of we’re going to the A&E.

I accepted a ride from our neighbour’s husband, and after loading everything I knew we’d need for an A&E visit off we went.  On the way I called our community nurse to let the paeds reg know we were coming.

Here’s the thing though – just as we were driving into the hospital gates, Kai started coming around. Kind of.

Oh. My. Days.

He went straight back to sleep, but it was the first sign of life I’d seen all afternoon.  We still went into the A&E (they were expecting us, so we were moved right through, no need to wait in the waiting room) and got Kai checked out. He got bloods taken, and had the consultant look him over. More importantly, while we were waiting for the blood results to come back, Kai got an NG tube put in.

Thank fuck.

Long story short, after several hours at the A&E, Kai’s bloods came back clear and we were given the thumbs up to go home. They suspect it was an issue with medication delivery, which is to say that because Kai dribbles when he eats, he wasn’t getting the full dose of medication. Because he wasn’t getting the full dose, his glycine levels were probably elevated and made him overly lethargic.

I was grateful we were able to go home, and that Kai was fine for the minute. I was furious though, that if the reason really was medication delivery – then our A&E appointment could have been avoided altogether by a less busy community team. If we’d had a spare NG tube, this wouldn’t have happened.

So, I did what any parent would do. I convinced our GP to write a private script for NG tubes and asked our pharmacy to order them in especially for us. However, because the minimum order of tubes is 100, I bought 100 NG tubes. It cost a bit under £200.

I know – I know. I know, the NHS should have provided the tubes. This shouldn’t have happened, and I shouldn’t have had to spend hundreds of pounds for something Kai needs. I know. But the reality is that we slipped through the cracks, despite my calls and emails and loud mama bear noises. The peace of mind that comes with having a huge supply of NG tubes is massive. We will never run out of tubes again, and I’m glad.

The reassurance and relief is worth it.

The other thing that’s come out of it is the confirmation that Kai can not manage without a tube. He can not manage orally. I did wonder, and I’m glad we tried but I will no long wonder. I am not keen for him to be tube free, and 100% oral anymore. I’ve seen what it does to our little guy, and he is 100% better off with a tube.

The things you learn, hey?

Either way, we were able to come home and Kai is fine now. I’m grateful that’s the case.

Oct 25
0

On the very first step towards a gastrostomy

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Hopefully, the days of that NG tube on Kai’s face are finally, finally numbered. We had our very first consultation with a lady from the paediatric gastro surgery team. She was lovely. I liked her. The initial appointment was to confirm that Kai was indeed a candidate for a gastrostomy. I didn’t even say anything, really. Moments after we walked in, Kai tugged on his ng tube and I lunged across the buggy to detangle his chubby little fingers.

“That,” she said, “is an excellent reason to get a gastrostomy.”

I wanted to hug her. I was prepared for a battle. In my head I’d listed all the reasons why I thought Kai should have a gastrostomy, I had the support from our Speech and Language therapist, our palliative care team and our primary care neurologist. But it was all for naught: she saw that Kai was intentionally pulling on the tube, the scratches over his face where he’d missed, known from his notes he had seizures and sleepy periods and that was enough.

Hallelujah.

Not having to fight for something, not having to convince and follow up and remind – what a relief. It felt like the stars had aligned.

We talked about the differences between a peg and button, we talked about what the surgery involves and the risks and the benefits. It was a quick one because I was mostly all up to speed and didn’t have any questions. Apart from one.

Turns out, if Kai is over 10kg, and fairly close to his first birthday, we can skip the PEG and go to a button. Woohoo! Kai is currently at 9.78kg and is one month out. Ish. Considering the waiting list is currently 6 weeks long, I’m confident we go straight to a button.

Side note:
PEG – stands for Percutaneous, endoscopic gastrostomy tube. Its a thin tube that a small bumper at the end. It’s sent down the throat to the stomach, where it is threaded through a small hole in the abdomen. It has a bit of a tube that sticks out, and unfortunately, to be removed requires another surgical operation.

The Button – Instead of the tube that’s held in place with a bumper, there is a little silicone button that’s held in place by an inflatable balloon. It means that it can be changed as required, and doesn’t need to have a tube attached.

I left happy. They’re going to schedule a fluoroscopy next to make sure Kai’s stomach and small bowel are all in the right places and work as expected.

Fingers crossed. As helpful as the NG has been, we’re so so ready for a gastrostomy!

Oct 23
0

On avoiding a minivan

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Before Kai, we had a zippy little two door Ford Fiesta. I say we, but it was really Sam’s car. It was blue and racy and drove like it loved being on the road.

Zippy Blue Car

Living in London, we basically never used it. Not unless we were road tripping or doing an extreme, pre-party grocery shop.

Except then we had Kai. Suddenly every day was a visit to the hospital. When we graduated from NICU, it was a way to get to our local hospital, and then PICU, and then hospice. It became our lifeline between home and wherever Kai was.

When we finally made it home (hurrah!) it became even more important. I’m not willing to take Kai on public transport just yet. Every time Kai got a cold, a sniffle or a cough we’d end up back in hospital. In London, public transport is where you catch all the things. Fine if you’re an adult with an immune system. NOT fine if you’re a small baby with complex needs that are exacerbated by a cold.

So, if we have to go somewhere, we drive. Hospital appointments mostly. The GP occasionally. Sometimes up to see Sam’s folks, when we need a break and some love and an extra pair of arms so we could do things like shower and drink hot drinks while they are still hot.

The thing with having a special needs baby is that he has a lot of STUFF. Just, stuff that goes everywhere with us. Trying to fit it all in a two door fiesta required the Tetris skills of a master. We’d spend forever packing everything down. Taking the wheels off the buggy, using every spare inch of space we could, while still making sure everything we needed and all the emergency just in case stuff was within reach.

It was insane.

So, Sam sold his zippy little blue fiesta and bought a much larger, blue Vauxhall Astra estate.

Bigger, less zippy car

Oh. My. Days.

Four doors?! Giant boot space?! We were in heaven. So much spaaaaaace. We still manage to pack everything in, but the difference is astounding.

The first is that we can turn the airbag off, so when it’s just me driving Kai can sit up the front with me. Game changing when it’s just the two of us! It means I’m not frantically trying to look in my review mirror through the mirror on the back of the seat to see if he’s having a seizure or not. Safer for everyone, really.

What’s more, when we’re going somewhere for a few nights, there is more than enough room for all the extra kit we need to take. Enough that no one needs to sit with gear on their laps.

So far, the new car? Big win.