6 years ago he went down the single ventricle path and today he is a stable bivent.

Tiernan has been talking a lot about this first Santa meeting. 

Wednesday we had his cardiology checkup. It was the first one since September!  That is the longest between visits since well before his bivent surgery. He continues to thrive and blow us all away with his energy and vocabulary. (He's especially fond of adverbs.)

Today also meant labs for the first time in 2 months. He was NOT as excited about this part of the visit. But when we arrived, there were Santa and Mrs. Claus waiting to see kids! He chose to do his blood draw first (see below for photos of that awesomeness) and then went and told them all about when they came to see him 6 years ago. He and Sammy left with some gifts and we made our way to cardiology. 

A bit more about the labs:

He has always screamed and fought it like crazy. I would have to hold him on my lap and hold his arm still so the phlebotomist could get the draw. Today he did this! Sat all by himself and acted like it was NOTHING!  

All smiles. 

Watched him put the needle in and only winced a little. 

Applied pressure and didn't even bleed or bruise at all. 

Later when I asked what his favorite part of the day was, he pointed to his arm and smiled. I asked why and he said, "Because I was so brave!"

Mine too little man. 

More about his checkup. 

Weight: He's gained a few pounds since September. I'm surprised it wasn't more,  given the amount of curry the kid puts away. 

Height: not showing significant growth since September but Dr. P doesn't think that's too surprising when considering his body has been focusing on more important things. IF he still doesn't shoot up in the next 6 months then he may need to see an endocrinologist to see what's going on. 

Sats: 96. Right on. 

Tiernan was SO great. He generally is these days, but I am just so impressed with how he deals with his lot in life. 

He was as calm as they come during his echo and so it was finished in record time. 

Dr. Park came in and set this graph down in front of me. 

This is a look at his BNP. (Brain Natriuretic Peptide) 

Here is a guide that shows what those numbers mean. The bottom line is that Tiernan's BNP has been all over during the course of the past year and a half. His number on Wednesday was 75! Normal!  So relieved. 

The other news is that while his valve regurgitation is a bit worse, his left atrial dilation (enlargement) has improved!  So it is pretty much an even trade which leads Dr. Park to declare his heart pretty much unchanged...read: STABLE. 

The one med change we've made is to increase his Enalapril (blood pressure) to twice a day. He had been cautious because it had caused his BP to be too soft when he was post op. It also has a negative impact on The Kidneys so, due to his kidney failure status, he was only on the smallest possible dose. 

Now The Kidneys are happy (all his labs revealed beautiful numbers even his nephrologist will be proud of....which is really saying something). 

So it's time to up the offense and increase his Enalapril. 

Dr. Park thinks it is reasonable to hope we could get 10-15 years before he needs Dr. Hanley to get back in there. Until now he hasn't said more than 5. Of course we can't really tell, but that is a great new prognosis. 

And then Dr. Park said he didn't need to see Tiernan for SIX MONTHS!!!!!  We don't need to go back before JUNE!  That is only the second longest we've ever gone between appointments. And even better, no labs until then either. 

Except we have an appointment with Dr. Hanivold (nephrology) on the 29th and she WILL want to see how The Kidneys are handling the new increased Enalapril. But after THAT, then hopefully we will have several months between labs. Fingers crossed!

Memories and Looking Towards a Bright Future

We have been enjoying going through old photos and videos recently.  This photo was from August 29, 2009.  Tiernan was 10 days old. 
This is the last photo I have of Tiernan before we nearly lost him to undiagnosed CHD. 
These are the next photos I have. 

September 1, 2009.  2 days had passed and we were learning all about the numerous ways Tiernan's heart was not normal.  I couldn't go 30 minutes without bursting into tears. 

But here we are, 6 years later, and this is all I see.

The next member of the Class of 2028. This boy starts kindergarten in 10 days.

Unless we tell them, no one would EVER guess the life this boy has already lived. 

Today I chased him and his imagination around our neighborhood high school pretending to be spies and hiding from cars and the cross country team and the custodian. All so I could get photos of this milestone. 

(Thanks to fellow heart mama Elin for her inspired idea....and speaking of Elin. I secretly/not secretly anymore hope that her mighty girl, Maija will be Tiernan's girlfriend someday. She is just about the coolest 6 year old in the world....loves superheroes and Star Wars and ballet and archery and...I could go on for a long time.  She's rad. Does it really matter that she lives in Minnesota?  Oh. And Elin is a U of M Griz too. Seriously perfect.)

Anyway. I am so excited to see what life brings his way. And life better look out. He doesn't back down for CHDs, why should he back down for anything or anyone else?  

Mended Little Hearts and Some Very Special Friends

Tiernan and I got to go to the annual Mended Little Hearts Picnic last Sunday. We missed last year's bash due to surgery but this is one event we really try to attend. 

I'm so thankful for this group. The first meeting we attended was while Tiernan was still in the Mary Bridge PICU following his Christmas Eve Glenn. He would have just been extubated, the second time, perhaps the week earlier (maybe days) and we were grappling with the uncertainty of his heart condition as well as the news he would need a GJ tube. Favorite Nurse Jodi told us to go so we went. Understand how difficult it is to have both of us gone from his room at the same time. It's possible we wouldn't go unless some family member came to sit with him in our absence. I imagine that's what happened, because we walked across the street to the basement of Jackson Hall and found some people we had never met but "knew" instantly because of our shared experiences. When you go through something like this others will often ask how you do it. I don't always know how to answer that and usually say something like, oh, you just do it because you have to. But I think groups like this one are really the answer. We survive because we have other families with whom we can talk/cry/worry/research/laugh at our "not so funny to people outside the CHD world but really funny to us" jokes and comments/get advice on everything from giving meds to what sort diapers hold up best to high dose diuretics to Medical 504 plans when they get to school to.....to......to....

But MOST of all, we give and receive hope and encouragement. There is NOTHING that compares to sitting down with another mom who has gone before me in this and seeing their child running around before us. And there is nothing (outside of getting to be Tiernans mom) that makes all this more meaningful than being able to be that source of hope and encouragement and advice for someone else who is just beginning this journey. 

I know I have written before about the incredible heart moms God placed before me, but I want to mention Dana again because God REALLY knew what he was doing on this one. 

I work as a choir director for 10-13 year olds in a wonderful community near Tacoma. This year will be my 13th year at this school. Dana is one of the 6th grade teachers there as well. When I got there she had been there for awhile already and really knew what she was doing. Seriously. I was a bit intimidated by the standards set there. No lie. So several years in and she has this gorgeous baby named Alex. And Alex has heart defects. And she was on leave for most of that year and I remember her talking about the different challenges she had been having and here I am, pregnant with Tiernan and blissfully unaware of what God has in store. I remember being MASSIVELY pregnant the weeks before Tiernan arrived and reading an email from another work friend about Alex needing a pacemaker and I sat at my computer and said out loud to myself, "I canNOT imagine what she is going through!" 

Oh boy was I about to get a whole lot of first hand experience with it. 

The next thing I know we are in the midst of diagnoses and surgery plans and feeling lost and terrified. And I had forgotten about my resource (Dana) until I was sitting in the back of the ambulance that was transporting Tiernan up to Seattle from Mary Bridge. The transport nurse, AKA Favorite Nurse Lori, (have you picked up on the fact that we have a few of those?) was there and asking me getting to know you/take your mind off the fact your baby is in an ambulance questions, like, where do you work?  And when I told her, she brought up Dana!  Because, of COURSE she was a friend of hers and had taken care of Alex after his surgeries. This reminded me that I needed to get in touch with Dana and at some point over the following days I called her. 

Random fact about myself....I'm insecure about phone conversations. I don't like them even when I am talking with my best friends. Sometimes I don't even like being on the phone with my own mother. (Sorry mom). So to me, calling someone I've never called before (and if you remember back a few paragraphs someone I was a little intimidated by (it just occurred to me that Dana doesn't know that part....Hahahaha. Now she does.)) was stressful. So stress layered on stress and here I am, a bundle of nerves calling Dana and she answers and I'll never forget.

She said, "Carolyn! (Because my work peeps call me that) I am SO relieved to hear your voice!"

And then "Everything is going to be okay because he will go to Northwest Childrens Heart Care and he will have Jodi and they will take such good care of him."

You just don't know the relief that filled my heart and soul with that conversation. And it's all because she had gone before. It means more than anything. 

Since then, our boys have grown into 6 and 7 year olds who run around together pretending to be a giant caterpillar in the spray park. And Dana and I have walked this journey together. Through hospitalizations and feeding/eating/puking issues and losing our amazing surgeon Dr. Woods, to traveling to Stanford and all the questions and challenges that go along with that. 

And Tiernan and Alex get to walk their journeys together which is AWESOME!  The older they get, the more special that will become. I hope they always have one another to lean on in this life. 

There are SO many more mamas I have met on this journey who are so important to me. I am thankful beyond words to the families we know through MLH and also those I have met on FB. I'm very certain that is the best aspect of social media in this world. 

There is no way I would be as sane as I am if I didn't have Dana and the MANY others by my side. 

Learning new meds is annoying

It just is. 

So we started the Atenolol on Monday night. No real issue to speak of besides the made up ones swirling in our brains. 

Jason and I each checked his heart rate and respiratory rate several times, and J even checked his temp a few times. All definitely normal. Including his 2 or 3 visits to our room. 

Tuesday night, however, he comes into our room around 11 and has what sounds like a croupy cough. 

Mental note and send him back to bed. 

He comes in again, like usual around 1am and gets in bed next to me. Coughs a few times and every few seconds takes in a gaspy sort of breath, like he can't really get deep breaths otherwise. 

So I realize I haven't really looked up any of the other possible side effects because Dr. P just said to watch for unusual fatigue during the daytime. 

1am Google session reveals:

Check, check aaaaand check. 

Okay, I know better than to diagnose "bronchospasm" on my own through Dr. Google, but it isn't far off. 

Aside

Do note the "Infrequent" nature of these, by the way. I definitely should have looked these up as the ones to expect given that Tiernan has ALWAYS taken the road less traveled. 

I have to add that he has, the last two nights also complained of stomach upset shortly after the dose. So I probably should have kept that throwing up one on my list. 

So yesterday I put in a call to cardio and he said to try cutting the pill in half and see if that helps. 

Aside:

His dosage for propranolol was 10mg TID (3x daily). 

The atenolol is only 25mg once a day. I don't know if it's a straight across comparison but now I'm hoping it's enough of a dose to control the HR. we will see, of course. 

So we cut the dose and there did not seem to be any of the breathing issues last night and all vitals were normal. But this morning there is this:

And this:

Aaaand this:

I can count the number of times he has fallen asleep again once coming downstairs on one hand. So I think we better switch it to a 7 or even 6 o'clock dose to be ready for school starting. 

And this is only day 3 of the new med. Like I said, learning new meds is a real big pain. Hopefully all these little things resolve soon so we can get into a good routine with all his meds. 

Until we change them again, that is. 

Let's Talk Weans and Regurgitation

Hello out there!  Figured it was about time for a little update.  We have had a very busy July and Tiernan's heart has been adjusting and readjusting to some changes. 

Back at the end of June Dr. Park loved how things looked so he dropped the Diuril dose. Shortly thereafter, we headed to Missoula for 2 weeks. We had a wonderful visit and Tiernan ran around like never before. He went on walks around the University of Montana (mostly because he could convince people to buy him chips and drinks at The Market in the UC).

He fit in PERFECTLY with the Williams Clan and worked on his photographer skills, starting with GG at her 90th birthday bash. 

Selfie lessons from the pro photographer in the family. (Uncle Dan)

Ladies and Gentlemen....the party has arrived!

We came home for a week and had to go in for yet another round of labs. 

Let me say that there is no place like Mary Bridge Clinic for blood draws, and Phlebotomist Gary is typically a magic man.  But this time, despite Child Life being there, it took him 2 pokes (almost unheard of) and he may have been a tad dehydrated so his blood wasn't flowing too easily. Regardless, we got it done and then made the 45 minute drive home only to be called back because someone had put his blood in the FREEZER!  

GAH!  

Round two went okay. Or so we thought. 

Within a few days we left for 5 days at Cannon Beach with my family. 2nd day there I got a phone call from Dr. Park. The labs had been run only for his BNP instead of including the Renal Panel for Kidney stuff. So this meant he would need more labs (3 in 2 weeks) when we returned. 

But most concerning was the BNP. it had LAUNCHED from a normal 90 up to 333! That is well above where it had been at any point since his surgery. Dr. P was definitely not happy with this but didn't feel it was an emergency. So we got home and the next day went in for more labs. Gary got it in one poke and did all the right tests and they came back looking much better. Pretty darn perfect in the Renal Panel actually. BNP had already dropped to 214 so we all feel better about that. 

What have we learned from this?  

Every time his BNP jumps, it correlates directly with a change in meds...specifically when we wean the diuretics. Initially his heart freaks out a little and then upon further reflection decides it's not so bad on lower Meds. And then it's happy again and we change the meds again and we start the cycle again. So I am really trying to take the jumpy BNP in stride. Especially since he looks amazing!  He feels great and it shows. He can run around with his neighborhood BFFs and hardly gets tired.

At the beach, by the way, he could have done this all. day. long. 

 

Since getting home, he has started Theatre Camp (his first day camp) and LOVES it. He's one of the Brick Peddlers in the Three Piggy Opera. 🐷🐷🐷

UPDATE:

 Tiernan's Heart Twin, Walker, had his surgery and did pretty well except that his Mitral Valve is not doing well. He had moderate regurgitation that was not improving with Enalapril like Tiernan's has.  So he is going back into surgery tomorrow to hopefully repair that as well. If they can't get it to work they will need to replace the valve entirely. That adds considerable complexity to life so please send out some prayers they will be able to close the cleft and stop the regurg. 

All of the talk of Walker's valve prompted me to ask for a little more clarity involving his Mitral Valve. So I asked Dr. P how he diagnoses moderate as opposed to mild or severe regurgitation. He said that Tiernan's is moderate because the "height of the jet" goes to the back of the atrium. 

Translation: the blood pulsing backwards through the valve from the LV to the Left Atrium makes it all the way to the back of the atrium. 

Also, the opening in the valve is pretty wide. 

The change he will look for to indicate a change to "severe" Mitral regurg (MR) would be that the blood trying to enter the LA from the Left Pulmonary Veins (from the lungs) would get pushed backwards due to the amount of MR.

Here's the best visual I could create given my limited tech abilities. 

 It is helpful for me to know what would change his diagnosis to severe. It's called Reversal in the pulmonary veins. So if we are on vacation and he gets sick and we are in an ER and they get worried about the MR. I can ask if there is reversal in the PVs and they will, first off, be super impressed with me, but more importantly, be able to discern if it's worse than his normal MR. 

MOVING FORWARD:

Tiernan starts Kindergarten in a month!  We are going to switch his Beta Blocker from the 3X daily Propranolol to a 1X daily Atenolol. That will mean meds only in the am and pm!  Wow!  

THROWBACK THURSDAY:

For comparison: 

The top spreadsheet was just the pain med wean from after his second surgery in 2009. The bottom two are from this past year post bivent. 

We will now have:

Lasix BID (2x daily)

Spironolactone BID

Enalapril 1x daily

Atenolol 1x daily

Aspirin 1x daily

This is HUGE! 

Atenolol does cause drowsiness so we will give it at night and hopefully a bonus will be that he finally sleeps through the night!  

I think that is all I have for now. Look for some fun posts about his performance and the start of kindergarten coming soon!  

Tiernan's Heart Twin

Tiernan has SO many heart brothers and sisters. TOO many, really. But none of them (that we know) are really living with the same constellation of defects as Tiernan. While that may not seem like a very big deal, it actually feels gigantic. 

When Dr. Woods and the team at Mary Bridge diagnosed Tiernan, he told us that even though he's seen all of these defects before he had never seen or even heard about them all in one heart. This meant he didn't have a whole host of cases to guide him in planning the surgical path for Tiernan. He knew he could possibly follow a few different paths but wasn't able to point to other kids who had gone before us and what their lives were like. 

I'll tell you this much. When your child is facing one or more open heart surgeries in their lifetime, it doesn't matter how many others have the same defects, except when it comes to the need to see others who have survived and even thrived. It is always scary. But doctors all now know basically what to do for a true HLHS or Tetralogy of Falot or Transposition. They don't always know what to do with a "Coarctation of the Aorta/Complete Unbalanced Right Dominant Atrioventricular Canal/Mildly Hypoplastic Left Ventrical/Big Ugly Floppy Common AV Valve Regurgitation."

There just isn't research to say difinitively what path a surgeon should take. 

So they guess. 

And things go wrong. 

And this forces their hand to treat him like a single Ventrical, even though he isn't truly a single vent kid. 

And then you think your path is set. 

And you wait.

And when people ask what defect he has there is no all encompassing phrase to describe what he has so you just say he's closest to HLHS.  

And you wish you had some people to really look to who have the SAME heart. 

So when I found my way to some FB group thread and there was a new mom on there describing her son's heart and I felt like I was reading Tiernan's diagnosis, I might have cried a little. 

Because that had truly never happened. 

Walker is Tiernan's little Heart Twin. Yes there are a few differences but the similarities are astonishing. 

And not only the defects. 

The surgeries they have had and the complications and recoveries following have been incredibly similar. They both had feeding tubes and issues surrounding all of that. 

They both were set on that single vent trajectory because doctors just didn't think their LVs were adequate to handle the job. 

And then Hanley looked at Tiernan's heart and disagreed. 

And Ashley thought why not ask?  And Dr. DelNido looked at Walker's heart and disagreed. 

And last year within a few weeks of each other, Walker and Tiernan headed down the bivent path!  

Walker was still young enough to do it in a 2 stage approach where they "recruit" the LV to encourage growth. I really don't know a lot about that one because Tiernan didn't have it.  

But yesterday Walker returned to Boston to have a cath to see how things went. 

His LV is now "of normal size"!  

Incredible!!! 

So tomorrow he will go in for his 4th open heart surgery in his short life and DelNido will perform the same repair  Hanley did successfully for Tiernan almost exactly 2 years ago. 

Please keep Walker in your prayers and thoughts tomorrow. 

I'm so hopeful that DelNido will be able to take down his DKS and Glenn with only native tissue and no conduit, just as Hanley did for Tiernan. 

I pray DelNido and the CVICU team will not push for a quick wean from diuretics so Walker's heart can adjust to its new jobs, just as Tiernan's has. 

I pray that the similarities between their hearts will mean continued similarities with the success of this repair. 

Because these two boys need to meet. 

Welcome to Stable. We're glad you've made it. Please sit down and stay awhile.

Selfie before Cardiology appointment. 

I spy a double chin. 

Tiernan was the model patient for his echo today. Which, by the way, was unchanged from the last one. Dr. P said if it weren't for the date, no one could tell them apart. 

The Mighty Tiernan was in rare (by which I mean COMPLETELY typical) form as the only patient in the office.  He showed off his muscles to anyone and everyone and did NOT show off his ability to listen and follow directions. (I'm pretty sure he doesn't possess those skills these days)

He played paparazzi with his favorite doctor. 

And needed a photo op with him as well. 

Why does Tiernan think Dr. P is super cool?

Well, the fact that he said no more fluid restrictions, effective immediately, might have something to do with it. 

"CHEERS!!!"

Other notables:

1: O2 Sats were 98!!!!  Rock. Star. 

2: Since his surgery (where he lost 3.5 lbs) he has gained nearly 7 lbs this year. 

3: He has gained nearly 2 inches (just shy of normal) since last year.  Given all he's gone through, we will take it. 

Side note: Dr. Pickens (GI) was NOT as pleased with such a "small height increase". No offense, but we will celebrate because cardiology says we should. 

4: Labs look mostly excellent. 

        •BNP is 90!  >100 is normal. 

        •BUN and Creatinine numbers look    "great". (Not sure what those numbers are exactly but he says it's excellent. 

        •Sodium and Chloride are still a bit low but we will wait to see how nephrology feels about that next Tuesday. Park is not concerned. 

         •Potassium is perfect. 

5: He is the healthiest Park has ever seen him. And he's known him since he was 4 months old. 

And looking like this...

  And speaking of those days, when we were on a walkabout after celebratory lunch at his favorite place (Mary Bridge Cafeteria) we found this guy. 

Dr. Paschall was one of our favorite PICU intensivists from back in the day. It was not difficult to jog his memory of Tiernan given the uniqueness of his surgery date (Christmas Eve) and his, eh, baby pterodactyl impressions. 

"Never wake a sleeping baby." Dr. Paschall, from his office across the hall fromTiernan's room. 

And even though he's nearly 6 and doing better than ever, cardio appointments never fail to leave him exhausted. So, because Dr.Paschall said not to, I didn't wake him. 

 His shirt says it.  The best IS yet to come. But today is pretty darn amazing. 

And we breathe easy again....until next time

Tiernan is still doing great!!!!!  

Still. 

Phew!  

Jason took him to cardio and then sent

my most favorite text in a long time. 

"All good news. BNP is down to 113. X-ray is clear. Echo is unchanged."

Thank you to all of you for sending extra special prayers and mojo our way this week. 

Maybe someday I'll get to approach these things with less worry and fear. 

I guess if I did a little more yoga and meditation with this boy that could be a good thing. 

I should have known better

Random person: How is Tiernan doing?

Me: Great!  At the moment. 

Random person: You must be so relieved now that he's doing so well. 

Me: Absolutely. For now. 

I never can seem to leave off the qualifier. 

I'm always waiting for the proverbial shoe to drop.

But just under a month ago Tiernan's labs came back with such great news. His BNP is under 50! Incredible!  So exciting. 

Potassium and another dose of Lasix d/c'd, and the living is easy. 

This must mean things are only looking up from here on out right???

Weeeelllll.....hold on a minute. Next labs in and not only was his BNP back up hovering around 100, but his creatinine (kidney numbers) was higher than it had been in a long time. 

Still. Could be a fluke. He wasn't super hydrated that time. Probably best not to put too much stock in those numbers. We'll get him drinking more and things will look better. 

So now the next labs come back with his kidneys looking great!  .35 actually. Practically perfect. 

Buuuuut....

BNP. 

Back up flirting with 200. Well, 178 to be precise. 

<insert defeated sigh>

I. Should. Have. Known. Better. 

So while I also know better than to get too far into the depths of despair, this is just one more example of why I keep finishing my sentences with

"...at the moment." and "...for now."

So please don't think I'm just being negative. Or pessimistic. 

These things change on the daily so celebrating small victories while maintaining a healthy dose of "this probably won't last long" is really the name of the game.  

The thing that never changes?  The cuteness factor remains off the charts.