Avleigh's Journey

Well today was a pretty long day. We got up early this morning to head out to Children’s for Avleigh’s Motility Test. This test is designed to monitor the contractions of the stomach through the intestines. Of course, we were scheduled to do this test because we had suspicions that it wasn’t functioning properly due to her symptoms. They started the test by putting her to sleep so they could guide the lines that would monitor her motility through her gastrostomy port on her belly. Once she was out of recover we went to a room where they began the test. The monitor had several lines where we could see the contractions of the stomach and several areas of the intestines. The idea here is to see a rhythm or organized chart starting with the stomach and then going down through the intestines.

We started with her on an empty to see her baseline contractions. It was pretty boring, a few small clusters here and there but nothing with any coordination. They then pushed through water on a slow drip. Still had the same result; random clusters. The next step was to add in a medicine that is designed to trigger the contractions. After adding the medicine there were a few normal contractions (3 and he would have liked to see 30-40) but the intestines didn’t follow suite. At this point the doctor wanted to try a bolus feed. We knew that she’s unable to tolerate the volume of a bolus feed, but he wanted to see exactly where the issue was within the motility system.

We gave her 120 ml over a 12 minute period. She seemed to start getting uncomfortable and 8 minutes later she projectile vomited the formula. This was good for testing because he could see where the initial gag was from her then it was followed by a huge contraction, what he called “true vomit”. The good that came of it was her stomach began having more of a normal rhythm of contractions after the vomiting. The bad news is the intestines never followed suite.

So what does all this mean? Her stomach doesn’t have normal contractions which would explain why her food was no longer breaking down. The contractions of the stomach is what actually “grinds” your food. Secondly, it means her stomach does not communicate with her intestines. Lastly, her intestines is just a big cluster! Without the intestines working in coordination the formula is slushed around instead of the upper section pushing it down all the way through the entire system.

The next question, how do we fix it? Sadly we can’t. He explained that this dysfunction wouldn’t have anything to do with the actual neurological issues that PCH causes because the motility system’s nerves work separately from the brain. He also said that she has a moderate to severe case of Neurpathy, meaning the issues with her motility are within the nerves rather than the muscles. She definitely needs to be changed from her G-Tube that feeds to her stomach to the GJ-Tube that will feed straight to the intestines. But we also have to keep her on a very slow feeding rate.

Here’s an easier way of thinking of it… he referenced her as the same as a 32 week premature baby that isn’t ready to eat yet. The only difference is a premature baby has the ability to develop and Avleigh is going backwards. So it’s very important that we keep her nutrition as good as we can and avoid any infections. Malnutriciton and infection could cause these issues to progress even faster.