We got home from our trip to LA 10 days ago, and I'm just now finding the time and the mental & emotional energy to write about the results that came from the testing. I am happy to say that thanks to all of the prayers on our behalf and a lot of preparation ahead of time, the actual trip itself went very well. It was not the same kind of exhausting that our last motility testing trip was and I am truly grateful for everyone who was praying for us. I have no doubt that we were watched over and that we were strengthened through the prayers of others.
The testing itself probably could not have gone any better than it did. She had an endoscopy first thing in the morning that day and the inside of her stomach and duodenum looked perfect. There were no signs of gastritis, erosion, ulceration, or the kind of damage that comes with eosinophilic disorders. There was no narrowing of the pylorus, and structurally, everything looked perfect. That was all good news that I was happy to hear. (mostly. I'll explain that later.)
Once the scope was done and she was awake enough to leave PACU, we went back to her room and Dr. P's nurse connected her to the manometry equipment. (for the details about how manometry testing works, click here) For the first 4 hours, she had nothing to eat or drink and data showing how her stomach and small intestine function in the absence of food & liquids was recorded. That gave Dr. P a look at her baseline digestive tract function. At the 4 hour mark, she was given a 30 minute infusion of IV erythromycin. Erythro is an antibiotic that can increase increase motility. The objective in giving her the erythro was to see if her stomach responded to the stimulation from the drug, and how well it responded. When the infusion was finished, we waited exactly 1 hour and then she had a 30 minute meal. The goal of the meal was to get as close to 400 calories into her as we could in 20-30 minutes. She ate about 50 calories by mouth, drank a little apple juice, and then was also given a 240 calorie formula feed through an NG tube threaded into her stomach through her G tube stoma. From that point, the data that was recorded showed how her stomach responded to and handled food and liquid. After about an hour from the point she finished her meal, the study ended.
Throughout the whole thing, I felt really good about what we were seeing and how her body was "performing" for us. I had been so incredibly frustrated during our first manometry study 4 years ago by the fact that she was such a vomiting mess for MONTHS leading up to the trip, and then just magically stopped vomiting as soon as we got to Ohio. For that reason, it was a huge relief to see a very typical stomach day during this manometry study. When she ate her meal, it was like a textbook feeding therapy appointment on a good day. She ate excitedly for 10 minutes (about 8 fries and a few nibbles of potato with margarine) and then just stopped, and did not feel good. We saw on the screen that at that point when she didn't feel good and stopped eating, her stomach had stopped moving too. I got a little excited/hopeful seeing that because I felt like maybe it was an indicator of something abnormal. When you KNOW there is a problem, all you can ever hope for is that someone or some test will pinpoint the cause of that problem. For the first time in a very long time, I started to let myself have hope that this would be the time that we would find "it".
All throughout the study, I kept reminding myself of my goals & objectives for repeating this testing, and reminded myself that no matter how the results came out, I knew that we were seeing a typical stomach day, and that meant that the test results would be accurate no matter what they showed.
I guess this would be a good time to back up and explain my objectives & goals:
1. This test would be the end of the road for us as far as testing goes. We went into it knowing that if this didn't turn up anything new, then we were done looking. We have turned over every rock we have found to turn over, and this would be the second time we'd be looking under this rock.
2. I needed to know if the way Raya's digestive tract is functioning right now might just be as good as it gets. I needed to know if there was anything we could be doing that we haven't already done, or if we are seeing what will be the normal ebb & flow of her digestive function. I needed to know these things so that I could decide where to set my expectations moving forward.
3. I needed to have the peace & reassurance that I have done right by her, and that we as her medical team have done right by her.
4. I needed to see evidence of her digestive tract function so that I could feel confident in the decisions we make moving forward. Over the years, her doctors have brought up different possibilities for more invasive ways to treat the problems she has had, but I don't think that any of us felt confident moving ahead with any of them without something more concrete to show us whether they were clinically appropriate treatment options or not. We're talking about surgical options, injections, and more invasive things like that, which could have long-lasting ill effects if they weren't truly needed.
I went to sleep Wednesday night after the testing feeling at peace (aside from Raya's incessant tooth grinding), even though I couldn't suppress the thought that I was not going to hear what I wanted to hear from the doctor. When the time came for Dr. P to come in and discuss the results, I was trying to hold onto the hope that she would tell me she had found something, but I was not at all surprised when I heard the words, "normal manometry study" come out of her mouth. Then she said, "Actually, it was pretty close to a textbook normal study. I haven't gotten the full report from the other hospital yet from her first manometry study but it looks like her small bowel function has actually improved since the first one." She said that they look for certain patterns and phases of contraction, and that they saw everything they were looking for. I asked about the point during her meal when there was nothing happening on the screen and she felt full and sick, and she said that what we were seeing was one of the normal phases of the gastric phase of digestion where the stomach rests. I was kind of speechless at that point. She said that because her stomach function is normal, we need to use her stomach (meaning no more GJ tubes). She also said that we need to work up to bolus feeds because her stomach needs practice at stretching/expanding as it fills, and continuous feeds do not do that.
That was the point where I had to take a deep breath and will myself not to cry. It is hard to help people outside of the medically complex world understand why the LAST thing I wanted to hear was "normal manometry study" and "work back up to bolus feeds." Isn't normal digestive tract function what we wanted? Yes and no. Of course we want for everything to just be normal. I would love for her to just eat a normal diet like every other 5 year old and not need a tube at all. However, that is just NOT how her body works. If her digestive function is normal, then why can't we feed her more than 3 ounces per hour without making her feel sick? Why does she take 5 bites of food and then feel so full she thinks she might throw up?
The answer to those questions is something we have known about for a long time now. Raya has a condition called visceral hyperalgesia, which is a fancy way of saying that the nerves in her digestive tract that perceive sensation are overly sensitive, and her brain perceives the signals from those nerves in an exaggerated way. Her problem is not a mechanical problem, it is a sensory nerve problem. Since there is nothing mechanically wrong with the way that her digestive tract is functioning, visceral hyperalgesia seems to be the root of her difficulties digesting food. We knew that she had this condition but never realized the extent to which it affects her. The best way I can explain visceral hyperalgesia is this. Everything she feels in her digestive tract is magnified. She feels as full after eating 5 or 10 French fries as we would feel after eating the entire burger and all the fries that come with it. To her, a gas bubble that would feel tickly or not even noticeable to someone else feels like a volleyball passing through her intestine. When she lays down to go to sleep at night, she can't hold still because she feels like she has a crawly feeling like restless leg syndrome in her belly. It's hard to know exactly how it all feels to her, but we know that every sensation is exaggerated and things that most of us wouldn't even notice or perceive as uncomfortable are extremely noticeable and uncomfortable or painful for her.
What all of this translates to is that there are no medical interventions we can take to help her. I realize that we are not dealing with an imminently life-threatening condition here, but it is a kick to the gut to actually hear someone say that there is nothing that can be done to fix this problem. Part of her is broken and we can't fix it. I asked her point blank if this is just going to be Raya's status quo, to make progress and then slide backwards whenever there is an illness or something that stresses her system. I didn't want to hear her say it because I already knew the answer to that question. She said, "Yes, her digestive tract is her weakest system. We know that, and so we know that anytime she gets sick, her digestive tract will be the first system hit, and will be hit the hardest. Things will continue to be up and down for her, probably for many years to come. Eventually her immune system will be stronger and her digestive tract won't overreact to illness by shutting down, but this is going to be something that she struggles with for a long time." She did also say that she doesn't expect Raya to do well with solid food until she is back on all bolus feeds. That's another thing that I anticipated hearing but still felt like a kick to the gut.
It was what I expected to hear all along, and yet it was still heartbreaking. I had allowed myself to hope for something more. More of what, I don't know. More suggestions, more options, or something besides "just keep doing what you've been doing only try harder." I never expected a miracle cure or a quick fix, but I had hoped that she would find something that we hadn't tried to treat before so that we would have a new angle to approach it from. But we didn't get that.
Here is what she suggested:
1. Keep her on her medications. We are already using a neurological medication to treat her visceral hyperalgesia, and at Dr. P's suggestion, we will keep her on it indefinitely, as it helps to calm the nerves that are overly sensitive to sensations in the digestive tract. Since she is overly sensitive to everything in her digestive tract, we will also be keeping her on her reflux meds indefinitely. A lot of people are critical of long-term PPI use, and I've had a lot of people "helpfully" suggest alternative treatments for her reflux, but it is not a coincidence that the inside of her digestive tract is looking so perfect right now. It's because of Nexium.
2. Work back towards bolus feeds. Being told to do that made me want to kick and scream and throw a tantrum. We've been TRYINGGGGGGGGG to do that for 2 years!! If it was as easy as it sounds, we'd be doing it already. I know she wasn't saying it in judgement and she understood that we are feeding her the way we are out of necessity, but she did encourage me to start breaking up the continuous feeds more and to start with one bolus feed a day. Her suggestion was to feed Raya 2 hours' worth of formula in 30-40 minutes and then give her a break, and then start her continuous feed. Once she's tolerating that, we can do 2 a day, and then continue to add bolus feeds until she's on all bolus feeds. It sounds so easy but it's really not. The way I feel when I think about doing bolus feeds with her is the same way I used to feel when I was in high school and our cross country coach would drive us out into the desert and make us run up a really long, steep hill a few times. Thinking about bolus feeds makes me feel like I felt standing at the bottom of that hill, looking up. I just want to lay down and curl up into a ball and not move.
And that's it. That's all the medical experts have to offer us. I understand the concepts behind what she suggested and I really do hope and pray that we can get to the point that she thinks we can get to, but it was incredibly frustrating and discouraging to hear it all. If I could have thought of questions to ask, I would have asked them but I was feeling such a turmoil of mixed emotions that I couldn't think of any.
Here's the flip side of all of that though. We can stop with the speculation. I don't have to lay awake at night agonizing over whether this surgery or that surgery might help, with or without causing harmful long-term side effects. I don't have to spend hours of my time fighting insurance to approve the pyloric botox injections that her GI doctor and I thought might help. We know that there's no point to putting her through GJ tube placements or doing the surgeries we've talked about. We know that it's not a problem with her digestive enzymes or the amount of stomach acid she's producing. We have not created this problem by putting her on medication for her acid reflux so I don't have to feel guilty for choosing to use traditional medicine to treat that condition. I have done every bit of research I could. I have gone to the experts in pediatric digestive difficulties. I have taken her to the people who understand the kinds of problems she is having and have years of experience treating children with these same kinds of problems. We have once and for all ruled out the things that we thought could have been the root of the problem.
We have already started working more aggressively on starting bolus feeds. We tried it on Saturday for the first time. I went slow but it still ended in vomit. I went slower yesterday and she felt yucky but kept it down. We did it in 40 minutes again today and got all 6 ounces in with only one close call where she thought she would throw up. The plus side is that doing 2 hours' worth of formula in 40 minutes buys her an extra hour and 20 minutes off the pump. I do hope that this will be the start of progress, but after 5 years of ups and downs, I admit that I'm feeling cynical. Maybe it will work out, and maybe it won't. We can't complicate things too much with her feeding schedule or else kindergarten in the fall will be even more difficult than I'm already afraid it will be.
*sigh* so there you have it. We went, we did the testing, the testing said her digestive tract function is normal, and that just means the burden I had hoped could be shared by her doctors is once again being placed squarely on my shoulders. It's overwhelming in many ways but we will keep pushing and keep trying. People ask me frequently if "this" is something she will grow out of. That question almost always makes me want to punch something because it implies that she will one day just magically be fine, and that will never happen. (don't worry if you've asked me that before, I'm not mad at you, just the situation) So the final answer to that question is NO. NO, she will not outgrow this! She will need the feeding tube for years to come and it will take a LOT of work to get her to a point where she doesn't need the feeding tube for nutritional support anymore. (more work than most people will ever comprehend) I'm okay with the tube. We are okay with the tube. We hope that the people close to Raya are okay with the tube and will understand that it's okay for her to have it because it is keeping her with us and helping her to thrive and reach her potential.