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)
