The title for today’s post comes from my own personal reaction to our appointment this morning in Milwaukee. Our wonderful doc comes in and tells me he is so relieved to see that she gained weight that he will not be sending her for surgery as he had called us up there to tell us. WHAT? I mean, I know he had mentioned it at our last appointment, but I really didn’t even remotely think that’s what the hullaballoo was about to get us up there faster. The surgery he was going to send us for was to have a j-tube placed to address kendall’s feeding problems.
Right now, she has a G-tube (gastrostomy, meaning hole into the stomach). We’re just bypassing her esophagus at this point since that muscle was having so much trouble moving food down. She has what is called a “mic-key button” placed into her G-tube site, and we hook a plastic tube (extension) onto her button when we hook her up to feed. There is a possibility of getting a “G-J mic-key button” placed, which would have one port go into her stomach still (as it does now) but would have another port with a tube that threads down her pylorus into her jejeunum (hence the J part, jejeunal feeds, meaning into the intestine). This wouldn’t be too large of a change for her, as she is currently on nearly continous feeds, and most kids need continuous feeds via their J-tube because your intestines can’t absorb at near the rate that your stomach can. Is that all clear as mud? Good.
So what her doc wanted to do was put in a surgical J-tube – meaning she would need a new hole put into her stomach with direct access to her jejeunum. For all my P2P tube feeding friends, I really have no idea the reasoning behind this except that she has already pulled her g-tube twice in less than three months so i am thinking he thought the g-j button would not be ideal? He didn’t get into hard and fast reasons with me.
BUT – the great amazingly awesome news is that she DOESN’T need to go on J-feeds yet, because by the skin of her teeth she gained JUST enough for him to say she is doing ok on her current schedule. In spite of a suspected 7 ounce loss from last week’s ped visit (she had a diaper on, different scales, etc plus her inability to consume her usual amounts of food for almost a week), she had gained 10 grams per day from our last visit. He wanted her to be between 10-13 grams of gain per day to avoid the surgery. So Praise the Lord! AND – the theory of murphy’s law in reverse does work. I packed a bag as “ju-ju” against having to go inpatient and it worked!
Anyways – I had no idea that that’s what we were going to be walking in to – and I am glad now that i didn’t know. I would have stressed out over whether she was headed for it or not if I had known. And for now, at least, it’s a moot point. I do love our doctor’s willingness to be very straightforward in that, she may hold off on it for a year, she may never need it, and it might be only a few months till we have this conversation again. No telling. He also made it a point to tell us that if she is exhibiting more coughing/refluxy/vomiting episodes that it is likely a sign that her fundo (nissen wrap around her stomach) is slipping, coming undone. The hope is that it stays in place long enough to give her muscles a chance to grow into a better coordination pattern and for her to grow out of a vomiting pattern. Around a year (for normally developing kids) is when they see that happen. If hers becomes all the way undone, there is a chance we could have it re-wrapped (some surgeons do it twice, others won’t touch it once its come undone once). If it comes undone and can’t be re-wrapped, we’d definitely be looking at J-feeds if she continues to vomit her feeds.
All of that led into a conversation about her current state of feeding intolerance/diarrhea/near dehydration status, and the fact that in spite of him trying to find a hundred other causes for it, it does seem to be suspiciously related to her getting the vaccine last week. One vaccine. Given the history of being inpatient after the only other two times we’ve tried to vaccinate her, it seems very reasonable that the bottom line is that her body just cannot do two things at once – function properly AND fight off infections/vaccine titers. We knew some of her immune numbers were low from a previous admission where we tested them – but everyone had hoped at the time that it was just due to system immaturity and (say it with me now) Malnutrition (*insert evil organ music here – Dun dun Dunnnnnnn*). CLearly that’s not so much the case as she received this vaccine during one of the healthiest periods she’s ever had, and look where it got us. A week of craziness. The problem is that even though she is improving today, it’s still going to take at least another 24-48 hours to get her back up to full strength full speed feeds. And five short days later, we go for another vax.
Dr. N said he would write a letter that kendall is absolutely not to have any more vaccinations till she can get in with Immunology to determine what is going on with her immune system. He also did a great job explaining that her food “allergies” that we are seeing aren’t so much true “allergies” as they are her body’s way of trying to fight ANY intruder – whether that’s baby pears or enterobacter organisms.
And lastly he emphasized that we need to get in with genetics/metabolics ASAP. I explained that I had been on the phone with them for about half an hour yesterday learning that they are essentially on a recommend-only basis for metabolic appointments, and that there is an almost year long wait for a general genetics appointment. I mean, good lord, I hope your poor baby doesn’t die of whatever it is genetics is supposed to diagnose while you’re waiting a year for an appointment!!! Seriously. So he said he would make some calls. His feeling is that this is a mitochondrial disease of some kind – it’s the only thing that ties all of her symptoms together and that makes her whole non-sensical self make sense. I guess by this point I’ve heard it tossed around so much that it doesn’t have the same shock value that it used to.
To close out the appointment, we got walked through doing a tube change with the nurse – FUN TIMES! At least it wasn’t the emergency procedure of a few weeks ago with blood and guts pouring out the stoma! And now she has a nice shiny new (working!) tube in! No more leaky formula, no more wonky balloon that decides to randomly deflate, no more spinning extension!!! All in all – a pretty good appointment – especially when you consider it was supposed to be for surgical set-up and instead came out with a good prognosis for the immediate term!
We got home to a message on the machine from the genetics scheduler (who I spent the half hour on the phone with yesterday telling me we had a VERY long wait ahead of us) telling me that they had an opening on Tuesday afternoon and the doctors would like her in then. Neat. Guess our doc has friends in high places! So back up to the land where the river diverges we go. They supposedly have all our records and all the most recent bloodwork. Now to just find someone who can put all those pieces together. I have to put a call in to her neuro tomorrow to figure out where the muscle biopsy results are at currently, and see if there is any other info they can offer for genetics. At this point, I have no idea what to expect, but i can tell you my expectations are pretty low. I don’t have any disillusions that they will be able to pinpoint her with one 2 hour long appointment. I don’t think they’ll look at her paperwork and know instantly what we are dealing with. I really don’t even expect them to have anything helpful to her every day care to offer. But a little teeny part of me kind of hopes. I think you have to keep some hope.
So that’s that. We survived it! Couldn’t have done it without the prayers of so many of you. And now we push on toward the next step – Tuesday! But in between then and now – we have a fun four day weekend full of barbeques and last blasts! I hope you all have a great time! Enjoy the last blast of summer (hopefully the weather cooperates!)