Nov 20
0

On still waiting for the corner to turn

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This past week has been tough. I’m going cabin crazy, but couldn’t possibly leave. I’m sleep deprived, on edge and the whole house is emotional right now.

Kai is still sick. The 3-5 days thing was bs. We’re doing NKH bronchiolitis- the kind that’s never ending, the dig deep marathon kind of ill. I’m over the fever spikes and mucus vomits and the suctioning. We even had to start oxygen yesterday, for a bit when Kai couldn’t manage to hold his own o2 levels.

Also, Kai is having crazy seizures. We’ve gone from 10-25 seconds to a minute and a half seizures. What was maybe two or three a day last week is now twelve a day, minimum.

And they’re violent. Kai is now very aware of them and will cry in the middle before he’s swept along with the shaking and jerking and tense horrid all of it. It stops when his brain can’t handle anymore and it konks out.

And as I’m holding his little body and crooning to him I’m crying too. Watching him suffer is tough. Everything comes second right now. I haven’t showered and my flat is a bombsite and breakfast happened way past lunch time… I’m too focused on Kai. Too consumed by his pain to do anything more than be right next time him.

I spend my down time googling more epilepsy research. More about seizures and what else can we do? We already listen to Mozart K448. He’s already on DHA (thanks Omega 3).

Neurofeedback is our next thing. I hope, with a longing I can’t even explain, that it helps his seizures. (Again thank you to everyone who has donated. If you’d like to donate to therapy for Kai you can do so at justgiving.com/crowdfunding/help-for-kai )

But while we’re looking for the next thing that will help Kai, I can’t help but feel we’re fighting symptom fires and not the cause.

I hate NKH, so much. It’s horrid. Come on UCL researchers. We’re cheering you on. You can do it. Make gene therapy work for NKH. Please.

Nov 17
0

On managing bronchiolitis

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We’re at home, which is more comfortable for Kai (and generally just better for everyone). We’ve battened down the hatches and are holding fort against bronchiolitis- its hard work hey.

I’ve cancelled all Kai’s appointments (six across four days not including the nurse visits. I’m gutted because some were so difficult to arrange!!) and it’s just me with my full focus on Kai. I literally haven’t left the house since we got back from the hospital. I designed and printed our own observation sheets and we do them mostly every hour. The benefits of design skill, hey? Mostly so I can see at a glance how Kai’s doing, and fend off anything before it happens. He’s managing, but it’s clear it’s a struggle. Poor little guy.

Kai requires regular suctioning out his nose and mouth, which is horrid for us both. It’s literally me suctioning the goopy flem out from his nose and the back of his throat. It’s thick and horrid and watching it go down the tube makes me want to gag. But Kai always breathes better after, so we do it. Not too often because we don’t want his body to go into overdrive and make more, just when he’s struggling. It’s pretty gross – but hey ho. The things we do.

We haven’t needed oxygen yet (thank goodness) but his temp keeps going up, and when it does his heart rate and respiratory rate skyrocket. I’m trying to manage it with Calpol (the magic drug for babies) but watching the fever bounce up and down makes me feel like I’m in a never-ending loop.

Observations, meds, chest percussions, feeds, suction, nappy change, seizures.

 

The consultant said it would be about five days of unpleasantness, with the worst of it being Day two/three (meaning today…. awesome). I know this is standard for special needs kids. I know it is. But it’s my first time through this particular rodeo – last time I let them convince me we should be on the ward (bad call). It’s hard work, and I do it because I love Kai (obvs) but also because I can’t not.

However the idea that we’ll be experiencing this on and off all winter long for all the years that Kai is with us? It’s like staring into a tunnel with no light at the end. I hate this. I hate that Kai is suffering, that he’ll forever be medically fragile. That anytime anyone is sick, they could pass it on to Kai and we’ll be right back here.

That, logically, it’s likely that these chest infections are what will bring about the end. From what we can tell, the end in kids with NKH is typically caused by respiratory issues. And for every chest infection Kai gets, the weaker his chest will become until one day he won’t be able to breathe. I hate the idea. I loathe it.

So while I’m here giving it my all, to help stave off this stupid stupid cold – I can’t help but feel like we’re edging towards the inevitable. And then I realise how overwhelming my fear is. My child has a terminal disorder, and I have The Fear. I hate this. NKH can suck it.

 

Nov 15
0

On the first hospital visit of flu season

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I’m not fighting with the registrar, exactly. We’re in A&E (of course we are – the start of flu season? We were bound to end up here eventually). It was a pretty typical lead up. Kai was working hard to just breathing and his chest was crackly. I’d been monitoring his o2 (92 – 95%, so slightly low), his heart rate (slightly fast), his respiratory rate (also fast) and his temp (37.9 – low grade fever).

Kai’s sick. Probably something like bronchiolitis.

I rang the nurses because I’d been oral suctioning after each cough. Not too often, because I didn’t want to increase his secretions. Just when he was having a tough time. What I wanted was a nurse to come do a nasal/trach suction (where they put a catheter down Kai’s nose and suction out the mucus he was having trouble shifting). I’d never done one and wasn’t about to do one maverick style now. Except that our nurses are a cautious bunch and instead of coming to do a suction, they were concerned with his o2 levels and suggested we take Kai to A&E.

While I’m always dubious of a ‘just in case’ hospital visit (dealing with the fear our team has and unnecessary escalation) I was keen at least to get swabs done to find out what was going on. So off we went. Kai rather magnificently timed a seizure which moved us from triage to majors (as always) and there we settled in.

Bloods, some suctioning, general monitoring – and yes, suspected broncolitis.

 

Now here’s where my polite but firm mama bear insistence comes in. They very politely told us that Kai’s white cell count is high and wanted to admit us, just for observation. We’d do repeat bloods and hopefully get discharged the next morning. It took a moment to absorb but when my brain finally kicked in here’s where it went:

  1. We only did bloods because Kai’s regular blood work is due. Not because they regularly do bloods with bronciolitis or with Kai’s general presentation/breathing concerns.
  2. White cell blood count could be high because of the elevated number of seizures (increased because: Kai is poorly) or because of bacterial infection.
  3. If it’s seizures, the white cell count will not go down tomorrow. Meaning they just want to watch Kai, just in case and we’ll be in the same position 12 hours from now.
  4. If it is a bacterial thing, the swabs will tell us. And the treatment is antibiotics, we have bottles at home for this very reason.
  5. We also have suction, o2 tanks and a stat monitor to manage symptoms.
  6. If we are admitted, Kai, as vulnerable as he is, is more likely to catch something else while we are here. Something worse, is my fear.
  7. The risk of sending us home is if Kai deteriorates rapidly overnight. The peak of bronciolitis is day two/three. So tomorrow and the day after.
  8. If Kai does deteriorate rapidly, we’re a short blue light ride from hospital but…
  9. Kai has a terminal disorder. We decided early on not to put Kai through extreme or invasive procedures to extend his life unnecessarily. So if we’re being rushed to hospital… there’s not much more they would do there than we would at home. IV Fluids, maybe. But IV Fluids in the face of a severe deterioration…

So. There is nothing they can do in hospital we can’t do at home. If we have clear parameters, we can come back if we need to. If we need to come back, it will be because Kai is very very poorly. The benefits of home outweigh the risks of leaving. I’ve talked to two registrars. We’re waiting on the consultant. I feel like we’re in here for just in case. The fear of the worst, really. Not because we need to be here.

I’m hoping we can go home. I essentially bought him in for nasal suctioning and the swab. I’ve already asked for an o2 plan and nasal/trach suction training.

We’ll see. Settling in and waiting for someone else to tell us we can go home is nails-on-blackboard mind numbing.

Fingers crossed.

Update: Took two hours but we’ve been discharged out of A&E and are going home, NOT up to the ward. We have some very clear parameters for what do and when, and for when to call an ambulance and go back. Which is to say, we got an O2 plan, and I got a run down on how to do nasal/trach suctioning. YES!

Here is the plan:

  • If Kai’s o2 stats drop anywhere near 92% we start o2, starting at 0.2L and go up as needed.
  • If his stats don’t go up, or Kai changes colour, we call an ambulance and blue light in.
  • If Kai has a fever we try calpol, and escalate to ibuprofen if need be.  If his temp doesn’t go down we try antibiotics and call the CCNs who no doubt will tell us to blue light it in.
  • If Kai is having a really rough time (increased heart rate or respiratory rate) and it doesn’t go down with the fever, we blue light in.

But you know what? While I absolutely would rush him back to hospital the merest hint of trouble, I’m pretty sure our little guy is going to be just fine at home!

Very very very glad we left. This is better for everyone.

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!