We are on our way back from Michigan. We went up to visit Lewis’s cardiologist in person for appointments, labs, and pre-opp for his heart cath procedure.

We got the best news about his heart. Lewis’s heart is looking the best it ever has in terms of function! They coiled off a few AP collateral vessels (arterial pulmonary) and that brought his Fontan pressures down from 14 to 12.5 which is also the lowest pressures he has had with his Fontan. He has previously been in the upper teens (20 right after his Fontan surgery). The collaterals send blood from his heart to lungs , but they aren’t helping him. Basically, his body realizes he has lower oxygen and tries to send blood (that should go out to his. body) back to the lungs to get oxygened, but then that blood is going  going back to the heart, so it kind of loops between the heart and lungs instead of actually being sent out to his body like he needs. This also increasing the pressures in his heart. So by getting rid of them, it takes away those pathways and lowered his pressures.

Lewis has a large fenestration (hole) that allows deoxygenated blood to enter back into his heart instead of going out to the lungs. It works to relieve pressure as almost a pop off value and he needs to have this with his history of PLE. It is very large right now and the hope is he grows into it as his body gets bigger and he gets older. This makes him “bluer” or causes him to have lower oxygen, but necessary to relieve stress to his heart. As he grows into it we may see him having less blood flow through it and higher oxygen. However, he is managing well, even with lower oxygen and his overall energy is great. In the cath lab they used a balloon and blocked off his fenestration (as a test) to see what it would be like if he didn’t have it. His Sats went up to 95%, but his pressures went up by 2 and if was up running around they would increase even more and be too much stress on his heart. But it’s nice to know that maybe someday he could have even higher oxygen levels. 

Essentially, if we can keep his PLE in remission his heart with this function can last him a long time! It’s the PLE that was causing us to look at heart transplant. With how good his heart is doing and how long the wait times are for transplant, we are going to keep doing everything we can to avoid that for as long as possible. While this past fall and the previous fall it looked like that was going to be happening  very soon, we know have hope that we can push that off for years!! It seems hard to believe that in just 8 months we went from thinking he was going to get a transplant to know seeing years in his future without intervening. With tweaks of his medications, “tune ups” in the cath lab, and remaining on his steroid we hope to maintain his current great health. Going to school and the many viruses that come with cold and flu season will be tough, but our hope is that by remaining on the steroid we can avoid the PLE coming back. We are on the low dose daily now and may be able to cut that back to every other day at some point to help increase his immune system as it does lower in immune system (but we want to make sure it helps keep his albumin up and be able to weather the viruses) it’s a fine line, but we will make adjustments as needed. It’s not ideal to have to be on the steroid, but at this lower dose we don’t see such significant side effects and it has worked so well for him, we don’t want to risk going off it again (last time we were only off it 2 months before his PLE returned). There is a new medication that may be a possibility for him in the future, but we will wait and see how it works for some other patients before changing what’s been going working for Lewis. We know anything can change and change quickly, but it’s amazing for us to be able to think about the future and see a future that doesn’t involve transplant relatively soon! 

We are going to try to come down on his diuretics, if he can, and try to find the best regiment for school, while also working on potty training. He most likely will never be able to fully come off of them with his current heart. That’s okay, we just have to manage them as well as the electrolyte replacements for what he loses. We have gotten pretty used to it and finding what his body likes so besides having more medications it’s not a big deal for him. We watch his bone density and growth as the lasix specially can be hard on his bones. But so far is been working good for him.

Something else we are watching for as he gets older is Scoliosis, as he is at a higher risk for it. Developing that would impact his ability for lungs to work properly. We will keep up with his PT and stretching and watch for it as he grows.  

In Omaha they have started an Fontan Clinic and that maybe something he can go to in the future as he gets older. 

We loved getting to see his cardiologist Dr. Ballweg! She is the best. We are so thankful for the great care and consideration she has for Lewis and makes us feel so welcome. Her dog Wilson, stayed up to make us cookies for the ride home ;) 

Lewis did awesome for the procedure and really the entire trip. He makes travel easy. I don’t know if it’s the fact that we only had one child with us or that he is 5 years old, but traveling and going places with him has been soo easy! He just comes along with us and is so pleasant. We visited the zoo, walked around downtown, and went to some fun restaurants, and it was so fun!

It’s just so nice to see Lewis doing so good. He has so much more energy. He walked all over the hospital to all our appointments and for surgery . Last year when we went he needed to ride. We carried him a few times, but mostly walked everywhere himself, which is a huge accomplishment for him. We took the wagon to zoo, but even though there he walked a lot of it. Getting to see him be just like normal kid warms my heart. 

Praise the Lord for how wonderful Lewis is doing and we attribute a lot of that to the many many prayers that have been said for him. Thank you all for your continued prayers for him. This could have gone a lot of ways for him the last couple years and he to be where is now is nothing short of a miracle.. 

On our way up we drove to the shrine of Our Lady of Champion in Champaign Wisconsin. It is the only approved Marian apparition site in the USA and Mary is known there as Our Lady of Good Help. We visited when I was pregnant with Lewis and this is our first time visiting in almost 6 years! It is a beautiful church and shrine. Lewis lite a candle and prayed before Our Lady in the shrine. We walked around the grounds as they have several statues, stations of the cross and did a rosary walk. In the church we went to confession, said the rosary, and had mass. It was very peaceful. Lewis liked looking in the gift shop and pick out a cross. It was a blessing to make the trip there. 

Jesus, I trust in you. Our Lady or Good help, pray for us!