Ellly

I talk a lot about grief, and grieving. I often feel like I’m being blindsided by something else I need to let go of.

Today I realised that as long as Kai is with us, it’s unlikely I’ll ever work again. Intellectually, I made the decision to close my company months ago. Today the emotion caught up and I struggle.

I was proud of what I did. I worked on apps that millions used. I worked behind the scenes on some big brands, making parts of the digital world better. Some of the work I did was average, I’ll admit. Timelines and limited resources and developer constraints often get in the way of excellence and I did the best I could when faced with those challenges. But some of the work I did was fucking brilliant.

It occurs to me that my time at being fucking brilliant in my career is now over. I won’t ever be able to demand the pretty penny I once did. I won’t ever be creating like I was, mingling in the high flyer world of agencies and amazing designers and interesting developers. The digital world moves on so quickly, new trends, new research, new tech and best practices.

Now, when I throw up a simple website or make some pretty cards, that’s it. I feel like I’ve fallen from design grace and have been left behind.

I love Kai, and will forever put his needs before mine, but leaving my job and putting to bed all the work I was doing is a bitter pill to swallow.

Part of it I think is letting go of the glory. Designing for big brands can be impressive. Being ‘just’ a special needs Mum has its own stigma and stereotype, and when I’m stuck in the mundane same same of our days… it’s hard not to feel down about it.

I feel all the feels. I didn’t expect to, but there we go.

Edit: Yes, I can still design things at home in my spare time if I wanted to. I have an option to do that and I’m trying. I’m working with two phenomenal developers but like I said, Kai comes first. Finding time to work in Kai’s day is like trying to capture a sunbeam between your finger tips. No good design work ever happens in distracted 10 minute intervals.

The more I hang out with our NCT kids, the less dagger like their developmental progression feels. It’s hard not to delight in this little group of 6 month olds. They’re all smiles and chubby cheeks and what else can they fit in their mouths?

We went to Battersea the other day, and oh my days, it was nice to be outside. We’d spent the morning at the hospital at some routine appointments, and meeting the researchers and I was desperate for time with non-medical people.

Four of us made it, and one of the other mothers asked if I’d like a cuddle with her wee little one. Oh my days, as much as it’s difficult to see kai not developing typically, this little sweet thing was such a delight! She was so strong, and determined and focused. She was so beautiful, and holding her was such a joy. I felt like I got a small glimpse into what it might have been (though I’m careful not to linger in the world of what ifs too long).

It was just an amazing afternoon. Fab company, the sun was out. Kai had pulled out his ng tube and because no feed was due I left it. Which means for the first time while we were out and about kai had bare cheeks. We walked the 40 minute meander home, and it was beautiful.

It’s amazing how something as small as a sunny day, a park and wonderful company can completely change your day around.

We were lucky enough to meet Emma and Joe (the famous Joe!) from Joseph’s Goal, the charity we support. It was after rushing about meeting Van Hove. They were also in London to meet Van Hove, and listen in on the research update. We went to them, in the lobby of a hotel near Regents Park.

Oh my days, I find such comfort in the company of parents who get it. Who understand, who hold the same hopes and fears and dreams for our kids. Who understand the day to day complications and just how demanding it all is. To spend some time with people who don’t shy away, who aren’t awkward and who just get it.

Joe, oh my days, Joe is amazing. He’s so long and lean, with the bluest eyes you ever did see. Joe is quite disabled, as I think Kai is likely to be. Emma and I were talking about our fears and hopes and one of the things she mentioned was about how different all our NKH kids are, how their mutations express their abilities in different ways, and how difficult it is to imagine that life our for babies. Joe is disabled, but oh my days, he’s beautiful. He’s able to vocalise, and it’s clear when he’s struggling and when he’s enjoying himself. Sitting with his Dad Paul, he was so relaxed, and happy. It was obvious to see.

I don’t shy away from these kids. I think we’re lucky that we spent so much time in hospice and were exposed to see many less able children, from something like autism to children with tracheostomies to children much more disabled than lovely Joe. And I have to say, Joe is eight years old – and just, I’m scared to hope for that long with Kai. We were told weeks, and then months and Kai is still trucking along. Our next milestone is a year. We’re six months in, and a year feels like too short a time. We sit between an awkward place of fear, being told that any ‘acute event’ could rob us of Kai, between trying to make the most of every single moment and trying to live our lives. It’s tense, constantly waiting, feeling like every moment is precious. I’m not really able to settle when I feel like every day we’re lucky to have Kai with us. 

To hope for years…. I’m scared to hope that we’ll get that long with Kai. Could we be that lucky? Eight amazing years, and Joe is still going like a trooper. 

We also talked about all sorts, what its like to run the charity, what the researchers are doing, the upcoming conference in August, how tricky it is to fundraise, how to trust other people with our children.  We also talked about having other children, and the fear of having another NKH child, the split between neuro typical children and our NKH kids, how to manage and teach through the oddities of strangers attitudes towards our kids. 

There’s just so much to navigate with a special needs kid, there’s so much happening with the NKH community and the researchers. 

Honestly, I wish we lived closer to everyone – meeting Emma, Paul and Joe, back when we met Kirsty, Jon and Alex, its so comforting to be in the company of people who get it. We spent a good few hours just chatting to Emma, Paul and Joe, and oh, I wish we could have spent longer. 

Hopefully we’ll see them again later in the year. It makes such a difference you know, to find your tribe. Such a good day.

I don’t know where to start. Last week I got wind that Dr Van Hove – an NKH researcher who has spent the last ten years looking at NKH, who has seen a number for NKH children, whose work has laid the foundation so other NKH researchers aren’t starting from scratch – was in London on a three day whirlwind visit.

I found out the day he landed, but with a frenzied amount of emailing Dr Van Hove graciously carved out half an hour of his time on his final day for us.

This felt momentous – I have spent the last six months on a crash course into the world of metabolic biochemistry and genetics. My knowledge is rough, but I have read an inordinate number of research papers by Van Hove.

On the day I was nervous. Excited. I wasn’t quite sure what I was expecting, but I had a long list of questions and was hopeful about learning a bit more from someone who would know. Finally! Someone who had answers!

Van Hove is a very softly spoken, genuine,  and very very clever man. A man of science, I’d say. He looked Kai over, and was very polite and he listened to my questions.

Well, my long list was a complete sham. I’d gone in with the wrong approach. I was asking questions like ‘would x help Kai’s brain development’ or ‘would x help the seizures’. I was asking because I couldn’t find anything myself. I was asking because I was hoping finally, someone would be able to tell me.

I was a bit of a fool. On my very first question Van Hove very gently, and very kindly reminded that there was very little evidence for how things work with NKH. The more we talked, the more clear it became – there are a lot of good hypothesis. But there is very little evidence, and there is very little evidence because there is very little research. There is very little research, because there is very little funding.

I, just, I can’t even begin to explain how deflated I was. I had the time with the person who has spent over ten years looking at NKH, probably the closest to an expert as I could get, and still… I was relearning what I already knew. NKH is so rare, we don’t have millions funnelling into research. Our experts eek it out on grants and what small amounts families can provide. There is no evidence, because there is not enough research.

I gave up my list at that point. Van Hove did have a lot of interesting things to say, lots of new to me things, about growth and formation. About the research he’s doing, about chaperone therapy and his hopes for the future.

I won’t go into detail, because his current research is currently unpublished, but I’m hopeful. It was such a privilege to spend time with him. I have hope in his research. I have hope that the foundation learning he is doing will propel other researchers forward towards a cure. I have hope that he will find the right compound, for the right protein, for the right mutation. I have hope in him, because without him Kai, Sam and I have a very bleak future ahead.

Again, we place our trust in the research. We fundraise. We hope. There’s nothing else we can do.

When we’re having a hard day, sometimes it’s just a small thing that will shake us out of it. Last week we were having a hard day – Kai was grizzly and having seizures, and at 3pm I was still in my pjs, the flat was a bomb site and I couldn’t get my wits about me. 

So, I said fuck it. I kicked a part of the couch clear of debris and Kai and I relaxed down into a mini nest. And then I painted our toes a delightful shade of pink.

Sometimes it’s the small things that make you feel better.

I’m only just beginning to discover how lonely it is being a special needs Mum. And I know, I know you’re all here, and we’re not alone through this. So many of my Mama social groups have typical kids, and I love them. I think they are beautiful, and so clever and just, so amazing. But that doesn’t make it any easier to bear when I can see they’re developing skills Kai might not. That all their babies are holding their heads up by themselves, exploring the world with their mouths, physically able to grasp things, and bring them up to their face, to make eye contact and respond, to be able to bear weight through their legs… I adore these little babies, but every time time I see them there’s a grieving process for Kai. Every time, a little unintentional dagger to the heart.

And it’s not that I compare our kids, because I can’t. Our beautiful little NKH baby is doing things his own way, at his own speed. It’s that I’m still grieving this whole situation. That we’ll never get the typical version, that there is a lot of pain and goodtimes and heartbreak ahead for us and Kai. I’m of two minds – do I plunge in and socialise as much with these typical babies as I can, effectively ripping the typical comparison band aid off and at the same time normalising disability for a few people, or do I quietly step back (my current strategy, having just been at hospice).

I had a look for Special Needs parents groups in my area. I found a lot for autism, and ADHD, and down syndrome. Able bodied special needs. There was nothing for severely disabled special needs. Or rather, nothing that a quick google/facebook search threw up. I’m so disheartened. Where are the parents who get tubes and feeding pumps and wheelchairs and drool? The parents of the nonverbal and non ambulatory? Where do they hang out?

I think we mostly hide. Our days are so chaotic, and most days I feel like I’m only just holding it together, so most days we hide. I figure the special needs families in our community are hiding too.

Edit:

<3 for all the comments and suggestions. Yes, there are services and we’re pretty aware of whats around us. The problem with services is that they are at a fixed time once a week – if Kai’s ill or we have other therapy on, we’re out of luck. Example: We’ve had a standing appointment for our local Small Steps for the last few months, and each time our appointment comes up Kai isn’t doing well or we’ve had more important appointments, so we’ve missed it.

Here is what I’d like: a relaxed group of local people who meet irregularly and informally outside of any services. Like the amazing-ness of our NCT social circle, but special needs. 

We’ve been trying to find our feet with fundraising. It turns out that fundraising is HARD. We know can’t continue to ask you wonderful folk of #teamMikaere to keep donating. So many of you have donated already, and we’re so so grateful. So genuinely grateful I want to hug all of you.

But every time we talk to our researchers, we get a similar message: there’s no evidence, because there’s no research. There’s no research because there’s no funding. It’s difficult to hear that, that between me and my son’s future is something as stupid as money. And as much as I’d like to be on Ellen, showing off Kai’s cute face, or coming up with the next act of viral craziness (the most marshmallows in your mouth challenge? The mannequin breaks into the running man challenge?) I really don’t think it’s going to happen. 

So. We’re trying events, things people can come to that they’d pay for anyway. Here is what we currently have:

The Fulham Brass Band Concert

When: Sunday 21st May, 3pm – 5pm (this weekend!)
Where: St Etheldreda with St Clement, Doneraile St SW6 6EL London
Tickets: £11 – http://www.wegottickets.com/event/396988

They’re doing a bake sale and a collection for Joesph’s Goal. We’re very very excited. 

It turns out two friends at our NCT group play in the The Fulham Brass Band, and they do charity concerts. This particular concert has James Fountain – the principal trumpet of the Royal Philharmonic Orchestra. They’ve spun an English composer theme, and the programme includes Queen, Andrew Lloyd-Webber, Gavin Higgins, The Beatles and Richard Rodney Bennett.

We’ll be there, if you’re in London – fancy joining us?

The Cooking School #teamMikaere Pasta Class

When: Friday 23 June, 7pm – 10pm
Where: 3 Enterprise Way, Wandsworth, SW18 1FZ
Tickets: £75 http://theavenuecookeryschool.com/shop/courses/teammikaere-charity-dinner-class/

We’ve managed to snag a class – dinner (Pear and Ricotta Ravioli with Beurre Noisette, Rocket Leaves and Shaved Parmesan), dessert (Lemon Posset with Rosemary Thins and Raspberry Coulis) and Bottomless wine (!!!!) included. 

The thing with the ticket price…. a large portion of it is donated to us. We’re so so lucky that the Avenue has agreed to help us out. Spots are limited to 24, and we’re excited! Please come. You know how much fun we are on wine.

We’re also looking at a wine tasting, a 5k, and reaching out to local businesses for christmas jumper days + schools for non-uniform days. We’re not quite sure how it’s going to go, but we’re hopeful. So so hopeful. 

If you can come to the concert, or join us for the pasta making class, that would be amazing. Please come. Please help raise the money for NKH research.

Since Christmas Kai has had a new community neurologist. I’m not sure how I feel about neurologists. Most are fairly logical and we get the ‘we can’t treat the underlying disorder so let’s just make Kai comfortable’ line over and over.

Our new neurologist… he’s a bit different. Typically you like your doctors, as someone of authority holding lots of trust over your health, to be straightforward and logical people. Our other neurologists with the doom and gloom line have been. Very logical. Very straight forward. Very doom and gloom.

Our new guy is much more stream of consciousness than linear and straightforward. His mind is clearly going a kajillion miles a minute and what comes out his mouth is a stream of this point and that point with this study and that ancetodotal story.

But. A huge bit giant but. He is the first neurologist to offer us help. To offer slightly out of the box treatments to help protect Kai’s brain. He hasn’t given any guarantee’s, and as far as we can tell, there is no harm. But, he’s given us something small to try.

I think you have to be a certain kind of person to be willing to go over and above the usual ‘we only operate within the box of typical management options’ to explore other options.

Our neuro guy? He is it.

We’re willing to try it, assuming the harm is limited or can be mitigated, that there is some supporting evidence (even if it’s minor) and we put measurements in place to determine its effectiveness.

So, on one hand, hope.

On the other, at the same appointment I was completely blindsided by another sign of Kai’s disability.

We measured the circumference of Kai’s head. As far as I can tell his head looks in proportion with the rest of him, I was sure it was growing like it should. And sure, it’s growing.

But not in line with his age, weight or height. He’s in the microcephaly range. His head isn’t growing as much as it would be in a typical child, because his brain isn’t growing as a typical child’s brain is. My babies brain is not growing as it should be.

Another physical sign of my son’s disability.

It’s hearbreaking, hey. It knocked all the wind out of me, and while the appointment moved on I was struggling with all the emotions.

When we got back to hospice later that night, Kai was having a speight of seizures and as I held him outside in the hope that the cooler night air would help, I cried.

I love my little guy so much. I have so much hope, but every day I grieve for him. Every day I grieve for the uphill battle he fights in his little body.

And I struggle – trying to weigh up what’s best for him, what treatment we should and should not do, what questions I should be asking. I have to turn away from the emotional to be able to go into an appointment and have those conversations, but you can’t deny the emotional impact. It comes out and when I take the time to feel it it’s overwhelming. I find it so difficult to align my logical treatment plan and emotional sides.

Fighting for Kai, it’s an everyday, every moment thing. To be honest, some days when it’s overwhelming I hide. I’ll lose myself in a movie, or a game on my phone for a moment or two. It’s hard to switch off. I feel like I don’t know enough, that I’m not talking to the right people, that I’m not doing enough research, or not organising enough fundraising events, or just, all of the things.

I don’t feel like I’m enough, and that’s hard, because I have to be. For Kai, and his future, it’s on me and Sam. We have to be enough.

Kai’s seizures have escalated. On Friday they amped up to around a seizure a minute. A *minute*. We thought we were managing. Kind of. We have nurses who were constantly on the end of the phone with us, and we ran through our emergency seizure protocol on loop.

By Saturday we fled to Sams  parents for the extra help and come Sunday Sam and I were sleeping in three hourly shifts to ensure not a single seizure was missed. They were never ending, with breath holding, weird apnea episodes where during or after a seizure Kai would stop breathing for a bit.

It wasn’t feasible – not long term. Come Monday we were back home and Sam was meant to be working, and we were both so sleep deprived. Our symptom care nurse called it, we went into A&E to see if we could figure out the cause and then onto hospice for a symptom stay.

We had a new registrar in A&E (and again we went through the spiel, hyperglycinemia is not hyperglycemia, I promise he doesn’t have a problem with blood sugar). We felt like old hands at this. I had the cheat sheet ready with all the contact info, the med schedule, and the big red writing that said cinemia, not cemia.

Truth, we don’t know why his seizures kicked off so much. It could because he’s teething. It could be because we recently tweaked his meds. He could be sick. Too hot. Overstimulated. Constipation. Or It could be NKH progression.

In the end, our Community Nurse snuck our Neurologist out of his office and down to see us in the A&E. I love our neurologist. He’s a guy who loves his job. Who likes his patients, and genuinely cares about Kai. He takes the time to greet Kai, rather than just talk over him at us. He’s a lovely man whose brain moves a kajillion miles a minute. We like him.

Essentially, he told us to go to hospice, and hang tight. We moved the medication thresholds back so we weren’t sedating Kai quite as often, and we waited.

I’ve never been so grateful to get to hospice. Sam and I were so fatigued, so sleep deprived that Sam was asleep before we even finished check in. I fell asleep in Kai’s room, not even making it up to the flat. I’m so grateful for having the hospice support. I’m so so grateful they were there for us. Grateful that there  are people who know us, know Kai that we can trust to take care of Kai. I don’t know what we would have done without them.

Sam and I spent the next few days recovering. I had no idea how sleep deprived we were, how much we were surviving on fear and adrenaline. Even having one good nights sleep under our belts, it took a few more days before we lost that hungover, groggy feeling. Sleep deprivation is horrid.

Kai had some hard days. Lots of seizures. Mostly sedated, very little awake time. I hate those kinds of days with him, I really struggle. I can’t settle, and my brain goes a kajillion miles an hour second guessing everything we’ve done, trying to figure out the correlation between seizures and everything else.

We had a conversation with our palliative care consultant. We talked about the meds, and how one of them, the one we upped, an anti-epileptic effects another one, one we hadn’t upped, also an anti-epileptic. They are apparently processed by the same enzyme, and while the hospital neurologists assured us they shouldn’t interact which each other, when we upped the second anti-epileptic the seizures reduce slightly.

Kai started having more awake time, and seemed a bit more with it. Enough, that after a week, we were able to come home. Thank goodness for that.

Even better, once we were home I argued for a reduction back to the original levels, and his seizures came down even more.

Kai is still having seizures. They’re still part of our everyday, and we still often have to give rescue meds. But, we’re away from every minute. We have clear gaps after rescue meds, giving his brain time to recover. We’ve worked out a routine which means both sam and I get some sleep (kind of. Kind of not really, but better than sleeping in shifts). We invested in an angel care monitoring thingamabob so if Kai has an apnea episode while sleeping in his crib, we will know about it. I’m investigating wearable monitors, but we will see.

So, we made it through another crisis period. Thank goodness for that. 

This last speight of seizures have done something to Kai’s brain. While Kai was in intensive care over Christmas a neurologist said seizures less than 5 minutes don’t do any harm. I’m not sure I believe that based on the changes in Kai. It’s so demoralising watching Kai become more and more, for lack of better word, disabled.

Way back in December, when Kai was not even two months old I thought the doctors were wrong. Kai behaved like a baby, you’d never know he had a disorder by looking at him. Clearly he was just fine and didn’t really have NKH (spoiler: not fine). Then after our epic stay in intensive care/hospice over Christmas I thought they’d got the severe diagnosis wrong, as he clearly looked mild. (Spoiler: not mild).

After this last round of seizures, it’s hard to deny. Kai’s stopped making eye contact. He almost always looks top left now. If he looks elsewhere his eyes don’t always look in the same direction. And he’s stopped using his arms unless he’s incredibly agitated. I know it’s not the seizures causing the changes per say, that it’s NKH underneath causing the seizures and the brain damage.

It’s just so heartbreaking. I know that I use that word a lot, but I feel like we’re being hit with one thing after another.

Nonketotic Hyperglycinemia. What a bitch.