I Didn't Choose The Scoliosis Life, The Scoliosis Life Chose Me...

To start, and I have probably mentioned in previous posts, Lyla has scoliosis - a right curve with associated kyphosis (curve protruding from her back) 

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Back in September 2012, Lyla had an orthopaedic appointment for a spinal check-up and x-ray; at that stage Lyla's spine had curved to about 70º+ in the space of 10 months, so it was quite a severe and progressive curvature.  At that September appointment the specialist told me (as we discussed this at a previous appointment) a spinal brace would not help Lyla - 1. because it is very constrictive and will not help her already bad breathing issues, and 2. her spine will more than likely return to this position over time.  He breifly mentioned surgery, but said she was too young so all they can do for the time being was to monitor it and return in a year.

So fast forward to last Friday when we had the follow up orthopaedic appointment.  We had a 9am appointment at the Women's and Children's Hospital - 9am is not usually a time we are up and in the city by, but the good thing about the early appointment was a semi empty clinic, therefore we did not have to wait the normal 2 - 2.5 hours to see the specialist... Anyways, straight away Lyla went to have an x-ray, this is a somewhat traumatic experience for Lyla, she is not normally awake at 9am, but here she was naked, on a cold table being pinned down and stretched by a stranger.  I try to make all these things as happy or as enjoyable as possible by doing all her favourite things, like explaining everything we are doing; 'one arm out, two arms out, boof out' as I take her shirt off, and 'arms up, up, up' as the radiologist tries to force her arms up in an un-natural position for her; none of this actually helps, but I guess it makes me feel better knowing I am doing what I can to try and make her feel better.

I got the x-ray image straight away and headed back down to the clinic, I thought I would take a quick peek at the x-ray as I waited for the lift, my heart sank as I started to pull it out and I saw all her little ribs squished and the curve of her spine.  I knew her back had worsened, but I don't think I was expecting it to look quite as bad as it did.  We waited about 30 minutes to see her specialist, he came in and measured the curve on her x-ray and he said it has reached and slight passed the 100º mark - 100 degrees!? 100 degrees!?  I remember the last time I saw him he said, "Once a curve reaches 90º people start to have lung and heart issues", Lyla is now at 100º what does this mean?  I asked if he has seen this degree of scoliosis on someone before, he said he has, its very rare but he has seen it with older people, and that it is very, very rare for someone Lyla's age to have this severity.

He checked Lyla out and did all the usual things, he said she still has some flexibility in her spine which is good, he also said her spine is rotating and we're now stuck in between a rock and a hard place and she needs something done as soon as possible, and surgery is the only option... Surgery was something we didn't want to do, especially when Lyla was younger, because her future was so unknown we didn't want to put her through surgeries just for the sake of it, but if that's the only option we have, we have no choice... The specialist said the plan is to return in 1 month for some more x-rays and then he wants her to have an MRI (under anaesthetic).  Lyla is having an anaesthetic in January for botox in her arms, so I asked if he could contact her rehabilitation doctor to combine them, which he did, but he wants it done sooner, so he'll try push the botox forward.  And after all of that, surgery.  He said because Lyla is so small and still growing, surgery will need to be performed every 6 months to replace the rods.  Apparently there are some other, newer options, but he is unsure if they're available in Australia, or if they're available in her size.

this is her x-ray from last week

This is a comparison - image on left is most recent October 2013 - 100º

image on right is September 2012 - 70º

Jess

My Child - the most 'normal', medically abnormal child out there

Two weeks ago we had a metabolic appointment and finally got the results from Lyla's liver, skin and muscle biopsies she had done back in June; the results were just as I expected - 

all normal!  

I'm have copies of the reports, none of which I can interpret by myself, but from what I was told some of her results did come back higher than 'normal', but not so abnormal that it would suggest or direct them to a diagnosis.  Her liver, which has been a bit of a concern to doctors (large, extremely high LFT, discoloured, etc) came back as slightly fatty; but once again nothing to worry about.  This was a relief to me, especially after hearing the surgeon who performed Lyla's gastrostomy, say her liver looked very unhealthy.  This made my brain go into overdrive thinking she is going to need a liver transplant, etc, etc.  Thankfully that's not the case. 

So what's next?  Her metabolic doctor will have a meeting with her neurologist and geneticist to see if she can have whole-exome sequencing genetic testing done.  Exome sequencing is a new, very sophisticated and quite expensive genetic test, which requires bloods from child and parents to look thoroughly through the DNA finding any defects and genetic disorders; this is our best shot at the moment.  Fingers crossed we get the green light for this.



Jess

Awareness Week...

I wrote this yesterday & published it, but it obviously didn't work, so try again today...


This week / day is very special for multiple reasons, 3 in fact, this first week of August is Cerebral Palsy Awareness Week (Monday July 30 - Sunday August 5), Jeans for Genes day (Friday August 3) and World Breastfeeding Week (Thursday August 1 - Wednesday August 7), all very important to me.

Every 18 hours an infant is born with, or will develop, cerebral palsy, which is roughly 600 - 700 children a year; the number 1 physical disability in children.  Cerebral palsy is not something you can catch, it is caused by damage to the brain during pregnancy, delivery, or soon after birth; the main causes being oxygen deprivation, or prematurity.  There are various forms of CP, but all affect the movement, tone, posture, coordination and reflexes of the body, and often people with CP also suffer from vision, hearing, learning and speech impairments, or epilepsy. 

Lyla's second cousin suffers from diplegia cerebral palsy (I hope that is correct), and we have been lucky enough to come in contact with other children whom also suffer from CP.  Several months ago Lyla was "diagnosed" with cerebral palsy, spastic quadriplegia actually, spastic meaning high muscle tone, movement difficulties and involuntary movements, and quadriplegia which mean it affects the entire body.  BUT, she doesn't actually have CP, confusing I know, but if you were to just look at Lyla's characteristics (movement, tone, etc, etc) cerebral palsy fits, this though, allows me to find out more in terms of therapies that may be able to help. 

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Jeans for Genes day, which is today, was a cause I never really thought much about until just recently. I, like many, used to give my gold coin donation at school just so I could wear jeans for the day and not really know why, or what I was giving money to, probably because it didn't affect me... Well turns out all these years later it has.  Every year millions of Australians give a gold coin donation in order to wear jeans for the day; these donations go straight to the Children's Medical Research Institute.  It is here scientists are able to find cures, treatments and diagnosis' for the 1 in 20 children who are born with congenital birth defects, diseases or genetic disorders.  Lyla is one of these children, as are her special playgroup friends, and my cousin Chelsea, who lost her battle to brain cancer, July 5th, 2011 at the age of 5. 

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And finally, World Breastfeeding Week!  Those who know me, know I am a huge advocate for breastfeeding, and those who know me would know that it was something we were physically unable to do and I was devastated, but that didn't stop me!  Due to Lyla's condition she was unable to suck, which is obviously crucial for breastfeeding, thus she had a feeding tube inserted.  For over 7 months I attached myself to a pump and 99.9% of her diet was breast milk.  But my days slowly turned from caring for Lyla, to trying to express enough so she would never need formula.  Determined to go for as long as possible I knew it was a battle I would not win; a child whom is solely tube fed cannot go on forever with breast milk, nor could I, so the time came and I stopped. 

Jess

Lyla's Surgery

I have been a bit slack on the updating, yet again... I think one of my last posts was just before Lyla's gastrostomy, which was about 7 weeks ago now, so that's what I'm going to write about :)

After all the stuffing around with Lyla's surgery date, it was finally scheduled for Wednesday, June 5th.  Lyla had to fast from 6am and was admitted at 11am that morning.  She was SO happy, usually going to the hospital is a bad experience for her, but for some reason she was in a great mood.  Her surgeon came in around 1pm to discuss the operation; she would be having a laparoscopic gastrostomy, a liver, muscle and tissue biopsy, and bloods taken for genetic testing.  I asked while they were actually doing the operations through her belly button if her hernia could be fixed - it only seemed right as she has an umbilical hernia and they were going through the site, they may as well just sew the hole back up on the way - he said that was fine!  We took her through to the pre-op room and at about 2:30pm she went in.  The anesthetist said the surgery would take anywhere from 1 - 1.5 hours, plus about 30 minutes in recovery, she would then be transferred to PICU where we would meet her... 5pm came by and I was stressing out, and no one knew anything, soon after the surgeon found us to say the operation went well, they just went really slowly and her liver biopsy took longer than expected, it took 6 attempts, he said her liver looked really unhealthy and discoloured, but he did not know why.  Lyla has always had a really weird liver, obviously no one has physically seen it before, but doctors have mentioned it is quite large and her liver tests have always come back abnormal, this was the reason for the biopsy.

About 6:30pm I was finally able to see her... She was very sad and crying / screaming like I had never heard her do before.  She was hooked up to the machines and had high and low flow oxygen on, but the NG was gone!  It took a while for the PICU staff to work out her medication that night, it was every hour on the hour she would start screaming in pain, but she would react to the stronger opioid, as they would pretty much slow her breathing down to nothing, so it was a long night.  The following day she was having blue episode after blue episode, up until that afternoon when they slowly started giving her some hydrolite, then her formula, it was like a light switch and she was back to her normal self, and surprisingly did not require any pain relief for the rest of the stay!  That night I found it extremely hard to sleep (I was in her room) as her apnoea monitor was constantly going off, early morning I went into the parents room for a few hours of sleep and one of the nurses said, I cannot wrap my head around Lyla and her breathing.... If this was any other person having these HUGE dips in oxygen levels, and the constant apnoea episodes, they would have died, but her body just adapts!  I always knew she was special!

I was expecting to leave on the Friday, but we weren't allowed until Lyla had built back up to her normal food intake, so we were transferred to a ward for another 2 nights.  As soon as Lyla was hooked up to the oximetry monitors they started going off, as her oxygen levels are naturally low, and eventually, after some reassurance (me reassuring the nurses) they felt comfortable, but decided to leave her low flow oxygen on as it kept her above the 92% oxygen level, occasionally.  Saturday came by and Lyla had 2 new nurses, who remembered her from her last hospital stay at 7 weeks old... 'The baby who went blue and constantly required the medical emergency team!'.  Anyways, Lyla continued to scare these 2 nurses with her episodes, lack of breathing, low breathing and "seizures"!  NEVER have I thought Lyla had seizures, and her previous EEG's, though along time ago, never showed seizure activity, but these 2 were so sure she was having seizures they started recording them.  Before leaving for the night, the day nurse begged me to ask the doctors to do an overnight oximetry and an EEG on her, no way was I bringing this up to anyone now, we were leaving in the morning!  Lyla had another blue episode overnight and her night nurse said she wanted to bring in the doctor to give her a look over and know her base line in case something happened to Lyla overnight.  The doctor came in shortly after and she told the nurse to pretty much relax, as I was not concerned about her, and there is nothing she (doctor) can do for Lyla, this is just how she is and she cannot be "fixed".  I was so happy the doctor understood!

Sunday came and the surgeon gave Lyla the all clear to go home!  

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Since the operation Lyla has changed so much, she is no longer coughing and gagging from the nasogastric tube, she has been making some small noises and loud cries, she is semi-interested in tasting food and will let us put a dummy in her mouth!  She is healing really well, and in about 2-3 weeks her PEG will be changed over to a Mic-Key. 

Jess

'Welcome to Holland'

I first came across this poem, by Emily Perl Kingsley, early last year and just like the our world from above post, it is an amazing comparison of our life with a disabled child.  More recently I have had numerous people mention and forward on the poem, so I thought it was about time I post it...

WELCOME TO HOLLAND

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

The Colour Run

As I mentioned on a previous post, Daniel, myself and our team the 'Rainbow Stars' (made up of staff, playgroup friends and family involved in Novita) were participating in the Adelaide Colour Run, well we did it 2 weeks ago and it was AWESOME!!  Our team has raised close to $3,400, well over our first goal of $1,000, for Novita, as well as the money from tickets sales, which will go towards Make-A-Wish. 

Here are a couple of pictures from the day...

Thank you to everyone who has donated, all this money will help Novita in every way from helping to provide equipment, to supplying our coffee for playgroup, which is an important part of Lyla & my lives. 

Jess

An Update

Sorry I have been MIA for quite a while, I have just finished my 2nd and 3rd uni courses, so the last month, or so, was pretty hectic with final assignments and an exam... plus all the normal everyday stuff which was squeezed in.

So what has been happening over the last month...

Lyla has started a new medication called baclofen.  The plan is for it to help relax her so she will not be as 'stiff'.  I have noticed a small difference, not exactly the difference I was expecting, like more mobility in arms & legs, she just seems to be a little more floppy in the trunk.  I was very hesitant putting her on this medication, and after close to a year of discussion with numerous doctors, I decided to just give it a go... When Lyla was born she was put on diazepan (without our knowledge, or consent) which sedated her for weeks until we had fully weaned her off of it; this is why I was hesitant.  Even though it is a different medication I didn't want it to knock her out, or change her.  

She also started a new formula, up until a month ago she was having your normal supermarket bought stuff, which is not best for a child whose only source of nutrition is a formula designed for 1 year old children who eat solids too.  I was sick of spending $40+ a week on formula.  So after a nutritionist appointment (which I was also not 100% keen on, that is another story) Lyla is now on Nutrison, a pre-made formula, which looks and smells like chocolate milk (you can now imagine the looks I get when I pull out a bottle of 'chocolate' milk) and it is normally for children over the age of 10, but nutritionally, this is best for her.  The bonus of this is, it's FREE!  The downside is the end result, no one is prepared for that!

We have now started weekly occupational therapy sessions, and the occasional speech (if she is around), in the hope to 'de-sensitise' Lyla, as she is very sensitive to unfamiliar textures etc, which, for example, makes things really hard when she has her little 'blue episodes' just because someone other than immediate family touches or holds her.  We are starting at her feet and working our way up. 

We had a follow up eye check 3 weeks ago, her eyes are FINE!!  However, we will be returning in 6 months for an actual test. 

The last couple of weeks Lyla was also very unwell, never has she been so sick before and it was horrible.  She ended up with a chest infection, which as you may know, or may not, for a child like Lyla can be VERY serious, especially with a high risk of aspirating; and sadly this is how many children like her pass away.  She was on 2 forms of antibiotics + a probiotic (which was powdered and got stuck in her NG tube, and ended with a tube change) she also had to have a chest x-ray to rule out pneumonia and avoid a hospital hospital stay, but luckily she's a strong little thing and after MANY sleepless nights, she's good as new!  Following this, we had a midnight visit to ER with a meningococcal scare, luckily that was also clear.

And finally, probably the most exciting news actually, 
WE HAVE A SURGERY DATE FOR A PERMANENT FEEDING TUBE!!!  
 We have been waiting for this surgery for a long bloody time!  There was alot of stuffing around with the date while Lyla was sick and last week her surgery was cancelled and rescheduled 3 times!  Now it is a definite for Wednesday June 5th, 5 sleeps!
The surgery involves placing a permanent feeding tube, either a PEG or Mic-Key Button, directly into her stomach, from the outside.  This means no more nasogastric tube and we will finally be able to see her face with no tube or tape!  Along with this, 3 biopsies will be done - tissue, muscle and liver.  The date cannot come quick enough, but as much as she needs this done and we want it done, I am VERY scared; I don't want her to go under anaesthetic, I don't want her to stay in hospital (staying in PICU), I don't want her to be cut open, and I do not want another repeat of our last hospital stay (I might write about this another day), but positive thinking!

Well, I think that is all that has happened over the last month in the life of Lyla, I will post a few pics too.

That is all for now

Jess