2 to 1... Nearly there!

It's been a while, but here's where our Orlaith is at...

At Gt Nanna's house

So for the past couple of weeks, we have been trialling Orlaith on just one injection a day (how frickin' scary?!). After the success of cutting down from 4 to 3 a day, we gradually went down to 2. So now, she has one daily injection of Octreotide therapy - a minuscule 0.05 mg - at 8am; which helps to stabilise her in the mornings, with the Sirolimus treatment, which is still twice a day at 2.2ml at 11am and 11pm.  So far, this is working really well and I'm pleased that she has come so far since she's been home - Orlaith previously had been on 0.28mg 4 times a day and I'm rather surprised it has happened this quickly! Her current BM range varies week to week but recently has been around 2.9-13.3 mmol/L, but only on a few occasions below the 3.5 mmol/L safety guideline and the lows have had explanations (e.g a vomit, is unwell and reluctant to eat etc) whereas before we had nothing to go on. All in all, she's doing really well at the moment!

                               Medicine....

Orlaith & her Daddy!

Ideally, there will be no more injections soon but at the moment, I'm hesitant to stop them altogether simply because that injection a day is is a back up if Orlaith's body is relying elsewhere for glucose. One of the downsides of the Sirolimus treatment is that it weakens and compromises the immune system, so Orlaith has been constantly unwell for around 10 weeks now. It started with a severe ear infection in both ears and has snowballed to various colds and infections. We never seem to actually get rid of any illness before it flares back up again and she currently has a bad chesty cough and phlegm on the chest, so because of this she's been on various medications and antibiotics; some increase blood sugar if there are any traces of sugar - for example calpol (unless it's sugar free). So because some tend to increase the blood sugar, it makes it tricky to figure out whether she's naturally becoming more stable - therefore not needing the injection, indicating it can be cut out completely - or the medications are keeping it raised and without it Orlaith can't hold the stable blood sugar by herself - therefore needing the one injection and to carry on as it is - so until Orlaith is better, it really isn't worth the risk of stopping it and then if something goes wrong to take backward steps and go back to 2, then 3 or 4 if necessary and so on. I'm happy with where she is at the moment, she's safe and with continued support from the specialist team at GOSH, we're going to stick to this for a while. We also had an admission at GOSH for three days back in September, it was just the usual BM profile/fast/blood work - which all went well, it was also her shortest admission yet! It was slightly harder than usual, as obviously we had Emila to think about as well as still recovering from the caesarean and the complications, but thankfully my parents helped out massively so she was looked after during the day by them then myself and their dad took it in turns being with Orlaith and staying with her overnight whilst the other goes home for Emila... hard work with the constant train journeys but we made it work.

Development...

With Orlaith's development, she fills me with pride every single day. She loves to count (although has a tendency to miss the number 3 out haha) and knows quite a few colours, she's just always chatting and running around, wanting to know what everything is and how it works. It amazes me how she speaks and every single day, she's stringing more and more sentences together. Orlaith's also very friendly and loves to interact with others, especially children... I can't wait for her to start nursery next year as she will love the interaction and social aspects! Potty training is coming along slow and steady, she's had a few hiccups previously so gradually we reintroduce it and back away if feel we need to and let her take it at her own pace. To think we were told she would be braindead by the age of one/two or have little to no brain activity, leaving her dependent and practically a vegetable for life... how wrong the doctors were!

 Food...

Definitely loves her food!

Orlaith loves her food, it's hard to think that she even has a blood sugar problem! If she had her way, Orlaith would be constantly munching on yoghurts, cucumber, chips, bananas and toast! Unfortunately, she's still heavily reliant on milk and is still unable to have cows milk so is still on the SMA high energy prescription milk - she currently has about 3-4 bottles, sometimes less, in a 24 hour period - this is purely to keep her stable especially overnight when she's dream fed. If her BM is good overnight then she doesn't need the milk so we do have some good nights like that. It's also great on occasions like today where she's lost her appetite from being unwell but will happily take milk so thankfully her blood sugar is not a worry.

Meeting Peppa Pig recently!

Likes...

This girl is also massively obsessed with Minnie Mouse, so much so that everything she could want or have has to be Minnie Mouse... such a diva! After watching the movies not that long ago, she's also pretty fond of Tinkerbell along with Peppa Pig (surely the bain of every parents existence is Peppa Pig?!), who she recently met at the local town Christmas event and loved it. Orlaith loves to sing and dance, if anyone can talk someone into 'row, row, row your boat' then it's her! She's so nosey too, always inquisative, wanting to know everything, adventurous and loves to play; but she can be a right madam and have the devil in her some days so she certainly keeps me on my toes but overall she's such a character. Orlaith's also the best big sister! The love she has for her sister is just beautiful, she loves her so much and loves to help us with her or smother her in kisses and squeeze her so hard for cuddles.


Christmas...
This Christmas is her second christmas but the first Christmas were she is starting to embrace traditions such as Father Christmas, the Christmas tree, presents etc which get her excited and she doesn't stop talking it. My parents have taught her 'HO, HO, HO!' so inevitably she's always saying it whenever Father Christmas appears, so she's getting really into it...including dismantling their tree and taking the decorations down every time she visits!!! I'm so excited for Christmas this year, last year she was only 8 months old so it was a long day where she wasn't particularly interested and buying for her was extremely hard but this year, I know it will be the complete opposite especially now we have Mila around!

"You can't play with that..."

"you can't play with that..."

 

Translating

I get emails sometimes to send translated blogs to various readers in other languages... I now have a translation bar. How exciting! This is best used via a desktop computer and can be located on the right, just under my profile picture.

Happy Reading!

Vomit, ew!

My goodness, someone - whether your child has CHI or not - tell me that I am not the only one?

Orlaith's new thing is making herself sick, like literally coughing and gagging until she successfully vomits over everything and everywhere. It tends to be when she isn't getting her own way or just wants attention. 

For example, I got her out of the bath recently and she didn't want to put her pyjamas on so what do you do in those situations? Make yourself sick, obviously. It was completely unnecessary, she could have simply of said no and gone down the verbal tantrum route but instead she just starts screaming uncontrollably and gagging, leading to the vomiting episodes. Orlaith's also done this in the middle of Starbucks whilst on a coffee date with friends, she then had to sit there in a vest and socks until her Dad came to the rescue with spare clothes and to take her home. Not at all embarrassing, right?! Another time, she didn't want to go out but unfortunately us parents haven't got a choice but to get the errands done - so again it was the screaming and vomiting scene... all over herself, the car, the car seat and then me. It happens at least once a day. Give me strength! 

I sometimes just laugh as it's so ridiculous and I look like at her like "REALLY?!" and then on other occasions, I just want to look myself in a cupboard and not be 'Mummy' anymore. I've heard, which I suppose is also the most logical, the best thing to do is ignore and divert their attention elsewhere. I  just want this to stop like right now, this phase will be the end of me - the washing machine is constantly on, I'm constantly scrubbing/shampooing/steaming the carpets and upholstery and she's constantly in the bath.... like everything is CONSTANT. 

Of course, then there is the matter of the HI and needing to keep blood sugars up which inevitably drop when she is vomiting. 

Has anyone else gone through this? I've had a ponder on google and it seems to be a popular phase for the majority of toddlers?! Let me know your toddlers irritating phases!