From M’s journal:

There has been another bump in the road.

At the end of April we started noticing Tiptoe’s sats getting lower, and it turned out that her ASD (hole between the atriums) had closed. It was talked about getting her in to do a blade septostomy (which is able to be done through a heart catheterization.) This is a pretty risky procedure even as a cath, so it was decided that we would wait and hope that her sats would stay stable(primarily in the 70’s).

Fast forward til now… Tiptoe has been vomiting for about 12 days now. Initially we thought she might have a stomach bug, but as the time has gone on, that scenario is losing it’s credit. No one else in the house has had vomiting, etc.. So I am pretty sure this is cardiac related. Initially she was acting fine. Playing, vomit, playing again. Not what normally happens when you have a bug.

Prior to the vomiting, Tiptoe has been having a difficult time eating again. She could go for a few days and eat practically nothing, and then she would pick back up and eat again. So it’s been a while with these eating issues.

I took her to the cards this week after 9 days of vomiting. He said it could easily be cardiac related, but not much we can do for her. Her heart function is down, and he noticed she has developed pulmonary venous hypertension which wasn’t there in April. Her sats seem to fluctuate, a day in the 60’s, and day in the 70’s etc. He thinks that her veins constrict and tighten, making the 60’s, then she is able to relax and the sats go back up. So she is in a cycle with that.

So what to do?? It looks like we are in a tight spot here. The opportunity to do the septostomy is gone. Apparently the scar tissue is too thick now to do this as a cath, and now it would require an open heart with a heart/lung bypass. Obviously that comes with great rick, and he wasn’t really sure if it would help her as much as it could hinder her to do another open heart.

She is too small to do the fontan surgery, and because of the vomiting issue, she’s not in a position to be gaining any weight. So the card told me that we are between a rock and a hard place with her right now. She needs to grow, but can’t, might benefit from surgery, but is it worth the risk? etc… If she can manage to grow enough, she would still need to keep her sats stable for a YEAR! That seems like a long time to me!

The problem with the pulmonary hypertension is that it can lead to permanent damage on the pulmonary veins. If this gets too bad, then she would no longer qualify to have the fontan surgery because of the damage. So we can’t let that happen, but at this point, they aren’t sure if they can prevent it. It wasn’t a great appointment, and it feels terrible to be helpless here.

The day after her cardio appt she started getting worse in a hurry. She was like a lifeless doll. Completely unresponsive, suffering from dehydration. Somehow Scott was able to get her to take Gatorade (I tried all day to get her to take anything and she refused., and miraculously (we’ve been praying a lot!) she kept it down. Yesterday she was feeling a little better, and today she seems to be even perkier. Gatorade seems to have re-hydrated her, but we have got to do something about her keeping things down. She didn’t vomit yesterday until afternoon, so all gatorade from the morning stayed, and this morning she is still okay.

She is scheduled for a cardiac cath on July 6th to check to see if they are missing something. They are also going to document the pulmonary damage and make sure it is still in a safe place.

I hate to see her feeling so terrible!