New “Normals”

It occurred to me yesterday when we went to the dentist office for the girls to get their teeth cleaned, that while we have adjusted and accepted some new “normals” here at home….have adjusted to the looks when we are out in public….for those that love us, some forewarning of these “new normals” is in order.  Our poor dentist!!!

A few weeks back, Madison’s nausea returned with a vengeance.  It was constantly there, would escalate for periods of time, then go back to the constant level.  We have been here before…it’s miserable and we started seeing some of the spunk we’d finally gotten back into her start to slip away.  None of us can stand by and watch her go back to where she was before, so with some help, a plan was devised, and Madi signed off on it.

For now at least, Madison is on partial gut rest.  What that means is that she is eating mostly soft, easy to digest foods, with an occasional solid food thrown in (when it’s just too enticing to pass up), and otherwise she’s getting Boost (formula) and fluids through her g-tube to make up for what she’s missing.  This means she’s connected to her feeding pump most of the time in order to get in what she needs.  This means she has another piece of equipment she’s lugging around with her.

This, most importantly, means she is FEELING BETTER!!

I’ve suspected for some time, based on times when she wasn’t able to eat normal food for a few days, that digesting solid food took a ton of energy for her.  There was no question that when her system wasn’t having to work so hard, her overall energy was better.  But while we had discussed the possibility of trying it, particularly the last two years when she has been so low energy, SHE had to want to do it, and who really wants to give up eating??!!

The difference is, bitter sweetly, amazing.

I am still praying that tomorrow, or next week, or next month something will change.  Somehow her system will get better and she’ll be able to eat “normally” again.  In the meantime though, I am going to embrace this new normal like she has, and celebrate the fact that her spunk is returning. 

A little more on the “spunk returning” I have referenced.  It’s still day to day, and one good day can still sometimes mean a few days of being lower energy and tired, but overall, she’s tons better than where she was in early August.  This baseline I cursed last summer because it was so much lower than where we had been the summer before, well, I am THRILLED to see this time.  I marvel at how much better a period of time can look when things progress to a worse place.  Our girl is still not where she was, but she’s living, and smiling, and participating, and laughing….we can live in this new normal. 

So, if you see my girl sometime soon, I hope you can look past the feeding pump & tubing, the Oxygen tank and nasal canula, and the wheelchair (she may or may not be in), and just see that smile and that sparkle in her eyes.  The attachments are less than ideal, and certainly make a statement we’ve not experienced making before, but for what it’s all giving her…..her life back…..we are happy to have them along for the ride.

Mish-Mash

Spirited

Not actually a word that I’d normally associate with Madison….her sister, yes….but Madison’s qualities have not usually lended themselves to that term.  Madison is my “old soul”, cool, calm and collected kiddo.  But right now….right now the term “spirited” feels right.

Seattle/Camp Korey restored my girl to me.

I knew….we all knew…..she was not doing well before heading to Seattle.  Her energy levels were at an all time low, she was declining to do things she typically loves to do, and, I realize now, her spirit was just not there.  She wasn’t smiling like usual, wasn’t laughing, just was not herself.  Her doctors that see her regularly were noting it too….the usually chatty Madison was laying on the exam tables, half asleep, not interacting at all.

Sometimes you don’t realize how bad things are until after the fact.

What it is about going to Seattle that does this for my girl I cannot say for sure.  The cooler weather no doubt plays a part, and forces us to face the reality that summer’s in Texas are so very hard on her (on all the kids, but especially her).   The air quality is better, and that likely plays a part.  Perhaps Camp Korey just has a healing power that we may never understand.  Whatever the reason, the fact that my kids improve so much while we are there is one of the biggest blessings that comes from our ability to be there every summer.

We watched as Madison “woke up” once we got there!!  And so far, she’s maintaining some of that energy.  Meanwhile, I & her doctors are realizing just how down she was before we left, and rejoicing in seeing her with a bit of spunk.  Mostly, it’s the smiles that make my heart happy. 

My girl is smiling again.

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Houston Trip #4

I am also rejoicing in the fact that we have actually managed to stick to our schedule for Houston this year!!  After two years of being down there 12 times each year, I needed to find the schedule that had the potential to work, and going every other month seems to have been the ticket.  We have NOT had to return unexpectedly at all this year so far (hoping this didn’t just jinx it!!)!!

We did have to head back down a short 6 days after getting back from Seattle, and while I would have loved to postpone it, I realized we just had too many important appointments and tests to get away with it.

Overall, things went well.  We got some additional testing done that was needed, saw a few of the kids doctors, and made some changes that we are all hopeful will improve quality of life for all three. 

We also were able to spend some time with our family and friends while we were there, and that ALWAYS does a heart good!!

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Adjusting

I am, slowly but surely, adjusting to having oxygen in the house and our new routine.  All three are wearing it at night, and Madison is wearing it most of the time during the day too.  This could explain some of her improvements as of late, and she’s dealing well with wearing it in public.  It’s not quite old hat to me yet, but we are getting there.

New med routines are getting easier too.  As soon as I say that, we are making changes again, but we are making the adjustment pretty well.  Just need some time and we will all get it better.

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Disjointed

And all over the place tonight.  We have some big (non medical) changes happening in our lives right now, and it has me stressed out, and in my world, stress = a lack of creativity!!  In other words, I tend to ramble!!!!

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Mito Awareness Week

September 18-24 is Mito Awareness Week and the kids and I are working on some plans for spreading the word about Mitochondrial Disease all this month.  Watch for it here and on Facebook!!

Check out the piece that ran on Channel 8 here tonight about some friends of ours that are making a difference and getting the word out!!

http://www.wfaa.com/news/health/Teen-raising-profile-of-little-known-disease-128652233.html

Way to go Delaney!!!

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Finally, Some Prayers

For our precious friends, Ali & Alex, who are in California as I type, starting the process of getting Alex on the study drug I’ve written about before.

For the McNeil’s as they prepare for Gary to get his new kidney.

For the Andrew’s as TJ fights a line infection.

For us for wisdom as we make some big decisions, faith that we are heading in the right direction, and peace in it all.

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.

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.