Ellly

A quick break from the doom and gloom seizure posts for just a moment – NKH Awareness Day was yesterday, and oh my days. So many people made their facebook profile pictures yellow. So many texted donations in. So many shared our posts and commented and liked and just… it was so comforting to see how much people care about our little guy. 

I’ve been quietly running a small campaign for the NKH families for funding the week before awarness day, over at fundnkhcure.com. I feel like we’ve spent so much time asking our friends and family for donations, that this one time, this one day of year it was okay to ask the NKH families for a small token.

Except that it spilt out around the edges, and then exploded on the day itself. Before I knew it facebook was yellow, and we managed to raise over £2000. 

I can’t even begin to express the love and genuine gratitude we have for the support. For every single person that changes their profile picture yellow. For every £3 donation. For every message, like, and share. We’re so lucky, so so so lucky that you all are on #teamMikaere.

You guys are amazing. You are literally the most beautiful, loving, fucking brilliant group of people I’ve ever had the privilege of being connected to. We’re so grateful for the support. Words aren’t enough to express how grateful we are. We love you guys. Thanks for being on our team x

Way back in NICU, when they told us about the seizures, I imagined that they’d be like the seizures you see on tv, the big grand mal ones with shaking and jerking and frothing. I was prepared for this big Acute Event, capital A, capital E.

Turns out our acute events are quiet. A small, tiny, twitch that won’t quit. I’ve been watching Kai’s hands rhythmically clench for almost two hours now. My babies brain is broken. So so so broken. 

I know which bits. I know why. I know which genes have a missense switch, I know how the p-protein isn’t doing what it’s meant to. I know his brain is broken. I’m  heartbroken there isn’t a way to fix it. So I watch the twitches, and wait for them to morph into fully blown seizures. We’ve already escalated. We did the first buccal five minutes in, after the first seizure that didn’t quit. The second fiveteen minutes later, when he was still twitching and seizing.

We even went as far as the next step, the rectal paraldehyde (that was good as far as lightening the mood went. Hard to be in tears when you’re holding your babies bum cheeks together and poo slides out in between your fingers). We’ve never gone that far at home before, and it worked for a moment or two.

Then the twitches were back. Even as I was holding his hands, his movements didn’t stop. That’s how you know it’s a seizure. So we escalated again, and gave a quarter loading dose of phenobarb.

The last time we did this was Christmas Day, the day he was admitted to intensive care. 

The fear, I have it. I have it in droves. This time I know we won’t go to hospital. We won’t be admitted to intensive care. Our symptom care nurse reassured me that we can manage this from home, that we were still a day or two away from a continuous infusion, and even then, we have a giant toolbox of meds underneath our bed to manage that. The emergency IV box for just in case. 

And I hate that it’s there and I hate that we need it and I hate that just in case is absolutely a possibility.

My baby is in a drug induced stupor, a groggy horrible dozey state, so so far from his happy self, and still with the twitches. It’s heartbreaking. I’m sleep deprived, because I can’t sleep when my baby is sleeping through the night. Sam and I are taking it in turns to sleep, which isn’t feasible in the long term, but allowing Kai just to seize isn’t an option. It just isn’t. 

I hate this. I hate NKH so much. This is why we fundraise. This is why we’re looking at putting on events. If you ever wanted to hold a bake sale, or a BBQ or a poker night for NKH, please do. Do it now. 

I have The Fear that with each escalation point on our seizure plan, we move away from symptom care into end of life. The Fear. I have it.

I hate giving him buccal. I hate it I hate it hate it hate it. The midazolam buccal is an anti-epileptic that comes ready drawn in a 2.5mg syringe. We squeeze it between his gum and his cheek, half each side. It’s horrid. It smells horrid, it tastes horrid. And worse, it’s a sedative. It knocks Kai out and makes him groggy. 

Because that’s how we deal with seizures we can’t control. We sedate Kai. And I hate it. Hate it hate it hate it. But despite hating it I follow the plan: we give one when he has a seizure of more than 5 minutes, or if he has three seizures of any length in an hour. 

I hate that that’s the choice: a sedated groggy baby or a baby that seizes over and over and over again. I’m grateful that we do have something that is currently effective against the seizures, because it was only Christmas when there was nothing that helped, that Kai was in a never ending seizure coma in intensive care. But I hate hate hate that sedation is our only option.

Kai hates it too. He screams when it’s in his mouth. As I syringe it in I’m almost crying myself, crooning apologies and platitudes and trying to offer what comfort I can. Some days I debate with myself whether it would be better to let him have the seizures. In between the seizures we’d be in the presence of our little guy, awake and here and with us.

I’ve never won that argument with myself though, his brain is too precious, as broken as it is. So I count how long each seizure is, I write down when each starts. And when we hit the threshold I give the buccal and then I cry. It’s heartbreaking hey, to have to sedate your baby to stop seizures. 

Some days it’s just about moving forward, and some days it hits me like a ton of bricks. 

Kai’s grandma sings in the Dunstable Rock Choir, and they held an informal concert with raffle for NKH research. 

Oh. My. Days.

I’m a bit overwhelmed. It was a lovely concert, with lots of fun tunes. Kai enjoyed it, kicking happily to the first few tunes before giving over to sleep. We had the opportunity to speak mid concert and Sam did. It was emotional. Telling a hall full of people that our little baby, the baby that’s just right here with the beautiful chubby cheeks has a terminal disorder with limited treatment options… that was rough. Both Sam and I were felt pretty emotionally raw, sharing these tough things with a hall full of strangers, but we were so well received. 

There was so much love. So many people stopped to talk to us, and so many people donated, either by buying from the raffle, donating to the raffle, or by putting money into the bucket. We raised a staggering £700 in a night! £850, if you count the donations that came in after.

I can’t even begin to share how much that night meant to us, as a little family. Having such an overwhelming positive response from so many people, having so many people donate towards research. Having so many people say hey, we believe in a future for your baby, and we want to help. It was intense. 

We’re so grateful. Thank to you to the choir, thanks to everyone who came out, thank you to everyone who donated. We’re so so thankful. 

YOU GUYS! We’re so grateful to everyone who has offered to help fundraise. From work Bake Sale’s and raffles, BBQ’s birthday parties and housewarmings, were so so lucky.

Because we have so much going on and there a few things all over the place, we thought we’d create one spot to find everything. So:

www.teammikaere.com

It’s got a list of how much money we’ve raised, what events are coming up, how to donate, links to my blog and Sam’s blog, and how to donate.

Its even got a store, were selling a few fun things with all proceeds going to NKH research. 

We’re so lucky for your guys support. Thanks for being in our corner.

We’ve been sticking close to home, because going further afield requires more kit and organisation than the effort warrants. Even a trip to the supermarket not two minutes away requires the emergency kit (ambulance directive, emergency meds, the small folder of notes, just in case).But sometimes there is a something that’s worth leaving for. Friends of ours, friends we adore and are so grateful for were having a baby shower and I was *determined* that we were going to go.

So, we packed the emergency kit, and the meds for the day and the pump and supplies and testing strips and syringes (as well as the usual baby stuff) and off we went, driving across London. We were several hours late but oh, the sheer glee at making it at all was brilliant. We were there! At a baby shower! With other adult people! With our friends!!

It was nice. It was more than nice. I felt like a superhero, that if we were managing to get out and about that we could do anything (my bar is set pretty low, just showing up right now is huge for us). Making it to the baby shower, hanging out with our friends, meeting people who didn’t know our story and didn’t start the conversation with grief/pity/awe was so refreshing. 

And our friends, oh my days. They came to visit us when we were in intensive care, so that they could love on Kai outside of hospital – I was positively ecstatic.

Sometimes the huge, crazy effort of getting anywhere is absolutely worth it. Good day. High five us!

Since being home we’re easing into a routine. It’s not smooth sailing, more like a clunky side jerk, but we try.

Kai’s routine revolves around medication and feeds.  We start at 5:30am and finish up around 1am, and in between it’s a dance of setting up feeds, drawing up medication, therapy, sterilising, washing and naps.

It’s a full on, and while we’d like to settle into it, we’re constantly thrown off. Seizures ruin everything. We have someone come and visit us pretty much every weekday. Nurses, therapists, dieticians, support workers. A visit or therapy that starts late or is commandeered by a nap will push out feeds (as if we did something like physio on a feed he’d vomit) which push out meds (as some of his meds require a full stomach) which push out the next lot of meds (as they need to be so many hours apart). 

We try though. We plunder through and try to manage all the ebbs and flows. In naps I do all the washing (we use cloth nappies, so there’s an endless cycle of washing on) and all the sterilising (every syringe, every bottle) and drawing up meds and formula, so it’s easier to manage. We try squeeze in homework. A few moments of tummy time here, leg stretching there, hands crossing the midline. We massage the old infected subcut site, to bring down the lumpy abscess that was left. We do a few moments with whatever portage has left us with – this week its a wind up music box. We hold it against his hands, so he can feel the vibrations, but can also pull away if he’s not enjoying it. The week before it was the space blanket, kicking and touching to get the crinkley noise.

It does make it tricky for fun stuff. Which is why I’m grateful for Sam. By the time Sam gets home he’s full of energy and happy to see Kai – he takes on the light hearted moments in the evening.

Sometimes we even manage a social something a rather. A walk with our NCT friends. Dinner with our nearest and dearest. Play dates with friends who have their own babes.

This is assuming, of course, that we’re having a good day. If Kai’s having back to back seizures, is inconsolable or requires a hospital visit everything goes out the window. In those times we operate on adrenaline. Meds and feeds are prioritised, everything else is dropped while we concentrate on managing whatever crisis comes our way. 

So, yes we have a routine. And we’d love love love to settle into it. And we do, on good days. On bad days, we do what we can to get through. So yes, we have a routine. We’re not pro’s at it yet, but we’re getting there. 

When I was heavily pregnant August last year, I was very very excited when I managed to procure two tickets to see Harry Potter and the Cursed Child. Fast forward to now, after months of waiting and our show date is finally coming up, and it became a bit of a logistical problem. While we were keen to go, getting care for Kai is difficult. He has an insane medication schedule, and he has equipment for feeding and there’s the testing that happens anytime we do anything with the tube.  Standard for special needs babies, but well over and above anything we could expect a babysitter to handle.

I have one friend (who I’m so grateful to) who comes on the occasional Monday and has taken on the task of learning how to feed Kai via the pump, and we have a hospice at home carer and a nurse who we’re allocated three hours every two weeks… however neither of those options were going to cut it for a whole evening.

So, we went back to hospice for respite. Logically it works out – they know him, Kai knows them and the space. Sam’s Mum came down to the hospice to spend some time with Kai. We stayed Tuesday night, and then on Wednesday morning Sam and I left to go to the theatre.

Well. 

Leaving Kai, as logical as it may seem, best case scenario and everything – I did not want to. I did not want to leave Kai for a whole day. I did not like it at all. Leaving Kai behind is always, always the hardest thing. I’ve come to realise that I know Kai best. I know how to care for him best. So leaving him to hospice nurses, who are amazing, and wonderful, is always going to be, well very very good, not as good as me and Sam.

I know that we can’t do 24/7. I know that it’s unreasonable to think that we can.  That doesn’t mean that emotionally I’m on the same level. I’m not. Leaving Kai was horrid.

We walked out, leaving Kai in the very capable care of the hospice nurses and his Grandma and went to the theatre. We had a lovely day in London, fancy lunch and dinners and the theatre. In a past life I would have been ecstatic. In the After, London is very loud and people are very rude, and care about things that aren’t important.

The show itself… some of it was very clever. Some of the magic tricks were brilliant. But I think that my idea of what is important has changed, and with my baby in hospice I found it hard to settle. I think that at the heart of it, the story could have been told in one show and there could have been less dancing wizard intermissions. I was very glad when it was finished, which was not how I expected to feel at all.  I think that’s what happens when something from a previous life is out of place in your current life.

If leaving was the worst, coming back was the absolute highlight of my week. When we arrived at hospice, I skipped the signing in and hurried down the hall. I could hear him crying (break my heart) and when I peeked into the door he was having his nappy changed. I waited till his bum was clean and dry and scooped in for a cuddle.

Oh the joy, the absolute joy. Kai stopped crying, and smiled. He even laughed, the first time I’d ever heard him do so. He was very very happy to see us, and knowing we were missed – though I hate the idea that he even had the chance to miss us – and that he was glad to see us made me so ridiculously happy. 

It was a nice night out, and I was glad we managed it. I don’t think it’ll happen again for a good long time though. Time with my baby is well too precious for things like theatre. I feel like we’ve had so little time with him, I’m loathe to give it up for something like dancing wizards.

I can’t even explain how grateful I am to be home with Kai and Sam. In our own place, with our own stuff, with all of that beautiful privacy. This past weekend we went for walks, and cuddled on the couch for family movie night (super not age appropriate, we watched War Dogs) and on Sunday? Sunday I spent the entire day in my pjs with my boys. Delectable.

No nurses, no home visits, no drama. I can’t even begin to tell how beautiful this time is. Sam even bought me tulips on a whim. I’m so lucky, I know the hashtag is overused, but genuinely, when I get to have a day like today I feel genuinely #blessed. Normality hey, it’s super underrated.

Well, fuck. I don’t know what to say. We’re in the A&E. At first it seems innocuous, we got Kai’s glycine bloods back from last week, and they were a staggering 177.

Now here’s the thing, 177 is good, it’s within normal glycine range…. but it’s not Kai’s normal. Kai’s normal sits at 250-300.

The thing with Sodium Benzoate is that if there is no glycine to bind to, then it gets toxic. If it’s toxic, then calcium and potassium get dangerously low. So I rang our CCN (community care nurse) and practically demanded bloods taken, so we could find out. She asked us to come in tomorrow, but I persisted. The glycine results are a good week old, and I worry. I worry because the last time his glycine was this low, we were admitted into intensive care.

On top of this,Kai’s been grizzly all day, and when he’s not grizzly he’s sleeping, for longer and longer periods (another worrying sign, similar to our pre-intensive care stay last time). And the seizures. Oh the seizures, they’re more frequent, and longer in duration. Add the vomiting and I’m all over the place. Is it that he’s teething? Is it that the vomiting is causing an absorption problem with the phenobarb? Is it sodium Benzoate toxicity? I don’t know. We got a taxi to a&e who were expecting us. We walked in to see Dr Lukas, one of our regular registrars from our last visit. I’m glad we knew him. I’m glad he knew us. I was so relieved.

So, we did bloods. Bloods for kidney function (sodium, potassium and calcium levels) and liver function (phenobarb levels) and for amino acids (oh hey glycine) and two pipes, covering blood/gas and a regular blood panel. 

But then Kai didn’t keep his feed down. And he was inconsolable. And then he had three seizures within an hour. Our emergency plan kicked in then, even when we were in a&e. We busted out our rescue meds, and Sam (having arrived after work, I’m so grateful for Sam and that he came) gave buccal midaz. Poor Kai. He was screaming at this point, and then the midaz knocked him for six. Oh my baby. 

I also called our symptom care team to let them know what was going on. I was very in control, but inside? All over the place. Emotionally well all over the place. I felt like I leaking out the edges, I was stressing out over his feed (we didn’t have a feed pump, and I kept forgetting to ask the nurses for what I needed, so every time they came back I was asking for something, a 20ml syringe for an aspirate, a 10ml syringe for a flush, sterile water to make up a feed, more for the flush… on and on. I was very glad when Sam arrived. I’m always much more centred when Sam is around).

We were moved into a bay closer to the nurses station, and then into the assessment unit. And then came the waiting. The endless endless waiting.

Fingers crossed hey. Fingers crossed he’s going to be okay.

Update: 

Bloods came back good hours and hours later. It’s teething. We think. It’s definitely not Sodium Benzoate toxicity. Thank goodness. It’s the not knowing that bothers me, so I’m grateful to know for certain. We’re going home.