Now that Sean is 20, I guess it is time for yet another update! It is hard to believe he is now 2 decades old. There are days that it seems like it was just yesterday that we were told about the CDH, that we were living in the NICU, the PICU, driving back and forth to the hospital or to doctors offices on a weekly basis (or more frequently!).

Since my last update Sean has had a few more surgeries including one more cardiac cath, line replacements, and one very scary hospital stay (ok, more than one!). It all started when we finally had a GI move into town that we all felt might be able to help him get off of TPN. Part of that step included doing a trial on medication to stop bacterial overgrowth, which *I* did not believe he had, as he had no symptoms other than dumping (lots of diarrhea). The GI didnt agree and wanted to try him on some antibiotics for it. Well, after trying a few, he ended up with a sever duodenal ulcer only Sean didnt tell me that he was stooling old blood (black) until it had been going on for a few days. Not so good... when I took him in to the ER and they did lab work he had already lost nearly half of his blood! SO... we had an admit with several units of blood, a scope, and the doctors decided he needed to have a reduction in his blood thinners. Against my better judgement, I agreed, though I was VERY concerned, having seen what happened when his blood thinners were reduced in the past, and the GI and cardiologist involved had not. 8-9 months later, he had a clot on the tip of his 1 1/2 year old central line, and it was not able to be removed despite every trick our surgeon tried (and if ANYONE can get rid of a clot, it is him! He is a magician when it comes to this kind of thing!

Thats when our horrific month started. Sean was admitted to remove the line, and replace it with a new one. During the line replacement, the tip of the new line hit some scar tissue on his heart and made a tiny hole in his heart. Because he had been on chronic blood thinners for so long, he bled into the pericardium, and over the course of a few days it became enlarged. IN the meantime, the IR department put their heads together with the surgeon and managed to place a trans-hepatic line (in the liver... not the greatest place for a line but it was a last ditch effort because he still needed TPN). The IR doc refused to put it in the correct way per the surgeon, but at least we had some access. (it was a very unpleasant line to deal with, painful to do dressing changes and way too close to his GT for my comfort level for infection control). We went home, but after a day and a half his breathing was really bad, so we went back for an echo. During that time the pericardium had gotten HUGE with the blood draining into it, and he had to be admitted to the PICU. He had several docs hovering over him, interventional cardiologists and our surgeon trying to figure out how to tap his pericardium to drain the blood. Neither of the cardiologists were able to hit the right place, but our hero surgeon (who has performed miracles for CDH kids over and over, especially our boy). hit the right spot first try. They ended up draining almost 600 ml of blood... keeping the drain in for a couple of days to make sure that no more blood was oozing out. Essentially Sean had suffered from a cardiac tamponade. He was in the PICU a total of a month between the two stays... and back 2 months later to replace that central line because it started to fall out, as expected. This time the surgeon was able to do it HIS way.

That line lasted until April of last year, when Sean was 19 years old. at that time Sean had finally weaned completely off of TPN, was completely tube fed and we were able to remove the line, we hope permanently.

4 months after the cardiac crisis Sean started having difficulty breathing. He was used to riding his bike around the park near our house once or twice without any problems, but for some reason his sats started dropping dramatically after only a short distance. He couldn't even walk around our house without being out of breath. We put him on O2, and took him in to the cardiologist. She did repeat echo's over the next few months, including exercise echo's (an echo, treadmill, followed by an immediate echo). Unfortunately it was determined that probably as a result of the cardiac insult and his maturing age his pulmonary hypertension had returned. For him it is considered to be exercise induced pulmonary hypertension, and we are able to manage it at this time with fairly simple (but expensive!) medication combined with his bipap/avaps machine (this machine is essentially a non-invasive ventilator as it not only gives pressure for breathing at night but also volume for the breaths). He uses it and oxygen at night as well as during the day when he isnt feeling well.

He is proud of his surgery count... 54 to date. He loves to swing, to write books, to take photos, to travel as much as possible, (going to Alaska this year, and all going well to Africa in the future!). He is an amazing young man! We are grateful to God for allowing us to have this gift of Sean in our lives, and to CHERUBS for allowing us to share this gift so that others might have hope as well. SO many times over the years the doctors would just shake their heads, not sure what his future would be... but he is still here with as much character, love of life, and hope for HIS future as any young man could possibly have. AND... as he likes to say... He got to VOTE in the presidential election too! Life is good!!!!!!!