Apr 02
1

On being post gastro

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We’ve had the gastro for a while now, and I was surprised at how some things have changed.  The biggest being Mikaere doesn’t have the NG tube anymore, which means there is nothing on his face. Spelling it out – when he’s in the buggy you can’t tell that he has a metabolic disorder. He looks neuro-typical.

This means that the attention he gets has dramatically increased when we’re out and about

The scared ‘there is something wrong with this baby’ looks and side comments and fear when people peer into the depths of the buggy has gone. Other parents give me knowing smiles (what is it they know? I feel like there’s a shared secret I don’t get) and people now want to ask about how old he is and how tall he is and tell me how precious he looks. That annoys me, because Mikaere hasn’t changed, just people’s perception of him. Initially I wanted to explain about his disorder, tell them in detail about NKH but I soon realised that one, they don’t want to hear it, and two it takes up waaay too much time and all I really want to do is finish shopping and get Mikaere home away from possible-germ-carrying strangers.

It’s not all bad though, Mikaere still goes for his nose when he’s upset, but we don’t stop his hands anymore. Which means he’s got full access to touching his face. We don’t have to pass tubes anymore and I’m delighted we’re not dealing with the screams from that. Not trying to get an aspirate has been liberating. We’ve started a pseudo-blended diet, which has been good for Mikaere, I think.

The wound is healing nicely, slowly but surely. I’m positive it would have healed even faster has Mikaere not pulled out his stitches yesterday. We’re a few weeks past the gastro now and the button is at the right spot for him to reach without having to support his elbow or move his shoulder in a big sweeping movement. It’s in the sweet spot of reaching. So reach he did and with one big yank had pulled the stitches off the top of the button and almost pulled the whole thing out of his stomach. I had a heart attack and my poor sweet boy screamed. Good thing we were already in hospital for another appointment. And rushed upstairs to the ward to find the on-call surgical reg, who pushed the button back in and restitched what he could.

So cheeky. Mikaere’s clearly been a bit more sore than usual and on top of that he’s not tolerating his feeds/meds as well as he usually would. Which means we’re in the world of all the vomiting. Like, projectile out the nose vomiting. All the time. Everywhere.

We’ve slowed down the rate at which we feed him, the volume and what we’re feeding him (no advice needed, is what I’m saying). Everything is gentle gentle. We vent frequently, the feed goes across three hours (previously took half an hour). He will eat a tiny amount orally, but no where near where he used to. So we spend a large portion of our days feeding.

I’m getting really good at getting baby food vomit stains out of things, too.

Hey ho. Give us a few weeks of healing time and I’m positive things will be better.

I can’t believe we’re on the other side. Mikaere has a gastrostomy. Hallelujah!

Mar 30
1

On the ward

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I’ve never been so grateful to be on the ward. Back to shared rooms and small cubby’s and nurses shared between three beds.

Mikaere tolerated his meds on a diarolyte solution. He tolerated his formula. He did NOT tolerate food. Womp. There was vomit. So much vomit. And with every vomit we tack a day on to how long we’re in here.

It’s not so bad though. We’ve got our own cubicle at the minute, having moved out of the shared room. We’ll revert back to formula at a rate he can manage (slow and steady). And our days are slow and easy as Mikaere recovers.

We take walks around the ward, and set up a rotation of toys from the play specialist. We read books and sing songs and take naps. (Best tip I ever got was asking for an adult sized bed instead of a crib. Mikaere doesn’t roll, let alone climb over everything so is in no danger of rolling off with the sides up. It means we’ve got more space to manoeuvre him, space for us to sit and a nice spot for a joint nap).

The other bonus is that we’re still doing the meds ourselves (it’s a bit of an ongoing thing, Kai’s meds are complex, and the nurses and the chart are always wrong, so we do it to make sure it’s the right med at the right dose at the right time).

We’ve also continued our at home nurse help, which has been amazing. Because the hospital nurses are there for emergencies, we haven’t had to stay the whole time – knowing that there is someone Kai knows and we trust has been amazing. It meant I was able to come home to sleep once or twice (a proper 6 hour sleep in a bed after a shower is pure bliss).

We’re in no hurry. We’d obviously like to be home, but this doesn’t feel like our other hospital visits. Mikaere is not sick. He’s not fighting for health. He’s not struggling.

There is the the occasional vomit while his stomach recovers, so when the surgeons suggest another day I’m not heartbroken. Kai is going to be okay, and once he goes a full day without vomiting we’ll be discharged.

Update: later that very day we went home. Five days end to end we were here, from fasting to discharge. I’d expected to be here at least a week or two, five days feels swift. Hallelujah we’re home!!

Mar 28
1

On coming out of surgery

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When the nurse came into the parent room and said Mikaere was 30 minutes away I let out the tiniest breath. When she came in 20 minutes later and said not only was Mikaere now in PICU, but screaming I wanted to cry. Screaming meant he was awake. Screaming meant he wasn’t on a ventilator. Screaming meant all the fear and worst case scenarios were not us right this minute.

I barrelled across the hall, washed my hands with lightening speed and rushed to comfort my baby. He’s cheeks were bare – no ng tube. He was covered in wires, and as he thrashed about he caught his hands in them.  His little fingers scrambling in wires to measure his head rate, his respiratory rate, his o2. Red, green, yellow wires. The white chunky ones for blood pressure, the cuff around his forearm. The probe for secondary stats. Tubes going to his cannulas. A nasal o2 tube. And there, bang smack in the middle of his belly was his new button, stitched into his lily white skin.

So many tubes. So many wires.

But the relief was overwhelming. He’s fine. He’s alive and here and very opinionated in his objections of what just happened.

Despite the wires I scooped him up for a cuddle – that’s the bonus of having been in PICU before. You know which tubes are precious and which aren’t and which to watch for when you want a cuddle. I wasn’t intimidated by the wires. Not even close.

And sure enough, once he had a dummy and was safely snuggled in my arms, he settled a bit and stopped screaming.

My beautiful baby. It felt like we’d gambled for an improvement of his quality of life and we’d come through. The relief really was overwhelming and I felt like I was constantly holding back tears. He was fine. He was here. No coma. No vent. Just baby outrage and cuddles.

As he calmed and we settled into the afternoon, everyone relaxed. We stepped back in the world of intensive care. We said hi to the nurses we knew, who knew us. They all marvelled over how big Mikaere had gotten. We knew the system, we knew the room. It was all eerily familiar.

Once Mikaere was on pain relief and happy bundled up in my arms, the weight of the world dropped from my shoulders and we relaxed.

It felt weird to be in intensive care relaxed. The last time we were here we were being discharged into hospice on end of life care. Mikaere got baptised in that bay over there, a just in case emergency baptism when we weren’t sure whether he was even going to make to hospice. That bay was where he met his Grandad Gedge for the first time. We spent Boxing Day last year over there, and hours upon hours in the bay we were currently in. This room was full of grief and memories. Our fears were all here. It felt weird to be there and not feel that. Mikaere was *well*, the most vulnerable moment had past and we were in PICU just in case.

I could feel the grief of other families though. I heard a ventilator beep and recognised it as an o2 drop. Maybe a blockage in the tube, maybe some suction required. I heard someone else crying over their baby. I heard a pump beep indicating that the syringe needed to be changed over, a mum try comfort her son, a support worker talking in hushed tones and a musical light show as a distraction attempt.

I heard all these things and remembered and was infinitely grateful that none of those things were Mikaere right now. We’d lived that before and instead my son had only observational wires and a dextrose/pain relief solution through a single cannula. That and his brand spanking new gastro button. All positive things. None of these things were dire, intensive care things.

Thank FUCK.

Even better, one of the consultants stopped by for a chat and some baby love. She mentioned that if they needed the bed we’d be the first ones to be evicted from PICU up to the ward. I loved her for sharing that. That if anything happened, Mikaere was the healthiest in the room. He’s never been the healthiest in PICU before.

And so we wait. We see how Mikaere goes overnight. How he tolerates formula. How he manages his pain. And tomorrow, fingers crossed, we’ll be discharged from PICU and make it up to the ward.

Mar 26
2

On between the MRI and the Gastro

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We saw him briefly. So briefly. We knew they were coming because we heard them readying him for transport. I was already standing to the side when the doors burst open. Our Tuesday carer, Wai, beside me.

He looked so little. He had a vent (horrid and wonderful life saving things that they are) and his eyes were taped shut. Two cannulas were in his feet.  Irritating, because we’d put numbing cream on his hands and inner elbows to help with the pain in going in.  His hands and arms were covered in red puncture marks where they’d tried and failed to get a cannula in. (Turns out the numbing cream is also a vascular constrictor.. fail).

He looked so little. So vulnerable.

Mikaere was being moved from the MRI clinic (an adult clinic which they’d commandeered for him because it was closer to the paediatric OR) up to theatre.

We rode up the elevator together and I gently held his tiny little hand. More for my comfort than his.

We stopped in the corridor near the OR – no parents past this point. Only patients and doctors. Saying goodbye a second time was equally rubbish. Still, I was grateful I got a glimpse of Mikaere. That so far he was doing okay.

We went down the hall to the Paedatric Intensive Care. We knew he’d be going there after and were told we could leave our stuff in the parent room.

It’s a tricky place, fraught with familiarity and grief from when we were there last year. I got flashbacks of Christmas and New Years. The microwave dinners we’d had on our knees, wolfing down food so we could go back into PICU. The tears, the quick power naps, the unbearable waiting that happened when we had visitors and it wasn’t my turn to be with Kai (it’s only two visitors to a bed, so turn taking is a thing). Mostly I remembered the fear. Of not knowing what was going to happen, of Mikaere sinking into a worse and worse hole. Of everything seeming so overwhelming and horrid.

I’m glad we weren’t under the same circumstances this time. Oh waiting. I willed time to pass quickly.

Mar 23
1

On Getting Underway

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I was left standing the waiting room with a handful of my babies clothes and his dummy, my heart in my throat. Walking away was rubbish, watching four strangers crowd around my baby, looking so little on the giant adult sized bed, blue gloved hands busy attaching probes and doing medical things as Mikaere lay there unconscious as we were ushered out the door. Leaving was hard. Trusting them to do their jobs and their jobs well when my little guy was asleep on the table… difficult.

I was unprepared for the leaving part. We’d been waiting so long for this all to finally go ahead, that I was more focused on tee-ing everything up, half believing it would, again, be postponed that when it was go time, I was unprepared. But there you have it, after months and months of waiting my son was right this minute being put under and I was being gently  pulled out of the cubicle.

I wanted to cry when we left him and I almost caused a motherly fuss – my fear was overwhelming. (I don’t know exactly what I’d be fussing over, the right to stay? It doesn’t make any sense, because I know we’d never be able to stay. Emotions – not always logical).

There was a girl on the ward last night, while Mikaere fasted. She must be 18, young enough to be on the paediatric ward but old enough have a 1 year old of her own. She’s dislocated her jaw and has all the fear about the pain of having it reset in place. I overheard a lot of her fears and anger and confusion. Hours of it. She desperately wants to see her baby but can’t get over the fear of the pain that comes with having her jaw reset. And until it is, she can’t leave the hospital.

I’m not getting down on her, because I was there when they tried the first time and I heard how that went. But at the same time, I think the moral of the story is there is a choice. A short, sharp spike of pain and its done – no more pain, or a long drawn out pain while you wait and sit with your fears and fuss.

The main thing for me was how her fuss made things difficult for everyone else. For her family, for the nurses who were trying their very best to help her with her pain, the consultant who was on hand if she wanted to try. Us, and everyone else in the room who silently listened to her process all the things.

And as I sit in the waiting room for the first of this long drawn out waiting process of my own, I feel like it’s best to just get on with it. Waiting is rubbish and my fear of it all going twisty and wrong is high, but there is no point making a fuss. I don’t want to be like that girl on the ward going on and on, when I have the power to emotionally move myself forward.  So instead I sit quietly and write you guys a blog post.

Mikaere’s under the general anaesthetic and is having an MRI. Up next, gastrostomy. I can’t sit still and I keep repeating that Mikaere will be fine. He will be. And I wait for the MRI doors to open.

Mar 21
0

An update on the superhero suit

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We got the suit! It’s only been a week so far and we’re easing into it but oh my days, what a difference. I know that it makes sense, with a supportive suit he should be able to support himself more, and he DOES. Sitting is better, it’s like his whole trunk is supported so he doesn’t have to work as hard, which means he can work on holding his head or swinging his arm or whatever it is he’s trying to do.

The only downside is that it is quite warm – making Kai warmer than usual. Difficult, with the helmet, which also holds all the heat in. We’re taking temperatures like nobodies business to make sure he’s acclimatising okay (and he is, what a champ) but it’s just something we’re aware of when we dress him in the morning. Thinking about layers and all the things. Checking his body for red marks to make sure the compression suit isn’t compressing him in a way that causes pain, or leaves red marks.

It’s weird to think about all these extra things, and what a difference it makes. It also makes me feel like we’re worlds away from the neurotypical path. Hey ho, onwards we go. I’m glad we’ve found something that helps Mikaere, that makes his life a little bit easier.

Hurrah for Superhero suits, hey?

Mar 19
0

On the head holding

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I’m about to share a thing.  Before I do, before I share – I want you to understand that these two photos were taken in The Moment. You know what I’m talking about, right? How in The Moment everything is good and perfect and everything looks positive and rosy. I want to share with you all the positive and rosy things, because I always want to delight in these beautiful milestones.  But I want you to understand that either side of The Moment did not look like this, and that The Moment? The Moment was fleeting.

So, for a few seconds, during physio, Mikaere held his head up.

For a few seconds, he held his head up all by himself. He was able to keep his head in midline AND look to one side.

There was a moment, a beautiful beautiful moment where Mikaere smashed out another milestone, because for those few seconds, my son could hold up his own head.

This one is bittersweet, because a neuro-typical baby learns to hold their head at around the 2-3 month mark… Kai has a full year plus some on that. But you know what? Dr Doom and Gloom told us we’d never get here. That we’d never see this – my sweet boy holding up his own head.

I live for these days, the ones that contain The Moment. Today was a good day.

Side note: Not shown: the moment Kai pulled out his ng tube just before physio started. Also not shown: the screaming fit that happened at the end of physio that caused a giant vomit. Our days are always up and down.

Mar 16
0

On not having a paediatrician because the NHS is underfunded

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Everyone knows Mikaere is medically fragile. I’ve talked before about how large our medical team is and how we have an appointment of some kind every day of the week. Our life is a series of appointments and therapy and special needs groups. We go to all these things because it helps Mikaere, it keeps him safe, minimises risks, teaches him skills and relaxes him and puts all sorts of services in place for when things go belly up and he needs them. It’s a full time job, organising and ferrying him about, being present, understanding the goals and raising concerns and following up.

Of all these services, our paediatrician is the most utalised. He’s the person who knows Kai the best, who is our first port of call. He orders our meds and the bloods and all the checks. He’s our go-to person when anything is wrong, if the meds are out or there are more seizures or we need something. He orders all the referrals, the X-rays to check for hip formation and scoliosis. The orthotic referral for the suit, the physio referrals, the gastro surgery referrals. He pulls checks on all the difference services, speech and language, physio, OT, nutrition. He’s the person we work with, the person we make a plan with and who helps us works the system to make it happen.

We’re lucky that our paediatrician is covered on the NHS, as are most of the services Mikaere uses. For those not in the UK, the NHS stands for the National Health Service. It means that the basics of our healthcare system are free at the point of care. Meaning, we’re lucky that for the bulk of Mikaere’s care, it’s covered by the tax we pay.

Here’s the thing though. The NHS is chronically underfunded. It may not look like it from the outside, but it is.

Mikaere’s paediatrician was a locum, which means he was temporary while they found a permanent person to take that role. Which is fine, we knew this and accepted it. However, a month or two before end of the financial year the paediatric locums were let go as a cost saving measure. And – get this – there was a gap in care. There was a gap where there was NO ONE to pick up the case load, where Mikaere DID NOT have a paediatrician – our first port of call, someone to prescribe his many meds or chase anything (like that gastro we’re waiting for). We didn’t know where to go for all the things Mikaere needed.

My small, medically fragile and vulnerable son did not have a paediatrician because the hospital trust was told it needed to save some money.

As you can imagine, I raised an absolute stink about it. I emailed the Medical Director, Andrew Rhodes. I also emailed the Head of Child Services, James Gavin. I made an official complaint to the hospital, wherein they couldn’t tell me why officially why there was a gap in care. I emailed my MP, Justine Greening. I also emailed Jeremy Hunt (https://www.jeremyhunt.org/contact – and if you can, I’d ask that you email him too, and tell him chronically underfunding the NHS is having a crippling affect on the most vulnerable of our population). I’ve complained to CQC and I’m in the process of a complaint with our local ombudsman.

And nothing happened, nothing changed. I got a few platitudes and apologies but not much more. And that’s because the problem is bigger than the trust that runs our local hospital and provides basic care for Kai.

Underfunding at the NHS is happening and it’s affecting my family in real and tangible ways – for the worst.

And I know. I know we’re lucky to live in a country that covers care for us, and we’re grateful for that. Except that we also live in a system where we couldn’t afford care otherwise. We couldn’t afford to pay for private care. I had to give up my (wonderfully well paid) job to care for Mikaere, and point blank, if it came to it, we couldn’t afford care for Mikaere outside the NHS.  Just to be really clear, we are not talking about luxury care for Mikaere. We’re talking the basics of what he needs to be safe and to live.

We rely on the NHS for Kai’s care, and it has a direct affect on his quality of life.

NHS underfunding is happening and it terrifies me.

Mar 08
0

On the superstitious good things

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There’s this thing about good times. When things are good, I feel like I’m holding my breath waiting for them to be not good. Like I’m waiting for the other shoe to drop. I’m told this not uncommon when parenting a child with a progressive disorder. When you’ve been on end of life and expect to be again, any good time is by nature of NKH, temporary. A nice, tiny haven where you ache to be with every fibre of your being, knowing full well you can’t live in the realm of ‘everything will be okay forever’.

So Mikaere recently enjoyed a good period. I got a bit superstitious about it and didn’t want to say anything in case it jinxed it. This makes zero logical sense and I know that Kai’s wellbeing does not hinge on whether I say he’s doing well or not. Nonetheless, there a words we don’t say unless we have to, and when we do we touch wood. (I’ve never been more superstitious in my life – when there are big, important things you can’t control you find ways to make yourself feel better about them).

So. Let me tell you a big thing. Mikaere hadn’t had a seizure since before Christmas.

Until last week.

I can’t begin to tell you how we watched him progress with no seizures. How his eyesight got better, how he started sleeping through the night, how his tone improved, how we saw more smiles and how he started vocalising more and eating better. Doing everything more and better. When his brain wasn’t fighting seizures it was developing. With new skills and new all sorts.

We don’t know why the seizures stopped. It could be he was well. It could be the medications were bang on. It could be the neuroprotectant meds we started. It could be that the planets were aligned or the sands were blowing east in Africa.

We don’t know and that bothers me. If I don’t know then I cant replicate it. This giant balancing act we’re constantly managing, we’ve hit on a magic time and I don’t know what we’ve done. Maybe we didn’t do anything. Maybe this is something we can’t control.

I hate that idea, because now the seizures are back and I don’t know why or how to stop them.

The first one I just caught out of the edge of my eye and I wasn’t convinced it was a seizure. It could have been a stretch, or a twitch. The second one was unmistakably a seizure. Fuck. Fuck fuck fuck fuck fuck. I don’t even know how to explain the despair. That terrible sinking feeling, the ‘here we go, battlestations’ type familiar mentality you have when your baby has frequent fits.

I’ve spent hours pouring over my notes of his meds, all the activities we’ve done in the last few weeks to figure it what was going on. Was his meds off? Had he outgrown his meds, has he put on weight or lost weight? Was he in pain? Was it something he ate? Did I feed him something with unlisted ingredients that he’s reacting to? Was it something I stored his food in, did something leach? Did he have a temp, was he ill? Was he teething? Is this NKH progression? The constant back and forth and second guessing.

And then the constantly whirling questions about the affects of the seizures. Was Mikaere going to lose all the skills we’d been working on? Was he going to lose his developmental momentum? Were we going to back to sleeping in shifts and constantly on guard? Was this going to slip down into something more sinister?

I don’t know. It’s tough not knowing. I did what I could, which was check and recheck his emergency meds, the rescue medication. I settled in the counting and timing and comforting after each one. I became paranoid about leaving the room, and would even move him into the bathroom with me if I had to use the facilities.

I just, I hate this. I hate that my baby is suffering seizures again. They’re so disorienting and horrible for Mikaere. They mess up our days and we’re all out of routine.

And then it got worse. The seizures started clustering, and we started having to give emergency rescue medication. I cried the first time, as a gently administered a round of midazolam. Kai’s grandad once told us that it’s midazolam they use the euthanise whales that have stranded themselves. I hate sedating Mikaere. I hate watching him slip into that groggy fog, I hate watching him fight to stay conscious. I hate watching him seize more though, so I do it.

But then he seized again, multiple times. And throughout the day I was using more and more rescue meds.

And I realise that this is exactly what I worried about in the good times. That we’d end up here, multiple clusters of seizures, having to use emergency rescue meds, watching my baby suffer continuously and not knowing what damage is happening to his little brain.

Seizures are horrific. NKH is such a bitch

—-

I know I’m always asking. Help us find a cure. Donate, or buy an Eva book if you’re able. Every bit helps.

Mar 02
0

On The Charity Quiz

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I feel like we’ve got a few charity fundraisers under our belts now, but the quiz was one of my favourites – a proper good fundraising event. People came, we had a great time, we made lots of money for NKH Research.

It was a fantastic afternoon. We had two fab quiz masters (thanks Tony and Tess!) – who provided several rounds of hilarity. Some questions required some thinking but there was enough knowledge in the room that the questions could be answered (because there’s nothing worst than a quiz where you can’t answer any questions). There was a round about music, and pictures with landmarks and general knowledge. Lots of delicious pop culture questions (I’m no good at knowing the answers but I still enjoyed those rounds the best).

We even had a round about Mikaere and NKH – because how better to raising awareness about a rare disorder than making people answer questions about it? I did a bit of a spiel at the beginning of the quiz and later pulled all my questions from that content. However, there was a slight blunder when I gave an answer in a question. Faaaaail. Quiz master I’m not. It was great though, people were kind when I messed up. They also must have been paying attention, because most teams got all the questions right! High five for awareness, hey?

We had a great turn out, maybe 40-50 people? I love fundraisers, if only because our nearest and dearest make the effort to come and support all the things. It also meant that since the Quiz Mastering was taken care of by Tony + Tess, the bar by Sam + our friend Gareth managed the bar, once I’d got the food sorted (which was nothing fancy – mini cheese boards and pizza pinwheels, one for each table, assembled not even by me but by helpers (thanks Hari and Becky!!) I got to sit with Mikaere and chat. Full, proper conversations, with adults, actual friends, with nowhere else to be. (Isolation: it’s a thing). I drank my full of social goodness, it was amazing. So many wonderful people came – we’re blessed. Seriously.

Thank you to everyone who came. You guys are the best. We’re also thankful to Chris and Majestic Wines in Putney for helping with the wine/beer. We’re grateful and even more grateful to the people who bought it (it made for a very cherry winter afternoon).

Thanks to everyone who donated the prizes and the items for the raffles. Thank you to Tony and Tess for organising and for the generosity of everyone on the day. We managed to raise £1450 on the night, which will be matched (!!!) to a total of £2900. Not bad for an afternoon, hey?

These funds are all going to Joseph’s Goal to help towards NKH Research, and the gene therapy work done by Prof. Nick Greene in progress at UCL.

We’re so hopeful for a gene therapy cure. Thanks everyone who came for helping with that hope – we love you guys. What an amazing afternoon. Stay tuned for more fundraisers throughout the year. We’re thinking the next one should be a wine tasting – who’s in?