Dr. Robert Campbell of Children’s Hospital of Philadelphia
Owen’s Story was Submitted by: Sara Sedey
Owen was born in March of 2010, with a rare disease called Escobar Syndrome (Multiple Pterygium Syndrome) which is the genetic form of Arthrogryposis. When we were first told of his diagnosis, we were informed that he has the lethal variant of Escobar Syndrome. However, our doctors assured us that clinically, he was doing fine and that the only thing that could get in Owen’s way in the long run, were his lungs. Some of the symptoms of Escobar Syndrome are contracted joints, webbing, scoliosis, and chest wall deformities. Owen has all of the above. His scoliosis was very severe and his chest wall was quite deformed. When Owen was less than a year, we noticed he wasn’t gaining enough weight despite having a healthy appetite. Owen is a twin and his brother Caleb was gaining rapidly. Our orthopedist at NYU put us in touch with Dr. Robert Campbell to discuss possible thoracic insufficiency. We met with Dr. Campbell at Children’s Hospital of Philadelphia. He ordered pulmonary function tests and a Dynamic MRI to assess Owen’s lung/chest condition. While Owen’s lungs were functioning normally despite the scoliosis and chest wall deformities, his lungs had no room to grow larger and eventually they would not be able to function at a normal capacity. At that time, it was decided that Owen would have VEPTRs placed, however, we needed to get his weight up so that he could support the rods. Owen had a g-tube inserted and in June of 2011, when he was at a healthy weight, Owen had his left VEPTR placed. In October of 2011, he had his right VEPTR placed and his left VEPTR expanded. Dr. Campbell was able to get a significant amount of space in Owen’s chest wall for lung growth and we are all hopeful that Owen’s lungs will grow now and use that space. Owen is scheduled for another expansion on February 15, 2012.
Owen’s VEPTR rods potentially mean the difference between life and death for Owen. And they certainly mean that he will lead a life that is far more active, far more similar to his brothers (Caleb and big brother Gavin) and far less debilitating than it would be if he couldn’t breathe. We have hopes that Owen will be able to walk and be self-sufficient someday. The VEPTR is life changing. Dr. Campbell has given Owen’s lungs the chance to function like yours or mine. It’s nothing short of amazing.
Owen is approaching two. He is happy. He loves to dance and play with his brothers. He loves Elmo. He loves music. He makes us happy every single day and is an inspiration to our family. He has come so far in so little time, I can only imagine what is ahead of him.
Owen’s Family Photo’s:
Owen and his brothers