April 2015

Wow, its been how long?!

Okay, so I've been terrible these past few months for updating this blog! Apologies for the silence; classic scenario of busy mum and HI child! Life is crazy busy at the moment, I can't keep up. So, I think it's about time to update the CHI family, or just the general readers who like to keep up with us...

Orlaith is doing okay at the moment - unwell and the HI is playing up, but she's her usual happy, charismatic self. Medication wise, we are still on Sirolimus twice a day and the daily injection. We actively worked for so long to decrease the injections, from 4 a day to 1 a day but we may have to increase back to 2 a day, but I'll go more into that later on. The Sirolimus is still working well with Orlaith's BM levels and so far it is still in therapeutic range, but the downside of the suppressed immune system is still apparent as she's suffered with illnesses and infections continuously since around November time and various things have landed her in hospital. Orlaith's had chronic ear infection consistently in the same ear and has been on antibiotics for what seems like forever, but the infection is now in the middle ear and we've changed to ear drops which seem to be working great. The past few weeks she has suffered with a strange rash all over her body and up until last week, no medication had worked but now it's seems to be a possible fungal rash and this particular cream which we are now trying is doing good and it seems to be clearing up. 

The mucus on the chest hasn't eased and you may remember the sickness post a while back - I thought it was a phase, you know the classic 'lets throw a tantrum and make ourselves sick and embarrass mum in front of everyone' routine? I stuck to my gut instinct - I still can't stress how important it is to be the voice of your children! - and after 6 long, painful months of vomiting constantly, we are on the road of solution and diagnosis. Don't get me wrong, sometimes it can be promoted if she is worked up but its gunky and full of mucus/phlegm (just want you need to read whilst eating your lunch, right?) but usually it's unexpected; we could be walking to the park, she could sneeze, be eating, even roll over in her sleep... she is always throwing up gunk by the bucket load and of course, it causes the havoc and starts the vicious circle when it comes to blood sugar levels. This is why it has resulted in horrific lows (recently she was as low as 1.4-1.7 mmol/L, so horrible to watch - I was genuinely scared for her but thankfully all picked up fine with no organ damage!) and a rush to the hospital on numerous occasions and why we may need to start the second injection at her vulnerable times to keep her stable at home. After chasing it up, our GP has started her stomach/reflux medications again gradually to see what works and what doesn't, whatever doesn't work then we'll stop and try another. I feel like maybe we should have kept the medications going, but this is just what happens and I'll take it as a learning curve. We start the first one today so hopefully it will go well and my washing loads can decrease with the lack of sicky clothes!  The past few days she has been in the low 2's and no higher than 5 along with a fever, a major cause for concern but she's got her visit to GOSH on Saturday 4th so hopefully we can touch base on her medication and see what can be done. It seems to be never ending! 

Onto the good stuff...

April is upon us which means it will very soon be Orlaith's SECOND birthday; I can't get my mind around it, how is it even possible! I swear I was in theatre just yesterday having her and in the blink of an eye she is going to be 2! I count myself very lucky that we are where we are, the road was meant to be so different but she's gone against it all and has come out such a strong, clever, beautiful little girl. In terms of her development, she is still on par for others her age, or the 'average' as others like to say. I decided to apply for a placement at pre-school for her and Orlaith got her place - at the same pre-school that myself, my siblings and my family went to and the lady who ran it when I was was a little girl is still in charge which is really lovely; they have been so accommodating with Orlaith and have gone the extra mile for her needs, I'm so happy that she can go to a good school and thrive in an environment that she loves.

Mila Moo, I see you! 

...And then theres Emila, who is now 7 months! Again, can you believe it?  She is doing brilliantly - she has two teeth currently cutting 4 top ones (major ouch!), crawls, sits up, pulls herself up and cruises the furniture and now she is trying to walk away... this usually ends up in her falling or cruising back, its as if she doesn't realise just how small she is! I have no doubts she'll be walking before she's 1 in August. Being 10.2lbs born, they do say the bigger baby does more sooner, Orlaith wasn't far behind in the milestones but I can't believe Mila seems to be doing something new everyday. Mills also loves her food, I've never known a baby like it - greedy guys usually wants the food as well as milk feed! She's definitely taking this 10 pounder thing to a whole new level!!

We recently had their pictures done, when Mila was 6.5 months (just like we had Orlaith's done at 6 months) and I must say, they are so frickin' cute!! Despite it being a small struggle getting them both to smile/pose, they came out great and I've started looking at frames and all the other boring decor stuff that us mums like to look at and buy so I can put them up! I'll post them within this blog so you can have a ganders, more than happy to show them off... even with Mila's open mouth smiles beautifully dominating every pose and picture! 

I couldn't love them enough, who needs anything else when you have your children around? They literally make my life. 

♡ ♡ ♡ ♡

Emila Coco

Orlaith Rae

Emila & Orlaith

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..."

 

Second baby so soon...

Life has been crazy busy, what with a 1 year old and an unexpected move... I sometimes I forget that I'm pregnant! It is completely safe to say that this pregnancy caught me completely off guard but I wouldn't change it for the world. Of course, with Orlaith's CHI being diffuse and from a genetic gene, the response has been mixed and I've had quite a few questions commonly asked....

I found out I was pregnant again around the end of November/beginning of December, I was around 5 weeks at the time and Andrew was away in Cannes with his work. I had felt nauseous and had the most dreadful migraines, it hadn't crossed my mind that pregnancy was a possibility but surely it couldn't be right?! I had felt like this a couple of weeks prior, during Orlaith's 2 week admission to GOSH but put it down to nerves and worry - I think I even joked that I hadn't felt that crap since I was last pregnant with Orlaith but again, it never entered my mind that it was a possibility. When the pregnancy was confirmed, I was unsure as to whether I would be able to cope with two children under two - as much as children a year and half apart is not uncommon, having a child who requires extra attention and 24 hour care is less common. We haven't slept a full night since Orlaith was born, surely we must be mentally insane to add a newborn into the mix? 

Genetically speaking, we have been told it could be a 1 in 4 chance for HI under our circumstances but the average being a 50/50 chance - it really depends on how the genes present themselves and what luck you have. This has put so many people off, especially if they have a child with HI and I believe that until you experience this condition first hand or deal with it on a daily basis as a parent or care giver then you will never truly understand - I can 100% comprehend why people go on not having any more children and why other ways are looked into... IVF via donor, adoption, fostering etc. As parents, Orlaith's dad and I said from the beginning that we would like more children if it was possible and that HI would not play a factor in our decision. Statistically speaking, as long as we have children together so its our DNA doing their business, the chances remain the same whether we have another child now, next year or in five years which is why it doesn't play a put in our decision process. Of course, as with any expecting mother, my baby's health is paramount and I want nothing more than a healthy baby but if they unfortunately have HI too then I would consider it 'lucky' that we have the knowledge we do now - we have a fantastic team looking after Orlaith, my family are supportive and I know I can count on Andrew for literally anything.

The downfall is, at this current moment in time you can't "test" for CHI in a pregnancy, so more care and monitoring has gone into this pregnancy. As many people know, Orlaith as a large ASD in her heart which thankfully at the moment hasn't worsened and surgery is a long way away, if it's still present at the age of 4/5 then keyhole surgery through the thigh will take place but if it get worse at this age then open heart surgery will need to take place. As far as we know, she was born with this and it got slightly worse after her central line was placed. This time round, I have been for fetal cardiology scans and appointments at St Thomas' Hospital in London for an a full examination of the baby's heart and luckily so far all is looking well; unfortunately it doesn't eliminate any future heart problems for definite but its relieving to know that for now all seems well. I also have consult appointments regularly along with scans every 2/4 weeks and I see the midwife more often than most to keep an eye on things. I feel completely supported by my community team, which reassuring on the non CHI side of things that all areas are being looked into. If CHI is detected then the team at GOSH will be on hand to monitor and treat. 

Our baby will be monitored in SCBU once it's born for monitoring, again the team there are amazing so I know she'll be in safe hands and it won't be as daunting the first time round.. but I hope I get the chance to take them home fairly quickly. I mentioned in a previous post, a mothers 'mutual longing' to take their baby home and I really struggled at first after having Orlaith - I relate heavily to anyone who has been in that situation of not being able to take their baby home straight away or if their bundle of joy is not as healthy as they'd like. I find that I practically throw myself at people who are or have been in a similar situation as I was with Orlaith. I struggled after Orlaith was born, not only mentally with PND but also with the fact that it didn't matter how many people tried to console you, they could never imagine what it was like. I found myself jealous of people having healthy babies and taking them home shortly after giving birth and I was racked with guilt always wondering why it was my baby. No one in my family had taken a journey similar to mine, except my Nan who unfortunately lost her daughter at the age of 2, so I found it hard to digest the practicality of having a poorly baby. So after all this, I had to question whether I could go through it all again but this time with a 16 month old. Despite all this, I'm not scared as I feel I know what I need to right now and I believe that anything else I need to know will come to me in due time.

Also in comparison to Orlaith's birth, I would like to have the 'ideal' birth that I longed for when I had her but of course, it never works out how you want it to so I would love it this time round. No theatre and 5 epidural top ups for me this time round, thanks... I also want to be able to walk and not have to be aided everywhere I go for the first few months after, practically learning to walk again! I recently had a meeting with the anesthetists to flag out any issues, discuss pain relief and to get a plan in place for when I go into labour. I have to say, I was dreading it but it turned out really well - due to my BMI (not going to lie, it's on the high side and I've worked hard to maintain my weight), it was a worry if I would be able to have pain relief, which could cause problems if theatre is needed. I want a smooth labour with as little pain relief as possible, so thankfully we were all on the same songsheet! I had my back checked and an epidural can be sighted should it be needed for local and my airwaves are also adequate if general anesthetic is required.

So in regards to questions you've asked me, I hope I've answered them. Don't let a statistic put you off, it is not worth the stress and worry; it's not healthy for you or your baby! Surround yourself with positive, supportive people and enjoy it. I have just over a month til I'm due and I couldn't be more excited and I can't wait for Orlaith to meet her baby sister! 

My little Rae of sunshine turned 1!

Our baby is 1!

So my baby hit the FIRST birthday mark on the 13th of April! The time has flown by, it seems so surreal to think that it's been a whole year since she was born. I've never known a love like it, I have never a little girl who is so loving and courageous and it makes me so proud to call her my daughter. There were times we thought we wouldn't get to this milestone so it's extra special for us... and of course, I had to spoil her rotten!

Unfortunately we had to cancel her party that we had planned for the day before her birthday, which I'm still pretty bummed about but there is always next year... and I'll make sure it's a big one! Despite this, her actual birthday was great. We had a lovely day; we spent some of the afternoon at her little friend Frankie's birthday party, everyone had a lovely time and Orlaith loved it!! Although it being Orlaith's birthday, they acknowledged every babies birthday too which was so nice of the parents, they hired soft play equipment and it was so much fun for them. We then spent the rest of the day with family; going for a walk, playing in the park with a spot of football and even adventured on the miniature trains; it was such a simple day yet so nice to spend it with our nearest and dearest in the sunshine! ☀️ Orlaith's also so close to walking now, she can confidently walk between objects and has taken quite a few steps - I can't wait for the time when she's independently walking! LOVE milestones, they always remind me of lucky I am and how clever my girl is.

Of course it wouldn't of been a M-Banks birthday without more family round in the evening for dinner, treats and plenty of birthday cake. Ah I was so excited for her birthday cake, of course with Minnie Mouse on it she loved it! This child is literally obsessed! So cute when everyone sings happy birthday to little ones and they just sit and stare as if to say 'what are you all doing?! ooooh fire, lemme touch it!'. That was definitely the case with Orlaith, I don't think she knew what was going on at all... she just wanted the cake! Along with the food, decorations and of course the attention, she had an amazing day. I thought I would be an emotional wreck, but somehow I managed to keep it all together! Thank you to everyone to helped us celebrate our girl's birthday with us, and for all her gifts too! Here's to another amazing year! In 18 weeks, she will be a big sister and I'm so excited to celebrate all the future occasions and moments with them! 

Here's to another amazing year; Happy Birthday our beautiful girl! xxxxx

Sirolimus what, Sirolimus who?

Wow, it's been well over a month... think that's the longest it's ever been?! Update time.  

So....

Orlaith recently had an admission at Great Ormond Street, surprisingly we were in and out within two weeks, so thankful!  

Orlaith was started on Sirolimus... a immunosuppressant which is more commonly used to prevent the rejection of a kidney transplant, which lowers the body's ability to fight infections and diseases and  it works but weakening the immune system to help the body accept the new organ. From my understanding, it came into the world of endocrine as patients on Sirolimus displayed higher glucose levels, so it is very new in Hyperinsulism patients but it appears to be working well in maintaining blood glucose levels. It is taken orally, very 12 hours so it's much better than injecting - Orlaith is still on Octreotide therapy every 6 hours via sub cut injections but hopefully she will be weaned off this as the Sirolimus levels in the blood reach a certain rate and gradually the Sirolimus dose will increase and the Octreotide dose will decrease, hopefully to nothing at all. It hasn't made the "medical load" any lighter as it's another medication for her to take but she's much more stable on it and it provides so much reassurance, especially overnight.

Side effects aren't particularly pleasant - there are the more common spells of sickness, headaches, possible swelling etc but on the other end of the scale is a viral brain infection which could cause disabilities or death. Luckily, we are only experiencing minor side effects which is trouble sleeping; before going in for this admission, Orlaith's sleeping pattern was fairly normal.. bed by 7/8pm latest and up 10/12 hours later but now we're lucky if she settles before midnight and is up between 10 am and noon - talk about teenage traits already! We're doing everything we can to shift the pattern back 4/5 hours but it's definitely taking it's time; we'll get there.. if trouble sleeping is all this brings us then happy days. 

With the treatment being an immunosuppressant, there is a lot to consider - for example going out can be a nightmare; if someone has a cold/infection or whatever or a friends baby isn't well then it's best to steer clear, as Orlaith hasn't got the immune system to fight it off. We have open access to F1 (Children's ward) at our local hospital anyway, which means we can skip A&E completely and just take Orlaith straight to the ward for treatment so that also provides reassurance given the circumstances. Also, Orlaith is currently up to date on vaccinations but any upcoming ones won't be possible as she will just contract the virus or whatever the vaccination is for e.g MR etc so I'm constantly having to ask people how they are before we see them or ask them not to come over if they are ill just in case ... I'm not being rude, it's just one of those things.

We visit GOSH every two weeks now for a blood test and monitoring whilst on Sirolimus and will have an admission in the new year for other things, so fingers crossed this continues to go amazingly with my little beauty.

The end of an era...

So I met with KT over the weekend and unfortunately, I am leaving Benefit/HOF. I can't thank you all enough for everything you have all done for me - you made going to work bareable on rubbish days and made me laugh when I really needed it. I am truly grateful for your ongoing support and love during the past six months.. and even during my pregnancy. Orlaith's health is more important than anything and due to the CHI, constant appointments and admissions, I can no longer commit to the role as account manager on Benefit. I have so many memories of you all, not just within the beauty department but throughout the store, with past and present employees. Although it was a difficult decision to make, it is for the best. I honestly can't thank you all enough, I'm so lucky to know each and every one of you. I hope we all remain friends and keep in touch! I'll be back as and when the time is right. ♡ you all xxxxx

The dreaded Diazoxide!

So Orlaith has been off the Diazoxide (a medicine which reduces the insulin production in the beta cells) for about 13/14 weeks now and I'm beginning to notice the lack of body hair... Finally!

I know it could take a while but it's such a horrible side effect for any baby/child/teen to go through so I'm pleased it's beginning to fall out. Hair growth is a big side effect of this medication and of course, the most dreaded too! Orlaith wasn't like a monkey or anything like that but she did sport hair on her forehead, upper and lower back and on her legs too. I know it can cause confidence issues in the older patients, especially those entering their teen years so I'm pleased Orlaith was distinguished as diazoxide-unresponsive sooner rather than later. 

Below I've put together some of the obvious forehead hair pictures from birth until now (well narrowed them down so you can see): 

Orlaith is diazoxide-unresponsive - this means pretty much that, diazoxide did not have any effect on her. On admittance to GOSH, Orlaith required up to 20% dextrose (this increased to 50% and a 30% top up from that, so effectively 80%!?) with confirmed paternal heterozygous mutation in the ABCC8 gene and her PET scan indicated diffuse congenital hyperinsulinism.

After reading up on and researching as much as I can on hyperinsulinism - I'm pretty much obsessed - here's some more information which completely makes sense to me; it's interesting to read that opposed to the normal divide of focal/diffuse in HI; a different way of looking at the condition is diazoxide-responsive and diazoxide-unresponsive. In most cases, diazoxide-responsive patients tend to be diffuse but have no known genetic cause. However, the majority of diazoxide-unresponsive patients are focal but quite a compelling amount are diffuse and a significant amount have a genetic cause. Most cases of congenital hyperinsulinism are transient - a patient is diagnosed by the amount of dextrose their bodies require. Then of course the stages of diagnosis by blood tests and PET scan if required. If patients respond to diazoxide then, from what I've experienced, they rarely go on to have the scan and can remain good levels of normoglycemia. I've also read up  that there are 7 known genes to cause this condition... it's interesting to see where Orlaith fits in and how much this makes sense of her condition.

Is your child on diazoxide? How are you finding it? 

HOME.♡

Not only are we sneaky, we've been super sneaky for the past week. 

Our coming home date was 17th July,

WE'RE HOME. 

....and have been for the past week.....

Yep, we have been home since the 17th July and it could not be more amazing. Orlaith is simply loving being home, she's settled in really well and she's kept to her routine and still sleeping through the night; even though our home is a completely new environment for her. It didn't feel right, it felt so weird and surreal driving into our street and taking her through the front door... Neighbours stopped and congratulated us as we carried 3 and a half months worth of hospital living into the house and then it hit us - we are home!! We decided not to tell anybody, we surprised those closest to us and have kept it hush ever since.... We decided to tell no one just so we had our time together first - and we're so glad we did it this way. Orlaith's medical schedule is so jam packed that to have visitors too soon would have been too much...coming home isn't as easy as what people may think; we have a medication schedule to stick to, a strict feeding routine, injections and BM's on the 3rd and 4th hour...but we wouldn't have it any other way, even if sleep is non existent. 

It still hasn't quite settled in my mind that we are home and able to live a fairly normal life with a baby, given the circumstances. I would still rather stay at home all the time in case anything goes wrong but slowly that feeling is disappearing. Orlaith had her first hypo at home on Saturday night, to 2.7 an hour before her feed was due; we followed the hypo plan and she brought herself back up again within the given time. The sense of accomplishment is over whelming, knowing that, as parents, we did that ourselves and Orlaith maintained the level and brought herself up - no medical intervention required, no breaking the bad news of "up on the fluids", no sinking feeling of knowing that home would be further away... just a feed and some gluco gel and she was stable again. Proud mumma! 

We nearly didn't come home, Orlaith had 4 major hypos during the night before we were due to come home (typical!) and just couldn't bring herself back up again, even with a feed. I literally felt gutted and I knew the news in the morning would not be what we wanted. Despite it all, I still packed everything up and tried to be positive; we were given the option to not go home and monitor Orlaith a bit longer or look into the options of her medicines and the dosage. After checking and reviewing with the big dr, the dosage was reviewed to her weight and we were allowed to go home under a new plan of 3rd and 4th hour BM's to monitor her. I know Orlaith can hold her levels which is why we made that decision - as her parents, we know her better than anyone and we knew it was just a frustrating coincidence that it was the night before we were due to leave! It's also common for new environments to cause hypos as they settle in but we've only had that one on Saturday night, phew!

One thing, I love is being able to go out if we want to. I went into the town where I work and it was nice to be able to take Orlaith with me! It also felt amazing that despite not currently being at work, the girls were all thinking of us and have constantly had us in their thoughts... although, I think Orlaith loved it just because of the attention she got!! Unfortunately we had to go shortly after arriving as Orlaith needed her medication but I can't wait to go back once we're a bit more stable. Small things like going to the doing a food shop, visiting family or going to Windsor to see a close friend.. it's all much more normal now and hopefully within time, we will be used to the meds and taking her out .... and eventually have a huge party to celebrate :D

We can't thank people enough for their support and well wishes, 

but we made it! 

HOME. ♡

Pet hates!

I've been reading recently on various blogs and outlets, peoples pet hates when it comes to having a baby or things that never bothered them but now do - I'm pretty sure I'm not alone in this but these things currently drive me crazy!!

 

Pet hates -

1) social networking

2) irritating people 

3) hospitals 

4) people debating who my child looks like

5) my new found love for 'take a break' magazine

Explained -

1) social networking 

I hate social networking for many reasons but since having a child I find myself sucked in - I always swore whilst pregnant that I would not post about my baby and a ban would be in place to stop me being that obsessive mother who constantly uploads about their child.... Well surprise surprise, I am that person! I dread to think how annoying I've become, but I really don't care - I want the world to know about how much of a beaut my daughter is and if you don't like it, do one. I now also despise Facebook and Twitter more than ever because of the 'share' option, I simply can't stand it when random people share things - especially photos and irrelevant statuses... Like who does that? It's weird. I don't know any of these people's friends and quite frankly, they could be molestering murderers for all I know. I love Instagram though... I used to love a good vain selfie or food upload but now (you guessed it) - I constantly upload pics of Orlaith... some people love the little lady though, she's getting quite a fan club! 

2) irritating people 

I keep finding people who I have never spoken to or at least not for a few years, coming out the wood work for the down low and update - I was more than aware this blog would attract the nosey ones but ever since Orlaith's birth people I hardly know have messaged me clearly just to get the gossip. Don't get me wrong, I can tell genuine support from gossip and some lovely people out there have sent me such nice messages but a "hiya, howz ya baby?" is something I won't reply to, especially if I haven't spoken to you in years FYI. Persistent people who can't take 'no' for an answer are also a pet hate; when we're home, I will have no problem with shutting the door in some faces if they can't respect an answer they were not looking for. There's also the so called friends and family which just haven't bothered with us at all - I have a fantastic memory (and I certainly remember all the times I've helped and supported others) so I hope they get the message when they come knocking on my door and no ones willing to answer. 

3) hospitals

For obvious reasons, I now can't stand hospitals. After a long traumatic, induction, labour, delivery, post birth, Orlaith's visits and times in various hospitals; it is safe to say I won't miss them at all. Staff are lovely but my god, I won't miss them coming in the room hourly with all sorts of things to do and I certainly won't miss the pull out beds, awkward shower times and  being so far from home and my family!

4) people debating who my child looks like

In case you've been living under a rock, I've had a daughter; Orlaith, who is my double. It's uncanny the resemblence and how much she looks like me, especially as a baby. However, you do get those annoying people who are like "oh doesn't she look like..." or "I think she looks like..." - no, she doesn't... Please feel free to shut up :D 

5) my new found love for 'take a break' magazine

Understandably, I have plenty of time on my hands and since being in hospitals - I'm now a fully pledged take a break addict. It all started when I picked it up one time as there was nothing else in a hospital shop and I had time to pass, since then I've been hooked on the ridiculous, trashy stories it has every week. I need help!

And rant over :) 

It seems most of us have similar pet hates.. What are yours? 

 

... I guess it wouldn't be right if I didn't list my pet loves too ♡

1) Baby daddy

I couldn't love him anymore than I do; he bugs the hell out of me all the time and I constantly want to kill him but I couldn't ask for a better father for Orlaith, they are  besotted with each other... Definitely a daddy's girl! He's also a loyal, caring person who would do anything for anyone and I'm glad I went through this with him... This won't put us off any more babies! haha ♡

2) Parents

I don't think I've ever appreciated my mum and dad more, along with Andrew's parents; they've never stopped loving, caring, supporting and forever giving. Family is such an important thing! 

3) and of course my babe, O

I've never loved anyone or anything as much as I love Orlaith. As her parents, we couldn't be more proud (or impressed!) of her and how far she has come. Everything thrown at her, she has handled it amazingly; a fierce soul just like her mumma ;-). She makes this rather shit journey totally worth it; love you crazy amounts cupcake xxxxxxx

Sunny Stroll

So I took my baby for a 20 minute walk in the sun yesterday -  just like her mama, she isn't too keen the hot weather! It was so nice to take her out though.... she's coming home very soon so I can't wait to do normal things like this all the time!

Orlaith, 13 weeks

Proud mum moments! ♡

So Orlaith rolled over for the first time yesterday! She's able to flip herself over from her side and get onto her tummy AND she's also not connected to ANYTHING(!!!!) no IV drip, no continuous feed, nothing! I don't think I have ever been able to walk around with her properly, this is something I will never take for granted. 

I'm way too proud of this little cupcake! The past week has simply been amazing for her and her progress. ♡

Bottle feeding.. It's the small things!

So my babe has been feeding by bottle during the day for the past 4 days! 😁

It's such a small thing, but its made a huge difference to her progress and I'm absolutely thrilled she doesn't need the ng tube for daytime feeds anymore! The tube is still used for her continuous feed during the night - this is where her milk is pumped continuously over the duration of the night, she has 21ml going into her system every hour and this helps her to remain stable. When she hypos now, we can give a bolus feed (to bring her sugars back up again) instead of her original hypo plan of hypo stop (glucose gel absorbed into the gums), then 10% dextrose bolus if that fails and so on; which is simply amazing. Orlaith's like a different baby now, her blood transfusion was definitely needed, too - she's back to her normal self, constantly chatting and smiling! 

Our next steps are to gradually get Orlaith to four hourly feeding as we are currnetly on two hour feeds and on the bottle during the night too. 

Go babe, go! 💜

SCBU Mummies.

In just goes to show that no matter what shitty situation you go through, there are always people in the same boat as you. Having the attitude of "MY child is sick" instead of "OUR children are sick" won't get you anywhere in a situation or environment like this. 

My first encounter with SCBU was strange; my baby was taken there and I was told I could visit her in a few hours time, I was hoping to go home that day so to have her not by my side was weird and we just didn't know what to do with ourselves. Me & the boy walked over (me in my pj's sporting the 'I've just had a baby' look, barely able to walk with drop foot and looking as rough as ever) to the baby unit; not having a clue what to do we pressed the buzzer and said we were the parents of Baby Banks. 

I literally had no clue but as we walked into the high dependency room, we saw our little cupcake all wired up and all I wanted to do was cry - I wouldn't want anyone seeing their newborn like that. We sat with Orlaith, we talked to her and just kept holding her. As I looked around, all couples were in the same situation but no one seemed to talk. 

Of course we visited everyday and sat with her all day and into early hours of the morning most nights; nurses were lovely and always supportive, but I rarely spoke to anyone except a smile here and there if you bumped into them in the corridor. It wasn't because everyone was rude, it was because we had all been thrown in this awkward situation and "how are you?" is such a stupid question in that environment. Some parents got on really well but before you had a chance to get to know them, they would get their going home date and they'd be gone. 

Orlaith made a few trips back and forth between the nursery and high dependency room; during this time, we got to know certain faces. Gradually as time went on, there were certain people you'd see every day and we would all just start chatting - finally, people that knew what you were going through and how tough it was but regular conversations brought a bit of normality into our situations. Over time, it become a set group of us and I actually began to enjoy spending my time there. 

The majority of the babies in SBCU at he time I was there were premature babies. Orlaith was only 8lbs but looked like a toddler compared to the other babies around her. We would always laugh and joke, they totally understood my humour (thank God...) and conversation would just flow. It's reassuring knowing you'll be spending your days in this unit but surrounded my mums who were going through the same thing and it was even more at ease because we got on so well and could joke about things. 

One by one they began to go home, of course I was a little bit envious but as I had gotten to know these mums and dads to a personal level; I was simply overjoyed for them - it was amazing news! We would also celebrate each others successes and support and comfort one another through the down times. 

I'm still in contact with all the mummies from the unit, I love hearing how they are getting on and what's new with them and their babies. I really hope Orlaith will grow up keeping in touch with her little friends from SCBU, I think it's an amazing foundation for a friendship. I would definitely of gone insane and started pulling my hair out if it weren't of these babes, really makes you treasure friendship more. 

SCBU feels like a lifetime ago, but I can't wait to go and see all the nurses when we're home and of course meet up with all the mums and babies - Orlaith's definitely missing her favourites! 

A spoon full of sugar makes the medicine go down, in the most delightful way!

Regardless of any medicine or side effect, my baby is the most beautiful being I've ever laid eyes on but some medicines have horrible side effects! Orlaith was put on diazoxide which is a medicine that aids the secretion of insulin in the pancreas and is mostly used on CHI babies.

Anyone who has ever met my baby or had a baby or known a baby on this medicine knows of the most horrid side effect - body hair. Most babies will develop hair on their foreheads, arms, legs, bum and back. After the medicine has stopped, the hair within a few weeks, sometimes months, will fall out... This is second nature to us now and we never even notice it; one mum who had a son with HI simply said it was just who he was and couldn't imagine him without it, well me too!

The reason I'm posting about this is some parents feel it's just their baby/child with these side effects.. when in reality most babies/children have it! Everyone says its hardly noticeable on Orlaith, but trust me..it's there! It is something minor in our eyes, treating our baby is the most important thing.. Who gives a shit what others think?

Many parents don't know how to deal with someone insulting their baby, of course you want to kill them - that's just human nature, but if we're all honest.. we have all thought about it and worried. I even have my "story" of being insulted....
One old hag/bitch from hell/c*nt even went to the extent of stealing one of Orlaith's pictures from my mother in law's facebook account and took the time to find a picture on the internet of a baby monkey in a dress, putting them side by side and captioning it 'Twins!!!' and then posted in on facebook. I mean how low can a middle aged women be? Claiming it was funny and meant to be a joke? I dont know about anyone else but a joke to me is something which brings laughter and uplifts your mood - this women is deranged and needs a good slap. Some people have mentioned Orlaith's "cute monobrow" and have jokingly called her monkey - I will rip you to pieces if you insult my baby, just putting it out there!

Orlaith's come off diazoxide now as it wasn't helping her much, she is now on Glucagon (a hormone which helps increase glucose levels) and its working really well for her - no hypo's to date - side effect of this is a rash on the face and chest and itchy skin, nothing a bit of piriton can't help with! She'll thank us one day.. but right now, she hates us! I don't think a 10 week old really knows what an itch is, must be a weird sensation for them!

Persevere people, your babies will be alright... cute monobrow and all ;) xxx

My beautiful babe!

This next steps a big one, Honey!

 

eeekkkkk so Orlaith had her PET scan on Wednesday.... Only one parent could go so moons went with her so I could sleep (total beaut, right?)
So now we're eagerly awaiting the results. This is what we've been waiting for, for 10 whole weeks.. the suspense is killing us!! Everything relies on these reports and results.. hopefully then we can have a plan on whats next.

Everything all boils down to this, not every parent gets the news they are hoping for, but for the sake of Orlaith's health we're hoping it's pretty straight forward! Anyone else's baby had a PET scan? How did you find it?
It reminds me of the MRI I had to go for after having her, a very noisy doughnut! O slept the whole way through, under sedation but we had to deprive her of sleep so it was affective enough to last for a long while. If anyone knows my daughter, you would know this is so hard haha.. like mother, like daughter...

So it's friday now, I hope we get the results today or early next week.

Congenital Hyperinsulinism (CHI) was the last thing on my mind when it came to having a baby...

Many people have not heard of CHI so I've written a quick overview using simple words, hoping this helps?! haha....

Congenital Hyperinsulinism (CHI) is a disorder where insulin is excessively secreted in the body, not being regulated by glucose appropriately - this causes hyperinsulinaemic hypoglycaemia (hypo) episodes where insulin levels are high and ketone bodies aren't being made - this is extremely dangerous for the brain and the protection of brain damage; as it means it isn't getting the fuel (glucose) or ketone bodies (which generate different fuels) it needs. A safe blood sugar level for CHI is 3.5 and above - a hypo is anything below 3.5. There are two types - focal, where a certain area of the pancreas (a lesion) is producing too much insulin, or Diffuse, where the entire pancreas is affected.

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It's a very brief summary, but here's my daughters story so far..

On Saturday 13th April @ 17.25pm; Orlaith Rae was born. ♡
I could go on and on about the feeling you come across after just having a baby but it really is the cliche feeling of feeling complete and unconditional love. All of sudden, nothing else in the world mattered except her existence.  I never thought I'd be a mother at 23, I had so much more I wanted to do in life but there I was, holding a beautiful baby girl and nothing will ever come close to it.

Orlaith was an extremely shaky and jittery baby. I assumed these were traits of hers, but it continued into her first night at the hospital, only to get worse. Orlaith would go through episodes of being floppy and in a daze to being vocal and screaming the place down, reluctant to feed. In the morning, she had routine OBs and it become clear Orlaith wasn't going home. Doctors kept coming in and observing her before telling us she needed to go to SCBU (special care baby unit) - she was admitted on a 0.9 blood sugar level - it should be above 2.6 for normal newborns. This didn't mean anything at the time but we were made aware the feeding and jittering was being caused by low blood sugar levels and she needed medical assistance - at first I was just confused, I couldn't understand a word the Doctor was saying, all I heard was him say 'antibiotics' over and over again.

Orlaith was taken to SCBU and was instantly put onto a 10% dextrose IV drip - this didn't do much and she went upto 12.5% then 15% .. She still lingered in the 0.'s, even dropping to as low as 0.2.
Due to Orlaith's veins being fragile - as with any new born - she had a IV line put into her belly button, as the dextrose percentage was too high for regular veins. Numerous canulars failed but Orlaith luckily remained stable. A few days after birth, one of the staff nurses told us they will be taking blood to check for "Hyperinsulinism" and briefly described what is was, but we didn't know the severity then. The results came back and she was on the fence, neither positive and negative. Our hospital stayed in daily contact with Great Ormond Street hospital, one of the only TWO centres in the UK which deal with CHI, to indicate what steps need to be made. Orlaith also developed severe reflux and would struggle to hold a feed down - resulting in the NG tube you see in her nose, this has been used since birth to help feed her. She's more than capable to take a bottle but with the reflux was extremely difficult (majority of CHI babies suffer with reflux and aren't keen on feeding, exactly like the day she was born).

As the weeks went on, SCBU tried numerous medicines and kept her on the IV drip constantly, as she'd only hypo without it. All together, it was THREE times we titrated the IV fluids with feed and she was able to come off the drip. However, Orlaith would only last a couple of days and she'd be back on the drip and meds were being increased. How Orlaith didn't slip into seizures, comas, or developed brain damage is beyond us. Drs indicated to us we were awaiting a transferral to Gt. Ormond Street but it could be some time until the bed was available. It got to the point where Orlaith kept dipping dangerously low and she needed a higher dextrose percentage, but she would need surgery to fit a central line as the glucose is too strong for veins and would damage them.

 Within a space of a day, I wasn't only told about surgery to fit the line- I was told she may have brain damage and a tumour on the pancreas. The next day we were transferred to Southampton hospital for surgery to fit a central line for the weekend, thankfully all was a success. We arrived back to our local hospital all fine - Orlaith was stable and the plan was to keep it that way until we could be transferred to GOSH to see the specialist team and top notch consultant. . Early hours the next morning after returning back from Southampton, Orlaith started having cardiac episodes and stopped breathing - I am forever grateful and thankful to the nurses who were on shift that night, without them we wouldn't have our daughter today. During all what was happening, bloods were taken which indicated low levels (of what, I'm not too sure!) and Orlaith had a blood transfusion which helped her remain stable. Orlaith had also had a brain scan which showed there was NO damage on the brain, overjoyed is an understatement.

By this point, Oralith was nearly 7 weeks old and we were finally being transferred to GOSH as a bed had become available. We've been here nearly 4 weeks and the team are simply amazing - coming from somewhere which had never heard of the condition, here it is like second nature. Orlaith remains on a 30% dextrose and occasionally hypo's, but here we have the ability to control it. After routine blood, her hyperinsulinism was confirmed and it indicated the CHI may be focal... This could be the best news, as hopefully it would mean surgery, recovery then home. Diffuse is more difficult to control and get rid of, but there is still a possibility of it so we're open to the outcome - Orlaith will need a PET scan to confirm the current findings. Fingers crossed it's focal and HI may well be cured!

I'm so proud of my daughter, at ten weeks old she has endured so much; she's so brave and words can't describe how much I love her. ♡♡♡