Yesterday morning Katja’s Dad found her convulsing on the stairs. She was rushed to SCH. She had multiple episodes while in the ER which was determined to be an AV block.

Katja entered complete heart block for 14 seconds last night. She was immediately started on isoproterenol with full cardiac watch overnight. Her teams made the call this morning that an emergency pacemaker must be installed in the next 24hrs.

So yet another cardiac surgery for our Heart Warrior.

On the one hand I’m sad and angry for her. That she is going through all of this. On the other I am relieved that a pacemaker will stop this from happening again and give her body some relief.

Seattle team has gone through every scenario on “what, why and how”. No one knows for sure. The underlying problem is we don’t fully understand the electrical connections in the heart enough to answer those questions. What we know are risk factors:

1) Multiple cardiac surgeries- Katja has had 4 now. Every time they stitch a new valve on the scar tissue is directly affecting the nerves in her heart. Each new surgery increased the risk of this happening (why we did PHT, so she hopefully will never need another).

2) Rejection- We don’t know what rejection looks like in PHT kids. What we do know is that there is a small donor muscle cap around her aortic valve. Could this possibly be rejecting and messing with heart signals? Possible but less likely. (They are currently working to rule this out and so far it doesn’t seem like the cause)

3) Medication- Katja is on a massive cocktail of meds. Azithromycin is known to cause conductive issues in the heart (prolongation of the QTc). While this could be what “pushed her over the edge” it didn’t “cause” it. Her QTc remains borderline/normal. To be safe her team is holding it.

What we do know: She didn’t have a seizure. She passed out/convulsed due to lack of oxygen to her brain (heart stopped). She had this same issue 7 days after PHT (a warning that her AV node was injured). It stayed silent for months (very common). We are EXTREMELY lucky we caught this. Had no one saw what happened she’d likely been found unresponsive after a full and complete stop.

What I know: I will never stop advocating for my child. I knew something was wrong. I pushed hard and long. Even when her EKG came back normal I made them keep her on continuous monitoring to wait for it to happen again. When she had an episode similar to this back in February I pushed for answers then too. We had a flight out to Boston in 1 week (set for Easter morning) for 2nd opinion consultation. I was not taking “everything is fine” for an answer. I know heart block is extremely hard to diagnose, it’s hard to catch.. unless like yesterday a “cluster event” is triggered. But no one knows my child like ME. I advocate because I have watched it time and again be the difference between life and death for her. I’ve watched it affect her life, her health, her mental health and her education. I won’t ever stop advocating for her. I haven’t taken my battle armor off in 10.5 years, I don’t plan to now.