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! 

Why shouldn't it happen to us?

I was a relatively healthy 22 year old when I fell pregnant with Lyla, I remember being ecstatic (and maybe a tad nervous) when I saw those 2 lines on that test.  I was in a hostel bathroom in Dublin and Dan was downstairs in the pub, and after waiting all afternoon to feel the need to pee, I texted Dan (romantic I know) that the test was positive and he replied with "Yay, I knew it".  

I avoided all the risky food and drinks while pregnant, I read all the books and attended all my midwife appointments.  I loved being pregnant, I was excited about the thought of giving birth, and I was looking forward to raising a beautiful and healthy child in a very natural and gentle way.  But I never expected to have a child with a severe disability, I had not prepared myself for this, I did not read about this, because this wasn't something that happened to people like me.  It was hard just as much as it was heartbreaking.  I has this picture in my head of my daughter running around with her cousins, who were just a few months old than her, going to school, telling me stories, playing at the park, making friends - all those things children do.  How could she do this when everyone was telling us she would live in a vegetative state and preparing us for her death?  Not only that, but no one actually knew what was wrong with Lyla, so how do they know her future when they don't even know anything about her condition? 

For the first few months of Lyla's life, whenever we had an appointment with her neurologist, geneticist or metabolic specialist I had prepared myself; that day we would finally get a diagnosis for Lyla.  I mean, how hard can it really be?  How naive.  Every appointment we were told the same thing we had from the beginning,'we don't know', and every time I left feeling deflated and frustrated...  There were a few possibles from early on though, Polymicrogyria, Schwartz-Jampel, Syalidosis, but Lyla never had all of right signs, or she had the wrong patterns.  It was never enough for a diagnosis.  After a while I no longer went to an appointment with that hope for a diagnosis.

Lyla is 2 and a half now and she still has no diagnosis.  My best friend is google, I google every condition and every medical word I see in Lyla's notes and reports.  I google every condition I read about, every syndrome I read of other children having and I try to connect the dots - Lyla has this symptom and this one too; I'm sure she has every condition out there thanks to Dr Google.  I need to know, I need to do all I can and I need to help her, I am her mother, but there is nothing I can do, I can't fix her, I don't even know what her future holds.  But I know it does not involve walking, being an independent woman, or getting married or having children... I have a severely disabled baby who will forever live in a child like state, who will be dependent on me for the remainder of her life, a life that will more than likely not be a long one.  That doesn't make me a horrible mother, I am being realistic, which may be hard for most to understand, but not for me and not for the people in similar situations.  I do not want to live with false hope and then grief, nor do I want to spend Lyla's life wishing for something better or thinking 'why me?', because I have something very special.  I have a daughter who has brought me so much happiness.  A daughter who, despite everything she has and will continue to go through, is gorgeous, happy, smart, cheeky, feisty and totes hilar.  A daughter who has taught me how to be a caring, sympathetic and more patient person.  A daughter who has inspired me to help other people, to share her story, to advocate for and educate others about special needs.  A daughter who has given me goals and ambitions.  A daughter who has shown me this place very few ever get the privilege of experiencing.  A daughter who has made me appreciate all those tiny little things most don't notice, and to live for every minute and never take anything for granted because it can be taken away in a second.  And I have a daughter who has shown me what true love is, she has reconfirmed why I wanted to be a mother.  It is about what you learn on the journey. 

We now know a diagnosis will not fix Lyla, but a diagnosis will help us.  Those dots will finally be connected, we will have a better understanding of what to expect for Lyla's future, and we will know what our chances are of having other children affected by the same condition.  Not everyone understands that risk as quite often I am asked when we are having more children... Caring for one severely disabled child is very different to possibly caring for two severely disabled children, and then caring for two severely disabled grown adults is very different from caring for one severely disabled child.  That's not a chance I am willing to take at the moment. 

I am the mother, who even though did everything properly, had a daughter with a severe disability.  But why shouldn't it be me?  Maybe I was prepared to have a disabled child?  I always had this weird feeling that it would be me.  I have the support, I have the love and I have the strength that was needed to raise a child with extra needs.  And even though I see my nieces and nephew doing the things Lyla should also be doing, I am no longer heartbroken, I am in total awe of the child we created and she is amazing just the way she is. 

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 year!

It's April, which means I've been a 'blogger' for a year, how exciting!

What has happened over the last year? 

- Lyla had her PEG surgery

- I finished my uni courses 

- We moved back into our own house

- We celebrated a 2nd birthday

- We went to Melbourne, twice

- Lyla's disability care moved to the new NDIS

- We have worked on new skills, like standing

- Countless hospital appointments

- Met new people 

- Lots and lots of fun times & memories!

What hasn't happened over the last year?

- We still haven't found a diagnosis

It's a little bit disheartening, but it is not something that I let affect me.  Of course I would love to have an answer for Lyla's condition and of course I would like to know what the chances are of the same thing happening again is, but I am sure an answer will come.  

One day.

In the meantime, as it is April it's almost time to celebrate...

Undiagnosed Children's Awareness Day! 

Friday April 25 is the day it falls on this year, well worldwide it is, in Australia it is Saturday April 26, but as it is not as widely known or celebrated in Australia, I am celebrating on the Friday with the rest of the world.

Here in Australia there doesn't seem to be much knowledge / support / research / information / funding / organisations / etc. focusing on undiagnosed conditions.  How sad is that? 

 Lyla and I decided to do as many positive things as we can in April to celebrate and bring some awareness to UCAD, here in Australia.  We started with trying to find someone or something that focuses on the undiagnosed - we wanted to raise some money that would go towards helping people find a diagnosis for children, but we couldn't find anything like that here!  The next best option was to fundraise for the Women's and Children's Hospital Foundation so that is what we are doing.  It is a place we spend A LOT of time and a place, that unfortunately, is very important to us.

Secondly, we made a video clip.  It was a last minute idea I decided we had to do Tuesday night and I knew I needed my playgroup mums & kids to participate.  So Wednesday morning at playgroup we filmed it and that night I edited it together.  If you haven't already seen it I urge you to watch it, it's great and I am not just saying that because I made it, it truly is special!

Thirdly, I am spreading the word.  I am using the video, the fundraising and Lyla's story to get the message out there.  Undiagnosed Children's Awareness Day is just as important as any other celebrated awareness day, it just doesn't receive the recognition it deserves.

Lyla and I are going to change that!

** Lyla has her own Facebook group now, which is easier for me to update everyday life through, so for more Lyla news, head over to...

www.facebook.com/LylaPoppysJourney

Rare Diseases Day...

Yesterday was World Rare Disease Day, a day for those living with a rare disease to come together, a day to spread awareness and a day to improve care for people with a rare diseases.

Did you know:

 There are about 7000 rare diseases

80% of rare diseases are genetic

100 million people world wide are affected by a rare disease 

About 50% are children

30% of children with a rare disease do not live past their 5th birthday

Receiving a diagnosis can take years, and often there is no cure

Lyla wore her denim jeans to show her support and her 'Team Lyla' t-shirt.  Lyla may not have an official diagnosis, but she is definitely proving to everyone that she is a one of a kind!

Please watch the video below

Travel Experiences...

How many disabled children do you think have flown interstate before?  A lot you would think, right?  Well it always seems like Lyla is the one and only.  The drama I have experienced trying to book flights, get approval for oxygen and sort out seating options for her is ridiculous! 

Lyla has flown interstate 3 times - Sydney at 12 months old, Melbourne  at 1 1/2 years and then a few days ago to Melbourne at just over 2 years of age.  The first time we travelled I wanted to take Lyla's oxygen with her, I had no idea how she would be on the plane, therefore I wanted it on hand just incase.  We flew with QANTAS and they had a very simple medical form that needed to be filled out, and on the days of travel we had no hassles.  

The second trip we flew with Tiger (rookie mistake) to Melbourne, once again I wanted to take her oxygen, but getting approval was hard.  I sent them a medical certificate which contained everything that the QANTAS medical certificate required eg. Lyla's condition, her symptoms, the oxygen tank size, the oxygen flow she needs if it was required, her carer (me), our travel dates, etc. but it wasn't approved.  I spent hours on the phone trying to sort this out, and I was being really annoying because they were being unreasonable.  Apparently all that was missing was a sentence that said "Lyla is safe to travel from such and such date, until such and such date".  I argued this because the medical certificate was filled out by Lyla's paediatrician, it included our flight details, including travel dates, and it was signed by him.  If she wasn't safe to travel it wouldn't be signed, right?  In the end I told them where to shove it and decided not to take the oxygen.  

And then most recently, Melbourne.  I knew that once a child turns 2 years of age they are required to pay for a seat and sit in it with a lap belt, this would be impossible for Lyla.  So before booking flights, and this was a 'last minute' trip, I called a couple of airlines to see what their seating options were for disabled children.  The options were a specialized CARES restraint, which I knew wouldn't work because of her kyphoscoliosis, limited neck support, tolerance and safety.  The next option was taking a car seat on the plane, and final option was a medical certificate which would have to say why Lyla cannot physically sit because she is disabled and it would be safe for her to travel on my lap, like a child under the age of 2 would do.  This was my preferred option, but I knew I wouldn't have the time to have a certificate filled out, sent off to the airline and approved, all before booking flights.  So car seat it was.  We ended up booking a flight to Melbourne with QANTAS, and back to Adelaide with Virgin.  Before booking, and after booking, I called the airlines to inform them I was taking a car seat in flight for Lyla and I went through all the safety checks with them, but ended up having to switch to an earlier QANTAS flight because the original plane didn't allow car seats.  It was all good...

That was until we arrived at Adelaide airport.  It took about 1 hour to have Lyla's car seat approved at check-in, but we managed to sort it out and had a few minutes spare before boarding.  We were let on the flight first so I could install the car seat.  We were on the very last row and we had all 3 seats in the aisle.  I asked the air hostess if it mattered which seat the car seat was on, to which she replied "You are meant to install the car seat" I said I will but I needed to know which seat it goes on, because it will obviously need an anchor point, she then told me to "Calm down".  My blood was about to boil, I cannot stand people like this.  She walked off in a huff and another air hostess came to help, I explained the situation, my ability to install the car seat myself, and I asked if she knew where the anchor point was.  Turns out they didn't, but an engineer came on and pulled the extension belt out from under the seat, which is what I needed to hook the car seat on to.  Easy. 

The day we left Melbourne, after the orthopaedic appointment, we decided to go to the airport early, have a coffee and look in the stores.  We arrived at the airport 2.5 hours early, and went to check our luggage in straight away.  We were flying Virgin this time.  I told the check in lady we were taking the car seat in flight and once again explained our situation, she said there was a note in the itinerary explaining this, but she was only new, so had not approved a car seat before so called the supervisor to check it.  The supervisor had never seen a car seat being used in flight either.  I had to explain I called Virgin and they suggested to use the car seat and that I went through all the safety checks with them and it was all approved.  After about 45 minutes of trying to work everything out, they ended up calling another lady who came down with a folder with all the safety checks.  They went through the folder for about 20 minutes trying to approve the seat, but apparently Virgin only approve AMERICAN car seats, that have to have a sticker on them saying 'this seat is approved for car and airline travel'.  Our AUSTRALIAN car seats don't say this.  They were at a loss.  I said our other option was a medical certificate so Lyla could sit on my lap, so they decided that was a good option and took us down to the airport doctor.  The wait to see the doctor was 1 hour, which by this stage we didn't have, so we nicely asked the 2 men ahead of us if we could squeeze in first, and they let us!  I go in with Lyla to talk to this doctor, I explained everything that had happened so far with the car seat drama, Lyla's condition and why we needed to see him.  I gave him a couple of medical notes I had in my bag explaining Lyla's condition, he then said "I am not approving Lyla to fly".  What the hell!?  He went on to say because Lyla has scoliosis that affects her lung function, therefore if she was to sit on my lap I would be restricting her lung function even more.  I told him Lyla travelled 6 months prior and sat on my lap, and her scoliosis has not changed since then.  He said it has increases significantly in the past 6 months, which is rubbish.  The only "safe" option for travel was to put Lyla in the car seat, the car seat that is not being approved.  We went back to check in and the lady was shocked and called her supervisor back.  Eventually they decided to call the flight engineers.  3 guys came down and said "Oh yeah, we can install this in to the plane somehow, we will go straight to the captain to get his approval first" (it's the captains choice at the end of the day) and then that was it, it was sorted, but by this stage we only had enough time to go straight to the gate. 

The ladies at Virgin were amazing, very helpful and very apologetic, but it turns out that they have never had anyone use a car seat in flight, except for Americans in international flights, so Lyla was literally the first disabled child to, which I find ridiculous.

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