Tag

Special Needs Life

Nov 12
1

On Stat monitors and o2 levels

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There are two little red numbers on the stat monitor, and I’m staring at them, willing them to go up. 92. 92. 92. 92.

92 is too low. I know the guidelines. He’s got to stay above 94 on 1L or less. I check the tank (is oxygen even coming out of this thing? Is there even any oxygen in there? But the needles on green, when I pull out the tube and crank it up I can hear the o2 rush out).

92. 92. Maybe he just needs a minute. I’ve put him down and he’s just fallen asleep. Asleep is when we need to pay attention, when he’s not moving about or working as hard. 92. 92. 92. 92.

Is the stat monitor probe even on correctly? I peel back the blanket to look at Kai’s chubby toes. He’s still, and the little red light is steady and exactly where I left it. It’s not the monitor. 92. 92.

I put the blanket back and reposition the mask slightly, bothering Mikaere in his sleep.

92. 92. 92.

Do I call our nurses? Not yet. I already know they’ll say if he can’t stay about 94 we have to go back into hospital. I don’t want to go onto the ward. I will, if I have to, but I don’t want to. He’ll catch something else there for sure, and I don’t fancy living in a half metre gap along side his hospital bed, sleeping on a plastic armchair that folds flat. With no sleep for anyone, gross showers and shitty food. No thanks.

92. 92….

Do I crank up the o2? Just to see if 1L isn’t enough?

Just as I reach over to the tank, the light flicks to 93. 94. 95. 96.

The relief is overwhelming. It’s not 92. He just needed a minute. I’m on edge, and have been the last few days. He’s back into the safe zone. I feel like I can breathe again.

We’re not in hospital yet, hey? Thank fuck for that.

Nov 09
0

The winter season

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I literally hate this time of year. Autumn back in September when school went back was hard enough, but now seems every child under the age of five has a snotty nose and a cough. And every parent, and every person that works with children. There are sick people everywhere.

To a neurotypical kid this is no big whoop. For us… it’s a huge deal.

There are so many NKH kids in hospital at the minute, fighting off what would be an everyday cold to anyone else. Their parents and medical staff working overtime to make sure they’re able to breathe, to prevent a snotty nose becoming a chest infection or even something more sinister.

For Mikaere… he’s on his third cold right now in as many weeks. Last week we were in A&E because I couldn’t wake him. He usually has a morning nap from 9:30 to around 10:30, 11.

This time he’d slept past 12. And then past 1pm.

It was a weird one. Mikaere had a snotty nose. Because of his low tone (or even possibly because of his medication) he’s not able to cough the mucus from his lungs up to his mouth, it gets about half way. Awkwardly it’s at the back of his throat and ends up dripping down over his airway.

His body, knowing this is a problem, gags. Mikaere has a super sensitive gag reflex, and this triggers a projectile vomit. Like, out his nose, completely emptying his stomach type of projectile vomit. Vomit shooting across the room kind of projectile vomit.

We suction the best we can, but we have to be careful not to over-suction least we damage his nose (going down his mouth causes a vomit every single time). For those who don’t know what suctioning is, it’s exactly like it sounds. We have a hospital grade suction machine. A bit like a tiny vacuum cleaner. We attach a flexible catheter (like a very very thin and flexible hose of a tiny vacuum cleaner) and have been trained to literally suction out the mucus Kai can’t shift himself. Usually we go down his nose. It’s traumatic for everyone, but is good for him in the long run, so we do our best.

Truth is we can’t stop all the vomiting. We might prevent one in three vomits with suction.

Those other vomits? They happen at any time. Before feeds, during feeds, after feeds. It wasn’t that he couldn’t tolerate the feed. It’s that he couldn’t tolerate the mucus.

The worst thing was that it meant Mikaere wasn’t keeping meds down. If the vomit happened within fifteen minutes of meds, we could regive them and hope he’d keep them down. But he rarely did. We couldn’t regive meds if we didn’t know how much he’d have absorbed and how much he’d vomited.

And so without his meds he became lethargic, and impossible to wake. Even with pain.

However, because he was sleeping, he wasn’t vomiting. He was positioned so his mucus would drip down the back of this throat, to his stomach, keeping his airway clear. When he was lethargic like that, we could feed him and give him meds. Which would improve his levels, so he’d wake up again and we’d be back to the mucus vomiting.

It was weird and cyclic, and we were in and out of the a&e while we tried to figure out what was going on.

That’s what happens when my kid gets a snotty nose. That’s not a chest infection or anything serious, that’s just a snotty nose and a cough.

Which is why we’re so particular about illness. About people not visiting if they’re ill. About not visiting others if we know they’re ill. About people staying home from work if they’re ill, even if you “feel okay”. If you feel okay and you pass it on to Sam, there’s a good chance Sam will pass it to Kai.

(So sidenote: people of BV, stay away from Sam when you’re ill. Don’t go into work, work from home. Please).

Illness and colds are huge right now. Hate it. This time of year can suck it.

Nov 07
0

On the isolation of the special needs life

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Over the summer my world got bigger, just for a moment. Sam had some time off, which meant that we could split caring for Kai. It meant some time for me. I worked, for a bit. Travelled some. Essentially I stepped away from the special needs life into what felt like taking back a bit of my life.

And I’m struggling to step back into the special needs world. Don’t get me wrong, I love Mikaere, with every fibre of my being and he delights me like no other. I love being his Mum.

But in the special needs life, I’m not just his Mum. I’m his nurse, and his chef and personal assistant and therapist and every moment is about monitoring and repositioning and anticipating his medical needs moment to moment. Watching the time for his meds, blends, flushes and sterilising. Repositioning him upright so he can cough, watching for those tiny moments where I can interject baby led physio, and a lot of the time not baby led Physio or OT or laser therapy. Keeping him entertained, or settling him to sleep.

It’s making myself smaller to fit into the very intense schedule of his day, managing the nurses and therapists and remembering appointments and chasing the thirty or so different things we’re currently juggling.

It was hard to leave Mikaere with Sam so I could have some time. It felt selfish. At the same time, it felt like falling into an oasis after walking months in the dessert. I got to speak to people outside of our nurses. I danced through some side hustles. People talked to me as if I was a competent adult. I didn’t have to fight anyone. I found some independence for me.

And now, making myself smaller, putting aside myself for the same same mundane, for the small moments, for the intensity of Mikaere’s day to day. I’m having a hard time with that. I find it hard to get moving, I’m leaning heavy on Sam and I’m spending too much time in the ‘what if’ daydream.

Most neurotypical Mums, they get to go work (or not, if they don’t want to). They can leave their kids with babysitters and have nights out with their partners. They can travel with their family. They can take public transport and take their kids where the crowds go. Their kids, eventually, grow to be somewhat independent. Where they can play without you sitting right there to make sure they don’t have seizures or aspirate or vomit or whatever else might go wrong.

I’m whinging, I know. The special needs life sucks so hard for everyone.

I read a book once, a fiction something a rather, where they played the champagne game. They’d open a bottle of champagne and bitch, for a moment. And then say something they were grateful for.

I’m gutted that I can’t go back to work full time, but I’m grateful we have the means so I don’t have.

I’m beyond gutted Mikaere lives a life less than the neurotypical life, but he’s here and he’s happy and content and I love him.

I’m sad that when we travel, it will always be split – Sam, Mikaere and I are unlikely to travel together internationally (boo immune vulnerabilities and planes), but I’m grateful that this summer I’ve had the chance to visit some phenomenal places, bucket list destinations.

I’m sad that our family doesn’t get to walk the neurotypical path, but I’m grateful I have a family, and we’re all well loved.

I’m sad I’m going back to my day to day with a heavy heart, but I’m grateful my day to day is still there to go back to.

Hey ho. Onwards we go.

Nov 05
0

On normalising death

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There are a few NKH kids right now who are struggling. Who are at home and ill. Who are in hospital on the ward. Who are in hospice, on what the doctors say is towards the end of their lives. It’s hard, being in a virtual community, physically so far away knowing these families are hurting, that these kids are suffering.

There are also many children who have fought their fight with NKH, who have gained their angel wings and are no longer suffering. Their families are still grieving, though. Still hurting.

And I say all these things, but I don’t have words for how deep the suffering is. How truly intense and horrific it is. I don’t have words for that kind of anguish.

I struggle. I don’t have words and I think this is all coming out wrong – how do I talk about this? How do I talk about the tears and the emotions just below the surface? How I’m constantly grappling with how I feel, with grief. Is it a relief for all the children who have passed? Will it be a relief for the children who are suffering right now, considering how much pain and hurt there has been in their lives? Is there relief for their families? Will there one day be relief for us?

Because that’s the thing, I can’t comprehend Mikaere dying. I can’t. But he will, one day, because NKH is terminal.  I don’t want death to visit us. I don’t want death to visit any of the families that are waiting for it right now. I don’t want the curtain of grief to envelop any of our NKH families. I just don’t.

And I feel powerless to help. The families who children have passed, the families that are struggling.  I don’t feel I can bring comfort to them. I don’t know how to support them. I send messages to let them know we’re thinking of them (and I am, all day every day) but it feels… inconsequential. I feel powerless to help our little family, and the endless waiting and fear for the terminal end.

There have been a few times we’ve waited for the news. A handful of times we’ve sat, anxiously waiting for that call, to hear when another child gained their angel wings. We’d send love and thoughts and cry. We’d talk about organ failure and seizures and that respiratory arrest is more likely to happen before cardiac arrest. We talk about the dignified death bill, and slower than slow breathing rates. We’re normalising the lead up to death.

I hate that this kind of conversation is normal for us now. That we as parents fight so hard to keep our children happy and healthy, and that we’re powerless in the end to stop the pain and suffering that comes with NKH.

I hate that our days with our kids are tinged with the word terminal. That sometime in our future with Mikaere we’ll be in the same position. That we’ll, one day, be watching his organs fail and be witness to his pain and his suffering. To his death. Just like other NKH families are right now.

I’m heartbroken. I’m absolutely heartbroken.

I’m talking about death and dying today because November 2nd, was NKH Remembrance Day. I’m late by a few days, but I want to pause and remember our NKH kids.

I want remember Alexander, the sweetest little sausage there was. I want to to remember Kaleb, and his cheeky grin.

Gregory, and his brother Elijah who is missing him. Halle Mae. And Cathryn. Maynak. Siem.

There are actually many more children. There’s a list. A list of 124 children who have died from NKH whose names should be remembered. There’s a slide show, even. And I started reading the names… there are so many names. Too many names.

And I’m aware that these names, they’re just the ones in our NKH group. They’re just the ones we know about. Other languages, other countries… they have their own groups with children who are dying before their time. They also probably don’t include the families whose babies died before they could even find support in the NKH groups.

Too many names. Too many children.

I don’t want them to be forgotten. I want to remember them, I want their families to know I remember them. To know their names, and their faces. They made marks, they made a difference. They were loved.

I held my little guy a bit closer today while I remember the others. And I think about how all of those children were so loved like I love my little guy. So loved and cherished. NKH can suck it.

 

Oct 26
0

On the first hospital visit of the season

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And just like that our little man is back on oxygen, working overtime to keep his o2 levels up (and failing). It’s the first stupid cold of the season. We had a nurse overnight and when I walked in this morning she was just getting ready to wake us – Mikaere’s o2 level was sitting at 89%. Typically it’s at 99% – 100%. 89% is LOW. Lower than I’d like, lower than anyone would like. And you could see it. My baby was struggling to breathe. His breath was fast and shallow, he had a tug and an intercostal pull. He was working overtime to breathe.

We have tanks of o2 sitting in the spare room for moments like this, and I pulled one out, grabbed an o2 mask. It’s been forever since he’s needed o2, and you can tell, because the mask is too tight on his face. He’s outgrown the paediatric baby o2 masks. Regardless, it does the job. I watch with relief as his o2 climbs back up to more normal levels, settling at 96%. In the safe zone. He relaxes a little, he doesn’t have to work as hard. Poor baby has a temperature, and we give him Calpol.

I take pause. Get dressed, say goodbye to our night nurse. Sam and I discuss whether we should go into hospital. He makes coffee and I think about calling our community nurse. His levels are fine on o2, but he hasn’t need o2 in I don’t know how long. He’s clearly got a cold of some kind. If it’s a cold, we probably could manage it just fine at home. We have o2, we have stat monitors and suction machines. He sounds a little rattly, but I’m pretty sure it’s all upper airway.

If I call our community nurse looking for reassurance, she’ll ask us to go into hospital. Go into hospital. Complex needs. Difficult case. Better safe than sorry. Go into hospital. They always tell us to go into hospital ‘just to be safe’. Everyone is scared of taking the chance to say does he really need to go right now? What are the risks? Benefits? It’s all go into hospital, and go now. The risks of being wrong is too high.

But the hospital isn’t safe for us. Mikaere could very well catch something else from the hospital. Something worse. Plus, it’s loud and tiring and disrupts our routine, and I’d really like NOT to spend our Saturday in the A&E.

In saying that… he hasn’t needed o2 in forever.

I make the call anyway, knowing she’ll send us in and alert the paeds registrar for us. Also, on a Saturday morning at 8am, none of the other kids have had a chance to hurt themselves yet. Their parents will just be waking up and they’ll take longer to decide to take their sick kids into the A&E. If we left in the next 15 minutes, we’d make it in just after the shift change when the paediatric A&E is quiet. I mean, if you’re going to go in, might as well time it for non-peak time, right?

Sure enough, our community nurse said to go in.  So off we go. We’re on our way into hospital. 🙁

Oct 24
1

On yoga and safe spaces to share

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I’ve started going to yoga. There’s a lady who does it in our local park for a fiver. When it’s warm enough, we go and we stretch and move and practise our yoga. I am not very good, but there is no judgement and my body feels better after. So off I go. Today there was a reading. I missed the beginning, I was in my own head.

But I caught the last bit, and it basically came down to don’t be afraid to feel what you’re feeling. Be vulnerable, explore your emotions.

I tend to bite down on my emotions. My logical, intellectual side is more mature than my emotional intelligence for sure. I usually only express those emotions in safe places, like therapy, or at home with Sam. Not in public. Definitely not in yoga.

But as our instructor urged us, I cautiously settled my mind, cleared my thoughts and gently took a peek. And then I slammed those feelings way way back into a tiny box and pushed it as far away from my brain as possible, bringing my thoughts back to the present immediately. The feel of the mat, where my body was grounded, the (more ragged than gentle) breath in and out. What I could hear. I thought frantically of what I was going to eat for lunch that day.

Fuck exploring those feelings at yoga.

There was only two of us, that day at yoga. And we chatted at the end. The reading came up, and the other girl said she really enjoyed it. It was helpful for her. She was feeling some residual conflict with an unbalanced friendship and was able to gently process her way through and let it go. I listened, fascinated. How amazing, genuinely, to have that be what needs processing. How healthy. I was jealous. Sure enough, the conversation turned to me, and what I thought of being vulnerable and open to feeling what I’m feeling.

I said it was difficult, because when I explored my feelings that morning what I got back was ‘please don’t die, please don’t die, please don’t die’ with such hope and desperation and love, it was overwhelming. My very rational fear of my son dying and it being a very real possibility is always right there just under the surface, it’s intense and overwhelming.

I had tears streaming down my face, and they were both taken back a bit. They clearly weren’t expecting this. They don’t know me very well, and they don’t really know me as the lady who has a son with a terminal metabolic disorder.

And then we did that dance. The not quite pity dance, but the ‘theres a definite need to comfort me but they don’t quite know what to say’ dance. The poor girl with the friendships felt that her problems weren’t problems (but they are, my problems don’t take away from anyone else’s problems) and they expressed how important it was that I look after myself and practise self care and how amazing and strong I am for parenting like I do.

I moved the conversation on to less emotional ground because that dance is awkward for everyone, despite the kindest of intentions (they really are the nicest of people).

Side note: if you’re at a loss for what to say, say “that sounds really hard. How are you feeling about that?” or if you don’t want to go deep and meaningful, “That sounds really hard. How is your son doing right now?” because chances are he’s fine and it gives me a chance to move the conversation to the positive.

We left shortly after, and my grief lingered all day. Long story short, yoga is not the place to explore all those emotions if you’re a special needs parent with a child who as a terminal disorder.

I’m grateful we have access to therapy and safe spaces to share. I definitely won’t be exploring all those feels in yoga again, that’s for sure.

Oct 22
1

On feeding into the world of pretend

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I talk a lot about the ‘what if’ daydream in my head.  The ‘what if Mikaere never had NKH’ daydream.  I think goes hand in hand with grief, with loss. When you grieve something has happened that you didn’t want to, the flip side is that there was another option, another path, another something that you expected/hoped for/wanted. For us, obviously, it’s that Mikaere didn’t have NKH. That he was neurotypical. That he didn’t have two little missense mutations in one tiny gene.

Daydreaming about what it would be like if Mikaere was neurotypical is a dangerous, dangerous past time. It’s like a sink hole, so I shy away from it. At the same time, I also want to confront what Mikaere is not – I want to rip that band aid off so that it doesn’t hurt so much the next time I’m faced with the gap. So that the developmental gap that’s widening by the day isn’t so unexpectedly large that it’s crippling.

So I straddle the line between hanging out with kids that are Mikaere’s age (thanks to our wonderful NCT friends and their gorgeous babes), the grief that he’s not developmentally where they are and imagining what it would be like.

I’ve noticed recently that when I’m having a hard day, when we have back to back appointments or Mikaere is projectile vomiting everything or the seizures are uncontrollable – I have a bad habit of leaning towards the daydream. If I’m writing posts, I’ll post the photo where Mikaere looks more neurotypical. That if you weren’t here behind the camera you wouldn’t even know. I like the photos where Mikaere looks neurotypical best.

There’s one photo I love. Friends of ours have a son that was born on the same day as Mikaere and we went to visit. I lay Mikaere down on the floor where he’s most comfortable and T roamed, as a toddler who has independent mobility is want to do. But there was a moment where T lay down next to Mikaere and they both laughed.

I love that photo. I love that T lay down and was the sweetest little guy. That he wanted to do what Mikaere was doing, with zero knowledge of disability or difference or anything. I’ll forever love T for that tiny tiny moment.

But I realised I love that photo because it looks like, just for a minute, what a neurotypical life could be like with Mikaere. It feeds into the world of pretend, if you weren’t there – it looks like two boys playing together. The reality is they’re not. T had a wonderful, kind moment with Mikaere before he toddled off and Mikaere had a moment of awareness that T was there, but the truth is they didn’t play together.

It’s insane how to the very depth of all I am I wish they had been. How much I wish Mikaere was neurotypical. He’s not, I know. But I struggle with the cognitive dissonance of knowing he’s not, and the emotional intensity with which I wish it was otherwise.

It’s been almost two years of this cyclic grief. Over and over and over again I battle with this. Over and over again I’m faced with such grief that Mikaere suffers. And when he’s not suffering, when he’s happy and content and smiling at us and making small gains – that his life will never be as full or as varied as a neurotypical life.

That he will never love romantically, to know those butterfly feelings when you meet someone. He’ll never know what it’s like to travel independently, to delight in discovering a new place with new people living differently to you. He’ll never know what it’s like to work hard and be considered an expert at something, he’ll never know the satisfaction of when your peers recognise something you’ve achieved. He’ll never know what it’s like to snowboard, to pick a line, to make fresh tracks down a powder black run on a bluebird day (Its been a long time since I’ve been to the snow, but it remains one of the greatest joys I’ve ever had). He’ll never know what it’s like to kite surf, or boogie board or cook an amazing meal for the glory of it. He’ll never sit in a side restaurant in Japan eating the best oyster of his life, or jump off the back of boat into crystal clear waters in Croatia. He’ll never be able to introduce someone he loves to the things he loves. He’ll never adventure with a group of friends, getting up to mischief. He’ll never feel the satisfaction of creating something others can’t.

These are my life highlights. I’m devastated the highlights of his life will be smaller than mine. And I know that these are all just the highlights, and he’ll also never experience the downsides of a broken heart or the culture shock of being somewhere out of your depth or break a wrist taking a jump with a bad landing. But fuck, aren’t the highlights worth it?

And the hard thing, the thing I really struggle with – this grief is cyclic. It’s never ending. For as long as Mikaere lives – and past that, I suspect for as long as I live this is something I’m going to carry. This big dark grief that my child will never have the opportunities I did, that he’ll not live a live as full as others and he’ll forever be disabled.

I know for certain it’s this grief that pushes me to fundraise. That pushes me to figure out the next thing I can do to raise more money for NKH Research. That this big, dark stupid grief fires that ‘do something about it’ tick I have. And so I push and I design and I build websites and read research papers and oh god, the whole time I’m furiously wishing my son wasn’t disabled.

He is though. So onwards we go.

PS – if you want to donate, as always, we’re fundraising at justgiving.com/team-mikaere. All funds go to Prof. Nick Greene, who is researching a cure for NKH.

Oct 17
0

On standing

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We’ve started a new exercise at our Monday Morning playgroup – standing. I don’t mean the standing that happens in the stander but proper standing, on his legs – not strapped in. It takes two people to support him and he’s always leaning against something (because head and core and legs is too much to think in one go) but you GUYS!

His feet are on the floor! His legs are straight (either with the help of us or gaiters) and he’s upright!! I’m glad this is a thing we’re working on. It feels like a step forwards. Its delightful to see how tall (!) Mikaere is, and it’s not hard to take that leap of imagination and think on what things could have been like if (there’s always that if and it’s a dangerous path to spend too much time on, but I let myself daydream for a moment or two).

I don’t know if we’ll ever get to unsupported standing, Mikaere needs a lot of encouragement (read: hands on his knees or leg gaiters and lots of cheering and big fusses) to straighten his legs. We currently have a floor baby – that’s his happy place, lying down on the floor. Where he can kick and wriggle and have some movement – the floor is where he does it. We always set up a safe space for him to wriggle, lugging about mats and baby blankets for him to lie on.

The idea that the world might be more accessible to an upright Mikaere, that standing is one of the (many) requirements of walking… standing is one of those gateway milestones for the hopes of a special needs Mama, obviously.

He’s not standing unsupported by any way, shape or form, but for a five minutes on a Monday morning Mikaere stands outside his standing frame. He’s standing, weight moving through his hips and legs and little wriggly feet. I’ll take it, absolutely.

Oct 15
0

On a hospice stay

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We’ve been working up to a hospice stay, overnight for ages. Technically Mikaere probably could stay overnight without us and be just fine. Probably. However my mama heart is NOT okay with him staying over alone at hospice. I’m just not. I have the fear, that fear of what if something happened and we weren’t there?! The guilt would be unbearable – the world of living with a terminal disorder is a bit shit. So I have the fear and we haven’t done an overnight at hospice since we left in March last year.

In kind of the same vein, I can also count on one hand the number of times I’ve been out with Sam without Mikaere. Date nights in are only so beneficial when you lived an adventurous always-out before-baby life. The mental refresh that comes with being out of the house is huge. Even more so when it’s without the mental strain of anticipating all the requirements that come with caring for a complex needs kid.

We’ve been seeing a therapist who is attached to our hospice (because the special needs life is hard, yo) and this is one of things we talk about frequently. For a hospice stay to be helpful we, as parents have to be relaxed and trust the people caring for our child. The idea of being relaxed while my baby is not safe with either me or Sam is a contradiction. So we worked on trusting the hospice. We go regularly for swims and visit their preschool program. We enjoy the hospice at home visits and like the nurses.

You guys, our hospice is GOOD.

And so, after months and months of talking about it and visits and reassurance we booked in a one night stay. Kai would stay downstairs with the nurses, Sam and I would be up in a parents flat upstairs. It would be on a weeknight, so things are less busy and Sam and I would go to out to dinner in the town nearby. There was a preschool group the morning off, so went out early for that which was excellent. Mikaere fell in love with the man who played the ukelele. He enjoyed a fab time running his hands and feet through raspberry jelly (seriously, messy sensory play is so fun), we had a swim and he fell asleep for his afternoon nap pretty content.

The overnight part of the trip had a wobbly start. The handover which was meant to be at 3 wasn’t until 4:30, which meant that essentially I was looking after Mikaere right up until after his 6pm feeds and meds (so zero respite, considering I’d done all the hard work) but after handover finally finished, I kissed my baby goodbye and left with Sam.

Dinner was lovely, spending time with Sam was without a doubt the highlight of the whole trip. I’d missed being just us. But I wasn’t super settled. I shook that off as nerves and figured that’s just how I was going to be the whole night, so leaned in.

When we returned Mikaere was fine, sleeping like a champ in the nurses station with the nurses. (He sleeps where people can see him, because silent seizures are a bitch)

Everything went down hill the next morning. I got up early, showered and dressed for breakfast (and to check in on my boy). He was sleeping, fine as you can be. The problem is when I started doing handover. Packing up all his gear (which was all over the place). I knew we’d need a blend done – we weren’t going to make it home before his feed and meds were due. But I’m not allowed to blend in the kitchen (parents aren’t allowed in the kitchen, no exceptions) and the there wasn’t enough room in the milk room. I was directed to the coffee station – a small tiny kitchen with a bench big enough for making coffee. I wish I’d had the foresight then to say fuck no, but I didn’t. I trusted the nurses and off I went, blender and food in hand.

Fuck me. Every second person that walked by wanted a coffee. Some were polite and waited. Most weren’t, most were reaching over the top of the blender or across Kai’s food as I was prepping. There must have been at least twenty people trying to get around me, one after the other. They were very British (Oh excuse me, Sorry, if I could just, hope you don’t mind) but this was my kids food and strangers reaching over me constantly getting the way made me furious and stressed. Just fucking wait for your damn coffee!! I was raging. I’m raging just thinking about it.

By the time the blend was done I was ready to scream, I was well upset. Fuck the hospice, if I can’t blend in the kitchen (because parents aren’t allowed in the kitchen – which is the stupidest policy, considering I would have been supervised by the chef, or fuck, if the CHEF could have blended his feed that would have been even better) and the place I’ve been directed to is the coffee corner – fuck off. Never again.

That’s not the worst of it, though. What happened next makes me want to never go back. When I went to go check out the meds, we found two boxes were MISSING. Considering we had to sign each box in and it was kept in a locked cupboard there were alarm bells. I wasn’t too worried, because what was lost was a supplement and one was a med we rarely use, but if it had been one of Mikaere’s main meds which are notoriously difficult to order and get in (they’re on special order from our hospital only. One is shipped in from the states for us especially) I would have been livid. What were they going to do for the next med round?

Then I found out the nurse on that morning hadn’t followed the care plan in terms of his breakfast. Wtf. She just decided it wasn’t for her and so didn’t feed Mikaere the food that had been set out by both me and his dietician. WTF doesn’t even cover it. It was CLEARLY in the care plan, and the care plan is not optional. It’s not fucking optional! I’m grateful it was just the food they decided to ignore, because there could have been disastrous results if they’d chosen not to monitor him overnight or given him the wrong meds (or not given him meds at all). Fuck me.

I was already stressed af and this was the breaking point. Done. There is no trust anymore. I’m done. No more overnights at hospice. We have night nurses, we have support during the day. We don’t need overnights at hospice. They’re not us, the building is not home, meds are being lost and careplans aren’t followed and strangers are reaching over as Kai’s food is being prepped. Too many things went wrong on a single overnight stay, too many things went wrong when I let strangers take care of Mikaere. This may be their jobs, but this is our child. Too many mistakes happened for me to be comfortable doing it again.

I’m not saying hospice respite is terrible. I think hospice is awesome for those special needs kids who don’t need constant monitoring, who don’t have immune vulnerabilities and who are able to sleep in their own beds the whole night unattended without their parents wondering if their baby will still be alive the next morning.That is who I think hospice respite serves best.

That is not us. So. No more hospice overnights. I don’t want to repeat that experience again. Despite the wonderfulness of spending time with Sam, it was not worth the risk.

Instead we’ll ask our night nurses to babysit for a few hours and go to dinner somewhere local, somewhere less than five minutes away. Not yet, I’m still reeling from the hospice respite, but one day soon. Maybe. We’ll see.

Oct 12
0

On the developmental quotient

By | #teammikaere | No Comments

One of the things newly diagnosed NKH parents worry about is severity. There is a range: Severe is at one end. Severe means that neither mutation is producing any protein that can be used by the body. Any protein that is made is probably unstable and falls apart to be recycled. Severe is what we’re primed for – disabled, seizures, developmental delay, early death. No walking, no talking, not eating. Perhaps smiling – a developmental milestone usually hit in the first few weeks – that’s the total brain development we’re told to expect of a severe child.

At the other end, there’s attenuated – that’s a whole different kettle of fish. Some children you wouldn’t even know have NKH. They’re walking and talking and going to school and are picture perfect (I met a boy who had very mild NKH in March. I literally couldn’t stop staring. I wanted to hug him, but as a stranger to this teenage boy, I refrained). It steps down in degree’s from there. Walking and running (or sometimes non-mobile) and/or talking up a storm (or non-verbal) and/or eating (or bolus only). There are a lot of other issues, too. ADHD, severe behavioural tantrums, autistic like behaviours, hyperactivity movement disorders (dystonia, chorea, ataxia).

As I understand it, it’s a huge range split across three categories: Attenuated Poor. Attenuated Intermediate. Attenuated Mild. You can’t tell which category is which until the child is much older (around 2), and they’re showing signs of development. The rate at which they develop – thats what defines which category you’re lumped in.

What this means first and foremost is that at the time of diagnosis (typically a few weeks after birth) it’s impossible to tell severe or attenuated, unless your child has two of the handful of mutations which have previously been expressed to show how much protein they produce. Regardless of this almost every parent is told their child is severe. If a doctor see’s your child as sleepy and lethargic (even if it’s just a bad day, or post seizure) they’ll lean towards severe. Severe is where we’re primed to be, and severe is what we fear in that newly diagnosed haze. Severe is the safe worst case scenario.

Thing is, it isn’t until our babes start developing that the severity of NKH comes to light.

There’s a slide in a talk done by one the NKH researchers that spits it out:

Attenuated Mild: Developmental Quotient 50-80
Attenuated Intermediate: Developmental Quotient 20-50
Attenuated Poor: Developmental Quotient <20

The Developmental Quotient is a score – it’s the developmental age divided by the chronological age. As the parent of a developmentally delayed child – that’s a scary score. In my ignorance, I know Mikaere’s delayed, but I don’t know *how* delayed. Very seems nice ambiguous score. I know he’s not even close to his peers, and he’s been passed by babies a year and a half younger than him, but there’s nothing concrete to latch on to.

And that was fine until in Boston I spoke to researcher, showed him a few videos of Mikaere and after a pause, he said Mikaere was unlikely to be severe, he was showing milestones that perhaps put him in the attenuated poor category. This same researcher had seen Mikaere a year before and had said without a doubt he was severe at his current presentation.

Attenuated Poor. That shocked me. What would attenuated poor mean for us? Would it mean more time with Mikaere? What would our future look like? Will I (dare I even hope) need to be looking at support and facilities and special needs schools? (Will we make that it that long?) – what kind of support will we need? Will we make it to the world of special needs vans and hoists and support with a teenage Mikaere (Can you even IMAGINE?!) Attenuated Poor threw me off my severe, live in the short term kind of mentality. What would attenuated poor mean for us as a family?

How do we make quality of life decisions for our family with such uncertainty in our future? You can see the kind of emotional mess my brain went to with the words ‘attenuated poor’.

But I’m also kind of a logical person, so my first port of call was can we confirm that Mikaere is, or isn’t right now, on the attenuated poor scale? Which is where that Developmental Quotient came in. It would tell me. There is a standardised test and a score and NUMBERS. I never wanted to know how delayed Mikaere was before and now it was all I could do to not do the test myself.

In the studies that discuss NKH Severity, there are three scales of development that could be used: the Bayley Scales of Infant Development, Mullen Scales of Early Learning, or Wechsler Scale of Intelligence. The Bayley Scales is the typical standardised measurement in the UK, and our physio was trained in assessment. So off we went. She came and Mikaere was assessed.

It’s heartbreaking to look at the long list of milestones by age and know your baby can’t do the majority of them. What a knock. Still, I cheered for everything Mikaere could do. We marked off some milestones, which is positive (he’s on the scale, you know?) and just – we got through. I had to remind myself that MIkaere hadn’t changed. Mikaere was still his happy little self and he couldn’t do anymore or any less than he was yesterday. He’s still just fine on his own little path.

Deep breath in. Slow breath out.

We got through the assessment and I put it out of my mind until the assessment report came.

Cognitive: Age equivalent: 3 months.
Language Receptive: Age equivalent: 3 months 10 days.
Language Expressive: Age equivalent: 6 months.
Motor Fine: Age equivalent: 2 months
Motor Gross: Age equivalent: <16 days

Mikaere was 21 months when we did the assessment with an average development of about 3 months. Oh my heart.

DQ Final Score: 3/21= 0.14 or 14%

But there we go. The numbers are pretty clear. Mikaere’s solidly in the Attenuated Poor Category.