Curveball...

There are millions of moments a person can experience that will
change their life forever; I think the positive pregnancy test with Lyla
was a big life changer for me.  No longer would I be caring for myself,
but I would now be caring for another human being; which apparently started a
few weeks before I even knew.  The next life changer was the moment Lyla
was born, there was my responsibility, this person I created.  I grew
this.  I made this.  This was a baby, my baby, and I changed from a
23 year old, carefree, responsibility-less woman, into a mother who like most
would do ANYTHING to provide, protect, care and nourish my child.  No
matter what.




But in that same moment all those dreams I had changed, just
like that.  Of course the providing and protecting, etc. etc. was all
going to be the same, but in that moment, and the following days, weeks, months
and even years, I changed.  To be honest, I hardly even remember the
person I used to be.  I don't know if that's a bad thing, or a good thing,
or maybe my brain is now just mush I honestly struggle to remember that far
back.  But I believe I've changed in good ways since having Lyla and
learning of her disability; like I feel I am more "knowledgeable" in
most things medical, I'm more patient and I'm stronger as a person.
 However, I feel as though I'm a lot more boring than before, because lets
be honest, my interest in all things medical doesn't keep a conversation going
amongst friends who don't relate.

But I am now used to the newer me, the me that I've been for the
last 3 years.  So sorry if I now bore you, but these kind of things can
happen when you experience those significant life changes.




Another life changing moment happened more recently,
specifically on Wednesday October 22nd, 2014.  You know when life starts
to go pretty smoothly, you've made some plans, you've got a few events coming
up you look forward to.  Life's pretty good.  You and your family are
happy; myself, my husband, our daughter and our dog, just the 4 of us.




Then, there's that curveball life decides to throw you...

Yep, that life changing moment. 

You're PREGNANT! 

Shit. 




You know that curveball was coming; you saw and ignored the
signs, hoping if you played dumb it would bypass you.  Apparently life
doesn't work like that.  I built up the courage to tell the innocent
bystander, though he was to blame, he went into shock.  My mind went in to
overdrive.  We didn't want another baby, well definitely not right now.
 How was I going to keep up with all of Lyla's appointments?  How was
I going to get around with 2 kids, one who was in a specialised pram?  How
am I going to care for 2 babies, and yes BABIES, even though one of them is 3
years old, technically she is still like a baby.  Then the biggest mind
f*ck... 

What if this baby is just like Lyla? 




It's been a number of weeks since we found out, it's also been a
number of weeks since I wrote the above, the worry is still there, but it has
definitely been overshadowed by excitement.  All of Lyla's doctors and
specialists knew before our family and friends, because that's what happens
when medical professionals know more about your personal life than most.
 I've recently had my first antenatal appointment at the Women's and
Children's Hospital, because it was felt I needed a more specialised care,
because of Lyla, involving extra scans and a possible MRI.  However, I'm
hoping for a transfer to my local and preferred hospital soon though, because I
don't particularly want specialised care, I want normal care with normal
midwives.




So I have no idea what the future holds, I do know it will be
crazy, but I do believe this unplanned, unexpected, and scary news is most
definitely a good thing for us all.




What ever will be, will be.

Week 5-7 Of Life: Our Journey To Palliative Care

This past week was National Palliative Care Week.  Palliative care sounds like a scary thing, and it is, but the services, support and care, palliative care providers provide for their families is unexplainable.  So, I thought it would be a fitting time to share our story of how we become apart of palliative care.


(due to my horrible memory, there may be a few blanks)


January 3rd, 2012, at 5 weeks and 5 days of age, Lyla was booked in to have an overnight oximetry and a barium swallow at the WCH.  Anyone who is unfamiliar with an oximetry, it is non invasive test that measures the oxygen saturation levels in the body, and a barium test watches a swallow (while drinking) under x-ray.  As soon as we arrived to the ward, we were told we were in for 2 nights, which was news to us, and just as quick as we arrived and started to get settled, Lyla had her first 'code blue', followed by another 2 that night; we became quite familiar with the Medical Emergency Team.  And needless to say, everyone in the ward was freaked out; the nurses moved Lyla and myself to the room directly in front of their desk, put her on oxygen and gave her a 1:1 nurse, who literally sat next to Lyla all night.  I was even told not to touch or pick up Lyla incase that set her off in to a blue episode again!  The following day, slowly started our week from hell, with the help from a very smug, inconsiderate, inappropriate, condescending and just all round asshole of a doctor ("Dr M."), he cut Lyla's feed amounts in half and put her on continuous feeds, incase all of this was reflux related (ha!).  Eventually we were moved to PICU, as Lyla's needs became too much for ward nurses to handle.


We came in for an oximetry, but we ended up in intensive care.  This was all new to us, yes we had spent a couple of weeks in hospital when Lyla was a newborn, but we had been at home for 4 weeks, 4 amazing (and slightly scary) weeks where we tried not to think about her unknown future and go about our new life as normal as possible.  It was overwhelming, but we had an amazing nurse in PICU, called Kate, and she was exactly the kind of person I needed around me, happy, helpful, caring; if I wasn't around Lyla she was there holding her and I knew she was safe.  I still think about her, she was the one person I could say inspired me to want to become a nurse.  I wanted to do for others what she was doing for us. 


Our time in PICU was not how I envisioned and it kept going from one extreme to another.  It went back to day 1 of life; to try and find a diagnosis for Lyla, to try understand why she was like she was.  They were no longer focused on the issue we came in for - respiratory.  To Dr M. she was science experiment, he thought it was exciting, coming in to see us with a bounce in his step, an inappropriate joke about the situation and then a laugh.  We met with a number of doctors and specialists over the first couple of days; general medicine, respiratory, pulmonary, neurology, genetics, ENT.  It was decided to take Lyla to have a laryngotracheobronchoscopy (camera down airway) to check for any obstructions in her airways that could be causing her to have these blue spells, after the scope she would have a MRI scan; all done under general anaesthetic.  It was scary, this was her first anaesthetic, despite everything she underwent when she was born. Thankfully she came back with no issues from the anaesthetic and no growths or obstructions in her airways; instead she had a continuous airway spasm.  The MRI showed possible 'bilateral open opercular syndrome', only Lyla's neurologist knew what it was at the time; a malformation of her brain that would affect face and throat muscles.


After raised concerns about possible seizure activity Lyla had an EEG done whilst in PICU, normally they attach sticky monitors to the head, but for a more accurate reading the monitors had tiny needles that went into the scalp.  Thankfully there were no recorded seizure like activities.  But things were not looking good for Lyla, it was blue episode, after blue episode, after blue episode; everyone knows what continuous lack of oxygen can do to the brain, and the biggest fear of ours and the doctors was she would have one of these episodes and not recover. 


We (myself, Daniel, my parents and his parents) had a big multidisciplinary meeting one morning with all the specialists we met with over the stay, to discuss what had been found and what we would do next.  Luckily by this stage Dr M. was on leave, so we had a lovely new doctor to lead everything.  It seemed the ONLY option we had to help Lyla was to take her to surgery straight away to have a tracheostomy inserted to protect her airway.  We didn't want this.  After a very long meeting, lots of discussion and lots of tears, we begged (almost) to let Lyla come home.  We didn't know what to expect, but I was prepared for Lyla to come home and die, as was everyone else.  The doctors were happy for discharge, but we couldn't leave without oxygen and because of our decision we were put in contact with palliative care.




 This was scary, palliative care was for people who were just about to die, so I thought.  Her name was Julie, she was very sympathetic to our situation explaining what she does and what P.C. do for families; it was a lot more than what I thought.  Julie sat down with myself and Daniel along with our parents and we discussed what our goals were for Lyla and many other heartbreaking things no parent should need to think or talk about.  Julie said we should make a resuscitation plan before being discharged, incase something was to happen with Lyla and we would have, in writing, how far we were willing to intervene.  We told Julie we didn't want Lyla on 24 hour continuous oxygen and feeds (like what was wanted by doctors), as this would be impacting on our quality of life with Lyla.  After these meetings Lyla was taken off all supports and monitoring and we were left in charge of her care, doing what we would normally do at home. 


Later that day we had a nice distraction as my grandpa had organised to have Brenton Sanderson come in to visit us.  I wasn't too familiar with Brenton, but he was the new AFL coach for the Adelaide Crows; everyone in our family is a Crows supporter except for Dan and myself, we're Geelong supporters, but Brenton was an ex Geelong player and assistant coach, so Dan was happy. He came in for an hour or so, we told him all about Lyla, had some photos and everyone had a nice chat with him about football.














Our final day in PICU was 'exciting', we were looking forward to getting out of this place.  We were given rundowns on how to use our new feeding pump and home oxygen, then came the time to fill out the resuscitation plan.  Thankfully Kate talked us through all levels of intervention and physically showed us what machines were used, so we knew what to expect if we were ever in the position.  Dan and I met with the leading doctor and Julie, we made our decision for resuscitation, much to their surprise, they didn't think we were making the right choice, but it was OUR choice.  There was then some discussion between the two, that the coroner should be advised 'to avert the need for involvement in unlikely event of death less than 24 hours post discharge'.  That was rough. 
















Then it was time to leave.  It took a couple of days to finally relax back into our life, but I still had in the  back of my head with every episode she had at home that this would be the last one.  




















The weeks and months after our discharge our family told us of some of the things Dr M. said to them while we were not around.  It started with Dr M. running into a support nurse we had with us during our PICU stay, and he was wanting to know why Lyla had been discharged, this was weeks later, he believed she should still be in.  Then more recently our parents told us Dr M. told them the best thing to do for Lyla was to let her starve to death.  The thought of a medical professional saying something like that to a family makes me physically sick, but thankfully this doctor no longer works at the WCH. 




















































(thats Kate in the background)


















































We are still under palliative care, we do not require as frequent support from them like we once did, but they are always there if we need them, for literally anything; in fact Julie is coming to visit us tomorrow, so we can fill her in to what has been happening over the last few months and weeks, and Julie hasn't seen Lyla in a year! 

Undiagnosed Children's Awareness Day 2014

Today marks another Undiagnosed Children's Awareness Day that we have celebrated for Lyla, and I am sure a day will come when we will no longer need to celebrate the day because Lyla is undiagnosed, but because she once was...

A Desperate Trip...

I'm sitting here this morning, with Lyla, doing our usual routine before we start our day, which involves watching ABC4KIDS while having 'our' morning coffee... Nothing exciting, but I like to start our day quietly and relaxing-ly (that's definitely a word).  Today is the day after yesterday, and yesterday was the day we had our worst fears reconfirmed, it was an emotionally draining day. 

...

A little while ago a very good friend of mine told me about a lady and her son, she saw on one of those morning TV shows.  This mum was on the show talking about her son, who had some major health issues (one being scoliosis) and she was doing all she could to help him, while studying to become a doctor and medical researcher, and she was currently doing her Masters of Science in scoliosis.  Straight away I found her on Facebook and told her about Lyla, and then just as quick, she got back to me with all this information and all these contacts.  With her help, we were trying to book some last minute appointments to see 2 of the best orthopaedic specialists, one in Sydney and one in Melbourne. 

Two weeks ago she messaged me to say we managed to get an appointment with the Melbourne specialist.  I was so excited, we were finally going to get a second opinion and we were finally going to see someone who could fix her, just what we needed after the stuff around with Lyla's scheduled casting, and just in time because the week later at Lyla's normal orthopaedic appointment, her specialist said he no longer knows what to do for Lyla.

Two days ago myself, my mum and Daniel's mum went to Melbourne, the big appointment was the following day, so in the meantime we walked around Melbourne and caught up with friends and family for dinner and breakfast.  Yesterday we caught the tram to the specialist's office; we were only able to get a half hour appointment, but that was better than nothing, or waiting a few months to get a longer one.  He was a lovely guy, we talked a bit about Lyla's condition and he had a quick look over her, then we sat there, in silence, but his face said it all... Dan's mum broke the silence and asked 'Do you have the miracle cure?', he looked at us and said 'No'.  He went on to explain every treatment that is available to help treat scoliosis, and a million reasons why every single one of those will not help Lyla, why they would do more harm than good.  She is too small, she is too weak, she has no diagnosis, she has no prognosis, her hips are dislocated, her back is too severe, she has respiratory problems... The list went on, and on.  Her body is broken and there is nothing that can be done to fix it.  I made it through he first 20 minutes before it broke me.  As parents we do everything we can to help our children, and that's what we've done, but to be told again that there is no safe alternative to help save your child's life is too much for anyone, no matter how many times you've heard it, and we've heard it a few.  Our only option is to continue going on with our lives and giving Lyla the best one possible.  

I knew this was going to be the case, but I didn't want to focus on that, I wanted the miracle cure.  I didn't want her tiny body to be too broken to fix.  After everything she has gone through and everyone she has proved wrong, I don't want this to be it.  But every day, every month her back will continue to curve.  Every degree decreases her lung function even more.  Every degree causes more heart problems.  How far can it go?  How long will it take?  Is she going to suffer?

As heartbreaking as the appointment was, I am very glad we went.  He said if he had the miracle, he would fix Lyla.  But if he can't help us in the way we hoped, he is going to help us in other ways.  He is going to contact neurologists to look at Lyla's brain scans to help us find a diagnosis.  After the appointment we walked to Melbourne's Royal Children's Hospital, not usually somewhere I would choose to go to hang out, but that's where we went and it was beautiful.  We looked at the fish and sharks in the aquarium, we looked at the meerkats, and we had ice-cream, the specialists recommendation.  Then we went home, to where we will continue on with our lives, enjoying our daughter, giving her all the experiences she can have, doing all the things she loves to do, and never taking any day for granted.  Her fate may now be sealed, but her fight is not over.

Jess

Feeding Tube Awareness Week...

February 9th - 15th is Feeding Tube Awareness Week, why?  Well, why not celebrate something that helps keep so many people alive? 

Prior to Lyla I didn't know what a feeding tube was, I guess I had no need to.  However, I am sure I would have seen people around with nasogastric tubes, or being fed through syringes before, and I probably looked at them and thought 'what on earth is that?'  Nowadays, I can spot them like a sore thumb, except I look at them and think 'we have one too, I can relate'.  Though they probably think I'm just like the next person looking at them and thinking 'what on earth is that?'. 

I love our feeding tube, we can feed Lyla when and where we want, it's not a fight to get her to 'eat' or drink, we can feed her when she's asleep, we can give her medication easily without a tantrum... Jealous?  Ha Ha! 

Our feeding tube journey started hours after Lyla was born.  She did not require one because she was premature (like many thought), she required one because she could not feed orally, she just didn't know how, she didn't have that newborn rooting reflex.  This we later found out was more than likely due to her brain malformation, something that meant her face and throat muscles were affected, making it very hard to swallow, eat, talk, etc.  The first time I saw Lyla with the nasogastric tube (NG tube) she was about half a day old and it was the first time I was really able to see and hold her, I don't even think I asked anyone what it was, and I probably didn't even care, I was just glad to be able to finally see her.  

I quickly became accustomed to the feeding tube way of life; I had every size syringe known to man and I had enough them, as well as PH tape, Duoderm, Coloplast and Hypafix stock a hospital supply room.  I knew all the terminology, I knew the different types of feeding tubes and I knew the different ways to give a feed.  I was set.  

It took over 8 months of being on the surgical waiting list before Lyla was able to get a permanent feeding tube (Gastrostomy / G-Tube), so by the time it happened she was 19 months old.  It was a date that couldn't come quick enough.  I was sick of the NG tube.  I was sick of ripping it out.  I was sick of Lyla constantly choking, gagging and coughing on it.  I was sick of rushing her to the hospital to get it replaced.  I was sick of having to put her through attempt, after attempt, after attempt of trying to have it put back in.  I was sick of the risk of it being inserted incorrectly.  I was sick of it possibly moving and constantly checking the placement.  

The surgery happened June 2013, it went great!  Straight away we saw a change in her, she wasn't gagging, she was making vocal noises, she let us put a dummy in her mouth and she began to lick food.  8 months on and it is still the best thing we did.  Lyla is fed full time through the G-tube and I have no expectations that she will eat orally, and that's alright.  We give Lyla 5 bolus feeds a day (a feed given over a short period of time) via a syringe if we are out, or an automatic feeding pump if we're home (that frees me up for 20 minutes).  She is on a specialised pre made formula, which looks very much like an iced coffee (and I have to reassure people that I am not giving my 2 year old coffee).  I have been wanting to transition Lyla to a blenderized diet for about a year now, but I keep delaying it for some unknown reason... Maybe I'll start next week ;)  

My first NG tube

My last NG tube - Operation Day

Gastrostomy / PEG

No Tubes!

Current G-Tube / Mic-Key Button

Jess

Operation Day...

Wednesday January 29th, Lyla was scheduled to have a spinal cast made (you may remember from previous post), along with the spinal cast she would also have a hip cast and botulinum toxin (botox) injections in her biceps. 

Now, the whole lead up to the casting was something I had to prepare myself for, it was a big deal; Lyla was going to be plaster cast 'jacket', as such, it wouldn't be able to come off, she wouldn't be able to have a bath or shower, she would have very limited movement, she would be heavier making it harder for me to carry her with my back issues, it would uncomfortable, it would be something that could possibly hurt her, she would be upset, she would be hot, and with her respiratory issues I was expecting lots of episodes.  As well as preparing myself for the procedure, I also had to prepare her therapies around the casting and botox, which was a hassle because she just received funding from NDIA for specific therapies with specific time frames, etc.  For this Lyla needed intensive occupational therapy sessions to make the most from the injections, cancelling hydro-therapy (water based physio) and transferring that section of funding towards extra 1 on 1 land based physio and adding travel so her therapists could resize home seating equipment, we would have to to order new standing equipment, remeasure and order sleep posture equipment and ordering a specialised bed for her.

Early December I emailed the orthopaedic surgeon with some questions I had regarding the procedure, as well as some questions her physio had.  I didn't hear anything from the surgeon, just a receptionist who told me he was on holidays.  Mid January the receptionist emailed me saying another doctor had read through Lyla's notes and my email and he said "casting was a no".  Obviously this was a shock, so I contacted the receptionist (after many confusing emails) and she told me, the hip casting was a no, but the spinal cast would still go ahead like planned.  A miscommunication on her behalf because 'she didn't understand'.  That was a relief.  

Tuesday, the day before the procedure, I received a call from the rehabilitation department asking if I would like to rebook Lyla's botox because the procedure had been cancelled.  I had no idea what was going on, so I was transferred to the orthopaedic department, where I was told by the receptionist that the procedure had been cancelled, and the surgeon was meant to call on Friday, but obviously hadn't.  

That was too much for me; the frustration, the mental preparation, the lack of communication; I had reached my breaking point .  After hours of calling asking for the surgeon to tell me what was going on, he finally called me back.  He said after reading my email, in which I asked what would happen incase of an emergency and she stopped breathing requiring CPR, he decided the cast would be unsafe for Lyla.  A completely understandable reason, but it made me think he has only JUST read my email, and the fact that I was only told, by another department, a day before it was scheduled was really discouraging.  If it wasn't for rehabilitation calling me, would we have just shown up to the hospital the following morning?

So the plan is another GA where a mould will be taken of Lyla's spine and a brace will be made for her.  The brace is removable, but not as effective as the cast, but if she tolerates the brace a cast can be tried, and then surgery down the track.

In the meantime I have contacted a lady who has worked with a orthopaedic surgeon in Sydney who specialises in infantile scoliosis.  I am hoping to hear from him and hopefully be able to arrange an appointment for a second opinion, I just want to know what we're doing is the best thing for Lyla, or if he thinks there is a better option.

Jess

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) 

...

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