Forney, Sean Patrick

[HeidiForney]

Wow, just found this... it has been 12 years now since I updated, and a LOT has happened! Sean is 15 1/2 years old, and still just as wonderful as he has always been. Briefly, his scoliosis issues ended up with a fusion at age 5, from T2- T12. They removed one of his ribs so they could do an anterior and posterior fusion, unfortunately it did not completely "take" and his curve started progressing in a kyphotic direction so at age 7 1/2 he had to have the hardware removed and was placed in halo traction with 25 pounds (half of his weight) hanging off his head and spine for 2 months in order to straiten it as much as possible. This was done in Seattle, and his spine was re-fused from T1-L2 a month before his 8th birthday. This time it worked, and instead of the 140 degrees that it could have been without the previous surgeries and rod expansions, it was only 70, and has remained at 70. He has a very large rib hump, and was on long term narcotic pain medication for 7-8 years through all of this, but he walks, runs, and is no longer wheelchair bound though he does tire easily. He also developed osteoporosis, which we are slowly working on reducing now that he can be more weight bearing.

Sean is still TPN dependent for about 50% of his caloric needs. Thankfully we can do this just a few days a week, so he gets a few days off with "just" tube feeds during the day every day. He is still on Compleat Pediatric, which has been a wonderful formula for him, though we no longer give glutamine or pectin. He just can't seem to absorb enough calories through his gut to grow without the TPN, if we go above a certain # of calories, he ends up with severe diarrhea, also called dumping, and loses weight instead. Not so good! He is a real light weight, at 75 pounds give or take, and just reached 4'9". He would be closer to 5'4" if were not for his spinal fusions, as those had to kill his growth plates in the vertebrae in order to stop the curve.

At age 5 we discovered that Sean had severe pulmonary issues. His lungs just were not doing well at keeping him oxygenated, as after one of his many surgeries he had to go back on oxygen and couldnt get off of it. We took him to Seattle to see pulmonology, and discovered that he had restrictive lung disease, partly from a partially paralyzed diaphragm (thank you patches!) and from the scoliosis and hypoplastic lung. He also has blebs all over his lungs, kind of like someone with emphysema. As a result he has been Bipap dependent at night as he can't remove CO2 easily himself, and has both kinds of sleep apnea. We found out that had we not pursued pulmonary when we did he would have been trached and vented within months. He came close to that again recently, though we changed machines and that seems to have helped, for now. Lungs dont grow if the spine can't grow you know... He also on O2 at night and has been since he was 5.

As if that weren't enough (I love my complicated sweet boy!) he also developed something called SVC syndrome. That is where he has a tendency to clot his blood vessels off. That is partly due to the central lines constantly going through them, but also, just part of being Sean. He has had 15 cardiac caths (essentially an angioplasty) to place stents to hold open the vessels and/or to balloon open the vessels. We came close a few years ago to where we thought he might need a graft (transplant) to replace a part of his SVC, but thankfully a cardiologist in Boston was able to place a few more stents instead and he has been fine ever since, along with blood thinners for the rest of his life of course. Sean is a traveling machine... loves to travel, thankfully, since we have had several different hospitals need to chime in on his care! Seattle, Boston, Denver, San Antonio, TX... Plus of course when we are traveling as a homeschooling family and "stuff" happens!

Sean was recently diagnosed with Aspergers as well as secondary mitochondrial disease. Nothing horrible, we just make sure he gets certain supplements and it seems to help with his energy levels. He is a happy young man, loves American History and animals, especially his dog and tarantula, loves to learn about hunting and wolf re-introduction, and has volunteered at our local aquarium helping them to write the signs for their new fish! If we had the $$ to travel half the year, we would as he can never get enough travel experience, seeing new places, finding out about the history of our country, and checking out every zoo and aquarium he can find.

I hope this helps to give families hope for the future.. though he has been through a LOT (47 surgeries), he is a fighter, and loves life. He doesn't know the meaning of the word NO.... God has blessed our family with him, and with his brothers (and the newest members, his new sister in law, niece and nephew!). What a LIFE!!!!

[HeidiForney]

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!!!!!!!