Ellly

Since Kai has woken up he’s pulled his NG tube out four times in three days. We’ve had to mitten his hands to keep him from yanking it out. I struggle, because I can’t imagine the tube is pleasant. He gets his feed pumped into him, 140ml of milk over an hour and half, followed by a 2.5 hour break. He’s so frustrated, when he’s hungry he lets you know, but he’s so unsatisfied by the slow dribble. He can’t suck in a coordinated fashion and we worry that he’ll aspirate himself so he has the tube.

Every now and then we’ll try a bottle, but he struggles with that. Sometimes he’s fine, sometimes he’s not. And until he can be consistent with the bottle, he must have the tube for his meds.

He may always have a tube. We’re beginning to think about a g-tube. A tube directly into his belly. I’m pretty sure it’ll be easier and more pleasant for everyone, though it’s definitely an idea we’ve had to come to terms with. In the beginning, I was determined to get him home from the NICU so I could pretend that NKH was some terrible terrible nightmare that we could shake off. And for three beautiful weeks, it was. Sure there was the daily meds, but it wasn’t this. It wasn’t 24/7 nursing care. With the feeds and pumps and syringe drivers, with the four-six hourly meds and hourly checking of his subcut – the needle his has in his leg so they can pump anti epileptics into his body around the clock.

I can’t change any of those things, and I’m sorry that when Kai pulls out the NG tube we have to hold him down to put another one back in. Still, every time he does it I want to high five him, and silently cheer him on for fighting it. Everyday, he fights.

He’s getting pretty good at getting the mittens off too. You go little guy.

PS – those cheeks without the tube though! 

Today we bundled our little guy up and went to see our metabolic consultant and to get bloods. It’s been a while since we’ve seen our consultant guy, but I won’t lie, I was disappointed.

NKH is rare. So rare. Our metabolic guy has a kajillion rare metabolic disorders to worry about, and we are one of many.

Originally, I wanted our metabolic guy to be our hero. To be the consistent doctor guy in a world of a kajillion consultants, the guy who would help us manage our NKH day to day, to slow any deterioration and halt the acute episodes, who would have all the answers to our questions.

He doesn’t. He doesn’t because there are no answers. He doesn’t have a magic wand. He doesn’t have the time or the energy. It’s gotten to the point, where three months in, I as an NKH parent know more about how to manage NKH than our metabolic consultant. I am the expert. And I know this because he came right out and said it. While NKH is small segment of our metabolic consultants job, NKH is our life.

But I’m scared to be the expert. I’m overwhelmed with the enormity of it. I feel like I can’t possibly know enough or be enough, that I will do anything and everything I possibly can for my little guy and it won’t be enough. That he’ll suffer because of my mistakes, because we can only move forward by trial and error now.

Like how my decision to go ahead with 8 week immunisations put kai in hospital. Or by not insisting on two weekly glycine bloods we had a six week hole where we were filling Kai’s body with meds willy nilly, instead of with data and intention.

I don’t feel worthy, or capable or enough to be the expert that Kai needs, and I’m scared to be the expert, and angry that our metabolic guy has been found lacking (not his fault) in what we hoped him to be.

Don’t get me wrong, I will and have stepped up to focus on Kai and NKH. To read the studies and fight the kajillion consultants for what I think is necessary, but I’m overwhelmed with the enormity of what lies ahead, and I worry that I’m not enough. It’s like needing a chainsaw to chop down an ginormous tree and all you have is a butter knife. That’s how I feel about me being the expert, and I feel like even my best causes mistakes that cost Kai dearly.

To put it clearly, here is the massive tree: my baby suffers daily. He is not a normal, happy go lucky three month old. He has seizures that cause screaming fits and comas. We pump him full of meds that are painful for his little belly. He has a tube down his nose for feeding, and a needle permanently in his leg for 24/7 meds.

Even small developmental things: he doesn’t smile socially, or babble. His gaze is odd, he can’t hold his head up, he doesn’t reach for things. Right now he doesn’t even have a coordinated suck like he used to. And sure, with time, physio and help he may gain some of those skills but regardless, my baby has a rare metabolic disorder and suffers every day. My babies disorder is terminal.

Putting all the raw emotion out there plain: I feel too small, and too powerless to be his expert. I’m scared my best may not be enough for him, and I worry that he will suffer when my best is all he has. I worry I’m a butter knife when I need to be a chainsaw.

I know I’m not alone in this. There is a wonderful community of NKH parents on facebook. Some of them are crazy, and some have courage I can’t even understand. But a lot of them are smart and amazing and they’ve been where I am. I know I’m not alone in this (I found my tribe) but that doesn’t mean I don’t feel all the things I do.

So. We went and saw our metabolic Consultant and I was disappointed. He is not the hero or expert I hoped he’d be. Turns out I have to the chainsaw hero, and I find myself lacking.

Edit: Sometimes when I think on this, I remember something that another NKH Mum said. I’m grateful Kai came to us, for a while there was a lot of ‘why us?’ but now I think of all the people I know, and how they live their lives and the choices they have made and sure, some of them I think could do what we do, they could live the special needs life and do their best by a special needs baby. But for a large chunk of people in my past, I think thank fuck Kai came to us and not them. Thank fuck.

He pooped!  it’s ridiculous how happy I am about that, even if Kai seems nonplussed about it.

Kai’s getting more awake with every day. He was a bit less dopey today, his eyes a little less heavily lidded. It’s so nice to see him have proper awake/asleep times. Nice to see him wriggle a bit more. He’s not yet moving his legs like he used to, but his arms can now put his fists within reaching distance of his mouth, much to his delight.

A few days ago it was clear my milk supply wasn’t able to keep up with him. We’ve used up the frozen stash, so I’m power pumping, and when I’ve pumped enough he has it, and when I haven’t we do half breast milk, half formula. However with this change (and him waking up a bit) has meant he’s not pooped. He hasn’t pooped in three days. He doesn’t seem uncomfortable, but my little guy used to be a champion pooper, pooping every 3-4 hours or so. Three days is definitely unusual for him. (Also, the pre-mum me would have been so unimpressed about sharing her hypothetical baby’s pooping habits. The post-mum me is obsessed, and sharing poop habits seems the least worrying of things to share).

Anyway, the hospice has a therapist here who does baby massage. She filled up a cushion with warm water, and we lay Kai out on it. He went to sleep instantly and had a lovely half hour baby massage. It was pretty lush, actually. He was so so relaxed. I was too, but I think it’s because there was some lovely calm music playing. I almost went to sleep sitting up next to him. I’m glad hospice has all these things available for us. Even if it doesn’t help the pooping, it was such a nice thing to have do. 

We possibly were a bit ambitious, but as Mikaere was more awake we took the opportunity to take him swimming. It’s something I’ve wanted to do since I was pregnant with him, and I wasn’t sure with all his hospital visits and illnesses whether we’d ever get the chance.

They have a pretty nice pool here at the hospice, where the air is as warm as the pool so it’s not too chilly when you get out. The nurses are keen to go the extra mile for us so we can swim, and there’s music and all sorts of floaty things. There was a small matter of his two ivs and his NG, but we put a bung on the end of the NG, and dressings over the midline and the subcut, wrapping the drivers up in a towel on the side and off we went.

We did our best to keep the midline out of the water, floating his arm on a floaty thing, but oh. Oh my days. It’s been a while, but I was so so happy. He seemed to enjoy it too, kicking his legs a little. He was wide awake the whole time, and cried his little kitten cries when we got out.

Oh my little guy, there is something huge about doing the things with you we thought we’d never be able to. Our dreams for you have gotten much smaller, but I’ll take the tiny wins. Swimming, I’m so so glad you were able to experience that. So so glad!

Also, our guy has crazy chipmunk cheeks right now. He’s put on a lot of weight and as he wasn’t moving so much before (he had two states: seizing or sedated) it’s all gone straight to his cheeks. The difference from before we went into hospital and now is crazy. I won’t post a picture, but he has the most beautiful thigh rolls I’ve ever seen!

 Pre three month curse cheeks

Yesterday’s cheeks

Kai woke up a bit today. Opened his eyes (!!!!!!!!), and looked around. His gaze wasn’t exactly a normal baby gaze, but I’ll take it. He opened his eyes!!

To be fair, he looked like a baby on drugs. Super dopey, eyes heavily lidded. Which he is, really. He’s on so many drugs so that he woke up today is really quite impressive. He’s also been babbling. Not a lot, not a stream of baby noises. Just the occasional sound but oh, hearing his voice!  I’ve missed hearing it. I cried when I first heard his little mewl.

There’s also been lots less seizures. We’ve been practicing baby massage and we discovered that with a bit of foot/head massage we could calm him and head of he seizure. So now he’s getting an awful lot of baby massage.

He’s also got a silly amount of wind, which we struggle with. He’s not really burping. Or pooping. I’m suddenly very very involved with the minute details of my babies digestive system. I’ve never been interested in his farts till now.

He’s a very different baby than he was a few weeks ago, before we went in. I can already see the beginnings of what the seizures and NKH has done to my little guy. It’s bittersweet, I’m heartbroken to see the deterioration but so so so pleased he woke. I’m hoping that as time passes he’ll improve and we can go back to being a bit more like we were. Fingers crossed!

PS You guys!! Thank you thank you thank you for the donations. They’re still rolling in and we’re so so so amazed at how much we’ve raised!! Also, for everyone that has shared our story or the just giving page, thank you. It means everything to us x

The hospice has shown no end of kindness to us. They’re constantly offering cups of tea, and feeding us copious amounts of food. Anything we’ve asked for they’ve tried to cater to, they’re so kind. They even offer us things we wouldn’t have thought of.

Today we took plaster casts of Kai’s hands and feet. It was an odd experience made darker when I asked if they do this for all the kids (only the terminally ill ones, was the straight forward response). Still, I enjoyed it.

We made up the mould mixture, and I held Kai’s hand while it was plunged into a ziplock bag with all the stuff. Slowly the purple mixture turned gummy, and solid. Like silicone. After a few minutes I was able to pull our hands out and tada, mould! We also did plaster casts of his hands and feet, and took his little fingerprints with magic ink (the kind that is invisible on skin, then after pressing to paper the print appears).

It was a lovely thing to do, but tinged with a little sadness, knowing that we’re creating remembrance pieces. Honestly, we’re holding onto every moment right now. 

Eeg results came back. It looks like Kai is having seizures all the time, even when it’s not obvious he’s having them. Oh my days. Devastated.

The nurses and doctors talk to me about meds. Going up on the phenobarb and down on the  midazolam  but only the continuous iv, the bolus will stay the same.  Perhaps we’ll try the buccal, even though he didn’t respond last time…. they come and make these decisions and they’re very good about making me feel involved.

Except that I move between stepping forward and stepping back. Before we went into hospital I knew the ins and outs of all the meds he was on. Now?

Now I nod. And hope. I don’t understand the significance of what’s being changed, I know the overall goals (wean him from midazolam/B6/prednisolone so he’s only on the phenobarb and zonisomide) and why (because we’re not sure his seizures are B6 related, because the prednisolone is only appropriate at a high dose for a short time and causes high blood pressure etc etc)

But he’s just on so many anti-epileptics. So many. I don’t know how they work, I don’t understand the interactions, I don’t know them. We change the levels so much, there are so many moving parts and looking at him, I have no idea what’s working and what’s not. Add in that almost all of them have a loading time before they work… I’m overwhelmed.

I consider myself a smart person. I know to research the shit out of whatever it is that I want. But this? There are so many meds, so many different moving parts and I’m exhausted. How do people do this? I’m torn between wanting to be up to speed on what medically is happening with my son, and dropping that ball from the pile I’m juggling and trusting the medical professionals.

It’s just… the medical professionals don’t always know what’s best. They don’t know NKH, they don’t know Kai. Two weeks ago a neurologist pretty much said that there was nothing more they could do with the seizures, that they’d already tried the ‘big guns’ and Kai wasn’t responding. Dick head. That makes me angry, because I know we haven’t tried a ketogenic diet no CBD oil. What else is out there that I don’t know about that we could try?

But then I doubt myself – is there a reason that he didn’t mention the diet or the CBD oil? Is there a reason they will not work, is that why he didn’t mention them? Did he not know about them? Or does he think that Kai is so far gone it’s not worth trying?

Struggle city. I swing through phases of wanting to trust the medical consultants and 100% not trusting them at all.

There are a few I listen to. The consultants at hospice are amazing, I feel like they’re on Team Kai where Kai is the focus. Where they can see him, and understand that NKH has an effect on how he processes. They suggest things, and are proactive.

Some others, like the neurologist who told me there nothing left, he’s NOT on Team Kai. He’s looking at symptoms and hospital protocol. Or the metabolic guy, now that he’s done the diagnosis he’s reactive, not proactive. I feel like he knows that NKH has no cure, and dabbles in Kai’s care rather than taking the lead.

I think that’s why trusting the medical staff is difficult.

Sigh.

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PS: Quick insight into Kai’s Medication Regime, he’s on a continuous feed of midazolam (into a midline in his arm) and phenobarbital (into a subcut in his leg). He has an NG tube, and orally takes nine different medications every day. Every four – six hours he has at least two of them. At 6pm he has 7 of them. It’s very very complex. I can’t believe my little baby has so many drugs in his system.

PPS: Still so grateful for the donations. Over £7k now! You guys are amazing x