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..."

 

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!

Congenital Hyperinsulinism Awareness

www.hi-fund.org

From 4 to 3....

Wow so we had some amazing news yesterday.

We are currently on a six week break from hospitals, appointments, tests, you name it (due to second baby due any day now!), which is lovely as the travelling was becoming too much for me and my ridiculously swollen feet! I've been in touch with the GOSH team more other the phone because of this and although we aren't able to test regularly if Orlaith is in therapeutic range for her Sirolimus treatment; we are able to determine whether a dose change in the Octreotide therapy is warranted by her BM level range. Amazingly, Orlaith is doing brilliantly at the moment and we have recently had another dose decrease and last night were offered to trial 3 injections a day instead of 4 for a week, making them every 8 hours and not every 6 hours. This is just great news, it means Orlaith is more comfortable as she's at the age where she knows what is happening and literally runs a mile, and it also means we have one less thing to worry about... and we can get more sleep!!  Some may say, and it was pointed out to be a risk by the specialist nurse, that undertaking a drastic change in medication is a lot to take on whilst being pregnant and ready to pop but I welcome the pressure as we have so much confidence that this is a step in the right direction and we will make effective progress from is.

Last night was the first trial of stepping up to 8 hourly and Orlaith slept beautifully, she always sleeps through the night but she's very unsettled around injection, medicine and feed times. I think this is because we have successfully dream fed her for a year now and her stomach subconsciously knows it's empty or needs milk, causing her to be unsettled and sometimes wake, although to completely wake is very rare. Similarly, we slept like logs and got 4 hours straight which we haven't had since before she was born! I woke up feeling so refreshed! I'm so happy we have made it to this point and I hope it continues so we can get her off Octreotide therapy completely and leading a normal life as much as possible. At 16 months, I think she deserves it!

The next steps are to hopefully carry on the week long trial with success so we can continue to put a new treatment plan in place and we are also looking into changing her feeding routine too; to make it more suitable for Orlaith, her age and dietary requirements. This will include looking into amounts, measurements and timings of certain meals, as she is still heavily milk reliant - some days she has her whole intake just in prescribed milk, on top of 3 meals and snacks - we would love to not need to give her so much; how she isn't overweight and still tiny in 3/6-6/9m clothes in beyond me!! I hope that over time we can meet these steps and become closer to being mediated through diet and not treatment.

Emails!

I love getting your emails and answering any questions or interest you may have about CHI. I'm glad i have been able to help and offer support to others, especially those of you who read my blog from all over the world! Amazing! It makes me happy that I decided to share our experiences.

If you would like to email me, please feel free. I will respond as quickly as I can.

limarabanks@gmail.com

Please note I AM NOT a medical professional, I will happily answer questions based on MY PERSONAL experiences but I can not offer medical advice or a professional medical opinion. Please seek medical advice from your GP, doctor or consultant. Thank you. 

Has it really been 4 months? Wow.

I can't quite believe it's April and I have yet to post anything in 2014! I've just shifted through my emails and questions relating to this blog and thought it's time to update the land....

Orlaith is still as beautiful as ever, totally biased doesn't even cover it. I am still amazed at how far she has come and what she's doing now, so crazy to think she is 1 in 11 days, where has the time gone?!.... no longer my ickle baby! She has literally gone from strength to strength these past 11 and a half months, I don't think I've ever been more proud in all my life! Her development is on par for her age, if not better which is just amazing - I always love hearing things like this because we were told when she was a matter of weeks old that she may have development and learning difficulties, so for her to be where she needs to be it just makes me so happy! It is still a fear in the back of my mind, I'm more than aware that these difficulties can develop at any time and slight brain damage could be present but she's doing really well for now and thats what I focus on.

We are still a journey and daily battle with Hyperinsulism, not quite on the transient path...but one day! The last time I posted, it was a post on 'Sirolimus'; a new drug which has been trailed on CHI babies. Orlaith started this treatment in November last year and this treatment is also our final step before surgery, but it's working wonders so hopefully this continues. Sirolimus has been in the press recently, and was also featured on ITV news, as it is such a new drug for these purposes but it carries so much potential. As I previously mentioned, Sirolimus is a treatment which is common in kidney transplant patients to stop the body rejecting the new organ - it was during this time that it found that it raises blood glucose levels. I'm no doctor or expert so I couldn't possibly tell you how or why this was linked to CHI and to trail it when persistent neonatal hypoglycaemia is present but it's working and has saved a handful of babies... so completely worth it! There are a few concerns which are quite regularly raised with this treatment, mainly in parents of patients of the treatment - the most popular being the little to no research in paediatric cases and that the treatment not being advisable for children under the age of 13, however these are questions which consultants are dealing with and there is definitely method in the madness here! Also it bares rather unpleasant common side effects; pneumonia, blood clots and an increased danger to skin cancer... obviously not advisably what you would put a baby on but we have seen a difference, which eliminates the worry of them being unstable at home and thankfully there are so signs of any side effects with Orlaith just yet.

 If you would like to read more, here's a link to the GOSH blog about the treatment and how it helped a baby boy.

In terms of medication, Orlaith is still on Octreotide therapy every six hours and Sirolimus as mentioned every twelve hours - gradually if Sirolimus levels reach and maintain a therapeutic range, the Octreotide can be decreased and Orlaith can be weaned from it. As Sirolimus is taken orally, it would make my life if she as just solely on that and the injections could stop, she's at the age where she knows its coming and it's heartbreaking! Similarly, as parents we decided to discontinue Orlaith's gastrto medication - all three of them - sometimes parents just know best, we believed she had outgrown the condition and after the all clear to trial this at GOSH we gave it a go and haven't looked back since!

Of course, her diet is still a big thing for her and it is very strict. I am very on the ball with what she is fed, if you know me personally then you know just how strict I am.. tough love if you ask me!! Orlaith is still fed every 2-4 hours, including overnight with BM monitoring pre feed and when a hypo appear s to be present. Over time, you get to know the food groups which benefit the most but it is always a case of trail and error with new food. Personally, I can't stand it when people just feed her because it's there and she's a baby, "something to gnaw on" they say; I don't think so.  SO much goes into preparing her food and it's great when we find something which has all the right food groups and nutrition but also keeps her blood sugar stable. Food which containing sugar is not always good, which is what most people believe - these foods could bring on a hypo as opposed to a sugar rush. It's important to consider carbohydrates, fat reserves, glucose, fructose, article sweeteners etc. 

Orlaith loves her food, it's sometimes hard to think she has a blood sugar problem!

Also, couldn't carry on without mentioning my second love - I'm currently five months into my second pregnancy, more than half way there. I think thats a whole post in itself, the responses I've received are rather interesting and some are rather intrigued about the HI side of things so I think that'll be the next one. X

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 classic HI vent

So I've been ridiculously busy these past few weeks, it is almost as if everything that could go wrong has gone wrong - you name it... everything from death, to giving up work, to credit card fraud and everything inbetween. Stressed to the eyeballs doesn't cover it!

However, despite all this.. I'm always coming across the same threads, comments and rants when it comes to HI and how others think they can say whatever they like to you without taking anything into consideration. Orlaith is six and a half months old now and I'm still having to deal with annoying people and their unpleasant, irksome comments which I could totally do without! One thing I've noticed since having Orlaith, not just with myself but other parents to patients too... is the frustration with people who really have no clue, who feel they can attempt to preach to you about my child and/or hyperinsulinism. I could simply mention that I'm tired and in response I hear, "Oh welcome to parenthood!","Oh the joys of being a parent" or the most irritating of them all; the dreaded "it can't be that bad". 

I find it ridiculous that someone thinks they can tell you how to look after your child anyway, completely healthy or not, but it's beyond annoying when they feel it's 100% necessary to all of a sudden become a guru in your child's condition. I stand there in shock sometimes with the utter rubbish that these morons come out with... I mean a Dr who has studied this condition for years and years - who is also the worlds best - knows a lot more than these people yet they still go on to act and talk to me like I should be taking their advice. 

"My baby had that", "I have gestational diabetes, my baby must have it", "oh my brother in laws friend has the same, just take care of it at home", "it's just like diabetes", "natural sugars will sort that right out, just try a banana"... A BANANA! If only that was the answer.. I'm sure plenty of parents around the world would be rejoicing, let me tell you! Chances are they have never come across hyperinsulinism. When something as serious as brain damage or organ failure comes into the mix and as a parent I need to monitor and care for my baby 24 hours a day, 7 days a week to prevent it - don't sit there and tell me "it can't be that bad". I would praise Orlaith's dad and I, we never ever complain; we simply just get on with things and accept that it is what it is.

I don't think we should even get started on the lack of sleep. You would think this particular sort would have gotten the hint but no... once again they want to bang on about the joys of parenthood. You have no idea! Yes, EVERY parent will experience lack of sleep at some point and yes, some parents will experience this longer than others; BUT, being up all night (whilst your baby sleeps blissfully through the night, completely undisrupted) conclusively carrying out the 24 hour care required for your child's condition and only functioning on anything from 20 minutes to an hour here on there (if you're even thay lucky) is a completely different ballgame! Unless you have to administer medication, injections, feeding every 2-4 hours, regular glucose monitoring all throughout the night to a sleeping baby, don't even make a judgement. The same goes for during the day but people just talk to me like I do nothing but sit around and watch TV all day!

Others really need to think before they speak, I've seen this happen again and again with HI patients and their parents- we have every right to complain once in a while, don't tolerate someone telling you anything that you don't want to hear. At the end of the day, you are your childs parent; you are their voice as they can't speak for themselves just yet, you know best!

Anyway, I'm glad I got all of the above off my chest. Happy Wednesday everyone! X

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

More babies?

So much talk of babies recently - there is definitely a baby boom going on, 2013 has been full of births and pregnancies! 

It's no secret that I didn't want more babies up until recently -  I had a far from great pregnancy, labour and delivery and I certainly did not want to go through any of that again. Also with Orlaith being how she was, I definitely did not want to experience that a second time round. I would have been happy with one child.  It's no secret that I now want more babies, I've always loved babies and the older ones too, and would go as far to say I'm quite a natural with children but now I definitely would like to add to the brood. 

I think deep down it's simply because I miss being pregnant, I adored the pregnancy excuse! I definitely had my hard times whilst pregnant (what pregnant women doesn't?!) but I just loved feeling connected with my child and feeling her move around; such a surreal feeling that I wish everyone could experience at some point in their lives. I am no longer contented on having just one child, I want Orlaith to experience siblings and have that closeness of a family like I had growing up; but I am 100% contented with her, so if she is all I'll ever get then I'll die a happy lady!

Here's the hard part, although it's "a very slim" chance of having another HI baby - statistically one in four - would I willingly put myself up for that this time round, knowing exactly what could be in store? I posted an article earlier on today (living with hyperinsulinism - for those who haven't seen it), of an amazing family who have two children with hyperinsulinism; I'm actually speechless at the courage of this family, hats off to them! It really is inspiring. It's an interesting read for you HI mummies as I know it's something we all have to consider at some point in your lives. 

So who knows, maybe next year ;)

To gastro or not to gastro PART TWO ♡

Thank you so much to everyone who called/text/emailed me today to wish Orlaith well on today's GOSH call after last weeks post - we're so lucky to have support from you guys! xxxx

♡♡♡

So here it is... Orlaith's range since Wednesday as been interesting to say the least - 2.9-7.4 - with seven occasions below 3.5, ranging from 2.9-3.3 - not amazing considering the vast increase on the Octreotide but understandable as the little madam is reluctant to feed.

Do you ever get to the point where you just want to pull your hair out?  Waahhh. I literally stayed home all day today just trying to pump her with food!! 

I touched base with the specialist team

today for the update on Wednesdays apportionment - as parents we have said no to the gastrostomy UNTIL we have more information and have looked into other options too. If feeding carries on this way then it would be inevitable. It's a shame my Nan couldn't come over today, she is like a bottle feeding queen when it comes to Orlaith - she can easily get 150ml of milk in her into time, thankfully we're seeing her tomorrow... Definitely putting her on the feeds!! ;)

Sirolimus is still now an option so we'll see what her consultant suggests - we are going to be re-admitted to Rainforest ward at GOSH as soon as possible, probably in the next couple of weeks... And the journey will be again all over again in finding what keeps Orlaith stable and within normoglycaemia levels. 

♡♡♡

To Gasto or not to Gasto; that is the question.

Yesterday was Orlaith's first outpatient appointment in the endocrine clinic with Dr Hussain and his team. We were going to not only follow up Orlaith's progress since being home, but we were also going to discuss the recent short stay admission on Kingfisher at Great Ormond Street Hospital.

If I'm 100% honest, I just had this feeling that we would be hearing news - whether it be good or bad. Dealing with Orlaith on a day to day basis, solely by myself whilst her dad is at work during the weekdays; I had this instinct that it might not be what we wanted to hear.  I do all of the liasing with the HI nurses, GOSH, GP's, pharmacists, appointments, constant prescriptions.. you name it and the past few weeks haven't been Orlaith's best, I could just tell - it doesnt make it any better that there was never a day where the BM readings have been amazing since we've been home. Similarly, I was thinking that the CHI would become transient by now. I'm not too sure whether that's me thinking realistically or naively. 

Here we were, sat down like before  with the what felt like the whole world in the room.  Orlaith's not made any significant progress in her levels for the past 23 weeks, since birth; she is still producing the same figures she was back then. That explains it then, she hasnt fotten any better - we've just kept her stable. Que the sunken heart feeling...

Orlaith managed just shy of 3.5 hours on her fast (10am to 1.30am).
10am - 6.3
11am - 5.7
12noon - 4.3
1pm - 3.2 (although a BM reading should never drop below 3.5, during fasting it's 3.0)
1.30pm - 3.0
Bloods were taken at this point - this resulted in a glucose level of 2.2mmol/L, NEFA level of 0.78 mmol/L and a ß-OH-butyrate of 0.37 mmol/L - the last two are good as it shows her body is trying to react to the levels of insulin in her system, but ideally need to be higher and well the glucose level speaks for itself.

In the previous post I mentioned the gallbladder ultrasound scan... they found a "sludge" on her gallbladder, at present this is nothing serious and is caused as a side effect from the octreotide but Ursofalk (Ursodeoxycholic acid) has been prescribed to prevent any stones from forming. Orlaith's also not on par for height for her age, although I think she's rather tall (?!) but again it's a side effect from the Octreotide and nothing to worry about just yet.

The consultant's plan of action is to increase Orlaith's octreotide therapy dose up to 0.25mg from 0.20mg every six hours, if there is no improvement they were originally going to consider Sirolimus (a fairly new medication popular as a last resort in unresponsive diazoxide patients) but the chances of it taking effect are slim so on Monday I need to call and let them know how she has been on this new dose and if it doesn't make an improvement....as if all this wasn't enough to take in..we came to her feeding - now of course with everything from GORD to sleeping through the night, we struggle to get milk into Orlaith and really have to push it with her. They want to fit a Gastrostomy. 

For those who aren't familiar with what a gastrostomy is or what it does - its a opening made surgically through the abdomen into the stomach and a feeding device is fitted onto it..it will allow Orlaith to be fed directly into her stomach, having no contact with her mouth or throat.  Orlaith will more than likely have the PEG (percutaneous endoscope gastrostomy); this will be fitted via an operation under general anaesthetic and held in place by a disk in the stomach wall.

This decision from what I can gather is down to us, not only for Orlaith's welfare but our own too - as parents we can't let her suffer with insulin secretion and fits, seizures and brain damage as a constant risk; her health is paramount, nothing comes close to how much we want Orlaith healthy and safe but also as parents, we can't go on being up all hours of the day and literally functionally on 20 minutes-3 hours sleep a day, feeding her at every possible moment to keep her glucose levels up and BM's above 3.5. If she was to have a Gastrostomy fitted, and this failed a full pancreatectomy would be stage two; which is a whole new ball game of complications.

I can't describe to you in words the amount of emotion which I was carrying around with me yesterday, I knew something was about to change but I was hoping for a medicine change or something more suitable but I would never of thought a gastrostomy would come into the equation. As a parent, do I carry on taking my child's care on the chin, having poor health myself just so I can be comfortable in knowing that she hasn't had to face yet another operation and hasn't got to be bound to a machine or as parent do I give consent, knowing that her glucose levels are always up, she'll be okay overnight and also knowing I can sleep too? My heart literally breaks for this, I haven't faced this decision with her father yet, I don't want to even face it at all. I've only just got my baby home and now I need to give her up again? As selfish as it is, I don't want to give her up and I certainly don't want her having, lets face it..in laymans terms, a feeding machine. We've requested information to be sent to us, we'll go through everything before a decision is made but if it's needed, it's needed - I won't let my daughter be at risk. Of course we still have hope that Orlaith remains stable on this new dose of Octreotide; in an ideal world, this would do the trick and a gastrostomy would not need to be considered until a later date or maybe not at all, or maybe even an NG tube instead..Who knows. I guess we'll see how it goes.

♡♡♡


On a happier note, Orlaith had her first train ride from the hospital as her dad works in London so we got the Waterloo train back to Bracknell; least she loved it!

Re-admission baby!

So today (sept 9th) we have Orlaith's re-admittance at Great Ormond Street Hospital. Every so often, Orlaith will be re-admitted for monitoring, fasting, profile building and investigating...to be honest, it could not of come at a better time, as she's been up and down lately with no real explanation as to why. This stay is intended to be short stay, hopefully it stays that way! 

We are on Kingfisher ward and as Orlaith is still a high alert patient, she has her own room...with an en-suite so I'm a very happy mummy, no communal showering for us! Haha! 

Today will be spent building a BM profile, so these will be done 2 hourly to see how she is coping with glucose levels and also to get an idea of where we're at in terms of her octreotide therapy and feeds. I'm feeling pretty good about it this time round; hopefully we'll get some solutions and questions answered! Orlaith also has a gallbladder scan today to see how that's getting on too. She's gonna be a busy lady! Its so strange being back though, its almost like this is home and we've been on holiday for 7 weeks at our house!! ♡

51 Days

We brought Orlaith home 51 days ago... 1 month 20 days... 7 weeks... 1224 hours and it's gone crazy fast, I can't believe I haven't posted in 7 weeks!!

I love being home and I adore having Orlaith home. Don't get me wrong, she definitely has the devil in her some days and is a diva every day of the week but as a baby, Orlaith is doing amazingly! I swear she is getting more and more mobile every day.. constantly shuffling everywhere (crawling soon, I hope!), has her hands and mouth all over everything, trying to sit up by herself, enjoys food (pureed, of course), sleeps through the night... I could literally list a million and one things as to how she has changed, it's unbelievable! Like where has my baby gone?!! 

Of course she still has CHI, and it is an on going battle to monitor it at home. We are constantly exhausted, her medical schedule is insane... for the last few weeks, we have experienced hypoglycaemic fits regularly... struggled as parents trying to grasp the disease... and of course it wouldn't be sods law if after 4 days of being home we weren't blessed with teething... but in all seriousness, throw whatever you like at us 'cos we're still over the moon to have our beaut home!

One compliment I get a lot (besides the amount of hair she has haha!) is how happy and smiley she is - she is constantly smiling and chatting away and always in a happy mood; it's strange because you would never believe anything was actually wrong with her sometimes, or has gone through anything like she has. I love waking up and seeing her face in her own cot, not on a ward being prodded every hour or setting off her own monitors... the experience of having a baby home is still new to me but I'm loving it. We are in the 7th week of being home, for most babies this would be at the age of 7 weeks so still a fairly small teeny tiny newborn (I have 100% forgotten what that is like, Orlaith is 14lbs worth of mini human now!), I'm still getting used to so much. I'm not going to lie, I do sometimes feel cheated. Like any other pregnant first time mum; I spent a fortune on clothes and getting everything the baby would need, getting her nursery all ready and making sure it was just right, picking the perfect pram, nesting to a worrying point of scrubbing skirting boards and banisters at 3am and re decorating the whole house so everything was right... I would never in a million years of thought that I would be in hospital for 15 weeks with my child suffering from a condition I had never even heard of! Just the other day I put O in her pram carry cot and she's the exact same length as it... what happened to my baby? Of course like any other 20 week old, she loves to kick and flap so the pram is definitely no good... time to dig out the stroller attachment for sure! Waah!

So we've been up to so much since being home.. for the first few weeks it was constant appointments; I obviously haven't posted in 7 weeks so here is a brief update for you;-

We go back to GOSH for re-admission on the 9th Sept, this is a monitoring and investigating period where the hyperinsulinism and hypoglycaemia are managed by her consultants and this is when and where any changes will take place, it is also a their chance to see how Orlaith is getting on, run tests they need to and all that kinda stuff; Orlaith's GORD (gastroesophageal reflux disease) is under better control now and is throwing up rarely, keeping her feeds down now and is generally more happy at feed times now... feeds are still hit and miss, she has only just grasped the concept of food and getting the feeling of hunger but it's great that she's come this far and learnt to feed in such a short amount of time; Orlaith still has a large atrial septal defect in her heart (possibly from her hickman line), a scan last week showed a hole which is 7mm wide but there is an island which has grown in the middle of it so effectively there are 2 holes along with the atrial septal defect, there isn't any pressure on the chambers so this will just be monitored and operated on a the age of 4/5 if necessary with 2 more heart scans to follow in October; we finally got to attend O's audiology appointments and after what felt like years they finally confirmed that she is not deaf in her right ear (despite what drs etc have said!) and that fluid is build up in the eustachian tube in the middle ear and will go over time; blood counts are currently normal so no more transfusions just yet... and breathe! So much to keep on top of, but overall this little lady is doing beautifully.  

Orlaith's still has subcutaneous (injection) Octreotide therapy every 6 hours, as a long term treatment for CHI. Octreotide is not currently licensed in children with CHI, but is used if the child is unresponsive to diazoxide (check my previous posts for more on this)... She's doing really well with them, at first I thought I wouldn't be comfortable giving my baby injections but we aren't phased by it, O takes it like a trooper with a tiny whimper then it's back to smiles or sleep depending on what time of day or night it is! BM management is still every 3rd and 4th hour of the day - this is 24 hours a day like everything else, they have been up and down recently and it cant be extremely hard especially when Orlaith is asymptomatic (does not show symptoms) in a hypoglycaemic episode but we have learnt to manage it and keep on top of the monitoring. I've heard this from a couple of parents, with children also on Octreotide therapy and I have to agree...one thing I can't stand is pity... the amount of times I hear things about Orlaith's medication is beyond irritating, at the end of the day Orlaith's health is paramount to me and any other parent would do whatever is required of them to keep there baby alive and healthy and NO I don't feel bad for doing it. 

 Of course this takes its toll; I can't remember the last time I slept more than two hours at a time and I'm constantly functioning like a zombie but you just make it work, I think it's more tough as Orlaith has slept through the night since she was 8 weeks old - she doesn't even wake for feeds - which for a normal baby is amazing but cares and meds go on throughout the night so lucky for her, but unlucky for us... she also has to be fed every 3-4 hours without fail otherwise she will slip into a hypo fit, so again it's hard because she is asleep so thank God for dream feeding! We sleep when it can (we  get 5 hours spaced throughout the day which are "free", so nothing meds wise needs doing, so we sleep in this time) but I do find that people don't understand and are sometimes not happy if we are unable to make plans etc but it's just how it is, gotta do what you gotta do! I speak to a lot of parents those children, especially newborns with the congenital disease, experience  this and find it difficult - give yourselves a break! Learn to be firm and say no...at the end of the day, your child's health is more important than anything in the world so if you want to sleep or do nothing when you can, forget these people and do it! I'm not afraid to admit it but I also became a recluse when we brought Orlaith home, as well as Orlaith being sick I had my own post-birth issues... I just didn't feel comfortable in public - especially having to do meds etc outside of the house; but I'm gradually getting there and lets just say I'm not comfortable in every social occasion anymore but my advice is that the feeling of anxiety will eventually go, we just need to get on with it at present - you've just had a baby/or just found out your child has this, so cut yourselves some slack...it's not something that will digest quickly, time is definitely the winning factor here.

I really hope the other parents are now home, if not have their coming home date..in which case I hope it's SOON. I've been asked a few times if I'm on Insta or Twitter - I'm the most easiest person to find - @LimaraBanks for both - beware I am the biggest mamarazzi ever and will probably bore you to death with pictures of Orlaith. Totally sorry not sorry ;) xxxx

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. ♡

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. ♡