On visiting our fifth hospital in four months (and the infected subcut)

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.

 

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On doing the normal things, even at hospice

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.

 

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On tentative going home whispers

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.

 

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On meeting Alexander

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.

 

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On the Rare Disease Conference

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.

 

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On not yet having a routine

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.

 

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Dear Team Mikaere

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?

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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

On glycine in food?!

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).

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... 

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!

 

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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

Dear Kai - Month 4

Dear Kai,

Four months! This past month feels like another saga, so much that is frankly unbelievable has happened. The biggest is that this month we lived in hospice. In *hospice*. 

In the beginning the phrase 'end of life care' rang out like an infected wound. We were in hospice... you hadn't opened your eyes since before Christmas, you were still seizing like no bodies business and the 'terminal' part of your little glycine problem felt much closer than I would have liked.

But it turns out hospice isn’t like you expect. It’s nicer. Nicer than hospital. It’s a bit more comfortable, a bit more relaxed, a bit more homey. We fell into a kind of routine. Every morning I'd collect you from the nurses, asking about your night and the number of seizures. Every day I'd sit with you, love on you a little, willing you to wake up. Daddy would be working upstairs, but would come down for frequent cuddles. We'd eat with everyone else, watch a movie with you in my arms after dinner and later, wheel you out to the nurses station before bed. Your seizures were ridiculous, sometimes you were having 20+ a day that required breakthrough meds. We'd wait the required 5 minutes before poking our heads round to the nurses, a bolus of midazolam would sedate you back to your seizure free coma. We had doctors wander in and out, and as the weeks went on, we got to know a lot of the residents here.

Still, like our little solider, you pretty much ignored the 'terminal' part. One day your midazolam syringe driver stopped working. Midazolam is horrid, and thick and syrupy, and for the whole day while the nurses fussed about pumps and drivers, you tolerated the extreme wean. Even better, when you didn't have any more seizures than usual, we tentatively decided NOT to put you back on Midazolam.

Shortly after that, you began to open your eyes again. Your seizures dropped off, and oh my baby, you seemed wonderfully aware and so very WITH US. Sure, you'd lost a few skills. Your ability to suck disappeared, and any kind of head holding up business, but you were awake and not seizing quite so much and your cry came back. Loud, beautifully loud. 

Now that you were on the NG and not refusing the bottle, your cheeks grew in, and you started collecting all that weight you were missing. You put on weight like a proper bruiser, and everyone, literally, would coo over your beautiful thigh rolls, or your delightful chipmunk cheeks. As your cheeks expanded, hilariously you started losing your hair. You had beautifully dark wispy locks, but now we think you’re going blonde! 

Life at hospice got much better once you woke up. We took you swimming, which you loved. Mostly. I definitely loved it. You chilled out in the pool, often going to sleep if we kept you in too long. You'd scream getting out, too. And if you lost a few skills, you gained others. You started smiling! Little fleeting grins. Not at us per say, but just quietly to yourself. You started holding on when we pick you up too, curling your fingers into our shirts, clinging with your arm around ours as if we'd ever dare drop you. Sometimes you’d coo at us which was such a delight. You also got better at focusing, and you’d look AT us, rather than through us. Oh my days, you can’t imagine how my Mama heart swelled when you were finally looking at us, making proper eye contact, and even turning your head to see us if you heard us speak. 

As you learnt new skills, so did we. We learnt all about feeding pumps and giving sets, running the feed through and cleaning out it became part of our everyday rigmarole. We’d fight you and those little fingers that ever seemed to be finding your NG tube, often yanking it out. We became competent at getting aspirates before every feed and med round. Rolling you onto your side, doing mouth care. I preferred to drip milk into your mouth, knowing that if the aspirate came back white it was still in your belly instead of your lungs. 

Eventually, hospice became our new normal. It was more relaxed than hospital, but still not home. Our family slotted in to hospice life. The nurses would jostle for cuddles. One nurse was even cheeky enough to steal a cuddle right out of Daddy’s arms! Every day there would be something. You especially enjoyed physio with Pam, often smiling for her, chilling out as she gently stretched your little legs. Or baby massage with Angela.  There was a time when you were so constipated from all the meds we were giving you, and magic Angela - she always managed to make you poop. 

We learnt an awful lot of songs while we were at hospice (which I think delighted you). Were we could we took part in Music Therapy (you seem partial to the flute) and Art Therapy… well, as much as you could do. We made you a sensory book for your feet. We even managed a walk here and there outside in the cold. We watched your first snow from hospice (and your second. And third). We stole as many moments as we could. You were particularly fond of Daddy cuddles in the early morning, as I'd wheel you back in from the nurses station, you'd grizzle until I put you into the big person bed, into your Daddy's arms for a quick cuddle before he had  to get up for work.

We had an outpouring of love from Team Mikaere. So many people donated to your justgiving campaign. So many people sent messages of love and hope and gifts (so many gifts!), so many people offered to help research (thank you!) and just, so many people helped.

We also had a few opportunities to participate in the wider NKH community, with a rare disease conference and meeting another NKH kid. Eye openers, both experiences, but more in the 'we totally got this' kind of way. 

In saying that, your schedule is insane. Your feeds are four hourly, but as you’re on a pump, it ends up being two hours on, two hours off. We start the pump. wait two hours for the pump to finish, clean it all out and then wait another two hours for the next feed. Your med schedule is ridiculous. Easily ten times a day with different meds. Some need to be on a full stomach, some half an hour before anything else, ideally with an empty belly. Some need to be given slowly (slower than a 1ml a minute) and some are goopey and some smell horrid. And if it wasn’t one thing it was another. Projectile vomiting with the meds. Constipation. Diarrhoea. Nappy rash. An infected subcut site. A face of baby acne from the steroids. Cradle cap. Every few days a new something would hit us, and you’d just continue forward. Onwards like it was no big deal, and I’m so glad you did. Weaning this, and talking to that doctor, and organising road trips to one hospital after another.

Every day with you is precious. Every day I’m grateful you’re still with us. I know that you don’t believe in your terminal disorder, that you, you wonderful little fighter you, just keeps going like life is meant to be this difficult. 

Here’s to another month down, you cheeky little monkey.

Love you more than you know,

Mama x

On seeing the neurologist and learning by eavesdropping

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.

 

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On five seizure free days

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.

 

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On all the good things

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.

 

 

On accepting help

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.

x

On accepting the special needs/disability badge

The last three months have been tricky... all of the baby/medical stuff of the day to day aside, this whole time I feel like I'm still coming to terms with the idea that I'm raising a special needs child. A disabled child. It's a concept/reality I've been mulling over while my everyday unfolds around me.

For a long time I ignored the future possibilities, my baby was beautiful and you'd never know he had a horrid metabolic issue. He was just a little baby boy and if no one mentioned anything about disabled futures I could live in the happy denial bubble indefinitely, and I did. Right up until we were back in hospital. Then we switched between no future with horrid end of life directives and whatever future would be allowed to us once the seizures were done messing up Kai's brain. It was made clear that damage was being done with all his seizures.

When we came to hospice we were shown what that second future might look like. There are some severely disabled children here. No one asks or tells about diagnosis's. There are enough children here in wheelchairs, who are nonverbal and different. Disabled. Children with special needs. And it's difficult, facing them at every meal, in the corridors, with the different noises and teeth grinding and the drool. The odd movements, the vacant stares, the different facial expressions. Almost all the nonverbal kids are either eating paste or fed through pumps. They're talked over the top of, or when they're talked to, it's in a funny sing song rhetorical way. And every time I see them, I think this is our future.

It took a three days or so at hospice before I stopped looking away like I would have in the before. Before, while I was out and about, if there was a disabled child in a wheelchair I would have said it was rude to stare and averted my gaze. Now I know it wasn't that it was rude at all, but that I was uncomfortable. I was uncomfortable with those less able than myself. Shame on me.

Now I greet these kids by name, and I talk to them. I ask about their days and I study them, while I wait for a response. Sometimes I get one, a noise, a movement. Sometimes I don't.

I watch their parents, who laugh and mop up the drool and talk and sing and love on their kids so hard. The parents here are joyous, like they've taken it all in their stride and are doing the best they can by their kids. Don't get me wrong, I'm sure there is a difficult side, but here at hospice when there is so much help and support respite parents are joyous with the time and help and the break.

I'm still coming to terms a very very different world than what I imagined. Before Kai was born I'd planned to go back to work. Letting go of that plan, the one where Kai is healthy and I can go back to work was difficult. But this week I emailed my accountant to begin the process of closing down my company. I stopped drawing a salary. I closed off all the services I won't be using. And I hate this, I hate that I'm not earning, that I'm not supporting myself independently or contributing financially and having to rely on Sam for money. I've had to swallow my pride and recognise that this is what's best for our family, that Kai's needs go over and above a typical 3 month old, and he will forever need help. That this phase of intense caring is not temporary, he will not grow and become more independent. He will forever need me to be his carer.

And so I filled out the 30 page form for the disability allowance. The first question took me a good day or two to answer. It asks if we're applying under special conditions. If, according to his doctors, whether his disorder is terminal and is not expected to survive the next six months.

I ticked the yes box, at the urging of our nurses. I felt like I was betraying Kai and my hope for him. The next 29 pages were equally brutal. Each question asked for extreme detail as to why my son was disabled. And with every question I answered the denial bubble took a beating. By the end of the form it had popped, and I was exhausted, and sad. So so so sad.

Accepting our reality and our future, grieving our dreams for our son, and living each day as it comes and no more has been horrid. I'm still wrangling with it, to be fair.

Pragmatically I know that I need to get through this dark patch to the other side, where I can move forward and do all the things I can for Kai, to improve his quality of life.

Emotionally I'm not there yet.

 

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On fighting NG tubes

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! 

PS - those cheeks without the tube though! 

On experts and metabolic consultants

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.

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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.

On poop updates

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

 

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On baby massage and poop habits

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. 

 

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On doing the unexpected

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!

 

 

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On the chubbiest of all cheeks

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

 Pre three month curse cheeks

Yesterday's cheeks

Yesterday's cheeks