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!