Little Jacob came along Oct 13, 2006. He was a big, healthy baby and we were thinking we were going to be going home in a day when we got the news he was born with several CHDs (congenital heart defects) and would have to be transferred to another hospital to have heart surgery. We had no idea there was anything even wrong with him.
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Jacob had his first heart surgery at 11 days old to fix a Coarctation of the Aorta, however after a routine surgery follow up, he was diagnosed with hypoplastic aortic arch and he had to go back in for open heart surgery at 2 months old to have an aortic arch reconstruction. He also has a bicuspid aortic valve that was diagnosed originally and will have to be followed throughout his entire life to watch for leaking and stenosis -thickening & narrowing and an abnormal mitral valve.His condition is called Shones Complex Variant. But, after another follow-up appt, his cardiologist discovered he had developed subaortic stenosis, due to a subaortic membrane (left ventricular outflow tract obstruction) which will require another open heart surgery to remove it when it grows to a certain point.r it starts to leak it will have to be repaired right away. Otherwise we wait to have the pressure gradient across his valve measured every six months to see if there has been an increase. No way to know when that will be, so it's my black cloud that follows me. I will never have an appt where his cardiologist says he's fixed...our news is either "stable" or "worse", which is not the most normal way to live, but it has become our "new normal". When the membrane is removed, there is no way to predict if or how fast it will grow back again (it's like a weed) and the only way to remove it is with open heart surgery.
Jacob also has Ehler’s Danlos Syndrome, which is a connective tissue disorder. It has caused his cartilage in his knee joints to be abnormal. He had his first surgery to repair his meniscus at 2 yrs old. At his next surgery, they found he also had Avascular Necrosis. His bone at the end of his femur was dead, causing the cartilage to be completely gone. His surgeon had never seen this in someone even close to his age before. He had a very invasive operation on April 7, 2014 to do a cartilage transplant. They took cartilage from a non-weight bearing part of his knee and transplanted it into the large space where he had none. His cartilage cells were sent to a lab in Boston. The original plan was that if the transplant fails, he will have his cells grown into synthetic cartilage and it will then be transplanted. Now it has been decided they would only use donor cartilage, since it wouldn’t be productive to put his cartilage cells back into his body. He then had a surgery in 2015 to remove scar tissue. In 2018 he had a “guided growth” surgery to straighten his legs to distribute the pressure on his joints. Hardware was put into the insides of his knees. It worked pretty quickly, so 10 months later the hardware was taken out. Jacob does not have a meniscus at all in his right knee, so his surgeon is going to make a 3-D model of his knee and figure out what the next step is.
Jacob is such a blessing to us and is so very brave!