2 week check up

Macsen had his 2 week check up after his pacemaker was fixed. I felt like the whole appointment was one big good bye - I don't think they could get rid of us fast enough. The EP doctor declined to see him even though he needs his pacemaker checked.  We've been seeing the team in heart failure  every 3-4 weeks for 14 months but we can no longer trust that Macsen is in the right hands.
For months after the pacemaker mysteriously stopped working we watched Macsen decline. We watched his labs get worse - his BNP rose, his kidney labs worsened. He was increasingly fatigued. He was miserable - uncomfortable in his own skin, unable to play, to sleep, to grow. His local cardiologist just told us that was just just his heart failure - he never even looked at a single EKG. If he had he would have noticed that his QRS had widened like it was pre pacemaker placement. AND HE COULD HAVE FIXED IT - right then. Instead, now, Macsen has been diagnosed with Stage 3 chronic kidney disease - no doubt bc for months with his heart beating out of synch again he wasn't getting enough blood to his gut and kidneys. He would cry because his stomach hurt so much. He would sit with his teacher or the nurse at school because his tummy hurt him. And that was completely avoidable. All that damage ....
And immediately after his doctor in Boston turned all the leads back on Macsen said "I feel better." and he's dancing again, and singing. He's doing that thing kids do when they make up voices for their toys and enact stories - all by themselves. He's not having stomach pains.His labs are all better! His BNP went from 3200 to 2800 in 2 weeks. His BUN is in range again after being very high 2 weeks ago. I don't know if it will be enough to keep from having to go the transplant route but I'm so happy to see him not miserable.He is not a meek little mouse when he doesn't feel well - he is a lion.
So - we are in the market for a whole new local team. I hate it - there is so much history and information lost when you change teams. Macsen has years and years of records - of labs and tests and surgical notes. It takes forever just to get all the centers to send them not to mention how long it takes to read them and or enter them into a new system. And all the phone calls and emails, the questions asked and answered that I've already explained many times to many other doctors. All the " Mac reacts to this, and that. and he had a terrible episode when this happened. And he got blood in his stool on this date and it turned out it was this after seeing 4 specialists." All the anecdotes. And on top of all that is TRUST. Macsen has been badly, badly burned bc I trusted the wrong doctors.
And changing to another facility capable of monitoring him means moving his local care to another state - again :( Means tacking on another hour to an already 3 hour long monthly drive.
And all that for hopefully just a few months bc I think he belongs at Boston and we need to figure out how we can do that. Looking at real estate prices in that area we have decided we can barely afford to live in a box under a bridge and that's is I sell my plasma lol. 
We have cath dates for mid May but that is too far away with no monitoring in between.
I've sent out so many emails - now just waiting for the responses to start rolling in.

This will be known as the pivotal moment of clarity

Mac's home cardiologist has reviewed all his EKGs and found the problem. Apparently in October somehow the threshold dropped for one of his leads - I'm still not clear how that happened but I'm told it just does somehow- and his cardiologist said it was obvious when reviewing his EKGS. He admitted to missing it at the time - he gets monthly EKGs- and said he could have easily fixed it at that time if he had noticed :( 
So since at least October his heart that was beating out of synch has been getting stimulated to pump only on one side - causing it to beat even more out of synch. He also said he never believed the pacemaker was going to help (even though we saw it helping) and that he felt it was giving us false hope and that not fixing the lead that wasn't working didn't do any damage to M's heart which is contrary to what Boston told us. I can't help feeling because he never believed in it he didn't do his due diligence to make sure it was still working correctly. And he mentioned that thresholds need to be adjusted frequently but he never mentioned that during all the months we were watching Macsen decline, he never said "Maybe we should check his thresholds since it can be a common problem they need to be adjusted frequently." I am so angry but it's that emotional anger where I will probably cry if I try to tell him exactly how angry I am with him, how disappointed and devastated. And that would be worse for me - I will never cry in front of that man. 
I told him I want to list Mac for transplant in Boston and he agreed that would be a good thing. 
Sometimes I just have to think that these crazy things that happen are pushing us somewhere else and I have to try to find God's plan in it. 
Boston has said setting everything up will take time and he needs to be monitored every few weeks as we up his meds, etc. As I hope this will be our last local visit and I couldn't possibly find a new local team in the next few weeks I will take him to the  Birmingham transplant clinic for the 2 week follow up. This sucks.  

Making plans and making memories

So we met again with the head of the heart failure department and tried to make a plan. As of now we will come back in 3 months for a cath and to see if the pacemaker is improving his function at all. Bc it may be too late for that we are also planning on doing the transplant evaluation at Boston Children's in the next month. I know the wait may be longer but honestly they are the only ones I trust anymore.
Listing here will mean Mac and I at the very least will have to move to Boston indefinitely. I will make this happen. We will work on finding a solution so the girls and Aaron can come too but for now I have to focus on getting him somewhere he will be safe ASAP. 
His Aunts are here and we are going to enjoy the next 24 hours in Boston - it's brutally cold so sight seeing may not be extensive. Macsen has requested a train ride and the art museum is free today so we will squeeze that in. Thank you everyone for your continued prayers

Heart is much worse

Macsen had a 2nd echo and a 2nd EKG done. His function is much worse then it was post pacemaker placement and his valve is leaking more. His cardiologist up here was visibly shocked at how much worse it looked. Alabama card never said he looked worse - just that he wasn't looking better :( Boston card confirmed my suspicion that bc his pacemaker was only partly working for so long it further accelerated his heart failure. EXPLETIVE EXPLETIVE EXPLETIVE! As if my baby could tolerate that! No wonder he was looking so much more tired and cranky. Why wasn't this caught in his monthly echos or monthly EKGS. The doctors here pointed out how it was CLEARLY evident when you looked at his EKG when the pacemaker wasn't working. I am so upset I can barely function - I just want to break down in tears but Macsen is so perceptive and already so afraid about transplant I don't want him to see. That's what showers are for right? Crying your eyes out in private?
Mac's Alabama pacemaker team wanted to see us every 6 months but Boston was shocked by this - a new pacemaker needs more frequent monitoring and adjustment apparently. I didn't know this. I thought bc it was remotely transmitting every night any problems would be caught but apparently it doesn't. I'm so angry with myself. I should have pushed harder when I noticed him more tired - especially since he improved so much immediately after surgery.

Holy Heck

We saw Mac's electro physiologist Dr today - Dr.Mah- and Macsen's pacemaker has not been functioning correctly . Probably for months!!! Dr.Mah was able to fix Macsen’s pacing so his pacemaker is now doing what he had it put in for in August - to get his heart to beat in synch - both sides squeezing at the same time. It was pacing like that in August and for a month we saw visible improvement in his function. That means that maybe now he will improve again!!!!! Key word is “maybe”though and it also means we need to figure out what the holy heck happened.It has not been pacing both sides for a while - one lead wasn’t firing at all so his heart has barely been getting any blood out to his body 😞 We don’t know exactly what happened but I noticed him declining in September. The Alabama pacemaker team changed some settings at that time. I don’t understand how monthly echos missed his heart was again beating out of synch. And I feel sick to think how much better he could be doing now if this had been caught months ago - I should have brought him to Boston for a 3 month check up instead of waiting for the scheduled 6 month 😞 
So now we start over - waiting to see if his heart function improves. This has set him back months but hopefully with time and prayers he will get better. 
It’s ironic that when we first saw his pacemaker dr here, Dr.Mah - I said “I’m hoping you can find something that is not working but is an easy fix” and boom! He delivered! We are setting up more frequent visits to see him for all Mac’s future pacemaker monitoring. 
A few more appointments today - thank you everyone for continuing to pray for Mackie 

Made it to Boston

Pilots for Christ is an amazing charity that has helped us get airline tickets a few times. We absolutely adore Johnny and Rosalyn Sales who run the local chapter. They helped us get from Dothan to Boston on Delta this time. Such a blessing!!!
Macsen loves to fly and is so excited about seeing his doctors and Boston again. Also Abby Gabby and Tante Trina are coming to meet us on Friday. Mac is tired and his tummy hurts him a lot. I think it's not getting enough blood at this point. I'm praying Boston has some miraculously easy fix - I can dream right? I'm thinking we are going to just discuss listing him though.