Change in plans...

Warning, this one isn't going to be fun to read. I need to write everything down so I can process it. But, it is going to be medically heavy.

We had to get through the three day weekend, which wasn't easy. I thought for sure I would hear something from her doctor on Tuesday since they probably received the results of her CT scan. Then at 6:30pm he called. He asked if Tom was with me and I said yes. He asked if he was on speaker and I said yes. Clearly this was going to be a lengthy call and he wouldn't want to do it twice. Totally understandable. 

He said they had conference and went over her CT scan, her most recent Echo (done in April), and her heart caths (many over the years, but her most recent one was in 2022, I believe). Here's where we are:

1. Pacemaker Revision: the surgeons feel that because of her heart anatomy (her heart is not normal - it is in a mirror image and has all kinds of craziness to it) they feel that the only way they can access her ventricle is by doing a full sternotomy (where they cut through the sternum to gain access to the heart). So, since they must do a full sternotomy, they might as well replace the atrial lead as well. So, that's the plan as far as her pacemaker goes. 

2. She has leaky valves in/around her heart. I've heard "tricuspid regurgitation" for years. Seemed like it was mild and not a concern. However, she now has some regurgitation with her aortic valve, or it is worse than it was. I don't remember ever discussing this issue. So, it is probably new. 

3. Her Fontan is too small. I really don't know how to explain this to people who have no idea what a Fontan is. There is a post I wrote that gave a basic description of her heart here. But, I really don't expect anyone to read that. So, the simplest way, I think is: 

In a normal heart the right ventricle pumps blood to the lungs to get oxygen. The left ventricle pumps the oxygenated blood to the body. But, she didn't have two ventricles. She had a VSD (ventricular septal defect) that is essentially a hole between the ventricles. Hers was so large, she was really a single ventricle. Basically, she only has one pump that goes to her body. The Fontan is a surgical procedure that re-routes the blood flow so that the blood coming back from the body (which needs oxygen) goes straight into the lungs first, to get oxygen, then goes to the heart to be pumped back to the body. 

Because of this new circulation, blood flow to the lungs depends on pressure and smooth circulation. It is definitely less efficient than normal circulation. Over time, the pressure (which can often be elevated) puts a strain on other organs especially the liver. That is called Fontan Associate Liver Disease (FALD). Unfortunately, Harlie's pressures were elevated for years. And we know she has FALD, and that her liver is congested and appears fibrotic on scans. 

Anyway, her Fontan being too small (think highway reduced to a single lane road) is a big deal and they cannot ignore it. So, since they have to do a full sternotomy, do they need to address her too small Fontan while they are in there? Well, a Fontan revision is a major surgery. So, they want her to have a heart cath first. They want to see if they can help her Fontan by putting in a stent to open it up and they are thinking that can be done during a cath. Her doc said she got a stent several years ago, but I don't remember the specifics. Well, I just searched my blog and wouldn't you know - I found it. Here is the post about it!  Now I remember! Haha! They also want to do an esophageal echo where they do the echo from inside her body versus on top of her chest (which does not give them great images/info). 

Also, if you read the post from December 2017, spoiler alert - she still has exercised induced intolerance. Just today, I asked her if she would like to go for a walk with me (well, she rides in her chair and I walk next to her). She said sure, but she had her pjs on. So I said you need to get dressed. She was like, ugh. So, I asked her if she wanted me to go upstairs to get her clothes and bring them downstairs for her and she said yes. That is not her personality. She is very independent and likes to do things for herself. But, physical exertion - that's a different story. She just can't. 

Anyway, they are trying to move some patients around to get in her next week. Then, based on how the cath goes and if the stent works, they will make a surgical plan. As of right now, they are thinking she would have surgery in mid-June.

Right now, we are hoping that the stent works. If it doesn't, then we might be looking at a Fontan revision and that sounds really ugly and scary to us. 

I just received a call that her heart cath is scheduled for Tuesday, June 2nd. 

Okay, so here's how I'm feeling. Terrified. I never want to speak for Tom, but he's probably terrified, too. Look, we knew all along that her heart was going to become a bigger issue. But, that does not help. Not to mention that this essentially came out of nowhere. We were doing just fine and now I feel like the wheels are coming off! We are not prepared for the what ifs, nor will we ever be.  No prior knowledge makes the future hurt less.

After we spoke to the doctor, Tom called his mom. And I realized that I can't call my mom. There is no one who loves your children like your parents. My mom loved Harlie so much. She was so proud of her. She was so impressed by her. I'm so grateful Harlie has her Grandma and her Grandma loves her like my mom did. But I am sad that she doesn't have her Nana anymore. I'm sad I don't have my mom to gasp and cry and have all kinds of reactions that - at the time - I called dramatic. 

Okay, well that's it for this one. I'm happy I was able to get her outside for a few minutes. Plus, we got to see a cute little family of geese. 

As always, thank you for reading and caring. 

Much love, 

Christy xo