Lewis had his cardiology telehealth visit today. He had  echo, and ekg  yesterday and labs last week all before this appointment. 

The good news: Lewis echo looked great (unchanged), his EKG also looked the same, and his stool sample came back in the normal range for protein! Since he doesn’t have a large amount of protein in his stool, it means he isn’t losing it like he was, and we are going to go down on his budesonide steroid. He has been absorbing the steroid well, as you can see from his very round cheeks and rapid hair growth, but it has also brought back up his albumin and protein. This PLE flair up (when it comes back after it being in remission) seems to be in control now and our goal is to go down on his steroid to a low dose and just stay on that continuously. Being on the steroid indefinitely will hopefully help him if gets future viruses that trigger his PLE in the future or keep it from getting triggered at all. We weight adjusted some other meds and are adjusting his diuretics as needed. 

The not so good news: Lewis is considered a high risk transplant patient due to his Fontan failing in the first year (the year he had his Fontan surgery, he developed the PLE and has had to be on a lot of diuretics which he has not been able to come off). Because of this it will be important what center he goes to for transplant, and most likely the only one he could go relatively close to us would be Denver. Our cardiologist is going to talk with their head transplant doctor and ask her where Lewis would fall on the risk category and if he needs some sensitivity testing in order to make that determination. If they need that, we could get that done without going through a transplant evaluation. Transplant is not an emergency right now, but looking ahead for whenever that time may be is needed.

This was a little tough news to hear, as we were hoping we would be able to have Lewis’s transplant here in Omaha once their program was up and running. Our hope was he would be able to do well on the steroid at least until Omaha’s program was ready. But with his high risk status that may not be the best outcome for him, even though it would be tougher on our family logistically. We have time and there will be future discussions when the time comes.  Omaha is having a heart failure/transplant doctor come back in January so we may be able to transition care to him once he gets on his feet (6months-1 year). We will keep following with Dr. Ballweg in Michigan now and go out to Michigan again for an in person visit this spring/summer. Even if we do feel comfortable transitioning to Omaha, Dr. Ballweg said she can consult with his care and she knows Dr. Cole. At some point we will get to meet with him, but not sure when that will be yet. It would be really nice to have our main cardiology care local again, and even if we had a transplant in Colorado , once they felt comfortable we could do follow-up care back in Omaha. Through all of this we will continue to pray and try to make the decisions that are best for Lewis and our family!

Lewis: As of now Lewis is doing great energy wise and is loving just living his life. We are praying we can make it through this cold and flu season with him in school as much as possible and no more PLE flair ups, but we don’t know. He is at a tough age for kids getting sick and some viruses trigger his PLE more than others.  We will try to space out his labs and infusions more as we can.

For now we are enjoying our 5 year old boy and trusting in God to handle the rest! He has never failed us, and I have realized we could never make it through this life without him. 

Hope you all had a Merry Christmas and Happy New year.