Over a Week and Counting

We've now been in the critical care unit for over a week.  Ethan's oxygen needs have been going down and he looks great.  He is well rested and in good spirits today.  He still sounds a bit chesty but is doing pretty well.

Yesterday morning he woke up at 4 am and decided to party for a bit.  He had fallen asleep before I arrived at 9 am and had a long nap.  He looked so relaxed...

His oxygen monitor reads 17 and 60...this morning he is at 14 and 45.  :)  We're really going in the right direction.  He was given the green blanket above during his visit here but it was thrown into the wash here and lost.  :(  He also received a really nice e card from his friend Sophie.  Thanks for putting a smile on our faces Sophie!  It is nice to know you're thinking of him.  :)

Unfortunately, this morning he blew his 3rd IV.  I'm not sure if you can tell just how puffy his poor right hand is right now...but it is interesting to note that his fingers don't look quite so long.  His geneticist had said that his fingers look SO long (everyone points it out) because he doesn't have much weight on.  And now with his puffy hand, I can really see that his fingers don't look quite so long anymore.

Five Days and Counting

This morning at about 3 am marked the fifth day that Ethan is in the critical care unit.  His chest x-rays show improvement and he is back to his usual self, but is still requiring a fair amount of oxygen.  Due to his oxygen requirements, we'll likely still be here for a bit longer.  He is on his 3rd IV, has continued to removed some of his leads/probes, tried to remove his nasal prongs again and they have restarted his feeds.  His sleep is still all over the map, but he does get a few hours through the night.

In the above photos: the first one was taken on Friday afternoon when he started smiling at me and being more aware; the second photo was taken on Saturday - he was looking for me, smiling and giggling at me and playing tickle games; the final photo was taken yesterday when he was playing with his iPad and watching TV.

A couple of days ago he also tested positive for c-difficile again so has additional antibiotics on board to combat that.  A usual course to fight c-difficile would be to stop the antibiotics he was on, but since his chest is still quite weak, the felt it was better to continue the course and add the c-difficile fighting antibiotic.

We still do not have any news on his upcoming surgery but do know that he will likely have to be home and healthy for 4-6 weeks before they will be willing to mess with his airway to sedate him for traction...

Potential Delay in Plans

So seems our plans may be changed again...

Ethan went to therapy last week for his monthly stay and unfortunately when he got home on Sunday within 9 hours he started vomiting and had a fever.  He continued to have a fever, vomit and started to have really foul smelling stool through to Tuesday afternoon when I brought him in to be seen by a doctor to see if he had an infection.  His ears seemed like they could be on the verge of an infection so he prescribed something to stop him from vomiting and wanted to see us in 24 hours.

Meanwhile, on Tuesday we got the news that Ethan's Shilla procedure has been approved!!!  :)  Plans for his traction and surgery are in the process and as I hear more, I will post it.

Back home with Ethan's anti-nausea meds and he did stop throwing up but his stools were getting looser and he started to have respiratory issues.  He did great with the anti-nausea meds for his first two doses but then he started to throw up just before his next dosings.  Just over the 24-hour mark, we brought him back to the doctor he'd seen on Tuesday and at first things looked alright...then they checked his oxygen saturation levels.  Now I know there is a chance their equipment wasn't reading well...but he was reading at 61at the low end and 81 at the high end but a lot closer to the lower end for much of the time.  They tried to do a nebulized (wet) treatment to help open his airway but then had to set up an oxygen tank.  They called the paramedics and we took a bumpy ride to the hospital ER.  His oxygen saturation was still an issue, but wasn't as low as at the clinic (he'd also received oxygen through the ambulance ride).  After several hours in the ER and discussions on where they would admit him, we were admitted into the pediatric critical care unit.  Since his only really need was for oxygen, they had hoped to get his needs low enough for them to send him to the floor to recover...but he just would not tolerate having his oxygen level reduced.  In the unit he was on 100% oxygen for over a day. 

Yesterday they tried to wean him down during the day and we got as low as 70% but at night we had to go back up to 80%.  Today while he was awake they were able to bring it down to 65% but as he falls asleep they have to raise it again.  We have sent several 'failed' stool samples - apparently formed means something different to the lab than it does to us - and his nasal cultures came back positive with metapneumovirus.  Despite still working hard to breath, ending up with a useless IV (that had to be restarted - 4 try trauma) and having blood drawn frequently, he is pretty alert and active.  At times a little too alert.

He pulled out his nasal prongs and tried to challenge himself without oxygen - he failed and has pulled off a couple probes, his oxygen saturation monitor, and keeps fighting sleep - which he desperately needs.  If we can get his oxygen needs down we can move up to the floor for continued recovery.

What does all of this mean though for the progression of surgery?!?  We're not sure yet, I'll touch base with the surgeon's office next week to see if we have a plan we can proceed with...

Best Laid Plans

Ever notice sometimes no matter how well you plan for something, a small thing can fully change your plans?

Okay, so in this case it wasn't something small it was something totally out of the blue.  We found out yesterday that Ethan will not be proceeding with spinal surgery next week as tentatively planned.

You might think it is because he is sick with a cold or something...but you'd be surprisingly wrong!  Ethan is in good health right now and doing well.  It is because plans have changed.  Ethan's images were shared with several orthopaedic surgeons over the past month and the one thing everyone can agree on (including us) is that he needs surgery.  It is just what type of surgery that is now in question.

Given the severity of Ethan's scoliosis, the surgeon would like to start by putting him in traction.  Not the type of traction you usually see in movies, but Halo traction...which the surgeon describes best as a crown of thorns.  This would be for about a month and then he would like to proceed with Shilla spine surgery.  It is considered experimental and new (has been around for about 10 years), but it borrows elements from the tried and true fusion or growing rods surgeries.  It would mean that a small part of his spine (where the scoliosis is the worst) would be fused and then rods would be placed along the spine with special screws that allow the spine to grow as it normally would and the rods would expand (or grow) with the spine.  It would significantly reduce the amount of surgeries he requires which would reduce the numerous risks for infection related to multiple surgeries.

We do not have any details yet on when and how we will proceed but hope to learn more later in the week after the surgeon consults with Ethan's neurosurgeon.  It sounds very promising though...

I keep falling behind in posting...

Late in January, Ethan got one of his 4-6 year immunizations and had a bit of a reaction to it again.  This is the second time he's had a reaction to an immunization but it wasn't as bad as the one from his flu shot.  He also saw his neurosurgeon for a follow up appointment and we discussed his December MRI.  His imaging is a bit of a mystery (as it always has been).  The space where his Dandy-Walker Cyst is is still quite large but it is not clear whether or not it is filled with fluid.  Since he is not showing any symptoms we're going to continue to monitor him especially since his back is more of an issue right now.  Ideally once things are underway with his scoliosis repair, the neurosurgeon would like to see him orally feeding.  Apparently it is a good way see how the cyst is affecting him...but it would be hard to tell if his swallow issues are because he hasn't orally fed since November 2010 or if it is a neurological issue.  Kind of a catch 22...

On the plus side, Ethan's neurosurgeon has agreed to be available for Ethan's tentative surgery date on March 26th should the orthopaedic surgeon be willing to have an extra person in the OR.  We need to find out where the VEPTR will be placed to be sure it avoids the shunt area.

We also saw his cardiologist last month and we won't have to return for 2 years now.  His ASD has corrected but his left pulmonary artery is still constricted.  It does not seem to be causing his heart any undue stress so we can just monitor it for now.

Recently Ethan was working with a communication iPad for about 4 weeks and he did pretty well.  He mastered the phrases, "I want to watch cartoons" and "I need help".  With a bit of help he was able to 'say' "I want to play with my iPad".  It was pretty neat to see the progression.  Since it was just a loaner, we were only able to use it for the 4 weeks.  They had a team meeting and decided that they support the idea of proceeding with another iPad for communication purposes.  We are now awaiting its arrival.  An interesting development recently is that he is pitching full out temper tantrums when things don't go his way.  I think a lot of it is age appropriate behaviour, but also think that he is feeling frustrated.

Ethan also tested a new standing frame recently.  It is the third one we've trialed.  It has been a long time since we've had a standing frame in the house and he did really well with it and seemed to enjoy it.  I think he likes the different vantage point.  Surprisingly we were able to trial it for longer than was initially anticipated which was great but now we are without again.  It is the one national product we tried and it has been our favourite.  We need to wait for everything to come through on his walker before we can submit a request for funding assistance with the standing frame.  While it was here, we had him in it 2-3 times a day as time permitted.

His respirology appointment went well, they said his lungs sounded really great and that he appears to be doing really well.  She thinks that the VEPTR surgery would be of benefit for Ethan.

He had his second hair cut and it went pretty well (although there were more people at the salon than we would have liked).  Lorelei joined us and wanted to be part of the photos.  He looks so cute with his new haircut (large one below)!

Also big news...my baby boy turned 5 last month!  Can you believe it?!  We were not able to celebrate with him on his birthday as he was in therapy.  We did pick him up early from therapy but that was because he started to show symptoms of being sick with a cold.  We celebrated with an ice cream cake and let him try a taste of whipped cream (top centre picture below)...I think most of it came back out of his mouth as he didn't quite seem to know what to do with it (or didn't like the taste/sensation).  He did enjoy opening his presents and has likes playing with his new toys.

 

With his upcoming surgery, we felt it was better to move Ethan from his crib to the larger bed that Lorelei was using in a previous post (she is now using a twin bed which friends have loaned us).  With spinal correction, there is a high likelihood that he will grow an additional 2-4 inches so we thought that it was better to have him used to the larger bed before he comes home from surgery.  I think he likes the extra space...but our bedroom is now even more cramped with his bed in there - we sure hope we have success in finding a new home soon...

 

 

A New Year and a New Plan

Have you ever watched those shows where they kind of flash forward, to the past and back?  Well, I thought I'd try to write that kind of post this time.

Early in January we met with Ethan's GI doctor who after feeling his abdomen suggested there might be some stool blocking his system and ordered a set of x-rays.  Since Ethan has been x-rayed and CTed so much that I'm quite surprised he doesn't glow in the dark, I requested (and he agreed) to combine x-rays with those for his orthopaedic surgery follow up later in the week.

When we went for Ethan's x-rays, I had explained to the technician that we were hoping to reduce some of his exposure.  She said that since a spinal x-ray is a tight picture of the spine we would not be able to reduce the amount of x-rays but at least it was just one trip. 

After the first x-ray she came back to redo it.  She said something like, "I need to retake that x-ray since I did not capture his cure in the image.  I knew he had a curve, but did not think it was quite that much of a curve."  I had a duh moment where I thought I should have told her that when she said it had to be a tight spinal x-ray.  I knew his scoliosis was quite pronounced (heck, I've been with him for every one of his x-rays).  So I joked about having been right that we could have limited imaging.  I also (in a straight face) said to her, "So...what you're telling me is that we're going into surgery?!?"  She was a bit flustered and said, "I'm not saying anything..."  And I cracked to let her know, we knew the curve was bad and we know we have to go for surgery, we just don't know when.  With their PCs down that day, I figured a little ribbing might lighten their Friday.

As you can imagine all kinds of thoughts were whirling through my head over the weekend.  On Saturday night, I had a dream and in this dream I so vividly (through two different x-rays) saw the poop in Ethan's GI track.  One x-ray showed it from the front (roughly 2 inches long) and about an inch thick from the side view.  My mom suggested, if I'm dreaming about it then he must not have a blockage.

The end of the weekend, we received an email from Ethan's GI doctor...it was his spine that he was feeling.  "It is incredible the degree of scoliosis," which he didn't appreciate before.  Ethan doesn't have any stooling issues and we're to keep things up as we have.  A bit of good news at least.

We had our follow up appointment with the orthopaedic surgeon the same day we did the x-rays and he is quite thrilled with how well Ethan looks overall and that he has been pretty healthy over the past year (we've been getting a lot of these positive comments lately).  He also feels that Ethan has gained nicely and should have enough padding for when we start with his next round of surgeries in March.

That's right, I've beat around the bush enough and come to the point.  The surgeon has decided it is time to start with Ethan's scoliosis repair.  We're still planning to move ahead with the VEPTR and the March date hopefully allows for a few things: 1) them to secure the equipment required, 2) it is later in the season so should hopefully not be a very risky time for Ethan to be in hospital, and 3) for Ethan to be healthy enough to proceed.  The surgery takes up to 6 hours and will require about a week long stay in hospital to start recovery.  It takes a couple months to fully recover and then he is monitored frequently as due to growth they have to expand the VEPTR or risk breaking his ribs/getting over correction causing the scoliosis to curve the other way.  Typically they are revised every 4-6 months and fusion surgery often doesn't occur until earliest 10 years of age...which will mean a minimum of 10 surgeries once we start.

I must say I wasn't surprised to hear that we're at the stage to start with surgical intervention.  We've been trying to put it off in non-surgical ways but are only able to maintain him a bit.  And if you factor in the above comments, it just illustrates how bad his scoliosis has gotten.  BUT...I'm nervous!  Before heading back home, mom asked me not to research the surgery or stress too much.  I can't promise either but am trying to at least hold off on some of the worrying.

Rounding out 2012

Wow!  I started this original post when there was just 9 days left until Christmas Eve...and now we're into the New Year!  It is crazy how fast 2012 flew by!  We weren't even close to ready for the holidays when I first started the post but we did get everything done in the end that we hoped to...or I've forgotten what it was I wanted to do if we didn't.  ;)

The kid's cousins on Clayton's side and their family have recently moved closer to us and we have had the opportunity for some really lovely visits together.  Lorelei is delighted to have her cousins come visit her and to share/show them her stuff.  (She doesn't often get to have friends over to play so she crams in all the playing she can with her cousins while they're here.)  I think Ethan enjoys their visits as well. 

This winter we did do something we haven't done before, and Ethan got to join us...

We went to the local Santa Claus parade!  We bundled up the kids, grabbed coffee & hot chocolate and headed out.  I think overall it was a pretty good outing...but it was bitter cold!

Ethan, Kalianna, Auntie Pengo, Mariella, Clayton & Lorelei watching the parade.

All bundled up, Ethan tolerated most of it, but by the end he was really cold and it took a bit for his hands to warm up again.  Thankfully his Aunt is better prepared and let us borrow some leg warmers to keep Ethan's footwear on.  Mental note: Next time bundle up better and remember mitts and hats for everyone.

Ethan's incision site has healed well and looks great!  He now has matching incisions (which was planned, I asked the surgeon ;)).  The only other medical thing for Ethan last month was his MRI...and we'll know more about the results later this month.

This year we decorated earlier than we have in the past couple years and Lorelei took the lead role on decorating the tree.  She did a great job and only needed a bit of coaching to make sure decorations were all over the tree and not clumped together.

Ethan watched from his play spot on the floor, even reaching out for decorations as they were being put up and from the tree at later times.

 

Ethan lost another tooth (or two?) mid-December, it has been a while since he lost a tooth but I was quite thankful that it wasn't one of his adult teeth.  We were lucky enough to capture it to be sent for analysis.  I do not anticipate it being different than all the other ones we've sent.

 

 

The rest of the month we pretty much got ready for the holidays and everyone but Ethan worked at recovering from a cold.  Ethan spent some time chill-ax-ing and watching TV in an easy chair.  (His right eye is patched to make him work his left eye and retain vision in that eye.)

 

Recently we'd picked up a larger bed that Lorelei has been using, and for the first time in about a year or so we put Ethan in with her for a little visit.  She loves it when he comes to her bed and this was no exception. 

 

 

Ethan did a great job opening presents this year.  We got through all of them in one day and he unwrapped (or at least partially unwrapped) all of them.  He ended up with a lot of fun toys.  The following picture is him opening up a really neat musical toy that I'm sure he'll love once he fully explores it.

 

 

One gift for Ethan was a big disappointment.   We'd picked up an expensive, geared to children, stylus for use with the iPad but it did not light up the choices/information so is useless for him. In an interesting turn of events I found a stylus for $2 that works perfectly for him. It is a bit slimmer in build but he was using it and choosing it for hand-over-hand connect the dots numbers.  :)

 

Kalianna, Mariella and their parents came for another visit over the holidays and the kids had a great time playing together.  We took them out one day to an indoor play space.

  

 

Auntie Pengo & daddy also got in on the action.

 

 

Then we took them sliding to wear out any energy that might be left...but I think the parents were more tired than the kids in the end.

 

 

While on the hill, Lorelei and daddy even got some airtime.

 

 

It was a great outing although quite chilly for the photographer - nothing a round of hot chocolates didn't fix.  Lorelei was quite thrilled to have so much company over the holidays!