On the Head Shave

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WELL.  We hit £5,000 in our fundraiser, here: www.justgiving.com/gareth-shave

So it was bound to happen, this head shaving business. I was nervous to begin with (because seriously: people have very strong ideas and gender norms/stereotypes around women and hair – I know this because several people tried to convince me NOT to shave my head – shame on them!) and I wasn’t sure how I would feel about shaving my hair. If I’m completely honest, I was mostly nervous about what my husband would think!

I hadn’t cut my hair since Feb 2014. It was long – mermaid length. Gareth, six foot ginger ninja stopped cutting his hair in 2015, back when he became a nomad. He has beautifully long, curly, red hair that makes him look a bit homeless when he doesn’t style it.

We agreed, one night over beers, that if we raised a certain amount for NKH Research, we’d shave it all off. Turns out our thresholds were quite low!

1. If we reached £1500, Elly and Gareth would both get number 2’s

2. If we reached £3000, Elly and Gareth would use razors, and be completely, 100% bald up top. (This turned out to be impossible, so we listened to the advice of the barbers and used the closest clippers we could possibly get).

3. If we reached £5000, Gareth would ALSO shave off his beard. Woah buddy!

Gareth and I did it together.  We donated all our locks to charity, so they can be donated into wigs (thats nice) and we held hands as our heads were shaved. Oh. My. Days!

I was unprepared for how freeing it would feel, and actually, how nice having your head shaved is (like a head massage, but nicer!)  Afterwards, my head was cold (I felt every single draft, even in a room with all the windows closed!) but I was surprised at how much I LOVED it! It feels incredibly tactile, and I can’t stop touching it.

So here we go – shaved head.  Thanks to everyone who donated, who cheered us on while we did it. Thank you to my partner in crime – Gareth – for shaving off his hair + beard with me, and for organising the barber from Flame and Steel (flamesteel.co.uk), who very kindly donated his time. I’m grateful! Thank you to every single person who donated.  Genuinely, thank you.

And I know I say it all the time – this thanking business – but I genuinely am. I’m beyond desperate for a cure for Mikaere. His seizures are daily. His vomits are awful. His quality of life is not even close to what we enjoy. When I go to bed at night, I’m not 100% sure that everything will be fine in the morning. That Mikaere will be with us, that he’ll be happy and able to move through his day. We live with uncertainty and grief and THE ONLY solution, the only long term, positive solution is research.

I have all my hope in the research. That it might, in Mikaere’s lifetime, be able to help him live a better life.

So with that desperation in mind, every donation feels personal. It brings us closer to a future with our kid, so yeah. Super personal. So THANK YOU again for all the support and love and donations.

If you’d like to donate – we’d love to have your support: https://www.justgiving.com/gareth-shave

So yes. Shaved our heads. Done!


Dear Kai – Month 5

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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

Dear Kai – Month 4

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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

Dear Kai – Month Three

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Dear Kai,

Oh my beautiful baby. I don’t know where to begin – this month started on such a high note. We spent a wonderful weekend with your Grandparents up in Toddington – your first road trip! You were so loved – fussed over by Aunty Josie and her friends, cuddles with Grandma + Grandad. You even met Great Grandma Majorie – it was amazing to see four generations hanging out on the couch together. 

We went to church too, and met so many of Grandma’s Church friends. After your rocky start, you’re practically famous in in Toddington. We were so pleased to show you off, to shake away the cobwebs of hospital and move forward towards little normality. You loved it too – there was so much love, so many people to coo over you and replace the dummy you kept spitting out just for fun. 

You did have a little bit of reflux, and we were worried about your weight plateau a little, but generally you were your happy grizzly little self. 

Your deterioration happened a little while after – the NKH Three Month Curse, popping up right after we got your immunisations. I’ll forever feel guilty because I think my decision to get your immunisations acted as a catalyst. Oh my baby boy, I’m so sorry.

You had seizure after seizure. We were admitted into hospital and an eeg confirmed it by showing hypsarrhythimia. Devastation doesn’t even cover it, we had hoped we might have longer before the seizures came. Nonketotic Hyperglycinemia can suck it. 

And oh how you fought my little guy. You tried and it was heartbreaking to watch. We stayed on the ward for a few days before we were discharged, when it looked like you were recovering. We lasted three days before, oh baby, you had your first five minute+ seizure. We were out with our NCT friends, and it was the first time I’d seen the difference between NKH and not NKH, even before your seizure happened. I guess this is our path now little guy. Where you go, Daddy and I will follow. 

You’ve not been home since that seizure. We spent a week on the ward, where you got more and more distant. You stopped opening your eyes. You stopped crying. You stopped moving. The only constant were seizures, happening more and more frequently.

And then on Christmas Day, when we couldn’t stop them and you were seizing constantly, you were admitted to Intensive Care. Oh my little guy. We love you so much, and in a place like intensive care that love is accompanied by so much fear. We were asked to consider terrible scenarios, and make horrific decisions for you. Through this whole time we worried for you, we did everything we could to ensure any decision made was what was best for you, but it’s difficult baby. Trying to differentiate what we’d like for you, and whats best for you is so so difficult. It was shortly after one of these conversations that you were baptised. We would have loved to have had a beautiful ceremony in a church, with you in one of those fancy long white Christening Gowns but alas, that was not our story. It was a sweet little ceremony done in intensive care, the curtain closed against the business of the ICU with your Grandparents.  

We didn’t celebrate your first Christmas like we wanted to either, but we did set up a fundraising scheme for your first Christmas. Our friends and family rose the occasion and we’ve raised several thousand pounds towards NKH research. We’re so pleased! We hold on to the dream that you have a future, that one day the research will provide the reality to our hope and your future will be comfortable and happy. In the meantime, we did try enjoy what there was. You were given a giant snowman balloon, and on Christmas Eve Grandma + Grandad came down to visit, as well as Uncle Michael + Aunty Mathilde. It was a fairly cheery time, even if you slept through it all.

Shortly after being admitted to Intensive Care, Grandad Gedge and Aunty Liss came to stay for a week. We settled into a routine where we could be with you, switching two people in and out. You spent every day in someones arms, as we held your hand and hoped, letting the nurses know when you seized, which you did frequently. 

When the New Year rang in, Daddy and I were right by your cribside in intensive care. There’s an excellent view from the fifth floor across the London skyline, and on the horizon we watched the fireworks that happened along Southbank. It was pretty, and scary – we don’t know what our future holds and we’re desperate for you to open your beautiful little eyes again.

It often seemed like you were so close to the surface, so close to breaking through into consciousness. But then a seizure would take you away, and we’d sedate you. Hoping to give your brain rest.

We moved to Hospice in the New Year. For us it seemed like the most sensible choice, and we hope that a more relaxed setting will help. I’ve been ignoring the End of Life part of Hospice. I don’t think we’re there yet. I’m hopeful that whatever wracked your little body just needs a little time and love to recover. 

We’re still here as we celebrate your third month. We’re so hopeful, and we love you so much little guy. Every day we spend with you, we’re so hopeful. You’ve changed the trajectory of our lives in such a fundamental way, and I can’t imagine a world without you in it. 

Arohanui my little man, more than you know.

Mama x

Dear Kai – Month Two

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Dear Kai,

This month has had so many firsts for you, your first ambulance ride with a full entourage out to meet your metabolic consultant. Your first time without any monitors, a proper free range baby. Your first time leaving the hospital. 

We got to spend your final few days in NICU rooming in and I think we were all drunk on the freedom. You expressed your delight with lots of screaming, but, thanks to our amazing NICU nurses we’ve got a few different tricks up our sleeves – the baby burrito swaddle works a treat, and you chill out so good.

We also had a big round the table of Team Mikaere too. Oh little guy, you have so many people on your team! It was pretty intimidating walking into a room with a good ten medical professionals around a table (and that’s not even all of them) but everyone is looking out for you. The system has a lot of moving parts, but we’ll figure out how it works, I’m positive we will.

But, after big meetings and a handful of goodbyes and forms and talks, my sweet boy we took you home! 

On the way out of the hospital, we met another couple. They were very fancy and had a boy a fair bit older than you. The lady was very taken with you, how little you looked in your car seat, how pudgy your cheeks. She was the first person who loved on you who had no idea you had a super rare, tiny little glycine problem. You were just a beautiful wee little baby.

Since we’ve been home there has been a learning curve for us both. One of us is definitely more patient than the other. Still, were managing. I’m slowly beginning to differentiate your cries, and we’re working out a routine of sorts around your many medications and hospital appointments. 

Getting out of the house always feels like such an achievement, but mostly I love the days we spend in. Hanging out in bed all morning, all cosy. The best bit of the day is when Daddy gets home, he scoops you up and your nightly routine starts. Meds, a feed and a bath with Daddy, before going down for the night in your little cot. You take to it so well! You’re a fan of the bath, chilling out in its warmth. Less a fan of undressing before or towelling off after. 

Oh little man, you have so many people who love you. We’ve had so many people come over to meet you, and you’re so gracious with all the cuddles. I’m such a proud mama, I love it when people talk about how cute you are, how chubby your cheeks, your long legs and your funny little friar tuck hair.

It’s not all super rosy, we’re waiting for the seizures to start, and you’re having these epic reflux fits. We know that your medication is sad on your little belly, and you’re so upset when it all comes back up. We’re working it out, trying to make you as comfortable as possible. You suffer us with grace and screaming, we’re still learning. Your facial expressions, like little old man grumps always make us laugh. 

Oh baby, you have changed our world. Everything has condensed down to you – suddenly it’s very clear what is important and what is not. I feel like we have a rough time ahead of us, but having you home and loving on you? It’s been such a delight. 

Arohanui my little man, more than you know.

Mum x

Dear Kai – Month One

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Dear Kai,

Happy One Month! What a month it has been. Your arrival and this first month was definitely not how I imagined motherhood to be. No one imagines they’ll spend the first month of their babies life in NICU. 

But what a fighter you are, those first few weeks you were so strong, despite the war raging in your little body. But oh, those first few weeks were so hard. So so so hard. To hear you make one little squawk after birth and then fall silent. To see you squint and open your eyes just that one time, and then to watch your little body stop moving and eventually become non-responsive. To have a machine breathe for you, with your little face and body covered in tubes and needles and wires. That time the vent got blocked and you turned a dusky shade of blue and needed to be resuscitated. Oh my sweet little boy, in that very first week I felt like I’d never loved so much and feared for everything all in the same go. 

Your diagnosis is a tricky one. So rare, so full of unknowns. I’ve really struggled with it. But you, what a little trooper. Once we had an idea what was going on and were able to give you meds (which came with a severe warning that you may not respond to them) you came around like a champ. Since then we’ve been celebrating every little victory you’ve given us. Every tiny one.

The day you opened your eyes for the first time I bawled – big great sobs of relief – your eyes are so beautiful! So so beautiful. When I was able to hold you I cried. I cried the day your vent came out, and the very same day you took yourself off CPAP and started self ventilating – high five for breathing by yourself!! That was a good day. So was the day you cried for the first time. The day you started taking the bottle, and then the breast. The day we were able to take your last cannula out. And your ng tube. When you moved from intensive care to special care. When we were took off the ecg cables, and I could cuddle you without being tethered to a machine. I’ve cried so many happy tears at each tiny win you’ve made. And oh baby, you’ve made so many these last two weeks. You’re a completely different baby.

Daddy and I have also been celebrating your general baby-ness. Your beautifully chubby thighs, and funny little poses. Your attempts to find your thumb. We laugh so hard at the bottom toots you make, and the pooping. Oh man, you’re a champion pooper. Youre also a comedian with the poop. Waiting until Daddy has just changed your nappy, just fixing it up and you poop. Not just once, but twice. Your timing is impeccable, and we laughed so so hard.  You like to be sung to, often quieting down for a moment to listen before you resume crying. You’re lyig next to me as I type this, and oh, you are snoring your little baby snores.

You also often get hiccups and goodness, do you hate them. With a passion, they always always upset you. It’s funny to see (less funny when it makes you vomit). And oh, your little hulk rages. You go from zero to dialled up past 11 furious in the space of two seconds. I think it’s something the NICU has trained you into, and it’s hard to tell your cues. We’ll get better at it though. Sometimes I can head it off with the dummy, or some singing and walking. Sometimes you just rage, with epic screaming (Kui calls it singing. Kui has never dealt with your rages though, I doubt she would call it singing if she had!). You’ll often hit me in the face with a flailing limb or headbutt me as I’m trying to offer quiet reassurance. But even in your rages I want to high five you. There was a time when you were limp and silent, so baby, if you want to rage you go right ahead.

I know we have an uphill battle ahead, but you are so loved. Our little family will get through it. I can’t even explain how much love there is for you. We have a village around us. We keep hearing stories about how friends of friends are praying for you. Church groups all over the world. And friends who aren’t into prayer, I’ve been told, are sending you visualisations and strength. It’s been revitalising, to know we have the support in our little corner. 

The best news is soon we (hopefully!) will get to take you home. That first horrid week taking you home was an unlikely possibility, so to be taking your sweet self out of the hospital, oh little man. We’re very excited. I can’t wait.

Arohanui my little man, more than you know.

Mum x