Aaron's spine-background

 

This whole story began in August, 2010, when right before our annual anniversary trip, we took Aaron for a school physical because he was getting ready to start Minnieland. We were so lucky to get an appointment with Dr. Chabra, our favorite pediatrician at the health center. While she was checking him out, she had him take off his shirt and bend over and touch his toes. Something seemed to concern her right away and she kept trying to adjust his shoulders as he was bent over. While she was looking at him, we saw it, and had never noticed it before, a curve in his spine. She thought he needed to have a scoliosis screening and we just couldn't believe it. When Aaron is standing up straight, it is not noticeable at all, and we found out why later. She arranged for us to have immediate x-rays at Ft. Belvoir and the rest of the day was a panicked blur. We got his x-rays done and we saw what his spine looked like. That was when true fear set in. It was so curved, right there in the center of his spine. To see an x-ray of your child that looks so very wrong is horrifying. Your stomach just drops out of your body. We had no idea what would happen next, we just had to wait until Dr. Chabra looked at the x-rays and got back to us, which she said could take 4 or 5 days.

Turns out she called us that very same evening. She said that Aaron did indeed have scoliosis, and that it was caused by two hemi-vertebrae, at positions T-8 and T-11 (http://en.wikipedia.org/wiki/File:Gray_111_-_Vertebral_column-coloured.png) that did not form correctly during my pregnancy. She also told us that since this was a birth defect, it was hard to know how it would be corrected so we needed to start visiting an orthopedic surgeon at Walter Reed. The biggest shock of all was the information Dr. Chabra gave us about the likelihood of associated defects. This condition is uncommon, but when it is present it develops during week 8 of pregnancy during which the spine forms. The heart and kidneys also form during this week so about 40 percent of children with Aaron's condition also have associated defects in one or both of those organs. The possibility that something could be wrong with his heart scared us even more than the scoliosis.

The next week we were scheduled to go on our anniversary trip with Aaron to Myrtle Beach and after the shock of the week it was exactly what we needed. We had a great time, we were reminded that our little boy was doing fine and we got in some good relaxation.

Over the next several months, Aaron had several appointments, all of which were at Walter Reed, an hour and fifteen minutes away from home, and all of which we had to miss school for. The stress of it all was very challenging and we are so grateful for an incredibly supportive workplace and group of colleagues.

Our first appointment at WR established our relationship with Aaron's orthopedic doctor, the director of spinal surgery for the army, Dr. Ronald Lehman. He is the best of the best and we are so grateful to have such an accomplished and competent doctor for our son. Our medical care is truly the best you can get in this country. He took another set of x-rays and explained Aaron's condition in detail. Because those two vertebrae are malformed, the scoliosis caused by them cannot be corrected with bracing. As long as those two vertebrae are in there, they will continue to force the rest of his normal vertebrae into a curve. Dr. Lehman explained that Aaron has "global balance" meaning his head is perfectly lined up over his pelvis. Because one hemi-vertebrae curves one direction and the other one curves in the other direction, they kind of cancel each other out. This is why Aaron appears totally normal, had no difficulties when learning to walk or crawl, and has even shoulders. Unfortunately, we learned that Aaron's condition cannot be corrected without surgery. That would involve removing the two hemi-vertebrae and inserting permanent rods into the vertebrae above and below the removal sites to fuse his spine together and correct the curvature that has been caused. This is a complicated and dangerous surgery, and it really knocked the wind out of us to learn this. There is a great description of Aaron's condition here: (http://www.srs.org/patients/congenital/) You can see illustrations of the hemi-vertebrae, which are wedge-shaped, and see how those cause scoliosis in the rest of the spine, just like a curved piece of train track inserted into your son's Thomas the Tank Engine track layout.

Because of Aaron's age, he would recover from the surgery very well if there were no complications, and his spine would fuse in a few short months, unlike an adult, whose spine would take years to fuse, if it did at all. Everyone we talked to was amazed that Aaron's condition was discovered so early and very positive about the possibilities for correction. Dr. Lehman advised that we proceed with the testing for related defects and come back in a few months to check the progression of the curve and make a determination about when surgery might take place. If the curve was progressing slowly or not at all, then we could just remain in a holding pattern, but if it progressed quickly, then we would need to talk about surgery sooner than later. As you may know, Aaron's surgery is taking place on Monday, July 11th, so we know how that part of the story went, but more on that later. Whether or not the curve was progressing would be measured by the degree of curvature. It seemed the bottom vertebrae, T-11, was the most curved, and that was really the one to watch.

So Aaron underwent a number of tests to check for associated defects. He was a great sport through all of it. We explained to him what a normal spine looked like and what his looked like and that we were trying to get information from doctors about what to do next. From the beginning, we have explained everything to him that was going on and he understands it the very best that a little mind can. We were very relieved to learn that his kidneys and heart were perfectly normal. It seemed that given the severity of his condition, we were getting some great news. Another concern Dr. Lehman wanted to rule out was the possibility that Aaron's signal cord could be tethered to either of the two hemi-vertebrae. That would require a first and even more dangerous surgery to separate the cord before the scoliosis correction could take place. This possibility became our new biggest fear, and we had to schedule an MRI to get the images Dr. Lehman needed to rule this condition out.

Aaron’s first MRI was in October, and since children have to be sedated for MRIs, it is an all day procedure. Aaron had never been through anything like this before so we were certainly anxious, but even more so, we were afraid of finding out his spinal cord might be tethered. The pediatric sedation team at Walter Reed, Raquel, Naomi, Tyrone, Dorina and Lisa, were amazing from the start, and we just love them. They are true pediatric geniuses and were positively excellent with Aaron. The sedation itself went fine but I got a little queasy watching his behavior change as he was given the sedatives and resisted falling asleep. I pulled it together and he was asleep pretty quickly. The MRI, because it was of his whole spine, took about 50 minutes. We went into the MRI room with him. It was freezing and so loud, but despite all that Chris and I managed to doze off, because we all got up at 4:45 in the morning for this appointment. After we got back up to the peds sedation ward, Aaron was allowed to sleep for another hour for the sedation to wear off. Chris got us some lunch from the Subway in the lobby, and we watched miners being rescued in Chile on the TV over Aaron’s bed. I remember thinking while watching those men be pulled out, “I need some good news. I need to know that things can still turn out well.” I was so heartened to see those men come out of the mine while we waited for our tiny little guy to wake up.

He was slow to wake up, and when he did, oh, it was like nothing else we have ever seen. He did NOT want to wake up, was completely hysterical, which they said was normal. He was kicking and flailing and even hit his head on his bed rails. Finally Chris picked him up and just held on tight while Aaron kicked and shouted and cried, eyes closed the whole time. Since he guzzled a little juice box and was saying sentences that were coherent during his flailing, they let us bring him home at 2:00. He went RIGHT back to sleep in his car seat, and he is still sleeping now. It was pretty scary watching him be so upset and disoriented from the sedatives, but we just let him sleep and gave him tons of water every time he briefly woke up. 

It took three longs weeks and a lot of frustration to get the MRI results, but they were clear, meaning his spinal cord is not tethered to his vertebrae. This was a huge relief for us because we knew at that point there was only one problem to correct. A difficult and serious one, but we continue to be grateful for all the good news we got during this time.

It was February, 2011 before we had to go back again for a new set of x-rays to check the progression of his curve. We got the worst news we could have at that visit; that his curve had progressed so quickly that Dr. Lehman said surgery was necessary this summer. It was time for us to start preparing ourselves for the reality that he was going to have this dangerous surgery. Back when Dr. Lehman had first explained that surgery was the only way this condition could be corrected, and after he explained the potential dangers of the surgery, we considered not doing it. All surgeries have risks, but this one includes a 10% chance that Aaron could become paralyzed. Chris asked Dr. Lehman what could happen if we elected NOT to do the surgery, and Dr. Lehman told us frankly that Aaron could go his whole life without having any complications, but if he did have them later in life, we wouldn’t know it until it was already too late to intervene. Like, one morning, a few years from now, he could wake up and not be able to use his legs. By then, there would be nothing we could do. Also, once Dr. Lehman saw the progression of the curve in February, he said the chances that it would not impact Aaron later in life were very small. How does a parent make a decision like this? Elect to give our son a surgery that could possibly have devastating results? Or choose not to give it to him, which could also possibly have devastating results? It was the ultimate gamble versus the ultimate gamble and the guilt of the decision weighs heavily on us. Ultimately, we knew that if there was something we could do to help him get better; we had to do it, instead of just letting his condition worsen.

So the wheels of surgery were slowly set in motion. Unforutnately, that included a second MRI just to make sure his cord was still perfectly healthy, as a precaution. Now this time, we felt confident that his cord would still be fine, but we were dreading the horrible effects of the sedation. The peds sedation team reviewed Aaron’s first sedation experience with us and decided they would give Aaron extra fluids before and after the sedation and let him have an additional hour to sleep it off. That was great with us, and ended up being very successful. Aaron needed even MORE medication the second time, unfortunately, because he had a cough and was moving too much, but his wakeup was so much better this time. Slow, but without the hysterics. He slept all the way home and through the night.

Aaron also had a CT-scan that Dr. Lehman is using to make a 3-d model of Aaron’s spine before the surgery to use for his own planning and preparation. The reason Aaron’s surgery has been scheduled for July 11 is that for one week a year, Dr. Lehman’s colleague, who is stationed in Hawaii, comes here to DC for complicated cases, which Aaron’s certainly is. Actually, during one appointment, to our dismay, Dr. Lehman rated Aaron’s surgery as a difficulty of 8 on a scale from 1 to 10. Yet another thing we didn’t want to hear. In any case, both surgeons will be operating on Aaron at the same time, one on each side of him, in order to cut the procedure time in half. It will take between 6-8 hours and we will be in the hospital for 4-6 days afterwards. I will save surgery day information for another post, but now the background is all here so you can follow along with the story.