To Fontan or not to Fontan

Hello!

It's been a hot minute since I've updated everyone with what's going on in Amelia's world.

When I left y'all, we had met with the Dr at CHOW, and discussed transplant. He did a fantastic job, explaining the process to us. We have a more accurate picture of a transplant, which is helpful, because before, the idea of it was pretty terrifying.

I talked to the Dr from CHOW, shortly after he presented Amelia's case to a room full of doctors, and the general consensus from them, was that we should give the Fontan another try. Dr. Kindel explained that although that was what they thought would be best, he didn't necessarily agree. Amelia's cardiologist in Madison felt the same way, she doesn't feel the Fontan is the best option right now.

Yesterday, we met with her cardiologist in Madison, to touch base, and to get on the same page. We love Amelia's cardiologist, who cares about Amelia so much. She feels pretty certain right now, that Fontan is not a good option for her.

Amelia will do the evaluation for a transplant in March, and assuming all the tests come back in favor of transplant, she will be listed. She is going to have a crap ton of blood work done, and in that, we will get to see if she has a lot of antibodies from all the stuff she's been through. She's received a lot of blood product during surgeries and recovery, so that number could be high. From what I understand, if that number is high, it makes her wait on the list a lot longer. There is also the chance that it's too high, and if that's the case, we will revisit the Fontan.

I thought I would feel a lot worse about this, but we are relieved to have a plan. We haven't had any idea of which way this was going to go, for too long. Honestly, its a bit of a bummer as well. The Fontan, given that everything goes well, is a nice, pretty package, compared to transplant. Pick a date, show up, recover, go home, and move on with your life. We would have done it this summer, so Kindergarten would have started without the worry of her stamina declining. With a transplant, we worry about a lot of things, but one is that she will eventually need a lot more assistance than her peers, and we hate the idea of Amelia feeling different than them. BUT, today, I choose to think about the positive thing, which is that we have a path. It is a bumpier, curvier path, but we have it. We also have Amelia, so that's a blessing for sure. 

Thanks for reading, friends. We love and appreciate your prayers and support.

 

What is happening?

Hello!

Whoa! Does anyone want to tell me where the last couple of years went? I cannot believe that Amelia is 2 years post Glenn 2.0! It's been a wonderful/scary/relaxing/anxiety ridden 2 years. 

Just really quickly, I want to fill you in on how Amelia has spent the last 2 years. 

She started school! She did an early childhood special ed program, when she was 3! When I say that she loved school and riding the bus more than she has ever loved anything in her life, I mean it. Homegirl, LOVES school. Since starting school in 2017, she also started dance, swim lessons, and 4k! In February, we had the fantastic opportunity to go to Disney World with Make A Wish! It was an amazing trip, so magical. Now here we are, just finished celebrating her 5th birthday. She's real ticked that her birthday is over, and that its time to move onto Mabel's birthday. haha!!

Health wise, Amelia has been pretty good. We have gone to regular cardio appts in Madison. When she started school last year, we had an IEP meeting with the special education director, and all of the people who would be working with Amelia. We had goals for her to hit, and she did great, hitting those goals. She was having physical therapy and speech therapy. By the end of the year, PT thought she would be fine without for this next school year. At the first meeting, back in the fall of 2017, I remember talking about what she was capable of doing, and what her stamina was like. She could walk the hallways without assistance, and she rarely wanted help or breaks. When we had her IEP meeting this fall, that was different. She's needing many more breaks, she's asking to be held more, and she can't make it more than the length of a school hallway before she's needing help. There wasn't an event that I can remember, where it was apparent that things had changed, but it did kind of happen all of a sudden. 

So now we have these conversations about next steps. Amelia is outgrowing the Glenn 2.0. She's not in dire need of anything right this very second, but we need to be making plans right now. As a parent, one thing that you really don't want to hear is, a brilliant cardiologist tell you that they're not sure what to do. Amelia isn't a good candidate for Fontan, but its not out of the question. Amelia had a cath, back in September, to measure many things, but most importantly, the pressure into the lungs. Ideally that number is low. A few months after Amelia's Glenn 2.0 surgery, she had a cath and those numbers were suuuuper high. Thats when she was put on a handful of medications to help with that. This last cath, the numbers came down considerably, but not enough to make this a super clear answer. She has other things going on that can't be measured with a cath or MRI, that could make the pressure number actually much higher. SO, This month, she had an MRI, to see if they could get an even better picture. Eh. They got more info, but it doesn't help answer the question, of what the next step is. 

So, we went to Milwaukee yesterday, to meet with a new cardiologist at the Children's hospital of Wisconsin(CHOW). American Family Children's in Madison doesn't have a heart failure/transplant team, and CHOW does. Going into the appt, I was already snobby against CHOW, because there isn't a better place than AFCH, but I was pleasantly surprised to see how much I liked CHOW. The Dr. is fantastic. His name is Dr. Kindel, and he did a great job, explaining the process of a transplant. He spent an hour and a half, talking about a fontan for Amelia, and a transplant for Amelia. We have zero answers with what we are going to do next, but we have a clearer, more accurate picture of what a transplant looks like. 

Now Dr. Kindel is going to present Amelia at conference, and see what other doctors have to say about her complex case. They could all recommend Fontan, or they could recommend transplant. Our regular Cardiologist has said that she will likely agree with what they say. 

SO, now we wait to hear back from Kindel in Milwaukee. It could be a couple weeks. If they recommend listing Amelia for a transplant, he thinks she could be evaluated and listed by February or March. If the plan is to go forward with the Fontan, then that will happen some time this Summer. 

Everything is so up in the air right now, and I don't even know how I feel about any of it. 

I really wish that Walt Disney World had a program where you can hide from reality, in the castle, for as long as you need. For me, it would be until Jesus comes back. 

We don't have answers. We don't even know what we are hoping for. Truthfully, the Fontan is so scary for us. We've done it, and it went terribly, but it would be great if we could do it, and everything went well. Transplant is complicated, and can happen at any time. 

Right now we would love prayers for Amelia's lung pressures. It would be wonderful if they could come down even more. We would also love prayer for the teams that are trying to come up with a plan for Amelia. A clear direction and answer, would be lovely. Also-if you want to pray for Jesus to come back, I wouldn't stop you. :) 

Love y'all! 

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

Fontan

Hello Hello! 

I was SO hoping to not have to make this post for a couple of years, and yet, here we are! I have to say, I feel very much like a small child, I just want to stomp my feet and yell "NO!". 

In November of last year, Amelia had a stent placed in her LPA to make it bigger. When they did this, it helped with a few problems she was having and the thought was that she would be able to wait until she was 4 years old, before needing the Fontan. That was wonderful to hear, because the bigger and stronger she was, the better. Plus, I got to pretend like she was perfectly healthy for a while longer. In June of this year, my family went fishing. David, Emma, Amelia, and I were walking a short distance to a lake when Amelia was huffing and puffing, and begging for me to pick her up. This was a huge red flag because Amelia wants to run and do everything on her own. Her breathing is always loud, but the second she stops moving, she breathes quietly again. Well, that day, it took her a good minute to recover....from walking. SO, I called her cardiologist and they got us in right away. They did an echo and her heart function looks great. They hooked her up to a pulse ox and had her run around. She ran for maybe 15 seconds when her oxygen dropped into the 50's. Her cardiologist thinks she is just outgrowing her Glenn surgery and is ready for the Fontan. The good news is that she isn't sick, she isn't going into the Fontan in bad shape, she's going in before things get worse. 

Although its not the 4 year mark like I was hoping, I know God's plans are bigger than my own.

She will be having her 4th open heart surgery(the Fontan) on August 30th. We will be admitted on the 27th, to start her on blood pressure medicine, to help her heart relax a bit before surgery. The surgeon(Dr. A) said that best case scenario would be 10 days in the hospital after the surgery, but to expect more like 1 month. 

I don't even know where to begin when I talk about my feelings on this. The anxiety is beyond anything I have ever felt before. I've started to experience panic attacks for the first time in my life. The simplest things set me off. The other day I saw a sign for an end of Summer party on August 27 and immediately couldn't breathe. This Tuesday I looked at the calendar and realized it was exactly 2 weeks until her surgery. 

I don't want to make this about me, but I do want to be honest. This is by far, the darkest and most scared I have ever felt. We've had a healthy, perfect Amelia at home for over 2 years and I don't want to give her back to the surgeons. I'm not so much scared about losing her(although, duh...that's there too), but I'm just not ready for the suffering again. Amelia had a cath in July to make sure there wasn't anything that needed to be corrected before sending her into the Fontan. Right before discharge, the NP was taking off her bandages. They are the giant, clear ones that are painful to take off. She had 2 in the groin that needed to come off. While the NP was taking them off, Amelia was screaming and looked up at me and cried "help". I SO want to help and take her place and make it better, but I can't and it breaks my heart. 

I think another thing that really bothers me about the Fontan is that we've always had another surgery in the future that would "make things better", but the Fontan is it. Thats our last stage surgery, but its certainly no fix. She isn't cured and she isn't set for life after this. Dr. A told us that this surgery punches the time clock for a transplant. She could be 7 when she needs it, or she could be 30. Who knows, and who knows what medical advances we will have by then. I know, I know, I know...I cannot worry about the future. Im trying to remember that today is what we have and tomorrow isn't promised for anyone, but she's my baby and I want her to have a long, healthy, full life. 

SO, David and I would greatly appreciate your prayers. Please pray that Amelia soars through the Fontan and the recovery. Pray for our anxiety leading up to the surgery and our hearts as we prepare for everything. Also pray for Emma. One of the hardest parts in all this is not having Emma with us all the time. Now that she's older, we can probably have her with us more than we did last time, but for the most part, she will be with family because she starts school one week after Amelia's surgery. Please pray for all the logistics, that we can have Emma taken care of and make things as smooth as possible for her. David will be taking the month of September off, to be with me and Amelia at the hospital. I am so grateful to have him for the whole month. 

If you read all this, pat yourself on the back! 

We love and appreciate all of you. Thanks for the love and support from the last few years. 

Love, 

Kelly

Future procedures

Hello! 

Long time no post, right? 

Amelia has been busy this last year! She had her g-tube removed after a super easy wean from it! I couldn't be more proud of her!

She turns 2 next month!! Holy moly!

This last year and a half has been nice, having her at home, and *almost* forgetting that she has half a heart. I say almost, because OBVIOUSLY you cannot forget, but we have been living a normal life. What a wonderful normal life it has been! I am so grateful to God for this time we have had, just being boring people, doing boring, everyday things. 

Since Amelia is turning two, we are starting to have conversations about her next(and hopefully last) surgery. Boooooooooooooooo! Waaaaaaaaaaaaaaaaaaah!

At the beginning of this year, she had a cath. During the cath, they saw that her LPA(left pulmonary artery) was really small. They tried ballooning it, but it wouldn't stay the size they ballooned it to. We were hoping that in this next year, that it would grow on it's own. Unfortunately, it hasn't. She had an MRI last month, to get an idea of where she was, heart function wise. The LPA is still very small, mostly due to the reconstructed Aorta being so large and squishing it against her heart. The MRI showed VERY little blood flow thru the LPA, resulting in very little blood flow to the left lung. As a result of the small amount of blood flow thru the LPA, she has started growing little collateral veins, that think they're doing a good job, but they're confused. haha

Anyways, all that to say, she is in need of a stent in her LPA. The cardiologist who will perform her cath, showed us a stent. It is a metal, mesh tube. She will need a few more caths over her lifetime to dilate the stent to the size the LPA needs to be. 

(From her MRI visit)

We were planning on having this done in February, but she is starting to show signs of needing her next surgery, so the cath needed to be moved up to before Thanksgiving. This time last year, her SATS were upper 80's, low 90's. The last few times she has had an appointment, her sats have been sitting in the low 80's upper 70's. There needs to be 6 months between her cath and her surgery, so we needed to move the cath up, so that we aren't backed into a corner this Summer and she isn't able to have the surgery yet. If that makes sense. It's hard to understand all the medical jargon, let alone repeat it for everyone else to understand! haha

We knew she needed another surgery, we knew this was coming, I'm just really sad. I don't want her to have another open heart surgery. I just looked at her scar the other day and thought, "man it looks so much better than it did just 6 months ago" but then remembered that it's not done yet. I am just remembering what it's like to be that kind of scared all over again. We've had a year an a half of relaxed(-ish) times with Amelia and I am so grateful for that. We are continuing to pray for things to go really well. I would love for Amelia's cath to go well, and for there to not be any emergencies between now and when she has her surgery. I would love for Amelia to not be really sick when they do the surgery. Things could be worse, the circumstances could be harder. I'm reminding myself that everyday. 

Her cath is this upcoming Monday. She will be staying one night. Would y'all pray that everything goes well and that we would have a good idea of when she will need her surgery? We love and appreciate y'all SO MUCH. 

Love, 

Kelly

For your viewing pleasure...

This kid is SUCH a joy and blessing to everyone who meets her. 

CHD awareness week. Being a heart mom.

With CHD awareness week coming to an end, I wanted to end on a personal note.

How does this all affect the mom's and dad's? Our sweet little babies are the faces of CHD, and adorable faces at that, but what about the mom and dad trying to hold it together?

When Amelia was diagnosed with HLHS, I had no clue what was in store. I mean I knew the facts, I heard the stories, but no one can prepare you for it.

Since Amelia's birth, I have nightmares. I wake up thinking that she's still on ECMO, or that she is in the middle of an episode that she won't snap out of, so they try to attempt intubating her, with no success. I wake up thinking we are still sleeping in the hospital. As you can imagine, I also have the gut wrenching dreams that she is no longer alive, where a dr tells me she died, or that I am at her funeral.

At least once a week I wake up thinking Amelia is really sick again.

She isn't, and praise God for that! I get to thank God every time, because she ISNT sick anymore.

I will always worry about her. Recently I watched a movie, where someone's daughter died while giving birth because she had a CHD. I hear those things and can't get it out of my head. I am constantly thinking about her upcoming surgery.

She will always have half a heart and will always run the risk of something going terribly wrong. I am always scared.

Amelia's lungs aren't great, her LPA is small, and don't even get me started on her SATS taking a decent dip in the last 6 months. There will always be something to worry about.

That said, I have a large community of women who live the heart mom life. They know and understand and I am so grateful for them. When Amelia was diagnosed, an organization called Sisters-By-Heart sent a care package to us and gave us links to blogs that gave us hope. If you're searching for a way to help the heart moms and dads, www.sisters-by-heart.org is an awesome organization to give to, because they were the first glimpse of hope we had.

If you're looking for ANY organizations to give to I recommend giving to your local Children's Hospital with a Pediatric Cardiology unit. Furthering research and bettering equipment in the units is important, because it raises our chances of bringing our babies home. Your local Ronald McDonald House is also a great place to give to. 

I am not looking for sympathy, I promise, just want to give a clear picture of what it's like to be a heart mom. We are so grateful for Amelia and would choose her every single time. She brings us such joy. 

Thank you, everyone, for your continued love and support. 

Love, 

Kelly

Livin' the trach life

I thought I would be interesting to show everyone what our house is like, all because of the tiny trach. 

These pictures of of her room. Just completely jam packed with supplies. 

This is her closet. We have the dresser packed with supplies that we use daily. The rest of the boxes are things we need to change out weekly. 

The dresser is where we do all her trach and g-tube cares. It's also where is dress her like a sweet little doll. ❤️

Sorry this one is blurry! More supplies that don't fit in the closet!

Syringes!!!(I seriously could have rolled around in these, I was so excited to get them!)

This is our bedroom, where Amelia sleeps. She usually sleeps in her swing, but occasionally sleeps in her crib. The iv pole holds the feeding pump(she doesn't use anymore, yay!), and the humidifier system, and water. The box like machine on the ground is the air compressor. It pumps air thru the humidifier. It's also loud as all get out and it makes our room super hot. We had to put a window unit in our room, even though we have central air. The little machine on top of the air compressor is the pulse ox, it has to be hooked up to her while she sleeps. 

Here's Amelia, all snuggled in and ready for sleep. we still swaddle her, because it's easier with the tubing and all. Once her trach comes out, we will wean the swaddle! Wish us luck! Haha

It doesn't look THAT bad, it's just a ton of clutter. 

Here is Amelia's "go bag"!

It's pretty, huh? I hate it. Hahah 

The bag is beautiful, but you should see us trying to get out the door with this darn thing and everything else that we need. 

This is everything that goes in the bag. 

Suction machine, a million suction catheders, extra g-tube button and extension, diapers(duh, right? Nope. I have definitely left the house without them), saline bullets, trach nose, extra trachs(size smaller and actual size), sterile water, manual suction catheder(used with mouth), Baggie with a bunch of trach ties and stuff used for trach cares), a ambu-bag in the awful instance that we would need to do CPR, and a pulse ox machine. 

I realized after the photo was taken that her nebulizer wasn't in the picture. We NEED that. Seriously. It's a crap ton of stuff. 

This is her bag all packed and ready to go. The black bag is her oxygen. We have to have it when we leave the house, just in case. The back of her closet is full of them. 

This last picture doesn't include her bag of meds and milk.  

This is the living room. 

Same set up, but with a pack n play and a ton of baby toys! My house a.l.w.a.y.s looks like this! We try to keep it up, really we do, but it's hard!!

Y'all this is my life! I wouldn't trade it. I love my girls and am so blessed to have them. I would live in a ditch with them...but not really😉

Thanks for all the prayers and love. Amelia is due to get a bronch on the 8th of July. We are hoping to pull the trach the next day!! Jesus take the wheel, I'm so nervous!!

Love y'all!

Kelly