Fontan Failure

Hello friends and family! 

We have made it. We can finally breathe a little, now that we have come out on the other side of this. 

We have been home for a little over a week and it's been a wonderful week, let me tell you! We originally came home 2 weeks ago, but Amelia must have missed her favorite nurses, because we were right back in the PICU for 3 nights. 

Let me recap y'all. Honestly, this is incredibly therapeutic for me, to write it out, so bare with me as I work through all this. 

Amelias Fontan was on August 31st. That morning was particularly difficult because she had been in such a great mood leading up to this and I didn't want to cry in front of her and send her into the OR sad. Once everyone started coming in, I couldn't help it anymore. I feel so bad that she is unknowingly walking into something incredibly difficult. David took her in the OR and she happily played with a toy, as she drifted off to sleep. The OR doesn't scare me, it's what happens after the OR that scares me. Amelia is proof that things can go really well in the OR and then all hell breaks loose when she leaves. She did really well during surgery and the following 24-ish hours were pretty typical, post open heart surgery. She took a few steps the morning after, she ate a lot of baby food pouches, and a shake. Mostly, she wanted to drink, but after the Fontan, patients are fluid restricted. So she was only able to drink a half an oz-1oz every hour. That was the hardest part at that point. She was just so thirsty. Then suddenly something switched and she was no longer consolable. Her tummy was getting bigger and harder. There was a lot of thrashing and screaming. David and I had to hold her down a lot. It was brutal. At one point, I had to leave to take a break. I intended to stay away for the night, but something in me told me to go back. When I got closer to her room, I knew something wasn't right because her room was crowded with doctors and nurses. I walked in at the same time her heart surgeon walked in. They were doing an echo on her, to see if there was something causing problems. They found a large pocket of fluid in her chest, where one of the chest tubes clogged and wasn't draining. A ton of fluid came out and by the time I left that night, I was told that, that was likely the cause of all the discomfort she was having. Just before I left, Amelia had an all out thrashing fit. It took David, Shardha(cardiologist), and I to hold her down. All I want to do is pick her up and console her, but she's so far passed being consoled, that we needed to get a drug into her, to get her to sleep. Definitely not how I wanted to leave her. 

Side note: Amelia's cardiologist is so dedicated to her that she rushed back to the hospital that night and was a little impatient, waiting for her garage door to open, and smashed her car into the garage door. Clearly, she's invested in Amelia. 

The next morning I got back to the hospital, where David told me that the doctors decided that Amelia will need to be sedated for a few days. The thought was that her body may need time to catch up to the changes that were made in her heart. I was really sad, because I didn't want to have her sedated, I wanted to have her awake and moving forward in the healing process. I left to get us breakfast and David text me to tell me that she was being put on the ventilator. Part of me was sad and then the other part was relived. At least she can just rest comfortably. The next couple days were so so. We did find out that the fenestration, put in during her fontan, had closed. Of course, because why not throw in another complication. On Sunday, September 4th, the decision was made, that she would go to the cath lab for her fenestration. We were hoping that if that was open, maybe things would get better. 

They did some pre cath labs and suddenly nurses were rushing in, giving a couple different types of fluid. I remember thinking it was weird because of her fluid restrictions, and they're just pumping her full of these fluids. I never thought it was serious though. I thought maybe it was just prep for the cath. They kept coming in and giving more and more of these fluids. Then the parade of doctors and NP's coming in and out of her room. Finally I heard someone mention Petros(Dr.A, her surgeon). We saw him talking outside of her room, he walked in and looked at her, and came over to us. "We need to put her on ECHMO if she is going to survive."

When I think of someone dying, I think of someone with cancer getting more and more sick, and passing away. I think of a freak accident, a heart attack, something other than what we experienced. On September 4th, death snuck up on us. We had no idea it was creeping in. The labs they did on Amelia, showed her kidneys and liver(I think) weren't working. Then they did an X-ray and it was horrid. Completely foggy. You couldn't see her heart or lungs. David and I were sitting next to Amelia and had no clue what was happening to her. 

I can't even put into words, the feelings I had when they talked about ECHMO. We just knew she was gone. She had a 1/3 chance of survival and it didn't look good. I've never felt so many things at once. Anger, desperation, sadness, and pain. It was all happening so fast and there was nothing we could do. How is it, that we walked a happy 2 year old into the OR and now we are losing her? I was angry that we were being robbed of her future. Angry that our last waking moments with her, were brutal. Ive never felt desperation like this. All my thoughts were screaming, and I just wanted to fix it, but couldn't do anything, and I was in physical pain, thinking about missing Amelia for the rest of my life. 

They moved her to another room and were able to take their time, placing her on ECHMO. 

That night I slept with her lovey. When I woke up at 1am, I pictured how she would shove the lovey under her cheek as she snuggled into her bed, and I wondered if I would ever get to see her do that again. I was up for the day and 1am and scared to go ask how her X-ray looked. Thankfully it looked a little better and that was enough to hold us over until the next day. Her x-rays got better everyday she was on ECHMO and we really felt hopeful that she was actually going to make it. 

Through all of this, we had such an amazing support system. Made up mostly by strangers. I can't even count how many people we don't know, who were praying constantly for Amelia. 

On September 8th, they decided it was time to take her off ECHMO, but she needed the Fenestration first. So they were going to take her to the cath lab, do the fenestration, and then see how she does off ECHMO. That morning, right before they were going to take her, she started pouring blood out of her mouth. When you're on ECHMO, you're on a lot of blood thinners, so it's likely that during a nose suction, something was knicked, and thats what started the bleed. It was definitely difficult to watch and not how we wanted to send her off. 

Not long after they took her for the cath, David got a text saying that a doctor was coming to talk to us. It was also the same time I got in the shower. Ive never showered, gotten dressed, and gotten to the hospital so fast. Thats when we were told that the Fontan had a large clot that was formed inside of it. They had talked to every cardiologist in the hospital, both UW and Childrens, and they all had decided that they were going to leave it there. The risks are that the clot could break off and travel to her brain, causing a stroke. It's a big deal. It looked like it had been there at least for 24 hours, and might just stay there. The alternative was that they would have to go in and take down the fontan, and put the Glenn back up, which we didn't want. If we could avoid another open heart surgery, that would be great. So they did the fenestration, and tried taking her off ECHMO, but her number just kept falling. So, not long after they told us about the clot, they came in to tell us that she was in the OR, and DR.A was starting to take down her Fontan. Talk about a huge blow. 

We walked a happy 2 year old, with a Glenn into the OR in the hopes that she would be so much better in the long run, and here we are about to lose her AGAIN and she would be back to a Glenn. 

I'm going to stop here and will pick up where this leaves off, in my next blog. 

This is a lengthy post, so if you made it all the way through, good for you!

Love y'all!

Kelly