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Monday, December 29, 2014

The results of our California trip

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.

Saturday, December 27, 2014

Manometry and motility testing

We returned home a week ago from our second and final experience with antroduodenal manometry testing (aka AD manometry). The first time we had it done, I was still very new to everything we were experiencing and hadn't had the time to wrap my head around all of it so I felt like a lot of it went over my head. I have spent nearly 5 years now trying to learn everything I can and trying to understand how the digestive system works, and how Raya's digestive system works. I've learned a lot in 5 years, both from experience and from textbooks, so I thought I would share what our experience was like for round 2 of manometry testing in the hopes that it will help someone else be more prepared going into motility testing.

(Our first experience was at Nationwide Children's Hospital in Columbus, Ohio in 2010. Read about it here and here.)

Here are a few key words to help this make sense:
*motility- the way that food and liquids move through the digestive tract
*dysmotility- problems with the way that food and liquids move through the digestive tract
*manometry study- a test that measures the strength and coordination of muscle contractions through the digestive tract
*antroduodenal manometry (or AD manometry)- a manometry study that looks specifically at the function of the digestive tract between the antrum of the stomach (the part that connects with the small intestine) and the duodenum (first portion of the small intestine)

This round of motility testing was done at Children's Hospital of Los Angeles.

We started with an EGD (endoscopy) to look at the condition of her esophagus, stomach, and duodenum. They all looked beautiful. My girl is beautiful on the inside and out. Literally. :)
While she was under anesthesia, her G tube was removed and the manometry catheter and an NG tube were threaded through the G tube stoma. The manometry catheter was guided into place in her small intestine and the NG tube was in her stomach so that she could be given a formula bolus and have the extra water from the manometry catheter drained out of her stomach during the study. The yellow tube is the NG tube and the clear tube is the manometry catheter:
(*Note: if she hadn't had a G tube, they would have placed the manometry catheter & NG tube through her nose instead. For that reason, I am SOOOO glad she already had a G tube because the catheter was probably about a 12fr, which would be huge in a little nose.)

Normally they would have waited for her to fully wake up on her own but because of how badly she usually wakes up from anesthesia, she has to get meds to help her sleep off the anesthesia drugs so that she wakes up more gently. We waited for about an hour but she was still completely out cold. We had to wake her up as much as we could get her to wake up so they could let her leave PACU and go back to her room. At Nationwide, she did not have an endoscopy and I was able to be in the room with her (in Interventional Radiology, aka IR) while they took out her GJ tube and put in the manometry catheter. There, the study was done in a special room in a different part of the hospital but at CHLA, they took us back to her hospital room and just brought the computer & equipment into her room. I liked that a LOT better. It made things much easier on me since the study lasts 6-8 hours. This is what the manometry catheter was connected to once we got her back to her room:
The blue tube coming from the lower left corner of the picture is the catheter that was in her stomach. Inside the catheter, there were 8 tiny tubes that each connected to another tube on the machine. You can see the dark blue caps connected to the lighter blue tubes in the middle of the picture. Here's a closer picture of that part where you can see each of the small tubes connected to the machine:
Each of the tiny tubes had a tiny pinhole at some point along the tube, and each pinhole rested at a different point in the stomach, pylorus, or duodenum. There was a constant flow of water from the canister (to the right of all the tubes in the middle of the picture) through the tiny tubes in the manometry catheter, and each time there was a muscle contraction at one of those points in her digestive tract, the muscle contraction would pinch the pinhole and obstruct the flow of water through the tube. The obstruction would cause pressure to build up between the pinhole and the computer, which would then register on the computer monitor as a spike on the reading.

On the monitor, there were 8 colored lines that each corresponded with one of the tiny tubes inside the manometry catheter. Here's what it looked like when her stomach still wasn't really doing anything:

And here's what it looked like when things started moving again after she finished waking up from the post-anesthesia fog:
That was the first real spike we saw in any of the sensors. We guessed (and the doctor later confirmed) that the 3rd sensor was in her pylorus. We could more or less tell which sensors were where based on the activity. The top 2 were in her stomach, the 3rd was in her pylorus, and the other 5 were in her small intestine.

The study was run on a very specific schedule. She was NPO (nothing by mouth) for the first 5 1/2 hours. At the 4 hour mark, she was given a dose of IV erythromycin to stimulate muscle contractions in her stomach. The medication went in over a 30 minute time frame and then we waited 1 hour from the time it finished. At that point, we had 20-30 minutes to get as close to 400 calories into her as we could, either by mouth or through the NG tube. When they first told me what the schedule of events would be, I thought it seemed backwards to give her the erythromycin before the food and I was afraid that doing it in that order would keep us from seeing an accurate representation of how her stomach behaves in the presence of food. They explained that the reasoning for the order of things was to first show us how her stomach & small intestine behave in the complete absence of food, then to show us if her stomach responds to erythromycin and how well it responds to it, and then to see how her stomach would respond to a meal. They assured me that her stomach would return to its baseline activity level in the hour we waited between the end of the erythromycin dose and her meal, and it did.

Most of the day was spent keeping Raya as still as we could keep her, telling her how many more minutes until she got to have a sip of apple juice, watching movies, playing games on the iPad, playing with toys, listening to a couple of different singing groups that came around to sing the kids Christmas songs, and staring at the computer monitor. I have to admit that the science nerd in me was totally fascinated with it. It was pretty amazing watching a visual representation of what was going on inside her body moving across the screen. One thing that the nurse pointed out to me (and the Chinese doctor that was observing) was something called a migrating motility complex (or migrating motor complex), also known as an MMC. Peristalsis is the wavelike muscle contractions that sweep food & liquids through the digestive tract in small portions. MMCs are a pattern of peristalsis that happens in the small intestine. We could see that Raya was having MMCs as we watched the screen and saw a pattern repeat progressively from one sensor to the next. It was pretty cool to see, and it was good to see an indicator that her small intestine was having normal function. This is what it looked like:
You can see that the MMC started at the orange line in the middle and then the contractile pattern continued to move through her small intestine, registering on each sensor as the muscles contracted in a wavelike pattern. You can also see in that picture that her stomach (the top 3 lines) was not active at all during that time.

Crazy things happened when they gave her the IV erythromycin. (Good crazy. It was supposed to happen.) Within a minute or two of the IV erythro starting, we started seeing a huge amount of activity in her stomach. As I mentioned before, the point of this was to see if her stomach would respond to erythromycin and how well it would respond to it. It most DEFINITELY responded!
The erythro started right around the where the red X is. There wasn't a whole lot going on in her stomach at that point. The blue X marked a spot where she had started laughing and it registered on all of the sensors. We marked it on the study because we were making note of how her demeanor was during certain points. Shortly after the blue X, the erythro really started to kick in, as evidenced by all of the huge spikes on the screen. The yellow X marks the point where she stopped smiling, stopped responding to verbal cues and questions, got very quiet, and became fidgety and squirmy. In other words, she didn't feel well.

When the erythro ended, we started the final 1 hour countdown before she could have some food and a drink of the apple juice she had been asking for all day. It feels really awful telling a child that we've spent 5 years begging/pleading/encouraging to eat that she can't even have a sip of water so it was a huge relief for everybody when it was finally time for her to eat. Since we knew there was no way she would eat enough food for the study, we also mixed up 240ml of formula so that she could get an 8 ounce bolus feed during the 20-30 minute mealtime while she ate her food. We ordered everything the cafeteria had that I knew I had a good chance of getting her to eat: baked potato with non-dairy margarine, corn, refried beans, French fries, and apple juice. {small rant: It was frustrating to know that even at a hospital, her food allergies were not fully accommodated but we did the best we could. Aside from cooking her fries in oil that was cross-contaminated with wheat and who knows what else, they also sent up crackers that we didn't ask for that she is quite obviously allergic to. It's kind of irritating to see that happen in a hospital where I had given her food allergies to multiple people when we were admitted.}

Moving on... She was really excited to get her food and she ate well for the first 10 minutes, just like she does on a great day at feeding therapy.
This is what the monitor looked like while she was eating and still feeling good:
 You can see the spikes in the top 2 lines that were measuring stomach activity, and also the 4 big spikes in the pylorus, which probably (if I had to wager a guess) showed that food was being segmented and pushed through the pylorus into the duodenum.

Also true to form, by the 10 minute mark, she was no longer very interested in eating her food. She was no longer leaning forward and engaging in the meal, but rather she was laying back against her pillows. She was having a great time talking to us, trying to distract us from the task of encouraging her to take bites, and playing with the fries, but they weren't making it into her mouth anymore. Within a couple more minutes, she just flat out didn't feel good anymore. It was written all over her face that she felt like puking.
It may sound strange but I was really encouraged at that point. Seeing that her body was doing exactly what it normally does meant that we were recording data that fit into the scope of Raya's "normal," which meant we were getting an accurate study. I didn't feel like we got an accurate study when we did the first manometry study 4 years ago because she had been vomiting several times a day for months, and only vomited twice that whole week, and neither time was during the manometry study. Having that prior experience, I felt really good about how things were going during her meal, and pretty much the whole day this time around. Interestingly, this is what the screen looked like at the point where she had stopped eating and felt sick:
There was very little activity happening. Since I really didn't know anything about what I was looking at, I had my fingers crossed that seeing her stomach stop moving was clinically significant but it turned out not to be. Boo.

We kept plugging along and encouraging her to eat as much as we could get her to while the nurse gravity fed her the 8 ounces of formula. (for the sake of reference, she has not had an 8 ounce bolus feed over 20-30 minutes in probably 2+ years, and I don't think she has EVER had an 8 ounce gravity feed, and I'm pretty sure the last time she had 8 ounces of formula in her stomach that fast was 2 years ago when she chugged a box of Splash and then threw the whole thing right back up. She hasn't been fed above 90ml/hour for close to a year.)
Then we got a beautiful serenade from a mariachi band:
It was seriously awesome. I wished they could have played music for us all day long!

From then on, we just sat around and tried to keep her stomach from leaking too much while we waited for the study to be over. There wasn't much of anything exciting going on in her stomach or intestine from what we could tell.
When the nurse thought we had everything we needed, the motility doctor came in to quickly look through and have a quick look at the data, and then the nurse carefully peeled off the tape, pulled out the probe, we put in the new G tube I had brought with us and we were done. (BRING YOUR OWN REPLACEMENT unless you're okay with getting whatever kind of tube the hospital happens to have or else getting the old G tube put back in. We were due for a change anyway so I brought a new one.)

So that's the basics of an AD manometry study. The hardest part is keeping an active child still and occupied all day long. There is also a LOT of extra pee, thanks to the constant stream of water from the manometry catheter. The nurse told me that for kids who are potty trained, they will bring in a bedside commode since they can't get to the bathroom with the manometry catheter in place.

The doctor came in the next day to go over the results with me (read about that here) and then we were discharged later that afternoon and flew home late that night. All things considered, it was a successful trip and even though the results were disappointing, the actual testing process went really well and I was happy with the way that CHLA does motility testing. The travel was really simple too. We flew into LAX, took a bus to the train station, and then got on the subway. There was a subway stop literally across the street from the hospital. The bus fare was $8 and the subway was $1.75 so it was really affordable and easy. I would highly recommend going to CHLA for motility testing, especially for anyone who lives closer to the west coast than to Ohio or one of the other motility centers.

Monday, December 22, 2014

Our trip to CHLA

It's amazing how much I have to say about something that only took 3 days. I have preliminary results but I haven't gotten the final report from the motility specialist yet so I'll save that part for another post. This post will be the story part and the nitty-gritty stuff will come later. :)

Our trip started at 4:00 Tuesday morning when I dragged myself out of bed to pump one more time before leaving. (Thank goodness for a baby that takes bottles!) Speaking of bottles, I chugged a bottle of this on the way to the airport:
I'm not much of a soda drinker and I normally pace myself but I was pretty sure that the TSA would not agree with me about Mtn Dew being a medically necessary liquid. It totally is.

We had checked in online and printed our boarding passes, and we weren't checking any bags, so we went straight to the security checkpoint. I hadn't connected her to the feeding pump yet so that made it really easy to get through security with all of our liquids. They were very kind to us and made it really easy for us. They inspected her medical stroller by hand since it was too big to go through the x-ray machine, and while they did that, I went through the liquids with the TSA agent. Since we hadn't set off the metal detector, I was able to touch our things. (not the case on the way home) She said we would need to take the pump bag out of the backpack and put it into their machine but I offered to open it and let her hold the little strip of paper over it to do the vapor test instead, and that was MUCH faster and easier for both of us. They visually inspected the 2 bottles of liquid medication but didn't have to do anything else with them, and our ice packs were frozen solid so they didn't count as liquids. Piece-o-cake. Raya is cute and charming at 5am so they gave her stickers and told her how cute she was. She loved the attention, of course. :)

We made it to the gate pretty fast so we had time to get a gate check pass for the stroller (which they technically considered a wheelchair) and they also gave us a pass to preboard. That was MUCH appreciated and needed. Everyone in Phoenix was very nice! Raya was just a teensy bit excited about getting on the plane:

We got onto the plane and that was when I realized that she had soaked through her pull-up (thank you, overnight feed) and her pants were wet so we made a quick pit stop in the bathroom. And of course, we had to take an airplane bathroom selfie:

Once we got back to our seats and got all settled in for the flight, I got out her special surprise. Last week, we had made 10 Tubie Friends and donated them to our GI clinic. Build-A-Bear was having a great sale when I bought them so I got Raya one too. I thought it would be nice for her to have something to snuggle on our trip. I had also gotten a $10 gift card for $5 as part of the sale, so we went to BAB last week and I let her pick out an outfit for "a very special little girl who needs a pretty outfit for her Hello Kitty." I just didn't tell her who the little girl was. :) Needless to say, she was really excited about her new "Hella Kitty" when I pulled it out of her backpack.

We sat near the front of the plane, and since we were some of the first people onto the plane, we watched a LOT of people get on. As the plane was filling up, Raya said, "Wow, there's a lot of people that have to go to this doctor!" Apparently she thought everyone on our flight was going to LA to see a motility specialist like she was. :)

Taking off was about the most exciting thing that's ever happened to her. She thought it was HILARIOUS. When the flight attendant brought me my juice and a cup of ice, I gave the cup of ice to Raya. She looked at me and got a big smile on her face and said, "What?! They have my favorite snack on this plane!!" When I asked her if she wanted to have the baggie of Kix I had brought for her, she said, "No thank you. I just don't want to get the Kick taste in my mouth right now."

Getting up for a 6:45am flight is not the most fun thing to do but nothing beats watching the sunrise from an airplane. It was beautiful!
We had packed just enough things to do to get us through the flight. She was in a fabulous mood and kept herself entertained until it was time to land.
It was bright and sunny until the plane started descending and dropped below the clouds. Then it was rainy, gloomy, and gray. We landed at 7:15 am and made our way to the bus stop to catch a bus to the next leg of the trip. While we waited, she insisted on putting her hood on even though we were in a covered area because it was raining.
We got everything loaded into the bus and then got ourselves situated for the bus ride, which ended up being longer than the plane ride. Traffic was bad (according to the driver) and the wet, rainy weather made it worse. The driver was very nice and gave me instructions on how to find where we needed to go next once we got to the train station, and which stop to get off at in order to find the hospital. His help was much appreciated!
When we got off the bus at the train station, the rain was coming down in a steady drizzle. It wasn't quite like the Arizona rain we are used to. Our rain comes down in heavy, fat droplets that you can feel when they hit you. These raindrops were small and sneaky. We didn't realize how fast we were getting soaked but we were soaked! There was a very nice lady that had been sitting near us on the bus and overheard the conversation with the driver about getting to the hospital. She kindly offered to help us get on the right train so that we could get where we needed to go. Raya was pretty excited to ride an airplane, a bus, AND a subway train all in the same day! Well, she was excited until she smelled the first elevator. Elevators in subways serve a dual purpose as toilets, as it turns out. She will probably plug her nose in elevators for a very long time. It took us 4 or 5 elevators to get from the sidewalk down to the subway platform and back up again, so the smell is quite etched in her memory. *gag*  (we are not city people)
She seemed to enjoy the train ride but I think some of it was a bit nauseating for her. About halfway from the train station to the hospital, I realized we were missing the large rolling backpack I had packed all of MY stuff in. To make a long story short, we did get it back later that day. It was nothing short of a miracle and it is a story for another blog post where I can do it justice.

Losing the bag caused a bit of a delay but we finally made it to CHLA at 11:00 that morning. I was feeling quite down and dejected by the time we got up to her room as I kept remembering everything I had packed in the lost bag. We walked into her room and saw that Santa had just visited and had left her a Hello Kitty jewelry kit on her bed. *cue the Mommy tears*
We got her weighed and measured and did her first set of vitals, and then got her changed into her hospital clothes. She thought it was neat that this hospital had "tiger pants and tiger shirts" instead of the gowns our hospital has. After all of that was done and we had learned to operate the fancy schmancy TV, I got word that my bag was waiting for me at the train station. I put on a movie for her and went back as quickly as I could to get my bag.
I was gone for just about an hour, and she was in exactly the same spot when I got back that she had been in when I left. Whew. It was a HUGE relief to have it back (even in spite of the $40 that was missing) and I said many prayers of thanks on the train ride back to the hospital. It really is a cool story.

There wasn't much to do the first day. When the weather cleared a little, I pulled up the shade and realized that we had a fabulous view from our room on the 6th floor. I am not familiar with Los Angeles geography and didn't realize how close the hospital is to Hollywood but we could see the Hollywood sign from our room. It looks farther away in the crappy cell phone picture than it actually looked from her room but now I understand why people say it's not as exciting in real life as it is on TV. :) It was cool to see it though. We had a beautiful view! (not evidenced by the following picture but we really did)

As the sun started to go down, we noticed how beautifully the garden at the main entrance of the hospital was decorated for Christmas. They had it closed because of the rain but it was pretty to look at, even from the opposite end of the hospital 6 floors up.

We had to face the inevitable that afternoon and get an IV placed. I had requested IV team as soon as we got to her room and thankfully, we had a WONDERFUL nurse who listened to me and called IV team. Raya is a very strong little girl and it took 4 of us to hold her and get the IV in, but they got it in on the first poke and it was perfect! They used an ultrasound vein finder and I hadn't seen that type be used before so it was cool to see how they did it. They also used a Bard StatLock to keep the IV in place and it made for such a nice, stable IV. It was not a mess of tape, gauze, and cotton balls that constantly had to be readjusted like most of the IVs we've had at our hospital.

We switched her from formula to Pedialyte around 3:00 that afternoon. After her IV was in and we had the plan lined out for Tuesday night & Wednesday morning, Raya and I snuggled up in her bed with "HellaKitty" and watched movies.
As lousy as all of this medical stuff can be, and as difficult as hospital stays can be, I truly do cherish the time I get to spend alone with my Raya. I wish that I could have had as much alone time with all of them as I've had with her and I wish that the alone time I spend with her could be under different circumstances, but I am grateful for the time I get to spend with her. She is such a tough, brave little girl and even though it is difficult to see her in these trying situations, I am always so proud of her for how quickly she bounces back and for how sweet she is to the people who are doing things like starting IVs.

Raya likes to surprise me. Usually at times when it makes me look silly in front of other people. Like when they asked if we needed anything and I asked for some juice and crackers for me but didn't ask for anything for her. She never drinks anything but water so I never even offer her juice anymore. It turns out that if it's in a cute little box with a straw, she likes it. Go figure.

I have to say that I was impressed by how quickly the hospital got all of her meds and formula to us. We actually ended up using our Nexium packets from home because they didn't have the same thing in their formulary (which I expected) and they were fine with it since I had brought them in the original packaging with the prescription label. We were only about an hour or so behind our normal schedule on her sleepy meds. After being up since 4:30 am, it was sweet relief when she was able to go to sleep. It's hard to put into words how I feel when I see her peacefully sleeping. Much like eating, sleep is something that does not come easy to her so it makes me happy to see her relaxed and resting.

She was supposed to go NPO at midnight (NPO = nil per os, or nothing by mouth) but we just turned off the Pedialyte at 11:30 when they came in to do night meds and the nurse started her IV fluids then too so they didn't have to come back in at midnight. I loved all of our nurses!

Transport came up at 6:15 Wednesday morning to take us downstairs for an EGD. It was kind of funny to wake her up and get her out of bed at an early hour just to take her downstairs and put her back to sleep for an endoscopy. She was a little anxious but she snuggled with "HellaKitty" and took it like a big girl. I'm kind of in love with this picture I snapped on my phone while we were walking down the hall:
We sat in pre-op for a while. It was pretty boring. The place was insanely busy though. It was much busier than our hospital's pre-op areas have been when we've been there. There was no playroom there so she was bummed. Not that it would have mattered anyway since she was hooked up to monitors and couldn't leave. We took selfies to pass the time.

And pictures of our glowing thumb:

And pictures of our tiger shirt with leads coming out the top:

And asked questions about how the nurse connects IV tubing to this little blue thing:

Meanwhile, I talked to all of the nurses & doctors that came by. I was able to chat with anesthesia about giving her something to keep her from getting nauseated and something to keep her from waking up like a wild animal, both of which have happened before. They didn't have the clonidine that we normally use but they used precedex instead and it worked beautifully. Once we had that all figured out, we met Dr. Punati, the motility specialist, who was doing the EGD. We talked about how the day was going to go with the EGD and then the manometry study and she answered some of my questions. A few minutes later, they hauled my darling girl off to have her procedure done. She had her beloved pink blankie with her and her new Hello Kitty, and she didn't get upset at all like she usually does when we get to the place where I have to say goodbye and go to the waiting room.

I felt like it took forEVER for them to call me. I was bored. There was nothing good on the TV in the waiting room, my phone didn't get reception there, I didn't have any money in my pockets to go buy breakfast with, and all the magazines were in Spanish and I didn't feel like I had enough brain power at that time of morning to try and read them. It was almost 8:00 when they took her back for the EGD and about 9:00 when they came to get me. That's a really long time for an EGD. It made me wonder what she had found in that little stomach. I started to feel a little bit hopeful that maybe she actually HAD found something, even though I suspected that she wouldn't. My suspicions were correct. She brought me back pictures of a beautiful, perfect stomach and small intestine.

I was fine with that. She hasn't had symptoms of ulcers or anything like that. I was hoping she would tell me there was a problem with Raya's pylorus (the valve between the stomach & small intestine) because there are things we can do about that but there was nothing visibly wrong with her stomach. They took me in to PACU (post anesthesia care unit) to sit with her while we waited for her to wake up.

I don't care who you are or how many times you've done this, it is always a little bit disturbing to see your child in a drug-induced sleep with monitors, wires, and oxygen connected to them. She's usually on blow-by oxygen after anesthesia so the cannula was something I wasn't expecting to see. One thing I do always appreciate though is that whenever she takes her blanket back with her, the nurses always cover her up with it when she's done so that she wakes up wrapped in her own blanket.
She has fairly low blood pressure anyway, but the drugs they had given her were making it lower than her baseline. Her pulse was lower than normal too, and I don't know what a normal respiratory rate for a sleeping 5 year old is but 11 breaths per minute seemed a little low. (the monitor thought so too, so it kept beeping) Her pulse pressure (i.e. the difference between the 2 blood pressure numbers) was low too. She set off lots of alarms. Nothing bad happened, they just had to keep an eye on her. As long as her O2 stayed in the upper 90s, I don't think anyone was worried about the rest of her vitals being low.

She probably could have kept on sleeping for another 3 hours but we had to wake her up so we could get back upstairs to her room for the manometry study. She couldn't stay awake but she woke up enough that they were okay to let her leave PACU. We got her back to her room and kept working on waking her up since she was supposed to be awake during the whole manometry study.

A few minutes after we got her settled back in to her room, Dr. Punati's nurse came to connect her to the computer for the manometry study. This is what it looked like:
That TV was a total lifesaver. I had planned a few things to keep her happy (and still) during the manometry study (6-8 hours...) but when she woke up, she really was not in the mood for anything. She didn't feel very good after being NPO for 11 hours and anesthesia, so she was really content to just lay there and watch a movie. (and when I say "a movie" I mean the SAME movie over and over) She really likes Despicable Me 2. Good thing it's a cute movie.
After a while, she felt a lot better and was ready to color in the notebook I had gotten her with the little bitty markers. I love how her tongue pokes out of her mouth when she's concentrating. :)

I will do another post and go into detail about the actual testing process but suffice it to say it was a long day trying to keep a very active 5 year old still and being patient with her when she got impatient with everything. I spent a lot of time taking the caps off of her markers, putting them back on, swapping out one set of toys for another, trying to get her to understand how much longer it would be until she could have a sip of apple juice or water (because 5 year olds don't understand "2 more hours"), and looking at the data being recorded on the monitor:
Lots of squiggly lines. The first time we did a manometry study, the lines on the screen meant absolutely nothing to me and I don't know why I didn't ask anybody to explain things to me, other than I figured they probably wouldn't tell me anything until the doctor had done his final review. This time, the nurse that was with us for the testing was very nice about explaining things. I think it helped that there was a doctor from China that was observing and learning how to set up and administer a manometry study. The whole thing was completely fascinating to me. It was real life, practical application of most of the chapter in my anatomy & physiology book about digestion. Pretty sure I understand that chapter a lot better now than I did then, and I thought I had a pretty good handle on it then!

In case anyone is curious, this is what it looked like when the G tube was out and the manometry catheter and NG tube were in:
This time, since she's a little bit bigger than she was 4 years ago, all of the sensors were inside her stomach so we weren't getting water leaked all over the place all day long like the first time.

When she got bored with the movies & TV shows and all her other stuff, she played on the iPad.
"Um, Mommy, I can't really see it very good. Can you take a picture so I can see it?"
The highlight of her day was when she FINALLY got to have some food and a drink. She had been asking ALLLLLLLL afternoon for a sip of apple juice or a sip of water. I hate telling her no when she actually wants to eat or drink something. For the test, we needed her to eat as much food as she could within a 20-30 minute window while she also got an 8 ounce bolus of formula. I ordered her everything the cafeteria had that I knew she would probably eat: baked potato with margarine, French fries, corn, refried beans, and apple juice. She was SO happy to get her food!
As per usual, she started off really strong with eating her fries and baked potato. She was excited to eat after having gone over 24 hours without anything nutritive, and not really eating anything at all for about 3 days. After about 10 minutes, her body language changed, she appeared uncomfortable, and she started doing all of the things she normally does to divert everyone's attention away from the task of getting her to eat. She started playing with her food instead of eating it, suddenly gained a lot of interest in whatever show was on, and had all kinds of things to talk about. She is the master of distraction!
By the time she had gotten the full 8 ounces of formula (which the nurse slowly gravity fed while Raya ate), she was feeling pretty yucky. She is not used to getting that much food & formula in a 30 minute time frame.

Just when we were reaching our limits with everything, we heard some beautiful music coming from down the hall. Raya couldn't get out of her bed to go look but I stepped out into the hallway and saw that a Mariachi band was playing requests for each of the kids on the floor.
The little girl down the hall had requested "Let It Go" and it was honestly the most beautiful rendition I've heard yet. I wish I had gotten the whole thing on video but I only got about half of it. (I'll post the videos at the end) As soon as Raya recognized the song, she perked up and started smiling again, and sang along in her scratchy, adorable post-endsocopy/anesthesia voice. After they finished at that room, they came to Raya's room. She asked them to play her favorite Christmas song: Rudolph the Red-Nosed Reindeer!
(pardon her hospital hair. try as I may, I canNOT keep it neat while she's in the hospital!) They had the voices of angels and could do incredible things with their instruments! It is the only time Rudolph the Red-Nosed Reindeer has made me cry, and I wasn't the only one! The nurse that was with us for the manometry testing cried too. :)
After they left, I found out that their group is called the Mariachi Divas de Cindy Shea, and they are 2 time Grammy Award winners. I really just wanted to hug all of them because they provided us with the perfect distraction at the exact moment that we needed one. It was another one of the small but much appreciated miracles that helped us through the trip.

Not long after we were serenaded, we reached the end of the study. We took one last picture of the funny tubes in her stoma and then it was time to take the NG tube and manometry probe out and put in a new G tube.
By the end of the study, there was fluid leaking out of her stoma because her stomach was not used to having as much food & formula in it in such a short period so it was overflowing a bit. Aside from having to wait for what felt like forever to get food & drinks, and then feeling sick when she got so much in her stomach all at once, the worst part of it for her was having all the Hypafix pulled off of her stomach at the end. It was a lot of tape. It was even stuck to the inside of her belly button, which she found to be amusing.
Even though it hurt pulling the tape off, she was very sweet and patient with the nurse and didn't get upset. She is such a good girl!

I thought she would have sworn off of food & liquids after that experience but she wanted her juice box back when we were done putting in the new tube. All the nurses wanted to see her tube because most of them had never seen an AMT Mini One before. It was nice to be able to explain to them why we chose to switch from the MicKey and what features make the Mini One a better tube for Raya. (I'm not saying the MicKey is inferior, just that it is not the best tube for Raya.)

After that, our sweet, wonderful day nurse, Maya, came to give evening meds and get one more set of vitals before she was off for the night. She was so great to Raya (and to me) and life at the hospital is SOOOO much easier when your child likes their nurse!

When all of that was finished, we got her changed into her pretty new Hello Kitty nightgown that I had bought her for our trip. Getting the sleeve over her IV was tricky but she was so happy to have it on!
She somehow managed to fight her sleep meds for an extra hour but finally drifted off to sleep. I'd say it was peaceful but there was SO much tooth grinding. She woke me up SO many times grinding her teeth. She probably wondered why I kept grabbing her face.

Thursday morning, we started off the day by brushing hair & teeth and getting out of bed for some much-needed activity. It's so much easier to do that when you're off of IV fluids! We had a lot of time to pass that day while we waited for the motility doctor to come talk about the results of the study, and then waited for discharge papers. We colored:
We took selfies:
We got vitals done and had our tummy listened to by our student nurse:
We made bracelets:

We tried to sneak away while Mommy was in the bathroom to visit the adorable little baby on the other side of our room, and dance behind the curtain in our candy cane slippers.

We tried on our (kind of) matching bracelets:
We ate more fries, potato, and corn and watched Despicable Me 2 for the 4th or 5th time:
I also said, "Raya, sweetheart, please don't put your French fries between your toes." for the first time in my life.

We paused for a moment to be amused by the size of the IV pump and feeding pump:

And also paused for a moment to appreciate how neat and tidy her IV site was, thanks to the StatLock they used to keep it in place. SOOOO much nicer than the IVs we get at our hospital that are kept in place by a mess of tape, gauze, and cotton balls and have to be adjusted every time somebody looks at them. This one was perfect the entire time it was in and never had to be adjusted at all. The adhesive left some marks (probably because of the way they removed it) but the IV itself was perfect.
We also had to take a picture with the menu screen where it said her name on the TV:
We brushed our teeth again too. She has an intense toothbrushing face:
We played Jenga with a nice volunteer lady:
Which gave Mommy time to pack up everything, including all of the frozen milk that the nurses had so kindly put in the breastmilk freezer for me. They really were so wonderful to us and it made the whole thing so much better.

Then it was FINALLY time to get out of there! (but only after refusing to change out of our Hello Kitty nightgown, which was totally fine because we had a really late flight)
We left the hospital at 4:00 and stopped for a couple of photo ops on the way out. There was a pretty tree in between the east & west sides of our floor (it looked a lot prettier in person than it does on my crappy cell phone pic):
And by the sign on the way out:

I thought about grabbing something to eat before we left the hospital but we were both antsy to get out of there and I figured I'd just get something at the airport. We crossed the street and headed to the subway stop, at which point Raya plugged her nose with both hands and said, "Mommy, do we HAVE to get in the elevator? It stinks in there!"
We got to the bus stop at about 5:00 and were fortunate to get to the ticket window just as their credit card machines stopped working (and they only take cards, no cash) so they were unable to allow us to pay for our bus tickets and we got a free ride back to the airport. It was another tender mercy. Not that I didn't have the money to pay for it, but it kind of softened the blow of having had the $40 stolen out of my bag while it was in possession of the same bus company. We made it back to the airport at around 6:00 for our 9:35pm flight to Phoenix.

Getting through airport security was a little less smooth on the way home but it was something I could have prevented. If you are ever traveling with a child who is on medical fluids, disconnect them from the pump before sending them through the security checkpoint. If I had remembered to do that before we got to the checkpoint, she would not have had to get a "pat-down" (that I don't think ever really happened) and they would not have had to inspect everything we were carrying on without me being able to touch any of it. I get that rules are rules but I did appreciate that when we were leaving Phoenix, the TSA agent was able to let me move things around for her and help her inspect things so that nothing got spilled and it was easy for me to put everything back how I needed it to be packed. Even with the extra inspections, it still wasn't bad getting through security and they were still really nice to us, even when I asked the lady if she had put clean gloves on. (she didn't like that I asked her) The worst part was trying to keep Raya from walking away while I put everything back together and put my shoes back on. They kind of took their time inspecting her stroller. Once I was able to strap her back in, everything was fine.

I have decided that LAX is an awful airport. Or at least the Southwest terminal is awful. There are very few food options, all of which are ridiculously overpriced, and it was far too crowded to navigate with Raya's stroller and our bags. I ended up settling for a coffee-free drink from Starbucks that was loaded with dairy (which Piper did not appreciate a day or two later) and a chocolate chunk muffin. We found a spot on the floor (yes, the floor, which was as crowded as the chairs) and waited for a flight to leave so that there would be a chair to sit in. I gave her the evening doses of her meds to keep her on schedule and then realized I needed to pump again before the flight. We went to the horribly tiny and crowded bathroom and waited for one of the only 2 stalls that her stroller would fit in while I pumped. I hate pumping in bathroom stalls but what else am I supposed to do.

She didn't last long once we got back to the gate. I found the perfect seat right on the end so that I could park her stroller right next to me without it being in everybody's way, but then I decided I'd better get a preboarding pass while there wasn't a line. The only rudeness I encountered the whole time we were at the airport was when I told the lady at the Southwest counter that I needed a preboarding pass for my daughter. Without even pausing, she replied right back to me, "We only give those to people with disabilities." At that point, Raya was passed out in her ginormous medical stroller with her feeding pump backpack attached to the handle and hospital bracelets still on her arm. I was a little dumbfounded at the way she shot right back telling me I couldn't have one because when we were in Phoenix, a woman who appeared to be quite able-bodied had cut in front of me and told the gate agent that she needed a preboarding pass, and when the agent asked her why, she said that she just needed extra time boarding the plane. If she can do that and get one, I thought the woman could have at least been polite enough to look for the child that I had said needed one. *end rant* I told the lady that my daughter was under the age of 5 and that we had medical equipment and that I would be having to carry her onto the plane. Once she finally looked around the counter and saw Raya passed out in the stroller, she said, "Oh, okay, I couldn't see her." Next time, I hope she actually looks first rather than being snippy at someone.
I honestly don't know how I could have managed this trip on my own without that stroller. Our regular stroller just doesn't cut it anymore. We finally got our preboarding pass and went back to where we had been sitting. While I was gone, a lady sat in the seat I had been in and put her bag in the one next to her. I thought that maybe she'd be polite and scoot over into one of the empty seats next to her but she didn't so I sat back down on the floor rather than parking Raya in everybody's way.

We were once again blessed by the kindness of strangers when it was time to get on the plane. As I was looking at everything and trying to figure out how I was going to manage carrying our bags, folding up the stroller, and getting a very much asleep Raya onto the plane, some off-duty flight attendants came over and took my bags from me, and then one of them held Raya while I folded up her stroller at the bottom of the jetway. They got us settled into our seats and I was so appreciative. Before long, we were up in the air and waving goodbye to Los Angeles.

Our plane landed at midnight and it was about 1:00 in the morning when we got home. I was greeted by some very thoughtful gifts on the kitchen table and a nice, clean house. It made me feel very loved.
The trip was a whirlwind and as soon as we got home, I realized that Raya was breaking out in a rash. By morning, her arms, legs and face were covered. I'm 100% sure that it was from the fries she ate at the hospital being cross contaminated with everything else they fry in the same oil they cooked her fries in. It was a bummer but on the bright side, we now know that we're not avoiding wheat for nothing. :)
So that's the story part of our trip. Next up will be the nitty gritty of the testing and the story about our lost bag. Here are the videos of the Mariachi Divas:
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