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

Congenital Hyperinsulinism Awareness

www.hi-fund.org

Emila Coco is here!

On Wednesday 20^th August 2014 at 9.45am, weighing an impressive 10lbs 2oz, Emila Coco made her dramatic entrance into the world via emergency c-section.

So far, all seems healthy and her admission to the neonatal unit at birth only lasted 48 hours - providing an incredible BM profile as well as no deformities currently being present... Which is all I could of ever hoped for!

The labour was not the 'natural with no pain relief' birth that I wanted. I turned down induction twice and I lasted 14 hours with no pain relief or intervention, birthing myself to 5cm dilated at home,  even with little to no breaks between contractions. I'm pretty impressed I got through it, I got to 9.5cm dilated and the 0.05cm was just not happening due to over medical reasons so it ended in a ECS.

Emila @ 12.5weeks
Emila's nearly 13 weeks now, I can't believe how quick time has gone... it's as if she's always been here.

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. 

Second baby so soon...

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

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

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

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

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

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

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

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.