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.

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

Why is more not being done?

After dealing with CHI for 25 weeks now and researching it in depth... don't get me wrong, I'm no professional but it's crazy the amount of time I've spent reading up on everything I possibly can from websites, blogs, information outlets, books, you name it! I am that annoying person that will randomly start a conversation about a condition you have never even heard of and start blasting you with facts and statistics. 

One thing I keep reading about and hearing over and over again is the mistreatment of CHI and the effects this causes; and it's a total shame that by doing ONE thing, misdiagnosing would be halved and mistreatment would not have to happen. 

Call me crazy, I believe every newborn should have a mandatory glucose test via heel prick at birth. I could not preach this any louder if I tried... and trust me, it's all I do go on about when I hear of these horror stories which are gradually coming out of the woodwork and into the press. This needs attention.

But babies have a blood spot I hear mums saying... I fully appreciate the importance of the five day blood spot, but I do not think it is enough. Do you know exactly what that tests for? The majority of people I ask have no clue. This mandatory blood spot picks up on disorders such as cystic fibrosis, sickle cell, phenylketonuria, MCADD and congential hypothyroidism which are all extremely important to detect in newborns; however, this screening does NOT test glucose levels so hypoglycemia is not detected so conditions such as congenital hyperinsulinism go undetected; therefore, it isnot treated  or it is misdiagnosed  and that is when the horrible risks start to kick in. More and more babies are being misdiagnosed, not given the right treatment and some unfortunately dying due to low blood sugar levels (hypoglycemia) - It's not just the brain which can be in danger if left untreated; the heart, blood vessels, kidneys, eyes and other organs can be damaged too. For the sake of a newborn's health, if a heel prick is all it takes then surely more parents would be willing?

I have no idea how glucose testing (BM's) are dealt with anywhere else in the world but in the UK it is not compulsory - in my opinion, it should be. The amount of lives which could be saved or made better, not necessarily of newborns, is beyond worth it.

My local hospital, obviously I can't name names, does not carry out any blood glucose measurement tests unless admitted to SCBU (Special Care Baby Unit) and this goes for many hospitals in the UK too. Tragically, many babies symptoms are not picked up and if symptoms are picked up, it is usually too late - if glucose monitoring was done at birth and monitored if needed then brain damage and even death can be avoided. 

I'm  continually reading how midwives dismiss mothers requests and concerns too - This article is about a baby who unfortunately died because his mothers concerns were not seen to by a medical professional. The mother raised concerns about her baby's feeding and abnormal crying -  she was simply brushed off as being 'anxious'. Any concerns should be followed up by a medical professional , mum knows best! From what I've read, midwives also need more training and equipping in the monitoring of blood sugar levels - what to look out for and how to treat a hypoglycaemic situation. Of course, my opinions do not go out to every midwife out there or every nurse or medical professional and yes some are trained in this but it's happening less and less often by the looks of it and something needs to be done about it. In other cases, even if patients symptoms are being picked up, some are still not receiving the right care and are not receiving the correct treatment - some doctors have not heard of this and it is misdiagnosed in numerous cases if not detected at birth. I got to know one mother at the hospital whose baby had been misdiagnosed, the drs did not know what they were dealing with - for the first ten weeks of her life, her daughter was pumped full of drugs which did not make any difference to the condition but instead made her really ill - thankfully she was transferred and put into the right care in time. 

This article is definitely an interesting read; it just proves the point that more needs to be done... brain damage should not be an option for any, otherwise healthy, newborn! I do wonder if the NHS sees this as a problem or not... Glucose is so important to the functioning of our bodies, without it or ketone bodies - what fuel does your brain operate with?  

The HI fund are constantly researching and attentively looking for lifelong answers to HI and the problems is can cause. Would you believe that NO research is being done in the UK for this condition and little to none being done anywhere else in the world... simply due to not having the funds. With the correct funds specialist staff could be employed, equipment could be brought and research carried out - this may be a rare condition but plenty of babies are being born with glucose issues, whether they be transient or persistent. 

All from one heel prick at birth, so much can be identified, especially if the baby is symptomatic, and if necessary then the right diagnosis and treatment can be given. It's so sad that this is even happening in our time. This is just so important, I can't believe how many deaths are caused by this and hardly none make it to the media or public knowledge! It won't go away unless something is done.

I can't fault the medical team that dealt with my daughter: I feel the staff listened to me, they knew I had issues with how my daughter was, especially overnight and they checked it out. This is how it should be! I can't even begin to imagine not having her here today, especially if it was just down to negligence.

Any more children I have with Andrew (as Orlaith is hyperinsulinemic on the grounds of a confirmed paternal heterozygous mutation in the ABCC8 gene) has a one in four chance of suffering from hyperinsulinism too. I already have a birth plan for my next child - the baby will be heel picked at birth, no questions asked; no risks are being run this time.

I'm proud to say I'm hyperinsulinism aware, are you? 

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? 

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

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. ♡♡♡