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

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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. 

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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? 

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.

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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!

SCBU Mummies Pt. 2

Eleanor 

After waiting what felt like years, Orlaith and I were finally able to meet up with our SCBU family when we got home and it was amazing to see everyone. At first it was so surreal, seeing the Mummies and Babies in a normal environment was just awesome! I loved catching up with them all, hearing about everything that has gone on since we all last saw each other and of course, seeing those gorgeous babies! I can't believe the babies are pushing on to five to six months old now, where has the time gone? 

Frankie and Orlaith

Lucas, Thomas and Frankie

It felt like only yesterday we would spend our days at their cots, feeling like we would never get to escape. Just to know this group of mothers personally is encouraging – the love, the dedication, the support that these women have is just inspiring. Despite what they all had to go through during their time at SCBU and even now, they are still smiling! It’s heartening to see that this particular group of people, and their children have taken all what they have been through on the chin and it’s just made them even more amazing. I would not have got through those 7 weeks without them. As for the babies… Wow, just wow... I am overwhelmed with how much they have grown and become such happy souls with their own personalities. It goes without saying ladies; we produced a bunch of beauties!! There are 6 babies altogether; 3 boys, 3 girls… and yes babies listen up; I am expecting at least two of you to marry! What a wedding that would be! Roll on the next SCBU mummy and baby meet up!! ♡♥ ♡♥ 

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!! ♡

And breathe!

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So lately I've gotten more and more irritated with certain people in my life - and all this is highlighting to me is their issues they have with me - some as a mother, which of course I won't tolerate -  most of which I didn't even know about! 

Firstly, I'm the type of person to call someone out and find it extremely hard biting my tongue - if I believe I'm right, I will stand my ground and my opinion. Secondly, I'm beginning to notice more and more how sly people really are - like making underhand comments knowing full well I'll see them or saying something which is rather covert to someone mutually, knowing it will get back to me. Again this comes back to biting my tongue in order to not cause conflict or upset... Which no one should have to do.  Why not come to me yourself and express your problems? Unjust comments on social media sites is just pathetic. 

Since becoming a mother, my priorities have changed dramatically for obvious reasons so I don't have time for petty, devious behaviour. I was raised to speak my mind, whatever the outcome and I will...I'll certainly be raising my daughter to do the same and stand up for herself and to make her own judgements on people. Why live your life pretending you like something or someone when you don't, just because of the circumstances? I don't think anyone should have to put up with ridiculous behaviour and "through the grapevine" gossip... I'm certainly not anymore. 

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On a more positive, happy note... I'm spending the day with my favourite boy and girl; family day before we head off for Gt Ormond St tomorrow. :D :D

How beautiful is she? Biased I know....

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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