Backing Up – Houston Day 3-5

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!!

The Mystery Continues

Answers remain elusive.

Still waiting to hear how Madison's labs look.

Sleep studies turned into daytime studies too......which means it's been a LONG 24 hours!!  We should hear what, if anything, the studies showed next week.  The girls say they slept well, and I should have...was definitely tired enough...and even had a bed to sleep in, but just didn't.  Being awoken at 6am makes for a sleepy trio!!  A bit jealous that the girls got to take naps all day!!!

Chance's cardiology appointment was a mixed bag.  The ECHO showed that his function is decreased...still just barely within the normal range, but much lower than it was in August....and that his mitral valve is regurgitating more.  Not something we were happy to see, BUT......not the answer to why he is losing weight.  The cardiologist wants to do her own measuring, just to make sure the numbers the computer was spitting out are right.

The "good" part of the appointment was that the cardiologist had some ideas on what else we need to look at and we got started on that today.  We did a chest xray and a bunch of labs, and she sent me home with a request for another test that we are hoping we might be able to get done in Dallas.  We are throwing the proverbial net out at this point....looking at endocrine possibilities, inflammatory markers, and following up on some elevated labs from November (that I didn't know about).  By next week we will either have some direction to head in......or be back at square one.

Getting tired of square one.

All appears to be a go for us to take off in the morning and head HOME!!!!!  We are all very ready.  Please pray for safe roads for us.

Held Up in Houston

Well.......we are here and will be till next Wednesday or Thursday now.

On the one hand, as jealous as the kids are of all you guys in Dallas getting to play in the snow, my thought is, your at the same time freezing your tushies off, so I am NOT jealous...not one little bit.  We are cold here, and were supposed to get snow, but managed to just get ice...fun fun fun. But we are still warmer here than there, and for that I am thankful!!!  We are missing the Old Man and the critters...a lot....but are being well cared for and loved here in Houston and are making the best of it.

While we knew earlier in the week that we were not going to make it out of town towards home today as planned, we thought we'd get out of here by tomorrow.  However, the girls sleep studies (that were supposed to happen last night) had to be postponed and the best we could do is Tuesday night.  We have accomplished all but those, so this will give us a couple of days of down time, which we really all need.

Thanks for the prayers for this week!!!  We have felt them and appreciate them more than we can say.  Since we are holed up here at the RMH today, I thought an update was in order since we asked for specific prayers and have some of the information we anticipated hearing this week.

Our pulmonologist was NOT too upset with me about the oxygen not being started yet, but it was reiterated that it's something we need to make happen.  This was further enforced by the scale.....Chance was down 6 pounds since we saw them in November, even with the supplementation.  While the pulmo is not feeling like the lower-than-we'd-like-to-see oxygen saturations are the WHOLE answer to Chance's weight loss, treating it with the oxygen at night needs to happen to see if it makes any difference.

Madison & Abigail will have their sleep studies and while we know we will be adding oxygen to Madison's bipap, we will see how things look for both girls and see where we need to go from here.

Our rheumatology visit went really well!!  While one of Madison's knee's still has some fluid on it, he was pleased with her response to the steroid injections.  After some talk about where we go from here, we got the exciting news that we might be able to try and wean Madison off her Methotrexate as early as this summer!!  As long as she continues to do well, no more joints become involved, and the ones already affected behave themselves, we are good to try. If we can get her off the methotrexate and keep things stable with the injections and an occasional oral steroid pulse, it would be huge!!!   All in all, it was a very good visit.

Halter monitor is done and now we wait and see if there is anything exciting to hear.

As I have already shared, unfortunately, Chance has lost more weight. I don't even know what to say about that.  We are more than a little bummed.  And concerned.

We saw our GI nurse yesterday and while I'd hoped maybe, somehow, the weight loss we had seen the day before at the pulmo's would have been a fluke, and more importantly, wrong, it wasn't.  He really is down 4 pounds since his last weight check. Blech!!!  Plan is to finish looking at other organ systems at this point, which means we have his heart left to look at. At this moment, he's scheduled for an ECHO on the 17th, but after talking to our pediatrician's office today, we are trying to go ahead and get him seen while we are here this week.  We shall see.

In the midst of all this, we have had the chance to spend time with people we adore, and that so makes your heart smile even when things are less than ideal.  Several of our friends have been at RMH with us, which always makes the "down time" funner when we are hanging around the house.  We were able to spend the evening with the Andrew's, eating, laughing, and watching the girls have a BALL with their bestest girlfriend, Thing 9. We were able to spend an evening with the Parker's and were again reminded how freaky it is to have our twin family down here.  Chance was able to spend the day visiting with Rachel, and the girls and I were able to meet up and spend the evening with the whole Adam's family, which always does the soul good.  And we had a lovely brunch with Aunt Gayle, Uncle John, Margie, Ashton and precious Livi Lou!!!  We discovered an amazing new dip we picked up at Bucee's on our way down, discovered an awesome place to buy pre-made gourmet food that is to DIE for, and ventured out long enough today to get SmashBurger's since we were all craving them!!!  So we have been eating well too....LOL.

Unfortunately, the Mito Fun Walk was cancelled for tomorrow, meaning our easy access to see all our mito friends at once isn't happening, so hopefully we will get the chance to see a few others while we are here.  Plans are slowly but surely being made to keep us busy until we finally get to head back home.

The kids are tired, but the down evening and day today have done them some good.  Abby had a rough tummy & head evening tonight, but I am hoping she is feeling tons better when she wakes in the morning.  Some more downtime tomorrow will be good for them.

Late at night, like it is right now, is when I miss home the most.  During the day your distracted, but when it gets quiet, all are in bed asleep, and there aren't the distractions, the being away from home blues tend to hit.  As always, Michael is holding down the fort well, and while CoCo acts as though she is miserable, I know she is happy to have had him home so much this week.  I am blessed.....more than I can say....to not have to worry about things there.  It's just hard to be away so much, and when the days turn into more than a week, well......

Hope all our friends and family in the snowy tundra of North Texas are staying warm and well.  We miss you....just not the cold that is freezing your tushies off<grin>.

{Medical Monday: Houston Bound}

Off on another wild adventure for the McNair’s!!  We head down today for the week and will get at least 8 appointments in while there…..actually a “light” week for us!! All three see Pulmonology on Tuesday, Madison will see her rheumatologist and be hooked up for a 24 hour halter monitor on Wednesday, and Chance will see the motility GI nurse and the girls will get sleep studies done on Thursday.  Easy peasy…easy peasy.

Prayer requests for the week…..

That our pulmonologist is not too annoyed with me that we have still not started oxygen with Chance & Madison.  There are a hundred excuses…..not one good enough….but I will spare you all.  If I am honest, it’s mostly me struggling with the idea.  I am still trying to wrap my mind around it.  Reality is though, it might make a huge difference for the kids and I just need to get over myself and help them go into it with the right frame of mind.

That our rheumatologist finds a decrease in inflammation in Madison’s knee’s, indicating that the steroid injections have worked for now.  We are almost 2 months out since they were done, so we should be able to tell where things stand.  If they have not reduced the inflammation, we will be faced with starting another medication to gain control, and no one likes the idea of starting something that will make her even more susceptible to infection.

On the halter monitor we’d actually like to see her have one of her heart-beating-hard episodes in hopes that we can see what might be happening during those times.  At the same time, I would be thrilled if it shows nothing and we are able to walk away with good news.

That Chance will have gained (even just a little) weight. 

And finally, that the sleep studies give us the information that we need to make adjustments to Madison’s Bipap, and that either there is a clear cut reason for Abby’s fatigue that are sleep related (with a correlating easy fix please) or that her sleep study looks pristine.

Some of these answers will come while we are there this week, while the rest will trickle in over the following week or so.  I will keep you posted.

Having a “light” week means we will get some good time to spend with family and friends and we are looking forward to it SO MUCH!!  We will no doubt be entertained, loved on, and very cared for while we are there.  Sure does make going down so much easier to do.  Cannot even imagine making it down like we do without that blessing.

In other medical news….

Chance did at least maintain his weight at his last weight check.  While we would have liked to see even a small gain considering the insanely high number of calories he’s taking in daily, no loss is good too.  We will see how his weight looks this week, then he’ll have another weight check on the 10th at the peds office.  With the lack of gain, he now needs to more consistently get in 3 supplemental drinks a day, and we are going into the next plan of action…..checking other organ systems in case they are stressed and consuming more calories.  We will see pulmo this week and will hope that perhaps the o2 at night might make a difference, and will do an ECHO to check his heart when we are back in Houston on the 17th.  We will talk with the Motility GI nurse this week on what the next steps will be if neither of these systems appear to be the problem.

Otherwise, the boy is doing pretty well right now!!!

Madison is having some “female problems” that I won’t share here and we need some prayers that we can find some solution for.  She’s worn out dealing with it and really, really needs a break.

She is <knock on wood>  14 days out since her last antibiotic and is staying infection free thus far!!  We are still in that 21 day window that she likes to come up with another one, so not quite out of the woods, but getting there.  Sincerely praying she can get a break for a while.  We will re-test her Mycoplasma levels on the 14th and would very much like to see those numbers come back not showing something chronic.

Fatigue is still pretty intense, but 2 weeks of no infection means I am seeing at least a little improvement. 

Abby started Florinef about a week ago to help with her low blood pressures and at least thus far, we are not seeing any negative side effects to it.  Not sure how much positive we are seeing yet, but watching for them.  Hope is that it will lower her heart rate and stop the postural dizzy spells she deals with daily.

Otherwise she is really behaving herself right now!!  Such a good girl<grin>.

Please keep our little friend Cooper in your thoughts and prayers right now.  He is, quite literally, fighting for his life and he needs comfort, as do his family and friends.

Thanks so much for the thoughts and prayers this week!!!  It’s been more of a trial than usual to get everything in place and taken care of this time, but all appears to be on the right track now and we should have a productive week.