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! 

Neurology...

Yesterday Lyla had an appointment with her neurologist, Nick Smith, at the Women's and Children's Hospital.  It was a pretty standard appointment, he was really impressed with Lyla's growth and development and he was excited to see Lyla was so happy and smiley.

I mentioned some of my concerns regarding her breathing episodes and how they have started to reappear quite bad, often causing her to pass out afterwards (there was a period of time where she was having very few episodes, or if she did have one she wasn't struggling to catch her breath as much, therefore not going as blue).  He thought it could possibly be due to low iron levels, which if the case makes sense because around the same time they were not as severe I was giving her extra iron.  He suggested to test her iron levels and a few other bits and pieces with a blood test.  

Along with that I mentioned the tachycardia spells she has (rapid heartbeat) with an episode, and when she was in PICU after her gastrostomy surgery the heart rate monitor was reaching 250 BPM!  He referred us to cardiology again to have an ECG to check her heart out.  I was hoping he would.  He also referred us back to respiratory (happy about that too), the only time we saw respiratory was when Lyla was 7 weeks old and spent the week in PICU.  I always found that strange because she obviously has respiratory problems.  He will also recommend a swallow study to see where everything goes when she swallows.  This was something we have had booked a couple of times, but it was always cancelled.

So I'm really happy that we're re-seeing a couple more departments, this was we will also know if the scoliosis is damaging her heart and possibly contributing to these issues.

...

After the appointment I had a few other errands to do at the hospital; we went to the HENS department (home enteral nutrition service) they showed me how to change Lyla's Mic-Key button, so I changed it by myself and now I have a back up one so I can do it at home next time.  We picked up some medical supplies and paid a pile of outstanding invoices (whoops).  I also filled out paperwork to request a copy of all her medical notes (there's A LOT of them) and put in a request for a DVD of her spinal x-rays for our upcoming orthopaedic appointment in MELBOURNE - Feb 19th!!

 We're seeing Australia's #1 orthopaedic surgeon!

Jess

Scoliosis Check-Up, Part 2

Yesterday Lyla had her 1 month orthopaedic follow up, at the Women's & Children's Hospital, for more x-rays and I guess a bit more of an idea / plan with the MRI and surgery on her spine.  

We started off with having several x-rays done with the doctor as he wanted us to pull and stretch her out as much as possible so he could see the amount of flexibility she has in her spine; this turned out to be very unsuccessful, as he told me afterwards, but I knew that anyways as she was upset, therefore tensing and not relaxing.  After this traumatic experience we headed back down to clinic where I was expecting to be told the big plan with the surgery, something I had prepared myself for over the last month.  Well, things apparently changed...  Dr. Selby (orthopaedic surgeon) came in and said 'I'm going to put her in a cast'.  Huh?  What?  It seems Lyla is still too small for surgery and the nuts and bolts don't come in little baby Lyla size, and she may not have another year or two to leave things how they are until she is big enough.  

As I mentioned in the last post about this degree of curvature causing lung and heart problems.  Leaving her spine as it is until she is big enough could, and possibly will, kill her; her lungs will not grow and develop.  

So she will be having a cast called a Risser Cast put on to control, not correct, her spine until she is big enough for surgery; he suggested this last year, but due to all the other health issues she was having it was not a good idea.  At the same time, she may be having her hips cast as they have always been dislocated, but he will discuss this with her other orthopaedic doctor, Dr. Allcock.  The casting is done under a general anaesthetic, so I filled in admission papers yesterday and it is scheduled for January 29th, 2014.  He still wants the MRI, so that will be done under the same GA.  The cast only comes off for re-sizing and will remain on for 4-6 months!

I've tried to research Risser Casting, but I can't find a whole lot of information on it, as it is an older style of casting.  I've read it goes under the armpits down to the hips, but I've also read it goes over the shoulders to the neck and sometimes past the hips.  I think the thought of her being in pretty much a full body cast (hips casted too if they decide to fix the hip dysplasia) is harder for me to accept than the surgery.  I've had so many questions pop into my head since the appointment - the worst I keep thinking is what if something happens to her and she needs CPR?  

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