Tag

Special Needs Life

Aug 06
1

On drama with buttons

By | #teammikaere | No Comments

Ahhhh. Fuck. I’ve talked before about how Mikaere’s button is in the perfect position for him to reach when his elbows are supported on the floor (so anytime he’s lying supine). He has pulled it out numerous times, and even worse – the balloon has burst and it’s fallen out.  Womp.

As a bit of a back history: this is what a button looks like. Essentially, there’s a feeding port with a balloon that holds it within Mikaere’s stomach. You deflate the balloon when you put it in, and once in you inflate the balloon to hold it in place. These buttons cost about £400 a pop and should last 5-6 months.

 

In the last 3 weeks Mikaere has had three (to the tune of £1200. W.T.F). I literally don’t understand why they’re not lasting.  When I was trying to explain to our nurse what was going on, it was very confusing. In the end I had to write up a breakdown just to get my head around it:

– 14th – Button got pulled out in the carseat fully inflated. (Button A). Went into A&E for a replacement, which we put in (Button B)
– 21st – Mikaere pulled out his button fully inflated (Button B). I put Button B back in.
– 22nd – Picked up a replacement button (Button C) for just in case
– 1st – Mikaere pulled out his button (Button B) fully inflated. I put Button C in.
– 3rd – Button C had a leak and fell out. I sterilised Button B and put it in.
– 3rd – Button B also had a leak and fell out. I sterilised Button A and put it in, and taped it so it couldn’t come out.

Just in case it was hard to follow:

Button A – Currently in. Inserted twice.
Button B – leaked. Inserted 3 times.
Button C – leaked. Inserted once.

I’m getting really good at putting the button back in, but YOU GUYS – it’s always a moment of panic. It’s so terrible. First it’s because someone notices that Kai’s onsie is wet, and there’s a wet spot on his onsie right over his belly button (which is a weird place for a onsie to be wet). Whoever is nearest will jank up the onsie to check, and sure enough, the button is out and vomit is leaking out of his stoma.

It’s a bit of a race to get something – anything – into the stoma to prevent it from closing. We currently use leftover NG tubes. We have so many and they’re thin and easy to get in and tape. The concern is that if the stoma closes then 1) we’re back to the NG for meds and feeds (boo!) and 2) he’d need surgery again to put another button in. Considering the huge risk of general anaesthetic and the emotional upheaval of the first round, I’m not keen.

Once we have an ng in the stoma we can relax for a moment and figure out next steps. Sometimes that’s going into A&E if we don’t have a replacement button on hand. Sometimes it’s sterilising the existing button, letting it cool and putting that one back in. If we do have an unopened, sterile replacement we’ll usually use that.

But oh, putting it back in. It’s not as bad as the NG, but it’s not fun. Because typically Mikaere pulls out the button fully inflated, his stoma is sore and a bit tender. When I attempt to push a new one back in (deflated, obvs), Mikaere screams and clenches his abdominal muscles, essentially stopping me from pushing it through. I have to wait until he takes a breath in – when he takes a breath in his lungs expand and his abdominal muscles relax for a millisecond. Listening to your baby scream because of something you’re doing is horrid. It’s heartbreaking and I hate it. But it’s necessary, so necessary, so on we go. Eventually it’ll be in and we aspirate stomach contents up to make sure it’s in the right place (testing with a PH strip).

Having to do this 5 times in the past three weeks has definitely upped the daily stress factor of our lives. The most current button is physically taped to his body, so he’d need to get through several layers of hyperfix to the button out. We don’t have a spare at the minute, so I’m really really hoping he doesn’t pull it out. I don’t fancy a trip to A&E for another one.

We’ll see. Hopefully things will get better soon. Fingers crossed this one will stay in longer than a few days!

Aug 01
1

On small wins

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Mikaere’s on two antiepileptics and a supplement to help with his seizures: Zonisamide, Phenobarbital and Omega 3 (with all of it’s fantastic DHA seizure support capabilities). Now, it turns out that phenobarbital is one of the most frequent drugs used to treat neonatal seizures, and considering Mikaere is palliative it seemed like a good choice when nothing else was working.
 
But here’s the thing, long-term use of Phenobarbital has enough studies to suggest it has a terrible effect on a developing nervous system, it has a detrimental developmental effect too (with developmental quotient declines (in both cognitive and motor skill) which are thought to reflect a slowed neurological growth rate) and causes an increase in the probability of a cerebral palsy outcome.
 
Whats more, side effects include a decreased level on consciousness (it’s essentially a sedative) and it’s addictive, causing withdrawal symptoms when weaning. Aaaaand it would make Mikaere vomit. If it wasn’t diluted and given really really slowly he would vomit immediately.
 
Also, it turns out phenobarbital is used in the lethal injection of death row inmates and also prescribed to terminally ill patients to allow them to end their life through physician-assisted suicide. Always nice to know we’ve been giving this drug to our baby twice a day for last 14 months.
 
A good 9 months ago now Mikaere’s seizures started becoming a bit more, dare I say it, under control? Mikaere started having a handful of seizure free days. At first, it was sporadic, one day here, one day there. It was amazing, so amazing to see. The fewer seizures he had, the more gains he was making. Eventually, he worked himself up to two or three day stretches. I was ecstatic, to say the least.
 
It was at this point we discussed weaning with our palliative care team. There wasn’t much harm, they said. As long as we went slowly. If there was an increase in seizures we could put the dose up, no problem. So we started a very very slow wean. Every three or four weeks, we’d reduce the dose by 2mg (which is about 0.2ml – a tiny tiny amount). We’d wait, and watch. We already track Mikaere’s seizures and I’d hold my breath waiting to see if there would be more seizures. Sometimes there would be if he had a cold or was having a tough time. Sometimes you wouldn’t have noticed the dose had dropped.
 
It felt tricky, weaning. We eventually got to a sub-therapeutic dose. We high fived that day. And today? Today, after months and months and months of tiny, incremental weans – we’ve managed to get Mikaere off phenobarbital.
 
It’s been amazing. He’s been more awake and aware, making more developmental gains. I have seen an increase in seizures if the Omega 3 is a bit old (and has oxidised) or his Zonisamide dose is later than it should be, but generally, as long as we’re on the ball he’s a happy little guy who is still having some seizure days.
 
I’m stoked to have Mikaere off one of the medications. Phenobarbital is still on our list of emergency meds, but I’m delighted we’re no longer giving it on a twice-daily basis.
 
Celebrating small wins, woohoo!
Jul 28
2

On cancelling

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Ahhh. Before Mikaere I couldn’t understand people who flaked. People who would agree to a time and date and event and just not show or would make excuses or whatever – I really struggled to empathise. As a person, I might be late but I prioritise getting there. If I say I’m going to be there, I am.

Today Mikaere and I were set to see a friend and her sweet 6 month old baby. It had taken us month to pin down a date and it was going to be in a lovely pub with a beer garden and the weather forecast was set to perfect. Except that just before 7am we were woken by our night nurse. Mikaere had a temperature. His chest was junky. He had a cold.

Womp. Wooooooooooooommmmppp. The offshoot of this is the more seizures he has as his body attempts to fight it off. Which has the delightful second effect of more vomits as he can’t handle the secretions when seizing. So much vomit.

I hate this. Was it because we were at the hospital yesterday? Because we had friends with kids over for dinner? Was it the small boy who made a beeline for Mikaere’s buggy and touched him before anyone could stop him? Was it someone at Sam’s work? Was it a stranger while we were on one of our walks?  Was it because I’ve been a bit more lax with the sterilising? That I just washed his spoons in soapy water and didn’t run them through the steriliser? I did say recently that it would be nice to build up Mikaere’s immune system a bit more, do less sterilising, have him hang out with more kids…

I’d forgotten how hard colds are Mikaere. So I take that all back, fuck building an immune system if it’s going to be so difficult for Mikaere to fight through.

I hate this. I hate that he’s sick. Hate that we’re constantly cancelling (I’m so, so sorry to everyone we’ve cancelled on, and I’m so grateful for your understanding and grace). I hate that my boy struggles so. Mostly I hate NKH. Positively loathe it. (So much so that I put it on a t-shirt and I wear it frequently).

Hopefully this cold will roll on by in the next day or two, but we’re battening down the hatches till it’s on it’s way. Send us immune-boasting-get-better thoughts.

Jul 13
1

On The NKH Car Sticker

By | #teammikaere | One Comment

Did you know the ‘Baby on Board’ sign isn’t so that other drivers will drive safer around your car? It’s so that if you’re in a car accident, the emergency team know there is a baby in the car and to adjust their priorities appropriately.

I’ve been thinking about that for a while, not sure how to help in that situation. We carry emergency medication and emergency files with us everywhere we go. But if we were in a car accident and I was unable to advocate for Mikaere… what would we do then? Mikaere is on six hourly medications – or what if he was in so much pain he started seizing? There are a bunch of medications he’s not able to have – most which are first line seizure medications.

What would happen if I weren’t able to advocate for him?  It scares me.

So I’ve been looking at medical alert seatbelt things.

Then another NHK Mum posted a car window sticker (thanks Amber!). It was pretty brilliant, so I altered it slightly and got more printed. Now on our car window there are stickers to let emergency teams know specifically about Mikaere. We haven’t got the seatbelt alert yet, but at least there’s a sign in the window to indicate something’s wrong. I’m positive that if worst came to worst, he’d be taken care of the best he could.

PS – NKH family I have extras. If you want some, £1 each + p&p. I’ll send them to you! All proceeds go to Joseph’s Goal + NKH Research.

Jul 11
1

On Bar-b-kai 2: The Meat Returns

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After the wonderfulness of the Joseph’s Goal Ball, we packed up the next morning and high tailed it back to Bedforshire. Penny and Tony were throwing the second Bar-b-Kai, a BBQ fundraiser. They threw one last year, in aid of Team Mikaere, Joseph’s Goal and NKH Research. It was our very first fundraiser and it set the tone for our attitudes towards fundraising.

I’ll forever be grateful for Penny and Tony for showing us how easy it could be, how insanely possible fundraising was. We’re so lucky, genuinely, to be surrounded by people who love Mikaere, who are willing to throw fundraisers for research. Last year, that first BBQ showed us that we could fundraise. That we could raise money for research, that we could, without a doubt, make a difference. It was possible, it was easy and we could do it.

So when they were throwing a second BBQ? We were going to be there. So we drove straight from Wigan down, about a six hour journey with comfort breaks for Mikaere and arrived just in time. YOU GUYS! There was a ridiculous amount of meat (cooked to perfection, and some kind of delicious butternut chickpea curry concotion with I inhaled thirds of), there was a super fun raffle and the wine/water game was addictive success. A special shout out to The Chocolate Deli in Worcester, who donated a beautiful chocolate hive which went up for raffle. It was very oh la!

We ate, we drank, we caught up and everyone loved on Kaikai. It was a lovely, lovely night. I’m so glad we were able to be there. In total they raised a ridiculous £1,200, which honestly – it makes me teary to think about how generous everyone was. Thank you, again, to Penny and Tony – you both have been so gracious to our little family.

Loved it. Charity BBQ’s are the best.

Jul 09
1

On finally putting on weight!

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Woohooo!!!!! Thank fuck – Mikaere is gaining weight again. It was scary to watch his weight fall off, the beautiful chub of his cheeks disappear, his little arms and legs get more defined. That with every vomit I knew he was losing calories, and I watched at our weekly weigh in as the numbers went down and down and down. He got to under the 20% percentile – which isn’t a huge deal. He looked healthy (everyone kept saying he he was growing up, but it was because he lost all his baby weight!) and was still alert and aware, still active. There was a reason he was vomiting (#borderlineGastroRegret) and so we were reassured by our team that it was okay.

But as a mama, it was NOT okay. There was always the fear from our dietician that if he couldn’t get his weight up a ‘nutritionally complete’ formula was in Mikaere’s future, and as someone who hates the idea of processed anything (particularly when some of the ingredients are high in glycine aaand its stored in plastic bottles – so terrible!) I went on the offensive.

The first trick was getting the vomits down – we reduced his feeds from 4 meals with three snacks to four meals period, increasing the calories and the feed time, reducing the volume. We got to three hours on/three hours off. We hadn’t been on this schedule since we were on end of life care in hospice.

We moved away from regular formula to the blended diet where I painstakingly worked out the calories of each blend, going for high calorie low volume (he eats a lot of avocado, peanut butter, coconut oil and coconut yoghurt). It’s hard work and I have spreadsheets coming out my ears but you know what? It WORKED!

Because my baby is putting on weight. There is something beautiful about all that hard work and extra effort paying off in tangible numbers. Something beautiful that is mostly epic amounts of relief.

It’s even more likely that his was putting on weight had little to do with me and more because Mikaere very gradually stopped vomiting as frequently. For every feed he kept down he gave himself a better chance of putting on weight. That’s not to say the vomits have stopped, because they haven’t. We’re still at at least a vomit a day. Sometimes too. But against the 4-5 vomits we were having? I’ll take that improvement. In fact, even yesterday he had his first vomit free day in forever, so that’s positive!

I feel like I’m on high. When he reaches the 50th percentile, I’ll reduce the calories to a more appropriate level and start adding in more nutrition. We’re not there yet but that his weight is going up? I’m pleased. So so pleased.

 

Jul 04
1

On getting a new carseat

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Mikaere has outgrown his baby carseat. His torso is too long and his shoulders are too wide. But because he’s so floppy there are few things we need: it needs to recline, it needs lateral support. We’d also ideally like a rear facing, swivel seat that fits in the front seat.

We went up to Milton Keynes and met the special needs lady at the In Centre Car Safety place. There were exactly two seats that might work for Mikaere, and only one was in stock. Womp. We wanted to wait for the second to come in before we committed. So we waited and waited, and Mikaere grew longer and longer and eventually his shoulders just plain wouldn’t fit.

A few weeks after, we happened to be at our friends place, who also has a special needs child. They offered us their old car seat which Max has grown out of. A Britax Dualfix Römer. It swivelled, it was rear facing and it reclined. We knew the (crash free) history. Amazing. We were grateful for their generosity (because we’d at this point be getting a charity grant for a new seat that may or may not work. Either that or we’d very gratefully fall on the generosity of our family).

So we fitted it and off we went. Now, it’s not perfect. Because of the angle of our car seats (boo racer seats) Mikaere is uncomfortably sat almost leaning forward when rear facing, even when the seat is reclined (when it’s not reclined he topples forward). So we swivel it around to forward facing, which works better (but I cringe on the inside, I know how much worse it is to be in an accident with a forward facing car seat. I’ve seen that gif and I hate it).

Also, there’s no lateral support. There’s room to grow, for sure, but in the meantime it means he kind of crinkles in on himself. It means we use Ellie Ears to support his sides and a trunki to support his head, and it doesn’t work well with the helmet so we pull that off every time he goes into the car. It’s a bit of a faff. You can see how rubbish Mikaere’s posture is when he’s in it:

Don’t get me wrong, it’s a hundred times better than being in a too small car seat, and a thousand times better than some of the other car seats we’ve tried. We’re grateful (beyond grateful) to even have the seat we do. It’s just not the ideal carseat. It’s about 80% awesome.

When we’re in the world of trying to provide enough postural support to fend off the future risk of scoliosis, I have the fear. 80% awesome is about 15% less than I’d like.

The last option we’re looking at is the 2018 Kilppan Kiss 2 – the car seat we’re STILL waiting to come in.  It’s been hailed as the all supportive, all singing and dancing chair that might be appropriate for Mikaere. But we don’t want to buy it outright (at £450 a pop) over the internet in case it isn’t inappropriate. So, we’re waiting for it to be in stock at the In Car Safety Centre for us to try (they’re the UK stockists).  Except we’ve been waiting almost 9 months now. So frustrating. Another 6-8 weeks, they told us. It could be 6-8 weeks. It’s more likely to be another 6 months.

Do you know what else? It blows my mind that there isn’t a car seat service, or place that caters specifically for car seats for special needs children who require extra support. It blows my mind. Frustrating af.  Even if there was just in insert that would bump up the supportiveness of our current car seat, that would be excellent. Blah that that service doesn’t exist.

In the mean time we try to make it work, we do the best we can. I try not to get down on the idea that this something that neurotypical parents don’t have to worry about it – but I can’t help put feel they have an easier job it in regards to car seats. Hey ho. I’m trying really hard not to be too bitter. I guess I should practice grace? We have carseat that swivels. Its safe for Mikaere, and we were so lucky to have such generous friends….

It is all those things, and I am grateful for all those things, but I still feel very blah about carseats that aren’t fully supportive. Womp. The special needs life is already difficult, so things like this which require lots of money, some puzzling and lots of time calling places and talking to people add’s difficulty to an already complex life. Blaaaah is how I feel about that added difficulty. Still, onwards we go.

Ps – our OT did some investigating and there is a charity that will do an hour long assessment to help you fit a car seat… for the heavily discounted cost of £150.  Wooomp. And that’s just for the assessment! That doesn’t include I’m sure any travel times, fitting costs or extra whats-its that we’re going to need. Did I mention that we’re a one salary family now because I had to give up my job to care for Mikaere?! How are are meant to afford all this extra stuff?

Jun 11
1

On the Van Hove Appeal

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I’m not sure how to write about this. Prof. Van Hove’s research lab is set to close at the end of June due to funding issues.

This man has spent more than 15 years in NKH research. It’s his research that laid the foundation for the other NKH teams. He discovered, defined and proved the difference between variant NKH + classic NKH, solving the mystery of why some kids present so so differently and don’t have mutation in the NKH genes. He proved that taking DXM + SB earlier in life has a positive effect on outcome in his sibling study. He’s set to prove that NKH causes growth issues in the brain (as opposed to formation issues) which is hugely exciting (and the opposite to what most metabolic consultants thing).

He’s the doctor our metabolic consultants ask for help. He’s the person the NKH Research teams want to pick the brain of. Notre Dame worked with him in the beginning of their research group, inviting him to speak to their students. Prof. Nick Greene from UCL has said openly, several times that there is no person with more NKH knowledge than Prof. Van Hove.

He has also, over the years, amassed a metabolic network that is second to none. When speaking to Nick, he said that every time he makes a new contact in the metabolic community about NKH, they already know and are fond of Prof. Van Hove.

He’s worked hard, a lifetimes worth of work, with his sole focus being NKH. Which is odd, considering neither the Notre Dame team or the UCL team are focused on NKH (Notre Dame’s main focus is Malaria. UCL is at least closer with Neural Tube Defects, which share mutations in the GLDC gene).

I can’t believe he’s closing, and when I heard, my initial reaction was ‘what can I do?’

It turns out, quite a lot. Not alone, of course. Both NKH Crusaders and Joseph’s Goal were already moving to help – they’ve both set aside a small grant for Van Hove. It’s not enough to cover his costs till the next grant comes in (due Feb 2019). But, if they can get it matched from University of Colorado, that would put Van Hove short only $20k. If we were also able to get matching, then we’d need to supply $10k.

$10,000. That feels like a lot of zeros.

But here’s the thing – if we can’t raise the full amount to keep Van Hove going till the next grant comes in, there is no point. We’ll end up in the same situation again a few months down the road. If Van Hove closes before the grant comes in, then any money we send is essentially wasted.

That’s the fear of NKH Crusaders and Joseph’s Goal. If we don’t get enough to cover the entire year, if the University of Colorado is not able to match what we raise, then those extra funds will go to researchers not on the brink of closing.

So – it’s all or nothing. If we, as a community, can raise $10k, we can save Van Hove’s lab from closing. That’s it. $10,000 (well, actually only $9550 now, because we donated the entirety of our allocated donation savings to get things going). If I rationalise it into more manageable chunks, 191 families would need to donate $50 each to make that much. Now, there are almost 500 members of the NKH Q&A Community, so I’d need approximately 40% of them to donate.

Seems doable, right? So, we’re asking that each NKH family donate $50. Not to share on facebook, not to ask their friends or family (who have no doubt been asked time and time again for money) but us. This is our community. This is our researcher.

Now, not everyone has $50 to spare – I know. It’s a big ask to pull $50 out of your pocket. But you know what? As a fundraising goal, it’s really low.  Keep any eye out for a few more posts on how to fundraise later in the week.

Here is what I want to say – I want to keep the most knowledgable NKH man in NKH research. I don’t want Van Hove to close his lab. So, will you help? Will you donate $50?

US folk: https://www.facebook.com/donate/312802479252342/

UK/EU folk: https://mydonate.bt.com/fundraisers/vanhoveappeal

Help me turn the grid blue. For more information: http://fundnkhcure.com/

Jun 06
1

On meeting Arthur

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There are a few NKH family support groups. A worldwide one, a UK one and one where you can ask questions and the community will answer. Depending on how Mikaere is and how much time I have dictates how active I am in them. But I’m active enough to know the regulars. These NKH community groups have been a lifeline to share my fears and delights and to help answer questions in terms of research or care or medication or how to deal with feeds or our medical team. They are an unparalleled line of support.  I think it’s one of the benefits of having a diagnosis, is that you can find others who live the same life you do. Who have the same fears and have had to make the same choices.

There is one family we’ve met who are just a delight. We’ve been talking for months and months and a few weeks ago we happened to be in their neck of the woods. So we went by for a visit. It makes such a difference meeting people who get it, who live the same life you do.

Arthur is the sweetest little guy you could ever meet. It’s hard to tell from the photos/videos on Facebook, but he’s well more aware, active and more beautiful in person than I realised. I found it really difficult not to stare, he was just so amazing. And I know, NKH kids all present differently thanks to the hundreds of different mutations that cause it, but I couldn’t get over how amazing he was.

His Mum, Charlene was also a delight. We’ve been talking for just over a year – and honestly, meeting felt like were just continuing an ongoing conversation. It was so nice, so nice to talk to people who got it, to talk research and nurses and support. It was also so nice to do meds and feeds with someone who was also doing meds and feeds at the same time. And oh Ellie, totally fell in love when we met her. She was shy to start, but Sam won her over first and before we knew it we were bff’s (she loves Totoro more than I do, which is saying something).

I was sad to say goodbye. I was sad that we live so far from each other, but you guys! It’s just like finding family. I’m glad we’ve got people in our corner who understand. I’m hopeful we’ll see each other again soon! Fingers crossed.

PS, if you’d like to follow Arthur on facebook, you can do so over at Arthurs Adventures with NKH

Jun 04
1

On Visiting Tenbury Wells

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Last year when Mikaere was well, we took him to Tenbury Wells to introduce him to his Great Grandad. Since a whole year has gone by (!) and we were enjoying another relatively calm period, we went up again this year. It was amazing – I adore his Great Grandad (he’s great – he even tried to teach me to read music this year) and it was nice to feel like we could take Mikaere on a road trip. That we could leave home for a bit of a break and not be on edge the whole time, waiting for the untoward.

I just want to take a minute and say how huge travelling is for us. Before Mikaere both Sam and I were super into seeing the world. We had huge aspirations for travelling with our little guy and that was all put on hold when we found out about NKH. Taking him home to NZ is completely out of the question. Something like taking a road trip four hours north of London felt like a huge giant mission. We went last year I prepped like anything. We had all the emergency kit, I knew which hospital was closest to where we were staying, I knew who to talk to in the paediatric emergency department and I knew who to call if there was non-emergency blip. I did the research. We had the emergency gear and I was constantly waiting for the emergency to happen. Long story short, it didn’t.

This year, because Mikaere’s been so well I didn’t prep nearly as much. Sure, we had all the kit and our car was packed to the brim like a tetris puzzle (SO MUCH GEAR) but it was a much more relaxed trip. Mikaere’s bigger and I guess we’ve got a whole year of experience under our belts.

Even better was that the trip was delightfully uneventful. There were walks and piano playing and lingering meals and a lot of fantastic family time. Our little guy is so loved, it’s really reassuring to see so many people love him so openly. I also feel like four generations is a pretty special thing, so I’m glad we went. It was also really nice to have the extra arms, which meant that both Sam and I got a chance to sit down together (!) and drink hot beverages when they’re still hot (!) and generally relax a little bit.

I often wonder how much of the complexity and stress of travelling is just down to my fears about the worst case scenario, and I wonder how much of that I’ve been primed for by our medical team and labels like ‘terminal’ and ‘acute deterioration.’ True, I’d rather have all the kit and plans and not need them rather than the opposite, but I think my mindset around travelling and being away from the safety of home has been sculpted by the fears of our medical team.

The truth is going to Tenbury Wells was positively delightful. I’m glad we went. I’m hoping that the more practiced we are at leaving home, that one day we’ll eventually be able to holiday somewhere that is outside the safety of family.