We had our second care conference with Adela’s main doctors today. We had a whole list of questions and they did a good job of answering them all. Granted, some of the answers were “we don’t have an answer”, but we’re gradually coming to terms that that may be the norm with some of Adela’s problems. The most exciting part of the meeting was the plan for us to be home near the end of next week. Of course, all this is subject to change depending on her progress, but the doctors all seem to think that, given her progress so far, being discharged at the end of the week is a realistic goal. Initially there was a discussion about us going home still on TPN and reducing the rate at home. We indicated that, even though it would add time to our stay, we wanted to wait until she was completely off TPN before going home, just for our own comfort level.
So, from the beginning, here are my main questions and notes from the meeting:
What caused the initial inflammation? We most likely won’t ever know. Adela has several genetic issues that are associated with higher incidences of severe colitis. People with duplication of a certain region of the ninth chromosome, defects in certain regions of the X chromosome, or some mitochondrial DNA defects all have a higher chance of suffering from intestinal issues like Crohn’s (or other Crohn’s-like conditions). Adela is two for three on those…and the third test is still pending. There’s the chance that this is actually Crones and we just happened to catch the initial flare, but there’s no way to tell at this point.
Will the inflammation recur?
Since we don’t know what caused the initial flare, we don’t know if it will recur. The plan is to have another scope procedure done in two to four weeks to look at the state of her colon. The thought is that if it looks like it’s recovered, we can possibly treat this as either a one-time issue or a flare of an colitis. Either way, we could come off the steroids, have some follow up appointments, and if all’s well move on. If there are signs of continued inflammation or damage, we’ll treat this as a chronic condition and we’ll switch from steroids to an immunosuppressant for long-term management.
Are there any outstanding tests that would answer the two questions above? If so, when will the results be back.
No. The only outstanding tests are a redo of her karyotype and a mitochondrial DNA test. While either may shed some light on that she’s more prone to colitis, neither can answer definitively what caused the initial flare or if it will recur.
Are there any remaining tests to be done before discharge? (eg – another metabolic cart test, other genetic tests, etc)
Yes. We’ll be redoing the metabolic cart test soon. The thoughts are that, when the initial test was done, Adela was suffering from the inflammation which caused her body to need more calories. Now that the inflammation is under control and she’s healthier, her caloric needs may have changed. Since they’re in the process of tweaking her TPN/Elecare ratio, having a better understanding of exactly how many calories Adela’s body needs will be very useful data. Other than that, just routine labs to ensure that the reduction of the TPN isn’t causing any nutritional problems that may arise if Adela’s intestines aren’t able to absorb the nutrients from the Elecare.
What’s the timeline for the reduction/discontinuation of TPN, the corresponding increase of Elecare, and the reduction of steroids?
TPN will be reduced over the next week and discontinued near the end of next week. Elecare will be increased to compensate for the TPN reduction. The dose of steroids will be reduced to a maintenance level over the next week and will remain at that level while we’re at home before returning for the second scope procedure.
Was there any proof this was food-related?
When can we reintroduce other foods? How?
In four to six weeks we can start reintroducing other foods. Even though there’s no proof that it was food-related, there’s the consensus that we shouldn’t add any more variables into the equation while she’s recovering. We’ll see how she recovers at home off the TPN with just Elecare. After we have proof from the labs that she’s doing well, we’ll start to reintroduce regular foods. We have a plan of which foods to introduce in which order and we’ll be following up with labs to ensure that none of them are causing the problem either.
What supplements/vitamins does she need taking into account her food allergies (that there are some nutrients she won’t get, eg – selenium)?
She’ll go back on her NanoVM vitamin that is a comprehensive vitamin supplement. Anything she doesn’t get through Elecare will come from that.
How long will she be on steroids?
For about two to four more weeks. They’ll be reduced over the next week then she’ll be on a low dose until we come back from the scope.
What follow-up appointments will we have and with whom?
We don’t have a schedule yet, but we’ll be following up with GI, Immunology, Metabolics/Genetics, and the general team. The plan is to try to get scheduled for all those on the same day so we’re not making a ridiculous number of trips over here.
How long will she keep the broviac?
We’re definitely going home with it. How much longer past then we keep it depends on several things. If everything looks good after the scope in two to four weeks, it may come out then. If there are concerns, we’ll keep it longer.
Will she need another flexible sigmoidoscopy? If not, what specific tests will be used in follow-up appointments to verify that her intestines are healthy and not getting inflamed again?
Yes, we’ll be back in two to four weeks for the flexible sigmoidoscopy. They can also look at her sed rate (ESR) to determine if there’s inflammation.
Did Adela’s karyotype results come back? Did they match her results from Duke?
They’re not back yet.
And of course, with Adela, she always likes to throw a curve ball just to keep things interesting. Enter her pancreas. So, several weeks ago, soon after the inflammation was discovered, she was having terrible tenesmus (pain and straining when on the potty). Her GI doctor ordered several pancreas-related tests just to rule out pancreatitis. The results did rule that out, however a fecal elastase test that can measure how well the pancreas is functioning came back with an abnormal value. The curve ball part of this is that pancreatic function has nothing to do with her inflamed intestines. It may be that we just happened to stumble upon another of Adela’s medical mysteries. So, here’s the plan — we will redo that test to see what the level is. It takes about two weeks for the results to come back, so they should be back about the time we’re coming in for her scope procedure. If the levels are back to where they should be, we’ll just keep redoing the test every so often to monitor it to make sure that we’re not in some kind of cycle of normal then reduced pancreatic function. However, if the levels come back low again, they’ll need to take a look at the pancreas in more detail. They can do an echo, however it’s usually not very reliable on the pancreas. Most likely, they’ll need to do an MRI of the pancreas, which means Adela will need to be still for about forty minutes. Since the chances of Adela remaining still for that long are, let’s face it, nonexistent, we’ll schedule the scope and MRI to be done one after the other so she only has to be sedated once. Granted, this is all a few weeks out and any of it may change, but that’s the plan as of right now.
Now on to some more good news, Adela has been getting off her pumps for a few hours a day and today was in the lobby play area for the floor walking around all on her own and just as happy as could be. If you looked at her, you wouldn’t have even known she was sick. She’s really hit it off with the daytime nurse she’s had for the past three days and is having fun with both the preschool teacher that comes each day and her visits up to the playroom with the recreational therapist.