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!  

...

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 

Colour Run

This year is the first time the Colour Run is being held in Australia... What is the Colour Run?  Well, the Colour Run is known as the "Happiest 5k on the planet"; it is a 5km walk/run event, but not just your average run, at every checkpoint (5 in total) you are covered in brightly coloured paint.  Sounds fun right?

My husband and myself, along with our very special friends are participating in the event as the 'Rainbow Stars' to raise money for the amazing organisation Novita (I will talk about Novita another day), who provide all the therapies for Lyla and her friends.  

Where : Adelaide - Victoria Park Racecourse

When : Sunday May 19th, 9am

Why : To have fun and raise money for your favourite charity or organisation 

How : Colour Run, join a team or participate as individual 

Please feel free to donate a few dollars to Novita and show your support 

Novita Everyday Hero's

I will try and find a group photo of us to add 

Jess

A day in the life

On a daily basis we are judged or judge people on their physical appearance, everyone does it.  Quite often my mum & I will go to our local shopping centre and 'people watch' whilst having a coffee, but never would I stop someone and question them about their fashion choices for the day; you wouldn't would you?

I know people can't help but look or treat someone differently based on any physical abnormalities they may have, but is it okay to go up to that person and ask them for an explanation as to why they are different?  This is what I want to know, because on a daily basis, numerous times a day if Lyla and myself are out, we are stopped so someone can ask a question, or we see someone performing some kind of Exorcist neck twisting manoeuvre because they just caught a glimpse of Lyla from the corner of their eye.

Monday for example, I was sitting in the new Rundle Mall Plaza, feeding Lyla with a couple of friends, a guy walks pasts, stops, walks back, looks at Lyla points to his nose and asks "What's that for?" to which I replied "For feeding", he walks a few steps and then said "Oh I know what happens otherwise" and stands there (in a packed food court, mind you) puts his hands around his throat and pretends to choke.  Are you serious?  This is the kind of stuff I get whenever we our out in public, okay, well maybe not this dramatic, but still, I don't think its fair to be making fun of my daughter.

These are some of the questions I get or comments I overhear...

"Was she premature?"

"Is she okay?"

"What's that thing in her nose?"

"Is that for oxygen?"

"Look at that poor, sick little girl"

or my personal favourite "What's wrong with her?"

I have no problem talking about Lyla if someone ask a question, but it all depends on how someone asks me the question; when I get "What's wrong with her?" which I get quite frequently, I reply with "Nothing, she is fine" (I want to say 'what's wrong with you', but I don't have the guts).  And "Poor sick little girl", well actually she's not poor, she has a great life and she is not 'sick', in fact she has never been sick! *touch wood* At the end of the day, or by the time they walk away, they've forgotten about Lyla and they don't really care 'what's wrong with her', so why should I be expected to tell them anything?  

So the next time you see someone who looks different or acts different - like that child throwing a tantrum in the supermarket, maybe take a second to think about how your looks are perceived to that child or carer, or those questions you ask; are you asking them because you really care and want to know, or because you are nosey and want to know why they look different, or that child throwing a tantrum, did you think maybe this child has a disability like autism? 

Just a thought.

Jess

Syndromes without a name....

As everyone is now aware, today is national 'Undiagnosed Children's Awareness Day', so I thought I share some information about Lyla's unknown condition.

Lyla's symptom or abnormality list is a long one, some are quite obvious, some are only obvious to the doctors who are looking for them and some are internal, which are only know about through blood tests, scans, etc.  We have heard a number of possible diagnosis's for Lyla, some extremely rare, which many specialist were even unaware of, to some of the more common, like cerebral palsy.  Not one of these conditions Lyla shows enough or the right symptoms for, or tests have been performed and like many, for some reason, all come back as 'normal'.  We have said from the beginning  it is just 'Lyla Poppy Syndrome', which could well be the case.  

People have said to us an overall diagnosis doesn't change anything, that is true, in some ways, but a diagnosis can provide us with information that can assist us and Lyla in terms of care, treatment, medication, development, or outcomes.  A diagnosis may also provide us with the information we need to know before we decide to expand our family - is it genetic and what are the chances of this occurring again. 

Lyla's symptoms are congenital, meaning they were all present at birth, since then some have worsen while some are not quite as severe as they once were.  Here are Lyla's most obvious or visual symptoms list...

• Brain formation - first thought to be Polymicrogyria is now Open Bilateral Opercular Syndrome, from my understanding it means the space that divides the left and right hemispheres is larger than normal.  This affects muscle functioning in the face and throat, like listed below.  There is some other brain abnormalities, which I cannot remember.

• Eyes - ptosis (droopy upper eyelids) due to this, people often assume she is sleepy.  Ophthalmology is also convinced she cannot see - which is absolute bullish*t!  Next appointment is in May, so I will keep you updated.

• Breathing 
• Stridor - a loud, high pitched noise which is normally heard while inhaling.  Caused by a 'floppy larynx, it is not as obvious as it once was, which was with every breath, it is really only heard now when she is upset.
• Airway spasm - discovered during a endoscope procedure
• 'Blue episodes' - This for a LONG time, and still to this day, is one of the major issues we have; often triggered when Lyla is upset and is associated with the above to breathing problems and possibly her brain.  Lyla's airways start to close causing her to not get enough oxygen and turns blue.  Once, I have had to perform CPR on her because she didn't get a breath in, in time.   We now carry O2 with us in case. 
• Apnoea - long space between breaths, usually happens while sleeping, and may be due to her brain not always reminding her to breathe. 

• Feeding - Lyla has always been fed through a naso-gastric feeding tube, as she has no suck / swallow reflex.  One of the main questions I am asked by people is 'when will she eat normally'. This is really the million dollar question, but also the least of my worries.  It's not that she is not interested in eating, she just can't... She does not understand it, therefore can't.  There is also a chance of aspirating (inhaling in to her lungs), which has never actually be tested or confirmed, but for that reason we've been told to give her nothing orally.

• Spasticity - in arms and legs, which causes increased muscle tone (stiffness - hypertonicity); involuntary movement (leg spams - clonus); limited & delayed movement; contractures. 

• Scoliosis - Lyla's spine is curved and has worsened over time; she also has 2 forms of scoliosis - kyphosis (curve protruding out of her back) and right thoracic scoliosis (spine curving to the right).  Her right curve, when last scanned, was almost at an 80 degree curve, so quite severe. 
• Hypotonicity in trunk (weak muscle tone)

• Bilateral hip dysplasia - both hips are dislocated

• Global developmental delay

Here are some 'Syndromes Without a Name' [SWAN] links 

SWAN Australia

SWAN UK

SWAN USA