We started our trip to St. Louis on Thursday afternoon with an upper endoscopy GI series to see if Josie's fundoplication was still intact where they wrapped her esophagus to keep her from refluxing. It looked great and they were unable to get any barium to leave her stomach and enter her throat. Also, they managed to get her to swallow a little barium and it made it all the way to her stomach so no blockage at all. This was all good news. Also, we met with the feeding therapist, but didn't come away with a whole lot of new information. She basically was going to wait to see what they learned in surgery before she could make any recommendations. Her initial thought is that Josie's inability to eat is a combination of things. Neurologically the message to swallow just isn't getting from her brain to the muscles that coordinate swallowing, there are some anatomical issues that make it more difficult, and behaviorally Josie hasn't had to eat in 4 years so that impacts it.
Friday was a very looooong day! We reported for surgery at 9 a.m. and she didn't go into the operating room until 1:30 so it was a long wait just getting her into the OR. It brought back a lot of memories for Chad and I and the multiple surgeries Josie has had. In Josie fashion, it was snowing, which seems to always be the case whenever she is in the hospital, since she was born in a snow storm in March:) The surgery started with the airway specialist doing an assessment of her airway and diagnosing Josie with a significant submucous cleft palate and an laryngeal cleft type 1. He came out and talked to us to explain what he observed and how he was going to fix it. The submucous cleft was much more prominent than he expected and he said down the road we may need to look at repairing this. This is why Josie is getting food into her nose when she eats. It isn't necessarily an easy surgery and children have to be at least four and a good size four before they would repair it. Josie is so small that she just isn't a candidate right now. He also was going to shave down her adenoids but she had no adenoid tissue at all so he left that area alone. The laryngeal cleft diagnose of a one was very good news. One is the smallest type of cleft and it is the most repairable. The surgeon said that it wasn't nearly as deep of a cleft as he was expecting and he filled it with a gel foam that lasts for about 2 - 3 months to see if we see any swallow or respiratory health improvement before he fixes it permanently. The permanent fix requires a stitch repair and then scarring to close the cleft. It is a more complicated surgery but he seemed to feel more comfortable with it after seeing her. The bad news was that her trachea, or windpipe, was very inflamed. This is the clearest sign of aspiration that we have had since her lung biopsies at Mayo Clinic when she was one. The surgeon described her trachea as looking cobblestoned. Basically, your trachea has little nodes that are minuscule running down it, when something is irritating the trachea they become swollen. As Josie swallows saliva, water, and/or food it is slowing trickling into her trachea and irritating it. This is dangerous because Josie's immune system is constantly fighting this irritation and thus far has done a good job of not developing a significant respiratory infection. The danger is that over time Josie will develop pneumonias and bronchitis because of the inflammation. The surgeon said the degree of cleft that she has doesn't always require surgical intervention but the fact that her trachea looks the way it does makes it critical that we try to fix the cleft. He said that it was "exceptional" that Josie hadn't developed serious respiratory illnesses. Chad and I think the combination of her diet and the constant use of antibiotics has really helped her in this area. The next steps are to see how she does with the gel foam injection and we will see the surgeon again in 8 weeks. If we see an improvement than he will schedule her for the permanent fix. I really think at this point we are more focused on her respiratory health rather than her eating. We are going to continue to let her eat and provide opportunities for her to practice eating, but until we get this fixed permanently we are going to be cautious. In the end, neurologically she may not be able to coordinate eating and swallowing effectively enough to get all of her nutrition by mouth.
After that, the GI doctor did a scope to see if Josie had any evidence of reflux or irritation in her stomach or intestines. She looked excellent which was really good news. Now we can start weaning her off of her reflux medicine which isn't good for her bone development and hurts our pocketbook too:) After the GI was done Josie went upstairs to radiology and did a sedated CT scan of her ears for her ear specialist in KC. We were hoping to get this done while she was under sedation at St. Louis because we were going to have to sedate her when we came back home to do it. Thankfully they were able to fit her in. Finally, she went back into the OR for an Auditory Brain Response test that precisely measures her hearing which took about an hour and a half. This was actually great news!! Her hearing hadn't progressed and she still has mild moderate loss in her good ear. We were afraid after her recent booth test that it had gotten worse.
Needless to say at this point Chad and I couldn't wait to see her because we were getting close to four hours in the OR. Finally, we got a call to go into recovery and as Josie likes to do she was holding her breath and making everyone nervous. They let me hold her while Chad tried to find something on the ipad for her to watch and calm down. It really wasn't working and even with oxygen her stats were dipping really low. Chad finally asked them to give her some pain medicine and with a little morphine she was able to calm down, still needed the oxygen, but at least was more stable. Since her airway was manipulated the surgeon wanted Josie in the pediatric intensive care unit over night for closer observation. If you have never been in a PICU before it is an experience we would rather have not had again. This isn't the first time Josie was been in there but the first where we stayed the entire night. They pushed Josie and I up to the PICU floor on a bed and as they pushed us into the room 8 doctors and nurses were all crowding in, asking questions, getting vitals, and putting a ton of wires on Josie. It is a little overwhelming to say the least. Luckily there were able to wean her off the oxygen before we went to bed and basically just got vitals every two hours and gave her a little pain medication. It is a heartbreaking place to be to see other children in such bad shape. We saw several kids being brought in from surgery with entire medical teams following them and they looked like they were really fighting for their lives. I felt so grateful to be sleeping in that bed side by side with Josie who although she had had a really rough day was going to walk out of that hospital the next. The individuals who work on that floor day in and day out just amaze me.
We thank all of you for your thoughts and prayers. We really can't tell you how much it meant. The words of support on facebook and through texts told us we weren't alone while we were there and it really meant more than you can imagine to both of us. Thank you so much!
