#teammikaere

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 

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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.

We were doing so good with the seizures. I’m sick. Kai is sick. My mum who is visiting is sick. With one of those head colds, where it sits in your sinuses and makes you feel horrid. Sam picked it up while we were out at the hospital last week and now we all have it.

This wouldn’t be such a bad thing if Kai processed illness like any other baby, with misery and building an immune system. Instead he has seizures. Considering he’s on so many anti epileptics this is crazy. They’re also presenting in a new way, with much more screaming and back arching.

Oh, baby. I’m gutted. He was doing so well, and we were looking at weaning some of the medications. Now he’s got a fever in the 39+ range (usual baby high is 37.1) and not holding down his feeds (extreme reflux) and he’s so so so uncomfortable. And the seizures. Oh my days the seizures. It’s heartbreaking, I hate it. I wonder if I could have avoided it with more hand washing or less cuddles or whatever. I think I’ll forever be second guessing myself.

Now I understand why people always say ‘don’t come into work if you’re sick.’ If someone at Sam’s work goes in sick rather than taking sick leave, and Kai gets it from Sam? Worst case scenario – we’re back in intensive care worrying if Kai will survive. That’s a likely scenario too, by the way. There’s a boy here on end of life care because of a chest infection that any healthy adult could shake off.

But Kai isn’t a healthy child, he’s vulnerable to all the bugs and colds, like all the special needs kids here. As an example, we got his eight week immunisations, and then the next day we were in hospital. Immunisations aren’t meant to hospitalise babies. Colds aren’t meant to devastate them.

It’s heartbreaking to watch, and I feel all the things. We were doing so well little guy! Please get better soon.

Oh my DAYS! So many good things are going on at the moment. The first, and the most exciting, is that every now and then Kai smiles.

It’s so heartwarming and I cried the very first time it happened. Usually we get windy grins, but to have a proper social smile. Well… I’m not sure if it’s a social smile. It’s not a reaction to anything we do, but there are just a few moments when Kai seems happy, and smiles like he’s got his own private little joke going on. My baby smiles!!!

There was one study that marked NKH severity on a bunch of developmental milestones, and the first milestone was smiling. And now my guy does it! He smiles!!!!!

The second good thing, just, gosh. We have raised an enormous amount of money for Joseph’s Goal. Almost £8k. (Almost £10k if you count the small fundraiser we ran with Sam’s work). We are overwhelmed and so grateful to everyone who has donated. To everyone who has shared our story. We’ve had friends of friends donate, customers of stores (thanks Tom + the chocolate deli customers!), pay it forward favours (heart to Stephanie Kaloi and her kindness) and just… so many people. So much love.

Emma Kendrick is Joe’s Mum, of Joseph’s Goal. She messaged me the other day to thank me (and really you guys) for all the fundraising, and then to say that they would be donating an extra £40,000 (!!!!!) to Dr Nick Greene’s research. That blows my mind. That’s not quite six months worth of research (with one post grad, on one hypothesis). Very very pleased. Nick even said that being able to plan ahead with the work is already allowing them move faster and start work that would have needed new grant applications. I think there is more research going on towards potential therapies than ever before. Woohoo!

The third amazing thing is that friends of ours play with the Fulham Brass Band. They’re doing a charity concert in May for Joseph’s Goal (expect to hear more later in the year, hopefully all of you London folk will come along with us?) AND (I love that there is an and) the lead Brass Band man is the editor for Drug Discovery Today (!) and has invited a few researchers to write a piece up for NKH! Charity work *and* amazing awareness. Ahhh!

I am blown away by all the good things. Love it. So happy!!! I feel like we have more downs than ups, but on days like this one, the ups are pretty up.

Okay. I’m a proud person. A fiercely independent proud person, who hates asking for help and doesn’t often accept help unless I’m drowning. However, almost every message or visit comes with a phrase ‘let us know what we can do to help’ or ‘do you need anything?’ or ‘what can we do?’  I’m never sure how to answer that, because answering became another thing to think about. Knowing what we need requires thought and some time to figure it out, my brain and heart are so full dealing with a kajillion other things that delegating just seems more difficult than it should. Add that to my pride and all those offers of help fall to the wayside.

Most my friends know this about me, so our closest people just show up. They make it easy, so we don’t have to ask. That worked really well when it was a visit at the hospital, taking us to dinner, visiting on Christmas Day with Christmas food, bringing clean clothes or collecting the mail. But as time has gone on and more people heard about our story, and the outpouring of love has been immense… and more people started just sending stuff. 

We have so much food. We’re drowning in it. I should mention the hospice feeds us three meals plus cake and snacks every day – so for people to send us more food on top of that is crazy. One of Sam’s friends was so so generous and sent us boxes and boxes of wet wipes and muslins and bibs (thanks John!).

That’s more stuff than we could ever use, so we’ve donated the lions share to the hospice. It was then that we realised that people were going to send things whether we asked or not, and it might be better to direct the goodwill into helpful directions.

So, we set up a page: How to Help.

I feel weird asking for help, or even having a page with things on it. However, there are a few things that would be more helpful than the copious amounts of food we keep receiving, so. We have page, with help requests. 

So much love to everyone for the offers of help. If you decide that you do want to help, please don’t send food. Pick something small off the list if you feel moved to. We appreciate every small action, and offer of help, so thank you. Thank you to everyone who has sent a message of love, who has sent food, and who has been there.  We love you guys. We can’t imagine getting through something like this without you guys.

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