We're back from our trip to San Francisco for Silas's appointments at UCSF and have some good news to report.
We left early on Monday morning and returned late Monday night. Silas's first appointment was an upper GI study, which was ordered back in August when Silas was still vomiting several times a day. Since he went on Miralax last month, his metabolism has become much more regular and he's been vomiting much less, but we were curious to see whether this study would identify anything anatomical that might make it difficult for him to keep food down and gain weight. Silas only had to fast for three hours before this one, so it was a much happier drive up.
We met briefly with the radiologist and helped get Silas set up for the exam. He had to drink modified barium from one of his bottles while they filmed his upper GI track using an x-ray. He handled the process pretty well even though he had to drink his bottle from positions he'd never tried before in order to get the best pictures. The radiologist had not reviewed all of the film in detail when we had to leave for our next appointment, but he said that from his initial review he did not see anything unusual anatomically and thought that Silas was taking in his food just fine.
Then we headed a few miles away to Silas's ENT surgeon's office near Mt. Zion Hospital. His doctor did another bronchoscopy to monitor the progress of his vocal cord recovery and check for any sign of infection or granulation tissue that might be affecting Silas's eating. She first put the bronchoscope down his trach to check for any signs of trouble. The scope produces a video image that appears on a monitor in the room with us, so we got to see everything she did. She thought that everything in his throat looked good--no evidence of granulation (extra tissue that can build up and cause problems and can need to be surgically removed) or infection.
Next she put the scope up Silas's nose and down his esophagus to see his vocal cords. (He absolutely hates anyone touching the inside of his nose, so this procedure is his worst nightmare.) He cried and cried which allowed her to see his vocal cords opening and closing again, especially when she plugged his trach. It was wonderful to see. The vocal cords are definitely moving now and appear to be opening slightly wider than last time, but are still opening about half as wide as she wants to see before we can talk about decannulation (taking the trach out). It is too early to tell exactly when Silas will get his trach out, but we were encouraged that she wants to see Silas again in March, the anniversary of when she put his trach in, to evaluate him again.
There is apparently a body of research that supports leaving a trach in an infant for a period of one year following Chiari decompression surgery, so Silas may be one of the babies who fits this description. His condition is so rare that it is hard to know what to expect. We are hopeful that his vocal cords will continue to 'wake up' as his brain stem heals. The good news is that they are no longer paralyzed--they're moving again. We just need to see more movement before we can be confident that Silas will no longer need the assistance of the trach to breathe normally. Once the trach is out, we'll have no way to suction him or help him breathe around his vocal cords if needed, so we have to be absolutely sure he's made a full recovery. We were also advised by Silas's chief PICU doctor not to decannulate in the winter during cold and flu season, so waiting until the spring may very well be for the best, although a big part of us is disappointed that the hard work of maintaining his trach will continue for at least a few months more.
Then something fun happened. Our pediatric pulmonary had doctor recommended that Silas get a slightly smaller trach so he could begin vocalizing, so Silas's ENT surgeon decided to switch his trach before the end of our visit. The trach he's had since the spring has been a 4.0, meaning the tube in his trachea is four millimeters wide on the inside, pretty snug. She suggested stepping down to a 3.5 to allow more air to pass around the trach and allow him to make more noise and get used to the sound of his voice. One of the challenging things about caring for Silas over the last six months has been that he is essentially silent. Since the trach bypasses his vocal cords he has no way of crying out loud or making himself heard. (Only in rare instances when he's really plugged up and in need of suctioning can he make any sound, and then he surprises himself and gets a little scared by his voice.)
When she put in the new trach Silas began to discover his voice. He can pass some air around this slightly skinnier trach tube now and can get out little "ah" sounds. He squawked all the way home and has been entertaining himself ever since by making little noises around the house. It's pretty fun. He's delighted that he can wake us up in the morning much more efficiently now, and he's getting better at holding a 'conversation' with us. He's still shy about using his new voice around other people, but he's bound to bust it out around others sometime soon. It's fun to see him add this new skill to his growing personality. For better or worse, he's getting more attitude each day and reminding us more and more that he's nearly a one-year-old. (Gulp.)
We'll post more pictures from this month and this summer soon, but we just wanted to pass along the news from these appointments. Thanks everyone for your continued prayers and support. Silas is doing well.
