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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On losing his suck

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

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

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

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

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

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

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

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

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

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

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

 

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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