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. 

It's a good thing all these blood draws show good news

Tiernan has been very busy putting his new 4 chambered heart to the test. He has resumed swimming lessons and started T-Ball!!!  He knows how to skip and can run and run and run, without a tinge of purple. 

And although it has been a slow process, we are finally starting to say goodbye to some of the many meds Dr. Hanley set in place nearly 9 months ago. 

N I N E months!  What??!! So slow and yet, 9 months!

We have appointments aplenty, whether with cardiology, nephrology or GI, and unfortunately, blood draws continue 2-3 times per month. Those are definitely a major bummer but we are thankful that the Phlebotomists at Mary Bridge are almost perfect at the "one poke" challenge. 

The purpose of the labs is to keep a close eye on several numbers:

BNP: enzyme released by the brain when the heart is failing. His BNP has been pretty consistently in the low to mid 100s. They were up over 200 back in September. They dropped initially and then jumped up again just before Park started him back on Enalapril. This worked well and the number had leveled out in the low 100s and then jumped again to 140 in early March. 

Creatinine: this number indicates kidney stress. The higher it goes the more annoyed The Kidneys have become. For somewhat mysterious reason, his creatinine levels remain a bit on the high side but his nephrologist, Dr. Hanivold agrees to stay the course with the diuretics and enalapril (both of which pack a punch against The Kidneys) because his heart is happier (as indicated by lower BNP) 

Potassium: diuretics make you pee. And potassium goes right out with the urine. Low potassium can cause issues with his heart rhythms which could, in turn, cause oodles of other problems for his heart. 

Typically the results have shown all things more or less acceptable and we don't make any changes. But a month ago Park made a bold move to finally start weaning the diuretics. Tiernan has been on 40 mg of Lasix 3 times per day. This, along with his Diuril and Aldactone is a bigger dose than any other patient Park has...including some kids in serious heart failure. (Hence the nephrologist being added to the team). So Park discontinued one of those doses a month ago and my anxiety level went up by  96%. We even ended up going in for an impromptu chest X-ray to check on things. (All was clear, so my anxiety cleared a bit too) 

But the proof is partially in the lab numbers, specifically the BNP because it would tell us if his heart is working harder. 

We went in for labs again yesterday at about 4:30. Dr. Hanivold called and left a message by 6:25 to make sure we get in touch in the morning. 

UGH!  Anxiety back in force. 

She called Jason this morning and had the following to say:

BNP is 46!!!! (I'm not sure his BNP has EVER been so low!)

Park wants to cut LASIX to twice a day at 20 mg. 

Creatinine is lower. Down to .47 from .7 last time. .3-.7 is normal range. So this is excellent news!!!

Potassium is slightly lower at 3.5 (3.6 is normal range).   But will probably go back up with LASIX change. So after the next blood draw it is likely he will get off potassium. 

This news could not be better!  I had to send update to Dr. Obayashi and he said:

That is just such good news! His left ventricle is growing and becoming normal! The left atrial stretch is lower and with a BNP less than 50 that is fantastic! As I said before it's a brave new World for Tiernan. The trajectory of his life changed tracks on a much more favorable expected destination. Unbelievable.

So for now we wait and hope this new wean yields similar results and then I think Park will shift his focus to ditching the Diuril. 

(Aldactone is here to stay.)

Getting rid of the majority of diuretics would improve things so much for us. Tiernan has to wear pull ups at night still and FILLS 2-3 of them every night. They often leak and I can definitely say we have changed and washed more sheets in the last 9 months than EVER before in our lives. The end of that season cannot come soon enough. 

The other big victory was that he finally passed his swallow study. Remember that his left vocal cord was paralyzed post surgery. He had been sent home on thickened Apple juice and water from a spoon. He wouldn't drink thickened milk (seriously....it's like Elmer's Glue) and the thickener just turned to snot-like globules in the Boost Breeze (high calorie/fortified juice) so that was out. He went for his first repeat swallow study back in November and he wouldn't cooperate very well. So Jason had to hold the cup while he drank the water. No surprise, he aspirated. 

So then we saw his ENT, Dr. Bosley. He was supposed to do a scope (down the nose) to check the vocal cord. He determined it was perfectly fine just by listening to Tiernan speak and then told me that he was not at all shocked to hear Tiernan had aspirated. He said anyone would if they had to suddenly drink free thin liquids from a cup when they had been spoon feeding it for months. So he directed me to wean him off the thickener and sent us on our way. 

All was great until last month when Jason took him to GI and they had not gotten the memo from Bosley and laid the guilt trip on hard. So I was pretty worried his next study would show aspiration and then where would we be?  

But the stars aligned and Tiernan passed his swallow study with flying colors so he is free to drink normally. (He was already doing this, but now we can feel confident that everything is working correctly.)

I have such difficulty telling people he is doing great without adding some qualifier like, "for now" or "all things considered". I still have the knowledge that his valve is pretty ugly and won't last forever bouncing around in my thoughts. But with each passing month and new milestone, I feel like I can finally stop thinking about it for awhile and think about things like Kindergarten registration and whether or not he will remember where third base is when playing T-Ball. (I'm not betting money he will remember that one, for the record)

 

Pre-school, Pumpkins and Potential Problems

I can't believe 2 months have passed since my last blog post!  A lot has happened but none of it seemed necessary to blog about at the time. But here it is. 
Tiernan and I spent most of September in a modified quarantine and both were MORE than ready to jump back into school with both feet!

We stopped in for picture day and he had such a blast with these crazy boys. 

Put a tie on this kid and he immediately decides he is the most important guy around. 

Tiernan and his buddy Landon had a great time playing while waiting their turn for photos.

Tiernan had his peeps: Adrian, Alex, Nicholas, Vivi and Elizabeth.  (By the way, L is only 3 in the picture above this one....shows how tiny Tiernan actually is when compared with his friends.)

Tiernan was feeling so good about getting back to pre-school.  His frist day back brought out his sassy side.

Our awesome friends (who are really family) The Whalens, found this perfect firefighter costume for T and knew he had to have it.  He has worn the coat to school every day since then. 

 Tiernan got double the pumpkin patch fun this year too.  We went first with our Mended Little Hearts group.  It was such a great day with his heart buddies. 

T, Alex and Christopher after romping around in the room of corn.  Corn is dusty.  I had no idea.

Tiernan found this duck race to be hilarious.  That kid loves ducks.  haha.

 What joy it brought to see these three boys taking on the corn maze together.  What a crew.  I am so thankful for all the heart mams in my life. 

Our fearless corn maze leaders: Luke, Tiernan and Alex.

Next up was Tiernan's pre-school trip.  Since I have next to no sick/personal leave to my name, I relied on my friends who made sure to get some good photos of him. (Thanks Mendy and Carmen.)  

Wow!  Half way up the white pumpkin this year.

Tiernan and his sweet friend Vivienne.  They sure missed each other.

These two found a great slug to investigate.

This little monster was hiding behind the bush at pre-school.  I decided to catch him and keep him.

 Tiernan was looking so forward to Halloween this year.  He was...wait for it....a firefighter.  He wore his full costume to pre-school for their party and, because he wears the coat everyday, Vivi asked him where his costume was.  Ms. Kay told him no one would think he was dressed up.  :)

Tiernan was absolutely the brave one of the group when it came to Trick or Treating.  Tristan and Michael were not nearly so confident at some of the houses.  But T2 marched straight towards door after door and faced some rather fearsome characters, but was only really startled once. 
"That guy scared me right off the step!  And some of my candy fell!"

At most houses, however, he just struck up conversations and enjoyed meeting any dogs who might come to greet him.

He brought in quite a haul.  More than his brother, and so heavy, he needed help toward the end. 
The most wonderful thing about the entire night was that he walked the entire time (save the last block and a half) and was not out of breath.  Last year he rode in the wagon from house to house.  (Very likely burning more calories and energy climbing in and out of the thing every 15 feet, but he was definitely purple and breathless.)

In medical news:
Tiernan has had a lot of appointments with many different doctors.  Some new ones as well as old favorites...including this dude.  Dr. Pickens is back on the job to help Tiernan gain some weight after surgery.  It has been slow going, and with the fluid restrictions and thickening throwing in some extra challenges, he has only gained a little of the weight back.  (He had finally made it to 38 lbs when he went into surgery, and he is just now back to 35.8)

Tiernan also sees Dr. Hanevold now as well.  She is a nephrologist, or kidney specialist.  He is on such a heavy diuretics regimen, that Dr. Park decided it would be best to have her involved.  She spends a lot of her time dealing with kids on diuretics, and he WAS technically in kidney failure.  So she now orders and reviews all the blood work which plagues this kid's life.  He is currently on giant amounts of Potassium Chloride pills to keep everything in order.  If his electrolytes were to get out of whack, then we would start to see some heart rhythm issues.  We definitely don't want to add that into the mix. 

From a cardiac standpoint, a month ago, things were looking incredible for those four chambers of his.  We knew, even by this point, that a valve surgery would be in his future, but were hoping things would last a good 10 years before that became necessary.  Current replacement valves don't grow with a patient, so any replacements in children are only temporary until they are old enough for an adult sized mechanical valve. 

The upper right part is the left atrium being measured at last month's echo.  

Here is the same view from last Wednesday.

His left atrium is quickly becoming dilated from the regurgitation through the mitral valve.  This is pretty disappointing.  The implications of this are that his heart cannot handle the amount of leakage like we hoped it could.  Dr. Park elected to first try him back on Enalapril. (Blood pressure med)  However, while inpatient, Dr. Reddy was not happy with how low it brought his pressures and took him off.  So we were told to keep an eye out for signs of dizziness, generally low energy, etc...

The first dose was Friday. 

I gave it in the morning and by the time I got there at lunch to give his meds, he was complaining that he didn't feel so well.  And then by the time I arrived to be there for the halloween party, he was sitting in the middle of the carpet, pale, and not participating in any of the party activities.  He looked at me and again said he didn't feel well. So we decided to skip the party and head over to see Dr. Park instead. 

Well, by the time we arrived, he had pinked up a bit and his energy was up.  His BP was a solid 96/58 (or something right around there) so we knew that was okay.  However, in order to really rule out the enalapril as the cause of this, Park told us to d/c the med for the next week and then give it again next Saturday.  If this happens again, then we know he won't tolerate the dose.  If it doesn't, then Friday was a fluke and we continue with the Enalapril. 

Please pray for the second response.  He needs the medicine. 

Regardless of whether or not he can take Enalapril, he is going to need that valve repaired or replaced much sooner than we had hoped.

And, while you are praying, add one regarding my insurance.  Last time it was covered because Hanley was the only surgeon who could do it in our area.  This valve would be different.  Our local surgeon, Dr. Chen could perform this surgery.  He does them often.  But I really would rather not have a 4th different surgeon opening him up.  Hanley did such unique work in there, and he would remember it.  Another surgeon would not know the intricacies of the patches and, although that doesn't mean they couldn't successfully deal with the valve, it does mean that Hanley could do it better, in my opinion.  So we pray that Chen will write a letter stating that Hanley is still the guy for this job, whenever that may be. 

Overall, though, Tiernan is still doing GREAT!  Dr. Park just wants to treat any changes aggressively to stay ahead of things.