#teammikaere

One of the things that was most heartbreaking was when Kai woke up, after essentially a month in a continuous seizure coma, was that he’d lost a lot of his skills.

He can’t hold his head up anymore, he wasn’t holding on to us when we held him, he stopped tracking with his eyes and he’d lost his suck. That last one was particularly heartbreaking for me, because it meant we were no longer breastfeeding. Kai’s swallow is good, but his suck is uncoordinated at best. It’s hard, because we used to use the dummy as a soothing tool, it worked like a magic trick! Now he pushes out the dummy as if it’s the most distasteful thing ever. 

I am pumping, so he’s  getting some breastmilk, but because I’ve been so stressed and we’ve been in so many different wards and had no routine my supply dropped. Dramatically dropped. I manage maybe half of his feeds now.

I’m so torn up about so many things – with no suck, he’s unlikely to be able to eat, and we take such pleasure from food. From eating. A lot of our social life revolves around it. Thinking of quality of life, how will Kai manage? Will his life be less because he may not be able to eat? That all his feeds will be put down a tube?

And then there’s the abrupt end to our breastfeeding time. Nursing was such a joy for me, they were the easy feeds of the day – no meds, just Kai and me.  I was able to soothe him with the boob, and I’d relax into it. I loved breastfeeding him. Highlights of my day, a little segment of normal.

Now we don’t even bottle feed. We try sometimes, but mostly he toys with the teat. We aspirate what we can from his stomach contents to check the tube is still in his belly, and then we hook him up to the feeding pump, hit a few buttons and away it whirls, beeping when finished. It couldn’t be a more different feeding scenario.

Even worse is the (completely unnecessary, totally irrational) guilt about having to supplement with formula. I know it’s best for him, that I’d never deprive my baby of food if he was hungry, but for me, I have a baby with a rubbish immune system. We know this. We know a cold can put him in intensive care. Feeding him breastmilk means he’s getting in part, whatever small immunity I’ve picked up.

This is a tiny tiny edge, but I’m clinging to it as long as possible, and the guilt that I’m depriving my baby of a possible advantage because my body isn’t cooperating is overwhelming.

I get on with it, because we’re in hospice and we need to. But that doesn’t mean I’m not sad, and guilty and trying desperately to increase my supply.

Instead, I’m doing my very best to appreciate what Kai can do. That he refound his voice, and has a very loud strong cry (healthy baby cry, sick babies are silent). That he so clearly enjoys physio and baby massage. That he found his smile for the first time (break my heart into a million pieces, I’m so happy he smiles), and he still responds to my terrible singing.

Small things get us through. Swings and roundabouts. Faith and fortitude.

Kai’s subcut site got infected. It’s a thing that can happen when you have a needle under your skin for a week, and now it’s all purple and inflammed and sore to the touch.

We’ve tried two different antibiotics (adding a few more meds to the kajillion he’s already on, even worse: these ones always cause him to vomit), and we’ve waited. I took photos every few days and we drew black lines around it in biro to see if it was growing or not.

When it was clear it wasn’t getting any better and was proper bulging into not quite an abscess but maybe an abscess, we went down the road from our hospice and visited our fifth hospital.

We packed loads, fully anticipating a long stay, so imagine our surprise when we were in and out in a few hours. Lucky for us.

We’re trying a new antibiotic and a salt dressing, and we wait another few days. If it doesn’t look like it’s healing, we’ll be sent back to our London hospital to have it cut open and removed. We’d like to avoid the knife if we can.

Fingers crossed.

The hospice is a few minutes walk from a canal, and one afternoon, between feeds and meds, when the weather was mild and the sun out, we took Kai for his first walk in a month. Leaving the hospice together, just us, was heavenly, and I felt (just for a moment) a bit normal.

It was lovely. There were people walking their dogs and children in gummies and just, the perfect way to spend an afternoon. We weren’t on our way to a hospital, or a medical appointment. It was just us three. Bliss. 

We don’t often get the chance to do things like this, so I appreciate it so so much when we do.

So. Going home has tentatively been raised. At first I wasn’t sure, he’s on four hourly feeds (that require two hourly attentions, for turning the pump on and off, setting up and cleaning down), meds ten times a day (more if we stagger them so can tolerate them a bit better), and I’m meant to be pumping every three hours.

I’m not sure how it’s going to work at home without the army of help we have here. Add in all the medical appointments and hooha and how am I going to fit time in for life? For showers, and grocery shopping and eating? With the two hourly feeding pump drama, how is sleeping going to work? How do I do the stuff that helps? Physio and tummy time and research?

The enormity of what life looks like outside the hospice freaks me out. But then, I think I’m getting a little too comfortable at hospice. A little too reliant on the nurses (they’re all enablers for three am sleep for me when it means baby cuddles for them). 

Even logistics aside, we’re in a nice cosy bubble. Here, an NG tube is the least out of place thing – all non verbal, non ambulatory kids are welcome here, and loved and are the norm. What happens outside the hospice, outside the nice safe bubble, where my kid goes from being the norm, to not?

I think now is the time we knuckle down. Now, in the tricky time when we have to reestablish a crazy routine is when we figure out what kind of parents we are.

Just, fuck. I am so ready to be home, but I literally can’t see how it’s going to work and I worry about all the unknowns. I guess we plow on ahead and do our best?

I want to face it in a determined ‘I totally am the best at life and will win at insane routines’ kind of way.

We’re not sure when yet, but now I’m totally ready to take my baby home. Fuck you NKH. I want more time with my baby.

Like I said in the last post, it’s such a comfort knowing there are other NKH families who get it. There must be 40 or so families in the U.K. To put that in perspective, the population of the UK is around 65.3 million (ish). Which means NKH kids are about 0.00006% of the UK population.

Lucky for us, while we’re at hospice, one family was only an hour away. So they came to visit, and we had the pleasure of meeting Alexander.

Oh my days, he’s lovely. He has the most beautiful eyelashes you ever did see, and his parents are a wealth of knowledge. Alex is almost five, and just, the sheer amount of stuff they’ve learnt in that time blows my mind, and makes me feel like such a new NKH parent. It got the point where just small comments were enlightening and I had to apologise while I pulled out my phone to take notes.

It struck me just how far we have to go, and how much further there is to go, if Kai gets that far. Alexanders parents are strong, super super strong. I’m aware that it takes hardship to build strength, but it was also easy to see how much they love their son. And I suspect that for us, that’s where we’ll find our strength too, in our love for Kai.

It’s comforting to know that they’ve been where we are, and they’re managing. They’re doing okay. It’s not easy by any stretch of the word, but that they have such a good handle on things gives us hope.

Every year the University of Notre Dame in Indiana does a two day Rare Disease Conference, and one of the diseases they cover is NKH. Because of a tired Mama daze I missed the research talks (fail!) but I made the family segment which was nice. We video conferenced in from hospice and short spiel about Kai, and we ‘met’ a few other NKH families. Some we knew already, from the facebook group, but it was nice to see them, to hear them, to listen to their stories.

There’s something comforting about being in the company of others with the same rare genetic disorder. Most people won’t get it, they can’t really. NKH is such a beast.

When you’ve watched your child seize every 15 minutes, when you’re fighting doctors over treatment and being told ‘there’s no point because we can’t treat the underlying disorder’ (I might be a bit bitter at that neurologist. I’d like to go back and punch him in the face. In the nicest possible way, of course). When you’re watching your kid in intensive care in essentially a seizure coma… twice… when you’ve done all those things it changes you. It was nice to know there were people who got it. I’d never wish this story on anyone, but I’m very glad we’re not alone on this walk.

There is another NKH conference in the U.K. later this year. I’m so excited to go (assuming, touch wood, the stars all align, and we’re in a position to be able to go). I feel like this is our tribe, they get it. Their babies have been along the same path as ours. It’s just such a comfort to know others truly understand.

In saying that, it’s double edged sword, because when I hear about an NKH kid seizing and in hospital, I really struggle. Especially if it’s one of the kids that are Kai’s age. Because I know. We’ve been there.

Like I said, I’d never wish this on anyone, but selfishly I’m grateful we’re not alone in this.

We’ve been in hospice for four weeks now (that’s longer than we’ve ever been at home) and we’re trying to take more on. Usually we would wheel him out to the nurses station every night, so they can watch him for seizures, but as he is seizing less (touch wood) he’s been sleeping in our room with us.

It’s a bit weird. The nurses creep in and out every few hours for his meds, and to check the syringe driver that pumps his meds in. But otherwise it’s on us.

I like that we’re slowly taking back our babies care. It irks no end that I’m sharing him with (very very lovely, very competent, well trained nurse) strangers. Here’s what I want: to be home with my baby, settled in on the couch in my pjs like every other Mum with a new baby. I want time with my baby. Out of hospital, out of hospice.

So, we’re doing the feeds and the crazy crazy med routine, and owning the aspirates. The only thing I’m not doing is putting the NG down his nose, because every time that has to happen I want to cry when he does.

We’re not in a routine yet, but we’re getting there. We’re totally getting there.

That’s you, btw.

I feel like a broken record, but we are genuinely so grateful for the love, the messages, the help and support.

We keep getting messages from all over, from friends of friends, from prayer circles in other continents, from the folk in Toddington/Beds. There has been so much love, and we’re so grateful.

Thank you everyone for the gifts – we have been inundated with beautiful onesies (our guy is so well dressed now! With clothes that fit, so thanks for that) and the amazon gift cards have been very very helpful (example: we just got an electric steriliser. After months of sterilising with boiling water this method seems like magic).

There are people I still need to get back to in terms of research (sorry for the delay, our day to day got a bit hairy as Kai went through a grizzly patch) but I promise I’ll get back to you. There is much I don’t yet understand that I’d like to.

The biggest thank you has to be to people who are fundraising in Kai’s name. Honestly, we know there are a few events in the works, BBQ’s with raffles, a quiz night, a brass band concert, maybe even a bake sale in a consulate and a teddy calendar!

So, the Christmas campaign will end tomorrow, and we’ve set up a new just giving page for Team Mikaere: justgiving.com/team-mikaere.

If you are running an event, or if you’d just like to donate, it’s available. We support Joseph’s Goal, because one, Joe (another boy with NKH) is awesome, but also because they are UK’s only NKH charity, and because they support the research being done by Dr Nick Greene.

I can’t even begin to talk about his research. It brings me such hope to think that treatment is being investigated. That there are models to test with, that hypotheses are being knocked down or validated. Hope is on the horizon, you guys.

With every pound we raise we bring the research a bit closer. Literally. Not even kidding, because if we can raise funds for research, the researchers aren’t wasting time applying for grants, they can just get started. I know, because Dr Nick Greene emailed me to tell me so.

So, we have a goal. £100k. (Did anyone else gasp a little on the inside??). We’ve chosen that amount intentionally – that’s how much it costs for one year, for one postgrad researcher, with labs and resources to test something out.

So far we have almost raised £10k, between our two campaigns so far. 10% (I’m still blown away you guys!!).

So, to Team Mikaere – thank you for being on our team. Thank you for fundraising. Thank you for following our story and sending all the love. Thank you for being in on this with us. 100k. Totally doable, right?

PS – we’re going to get Team Mikaere shirts made, all profits to research of course. The kicker? Mikaere will get one that just says ‘Mikaere’ – Sam laughed so hard when he thought of it x

Well poop. I didn’t even think about this. Of course there is glycine in food. Glycine is an enzyme that makes up protein, so of course it is in food. And then if I eat it it’s in my breast milk, which Kai eats. Which then effects his glycine levels. Which he can’t process because he has non ketotic hyperglycinemia.

Now I’m trying to figure out whether to avoid all the things, and how that will effect his levels at all. I’m frustrated that I found out about it from the FB group rather than our metabolic consultant (although: looking back on my notes, he did mention a ‘glycine diet’… no explanation on what it was or its significance or if we should be doing it, and if so the best way to implement it… gah).

Honestly, I constantly feel like I’m being blindsided by things I feel I should know, especially if they may help Kai. There is not enough time in the world for me to research all the things!

Sad face. It’s so discouraging. I had this idea of what it was like to be a Mum. It didn’t include marathon research sessions at three in the morning, meds a kajillion times a day, all the physio exercises and all the medical appointments and hospital visits and scary mystery future. Honestly, I feel like having a typical baby would be a walk in the park at this point. Hard, but not this hard. 

I guess we plow on ahead. Every day we try accept the hand we’ve been dealt… the best we can do will have to be enough. Even if it means I’m learning things from facebook groups (honestly, thank goodness for the other NKH parents, I don’t know what I’d do without them, even if they are strangers on the internet).

… 

Edit… 

Knowing that I’m being Miss Negative Nancy and it takes at least five positives to strike off a negative, here is a positive thing. I’ve successfully mastered the giving set, aspiration and feeding through a tube. It’s a small thing, but at four am when you’re determined not to go out to the nurses station and on the sixth time you finally get an aspirate that’s not super black? Dance parties and high fives all round!

PS – if anyone wants to help me research some stuff, I’d be super grateful. You don’t need a medical degree or anything (I don’t have one). If you have fifeteen minutes and an internet connection, I have a list of questions that I could use some help with. Starting with glycine in food, and whether a low glycine diet would help Non Ketotic Hyperglycinemia kids, and what foods are high in glycine, and what foods to avoid. Email me – elly[dot]gedye[at]gmail.com 

x

As we’re getting over our colds, we went back into London to see our neurologist. Which is to say, for the first time since he’s been seizing we finally have a neurologist instead of consultants passing the buck (so frustrating).

Here’s the thing: the neurologist didn’t tell us anything new about Kai’s seizing plan. The plan is still the same. What we did learn are new snippets that other consultants didn’t tell us.

Like that our metabolic consultant told our neurologist consultant that Kai is only expected to live five years or less, but he never told us that.

We also found out that his developmental level is at that of a four week baby, rather than the fourteen weeks he is. No one told us that either. We heard it as aside when symptom management nurse was filling in our neuro guy.

We also found out from the neuro consult that babies aren’t likely to survive with burst suppression eegs – shown on Kai’s very first eeg way back in NICU. No one told us that that was what burst suppression signified. We knew it was bad, and we knew that Kai’s condition meant a rubbish survival outlook, but we didn’t know that it was because of what was happening in his brain right that moment.

I’m so frustrated that this is how we find out information about Kai – through eavesdropping. All doctors treat us like this – they talk amongst themselves and attempt to ‘protect’ us I think. Fuck that. I don’t want protection. I want it straight and I want all of the information. There is no one more involved in his care. I am the expert. I am the one that knows his meds. I am the one that decides what he does and what battles we fight and what care he gets.

So if I am the one who does all the things, and decides all the stuff, why am I not being told all the things? Why are others deciding what to tell me and what to not? How am I meant to make any informed decision when we don’t know everything, or even the significance of everything?

On the other hand, I half think that our medical team can be hit or miss. I’m not into our metabolic consultant telling me that there are no clinical trials when I ask about NKH management (it wrankles when I’m facebook friends with the very clever researcher leading the way with NKH treatment). I’m not into that neuro consultant that told me there was no point to treating Kai’s seizures because we couldn’t manage the underlying cause. I’m over doctors telling me about the doom and gloom,  but that doesn’t meant I don’t want to hear every single scrap of information they have about my son.

It bothers me that we’re not included. There is a whole system that they’re on that we’re not included in. It’s as if the entire Medical Team Mikaere was on facebook except me and Sam.

Frustrated.

Anyway, the plan is the same. Wean the phenobarbital, switch from the iv in his leg to an oral version, and then we wean. C’mon little baby, we love you so much. I can’t help but feel the less meds you’re on the better.