Waiting on dates

ALMOST have our dates for cath and surgery in Boston. We are hoping for the first 2 weeks of July so Mac will have time to heal before starting kindergarten the first week of August. I pray these interventions work so he can feel better, start growing again, go to school and make friends, and do normal 5 yr old stuff ❤️ I know we are not supposed to get our hopes up but I’ve never been very good at that. That is how we have made it through everything so far - hope and prayer and on Mac’s part sheer will.
*** He made this cool stegosaurus hat with his new kindergarten teacher Ms.Rhonda Kraft who he loved!

Second opinions are finally coming in

I reached out in December to Boston for a second opinion. I have had a  ridiculously hard time getting his records sent from all his different doctors. Seriously - what should take 1 phone call instead required a dozen calls, faxes, emails. We still don't have any actual records from Nemours in Pensacola. Frustrating doesn't even begin to describe the process.
So after reaching out to other Boston heart moms on FB who emailed and called and texted their cardiologists at BCH within 24 hours the cardiologist called us.
He said Macsen's heart is beating out of synch - disynchrony. Instead of starting the beat at the top and squeezing down the heart to have an efficient pump,  his is beating erratically. He is still in heart block months after being on the digoxin.  So Boston wants us to come up for a cath - Macsen's 2nd this year- and if the electrophysiology study goes well they will perform another open heart surgery, (#4) on my little man to place  a pacemaker - something called CRT (cardiac resynchronization therapy.) He is too small to have it placed via cath or small incision.
His CHOP team doesn't do CRT in single ventricle children and they think we need to list him for transplant - that his heart is just done. But his cardiologist there is doing more research - reaching out to her contacts at other centers that use CRT. There is very little published research on it and even the Boston team keeps urging me not to "get my hopes up." They tell me "sometimes it's a homerun" and sometimes it doesn't help at all. If it was all for nothing that would be devastating after putting him through the trauma of another surgery - he's older now. He will remember this one. But if there's a chance it will let his heart function better and keep him off the transplant list a while longer is it worth the risk?
We are now waiting for scheduling to contact us with the date for the cath. I pray this pacemaker is the answer to our prayers.
Macsen is still his funny, smart little self. Today he wore his cowboy boots and Woody cowboy hat with his Lightning McQueen shorts and shirt to his sisters awards day at school. He makes me smile all day long.

Adventures with fluid intake restrictions

Day 2 of monitoring Mac’s fluid intake. He had already had a lot of water this morning but kept asking and I had to say no. Finally he took matters in his own hands, pushed his chair to the fridge and climbed in (our water dispenser is inside the fridge.) Then he panicked and couldn’t get down - still holding his cup of ice ready for the water. Poor buddy
We heard from Dr.Marx again who has been discussing Mac’s case with the surgeon and also with Dr.Mah, the director of BcH’s pacemaker and cardiac device program. They are still thinking a pacemaker may be the way to go but urged me not to get my hopes up yet. Still waiting on the final word/plan

Too much water is bad?

I talked to Dr.Blume Director of the Heart transplant and heart failure management clinic at BCH today and she gave me so much info I am amazed, and embarrassed, I didn’t know. 

Mac is on 3 diuretics but still holding onto fluid. She said bc he is in heart failure and his heart is not putting out enough volume his body is sending out a hormone ADH telling him “drink, drink, drink! We need more volume!” So he does - all day - which his other centers have said was good. “Dehydration is bad” is what we always heard. But she says the extra volume is too much for his overworked heart, it’s accumulating in his tissues and we need to restrict fluids. She also guessed his favorite food - salt. His favorites are bacon, popcorn and boiled peanuts, avocado but only with salt, on and on that make him drink even more. So he drinks more and his heart is overwhelmed even more. We have to start weighing him everyday and she wants to see him lose weight - water weight. I am happy to have some input that may help him - his bnp is not going down- but the thought of taking away most of the only foods he eats and telling him he can’t drink when he’s thirsty 😳😳😳 It’s going to be tough. But we will do everything to help him feel better even if he hates us for it. The dr needed a few more days to consult with another doctor but hopefully vet soon we will have a plan for the next few months.

SOS

After 5 months of waiting for Mac’s second opinion from Boston - calling every week and being told to continue to wait- I was fed up. I sent out an SOS to other Boston Heart Moms on FB to help me get a dr to talk to us. They got to work emailing and texting their cardiologists at Boston and boom! We got the call this morning at 6:30 am. I am beyond grateful! 
The dr there says Macsen’s heart is not beating in sync. Apparently this is written in his mri report from January but it was never brought up or explained to us as being significant. He believes with a special pacemaker we may be able to get Macsen’s heart to beat in synch. It’s a big “maybe” and “hopefully” and not a guarantee that it will work but if it does it could mean improved heart function and more time before he needs a transplant. He will talk to the surgeon up there so we will know more next week.
Through another new heart mom friend I was able to get in touch with the Director of Transplant and heart failure management at Boston and she will call me next week as well.
So the ball is finally rolling!

BNP is up again

BNP was higher :( 1600 up from 1400 when he was last hospitalized. Normal bnp is less than 100. I just want to cry - I feel so devastated. His heart failure is just not getting better despite all the new meds.
More sad news - our foster kitty Layla got out after we got home from Birmingham and is missing. She’s never been outside and we’ve been looking for her all day. **** update! We found the cat. She was mad at us for leaving her when we went to Birmingham.

Appointment

Update: labs looked good indicating his liver and guts are working ok. Still waiting on the BNP.
Echo looks unchanged - no better but no worse. They had a very hard time getting his bp and I don’t feel the number they got was accurate (much higher than normal.)

Blood draw and blood pressure done and now onto echo.