PLOT TWIST!!!!!!!

I would also say this applies to when your child's cardiologist calls you and tells you the surgeon at Stanford feels strongly that he could close up the AV canal, thus giving him a...

....are you ready for this?

 four. chambered. normally. functioning. heart.

So I have to back it up for a minute and start with Seattle Children's and Dr. Chen.

Several weeks ago, we were told that, after presenting Tiernan's case to Chen, he STRONGLY recommends proceeding with the Fontan and attempting the <very scary> valve repair, as soon as possible.

Rationale and Ramifications:
Why not just do the Fontan and wait to repair the valve?  The longer we wait, the worse (leakier) the BFUG will become.  IF he has a shot at a successful repair, now is the best opportunity.
During this surgery, because stitching the valve is somewhat like trying to stitch wet tissue paper, (according to Bellotti) Chen (or any surgeon) could cause more harm than good.
Enter PLAN B:  a  mechanical valve.   (this link is re: a bicuspid valve, but there is good info)
Problem with the mechanical valve is that the "geometry" of Tiernan's common AV valve is strange so they may not be able to get a good fit.  But let's say they do.  That means he has a functioning valve.  Good.  But it will be a magnet for blood clots.  Very VERY bad.  This means lifelong Coumadin Therapy.  If you know anything about Coumadin, you know that is a bummer of a sentence for a kid.  Easy bruising, constant pokes to check INR levels (which are really difficult to regulate) and bleeding would be difficult to stop.
What if the valve does not work?
PLAN C: He is stuck with no functioning valve, gets placed on the Berlin Heart and goes to the
top of the transplant list and we are inpatient until that occurs.

This information was not exactly the most encouraging.  At this point we were in a holding pattern waiting for our second opinion from Dr. Hanley and his team at Lucile Packard Children's Hospital at Stanford.  We fully expected him to come back in agreement with Chen.  At that point we would be faced with the choice of who we felt most comfortable with on this scary surgery.

A: Stay at Seattle and wish we had gone to Hanley...undeniably one of the best in the country.
B: Go to LPCH in Stanford and maybe have the same results and need transplant and then we are stuck in Northern Cali until that happens and for months (minimum) after.

These are not the most amazing choices.

But then, last night, Dr. Park called with news of the letter from Hanley.  Not only does Dr. H think it's possible but his entire team is in agreement.  They met extensively re: Tiernan's case and they unanimously support this recommendation!

As a reminder:

Tiernan has, among other things, a big hole through all 4 chambers of his heart.  Way back when he was diagnosed, the hope was that they could reconstruct his Coarctation (pinched aorta...CoA) and put a band on the Pulmonary Artery to discourage the blood from going so much to his lungs and encourage more through his now bigger aorta.  If all had gone as they hoped, then they felt confident the improved bloodflow could help his smaller Left Ventricle grow to a size that could handle the necessary workload.
The problem was that his CoA closed down again and his heart then had to pump against a double obstruction (tight PA band AND CoA).  Dr. Woods (Mary Bridge) AND, incidentally, a second opinion from Dr. Hanley, both recommended we had to proceed with the DKS and Glenn and plan to treat him as a single ventricle heart.  At that time, and occasionally since then, we have discussed the rare occasions when it is possible to take down the DKS/Glenn and patch the AV Canal and repair the valves to convert back to a 4 chambered heart.  Honestly, I never really let myself believe that was an option.
Even as recently as our last cardiology appointment, however,  Dr. Park and Dr. Bellotti were discussing this option.  They agreed it was juuuuust ever so slightly too small to consider it.  And so, again, I abandoned that option.

But now Dr. Hanley says he thinks it can work!!!!!

This could be a miracle.  Please pray for this miracle.

Tiernan could grow up with a normally functioning heart.  A normal life expectancy.  A normal opportunity to be whatever he wants to be.

Like a fireman.

This boy so DESPERATELY wants to be a fireman.

The decision to take Tiernan to Dr. Hanley is sort of a no brainer but we will be meeting face to face with Chen to talk at length about why he does not recommend this.  That information is really helpful.  We will also need to schedule a trip down to LPCH to meet Dr. H and let them do their own echo and exams.  We are thinking that will be as soon as November.
The surgery could maybe take place in April.

But it won't be a quick and easy recovery.
It will likely be long and, if we know Tiernan at all, complicated.
We could end up there for a long time.
It's going to cause lots of things to change.
What do we do with Tristan?
It will be financially scary.


But I just don't know how we can say no to this opportunity for a normal heart.