Update 11/11/19

With several months behind us since starting her new medicine, it has yet been difficult to get a full grasp on how much it will help Evelyn in the long-run. After only one dose of Gattex, she definitely showed signs of less dehydration and a lot more energy. Over the first few weeks, her stool output decreased while her appetite went up and her need for IV fluids lessened. We were very optimistic as we were able to taper her TPN relatively quickly; she went from needing 1700 mLs daily to only 600mLs in 3 weeks! Since then she has tapered further to 400 mLs daily, and has transitioned slowly over the last month to cutting out two nights a week. With this has come the pressure for her to get the calories in that she needs, as she doesn’t seem to require as much fluid-intake, which has been a relief for her. We used to think that if we could just get her fluid needs taken care of, she could enjoy eating and do fine.

But, every day has not been perfect, as she seems to continue to have her gut-upsetting days. With her slowed motility, there are days that her stomach doesn’t empty as well. Her TPN taper has stalled because she has the occasional need to vent her g-tube of extra fluid buildup that just didn’t want to digest that day. This slows her eating as well, so her weight has come down in the last few weeks. She went from having the occasional bad day to every few days having set-backs, and this last week she is back to only the occasional bad day again (maybe 1-2 a week). Because Evee isn’t the typical “short-gut” patient that they give Gattex to (her small bowel is actually a decent length) we are still experimenting and learning what this new drug can do for patients like her, and given the initial progress she made, it is obvious she is benefiting somewhat. Dr Mercer told us that as the Gattex is changing her bowels, her body has to adjust to the new square-footage that brings this feeling of “fullness” with it. It is hard to say at this point if it is Evelyn’s underlying motility issue trying to shine through or just the adjusting. They like to give the patient a full 5 months on the drug, as this seems to be the amount of time it takes to max out the benefits of the drug. So, we try to be patient and wait for her body to make the changes, if it will.

On the plus side, she hasn’t needed any hydration fluid at all since we started the medicine; this is great, since during a gut-episode she usually will need supplemented, sometimes daily. We are hopeful that even if the Gattex hasn’t “cured” any underlying issue, it has at least helped her recover much quicker and if a bigger “storm” is to come in the future, we can perhaps lessen the need for IV fluid.

Throughout her life we have tried unsuccessfully to manage the ups and downs with no idea what her intestines have needed to succeed; they’ve usually recovered on their own to a mediocre state that enabled us to “get by” until the next storm, whether it was a line infection or gut-motility flare. We cannot forget that only 9 months ago after her recovery from surgery, she was totally line-free and thriving wonderfully, without the need for Gattex. So, it seems we may not have a full picture of what her guts need and the medicine may be the bridge to be less dependent on IVs, for now, but doesn’t seem to be the cure-all. Time will tell. Meanwhile, in the last week she has an ankle that is giving her grief, this time the left foot, and we are praying that it is a simple muscle problem and not a brewing storm. May God continue to guide and give strength. Please remember us in prayer, that we could be given strength to not weary in this journey and accept with patience the ups and downs as they come. Thank you for all.