It's been a day, and this is going to be very, very long and I really don't expect anyone to read it unless you're wondering what it's like inside the mind of a "tubie mama" on a stressed out day. Actually it more or less started last night when I was driving to the grocery store. I was already tired, it was after 9pm, and I was thinking about what I needed to talk to Raya's GI doctor about at the appointment this afternoon. Next thing I know, I'm bawling in the car and trying to compose myself so I can go in the store without looking like a crazy person. Most of the time I think I do a pretty darn good job of being positive. I know I could be working harder on...well pretty much everything, actually, but I think as far as Raya's medical stuff goes, I do the best I can with where she is at as far as food and her stomach go. I can remember what her worst times were like and I know how very far ahead of those times we are now, and that usually makes it pretty easy to stay positive. But.
Right now I'm incredibly frustrated. For a year now, she has been stuck. A year ago this month, she had a huge backslide and went from getting her bolus feeds of 250ml each (8.5 oz) in about 25 minutes at the maximum pump rate of 600ml per hour to not being able to handle that same amount in anything less than 2 hours (rate of 125ml/hour). Here we are a year later and she is still at 125ml/hour. I've tried a few times in the last year to increase her pump rate. She does okay for a little while (sometimes a day, sometimes a few days) but inevitably she starts to seem "off" and we go back to 125 and she's fine again. We even see it when she eats solid food. I thought it was so funny (but not necessarily in a ha-ha way, more in a how interesting way) that when she would drink a full box of Splash, which is also about 8 ounces, there were a couple times when she drank the whole box in about half an hour or less and then puked. When she wasn't being pushed to drink it that fast and could go at her comfort level, she "grazed" on it and finished it over the space of 2 hours. Coincidence? Probably not. Clearly, that's a rate that she feels comfortable at, gosh dang it. Does YOUR kid need to take 2 hours to drink 8 ounces of liquid?? This is the part that frustrates me and makes me stress a little about how her future is going to look.
Since the day she got her first NG tube at 10 weeks old, people have asked me if this was something she would eventually grow out of. It's a fair question and I completely understand why people ask, and I have never faulted anyone for asking. I'm sure if the tables were turned, I would be asking too. That being said, that question has brought me so much frustration and even anger and sadness at times for 2 reasons. (not at all directed at whoever is asking though, I promise) One, we have never been able to really nail down what "this" even IS so we have no idea what to expect. We can speculate that yes, she will eventually be able to eat & drink enough to not need the feeding tube anymore, but nobody has any idea when or how that will happen. The other reason that question stings is because the phrase "grow out of" implies that she will just one day be all fixed and magically be "over it". The hundreds of hours we've spent at therapy appointments, doctor appointments, and the thousands of miles we've put on the car & the money we've paid in insurance premiums & medical expenses are pretty sufficient evidence that there will be no magical "growing out of it" happening here. Not one single step of this has come without a huge amount of effort on the parts of everyone involved.
With the slow expansion we've been doing of Raya's diet, I have once again been smacked in the face by how unnatural it is for a child who is almost 4 years old to still be learning to eat. Every single bite for her is a process. When I want to eat lunch, I decide what to eat, make myself some lunch, and sit down at the table with my food, a glass of ice water and something to read, and then I eat it. I take drinks of water after every few bites and I'm usually finished in about 15 or 20 minutes. That's about all the thought that goes into it. For me, the hardest part is deciding what to eat.
Now let me explain Raya's eating process. First of all, she usually doesn't ask to eat a meal, and especially not lunch. Most of the time, she is happiest and feels the best when her stomach is empty. She asks for snacks once or twice a day and if everybody else is having dinner, she is now to the point where she expects to have a plate at the table too but most of the time would be pleased as punch to just eat a few chips or a cup of ice for dinner. Lunch for Raya usually starts with me telling her that it's time for lunch and giving her 2 options for what to eat. That is followed by her either dodging the question or telling me she doesn't want potatoes or peaches, she wants potato chips. Eventually she gives in and decides she wants potatoes. While I cook her a baked potato in the microwave, she gets crushed ice and water in a cup, picks out what plate she wants to eat off of, gets herself a fork, and plays musical chairs at the table since nobody else is home & she can pick whatever chair she wants. I bring her plate to the table and start to peel and cut up her potato. She gets upset and tells me she LIKES potato peels now and wants me to leave them on so I leave them on. I give her the plate. She wiggles in her chair, talks to herself, plays with the fork, eats a chunk of crushed ice, and finally takes a bite of potato but uses her fingers instead of the fork that she insisted on picking out and getting by herself. After she chews and swallows that bite, she starts playing around again. I tell her to take another bite. (telling is the only thing that works, you can't ask her if she's ready for another bite or she won't do it) She finds all kinds of ways to distract herself and usually only takes one or two bites before she needs to be told again to take another one. Then I remind her to take a drink of water but I also have to remind her to drink it slow or she will choke on the water about half of the time. After about 5 bites, she starts picking the peels off of the potatoes. (remember, the peels that she insisted that I leave on?) Then she needs to wipe her hands on something because she has too much potato on her fingers. Then I poke a piece of potato with the fork and hand it to her. That's usually followed by her pulling that bite off of the fork and poking a different piece of potato with the fork and eating it, because heaven forbid she eats the bite that I gave her. This process continues until I can see that she's slowing down (ha ha, yes, it can actually get slower than that) and/or that she's starting to look uncomfortable. There is a definite change in body language when she gets to her limit. 45 minutes after we started, she has eaten 1/3 to 1/2 of the potato (either a small russet or a medium sized red potato) for a total of about 50 calories. After that, she's too full to eat any more and 45 minutes is a LOOOOOOONG time for her (and me) to sit at the table and eat.
Now that was just for something easy to eat like a potato. It's soft and easy to chew and although it makes you thirsty, it's not particularly difficult to eat. She LIKES potato, but that's still the process she goes through to eat it. When we give her something new or something that's physically more challenging to eat, things get harder. For example, a few weeks ago we introduced pork loin. It was sliced very thin and lightly seasoned with salt and then cooked in a frying pan until it was done but still tender and juicy. I thought it was delicious and as far as meat goes, it was very soft & easy to chew. Of course I'm USED to chewing meat but Raya is not. For her, the amount of effort it took to chew each bite enough to swallow it quickly outweighed any positive feeling or enjoyment that she got out of eating it. For a child like Raya, as soon as eating gets difficult, it's no longer worth the effort and they quit. She didn't dislike pork, but she lost interest in it very quickly because of how much more work it took to eat it. The other thing that was difficult for her and kind of eye-opening for me was that she had a hard time understanding when the piece of meat had been chewed enough for her to swallow it. Sometimes she would chew a bite a couple times and then swallow it. Other times you'd have thought she was chomping on a piece of gum for how long she kept chewing on it. As difficult as it is for me to try and figure out how to get her to eat more, and to eat things more efficiently, I can only imagine how difficult and confusing and foreign the whole process must be for her. Nothing comes automatically to her because she is beyond the age where that is an instinctual part of the developmental process, so every single bite takes a conscious thought process. I can't even fathom how a 3 year old accomplishes that process. As she practices, I do see some bites becoming effortless and automatic, and I'm grateful for that, but those are much fewer and farther between than the conscious effort bites, and especially when it's something that she doesn't totally love or that is new to her.
And with Raya, if it's not one thing it's another. What I did not mention in the previous examples was that before we can get her to eat that much food (half of a potato), we have to wait about 2 hours after she's finished a formula feed. After she eats, we have to wait at least another 2-3 hours and sometimes longer before we start the next formula feed or else it hurts her stomach and makes her feel sick. To put it into other terms, if all she's getting is formula with no solid food, then she gets about 500 calories over the space of 8 hours. The example I just gave would mean that in that same 8 hour time frame, she's getting about half that because we have to give her time to get hungry enough to eat the potato and then give her time to digest it before we start filling her up with formula. That makes it difficult to get all of her calories in for the day. We've been off of overnight feeds for quite a while now, but on days where we give her more time in between pump feeds to try and increase what she's eating by mouth, we end up running the pump until the wee hours of the morning. I prefer not to feed her all night long because let's face it, digesting in your sleep is not normal or natural. Necessary sometimes, yes, but having to feed her at night is pretty much another step backwards. So the slow motility definitely works against us in the game of oral eating.
The other giant brick wall we keep hitting is food allergies, intolerances, and sensitivities. (yes, those are 3 different things) She has several foods that she is just flat out allergic to that elicit immune system responses and a couple could potentially cause anaphylaxis. There are other foods that she is intolerant to, and those elicit digestive system responses (i.e. abdominal pain and diarrhea that causes blistering skin rashes). Then there are other foods that she is sensitive to, that she doesn't have immediate reactions to but chronic ingestion over time leads to symptoms like skin problems, abdominal discomfort, dark circles under her eyes, a change in her disposition, and more subtle things like that. Trying to find foods that she can eat is a challenge. Trying to find foods that she WILL eat is a whole other challenge, and trying to find foods that she can and will eat that have a decent amount of nutrition in the amount she'll eat of them...well you get my point. Not one single aspect of feeding Raya is simple, natural or effortless and there certainly won't be any magical "growing out of it" happening. Like I said, I completely understand what people mean when they ask that question and it doesn't offend me, but the real honest answer to that question is just beyond most people's realm of understanding. It's unfathomable to most people that a child would just flat out not be able to eat, not know how to eat, and not care if they ever ate anything at all but that is the reality for Raya and so many others.
Raya had a make-up session of feeding therapy today since she was sick on Friday & we had to cancel.
Since it wasn't our regularly scheduled time, we had to use a different
room than we normally do, which seemed to affect her ability to focus.
The chair was just a regular child-sized chair rather than the chair she
usually sits in at therapy that has straps & buckles on it. That meant she was free to wiggle as much as she pleased, scoot her bum to the edge of the chair, move the chair around, etc. It was
also the first day of Raya's periactin week. She was in rare form all
day long (not necessarily in a good way), and although she wasn't crabby for most of the day like other
periactin days and she didn't sleep all day long like last week, she was
not herself either. She acted so silly all through therapy and had ZERO
attention span. She also didn't seem hungry at therapy in spite of having plenty
of time between her morning feed and her therapy appointment. I think there were more verbal cues tossed around in that feeding therapy session that we've EVER had to use in one session before. When all was said & done, she had eaten about 60 calories' worth of potato and about a teaspoon or two of whole black beans, which was probably a dozen beans. 50 minutes of work for a lousy 50-ish calories. (negative, perhaps, but today I'm not feeling like my usual "glass half full" self) Even her therapist made several comments throughout the session about how "off" she was acting. She's still feeling a little under the weather and it wasn't her normal appointment time, so I think part of the behavior was an effort to stay awake but yeah, I hope it was a one time deal because I just don't have the energy for that every time she eats.
The one funny thing that came out of feeding therapy was that at some point when I wasn't looking, she took the cap from my water bottle, put a bean in it, and put it in front of her stuffed lamb on the table. I got a good laugh out of that.
While I was sitting in feeding therapy, my phone rang. I never answer the phone during appointments but the number that popped up was the GI doctor's office, and we were supposed to be seeing them half an hour later so I figured it was probably something important and answered it. Turns out that somebody made kind of a dumb scheduling mistake and didn't catch it until 30 minutes before our appointment. There was a family with 3 kids that were all seeing our GI but somehow Raya got scheduled in between 2 of them. My guess is that Raya was scheduled first and those kids got filled in around her. They wanted us to come at 3 instead of 2 so that the family could be seen all at the same time. It was completely understandable and wasn't a huge inconvenience for me but it was kind of annoying that nobody bothered to notice that or call me sooner. I had scheduled feeding therapy at 1 because the clinics are about a mile apart so it was very convenient. Or it would have been, anyway. Instead of leaving FT early like we had planned, we finished out the whole appointment time and then went to the pharmacy a couple miles away to pick up a prescription, and then headed back to the GI office. Picking up the prescription took all of 15 minutes so we still had 45 minutes to kill. Raya had fallen asleep in the car on the way to the pharmacy so I just parked in the shade of a tree at the GI office and we sat in the car until about 20 minutes before the appointment.
As we were sitting there, I suddenly wondered if the back office MA that had called me had thought to mention to the front office staff that they had swapped us with the person after us. Of course she had not, so when I did the automated check-in kiosk, it told me to report to the front desk because we were more than 10 minutes late. The lady at the desk called us up right away and started to say, "Ma'am, do you realize you're 40 minutes late?" but I quickly cut her off and said, "Let me guess, they didn't bother to tell you that they called me an hour ago and asked me to come at 3 instead of 2, did they." I explained what they had said when they called about the family's schedule being split up and of course she had to go ask if that was really the case or not but got us checked in and we sat down. At that point, Raya was still sound asleep so I was enjoying some cuddle time, especially after having just sat through an exhausting feeding therapy session and been bored out of my mind for an extra hour between appointments.
She was still asleep when the MA called us back. He was new and it showed and we'll just leave it at that. I handed him the med list so he could copy it into the chart and by then she was awake enough to go get weighed & measured. By the time I had her changed into a gown, she was WIDE awake and feeling bright eyed & bushy tailed and I ended up having to hold her hand for the 20 foot walk from the exam room to the scale in order to keep her from running off in some other direction. (she thinks she owns the place) I was happy to see that since her last appointment 6 weeks ago, she has gained 0.4kg and is now up to 15.0kg (33 lbs 1.1 oz) and is 39.05" tall. That puts her in the 36th percentile for weight and the 38th for height and for our family, that's pretty darn good. I was just glad to see that she gained weight this time because she had gone pretty much from May to September without gaining an ounce. We've been monitoring her calories more closely though and she's been on the pump more and it's doing the trick.
There are some GI appointments where I leave afterward feeling great and others where it's all I can do to get out to the car before the tears start falling, and today was one of the hard days. It wasn't anybody's fault, it's just a very frustrating situation. For several months now, we have been talking about the fact that Raya just isn't making any progress with motility or reflux. The reflux meds she gets keep her symptoms at bay, but I don't love that she's been on high doses of PPIs her whole life and that we have seen that she is not able to function well when we take her off of them. There is no light at the end of the reflux tunnel right now, and that stinks. (and lest anyone is tempted, this is NOT the day to tell me about the home remedy or essential oil that will cure her reflux...) The subject of a fundoplication surgery has come up several times since she was very little but her GI doctor, surgeon and I all agree that she is not a good candidate for a fundo because of her history of retching/vomiting and because of her ongoing gastric dysmotility. A child with gastroparesis or delayed gastric emptying is not a good candidate for a fundo because when the stomach doesn't empty properly but the fundo takes away the ability to vomit, the stomach becomes like a pressure cooker and the child is miserable. We don't feel like it's worth that risk for Raya because she is not having problems with aspiration pneumonia, which would be the only reason we would ever consider a fundo for her. So that means the PPIs are here to stay.
Issue #2 is the gastroparesis. As my friend & Feeding Tube Awareness colleague, Traci, likes to put it, motility meds are piss poor at best. There are really not any meds that have the primary purpose of treating dysmotility. The meds that are used for dysmotility are pretty much all meds that are intended for other things but CAN have an effect on motility. We have tried 3 different ones and really didn't see any results with any of them. Pyloric botox injections would give her a good shot at having a more normal rate of gastric emptying by relaxing the muscle that controls the rate at which food leaves the stomach, but we either have to convince either or both of her insurance companies that it would be worth the expense or pay the $1200 out of pocket ourselves. Financially, it makes more sense for an insurance company to pay for pyloric botox that could potentially help her make good strides towards getting off of the feeding tube, which would benefit the insurance company in the long run. The alternative is surgery, which would also be more expensive than botox and could have potential side effects that would increase her dependency on the feeding tube, which would cost the insurance company more money than they're currently paying. Unfortunately, the people who make decisions about things like my daughter getting botox or not are not medical professionals, believe it or not. They are businesspeople who, as a board, discuss cases like this and decide whether or not they have merit, and if they do, THEN they go before a medical professional (either an RN or doctor, not sure which) to have a decision made about whether the insurance will cover it or not. It is ludicrous if you ask me. These people have no idea what this one simple procedure could mean for Raya, nor do they care enough to even give her GI doctor, who could adequately explain it to them, a chance to plead our case. It infuriates me but I don't even know who to argue with about it. Her doctor & nurse are still trying to push forward with getting it authorized though. Her doctor is also consulting with a motility specialist in Boston because she thinks that there's a possibility that if we had it done at an actual motility center, the insurance would possibly not look at it as "experimental/investigational" since the motility center specializes in procedures like that for that purpose. Still a big question mark as to whether or not it will ever happen though.
I also brought up the pyloroplasty option again. Deep down, I know we shouldn't jump into that just because the botox was a dead end, but I am just so tired of trying to be patient and wait for her body to magically start working right and I want to DO something. We KNOW that a pyloroplasty would increase her gastric emptying. The problem is that there's a good chance it could increase it too much and cause dumping syndrome and other complications, and the procedure is not reversible. Right now, it's too big of a risk to take without repeating motility testing and trying botox first. (i.e. the motility testing we had to wait months and fly across the country to do 3 years ago and the botox that insurance has refused to cover thus far) See why I feel like we're running in circles and getting NOWHERE?! At one point, her doctor looked at me and said as nicely as she could, "You know there's not going to be a magic fix for this." And I know. I think I've known that since about the time that I finally resigned myself to the fact that it was time to get rid of the "temporary" NG tube and switch to a G tube. It still feels like a kick to the gut to hear it though. It also felt like a little bit of a kick to the gut to hear her say that we need to increase Raya's caloric intake again. Without having been able to increase her feed rate in the past year, adding more calories just means more time on the pump. Now that we're trying to push oral eating, that means more time on the pump at night, which feels like a step backwards. The feeling I had when she said that reminded me of how I used to feel when I was running high school cross country and we'd go run at the butte outside of town. There was this one hill in particular that we would run up. You'd run until your lungs felt like they were going to explode and your legs felt like lead and then you could see the top and it gave you enough of a surge of energy that you felt like you might actually make it. Then you got a few steps away from what you thought was the top only to realize that there was more hill to climb beyond that, you just hadn't been able to see it before. Or like the orphans on Annie when they get all excited that they've found #1 5th Avenue and then remember that they have to get to 967.
Well anyway, the appointment was not my favorite one we've had. Nothing that we discussed or that I heard her say today was anything that I didn't already know, but sometimes hearing what you already know sucks. For now, the plan is to keep her meds the way they are but start weaning her off of the zantac when I feel like she's ready, increase her calorie goal to 1100-1200 calories a day, and come back in 2 months. In the mean time, she's going to contact the doctor at Boston again, as well as the one we saw 3 years ago at Nationwide (who I hear is now in such high demand that he's scheduling out 1 year in advance for motility testing) and see if they have any thoughts or suggestions.
Issue #3 is food allergies/intolerances/sensitivities. We saw the allergist on Monday and that appointment actually went pretty well except that I forgot to ask for a new Rx for her Epi-Pens. We discussed what foods to add into her diet, especially in the way of protein sources that won't sit in her stomach for hours after she eats them like the beans do. His suggestions (in addition to the salmon we just tried last week) were tilapia & other white fish, and home ground pork. (buying ground pork increases the risk of cross contamination with chicken, which she's allergic to, and beef, which we don't know if she's allergic to or not) I think both of those are good suggestions & we'll definitely try them. We also talked about grains and the plan going forward is to try quinoa again, as well as millet. He said we could also try tapioca again and since she tested negative for soy allergy, we can attempt soy too. That would definitely give us some options. We're also starting on some winter squash now. We'll see him back in 6 months unless something comes up in the mean time.
That's pretty much the rundown of where we are right now. Treading water, and trying to move forward without falling backward. Hopefully now that I've gotten all my word vomit out, I can go back to being my usual mostly optimistic self and be content with what we have to work with because I'm tired of feeling frustrated. The end.
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