So glad to be on the other side of that!

As much as I know about Tiernan's heart and the impact it has/will have on his life, every time he has a procedure or a check up I come away with new knowledge.  Some good, some overwhelming, some mind blowing and some really defeating...IF I allow myself to dwell there. Which for now, I don't.
But I digress
.
Pre-Fontan Cath Results:
Hemodynamics look good and there is no reason to suspect he won't accept the Fontan physiology when the time comes.
The question, of course, is when will that time come?  Soon-ish.  Because there isn't an emergent need, (like there was for his first two surgeries) we can negotiate a little.  There is a difficult balance between doing what he needs and getting a timeline that works for everyone.  The docs came to the table with October.  Git er done before the dreaded CAFS (cold and flu season) kicks up.  But as teachers, that is pretty bad timing because we would need to be out for however long his hospital time dictates, AND we wouldn't want to put him into preschool once the CAFS is in full swing which means much more time off work.  So that is not our best option.  We came back with May.  Dr. Park countered with Spring Break (April).  Either of those is do-able since we would work it out for him to be home until next September.  But for now there are no definite decisions so we will just wait and see what they discuss when Dr. P presents his case to the other cardiologists.

Collateral Vessels (CVs):

So, Dr. B discovered several CVs but only one that he could coil.  It had grown between his aortic arch and his left lung.

As a reminder, his heart as it is now:

The blood flow leaves his heart, pumped by both his right ventricle (RV) and his too small left ventricle (LV)  It goes through his combined Aorta/Pulmonary Artery (PA) also known as the DKS and through the arch up to his head and down to his body.  Blood from his head comes back down through the Superior Vena Cava (SVC) directly into the PA branches to go out to his lungs for O2.  The blood from his body comes back through the Inferior Vena Cava (IVC) and does not go to his lungs, but rather, mixes around with the red blood from the lungs, thus diluting it, and then goes back out to the body again.  This is why he has O2 saturations (sats) in the low 80s instead of high 90s.  A good portion of his blood never goes to the lungs and the blood that does gets mixed back in with the blood that didn't.

So this little CV appeared at the point where red blood is going out to the body.  But because the CV was there, it siphoned off some of that red blood and took it back into the left lung, where it would get more O2, which it didn't need because it was already mostly oxygenated.  So it is completely useless blood-flow.  So Dr. B got a coil in there to stop it up and keep the oxygenated blood going where it needs to go.  Dr. B also said that before the coil was placed, (or as the nurse said, "deployed" lol) the sats in his left lung were nearly 20 points higher than the right lung!  Now they are dead even.

Okay.  So here are the rad photos.  I am seriously obsessed with them.  I love it when doctors take pictures so I can geek out a little.

Pre-coil:

Post-coil:

WHAT?!!!  So amazing!

Anybody?

Nobody?

Ahem.

Well I'm impressed.  I just can't imagine this won't help him feel considerably better!  Hopefully it will mean he won't get blue and winded quite so fast.  The other hope is that his heart will not have to work quite so hard with that coiled off and will ease some of the work on the BUFV (big, ugly, floppy valve).

Aaaahhhh yes.  The BUFV.  That is really the root of all evils for this little boy.

When the surgeon (as of yet, undetermined) does the Fontan, he will decide if he can also go inside the heart to put a few stitches in the delicate valve to stop the leakage.  The problem is that it is, as Dr. B described it, a bit like trying to stitch up wet tissue paper.  So the surgeon could decide that the risk of causing more damage is too great and they won't try.  So where does that leave us?  Because it is a giant abnormal AV valve (remember, because of the canal, he does not have two separate valves), they do not replace them.

They just don't.

So here is the deal.  No sugar coating.

Transplant.

Yep.  That is the only option if they cannot stitch it.
There it is.
And if I allow worry and anxiety over that word to take up residence in my mind, I'll go bonkers.  So I won't.  Because, as I've often said, modern medicine is advancing by leaps and bounds.

I mean, seriously?

No. Really.

Click that link and hold on to your jaws people because if you don't, they will hit the floor.

Not. Even. Joking.

So right now we are just happy we made it through that cath.  Honestly, I was a bit surprised at the level of anxiety I experienced surrounding this.  Having had such a blissful 2 years (nearly) of no surgeries, this was rough.  Emotionally speaking.  But it reminded us, yet again, how blessed we are by the doctors and nurses and hospitals available to us.  It makes the whole process that much easier.

And by easier, I mean completely-awful-wouldn't-wish-it-on-my-worst-enemy-but-not-as-bad-as-it-could-be sort of easier.

Right?

Riiiiiight.

So enough of that.  Check out some of the photos of our little adventure at Mary Bridge Children's Hospital.

 Super Heart Hero Tiernan was ready for action

 Dr. Bellotti brought him a Happy Birthday-gram.  He didn't sing anything though.

 The pre-op nurses also put together a birthday gift for him, including new cars and a cool garage.  He read the directions and instructed me in the construction process.

 Post-op Tiernan was so calm and cooperative.  He had George, The First Male Nurse Mary Bridge Children's Hospital Ever Hired.  He was super cool.  He bent the No Kids Rule of the PACU and allowed Tristan to come in and see Tiernan.  He even gave him a Popsicle and  Goldfish crackers just like Tiernan.

 Benefits of a super sweaty, chilled out boy.  You get to give him a rad faux-hawk.  

Awwww yeah.  :)

 And now for a few photos of the newly remodeled MB rooms.  This is in the hall outside of the Med/Surg Floor wing.  There is one with different colours outside the new PICU.  Some of those glass bubbles contain blown glass sea creatures.  Sort of an I Spy type of thing.  Super cool.  

It is an underwater theme and all of the rooms on the Med/Surg Floor and PICU are like this.  Totally amazing.  The old rooms on The Floor were not much bigger than his bed.  

 Auntie and Uncle Matt came to visit.  That many people would never have fit in the old rooms.  I could have taught an OULA class in the extra space!  hahahahah

 Every room has a different under water theme and a beautiful mural wall, along with different coloured lights above the bed that you can turn on and have alternate at various speeds or just stay on one favourite colour.  A simple thing but it really made Tiernan happy.

 Okay.  This may just look like a bathroom to you but you have no idea.  

1. Those tiles were hand made by kids back in the spring.  Tiernan got to make one too, thanks to favourite PICU Charge Nurse Lori.  Every room has those.  Who knows where Tiernan's is.

2. There is a beautiful tile mosaic on the wall to the left of the shower.

3. Before this remodel, when in the PICU, in order for a parent to use the bathroom (much less shower), one had to leave the PICU, go down a floor to the closest available public bathroom.  And still no shower.  Now even the PICU parents have this in their child's room.  It may as well be the Hilton.  It's that luxurious.

 Once all the visitors left, Tiernan feasted like the prince he is.  He devoured that bowl of mac and cheese, emptied a bowl of grapes, ate several bites of peas, most of a banana, drank two cartons of milk and half his birthday brownie.  And that was AFTER his Popsicle and crackers from George.  This kid was insatiable.  

 I just love the spirit in this picture.  Clearly he is still pretty worn out from surgery, but he didn't have any pain meds on board and was still smiling and giving a big thumbs up.  

  Finally, after a wakeful night (up every 2 hours or so) he was up for good by 5 and had a grand time being in charge of the cool bedside  remote/speaker/pretty nurse summoner.  

Maybe the best part of the morning was that Dr. B showed up before 8am to bust us out of the joint.  Such an early discharge has happened....well....never in the history of hospital stays.  So I can absolutely say now that Dr. Bellotti makes a strong first impression.  And if you have to lose a Dr. Chris Stephanelli, then it's good to replace him with a Dr. Chris Bellotti.  :)

If you are still reading this novella, thank you ever so much.  I really can't explain how much it means to me to have so much virtual and local support, and so many prayers and good juju coming from all over the world.  Literally.  So. Grateful.   

We have had a good long run of normal

 Tiernan went to cardiology today.  He got to see his buddy Christopher too!

 He was a super patient for Super Nurse Jodi. His sats were awesome at 86 +/- and HR in the 98ish range.  Weight was 34 (fully clothed, mind you). Height is just under 39 inches.  As far as I can tell, that puts him at the 25th % and 15th % respectively.  Still sort of a peanut. He got to learn how to take his blood pressure.  (upper was 104 over something and foot was 110 over something)  EKG looked great.

Tiernan was so cooperative for Echo tech Chris.  He chilled out watching Scooby and enjoying a lolly.  Even when we had to go back in for some more pics of his arch.  

After rocking the cardio part, Tiernan did a superhero job with his blood draw and chest x-rays.  He also did a little rowing at Mary Bridge clinic and was rewarded with crazy delicious milkshake at Shake Shake Shake in Tacoma.  YUMMMM!  (mommy's nutella milkshake was incredible too.  That's right.  N-U-T-E-L-L-A)

Back to the results:

Dr. P says the echo clearly shows he has Collateral Vessels.  In short, these are vessels that grow and cause extra work for the heart.  This does not help his leaky valve either.

Speaking of the leaky valve:

It is his "tricuspid valve".  I always thought it was his "mitral valve" that had the issue.  Not so.  Dr. P drew me this picture.  (even though when I asked him he looked at me in terror because "he doesn't draw".  I think he did fine.  

Below you can see the two ventricles.  The left is actually drawn on the right.  (just go with me on this) It is just slightly too small.  The Z score is the measurement at the top.  Way back before his second surgery, the docs were all kicking around the idea of closing the AV canal and hoping his LV was big enough to do the job.  A normal Z score would be 0, as he noted.  Tiernan's is -4.5.  In order to have convinced them to go for the 4 chamber fix it would have needed to be around -2.5 or less.  T's is juuuuust a bit too small.  Anyway, I've labeled the tricuspid valve and he has drawn the leaflets as big floppy things.  And that is what they look like on the echo too.  Big and floppy.  They don't close all the way and so quite a lot of blood flows backwards.  They are meant to be one way valves.  His most definitely is not.  

So.  Because of this ugly valve, he needs those collaterals to be coiled in a cath this month.  His arch seems okay on the echo but might need to be ballooned.  Dr. Ballotti (spelling?), Dr. Stefanelli's replacement, started at NWCHC today.  He will be performing Tiernan's cath and will determine if he also needs to balloon his arch again once he is in.  

 I took a video of the echo and I **think** you can sort of see what Dr. P is talking about when he says big floppy leaflets.  I don't know for sure.  Not being an echo tech and all...still, it's interesting to see.  Maybe.  Or not.  

The other part of the cath will be to get the pressures and measurements for the Fontan.  
The Fontan.  
We do NOT know when it will take place for sure until after this cath.  (a lot hinging on it, no?) But it will either tell us he needs it soon.  As in October soon.  OR it might tell us he's good to wait until Spring/summer.  But it doesn't seem at all likely we will wait longer than that.  The darn valve won't let us.  
So now we need to start paying much more attention to the new team up at Seattle Children's.  Dr. Chen has arrived and begins surgeries as Chief Surgeon on Monday.  He as an excellent reputation but it's a wait and see him in action sort of thing. 
We are hoping, and please pray, that whenever the Fontan must take place the surgeon is able to repair his valve with a few stitches.  However, this complicates the surgery much more because even though the Fontan is called an "open heart surgery", it actually happens entirely on the exterior of the heart.  If they decide to try and repair the valve they will have to literally open the heart and work inside, thus upping the level of complexity.  
If they cannot repair the valve then they will need to wait and replace it eventually, but that is a whole different ballgame due to the size valve he would need.  

The cath will be scheduled tomorrow and I will update when we have a firm date.  I suspect this blog will be seeing a bit more action this year.  I can't believe the last time I blogged was in OCTOBER! That was, indeed, a good long run of normal. 
And now on to the next chapter.


PS...please send good thoughts/prayers/mojo to one of Tiernan's besties, Alex.  He is down at Stanford for his Fontan which takes place tomorrow.  Tiernan and Alex go to preschool together and his mom, Dana and I have worked together at NVI for the past 10 years.  They are so important to us.  We wish them all the best as they start this chapter.

Cardiology Report

Today was Tiernan's first full cardio appointment in over a YEAR!  Crazy!

It was also our first cardio appointment with Dr. Park instead of Dr. Stefanelli or Dr. Obayashi.  We are I am still a little sad that they won't be around, but I think Tiernan loves Dr. P.

 Tiernan has a few favorite things to do at NWCHC:

He likes to chill in the little chair in the waiting room

 



and he has to keep an eye out for the "ambences" across the street at Mary Bridge.

 

 

But this time he really decided to take charge of the appointment from the beginning:

 



He was Mr. Cooperative with the sats check.  They are holding steady at 86. 

 He showed how he can put on his EKG stickers.

 

and then he attached the leads

and finished up by reading his EKG results.  :)

They were unchanged from last year.

 

While waiting for his Echo, he pretended to be Dr. P and took a few spins on the Doctor chair.

 

Next he decided to be a ROCKSTAR in the Echo room:

 



He was so super chill watching The Little Engine That Could that I was able to sit on the comfy couch across the room for the first time since the days when it took both Jason and me to get him to any appointment.  Not that I got to relax for long though, because apparently the tech is able to get pictures in under 20 minutes (as opposed to 45 to an hour) when the patient is not screaming his head off the entire time. 

Results: UNCHANGED from a YEAR ago!  Praise God!

 

And because he didn't need a sugary distraction during the echo, he got one as a reward.

 

He actually dropped that on the floor moments later. 

Luckily, I had brought 2 more so he got a blue one...which landed on the floor as well.

He finally got a green apple dum dum and made that one last until the very end. 

 

Overall, the appointment was fantastic.  I have some new understanding surrounding his leaky valve though.  Because he has an AV canal (hole through all four chambers) he doesn't really have the typical Tricuspid and Mitral Valve.  True Hypoplastic Left Hearts have a small mitral valve.  Tiernan has what is called an AV valve...it is a one valve instead of two and is therefore larger than either individual valve would be.  In addition, because an AV valve is abnormal, they would be faced with the problem of finding a valve big enough to use as a replacement. 

So.  What all this means is that if they cannot repair at the time of the Fontan, it would be highly unlikely they would attempt a replacement.  The point of the Fontan is to take the workload off the single ventricle.  It will do this, but his leaky valve would continue leaking and likely get worse over time causing more stress on his heart anyway.  This means that if the valve causes too much trouble post Fontan, it could mean an earlier transplant listing. 

Of course, this is an example of the the proverbial cart being waaaaay before the horse.  And Dr. P reinforced our mantra:

"We don't know what science and technology will bring in the coming years so let's not worry too much about that now."

 

We will not let this steal our peace.

 

Especially in light of the highly encouraging news regarding the search for a new Chief of Pediatric Cardiothroacic Surgery at Seattle Childrens.  When Dr. Woods left, we were pretty much set on needing to travel to Stanford for any future surgeries since we did not feel confident having Cohen again.  But then Cohen left as well, and hope returned that we could stay local.  But the search is a long (understatment) process and so we've been in a limbo, so to speak. 

But today Dr. P shared a really cool update. 

Seattle Children's is gearing up for the round 2 interviews and 2 of the candidates (maybe the only candidates) are a guy from Dallas Children's with a pretty great reputation and, inexplicably,  

this guy...Dr. James Tweddle.

Dr. Tweddle is the guy Dr. P studied under in Wisconsin prior to coming out to NWCHC.  Additionally, he is the guy Dr. Woods left us to go work with in Milwaukee.  He is one of the top rated Pediatric Cardiothoracic surgeons in the country.  His outcomes with HLHS and Fontan patients is stellar.  No one at NWCHC can explain why he would want to leave what he has at CHW to come to SC but clearly he's etertaining the idea.  Crossables are all crossed. This would leave no doubt about staying in Seattle for the Fontan.

 

Speaking of the Fontan...

They only schedule Fontans for the summer to avoid potential illnesses during cold and flu season.  Because of the weird valve issues, Dr. P seems prone to at least keeping open the possibility of the Fontan taking place next summer.  [cringe]

This all depends on how he is doing in May.  If all is well and largely unchanged then I expect we might wait another year.  But if things are starting to deteriorate, then they will start scheduling caths and surgeries.  [sigh]

I won't lie.  This news sort of dampened my glee a tad, but once again.  It will not steal my peace.

 

Well, after cardio, they sent us over for bloodwork and he continued his amazing superstar behavior and didn't make a peep during the entire thing.  Shocked the phlebotomist as well.  :)  We will hear Monday about his kidneys and liver BNP or Brain Natriuretic Peptide levels.  This is something that is produced in the heart when it is failing.  Today's levels don't matter except as a baseline to which they will compare levels in May or whenever.  A discrepency will indicate need for intervention. 

While at Mary Bridge Clinic following the blood draw, we randomly ran into our old friend Dr. Pickens, GI doc extraordinaire.  He was thrilled to see how well Tiernan is doing and (as per his norm) gushed over my weight loss.  hehehe.  Love him.  :)  That said, I'm not sorry we don't have him on speed dial anymore. 

 

Finally we headed for home but made a stop for lunch and plane watching at The Hangar Inn near our house as a reward for Tiernan's outstanding appointment. 

Happy HAPPY Tiernan.