No More Tears

Today was the first of the two eye surgeries that Lily needs to have done. We, mistakenly, thought that they were going to do both procedures at once but since that is not the plan we went ahead with the first one today – a simple tear duct probing. Lily, along with a ton of other kids, has had an issue of blocked tear ducts. It’s especially common in kids with Down syndrome and a lot of the time it resolves itself. When it doesn’t, as was the case with Lily, they go in and do a simple procedure to unblock the tear duct. We were actually shocked at how fast it was (less than 45 minutes) and while they had originally recommended that we spend the night, anesthesiology was so happy with how she held up that they let us go home right away. While we were originally a bit hesitant (our post-traumatic stress coming out), now that we’re home, we are grateful to sleep in our own beds.

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It was an odd place to be in emotionally today. We knew, logically and without a doubt, that this was the easiest surgery that Lily has ever had and we love her ophthalmologist so we knew she was in great hands. But we kept flashing back to June 2011 and thinking something similar – this is a regular surgery, it’s common for kids with Down syndrome and they see it all of the time. But that surgery changed our entire lives and while we knew that it wasn’t going to happen again that worry is a hard one to put to rest. It certainly didn’t help that as we were having our last discussion with the anesthesiologist a code blue was called in the recovery unit. It was just another reminder than even when you’re not expecting it life can hand you totally new cards. But we had to out those fears aside, kiss our girl goodbye (who barely even noticed we were gone – she went happily with the nurse!) and hope that we had time for lunch (we barely did).

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So now we’re home – safe and sound and with way fewer tears than before. Lily did not enjoy the recovery process and cried/shrieked for the entire car ride home. It wasn’t until after she crashed for a good long nap (with me right beside her) and got some food in her tummy that she started to come around. And now, even though she still seems a little punch drunk from the medicine, she’s back to her funny ways. In the end, while it wasn’t a good day, it certainly wasn’t a bad one.

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Head and Shoulders…

I sometimes feel as though that as a whole, we need to look at our calendars and start thinking about adopting Rosh Hashanah as the new year for everyone.  I don’t think that I’m alone in thinking that September always feels more like the new year than January does.  Be it the years of school starting, the fresh winds blowing out the hot stale air of summer or the fact that I work in Aquatics and at the end of every summer we get to relax a bit and breathe a little easier, but in my heart of hearts, fall is the start of the new year for me.

It was felt incredibly true just this past week, as Lily and I strolled out of Holland Bloorview (a kids rehab hospital here in Toronto), feeling as though we had uncovered some holy grail of support that we didn’t realize had been missing from our lives.  Well, that’s not entirely true – we knew it was missing, so maybe it’s more accurate to say that we felt as though we had uncovered some holy grail of support that we didn’t realize we were longing for.  But, in that moment, walking in the sunshine, I felt as though we were starting fresh.

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If only you knew how long it took to get her hair into braids. Sister hates having her hair done.

For the past year, Jess and I have been watching Lily’s weight and growing more concerned.  Lily is a tiny girl and always looks much younger than her actual age (we call it her chronological age – more on that in a bit) and while we tend to embrace it and laugh about how lucky we are that we get a baby for an extra long time, since December we’ve been noticing that she hasn’t been gaining weight.  Notice that I didn’t say “much weight” or “weight steadily”, I’m saying weight – any weight.  In fact, Lily has only gained 1 pound in the last 12 months – from 21 pounds in October 2012 to 22 pounds in September 2013.  Obviously, this is not an ideal situation – kids are supposed to grow, and while she’s clearly grown in height, her body weight is not keeping up with her.  Our pediatrician kept telling us that she was “growing so nicely”, and other people would comment on how big she was getting, but we knew that this wasn’t right.   We kept saying to each other that what she really needed was to switch to a specialized formula so that she could take in more calories, but when we asked the dietician, she simply switched us to a different formula with the same calorie content and told us to “make her eat more.”

This statement simultaneously made us laugh and want to outlash violently at her.  “Oh, is THAT all we have to do?” Make her eat more? Clearly that’s the answer we’ve been missing all of our lives!”

I’ve talked a lot about Lily’s heart and her brain and her eyes, and while I’ve mentioned the g-tube and the need for it, I don’t think I’ve explained the history of why eating has been such a challenge for her.  When Lily had her first open-heart surgery, she was 7 days old and incredibly tiny.  At somepoint, during her intubation, they severed one of her vocal cords (sadly, this is common in pediatric heart surguries).  In order to give her vocal cords the time to heal, they began to feed her through an NG (nasogastric) tube.  This is just a thin tube that winds down from the nose and down into the stomach.  Once it was safe for her to eat again, it was discovered that she had developed a feeding aversion.  Again this is something that is very common in kids who’ve had a surgeries at a young age, as they’ve been traumatized from the multitude of intubations, tubing and masks that they’ve had to put up with and now don’t trust anything around their mouths.   Because of that aversion, Lily stopped eatting anything orally and then lost the ability to suck, swallow and breathe while drinking from a bottle.  When we adopted Lily she was still being fed exclusively through the NG tube and that just became part of our life.

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The hippo was just one of Jess’ many amazing hypafix animals used to hold the NG tube in place

After her second open-heart surgery and cardiac arrest, we thought we had come across a small miracle – in what we started to call, “the reset”, Lily suddenly gained the natural inclination to suck and so we started to work on feeding her again with the help of our amazing OT Lisa from Sick Kids.  Sadly, the miracle was a little short lived, when we found out, via a video swallow study, that Lily was doing something called “Silent Aspiration”.    For most people, when food or liquid goes down “the wrong hole” (the trachea), we cough or choke or gag because this is our bodies way of protecting our airway against something that’s not supposed to be there.   In Lily’s case, her body doesn’t protect itself and anything thinner than the consistency of pudding would go right down her trachea and into her lungs.  When this happens, it puts a child at risk for many complications, including aspiration pneumonia (think of secondary drowning).  What this means, is that while we’ve been working on feeding we have had to be incredibly mindful of what her body is doing when we’re trying to get her to use her mouth to eat.  We’ve had to limit the types of food we can give her, thicken them to make them safe and then constantly monitor how’s she swallowing them and whether she’s just letting the food go down or if she’s controlling what happens to that food.  The other part to that is that when she’s sick or even when she’s teething – anything that causes some extra buildup, we need to limit what we’re giving her because her body can’t handle the extra work.  Once we were released from Sick Kids, she was doing very well and we were really happy with the progress she was making, but then we had a much bigger set back because of the seizures that she developed.  The medication that they used to treat the seizures caused Lily’s muscle tone to weaken, which in children with Down Syndrome, is usually quite weak to begin with .  This meant that she no longer had the muscle control to eat safely.  That put an end to feeding for quite some time and it’s been a large struggle since then to interest her in eating and she’s lost most of the natural skill that comes with doing something over and over again.  So clearly, when we’re listening to someone say, “just make her eat more,” we laugh at them because if we could be doing that, we would have been doing that.

That’s where Holland Bloorview comes in.  This amazing facility has a feeding clinic that is staffed with doctors, occupational therapists, speech and language therapists and dieticians.  They all work as a cohesive team to observe and treat patients who are struggling the same way that Lily does.  Within an hour of our first appointment, they were giving us new techniques – ones that were right for Lily (which we could tell because they started working right away!), a plan for a follow-up swallow study to re-evaluate what is safe for her to eat, and they were putting her on a newer, high calorie, formula for her g-tube, which will help her gain weight and really start to grow.  Beyond that, they simply validated all of the concerns about her weight that we’ve been pushing for a year and really truly listened to what our concerns were for Lily’s long term ability to eat typically.  As nice as the g-tube is when we’re out and about, it would also be nice to watch Lily scarf down a bowl of pasta with her cousins.   I walked away, feeling empowered knowing that we had been right all along and we really do know our daughter best.

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Trying to poke Hef in the eye under the watchful gaze of Uncle Jeff

Just a bit north of her mouth, we had Lily’s pre-operative appointment with the anesthesia clinic at Sick Kids today.   A cancellation opened up a spot for Lily’s eye surgery sooner than we had anticipated and so she will now go in for the procedure on September 23rd.  There’s some confusion around what exactly is being done (one procedure or two), and so we won’t know how long we’ll be at Sick Kids until early next week when it’s clarified.  If they do the more difficult surgery – to help fix the strabismus – then anesthesia is going to recommend that she stay a bit longer for observation, because they will have to intubate her fully and that carries higher risks because of her lungs.  To be entirely honest, Jess and I would actually prefer that option.  We just know that Lily has a history of being a bit….tricky….so we’d rather be overly cautious, even if it means a night away from our own beds.

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So relaxed during her ultrasound – you’d think she had done it before…

We also made time for an ultrasound this afternoon to look at a funny anomaly on Lily’s chest.  Over her left nipple, just up from the scar from her first open-heart surgery, there is a small section that, for lack of a better word, pops out whenever she is crying or whining or doing anything that takes a little extra effort.  We were not very concerned with it previously, but mentioned it to our paediatrician with the thought that if it could be repaired, it would be ideal to have it done while Lily was already under anaesthesia, as opposed to bringing her back at a later time.  After looking at it today with the radiologist, it seems that it’s actually a section of her chest muscle that is thinner than it should be and so what we’re seeing is her lung expanding when she’s breathing harder.  It wasn’t confirmed with us, but it sounds as though it’s not something they can repair and so it’s just going to be lumped into another one of Lily’s lovely quirks.

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Making faces at Abby – because they’re best friends.

Can You See the Difference?

Guys, do you remember what you were doing one year ago today? Because I do…well, kind of.  At this time, one whole year ago, I was actually pretty out of it because I had just come out of my second open heart surgery.  You see, when I was born there were some big time problems with my heart – I had something called an AVSD and coarctation of the aorta.  When I was just 7 days old, and still with my birth parents, the doctors at Sick Kids did my first open heart surgery to make my heart a little better so that I could get strong enough and big enough to have the second surgery and on June 16th last year, they decided that I was finally ready.  Now, I don’t remember a lot of the details (mostly because I was on a lot of drugs) but the mom’s say that today was the first day of a very scary 3 months for them.  My heart surgeon was actually really happy with how well he was able to fix my heart, but as most you know, it was during this surgery that he officially diagnosed me with Pulmonary Vein Stenosis.  He was such a great surgeon though, that he tried to fix that (as best he could) at the same time, so that I wouldn’t have to have ANOTHER surgery, but because he spent so much time playing with my heart, it was too swollen for them to close my chest and I spent 3 more days with my sternum open to let the swelling go down.  Those three days were pretty scary for the mom’s.  I hadn’t even been home with them for 3 weeks and suddenly the heart problem that they had expected from me turned out to be way worse and now they were hanging out in a CCCU room actually being able to look down and see my heart beat.  It was pretty surreal.

 

And now here we are, one whole year later, and not only is my chest closed up all nicely and my heart is amazing and healthy and my right lung is strong and working hard, but I’m also sitting and seeing and have a tooth.  And I’m playing in the sand for the first time ever, and hanging out with the mom’s and their friends at the park and trying ice cream and slushies (thanks Rachel!).  I don’t know if you guys know, but us babies do a lot of growing up in one year…..

 

 

 

 

 

 

A Rare Face…

A mom post tonight.  They were able to pull the temporary Ng tube yesterday because our Lily is doing so well with her G-tube feeds.  It’s a gorgeous face that’s been lurking behind that tube.  A tube, that we’re now seeing must have been far more irritating and uncomfortable then we had ever thought because the personality change we have seen in the last 24 hours is remarkable.  Lily has always been a happy, content baby but now she’s smiling all of the time, she let me brush her hair (getting all of the tangles out) and put in the pigtails without a peep and even let the nurse and I do her dressing change (which normally causes a total breakdown) without a single tear.  Between stretching out her vein and getting rid of the Ng tube, it feels like we’re bringing home an entirely new kid.  She’s amazing.

Lily – the (Ng) Tubeless Wonder!

I have had a really great week.  I was worried about saying that because it seems that I sometimes manage to jinx myself and say that things are going well and then something happens and then I’m stuck telling you about something crappy.  But so far this week is pretty good – I mean it is only Tuesday, but we take the good things as they come.

I had my heart catheter last week to try and patch up my tiny pulmonary vein.  In the end, the surgeon was not able to put a stent in because it’s made of metal and it would have blocked another vessel (which kind of defeats the point of putting the stent in).  That was not ideal because the stent really was the best option for my last left vein.  In the end, they were able to open a tiny little bit of my vein and it’s helping because my oxygen levels are sitting much higher but it wasn’t really a lot.  Now my left lung is only doing about 2% of the work, and my right lung is doing 98%.  It’s a lot of pressure on that one lung but hopefully this catheter. procedure will help to keep the pressures in my lungs lower than they were before I went to the catheter lab.  Now we just have to hope that it works for way longer than 6 weeks this time.  I get to see my cardiologist in 6 weeks and we’ll have a better idea then on how we move forward from here: how they’re going to monitor my veins and what other treatment options there are if my right lung decides that it doesn’t want to do as much work as we’re asking it to do.  My cardiologist did say that as long as my heart is good, she has kids who are 6 and 7 years old who just have one working lung so that’s what I’m aiming for!

The doctor’s were so impressed with how I was doing that they decided that I could come off of the ECG monitors completely and off of the oxygen monitor while the mom’s were awake (it goes back on when they’re sleeping so the nurses can track me).  I think that’s what made it the best week ever.  On Sunday, I had lots of visitors because it was Grandma’s birthday, so she and Grandpa and Uncle Jeff and Auntie Marina came to have something called birthday cake.  I got to try a little bit of this icing stuff and I really liked it.  But mostly, I liked being able to cuddle with Grandma – she’s so comfy that I went right to sleep.  I would have slept for Uncle Jeff but he kept sticking his tongue out at me and trying to make me laugh.  I decided then that I like Auntie Marina better.

Then on Monday, I got to eat from my bottle and I did really well.  Just as Mama C was attaching my Ng tube to top off my dinner, Auntie CC, Uncle Rico and my Thor came to have their dinner with me!! (And bring Mama C some dinner too).  I had so much fun! Mama C put the mat down on the floor for me and my Thor and we hung out and played for so long.  He cuddled me and called me his “sweet sweet baby”, and he patted my tummy, sang me songs and then tried to teach me how to hold my feet in the air.  I’m not quite strong enough to do it, but if Thor just waits a little bit I bet that I’ll catch up soon.

Then today was G-tube day! Finally, after all of these months of having this stinking Ng tube in my nose, the doctor’s took me to the operating room this morning and put in my Gtube.  I still have a temporary Ng tube in, just in case we run into any problems and they need to feed me or give me medicine, but later tonight (12 hours later), the nurse will give me some clear fluids and I’ll start using my new tube! The mom’s are really happy that they’re going to be able to see my gorgeous face all of the time now but they were a little sad when they had to go to the Specialty Food Shop and buy my new G-tube pump, feeding bags, extenders, clamps, clips and doo dads.  I don’t know what any of this stuff means, but if it means that I get to eat without throwing up, then I’m all for it.  Plus it did come with a cool backpack that made Mama C happy – she says that it’s the perfect size to throw on my stroller.

So, I’m in some pain tonight, because the G-tube does hurt a little bit at first.  They gave me some morphine earlier but now I seem to be doing okay just on Tylenol.  Mama C asked the nurse to give me a little more morphine around midnight so that I sleep really well and the pain doesn’t wake me up, but hopefully tomorrow it will be even less and I can go back to being the happy, smiley girl that I usually am.  Then the doctor’s say that if everything heals well from my G-tube, I could possibly be home before the weekend! I’m not counting on it, but a girl can dream….

Another Week End, Another Surgical Adventure Begins…

The general consensus seems to be that I look really really good for someone who has so many things wrong with me.  I’ve been eating like a champ this week and more importantly, eating like a champ from a bottle.  My OT Lisa, says that it’s safe for me to try 3 meals a day from a bottle and at each of them I’m getting more and more confident about how I eat and I’m taking around 60mL each time.  Impressive, if I do say so myself, for a girl who couldn’t even suck a few weeks ago.

The rest of my OT exercise is coming along really well too! Lisa is very impressed with how strong my neck muscles are getting again and how strong my right arm is now, especially since it was just last week that it was laying limp beside me.  It seems that at some point this week, I not only remembered that it was there, I decided to start using it all of the time! I’m sucking my thumb again and grasping things (like the mom’s hands) and I’m even holding on to a set of jingle bells and moving them all around so they make beautiful music.  We won’t talk about how I often hit myself in the face with the jingle bells because, well, that’s just embarrassing.  My legs still need some work – I move them a lot when I’m angry or frustrated, but they don’t move around a lot more than that.  I also need to work more on my tummy time, because I really really hate it, but it seems that no one is doing anything to really make me cry these days.

On Monday, I had another Echo done so that the doctors could look at my heart and see how the blood clot was doing.  About an hour after I got back to my room, my cardiologist came upstairs to “see me with her own eyes” (her wording, not mine).  It seems that after all of that worrying we had done since Friday, the Echo showed that my blood clot was gone.  They didn’t know for sure where it seemed to be – either it had been broken down by the Heparin that they started or it had dislodged and passed into my lungs but in small enough pieces that it didn’t cause any problems.  My cardiologist was shocked and said that while this was the absolute best case scenario, she wasn’t expecting it at all because the clot was quite big in the pictures and they were pretty certain that it was going to cause way more problems.  She told the mom’s that they were still going to do the MRI that they had scheduled for Tuesday, just so they could see if it was in my lungs and to look at my brain to make sure there wasn’t any bleeding (because of my brain injury, Heparin can make bleeding in my brain more likely).

I had the MRI on Tuesday and that was a long day.  After it was all done, I really didn’t want to sleep and so I stayed up really late and even pulled out my Ng tube (I told you my hands were getting better!) just so that I could stay up late with Mama C.  The mom’s didn’t hear anything about the MRI results though until Wednesday morning.  That’s when my cardiologist came upstairs very quickly to let the mom’s know that they had decided to move me back down to the cardiac floor (my home!).  During the MRI, they were able to see my pulmonary veins (the one’s I talk about here…. https://giggleandhugs.wordpress.com/2011/06/18/you-take-the-good-you-take-the-bad/).  It turns out that my surgeon tried to open up the veins a bit during my AVSD repair and they were hoping that this would buy me 6 months of time before it became an issue again.  In the end, that work only bought them 6 weeks of time.  On my left side, the lower pulmonary vein is completely dead and the upper one only has a tiny bit of blood flowing through it (about 10% of all the blood – my right side is filtering through the other 90%!  This isn’t fair to ask my right lung, especially without a lot of notice, so the hope is that they can fix my veins for a long enough time to allow my right lung to learn how to deal with all of that blood working it’s way through.

So, tomorrow morning at 9am, they’re going to come and take me to the catheter lab, where they will insert a catheter which will snake it’s way down to my vein and use a balloon and a stent to open it back up.  They don’t know how long this will last and they can’t even say what they would do for a “typical” child – because apparently this is incredibly incredibly rare (2 in every 10 thousand!) and so they take each case at with their own set of challenges.  The plan is to avoid a heart-lung transplant, which could be an option but only in a worst-case scenario.  Apparently the survival rate for infants/babies and transplants is pretty low, so we want to make sure, that if we get to the point where the doctor’s think a heart-lung transplant is my only option, I’m as big and strong and healthy as I can possibly be!