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!!!!