Here is where we stand after all is said and done from our visit to Cincinnati Children's Hospital. All of the tests with the exception of the "subtle" finding in the manometry test came back normal. This is really good news that almost everything came back normal.
From our follow up conversations with Dr. Kaul and his nurses, we are a little bit of a waiting mode over the next few months. We are waiting to see how he responds to the Botox and dilation procedure as this should tell us a lot of information. What we think we've been dealing with is a sensory issue coupled with some complications from his small bowel not firing quite correctly. Dr. Kaul also mentioned that Jonathan seemed to have a small stomach. I think this is partially due to him not eating large meals over the years to stretch it out and his small stature.
If the core issue is sensory, that he feels full when he really isn't, made worse by a smaller stomach and periodic misfiring of the muscles in his small intestine, then we should see some pretty good progress with his appetite and food intake from the Botox and dilation. This should give us confirmation of what we now suspect is going on.
We can also approach his treatment in the meantime under this assumption and do things like give him medicines that target those areas, feed him smaller and lower fat meals, etc. The really good news is that although it is hard to diagnose and treat kids with these sensory issues, they typically improve over time and usually resolve themselves. All the lack of eating and vomiting when we fed him via his tube when he was younger (at rates and volumes he should have been able to tolerate physically) could just come down to the fact that he thought he was full or too full and needed to vomit. We aren't even sure how he feels if it is a sensory issue. I am just referring to it as full since that what he says, doesn't eat, and says nothing hurts. It could be a completely different feeling that you and I experience when things are working normally.
Over the next several months we will be monitoring how much of his daily intake will come from him or come from us feeding him. We are already seeing good indications that he is making progress eating, and we know he is getting enough calories and using them properly, so his size is probably a non-issue at this point. He is going to be as big or small as he was always created to be.
The manometry test was one of the tests Jonathan had done for the first time. I found the whole thing really interesting. The purpose of the test was to measure the strength and coordination of Jonathan's upper G.I. nerves/muscles after he ate a meal. In some kids there are issues with the nerves and muscles firing at the wrong time farther down the pathway, slowing down the process. Imagine how hard it would be to squeeze all the toothpaste out of a tube if you started squeezing from the bottom but halfway though the process squeezed at the very top. A lot of toothpaste would go the opposite direction back down to the end of the tube.
At some point in the past some really smart people figured out that you could measure these contractions using changes in pressure in millimeters of mercury, or mmHG, the same thing meteorologists use for the weather (barometric pressure). So, they removed Jonathan's g-tube and placed a catheter through the same hole to measure these changes in pressure. If you look at the drawing below, the line represents the catheter placed and the dots on the line represent the sensors that are located all along the catheter. These sensors sit in precise locations, ready to measure the pressure (and therefore contractions) when food is eaten.
After his procedures on Monday (including the placement of the catheter) Jonathan wasn't allowed to eat or drink anything the rest of the day. The next day, Tuesday, the nurse came into his room and hooked up the catheter to the machine and computer that would record the results. The system started recording for a couple of hours before the test started to make sure everything looked good and all was calm with Jonathan's system. The first picture shows the contraption that Jonathan was hooked up to that then fed data to the computer.
Once we were all ready to go, Jonathan had about 30 minutes to eat a 300 calorie meal of his choice. We ordered breakfast for him about an hour prior to the test time...a double donut, juice and Cheetos breakfast of champions. He only ate half of one donut, but with everything else it was enough. For the test they needed to cut his eating and drinking off after 30 minutes so that we could measure a discrete set of food as it moved down the line. The third picture shows a sample of how the readings showed up on the computer and were recorded. This picture was taken right after Jonathan started eating, which is readily apparent when looking at the aqua colored line. The left side of this picture are readings taken before he started to eat and the right side are the readings after her started to eat. The difference is pretty obvious. Jonathan didn't mind this test too much because all he had to do was stay in bed without moving, finally get to eat yummy food, and watch Toy Story 1, 2 and 3 all in one sitting.
Although the doctor reviewed the results the following week, I know them as I type this blog. Overall things looked good. This test did not reveal any major findings. However, there was a small "misfiring" of one of the areas in the upper small intestine. The problem wasn't major, but it could be periodically slowing down the speed at which food moves through his system. Considering his motility test came back normal, this isn't a huge concern for me. It's something to be aware of and keep in the back of our mind for the future. It certainly, given the results of everything else, is just one factor of a couple of factors (according to the doctor/nurse) of what we have been experiencing with Jonathan for the last 5 years. We think what is really going on is a combination of a few things, which I will get into in an upcoming post.
For now, we are glad to check this one off the list since it is one of the last things left to do. Although there is probably an issue here worth monitoring, this could have been a lot worse.
We were so glad that the major procedures were a go and that our trip out to Cincinnati wasn't going to be rescheduled. Because we had prior experience with Jonathan going under anesthesia, we opted to give him an intravenous boost of something prior to wheeling him back to the operating room. It was pretty funny to watch him after getting the medicine. All of a sudden he became very relaxed and then looked up at Stacey, eyes half open, with a loving and cheesy smile. The effects seemed similar to Jonathan taking a couple extra doses of "Grandpa's old cough medicine", if you know what I mean.
The rest of the family waited for about an hour and half for everything to be completed. Afterwards we met with Dr. Kaul and his nurses to discuss how it went. Overall it went really well. Dr. Kaul did not see anything obviously wrong (visually, at least). He did mention a few things though. He mentioned that the g-tube was probably placed too close to the pylorus (opening at the bottom of the stomach). He also asked if Jonathan had been tested for Celiac, as the very beginning of his small intestines were pretty smooth/flat, which can be a sign of this disease. We have had Jonathan tested for this, but it is something to keep in mind. This is also the second doctor to mention that area of Jonathan's intestines - last time there were some questions about friability in this area. We will have to wait for the biopsies to be examined before anything can confirmed, but chances are nothing will come from this as things looked pretty good.
Below is a list of things Jonathan had done, any notes on this item, and the corresponding pictures below.
Upper Endoscopy w/Biopsies - images 1-3 are Jonathan's esophagus, 4-5 are the top and side of his stomach (including parts of his g-tube visible), and 6-7 are of the pylorus (the bottom opening of his stomach). There was nothing to note from these pictures. 8-9 are the beginning's of his small intestine, with the foreground in image 8 being the smooth area I mentioned above that is consistent with Celiac patients.
Pyloric Botox and Balloon Dilation - images 10-12 show Dr. Kaul injecting Botox into Jonathan's pylorus and then dilating it with a balloon. The effects of Botox and dilation are supposed to last about six months. Botox relaxes the muscles that control the opening at the bottom of the stomach (as is moves into the small intestine) and the balloon, enlarges this opening. The idea is that if there is a problem with this area or a sensory problem with his stomach feeling prematurely full, we will see improvement in his appetite and calorie consumption in the next several months. It's really as simple as relaxing and enlarging the drain hole from his stomach to his small intestine.
Colonoscopy w/Biopsies - images 15-20. Biopsies were taken, but nothing of note for right now.
Antroduodenal Manometry Catheter Placement - not pictured. After these procedures Dr. Kaul removed Jonathan's g-tube and replaced it will a catheter for the upcoming manometry (pressure test). They kept all the sensors that would be hooked up to the machine in a plastic bag that was taped to Jonathan's stomach.
Yesterday afternoon Jonathan started to cough...a lot. Last night none of us slept very well because he was coughing all night (and it didn't help that some loud punk kids had rented a couple of rooms across the hall). I actually left the hotel room at 3:30 in the morning, half asleep, and went to the grocery store to get him some cough medicine. I also needed some ibuprofen, since the night before I had slept wrong and it was really hurting my back to move or sit up.
When we all eventually woke up Jonathan sounded sick and was coughing on a lot of phlegm. A couple of days ago at our anesthesiology consult we were told that if he starts coughing or gets sick that we should call them as soon as possible. So, we tried but there really wasn't anyone around since it was Saturday. We eventually got in touch with an anesthesiologist who was on call. Long story short, we weren't totally sure what the next steps were after talking to her on the phone. It was possible that she was going to talk to our main doctor and cancel the procedures, but she also said we wouldn't have any information on rescheduling options until Monday. The problem for us is that we are supposed to be admitted tomorrow morning to start the process.
It's possible that this whole trip is over because Jonathan is getting sick. We understand that it's not a good idea to put someone under when they are coughing and hacking up a lung. We also have a test scheduled on Tuesday morning (after a catheter is placed on Monday) where he will need to sit very still for almost 6 hours. If he's coughing a lot or throwing up, this test won't be able to be completed.
So, we went up to the hospital late in the morning to get more information. No one was really around but we were able to check with admissions (and get their number) to make sure we still had everything scheduled in the computer for tomorrow. We will check back tomorrow morning before we head over there if Jonathan is doing better. Tonight he vomited in his sleep, so things aren't looking very good for the rest of this trip. We can always reschedule and come back out here if we have to, but obviously that would be less than ideal.
Here's hoping he wakes up tomorrow morning, his labored breathing is gone, he feels good, and our tests haven't been canceled. If things don't work out we might want to come back earlier, but I am not so sure there will be a ton of flight options over Thanksgiving week. If we keep our currently scheduled flights back, there is a chance the snow forecasted for Thanksgiving will complicate things as well.
This afternoon we went back to the hospital for Jonathan's next test. Earlier in the morning we had met with the feeding therapy team (as expected, nothing really came from that). Jonathan wasn't allowed to eat for 4 hours prior to the test, so I felt really bad him when we stopped for lunch. He was really hungry and wanted to eat some lunch (and kept saying he wished he could take a bite of Julia's yummy lunch). So, we had to buy him his own lunch when we left so he could have some food with him when he was allowed to eat again. The irony of this was painful.
The test was pretty straightforward. Swallow some barium over 40 minutes, lie down on a table, and let them take a bunch of x-rays of Jonathan's abdomen. The barium would illuminate the lining of his intestinal wall, allowing the multiple x-rays to paint a picture of the section of plumbing between what an upper endoscopy and colonoscopy will see.
Unfortunately, Jonathan didn't really like the whole experience once the nurses put in his IV. There were a lot of tears and irritation after that, so we were glad we still have his g-tube. He was tired, "hangry", and his arm was really hurting so he refused to drink the solution. Every five minutes I put an ounce of barium through his g-tube until we had evenly spaced out the dose over a 40 minute period of time. We moved across the hall to where the CT machine was located and I finished priming him with a nice flow of barium. By this time he had calmed down, so the few minutes we spent listening and hearing the CT machine go round and round in circles went by pretty fast. We will find out the results of this test soon. We never done this test so it will be good to know either way. I don't expect anything structurally to be wrong, but we are covering all of our basis once and for all.
Thought I would post some pictures of one of the main towers on campus. This is the building we see when we drive up each morning, with several more buildings to the left of this shot.
Got Jonathan's official gastric emptying results back today. They are:
Liquid - 56% at 1 hour, 84% at 2 hours
Solid - 28% at 1 hour, 79% at 2 hours
These are well within the normal range. I looked up ranges on the internet, and according to one reputable site, the abnormal range would be anything less than 10% at 1 hour and anything less than 40% at 2 hours.
