Houston Part I: Medical

Overall, it was a light week without too many revelations. 

However, it’s never quite that easy…..while true that we didn’t have too many appointments, there seem to be so few anymore that are just “routine”, for lack of a better word.  Even if one, or even two of the kids seen alone might produce a “routine” appointment, there is always one that takes it in another direction.  And while nothing discussed was a “revelation”, it doesn’t mean there are not changes that are emotionally difficult to swallow.

On some level, I loathe writing this post.  I need to….for me as much as anyone else….but what I really want to do is write about the fun stuff from the week.  The next installment will be nothing but FUN….this I can guarantee!!

We started the week with Madison still trying to recover from her gut shut down of the week before, which has been a slower process than I would have expected.  Although, considering how run down she has been, for so long now, I guess nothing should surprise me anymore. 

While we have had oxygen sitting in the house for a couple of weeks now, because we are still lacking the pulse ox machines, we’d been waiting to get started.  However, last Friday night Madison was having a lot of shortness of breath at bedtime, and since we’d heard from Pulmonology that we could go ahead and start while we wait for the pulse ox machines, I offered her oxygen for the night to see if it would help.  And it did.  We’d continued with her over the weekend with plans to get the other two started when we got to Houston.  In preparation, I had gone ahead and filled our portable containers to take with us, knowing we might need to get through a night before the O2 company could deliver what we would need for the week.

When we made our usual pit stop at Buc-ee’s, I noted that Madison was not looking so hot and offered to get the O2 and let her wear it the last hour and a half till we got to my Aunt & Uncle’s house, which she immediately agreed to.  On the one hand, I wasn’t pleased that she was feeling cruddy enough to be willing to wear it….on the other hand, I am pleased that she is already feeling a positive affect.  Always a mixed bag.

We were not able to get a room at RMH for our first night…..something that’s only happened one other time, but is the risk we take.  Thankfully it meant we got an evening with my aunt and uncle with some time to visit, making the delay in getting in to RMH thoroughly enjoyable!!  At least for the first night.  We were hopeful we might get in on Tuesday though, especially now that we need to have O2 delivered and not knowing where your going to be makes that a bit more complicated.

On Tuesday, Chance saw our pulmonologist.  I honestly didn’t think there would be all that much to talk about since we were just getting started on the O2, but for the second time as of late, when they did the spot check of his O2 sats there in the office, he was sitting at 96 for an extended period of time.  96 isn’t bad by anyone’s standards, but IS different for him, and a little lower than the pulmo wants him sitting at.  Had this been the first time I had seen it, I might have blown it off, but that it was the second time in a row & I suspected it might be more accurate than I had given it credit for the first time. 

As I have said before, we really love our pulmo.  She was, as always, very attentive….and more, she wants to know everything that is going on when she sees the kids, not just the breathing related issues.  While it makes for a longer appointment, it is always good to have as many smart people as possible thinking about what we are seeing.

In light of the sats we were seeing during the spot check, she hooked him up while we visited to see what was going on.  Part of me expected it to be fine, now that we were actually looking at it, but sure enough, she hooked him up and he just sat at 96.  She had him sit up and while we were able to get him to pop up to 98 for a little bit, after a few minutes he dropped back to 96 and just sat there for the rest of the appointment.

In light of this, our O2 plans have changed a bit.  She wants me to spot check him during the day and if he is sitting below 97, she would like us to supplement with O2 and see if we can get him consistently satting higher.  We will keep her posted as we start being able to monitor with the pulse ox, both night and day, and will see her again in August to go over how it’s all working out.

Unfortunately, we did not get into RMH on Tuesday, but with early morning appointments the next morning, staying anywhere but close by the medical district didn’t make sense.  We were able to find a hotel room not too far away that worked out okay…..and I was able to go ahead and get O2 delivered there. 

Wednesday morning, all three kids were seen by our immunologist.  For Chance & Abby, the appointment was quite routine.  Other than a few changes in Abby’s meds to deal with the sinus issues she has had this year, there really wasn’t much else to talk about.  For Miss Madison, the big news was that she HAD been able to build titers to the vaccine we had given her in April.  This is good news in that it means she has all the parts to her immune system to do what needs to be done……and at the same time, was bad news in the sense that clearly her immune system is not working right (all the right parts, or not), and this took any hope of having a treatment that might help support her immune system off the table.  It was not a shock, and I had already started wrapping my mind around our alternative….we can only support her as much as possible, as quickly and aggressively as possible, when she starts showing signs of infection.  Already working on it.

While there, we had a nice visit from 2 of the ladies working in our mito specialists office, both there to talk to us about some fun things they are working on, and needed our input and the kids artistic abilities for.  When all was said and done, our immunologist being behind schedule turned out to be a good thing, and we had plenty of time to visit and work on our project.  Always nice when things work out that way!!

Thankfully, very shortly before we needed to make a decision about staying in the hotel for another night, we received word that there was a room available for us at RMH.  I had no idea how discombobulated it would make me to be in limbo that far into the week.  No doubt in part because this was also the first time we were dealing with the O2, but I was exhausted and out of sorts by this time, and never so happy to get settled somewhere familiar with people we know care about us!!  Even when that means re-packing (at the speed of light so we could check out on time), and unpacking AGAIN.  It was, perhaps, a good reminder of how blessed we are to have this home away from home….not that I ever forget it!!

Bright and (far too) early Thursday morning, Chance and I checked in to the hospital for a 23 hour video EEG.  Chance has been having some episodes that seem seizure-like and so the EEG was ordered to see if we could catch something to give us some clues.  It’s always unclear whether you’ll catch something while your there, but as often as he has been having these episodes, I was more optimistic than usual. He did have a mild one during the strobe light/hypoventilation portion of the test, and it was noted, but otherwise we were a bit jinxed and he managed to make it the whole time without another one.  In the end, it may or may not show something. 

My mom came over at about 11:30am to take over the bedside vigil so I could take the girls to see our pulmonologist for their appointment.  Like for Chance’s appointment, I didn’t anticipate having all that much to talk about since we had just started the O2, and for the most part, this held true.  Madison though was looking horrible by the time we got there, and did nothing by lay on the table, essentially refusing to interact.  VERY unlike my girl, especially when we are seeing this doctor whom she loves to chat with.  With all we have been seeing, while using O2 during the day with Madison had been discussed as something we might need to do, the pulmo talked with Madison and I both about the need for it considering how she is doing right now.  While Madison is still insistent she doesn’t want to wear it when other people can see her, she agreed to use it otherwise.  The only other change we are making is to try a nose mask for Abby since the canula is bothering her.  It has a kitty cat nose & whiskers on it, so Abby thinks it’s adorable!!  In a very positive turn, both girls were already feeling a positive effect of using the O2.  Considering we were away from home and all the stress that comes with that, I honestly wasn’t sure if we’d be able to tell whether there was anything positive with it or not, so as much as my heart wishes it wasn’t needed, I am pleased to see it’s already making a difference.  I am anxious to see how they are doing after a week here at home on it!!

Mom and I switched out for the night so I could be there with Chance until he was done Friday morning.  Friday was spent taking care of some loose ends that needed our attention while we were there, ordering one more night of O2, and taking Abby over for labs.

Madison is still struggling.  She’s swelled back up again, not quite as badly as she was a few weeks ago, but only barely better.  She’s exhausted & cranky & just generally not feeling well.  Whether she’s still recovering from the gut shut down, and last week just added to her already stressed system, or if she is brewing something new isn’t clear yet.  I don’t even know which one I am hoping for!!!  Watching her closely and hoping there is a clear indication when/if we need to support her more than we already are here at home.

Next installment…..FUN!!  Oh how very grateful we are that even in the midst of all this stuff, there is fun to share.

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!!

Yes Dear, It Does Suck

While there was a time when I believed that it was very hard to raise an emotionally intact child with a chronic, degenerative disease…..and still do…..I am coming to the realization that it has never been harder than it is right now, when they are all teenagers.  So many things come into play….hormones, a higher level of understanding and recognition, thoughts of life AFTER being a kid living with their parents at home, AKA “what I want to be & do when I grow up”, and the list goes on. 

To further complicate matters, there is the ever-present DIFFERENT ways that all three deal with things.  It’s ALWAYS been there and it’s something I have pondered on more than one occasion….just how amazing it is that three children, all raised in the same home, with the same rules and parental involvement, with the same level of comfort, can somehow approach life so very differently.  It certainly goes to the whole “nature vs nurture” question, and proves, on some level, that our nature is just there and only partially influenced by the nurturing aspect.

That is NOT to diminish the effects of nurturing, don’t get me wrong, just shows that even nurtured the same, different people react differently to their situations.

In this house, I somehow have children that cover the entire range of possible reactions to the exceptional life we have been given.  And I mean, I have one on one extreme, one on the other extreme, and one who would likely fall somewhere right in the middle, at least most of the time.  The reality is it changes from time to time, or one moves into another’s position for a short time, but mostly, they do their own thing.  Mostly they make their mom seriously stay on her toes!!!

Madison is my extremely (almost abnormally) stoic child.  She tolerates it all, doesn’t stress about what’s going on (at least not outwardly and swears not inwardly either), responds to bad news with a total lack of surprise (I am Madi, what do we expect), and she has ALWAYS been like this, even as a small child.  We could dig for a vein, and poke her multiple times to place an IV and get little more than an occasional ouch.  In some ways, she rides this ride with absolute grace, and we could all hope to be as at peace as she seems to be.

Then there is my middle of the road child….at least most of the time….Chance.  Chance will complain and be unhappy about things, but has always very quickly come to peace with it, accepted it, and moved on.  There has always been too much going on in his head for something like medical crap to take up any of that precious space. He does though sometimes react emotionally to what’s going on, and generally speaking it’s when he comes to revelations about what something or another really means.  It’s rare, can be intense, but is generally short lived.

Then there is Abby.  My child who has done absolutely everything else opposite of how her siblings have, continues that tradition into adolescence.  Abby has always been an emotional child who wears it all on her sleeve.  You know immediately when she doesn’t like something being discussed…..she makes it clear that talking about anything medical, even when not involving her, upsets her…..she avoids watching medical shows on TV…..and not surprisingly, she deals with more anxiety, frustrations, and fears in general.  It is almost always intense with her, even when it’s something our crew considers rather minor with the perspective that we have.

What further makes this interesting to me is that this reaction each has is, in some ways, the opposite of what I would expect based on the level of intervention needed for each.  So while Madison is my most stoic, she is the one that endures the most regularly….while Abby is my most reactive, she endures the least.  And Chance is somewhere in the middle.  Of course, living it, I get that in some ways this is exactly the reaction I should expect from each based on their level of intervention.  Although, honestly, I am not sure we would have seen anything different had their roles been reversed.  It’s their personality, their make up, that lends itself to these responses.

Generally, just as I think I have some handle on it, the kids will change it up a bit and make me stop in my tracks.  A couple of weeks ago Madison came to me out of no where, wanting to know if she could take one more night a month off her BIPAP.  As it is, to maintain her sanity, we made the accommodation that she could take one night off a week.  She needed that night to look forward to…she needed a break.  While theoretically the BIPAP is helping her overnight, we have not been so fortunate to SEE a real difference, or for her to FEEL one.  There are multiple factors contributing to that, ones I have no control over (no one does), and when your 14 and HATE something, sometimes a compromise has to take place.  Looking back, I wish I had compromised initially with a night off a month, or every two weeks, but when faced with a stoic child that is absolutely distraught, you sometimes compromise more than you should. Anyway…..Madison had asked me about it one day and I told her I honestly did not have the time to discuss it at that time.  It was honest, but it was also so I had a chance to ponder the proposition. 

After a busy Saturday, a very worn out, tired Madison came to me Sunday evening to ask again.  I’d had some time to think and had come to the conclusion that I really could not, in good conscious, okay this latest compromise.  For one, she’s already off the vent 4 nights a month, which is likely more than is best.  Two, she rarely ever keeps it on all night long any way, taking it off in the dead of sleep completely unaware why or that she is doing it.  While she generally is at least getting 6 hours on, when we know she struggles the most in REM sleep, and we know that happens mostly in the early morning hours, that she is off the vent at that critical time is not good.  Finally, I really needed the doctors okay AND for Madison to not be in a tired, emotional place while making this decision.

Madison’s response was anything but expected.  My very stoic child who rarely complains and never cries, just sobbed.  And sobbed some more.  It was heart breaking, but I really couldn’t give in on this one.  Not at this time.  After a while of begging then sobbing, then begging again, she finally started to calm down.  She calmed down because, what little energy she had after a busy Saturday, was pretty much exhausted and you could see it in her face.  And just when I thought it was over (for the evening at least), she burst into tears again!!  When she finally calmed enough to speak, she explained that she had just realized it was Sunday night….our weekly Methotrexate shot night.

 “For the record Mom, I hate my shots too!!”

Very thankfully, this was something I could give in on…..it could wait till the next morning if need be.  She took the offer quickly and started calming down again.  My heart hurt so badly during it all, and all night long.  Yes Dear, it does suck.  Really, really sucks.

True to form, the following morning I asked Madison how she was.  She said she was “tired” which prompted me to say I was asking more how she was EMOTIONALLY doing….”Oh….I’m fine.”  And she really was.

Sigh.  I’d be whining, complaining and sobbing ALL the time if I had half the stuff she deals with going on, particularly when she has generally felt cruddy for a LONG, LONG time now.  But, that wouldn’t be living life, so I am very grateful she mostly can handle it all so well.

Several things have come to light as of late when it comes to Abby.  The first is, in her zealous attempt to not even allow a medical thought to enter her mind, she is ignoring a lot that’s going on with her body.  Things I need to know so we can do something to help.  Things that left untreated could cause some serious problems down the road.  I love that she can ignore things so well from the LIVING standpoint, but know that ignoring things can sometimes just make everything worse.  She still needs some guidance on when it’s something okay to ignore, and when it’s just not.  But that requires a LOT more conversation between the two of us.  She’s working on it….trying….and that’s all I can ask.  Abby will NEVER be my one to tell me it all, and she will ALWAYS keep it all “out of sight, out of mind” as much as possible.  We just need to find a happy median.  Someday I won’t be there to tell the doctors what all has been going on, and at this point, she’d tell them all she’s perfect if she could get away with it.

While I have come to the realization the last year that Abby is a tad bit ignored medically, her desire to ignore it all has contributed and I am realizing that more and more.  We both just need some work on this.

Of a more pressing nature, Abby goes through some serious cranky periods on a pretty regular basis.  And she can be seriously CRANKY!!  She tends to get herself into trouble at these times, and it’s only once she’s being griped at/punished for unacceptable behavior that she shares how tired she is, or how bad she feels.  There are several issues with this scenario……once in trouble, to then say you feel like crap feels like an excuse.  The thing is, I rarely question that it is what’s going on, however, it’s at that point too late for me to really back down.  I always tell the kids, feeling bad or not, if you choose to behave badly, there will be a consequence.  Second, I WANT to comfort Abby when she is feeling cruddy.  I WOULD comfort her, but she has always been one to act out rather than ask for some loving.  It hurts my heart though because I so badly don’t want to be griping at my child when what she really needs is her mommy to take care of her.  Finally, as I told Abby, I can see it now….she will be in her early 20’s and will, likely in the heat of some argument, tell me that all I ever did was gripe at her when she was feeling bad.  That thought KILLS me.  But as I told her, in that moment I pray I can remember that the way it really plays out is…..

She never tells me she feels bad TILL I am griping at her.

I wish it was obvious….I wish I knew when it was Abby being a stink and when she doesn’t feel good.  I want to comfort her….I want to empathize with how cranky you feel when your feeling bad…..I want to tell her siblings to be extra nice to her that day….I want to offer some extra hugs and maybe some snuggles.  But I just cannot tell usually, and she’s not real forthcoming in this area.  I am glad she doesn’t dwell on it, but I need to know sometimes.  Just need to.

We are working on a plan for both.

Madison is patiently waiting for her upcoming appointment with the Sleep Doctor/Pulmonologist where I have told her she can talk with the doctor about taking another night off.  I really don’t know what the doctor will say under the circumstances.  At some point we will not be able to reduce her time any more, and there is a very real possibility she needs MORE time, especially with how much she is struggling right now.  I wish I had some way to make this better for her.  I am incredibly proud of how well she has tolerated it.

She and I are both praying her arthritis stays stable between now and August.  Our rheumatologist has said that if we can keep her stable, with no more joints involved, we can start trying to get her off the Methotrexate injections.  She cannot wait!!  And I just pray daily she is not disappointed if things don’t go like we would like for them to.

We will be getting Abby a small journal where she can make notes about how she is feeling, particularly at times when she doesn’t feel like letting me know.  If nothing else, at least then we’ll have a better resource for determining how much certain issues are occurring.  Her perception of how much something is happening is very off, most likely because she so badly just wants to ignore it.  I have told her that she can write it down and forget it till the time comes we need to discuss it, but at least this way we know we are accurately conveying the situation to the doctors.

Personally, I am working on new ways to work with Abby and meet her more where she is.  I HATE punishing her not knowing pain and/or exhaustion is playing a part.  There is still a consequence to our actions, but it does need to be tempered sometimes based on what all is going on.  Mostly though, I just want to love on her more when she is feeling cruddy.

We will, no doubt, make the best of it when all is said and done but, sometimes, it really does just suck.

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.