Our Life is…

May was full of medical appointments for our girl that I haven’t had time to write about AND she had an extra big appointment this morning, so I’m going to start there today and then work backwards…

Today was K’s first visit with the motility specialist. It was a long wait, but well worth it! K’s gastric motility (how well the stomach moves food through) has been in decline for about 2 years now. We’ve tried a couple of things, but there hasn’t really been a fix OR a reason why this suddenly became a problem after several years of great stomach function. On top of the slow and steady decline, she also REALLY struggles whenever she gets even a little sick (think sniffles) and sometimes things seem to sloooooow down for no discernible reason. In the past week, she has vomited twice, had us stop her feeds three times, and asked to be drained (venting out her stomach contents through her button) twice. When we have to vent her (or when she is vomiting), we can’t also feed her…so it makes it difficult to give her the calories and fluids she needs at times. It is important that she gets a steady supply of these since she is prone to ketotic hypoglycemia and her metabolic disease increases her need for fluids.

Currently, K is on Augmentin (an antibiotic) in an attempt to help her gastric motility…but it has never really been great at that. Instead, it helps with her intestinal motility and we’ve seen a big reduction in bowel accidents since she has been on it (I think because it makes it easier for her to really empty her bowels which she wasn’t fully able to do before). She also takes 2 tsp of Miralax twice a day for constipation. Dr. C wants to wean her off the Augmentin, since it isn’t doing its assigned job (and she has been on constant antibiotics for over a year now)…so we are increasing Miralax while weaning Augmentin and adding in Senna as needed (a stimulant laxative in case things slow down too much during the transition). He is also adding Periactin to her medications. She took this as a toddler as a migraine preventative without luck, but it is being used here because it can aid gastric accommodation (the ability for the stomach to stretch to comfortably hold food). It can also increase hunger which would be a beneficial side effect. Once we’ve switched these things around, she needs a sitz marker test done. Basically, it is a capsule filled with small circles that will show up on x-ray (see photo). We need to somehow get them into her (hoping we can hide them in baby food peas, because we can’t put them in her tube!), so they can do a week’s worth of x-rays to watch how they travel through her insides.

Additionally, he wrote an order for a GJ tube. A GJ tube will replace her current G-tube and look pretty similar (though it will be bigger), but it will have a port to feed into the stomach and another port to feed into the intestines. The plan will be to continue feeding her into her stomach the majority of the time and then feed into the intestines when her tummy is not tolerating it. He suggested that once it is placed, we should only do J feeds for a couple of days to test whether the problem is JUST the stomach or also the small intestine. If J feeds go well, then the problem probably isn’t also in the small intestine at this point. Since her issues are intermittent, this might not paint a real accurate picture, but worth a trial run. He is also going to see if it is possible for him to order a tilt table test to see if dysautonomia (over-simplified explanation: the body incorrectly switching itself into fight or flight mode for no reason at all) is contributing to her GI problems. We follow up with him in August and depending on how things go, she may need some further testing (endoscopy, manometry testing)…but those are NOT fun, so we’re starting with the easy stuff first.

He said that the root of these issues is probably the dreaded disease progression, but as all of her docs have said recently…SHE LOOKS REALLY GOOD! It is true, she is still growing very well, she is active, happy, silly, smart, most days she feels pretty good…all good things! We just want her little wonky insides to match those good-looking outsides…so if these changes might help with that, we’re all for it!