On the Heat

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Oh this HEAT! London is in a heatwave. We’re doing our best over here (cause increased heat usually means increased seizures) with fans and portable aircon, feets in paddling pools and mist from spray bottles and closing the curtains. It feels like we’re hunkering down, trapped inside (because outside is too hot, it’s just too hot rn). But this little guy is such a champ, he smiled through physio this morning, and is always up for a round of acoustic noise making, or smashing the space foil, or just laughing at his brother. He’s so patient and kind with us, I’m grateful.

We’re trying to make memories, but feeling like we can’t go anywhere is hard (constantly feeling like we’re not doing enough, while also knowing we’re doing the best we can. It’s not a competency thing, it’s a capacity thing). Still, I’m grateful he doesn’t seem to mind, and is happy to make memories at home.

We have some friends who are in tricky places right now, in PICUs and wards all over the country. It’s hard to see their messages, to both despair and love with them, to be overwhelmingly grateful that Mikaere is at home, and in great health and *happy* – I feels wildly unfair that others are in tricky places (while at the same time knowing we’ve been there, and are likely to be there again, at some point). So things right now are tempered, hugging my kids close, wishing I could do more, wishing my capacity was infinite (and equally knowing it’s not). Holding on tight to gratitude, with a bit of memory making on the side.

I guess that’s how this summer is going. How’s your summer shaking out??

On the days following our A&E visit

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Mikaere’s been a bit flat since our a&e visit. Many hours sleeping, but he has some active/awake time. As It’s so warm, we’re out on the balcony for portions of the day, when he’s up for it, willing the breeze to blow our way. Warm weather is always bitter sweet for us, it’s delicious but the risk of seizures increases.
So we pulled out the cool blanket, the magic mat that’s filled with gel, so it’s cool to the touch. It lasts for about 15 minutes, and then you move to a new spot. It’s worked wonders to keep Mikaere cool.
We’re still not comfortable going out into the world, and the pictures of everyone else enjoying their break at the beach or in crowds of just, out an about with a bit of normality has me feeling very green. But onwards we go, enjoying the balcony, a bit of water in a tray, paddling pool static nonsense.
Onwards we go. Managed to get some smiles, even 🙂

On visiting the A&E

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It’s not the first time we’ve had to call an ambulance this year. It doesn’t get easier. The counting of the seizures, timing them, logging the presentation. After we give the second buccal, we’re meant to call an ambulance, but I don’t want to. Not really. I don’t fancy spending hours in a&e, holding down my boy for bloods. (That’s not why, though. The why is that I don’t want this to be beginning of the end, I don’t want this to be a deterioration, but I don’t let myself think those things. Instead I think about the uncomfortable chair and the boredom and the endless waiting and the child with the disorder so rare people just ‘pop in’ to introduce themselves and say hi).
So instead I call our critical care nurse, and let her convince me. She clears the way. When they arrive, the ambulance service already has Mikaere’s directive. We’re ready and waiting, and so we head right out.
Thanks to our nurse, the paeds A&E knows to expect us, which means we go right into Majors (it’s busy. The other two bays are already full with a broken ankle and a concussion. Usually they’re empty).
Triage is quick. Seizure frequency is up and drastically different from his baseline. We’ve given emergency meds twice and the seizures are still breaking through. We’re here for a review, and to rule out any acute indications that would mean a symptom stay.
The physical review is clear. No red flags. The bloods come back clear. Infection markers are low (so no infection). Everything looks fine.
At this point we’ve been in the a&e almost seven hours. The last four have been seizure free, and as there are no acute concerns, they’ll send a message to his neurodisability consultant to follow up, and they wave us out.
Grateful to be home. Hate seizures. Hate that this is happening with more regular frequency.
Hey ho. Life with NKH, hey?

Neverending Isolation

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This guy. So, I think we’re (touch wood) out of the last seizure war and into a place of relative calm (I say relative calm because he’s not seizure free, but it’s not the 20+ episodes with weird new presentations we were seeing before). We did lots to bring him out (mainly med adjustments for weight, and opened a new bottle of possibly oxidised oil), but as always its slow and takes time to kick in, and the recovery back to baseline is heartbreakingly slow. But there have been more smiles, and more awareness and more vocalisations (this kid has opinions!)

But. We’re still in this place. 25 weeks of isolation. Today is day 175. We’re still sleep deprived (getting up at 3am is not any easier 5 months later) and I still have all the anxiety about what’s safe for us.

Here’s what we know: the risk of Mikaere getting covid19 is worst case scenario (aaand we live the palliative care life, so worst case has a different, very real meaning for us). We know that covid is still rife outside our flat. Over 1k of people in the UK tested positive YESTERDAY. Last week over 9000 people died (400+ more than the week before).

Just, not fully knowing what needs to happen for us to be able to be safe outside is really doing my head in, because the honest truth is that it may never be safe. And if it’s never safe, then it becomes a balancing act between quality of life and the possible quality of death.

And the never leaving the apartment isn’t the kind of quality of life we want for Mikaere… but, the thought of taking the risk and going out, with the very real risk he might suffer unnecessarily/die is paralysing, you know?

I don’t know how to align those two things, and I’m all over the place about it. How do we provide the best possible life for Mikaere, considering the dangers? I’m struggling so hard, and I don’t have an answer.

I suspect this is even more difficult because in our little bubble we’re sleep deprived and without a wider perspective and without all our usual support (from our teams, therapists, groups, friends). It feels like we’re trying to make safety life/death decisions in the dark.

So. What would you do? Would you go out if the risk was death?

Trying to navigate blind

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I’m wary about posting this. When there are so many things out of your control, and those same things are basically what makes your heart beat, you become way more superstitious and faith going person than before.

So I’m wary about telling you that Mikaere’s seizures have lessened because the universe might hear and laugh and send more seizures our way.

His seizures haven’t stopped, but the frequency has definitely decreased.

Because the seizures were presenting differently, because they came on so suddenly, because they were relentless, it’s been a stressful few days.

Do we take him in to a&e to be reviewed? No, because all they’ll do is take bloods. They’ll swab for an infection he doesn’t have (because we’re still in isolation, day 164 today) and we won’t get the important plasma results back for two weeks, so going in is basically a risk (cause global pandemic, with little to no benefit). And at that point, it’s on *US* to figure it out.

So we do all the things. We weigh and adjust his meds based on any weight gain, and we video what the new seizures look like (is that movement really a focal seizure? Could it be dystonia? Could it be spastic movements or chorea?)

We go down a rabbit hole of research, reaching out to people we know in the community to understand what it typically looks like, how it’s treated, when it came on for other kids.

Spoiler: not dystonia or spastic movements or chorea.

We check the use by dates on all the meds, make sure the meds are what they say they are on the label, check they’ve been stored correctly and haven’t oxidised.

We try figure out what he might have come into contact with. Is he too hot? Too cold? Is he in pain? Are any of his muscles unbearably tight? Has he lost any range of movement in his limbs that might indicate pain? Are there any rashes or spots or anything that might give an indication?

Is this NKH progression? (I hate considering this, because it means there is nothing we can fix).

And so we sit. With our anxiety, asking Mikaere to please breathe while he’s having a seizure, cuddling him close after, reassuring ourselves that he’s still here, still with us.

We’ve already made the med changes, so we need to wait and see before we make any other changes.

But it’s hard to wait, and it’s hard to just watch and there’s definitely a toll on everyone (it’s hard not to feel like a failure, when you can’t help your kid when they’re suffering).

And slowly, the time between seizures stretched out and the frequency came down and the weird focal seizures went away and the tonics we’re used to seeing took precedence.

But it’s hard to trust this lull. It’s hard not to be on full alert, hard to trust that there might be less seizures today. That we might get awake time and maybe even a smile. It’s hard to relax into this deescalation and trust it will hold, while at the same time desperately wanting it to.

The special needs life, hey? Seizures can suck it.

Seizures are awful :(

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I’m holding the sedative meds, I’m ready. Waiting. Because I know it’s coming. I know that I’m going to have to sedate my child. Again. And that if I don’t, he’ll keep having seizures. Over and over and over again.

Around 4am there was a twitch. A slight lifting of the elbow and a tense shoulder. For a *moment*. I saw it, but before I could figure out if it was a seizure he’d relaxed. And then it happened again. And over the course of an hour it happened over and over, for longer and longer until they were recognisable as seizures. I need to wait for one more, as per the seizure care plan. Three in an hour, that’s the threshold. But I know it’s coming.

You’d think I’d be used to it now, but knocking your kid out isn’t an easy thing to do. Well, that’s a lie. It isn’t hard: position him so he won’t choke, pop the lid, squeeze half between his cheek and gum on one side, the half on the other side and rub his cheeks until he crashes out. Physically it’s one of the easier cares (way easier than nasal suctioning or button changing or fighting with the Lycra suit).

Emotionally? I feel like I’ve been pummelled. Emotionally I would like to never see my kid suffer seizures again. Emotionally I wonder if this is the beginning of the end (it’s not, universe. I didn’t put that out there so you could run with it).

Because it’s a never ending fight. Trying to figure out what’s causing them, trying to get them to manageable levels so he can experience some kind of quality of life, with awake time and development and (and in my head, I want to cry, because my sons quality of life comes down to him BEING AWAKE, and not, you know, every other joy a three year old might experience).

Honestly, the weight of this hand we’ve been dealt gets harder to carry. The never ending seizure war. NKH, you’re awful and I hate you.

The correlation between heat and seizures.

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Another warm day, another day of seizures and struggle. He has both a portable ac unit and a fan pointed at him. He’s lying on the floor because it’s 1.4 degrees cooler than the couch, or a chair. I love the warmer weather, but not when it causes seizures. They’re happening every few hours. Just long enough for a recovery nap and some minor awake/aware time before another one hits. Blah.

Seizures are the thief of everything, and I hate them. They take away so much. At this point, what kind of quality of life is this? Seizing and recovering, not moving, no joy.

This is what NKH looks like. Seizures because it’s warm. The brain damage that’s happening in my little guys brain because of a tiny tiny mutation which fucks up a large chunk of his carbon folate metabolic system….

Living the super special needs life atm. We’re one of many, our story is standard, not exceptional. But that doesn’t make it any less heartbreaking 🙁

On the A&E during lockdown visit

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The thing with isolating is that safety stops at your front door. Inside: safe. Everything else: unsafe. We’ve been living that tenet for what feels like forever (funny how long 4+ months can feel).

But about a couple of months in, Mikaere started having seizures. Not just one or two, but many. The kind where it knocks him out, and as he’s waking up he has another. He was having very little awake time, losing skills and honestly, they were the kind of horrific seizures where between having the seizure and his brain shutting down, he’d literally scream before becoming unconscious. Melt your heart, panic when your heard it type scream (can I just say now I loathe NKH to the very depths of all that I am capable of feeling). We ended up sedating him, several times. We haven’t had to do that in almost a year.

The other concern was his heart rate. Usually when sleeping his heart rate is 90 or under. Fairly typical, as far as heart rates go. But when I came on shift after The Day of Awful Seizures, I watched as his heart rate went up and down, up and down while he slept. But by morning his heart rate was over 160bpm, despite him still being asleep.

His heart when he’s active and awake and happy is somewhere between 120 and 140. 160+ when sleeping with seizures indicated something was Wrong. Capital W.

Obviously being in isolation trying to figure out what to do adds complexity. Do we go into the hospital and get a review? Do we leave the safety of our flat, and take him into the A&E to get checked out?

Let’s be clear. At this point, we were mid peak. Thousands of people were dying a day. Four times that were testing positive daily. Outside our door seemed a dangerous place, and we were deeply distrustful of everyone. They could be asymptotic, they could share it and we could be facing something else. The risk felt huge.

Trying to figure out logically what was going on, I went through the list of the possible causes in my head:

Had he pooped in the last day, was he constipated?
Had he gained weight? Was his meds dosages off?
Has any of the meds changed brands/formula?
Had one of his meds oxidised and become unusable?
Was his glycine levels too high?
Or were they too low? Did he have Sodium Benzoate toxicity?
Did he have a fever, was he too hot?
Was he in pain?
Was he ill? Did he have an infection?
Was it a full moon, were the planets out of alignment (kidding, mostly. Sometimes it feels like there’s no rhyme or reason to these episodes despite me bending over backwards to figure it out)

A lot of those required blood tests and swabs to eliminate. After some in depth conversation with our CCN (community care nurse) we weighed up the risk. She called down to paeds A&E to assess the risk with what kind of patients they had on the floor and we talked to our neurodisability consult. And then we made the decision to go in. The truth is, with a heart rate that high, with having to sedate him for the first time in a long time and with an abnormally high number of seizures, he should be seen and reviewed. Just in case.

So we went. We assembled enough supplies for the day and for the first time since March, we all left the flat. Mikaere hadn’t really woken since the day before yesterday. He’d been having seizure after seizure. (I hate NKH. I hate it I hate it I hate it).

It was less risk for us to drive than take an ambulance, so that’s what we did. We faced an issue at the door, as only one parent is allowed to accompany a child at a time. We got around that later, by coming in separately to do a handover.

The paediatric waiting room was empty. The staff were careful to stay well back, and had gloves and masks. We were shown to a private triage room immediately. Sam and I were both able to stay through triage while we made a plan. Bloods, swabs and observation while we waited to see what his heart rate did.

We were eventually moved through to majors, and Sam left. I struggled. Sleep deprivation is a real thing, and I’d already missed the lunchtime nap that gets me through the day. Still, they did bloods and the swabs, and took away a urine sample. His heart rate came down, just a few beats a time. Mikaere was still sleeping, bar the seizures.

I also had a minor argument with the reg while he was taking bloods. Mikaere is hard to cannulate and never gets a cannula in on one go. He ends up with multiple bruises and scabs and it’s always awful.

The cannula they used to collect the blood wouldn’t flush (it never ever does). The reg wanted to insert a new cannula against the possibility he needed to be admitted. I argued that he may be causing my baby pain unnecessarily, that he might not be admitted and might not need the cannula. Also, that the emergency medication they’d administer was rectal paraldehyde and wouldn’t be going through a stupid cannula anyway. I insisted they stop. If he needed one later they could try later. It turned out I was right and saved my baby a tiny speck of unnecessary pain and suffering. It’s not often I can do that, so I’ll take it where I can.

While we waited, I convinced Mikaere to wake long enough poop, which was good. He wasn’t hot, not temp. He hadn’t gained any weight that would make his dosages change. We gave him a dose of calpol, just in case he *was* in pain. I was crossing off possible causes in my head. Watching as his heart rate came down a bit more.

And we settled in to wait for the blood results, my eyes constantly flicked between the sat monitor and Mikaere as we waited between seizures. Hours and hours later, the bloods came back clear. No infection (I’d hope so, we’d been in isolation for what felt like ever). Sodium, calcium, potassium all in normal range, so no indication of Sodium Benzoate toxicity. Glycine levels would come back in a week or so. Carnatine looked fine. Liver function fine.

Mikaere woke up enough to play with some toys shared (and disinfected) by the units play therapist. His heart rate was nearly in normal range, considering he was awake.

The consultant came down to have a chat with me. Essentially, he wanted Mikaere to be admitted on the ward for observation. Because we’d already given two doses of buccal midazolam, he’d hit his max 24hr quota. If Mikaere needed more rescue medication because of seizures, the next medication was rectal paraldehyde, as laid out in his seizure care plan.

The problem is that it’s not always easy to get on short notice. We didn’t have any at home (our stash expired, and it had been so long since we’d needed to use it). They had some on the ward, but wouldn’t be able to get any from the outpatient clinic.

If we left we’d be taking a risk. If he was admitted to the ward, we’d be taking a different risk.

We talked it out. Essentially I decided that between solo parenting for the entire night on the ward with sleep deprivation, Mikaere’s seizures decreasing and his heart coming down, and that we’re an 8 minute blue light from the hospital, we should go home.

The consultant argued until I asked if any of the nurses were sharing shifts across wards or working with covid positive patients. Then I asked how many kids on the ward had pseudomonas or rhinovirus or any of the other respiratory illnesses that would also be a danger to Mikaere. He shut up after that. I guess things on the ward weren’t ideal. We were towards the end of winter, so I’m not surprised.

Either way, Mikaere was discharged, with strict instructions about what to do if things got worse. Conveniently Sam had arrived and we went HOME. It was glorious to be back in the safety of our flat. After disinfecting ourselves with baths/showers/changes of clothes I went through all of Mikaere’s meds.

There’s one particular supplement he gets once a day, at lunchtime, called ubiquinol. It’s a supplement that supports cell repair and is a potent antioxidant.

However, the pharmacy had supplied ubiquinone, the oxidised form, which your liver needs to process into ubiquinol. Considering that Mikaere’s liver is already doing so much, and thanks to NKH, it’s not clear what exactly is happening in his liver, anything out of the ordinary could cause things to go sideways.

The horror of it was that because the dose required more capsules than was supplied in the packet, the pharmacist had supplied extras in a jar, and had mislabelled the jar ubiquinol and not ubiquinone (which was the drug supplied). Sam, who doesn’t usually do the 12 meds’ didn’t think anything of it. The jar had the right label on it, so it should have been the right med.

I was livid (with the pharmacy, not with Sam). Our prescription clearly states it should be ubiquinol, not ubiquinone. I kicked off a formal investigation and it turned out a locum pharmacist had made a mistake, and had thought there was no difference between the oxidised and reduced form. They didn’t have ubiquinol in stock, easier to substitute than order in. No harm interchanging them. He could have been right, in a typical patient. But let’s not make guesstimates when the patient has a rare metabolic disorder.

I was gutted, because I really like that pharmacy. They’ve been so good to us, and this was the first mistake they’d made in the three years we’ve been with them.

Luckily, the owner of the pharmacy knows us and was equally horrified. He implemented a whole range of safety checks to make sure it wouldn’t happen again (to us or anyone else) and the locum was let go. I’m sad someone had to lose their job, but I’m also frustrated that the safety, health and quality of life of my baby boy is so heavily reliant on others doing their job as they should, without guesstimates or convenience-based substitutions.

We stopped giving Mikaere ubiquinone, and the pharmacy shipped out ubiquinol quick smart.

To be safe, we also opened new bottles of meds we thought might have oxidised. Between having pooped, ubiquinol and unoxidised meds, Mikaere’s seizures eased, and we settled back into our regular routine.

Sam’s convinced it was the pooping. I’m sure it was the ubiquinone. It could have been some unknown pain/calpol. (In my head I can hear Kai’s Grandma telling us the moon was full). Either way, I’m glad we went in to get him checked out, but I’m more relieved that we all got through to the other side safe.

The special needs life is no walk in the park. Hey ho, onwards in isolation we go!

On Seizure Monitors

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Gadgets. We have them coming out our ears. One problem we have is knowing when Mikaere is having a seizure when he’s sleeping. His seizures are silent and horrid and we need to know when he’s having one so we can give relief medication if he needs. At the minute we have night nurses a few nights a week, which is helpful. They stay awake and watch Mikaere. When we don’t have night nurses, Sam and I sleep in shifts. But the problem is in the wee hours. Sam’s shift usually goes until around 2am, and typically I’m up for the day around 5, 5:30am. In those inbetween hours, it’s usually whoever is around, or whoever is the most awake.

Except, you guys, we’re SO sleep deprived. We’re special needs parents, so of course we are. But when you’re sleep deprived, we run the risk of missing something in those inbetween hours. It scares me no end. So, gadgets. We have all the gadgets.

We have an angel care mat, and while that’s a relief, it only alarms when Kai stops moving for more than twenty seconds (which is to say if he stopped breathing and was completely still for twenty seconds). But with seizures he jerks.

We tried wearables next. Specifically the neebo, as his feet are now too big for the owlet. Except the neebo wasn’t accurate enough for us. Additionally the strap left red marks on his arm.

Then we tried the RayBaby, a gift from a friend. Ray baby is a baby monitor that tracks breathing. As Kai breath holds and then hyperventilates when he’s having a seizure, we’d hoped that RayBaby could help. Except that RayBaby couldn’t distinguish between an empty crib and one with a baby in it. It’s still very very beta. Womp womp womp.

Our saving grace is the pulse oximeter my Mum got Mikaere. It’s an old Massimo Rav 5 that a client gifted as they were upgrading their kit. It has a probe that goes on Mikaere’s toe and measures his heart rate and o2 saturation levels. When he has a seizure his heart rate sky rockets and his o2 drops – we have alarms set to go off when they hit certain thresholds.

He wears it every evening when sleeping, which means we don’t need to be as hyper-vigilant as we used to, game changing when everyone is exhausted.

The only downside is he can’t wear it all day. It’s too big, and the minute he’s awake he’ll kick and wriggle and the probe comes off. Still, it’s good when he’s sleeping, which is when he’s most likely to have a seizure.

We’re also on the waiting list for a seizure movement monitor from Muir Maxwell, and Epilepsy Trust in the UK, but I’m not convinced it’ll be any better than the pulse-ox to be honest. I don’t know you guys. Seizures are awful. They’re really really awful and I’m terrified I’ll miss one and that will be it. SUDEP (Sudden Unexpected Death in Epilepsy – it’s when a person with epilepsy dies without warning and where there is no other obvious cause of death) has me incredibly fearful of falling asleep and missing something. I guess it’s a special needs parents thing, where you go to sleep but you’re not 100% sure your baby will still be alive when you wake up. The Fear, I have it.

Epilepsy parents, what do you use to monitor your child?

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!