Thursday, July 14, 2016

Post Cath and Meeting w Hanley

Alright so, a lot has happened in the last few days. Following Micah's cath, the cath doctor - Dr. Peng - gave us some encouraging news!! Based on the pressures out in the distal branches, she doesn't think Micah has pulmonary hypertension!!! This is AWESOME news because this is the diagnoses that suddenly put a shorter life expectancy on Micah. One less disease to worry about! Furthermore, she proposed TWO options that Dr. Hanley might take to fix Micah's left pulmonary artery. TWO! We left Children's with no surgical options so this totally blew us away. She said there are some narrow branch arteries in the right lung that need to be addressed in addition to reconnecting Micah's left pulmonary artery. We were so full of hope after meeting with her. There is a plan!

Even though we got some good news, Micah came out of his cath with bleeding in his lungs so they kept him intubated. They put him in the PICU because there was no room in the CICU - hopefully we'll head to the CICU today. They've been trying periodically to sedate and paralyze him to allow his lungs to clot and heal so the bleeding stops.



Unfortunately, on Tuesday we were told that Dr. Hanley didn't want to perform the scheduled surgery because of risk for hemorrhaging. We agreed that postponing surgery was the best option... even though we just want to see Micah fixed.

We got to meet with Hanley on Tuesday morning which was another huge hopeful moment! He said for kids with this particular CHD, Micah is a 3 out of 10 in complexity! He is very confident that he can fix Micah. This man appears to have this diagnoses down to an exact science. Dr. Hanley pioneered the process for fixing Micah’s condition - so we know he’s in good hands.


Dr. Hanley elaborated on all of the details, considerations and options for Micah. The surgery has 3 main elements. Right pulmonary artery, left pulmonary artery, and the heart.


(Read on at your own risk - it gets complicated and we’re not 100% sure we’re explaining it correctly - this is just our understanding. We provided a drawing of Micah’s anatomy as a visual.)




First, he wants to fix Micah’s right pulmonary arteries. We didn’t expect Dr. Peng to talk at length about fixing the right side - we were so focused on IF Micah’s left side could be repaired that we didn’t even consider how Dr. Hanley would also want to repair the entirety of his heart. Poor Micah is currently sitting in the unfortunate position of his left lung being in excellent shape but the pulmonary artery is cut off, meanwhile the right pulmonary artery is OK but his right lung has poor distal branches. It would be convenient if at least one side was all good and the other was all bad. His right side is what lead the doctors at Children’s in DC to diagnose him with pulmonary hypertension. There’s so much pressure in his heart - but it’s due to the narrow passage of his right arteries to his lungs. Dr. Hanley can surgically go all the way out towards those narrow passages and enlarge them - something we only thought could be done via a cath!


Second, Dr. Hanley explained the two options for the left arteries. Dr. Peng called them “Ghost vessels” because Micah’s various branches from his left lung are disconnected from the main pulmonary artery - and without blood flowing through them, they’re hard to see.


OPTION 1: Connect the right pulmonary artery to the ghost vessels. This would be the ideal fix. Dr. Hanley started saying things like “full repair” (!)


OPTION 2: Create a shunt that leads oxygenated blood to the ghost vessels - so that they can grow and eventually (somewhere around 6-9 months from now) be connected back to the main pulmonary artery (full repair).


One of the main factors that influence the left side is how small his left arteries are (currently measuring 2.5mm) and if he’ll be able to branch all the way out to them. Dr. Hanley suspects that Micah’s left side became discontinuous due to a “kink” (like a kink in a water hose). He suspects this occurred due to the very large pulmonary conduit being placed next to the very small left pulmonary artery.


The last points that he talked about were how he decides whether to close or leave open the hole (VSD) in Micah’s heart. This was particularly interesting to us because Dr. Hanley can pretty accurately measure how leaving open or closing the VSD will affect pressure in the heart and arteries. Micah was born with a VSD, but during his last surgery - Dr. Jonas partially closed the hole, leaving it fenestrated. The intention of this is to regulate the pressures of the blood flow to the lungs. When there’s too much pressure in the right ventricle, blood can shoot to the left ventricle which leaves Micah with poor oxygen saturation. The part that gets interesting is that Dr. Hanley plans to perform a flow study - right then in the operating room - to determine whether or not to close the VSD. We had never heard of such a thing. As you might remember, Micah gets caths all the time (already had 7) and the caths are the best way to get an understanding of blood flow through his arteries. After Micah’s last surgery, he struggled, went home, did poorly, went back to Children's, had a terrible episode, then had a cath to figure out what was going wrong. Well, Dr. Hanley will basically do a cath -during surgery- so that he knows if the pressures are correct or if he needs to keep going and make further adjustments. This flow study will hopefully prevent a similar episode Micah experienced shortly after his last surgery.


Dr. Hanley is clearly going to have a lot of judgment calls during the surgery, it sounds like the ideal plan is to 1) replace the main pulmonary conduit 2) fix narrow pulmonary branches on the right side 3) see if he can connect the ghost vessels on his left side OR place a shunt 4) perform the flow study.  


We also asked if Micah's diaphragm still looked elevated (something we thought was contributing to Micah's low sats). Thankfully, Dr. Peng said that Micah’s diaphragm looks fine. (I suppose all that praying a few months ago paid off! haha). We assumed that if his diaphragm was OK, then Micah’s oxygen saturations would be OK (which obviously isn’t the case). When we brought up our concerns with Dr. Hanley, he reassured us that he carefully identifies the phrenic nerve (controls the diaphragm) during surgery and moves them out of the way - so that the risk of diaphragm pushing up into his lungs is reduced (but even the act of moving it could cause trauma). These types of things get complicated when there’s multiple surgeries performed - and thus lots of scar tissue - making things harder to identify.  

We really feel that we made the right decision to come to LPCH. We are blown away by Dr. Peng, Dr. Hanley, and the whole team. Their strategy, practice, and team is set up to best help kids like Micah - who have complex pulmonary issues. Perhaps the only way we would have ended up at LPCH is with things becoming as bleak as they did back east. Sometimes roadblocks and hardships in our life are the only things that can redirect us to where we are supposed to be.

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