Considering out next trip to Houston is fast approaching, sharing the rest of the last trip is becoming more imperative. My oh my, where DOES the time go!! It feels as though, like here, I am several weeks behind in certain aspects of my life, while other parts are current, and it all makes for a bit of discombobulation, so bear with me.
When I last shared, we had gotten to Tuesday night and were headed to Cardiology with Madison and Pulmonology with all three the following day. I will finish up the medical side of our trip down in this post, but want to do another post about the fun, wonderful things that happened while we were there too and will get that posted very soon.
Wednesday morning Madison had her ECHO and EKG and we had the privilege of seeing our cardiologist to go over Miss Moo’s stuff. The lead up to a visit with this specialist is always a bit fraught with concern, just because it’s so hard to know what we will find to be going on. The stress, as we continue getting positive test results, is lessening, but I am not real sure it will ever really go away. The reality is, too many of Madison’s issues, and the associated symptoms, overlap, and heart function can change on a dime, sometimes silently. Personally, I prefer issues that I can clearly define and have a pretty good idea how tests are going to come out. But alas, that’s not the case with this system and I am slowly but surely learning to accept that.
Madison’s ECHO and EKG looked great!! Pretty sure my sigh of relief was audible…LOL.
While the appointment from the testing/result end of things is something I don’t really look forward to, meeting with Dr. M is always informative and reassuring, and this one was no different. I love how Dr. M thinks outside the box and doesn’t stop at “just” heart stuff….she wants to help us understand whatever might be going on. Unfortunately, with Madison, there always seems to be something “going on” and Dr. M is incredibly tuned into that and provides some further thinking on what is happening and how we might address it. Her insight is invaluable. Absolutely invaluable.
With the stabilization we have seen in Madison’s heart issues, the possibility that she might have had Myocarditis, or an infection in the heart, at the time of her Dilated Cardiomyopathy diagnosis is gaining some footing. Unfortunately, there is just no way to know, and it still doesn’t tell us what her long term outcome with this will be, but provides just a little more hope that she might improve and someday might even be cardiac medication free. We can hope at least, and maybe with just a little more zeal then we felt confident in having before.
We went over all of Madison’s other issues happening, most specifically the increase in her autonomic complications, and I learned from Dr. M that in part, it’s the cardiac medications (ACE inhibitor and Beta Blocker) that are contributing to the autonomic issues. It turns out that these two medications effectively block some of the bodies normal responses to the stress infections impose on the body, so in a sense, it could be contributing to the increase in the autonomic symptoms she is experiencing. With the continued stabilization in her heart numbers, we are trying a reduction in her ACE inhibitor, with removal of the Beta Blocker when she is in a crises and we are seeing the low blood pressures. It’s worth a try and might make a difference in her responses to the infections. It also allows us the chance to see how her heart handles the reduction of the medications, something that will eventually have to happen if the thought of getting off them all together really enters the picture.
Additionally, she would like to add Paxil into Madison’s medication regime. They have found that for some of her autonomic kids, this medication can provide an increase in energy levels during the day. While I am a bit loathe to add something new in, the thought that we might be able to help with Madison’s energy levels is awfully tempting. I am waiting though for now. We have some other changes in the works at the moment, and I really need to do things one at a time so we can see what’s helping and what is not. We have the script and will give the Paxil a try at some point, just not quite yet.
Finally, we have an event recorder for the next month, in an attempt to try and catch one of the times when Madi feels her heart is racing, and we drew labs to check on heart numbers and a few other things. The labs all looked good, but her CPK is a bit elevated, no doubt because of all the crud she has been fighting lately.
Wednesday afternoon, the kids and I headed over to see our favorite pulmonologist/sleep doctor, Dr. J. Again, another brilliant clinician who happens to also have a fantastic bedside manner and a desire to help figure out what may or may not help the kids. In some ways, this appointment was actually pretty simple and straight forward…..emotionally for me though, not so much. I knew that my reluctance to get things in place to get night time oxygen started had reached its end and I needed to get on board for the kids sake. In that vein, it was likely the very best time Dr. J could have chosen to share that she really felt Abby needed it too. Starting two or three at the same time, suddenly didn’t seem all that different.
How did we get to this place? Sleep studies, actually happening since last November, have been showing us that all three kids are dropping their oxygen saturations at night, particularly in REM sleep, to low enough numbers that it is concerning. Add to this that they are seeing AMAZING results in mito kids with supplemental oxygen, and that we need some improvement in energy levels in general in all three, and we have the perfect mix of good reason to give this a chance. Chance and Abby will be on nasal canula’s, while we will pipe it in through Madison’s BIPAP mask, all at night while they are sleeping. Madison may try some supplemental oxygen during the day too, but has made it clear she is not keen on the idea of anyone seeing her with it on, so it would be for here at home and while getting from point A to B if/when we give it a try.
This really is a GOOD thing……it could make a real, tangible difference for them….but it’s one “new normal” I am definitely needing to adjust too….seemingly more than the kids even (which is really a good thing).
Early Thursday morning Chance and I headed over to TCH for a Resting Metabolic Rate (RMR) test. This is a test I have heard about, and is being used by one of the mito specialists in Atlanta to aid in diagnosis, but had not realized was available to us. Because of Chance’s weight loss it was ordered to see if it would show whether his metabolic rate was higher than normal providing some explanation for the much higher than normal calorie intake needed at this point. It was an interesting and benign test that provided us with some useful information!! I like those kind!!!
Later that day we headed over to see our motility GI’s NP to go over Chance’s results, check where his weight was sitting, and talk about Abby for the first time and see what we might could do with her and her finicky belly. As always, C was awesome, easy to talk to, and was able to give us a plan which always makes these things a little more tolerable.
The RMR did in fact show that Chance is hypermetabolic which helps explain the increased need for calories. Why that’s the case, and why now and not before is unknown. Further, this is the first time they have done this test through the GI department, so we didn’t really have any other kids, much less mito kids, to compare to. It’s information, from an easy test to do, so it was worthwhile in that respect, but I am hopeful they will perhaps start doing them more and have some information to share about it in the future. I am also hoping to find someone that has had it done during their workup in Atlanta that might know more how it’s being used there.
Thankfully, Chance’s weight was holding steady, so the protocol we have in place right now seems to be working!!
We left with some new ideas and plans for Abby too, so all in all, a good appointment with some options left to try before we contemplate attempting the Cisapride study. We could not get started on it till after camp in August anyway, so for the time till then we have some other things to try and that’s always a good thing.
And that, my friends, concludes the medical portion of our final days in Houston!!! Next up….FUN we had while there!!
