On getting into a routine.

Since being home we're easing into a routine. It's not smooth sailing, more like a clunky side jerk, but we try.

Kai's routine revolves around medication and feeds.  We start at 5:30am and finish up around 1am, and in between it's a dance of setting up feeds, drawing up medication, therapy, sterilising, washing and naps.

It's a full on, and while we'd like to settle into it, we're constantly thrown off. Seizures ruin everything. We have someone come and visit us pretty much every weekday. Nurses, therapists, dieticians, support workers. A visit or therapy that starts late or is commandeered by a nap will push out feeds (as if we did something like physio on a feed he'd vomit) which push out meds (as some of his meds require a full stomach) which push out the next lot of meds (as they need to be so many hours apart). 

We try though. We plunder through and try to manage all the ebbs and flows. In naps I do all the washing (we use cloth nappies, so there's an endless cycle of washing on) and all the sterilising (every syringe, every bottle) and drawing up meds and formula, so it's easier to manage. We try squeeze in homework. A few moments of tummy time here, leg stretching there, hands crossing the midline. We massage the old infected subcut site, to bring down the lumpy abscess that was left. We do a few moments with whatever portage has left us with - this week its a wind up music box. We hold it against his hands, so he can feel the vibrations, but can also pull away if he's not enjoying it. The week before it was the space blanket, kicking and touching to get the crinkley noise.

It does make it tricky for fun stuff. Which is why I'm grateful for Sam. By the time Sam gets home he's full of energy and happy to see Kai - he takes on the light hearted moments in the evening.

Sometimes we even manage a social something a rather. A walk with our NCT friends. Dinner with our nearest and dearest. Play dates with friends who have their own babes.

This is assuming, of course, that we're having a good day. If Kai's having back to back seizures, is inconsolable or requires a hospital visit everything goes out the window. In those times we operate on adrenaline. Meds and feeds are prioritised, everything else is dropped while we concentrate on managing whatever crisis comes our way. 

So, yes we have a routine. And we'd love love love to settle into it. And we do, on good days. On bad days, we do what we can to get through. So yes, we have a routine. We're not pro's at it yet, but we're getting there. 

On leaving Kai behind and hating it

When I was heavily pregnant August last year, I was very very excited when I managed to procure two tickets to see Harry Potter and the Cursed Child. Fast forward to now, after months of waiting and our show date is finally coming up, and it became a bit of a logistical problem. While we were keen to go, getting care for Kai is difficult. He has an insane medication schedule, and he has equipment for feeding and there's the testing that happens anytime we do anything with the tube.  Standard for special needs babies, but well over and above anything we could expect a babysitter to handle.

I have one friend (who I'm so grateful to) who comes on the occasional Monday and has taken on the task of learning how to feed Kai via the pump, and we have a hospice at home carer and a nurse who we're allocated three hours every two weeks... however neither of those options were going to cut it for a whole evening.

So, we went back to hospice for respite. Logically it works out - they know him, Kai knows them and the space. Sam's Mum came down to the hospice to spend some time with Kai. We stayed Tuesday night, and then on Wednesday morning Sam and I left to go to the theatre.

Well. 

Leaving Kai, as logical as it may seem, best case scenario and everything - I did not want to. I did not want to leave Kai for a whole day. I did not like it at all. Leaving Kai behind is always, always the hardest thing. I've come to realise that I know Kai best. I know how to care for him best. So leaving him to hospice nurses, who are amazing, and wonderful, is always going to be, well very very good, not as good as me and Sam.

I know that we can't do 24/7. I know that it's unreasonable to think that we can.  That doesn't mean that emotionally I'm on the same level. I'm not. Leaving Kai was horrid.

We walked out, leaving Kai in the very capable care of the hospice nurses and his Grandma and went to the theatre. We had a lovely day in London, fancy lunch and dinners and the theatre. In a past life I would have been ecstatic. In the After, London is very loud and people are very rude, and care about things that aren't important.

The show itself... some of it was very clever. Some of the magic tricks were brilliant. But I think that my idea of what is important has changed, and with my baby in hospice I found it hard to settle. I think that at the heart of it, the story could have been told in one show and there could have been less dancing wizard intermissions. I was very glad when it was finished, which was not how I expected to feel at all.  I think that's what happens when something from a previous life is out of place in your current life.

If leaving was the worst, coming back was the absolute highlight of my week. When we arrived at hospice, I skipped the signing in and hurried down the hall. I could hear him crying (break my heart) and when I peeked into the door he was having his nappy changed. I waited till his bum was clean and dry and scooped in for a cuddle.

Oh the joy, the absolute joy. Kai stopped crying, and smiled. He even laughed, the first time I'd ever heard him do so. He was very very happy to see us, and knowing we were missed - though I hate the idea that he even had the chance to miss us - and that he was glad to see us made me so ridiculously happy. 

It was a nice night out, and I was glad we managed it. I don't think it'll happen again for a good long time though. Time with my baby is well too precious for things like theatre. I feel like we've had so little time with him, I'm loathe to give it up for something like dancing wizards.

On #blessed

I can't even explain how grateful I am to be home with Kai and Sam. In our own place, with our own stuff, with all of that beautiful privacy. This past weekend we went for walks, and cuddled on the couch for family movie night (super not age appropriate, we watched War Dogs) and on Sunday? Sunday I spent the entire day in my pjs with my boys. Delectable.

No nurses, no home visits, no drama. I can't even begin to tell how beautiful this time is. Sam even bought me tulips on a whim. I'm so lucky, I know the hashtag is overused, but genuinely, when I get to have a day like today I feel genuinely #blessed. Normality hey, it's super underrated.

On being in the A&E

Well, fuck. I don't know what to say. We're in the A&E. At first it seems innocuous, we got Kai's glycine bloods back from last week, and they were a staggering 177.

Now here's the thing, 177 is good, it's within normal glycine range.... but it's not Kai's normal. Kai's normal sits at 250-300.

The thing with Sodium Benzoate is that if there is no glycine to bind to, then it gets toxic. If it's toxic, then calcium and potassium get dangerously low. So I rang our CCN (community care nurse) and practically demanded bloods taken, so we could find out. She asked us to come in tomorrow, but I persisted. The glycine results are a good week old, and I worry. I worry because the last time his glycine was this low, we were admitted into intensive care.

On top of this,Kai's been grizzly all day, and when he's not grizzly he's sleeping, for longer and longer periods (another worrying sign, similar to our pre-intensive care stay last time). And the seizures. Oh the seizures, they're more frequent, and longer in duration. Add the vomiting and I'm all over the place. Is it that he's teething? Is it that the vomiting is causing an absorption problem with the phenobarb? Is it sodium Benzoate toxicity? I don't know. We got a taxi to a&e who were expecting us. We walked in to see Dr Lukas, one of our regular registrars from our last visit. I'm glad we knew him. I'm glad he knew us. I was so relieved.

So, we did bloods. Bloods for kidney function (sodium, potassium and calcium levels) and liver function (phenobarb levels) and for amino acids (oh hey glycine) and two pipes, covering blood/gas and a regular blood panel. 

But then Kai didn't keep his feed down. And he was inconsolable. And then he had three seizures within an hour. Our emergency plan kicked in then, even when we were in a&e. We busted out our rescue meds, and Sam (having arrived after work, I'm so grateful for Sam and that he came) gave buccal midaz. Poor Kai. He was screaming at this point, and then the midaz knocked him for six. Oh my baby. 

I also called our symptom care team to let them know what was going on. I was very in control, but inside? All over the place. Emotionally well all over the place. I felt like I leaking out the edges, I was stressing out over his feed (we didn't have a feed pump, and I kept forgetting to ask the nurses for what I needed, so every time they came back I was asking for something, a 20ml syringe for an aspirate, a 10ml syringe for a flush, sterile water to make up a feed, more for the flush... on and on. I was very glad when Sam arrived. I'm always much more centred when Sam is around).

We were moved into a bay closer to the nurses station, and then into the assessment unit. And then came the waiting. The endless endless waiting.

Fingers crossed hey. Fingers crossed he's going to be okay.

Update: 

Bloods came back good hours and hours later. It's teething. We think. It's definitely not Sodium Benzoate toxicity. Thank goodness. It's the not knowing that bothers me, so I'm grateful to know for certain. We're going home.

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On tea with the Mayor of Wandsworth

The Mayor of our borough is a patron of our Hospice - run by the charity Shooting Star Chase. And as such, he invited the hospice goers to tea in his parlour. Sam was keen to go, so we dressed up in our Sunday best and off we went. To tea with the Mayor of Wandsworth.

Can I just say first, the Mayor has it well fancy. You walk into the lobby and all the floors are marble, and fancy panelled walls and it's all very very oh la. He's also driven around in a limo. 

It was a lovely little meeting. We talked about Changing Rooms, and generally about Kai. The current mayor is Councillor Richard Field - he's lovely. Before being Mayor he worked at Twickenham - he had some lovely stories to tell about Sean Fitzpatrick.

It was a pretty nice tea, actually. Very fancy. It's nice to feel like we're part of the community, and I appreciate that the hospice invites us to events like this. 

Update: on the seizures

Okay. So it looks like we have them under control. Ish. We've adjusted his meds for his recent weight gain. We give the phenobarb and sodium benzoate slowly, at the end of a feed, in the hope he'll keep them down. If Kai doesn't, hes already had some time to absorb the others.

So, we move forwards. Seizures are back as part of our days. We manage them. We keep them under control. We can totally do this.

On the seizures are back

And just like that, after such a good spell, the seizures are back. They look different, having changed their MO, and I didn't identify them as seizures, but the more they happened, the more sure I became. 

I've just faced the fifth seizure of the day, and I'm heartbroken. More than heartbroken, that word doesn't even begin to convey my despair, my fear, that sinking feeling you get when you suspect your world is tipping over sideways again. Do the next few days mark the beginning of another stint in intensive care, in hospice? We've only been home eleven days. 

We suspect it's because Kai is not tolerating the phenobarbitone. He vomits consistently after each dose. Doesn't matter whether he's on gaviscon, before the feed, after the feed, diluted. I hate pumping his little body full of drugs, I hate when he vomits, he gets so upset, and holding your baby while he screams is horrid. I always want to cry along with him, it's so unfair. So horrific.

It's in such contrast with happy Kai. Sometimes, after a feed with no meds I'll get a glimpse of what it could have been like. With a clean nappy, a full belly post burp, he's positively delightful. He smiles, and coos and makes eye contact. He lights up my whole world. He's happy, and will interact with us. 

I love that little version of him, and I'm constantly striving to keep him in his happy place, but it's so fleeting. We give him so many horrid meds. He vomits so frequently. And now the seizures are back. 

I just feel battleworn. We're only four and a half months in, and I'm battleworn. The only way I get through today is by hoping tomorrow will be better. That tomorrow there will be less seizures and more smiles.

Please, if there is ever going to be an end to this, it's in a cure. A proper, honest to goodness conditional gene therapy cure. 

I know I've asked a kajillion times, but on a day like to day where it's so clear that every moment we have is precious, that every day could turn, I'm going to ask again:

Please donate towards research. Please. 

If not via donation, please fundraise. 

Just, please help us fund a future for our son. 

On pulling off the neuro-typical band aid

I decided that, despite my dislike of being reminded just how disabled Kai is, I couldn't avoid neuro-typical babies. That to try would mean becoming a hermit lady and never seeing any friends. Not the most viable plan I've ever made. As much as I'm still grieving Kai's alternate healthy future, I decided that I would just pull the band aid off quickly and surround myself with neuro-typical babies. I wanted to be over this.

Conveniently, NCT has provided a whole group of lovely beautiful neuro-typical babies who were about Kai's age. Since we were in hospice, I've ignored the whatsapp group we had. I hadn't said we were back, and when they organised a trip to our local Baby Bach (a small concerto for babies in a local church) I was determined we would go. 

Can I just say that getting out of the house when it's just me and Kai is a giant faff of a mission. Not only do we need the typical round of nappies, wipes, change of clothes and a bottle, but we also had to have the meds, the emergency notes, the emergency meds and the escalation meds. We had to have the feeding pump, the strips to check the ng, syringes for aspirates and the power cable (just in case). The last time I went out with our NCT ladies, it was coffee and I ended up calling an ambulance for all the seizures. I'd forgotten half the emergency meds and the power cable and ended up being quite stuck. Never again, now we travel with everything on the off chance we need to go to the hospital.

So, I drew everything up ready to go, for both the feed and meds we'd hit the end of, and the next lot just in case. I managed to load Kai into the buggy (I actually did it three times, after a nappy and a vomit required two hasty buggy evacuations) but when I locked the flat door behind me, I mentally high fived myself. We, at the very least, had left the flat.

I wish I'd looked out the window before we'd left, I might have changed my mind. It was windy. Hurricane windy. Pull tree's down windy. From the gate it didn't look too bad, so I chanced it. What a terrible, terrible idea.

The church was a 20minute walk away, with a big park in the middle. The walkway through the park was like a channel for the wind. Twice the buggy was almost turned over. With Kai in it, and my holding on. I might as well have been walking a giant sail out and about. Worse, was that I hadn't realised that our weather shield was actually for the carrycot. Kai has grown out of the carry cot with his long legs, and was sitting the pram proper.  Which meant that the weather shield plain didn't fit. 

Needless to say, Kai now hates the wind and screamed the entire way there. 

When we finally got to the church, I had another high five moment. We made it out of the flat, and despite the weather, to our destination and just as the concert was starting. No matter what else happened that day, we'd already gone over and above my expectations.

Which was pretty lucky, because Kai did not care for the concert. Not even a little bit. There was lots of screaming and at least half a dozen poopy nappies. It's like he saved up all his poop and then released it all at once in protest. We spent most of the concert in the bathroom. Which, by the way, the baby changing station was in the disabled bathroom which I'm pretty sure no disabled person was going to fit in, considering how small it was. On top of that, perfectly capable non-disabled people had used the bathroom and it smelt rank. People are so gross.

So, instead of spending our morning like I imagined, blissfully listening to a world class pianist play some pretty music, we spent it stressed in a horrifically smelling bathroom. 

By the time Kai was empty, we had managed to catch the last song, which was pretty. And then the nursery rhyme time - that was pretty good too. I think Kai enjoyed that, it was much more upbeat.  And meeting our NCT friends. I'm so lucky to have banded with such an amazing group of ladies - they were openly happy that we were there and not at the hospice any more. Honestly, once I was there it was so easy to shrug off the fear. And oh, their babies. They have such beautiful beautiful babies. 

We spent the afternoon over coffee and it was just, so nice. A nice slice of normality. By the end of the coffee, I wouldn't have said I'd thrown off the comparison fear, but I'd at least made dent in it. It was nice to hang. Nice to see their beautiful babes. Nice just to be out and doing things.

It wasn't an easy morning, but I'm so so pleased we went. 

On being socially flakey

We've had to reorganise plans for visitors several times, just because Kai hasn't been well, or there's been unexpected hospital visits or whatever. It's the hardest.

I loathe being socially flakey, I know that friendships are built on time together, on being reliable and just, being someone people want to be friends with and come and see.

We're pretty isolated at the moment, so when we do see friends it's more amazing, more touching more everything. I have so much love for the people who have gone to lengths to come and see us, even when we haven't reached out.

So when we've had to rearrange plans again and again, it irks. I understand why we do, and I'll forever put Kai and his wellbeing over a social visit but it still irks.

I love my friends, I love hanging out with them. We've got a great crew around us, really, so to have to rearrange, it just sucks. I fear that eventually we'll be too difficult to hang with, that we'll be too unreliable. I haven't figured out how yet, but I'm determined we'll have some kind of social life!

So please have patience with us, we still want to see you and know you and we miss you guys. If you're in London and want to visit, we'd love to see you for something low key. If you're okay to come and see us, we'd love you even more for it.

Message me with some dates, okay? Seriously - please do. We miss you guys! 

On a weekend of firsts

Our first weekend home, we hit a series of milestones. Sam's Mum has been with us this first week to help ease us in, and she's been amazing. Small things, just being helpful and making sure we're fed and clean and have everything we need. I love watching her love on Kai - medical people aside, she's the person beside us to have spent the most time with Kai and he responds so well with her.

When we had to say goodbye, we felt a bit uncertain about how things were going to go. Sam and I managed - we knew we would - but it wasn't smooth sailing. In saying that, this weekend was the first time in forever that it was just us three. Just our little family, and oh. I can't even begin to explain how amazing that was. No medical people checking in, just time for us three. It was shakey, but so so freeing, and so so needed. I revelled in the joy that was the just-us time. I wonder if this is what it's like for other new families all the time?

Kai didn't make it easy for us though. There was a lot of vomit. So so so much vomit. And he pulled his NG tube out. I love seeing his cheeks free of tubes, and I love his determination to yank it out but the necessity and putting it back in is heartbreaking.

I'm glad Sam had the training, because it was on him to put the NG tube back in. We called a friend of ours who lives around the corner just to watch, just in case. She's a paediatrician, and spent some time in Africa developing an infant help program (very familiar with ng tubes). And with a swaddled squawking baby Sam inserted the NG tube like a pro. It was a bit nerve wracking for me, but I'm glad Sam was able to step up for Kai. 

While Kai was squawking - we noticed his first tooth come through. I was unprepared for that little toothy peg, but there it was. Small little baby tooth, right there in his mouth. It blows my mind, because I forget that he's growing. I feel like we only just managed to get home, that we've only just got our feet under us before Kai is changing it up again. 

Still. Loving being home. Loving being here with him. Fingers crossed we're here for the long haul, hey. 

On words and how they matter

I can't even begin to tell you guys about the outpouring of love we get every day. With every post or update there are emails, facebook messages, whatsapp messages, comments and texts. So so so much love. We're so grateful, we really are, that there are so many people in our corner. So many people thinking about us, and following our story.

But sometimes, some of the messages, while good intentioned,  aren't as helpful as one might think.

I read a thing today, an article by Tim J Lawerence about how things don't happen for a reason.

It's a good article. I've struggled with the words 'everything happens for a reason'. People say it all the time, but I'm not a fan. That Kai having a life of pain and hardship 'had to happen' for something better to occur? For some kind of improvement to happen? For us to raise money so a cure can be found? So we could discover what kind people we are or friends that we have?

Tim says it better in his post than I could:

That’s the kind of bullshit that destroys lives. And it is categorically untrue.  These myths are nothing more than platitudes cloaked as sophistication, and they preclude us from doing the one and only thing we must do when our lives are turned upside down: grieve.

You know exactly what I’m talking about. You’ve heard these countless times. You’ve probably even uttered them a few times yourself. And every single one of them needs to be annihilated.

Let me be crystal clear: if you’ve faced a tragedy and someone tells you in any way, shape or form that your tragedy was meant to be, that it happened for a reason, that it will make you a better person, or that taking responsibility for it will fix it, you have every right to remove them from your life.

Grief is brutally painful. Grief does not only occur when someone dies. When relationships fall apart, you grieve. When opportunities are shattered, you grieve. When dreams die, you grieve. When illnesses wreck you, you grieve.

So I’m going to repeat a few words I’ve uttered countless times; words so powerful and honest they tear at the hubris of every jackass who participates in the debasing of the grieving:

Some things in life cannot be fixed. They can only be carried.
— Tim J Lawarence

 

Kai's disorder can't be fixed. I have hope in NKH research, which is why I bang on about fundraising and how if you really want to help us, fundraise for NKH. But right now, the dreams we had for Kai, the experience of raising a typical baby, the imaginary life we thought we were going to get, we can't fix them. We grieve them. We grieve Kai's future, and the pain he's in, and the future we thought we were going to have.

Which is also why the phrase 'I can't imagine' isn't helpful for us. Because we can't imagine living this life either. Because when someone says it, it's impossible not to silently add 'and I'm glad it's not happening to us' on the end of it.

Here is what is helpful for us to hear:

"We have donated. We are organising a fundraiser. We are raising money for NKH. We are doing something."

I know this is a difficult one. I know it means work and effort and it's harder than sending an email telling us you're thinking of us. But this is the single greatest thing that can have meaning for us, this is one that is most important - the doing that says we believe a future is possible, and we're helping you get there.

Research is so significant for us, because it is the sole ray of hope we have for Kai's future. So many of our doctors and consultants are doom and gloom, who say that because we can't treat the underlying disorder, we should be concerned with keeping Kai comfortable (silent addage: and wait for him to pass on). We hear this at every visit, every doctors appointment they reference his death and how hopeless treating anything would be.  What keeps us going is the hope. Hope that a treatment will be found through research. Raising money for that research, that is significant for us. As one of the other NKH mums says, in regards to research, hope is on the horizon.

It's also helpful to hear: "You can do this. We hear it's hard, we hear that you are struggling and we hear that times are difficult. But you've got this. You can do this, you can face the scary hard unknown, and you don't need to do this alone. We are here, and here is an actionable thing we will do with you."

That last bit? The actionable thing? Better than 'if you need anything, just ask.' Or even "just letting you know we're thinking of you."  The actionable thing that would be most helpful would be if you had ten minutes, half an hour, an hour to google some NKH stuff for me.

About what seizures are and what supplements can help him recover (Taurine? Tyrosine? Omega 3? Alpha lipoic acid?). About how a type of medication works. About how to teach a kid that can't swallow how to swallow. What a keto diet is and can you do it while breastfeeding? Is the keto diet good for glycine? Is it better or worse than the low glycine diet for NKH? When should we introduce solids? How do we do solids with no swallow? When we can get a g-tube and what kind of solids can we do with that? What physio/OT things should we be doing? What benefits can we claim? What research programmes should we be a part of? Should we be getting a tumble form? Is there a grant we can apply for for things like bath equipment, extra physio or osteo sessions? When do we start worrying about scoliosis? What things should we be worrying about that we don't know yet?

My list is exhaustive and goes on and on and on.

Or the actionable thing could be something from the help list. Or it could be you just showing up. "I'm coming to visit next week so we can gossip and you can experience some normality. What day is good?"

I don't mean to complain, or ask for anything. No one owes us anything, and we're so so so grateful for the love, and the immense waves of support. Just, please no more 'everything happens for a reason' or 'I can't imagine's. Also, if people could stop using the word 'retard' as an insult, that would be good too.

 

On not being in the bubble

Since we've been home we haven't really left the flat. Not really. Small walks around the park, trips to the tiny corner supermarket.

Mostly we've hunkered in, part of it was trying to find our routine and a sense of normality. Trying to reclaim a sense of us after two months in hospital and hospice.

Part of it is fear. I'm very very aware of what it's like to be outside the safety of the hospice. The looks at Kai's NG tube and the look away, with side eyes after (just look for goodness sake. Acknowledge him and all his tubey glory. Look and smile like you would any other baby with ridiculously chubby cheeks).

There are a kajillion Mums and babies out during the day. Mostly I smile and my gaze kind of washes over them, I don't look too closely. I don't want to compare my baby with theirs, I don't want to investigate how my little guy is doing developmentally. I know he's behind. I know he's not holding up his head or putting his fingers in his mouth, but what he is doing is more than enough. I'm so attuned to Kai as he is, I'm scared to know what typical development looks like.

It's the fear, I know. We've spent so long dealing with the intellectual side of Kai's disorder, and getting on with everything that we haven't really dealt with the emotional side. To be honest, we went into hospice on end of life care, so that we managed to make it home again was joyous enough. I didn't know this was a thing I needed to deal with. Not until small typically developing babies were everywhere.

In the end it happened at the pub. Our very first pub lunch with Kai and Sam's folks. Friends of ours from NCT stopped to say hey, and how pleased they were to see us out of hospice. I was so so glad to see them, but was completely unprepared for how my emotions dropped out when I greeted their gorgeous little girl. She's beautiful, hey. The most beautiful amazing little child you ever did see.

The difference developmentally between her and Kai was like night and day. She was born a week before Kai and oh my days. She's so present and alert. Able to focus and hold a gaze, to recognise a face and smile in response, so social. Her movements smooth, and arms crossing her midline in delight.

I smiled and held it together, because grief is a private thing and I try not to process in public. Besides, despite the emotional turmoil that I was experiencing knowing just how developmentally delayed my guy is, she truly was beautiful and amazing. I was so pleased for our NCT friends (and I say that genuinely). They seemed well, and happy.

We said our goodbyes, and it wasn't until Kai needed a nappy change that I cried it out in the privacy of the bathroom.

Every day I grieve the alternative to NKH. Everyday  I grieve what it might have been like, the future Kai might have had, the future our family might have had. I love Kai and Sam more than anything, and it's hard to let go of the what ifs. The alternate reality without NKH.

I often remind myself that one what if was that Kai could have died and we're so lucky to have him with us. But still, I would be remiss if I didn't acknowledge the extreme sadness I have that we don't walk the typical path. I think this is something all special needs parents go through, right? This is the still longing for Italy, when you're bang smack in the middle of Holland. I mean, Holland is nice and all but it's not Italy. No offence, Holland.

Sigh. I do my best to acknowledge the craziness of this whole situation, and then we move forward. Nappy, feed, meds. Another day down. We'll get through.

 

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On guilt and fucking up his phenobarb dose

Some days I'm okay. On some days I feel like I have things under control - Kai's clean and in a dry nappy, the washing is happening and the sterilising is done and Kai's feeds and meds are happening when they need to. I've showered and eaten and brushed my teeth, and the flat looks somewhat normal. On those days, I feel capable. More often are the days I feel like I'm barely holding it together with sellotape. On those days if Kai is fed, had his meds and his nappy is not too stinky, I'll hold fort until Sam (thankfully!) gets home. I feel like I'm juggling all the things, and just barely making it work.

It's so easy to miss things, to drop one of the balls and mess up. Like I did with Kai's phenobarb dose.

We give Kai's medication through the tube in syringes, so everything is measured out in mls. Because everything is at different strengths, the scripts are written in mgs, and we work out the mls from the strength. As an example, Kai takes Sodium Benzoate. The script says 500mg/kg/day which we split into four. Kai's working weight is 6.88kg, so the total for the day is 3440mg, which split into four is 860mg per dose. At a med strength of 500mg/5ml that works out to 8.6ml per dose, and that's what we draw up in the syringe and give to Kai.

Fine for Sodium Benzoate,  but there is a lot of working out and thinking and rechecking that happens. And Kai's on 9 different medications each day. So it's not a small undertaking every time we get a dose change or a new stash from the pharmacy.

My fuck up happened with phenobarb. Phenobarbitone is an anti-epileptic. We've been trying to wean it since before we left hospice. It's horrid, it makes Kai vomit and it's just, blurgh. We're trying to increase zonisamide (another anti epileptic) as we wean phenobarb. Phenobarb takes forever to come out of your system, and it takes forever to load up. (Note: forever = 3/4 days).

We'd been home not even a week and I was freaking out. After a particularly unsettled day - when I was checking my calculations I thought I was meant to be giving Kai 7ml, but had been drawing up only 2.1ml - that's an extreme wean. What happens on an extreme wean? Seizures and vomiting - tick and tick. Poor Kai. I rang our hospice symptom team all in a panic, we went through all the doses and decided on a part way (3ml) doses to help ease the drop.

Here's the thing though, seizures and vomiting can be caused by a trillion things.

So, while we were in hospice, the strength of the phenobarb was 15mg/5ml. Once home, we had a new bottle with a strength of 50mg/5ml. And for a hot moment, I'd mixed them. Two days after freaking out I sat down with the bottles and redid all the calculations. The bottle said 50mg. The 2.1ml dose was the correct one all along.

I felt like such a wally. Horrified that I could have made such a mistake that effects Kai's care and well being so drastically. The guilt at my best not being good enough, the fear of hurting Kai and the anger at myself for messing up took a few days to dissipate. I know Kai's med schedule the best. I know him the best. I still can't believe I fucked it up, but there you go.

After talking to our symptom care nurse, we reduced the dose to a lower 2.4ml. She came and did a visit (and when she did, she looked at the bottle to make sure I'd worked it out correctly) and we restarted the weaning schedule.

The enormity of my responsibility for Kai feels overwhelming sometimes. Honestly, sometimes I wonder what it would be like to have a 'typical' baby. Walk in the park by comparison. But then I think again, about people I know and just how some of them would be completely rubbish at being special needs parents (not you, FYI. I don't socialise those people anymore so probably not you) and I go back to 'thank fuck he came to us' - even despite my small mess ups, and despite bad days being held together with sellotape, we've got this. We're capable. His best shot is with us. And then I feel much better.

 

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On losing a little bumblebee to NKH

So they say that bumblebee’s aren’t designed to fly, that their bodies are too heavy for their little wings - wings that aren’t meant to enable flight according to the rules of aerodynamics. And yet, they fly. They don’t know they’re not meant to fly, they just keep on keeping on.

Because of this, the NKH community has adopted the bumble bee as one of their own, a little reminder to keep on keeping on. Every NKH parent is told their babies aren’t meant to live, and yet they do. I talked before about how Kai could go either way: Kai doesn't know his brain is broken, he doesn’t know that he’s got a little glycine problem messing about with his brain development. He doesn’t know that his disorder is terminal. 

Today one of our NKH kids passed away. A beautiful little four year old who deserves to be remembered. He deserved more time, more life. He deserved so much more than NKH allowed him.

This beautiful little guy is the first NKH kid to pass since Kai was born, and I won’t lie, I’m devastated. I didn't know him personally. I don't know his parents. I can't even imagine their grief.

But I do know it could easily have been Kai, and one day I’m sure it will be. I’m so angry - I feel like the research is SO CLOSE. I feel like we just need to hold out, protect his brain as much as we can. That hope is on the horizon. 

When an NKH kids passes, one of the phrases we say use is ‘Fly high.’  It's all so heartbreaking, and today I'm loving on Kai a bit more than usual.

 

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On being home

And just like that, we're home. We haven't  been home since before Christmas. Sam put away the tree on one visit earlier in the year,  but we still have all these unopened gifts sitting in the lounge.

It felt weird to leave the hospice, we said our gbyes quickly, and when we were finally out of the building, we packed up the car so quick! As it's as if we were afraid we were going to be called back.

Being at home... it feels so good and so odd. We don't have a routine at home yet, and all the added pressures of things like cooking and shopping and remembering to take the rubbish down have me a bit off balance.

Still, I'll take it - sleeping in a proper bed again is so so amazing. Wearing pjs all day and not worrying about what your hair looks like. The sense of privacy, no nurses sticking their heads in to see how we are! All the things we have! I'd forgotten how much stuff we just have. How much stuff Kai has (he's outgrown almost all the clothes we have for him!)

It's amazing how much we take for granted, but I'm truly grateful that after a month in hospital, a week in intensive care and another month in hospice on end of life care, Kai basically said fuck it and got well enough to come home. I love him.

 

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Dear Kai - Month 5

Dear Kai,

Another month - I can't believe how much you've grown! Those cheeks! They're so big, one of your nurses insisted we weigh you to see if you'd jumped a percentile. You haven't, you're growing just as you should be, but apparently it's all in your scrumptious cheeks.

This month we got to take you home. Can we just pause for a moment and celebrate, we went into hospice on end of life care, and we were able to take you HOME! Being home as been wonderful and crazy. So wonderful, so crazy. So much chaos. I'm loving this time with you though, I love spending my days with you, though they seem to pass in a flash with your schedule.

Your schedule is crazy hey. With the meds, and feeds and all the therapy. It's been the month of a meeting kajillion new therapists. Physio and dieticians and nurses early support and portage. The list is never ending. We're getting into the rhythm, we have so many people through, easily someone every day. I very quickly started reserving Mondays 'just for fun' else we'd be overrun. You're best in the morning, and enjoy physio and play therapy with portage. You easily charm everyone, your hospice carers, our health visitor, the slew of nurses and dieticians. Our people often say they're not meant to have favourites,  but they do so enjoy spending time with you! I'm so grateful they come to us, because schelping you about with all your kit requires a plan executed with military precision.

We do manage to get out, though. It took a week or two - for the longest time we weren't able to settle in, just us three. We were always at the hospital, or hospice. So for the first few weeks at home we hunkered down - loving on you, trying to get into a routine and come to terms with your insane schedule of meds and feeds (we're still waiting for it to break in a little, for it to feel more like second nature rather than like we're always behind the ball).

Once I got over the babies-without-rare-disorders fear, we went to all the (very local) places. We tried Baby Bach (not your thing) and we're doing pretty good at walks around the river. Hanging out with our NCT friends has been pretty fun too.

It's hard not to compare developmental milestones, so we try extra hard to celebrate what you can do. Your suck returned, and with it your love for the dummy. We also do a bit of a bottle feed before each meal, if you're awake. Your first tooth came through (!!) and we discovered that if we roll you onto your side, with some determined kicking you can roll yourself onto your back. Every day we manage a little bit of physio, and a little bit of portage homework. You're not a fan of tummy time. Not even close. Your head is still wobbly, but sometimes you'll give it a go, holding it up unsupported for a microsecond here or there. You're much more active, and talking to us. You've found good use for your voice! It's such a delight to hear your happy gurgles.

We're not much into a routine outside therapy, meds and feeds (your medical needs make it difficult) but when we can you enjoy baths with Daddy and afternoon walks. You've outgrown the carrycot, so now you sleep in the big crib at night, and ride about in the pram proper. We've had a right old time with the weather shield (and we now know for certain that you hate wind). It's better now that I've figure out how to put it on correctly.

You're getting pretty good at pulling out your NG (though I'm a bit better at stopping you). You almost always end up in our bed in the mornings (one, cause it's easier to give meds if your next to me and two, baby snuggles). Though throughout the day you nap wherever you happen to be lying.

Your meds continue to be difficult, you struggle to keep them down, so we spend a long time dancing about to give you the best shot at not vomiting. You've also had your first proper cold, and with it came a slew of seizures. You were also on antibiotics preventatively, to ensure you didn't get a chest infection you couldn't fight off. Helpful because you weren't able to cough up all the mucus that was sitting on your chest. We spent an awful lot of time trying chest physio!

This month really has passed in the blink of an eye. Daddy dressed you up in an England onsies for the six nations (though rest assured, you'll be in an All Blacks onsie when they thrash England). I managed to carve out some time to crochet a hat with ears, which you're not a huge fan off. We've also been socially able to meet more of our friends. It's slow going, as we're wary of overwhelming you (and then there's the fear of you getting ill as people touch you with unwashed hands, or worse, if people try to kiss you). Still, I can't get over the pride I find in you when my friends (appropriately sanitised) get to love on you a little. My bonny wee guy!

You've done well this month with hospital visits. We only had one trip to the A&E, which you handled like a pro. We were lucky enough that instead of being admitted, we were sent back home! Good job, little guy. We were so pleased.

Another highlight was meeting the Mayor of Wandsworth for tea in his fancy parlour. You seemed not fussed, but it was a a nice song and dance for Mummy and Daddy! We also managed some Mummy/Daddy time for Daddy's 30th. We left you for the first time in the capable care of our hospice carer and went out to dinner for a few hours. It was both the longest and shortest few hours ever. I hate leaving you baby, but it was so refreshing and weirdly bizarre to slip back into a parody of our pre you life. I've never been more grateful to be home, though. I feel like every moment with you is a precious one, and I don't want to miss a single moment.

I'm so grateful you're home, baby. You've changed our lives in such a fundamental way, and I can't imagine life without you.

Love you more than you'll ever know,

Mama x

On Sam and Adam

One of the worries about being home is what happens when Kai pulls out the tube. We won't have nurses in our living room, so we can't just pad down the hall and have someone put another back in.

They asked if I'd want to learn how to put it in, and the truth is: no. No I don't. When I have to hold Kai's head still, and he's screaming and there are a kajillion hands over his face putting tubes down his face I want to cry with him. I hate it, I positively absolutely loathe that this is a necessity.

Put in perspective, it's not the worst tube. It's not a cannula into a vein, or a vent to help him breathe. It's a small little one, and by far not the worst tube.

That doesn't mean I don't hate it, because I do. I hate that it messes up his face, and that we need to mitten his hands to keep it in. I hate that when he screams you can see it running down the back of his throat. I hate that Kai clearly doesn't like it, and wants it gone.

So when we're putting a new tube in, I feel all the things. And I'm crooning platitudes and trying to comfort my child as I forcibly restrain his head and just... it's rubbish.

Once we're home we will have community nurses available to us during daylight hours, who can come out and put a tube down if we need.

At night? At night Sam stepped up and said he'd learn. To be fair though, he stuck a tube down his own nose at the prompting of a nurse the other day, so yeah.

He's spent some time hanging out with Adam. Adam is the creepy child mannequin where you can learn all the things (NG, trachie, g-tube button among a dozen other things. Poor creepy Adam has it rough).

Still, Sam has been signed off by two nurses, and has been given the thumbs up to put an NG tube in for Kai if need be.

Honestly, I was so blissfully unaware of what special needs parents learn to do in The Before. I guess this is just one thing of many that we'll learn to take care of.

 

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On the now

We're gearing up to go home. I've taken on the meds and bulk of the care. Kai sleeps with us now, instead of under the watchful eye of the nurses, which is nice. I feel a bit like I'm getting my baby 'back'. The nurses pop their heads in to see if we're fine, and if we are they leave us to it. It's pretty refreshing, actually, to be the main carer for Kai, like I'm meant to be.

In saying that, it's not a walk in the park and I'm grateful the nurses are close by if we need them. Kai's horribly difficult to aspirate. Horribly horribl difficult. It's frustrating for everyone, Kai especially. I hate it, trying to pull up something from his belly to make sure the ng is still in the right place. If he's sleeping and we can't get one, we reposition him or do mouth care, often waking him. He is not a fan of the NG either. He's always trying to pull it out. The nurses say he'll 'get used to it' but I suspect he'll get used to it in the same way you would if you had a thorn in your ankle, and you didn't know it wasn't meant to hurt.

We have to mitten his hands to keep him from pulling the tube out if we're not hovering over him. It's so frustrating, and I worry we're impeding his development, I'm pretty sure he's meant to exploring things with his mouth, and would if he knew he could reach his mouth with his hands. He's already showing signs of shoving his gloved fist into his mouth. 

I'm also anxious to get the referrals sorted. We're waiting to see a gastric surgeon for a g-tube, so we won't have put meds down his nose (instead we can put them directly into his belly). And we're waiting to see a speech and language therapist about his suck. And a dietician. It's taking forever to sort and is so frustrating. While I'm grateful for the NHS, it's so slow moving.

Kai's also having a hard time keeping meds down, especially the phenobarbitone and sodium benzoate, medication used to keep his seizures at bay and the glycine in his blood down. You know, just the important ones. We do a big song and dance around giving them. They must be diluted. They must be given on a full stomach. They must be given slowly (0.1ml/ten seconds. SB is 8.4ml, so takes a good quarter of an hour). Once in, we don't move Kai, we don't touch him, we love him with our voices. We make sure his nappy is fresh before we start so we don't need to do it later. We stagger the meds too, some at the beginning, some mid feed, some at the end, just in case it s a volume issue. Sometimes it works, sometimes he vomits no matter what we do.

This whole medication tube business is rough. We do it, because there is no choice in the matter - Kai needs it. His brain needs it. So we do it, but the now is rough, and we're not even home yet.

Still, worth it. For every single day we get with him. Worth it.

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On the infected subcut site - an update

So it's been a few days and the antibiotics aren't doing their thing. The abscess hasn't burst, but it does have some fluid. It's hard around the outside and soft in the middle. On the hospice gps advice (they come visit us, it's the most convenient gp visit ever), anyway, on the gps advice we went back to the local hospice hospital to see about helping it along.

Two things happened.

The first is that they whacked on some cream that numbs while it breaks down the skin, making it thinner and therefore more likely for the liquid underneath to burst through. That sounds horrific, and I worry that his poor skin is going to be so irritated and raw, even after the numbing has worn off.

The second is that if it hasn't burst, because the local hospital won't do anything surgical on children under 5, we have to go back into London to see a surgeon about cutting it. This could mean anything. I could mean a local, it could mean a general. It could mean a bit of numbing cream. We won't know until we get there.

This of course freaks me out, because any major stressful event may cause seizures and coma. We only just got over the last lot, that came on from two monthly immunisations and a cold.

We've got our fingers crossed it bursts.

 

Update:

It didn't burst. We went into London to our regular hospital but we were lucky - the day unit wasn't busy and the surgical resident Dr Julie was available, she was so wonderfully nice to us. We talked about the best approach, which was to lance it and let it drain. So we went into the treatment room and did it, with some numbing spray.

Oh my days. It was disgusting,  but at the same time I couldn't look away. The pus was a horrid green/brown mustard colour and gushed out. It was foul, I can't believe that was in my babies leg! There was so much of it too, it was the most crazy thing, it just kept coming! Poor little baby.

In the end it was dressed, and we were given a script for antibiotics. We spent more time in the pharmacy than in the ward.

Still, very very glad it's taken care of! 

 

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On faith and superstition

In the Before, I wasn't a superstitious person. I wasn't a religious person, either. I can't remember who it was that told me that largest percentage of middle class, comfortable people are atheists, mostly because their lives are so comfortable and they don't often face hardship out of their control. If you're not in a situation where something horrid is happening, something truly horrid, then you don't need faith or superstition to get you through.

At hospice I've picked up a few superstitious habits, because there is so much with Kai that we can only face with hope and cuddles. If we don't want something to happen, we don't mention it for fear of jinxing it, having it come about. We talk around it. If he's sleeping and settled, we won't say something like 'he's keeping down his meds! Hopefully he'll keep down the next lot' because then for sure Kai will vomit.

When we do talk about things we're hopeful about, we always always prefix or end it with 'touch wood'. And then both Sam and I will touch the nearest bit of wood, hopefully unpainted. Some of the nurses do it too, which is where I suspect we got the habit from.

I also throw up small tiny prayers of hope when I'm faced with a junction where Kai could go either way. I've never prayed as much in my life as I have since Kai was been born. Honestly, there is a lot of prayer.

When you're faced with such extreme love and against such extreme hardship and pain, with the possibility of extreme loss, I think that's where faith and hope are the strongest.

 

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