Houston Part II: Extended Family

One of the many blessings that is revealing itself with the increased control over our visits to Houston is that we are not pushing as hard as we were before.  We are not generally sitting in doctor’s offices ALL day, then attempting to “squeeze” the very important fun times in.  As much as my better judgment at times would say we needed to stay in and rest, the very real need to make the fun memories too would often overpower the logical part of my brain (and heart).  With the new schedule, the fun times are not at the cost of needed rest like they were before!!  SO much better!!!

As I mentioned in my last post, Monday night we were blessed to have beds to sleep in at my Aunt and Uncles house.  We had a lovely meal, and more, we had some good quality time to visit and love on them.  Never can get too much of that!!!  They graciously fed us a lovely breakfast the next morning before we were off to start our day.

I also mentioned in the last post that I was discombobulated this trip down, and while this was mostly related to not quite knowing where we were sleeping from night to night, and unpacking and re-packing more than I ever want to again, there was one other reason I was a bit out of sorts….a good reason!!

My precious child, Madison, was planning a surprise for me for my birthday Tuesday night.  Little did I know at the time, but she’d actually been working on it since we were in Houston in April, when she found out that we would be there over my birthday again this year.  She and my precious friend, Jeanne, were working out the when’s and where’s, but it was ALL on Madison to then contact the other people she wanted to be there for my special evening.  She, quite literally, stressed herself out getting it all arranged, then dealing with the fact that we were not where we thought we would be Tuesday night!!

Madi, Jeanne, Kyla & Beth

In the end, it all worked out beautifully!!!  I was dressed from head to toe in items my precious family had gotten for me and felt like a million dollars…I was directed to get us to RMH where the meet up would take place…..and there, one by one, some of my most precious friends came through the door to help celebrate my special day!!!  They were all there to take ME…little ol me….out for a special dinner!!!

I will simply say Perry’s Steakhouse is amazing!!!!  There we met up with my cousin, and while two other people I love dearly were not able to make it, they were definitely there in spirit!!! It was an incredible evening, and I am pretty sure that had someone been looking in on our table, they would never have guessed that prior to this evening not a single one of these precious ladies had ever met before!!!  It made my heart swell seeing these people that I love, start loving on each other too.

Margie, Jeanne, Kyla & Beth

My precious Jeanne

Miss Madison put her heart and soul into this surprise and it could not have been any sweeter!!!! 

Wednesday we finally got in and settled at RMH and I started feeling a bit less out of sorts thankfully!!!  We had some down time after our appointments that morning that we took advantage of, spending some time loving on our RMH family, and then headed over to visit with Jeanne and her precious girls.  As always, we walked in their apartment and one would think we spend every day over there.  There is a comfort that we have with this family, and no matter how long it’s been since we have had the chance to see them, we just pick back up like no time has elapsed at all.  It’s perfectly lovely, and so awesomely real.  We had a wonderful time visiting with them and having dinner.

Thursday evening, we were without the boy & my mom who were both stuck at the hospital.  After finishing up our appointments and errands for the day, the girls and I were in need of a little more time with the female portion of the Andrew’s family!!  Beth & Madison were gracious enough to come back over to our part of town and have dinner with the girls and I, getting me back to the hospital for the night just in time to catch the end of game 5 of the NBA play offs with my Mavs loving son!!  Another family we are just so blessed to just mesh with in a really incredible way!!

Finally, Friday afternoon part of us were off to yet another family we have become an extension of (Madi wasn’t feeling good and stayed back with my mom)……

How incredible is it that we have this many families that we feel this kind of belonging to!!??

The Parker’s welcomed us into their home with open arms as usual, and we picked right back up where we left off last time….just so very cool.  Sean and Chance were almost immediately playing music together….we had the chance to congratulate Lauren on her graduation and give her a little token from each of us that we hope brightens her day when she sees them…..Abby & Skye were off exploring who knows what….and Teion and I were immediately in conversation about anything & everything, over glasses of wine.  We were further blessed on this occasion to meet another family that the Parker’s love, and had an amazing time getting to know them!!!

We are SO very blessed to have this amazing extended family.  I cannot imagine our time in Houston without them there.  They rejuvenate me when I need it the most…..and though we come home weary, at the same time, we come home feeling all warm & fuzzy, and so very loved.

Family….friends…..THANK YOU!!!  We love you all so very very much!!!

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.

Tortured Hope

Having a disease that has no real treatment, much less cure, I have chosen the route of finding hope in the things there is some control over. 

I hope for a good medical team, for treatments for each system involved that is effective with the least amount of side effects, and for the wisdom to know how far to let the kids push so we have more good days than bad.  I hope for cool days that allow us the chance to spend time getting some fresh air, for the emotional strength we all need to continue to function, and for maintaining the ability to not take a single day, a single moment for granted.

Ten plus years into this journey, having so little on the horizon in terms of direct mitochondrial treatment or cure, for fear of being brought down by that reality, I have not allowed myself to hope for a real treatment for our family.  I needed to hang on to the things that seemed plausible, at least in the foreseeable future, just for my sanity’s sake.  I pray for the treatment and cure daily……just haven’t allowed myself the option of spending any real time considering hope for it.

I suspect some would consider this faith related, and I suppose in some way it is.  Perhaps, if my faith was stronger, I could hope for that thing that seems so unreachable.  Perhaps I could hope without feeling the pain of disappointment when something doesn’t come to be.  I really don’t know…..I only know how I have managed thus far and have feared allowing myself to “go there”.

A little while back the mito community at large learned that there was a new treatment being tested that could be promising.  However, because the study group was so small, and exclusively a type of mito we did not have, I allowed myself to follow the progress, while tempering my response to it.  The truth is, very little was leaking out, so I could mostly ignore the possibility.  It was not the first time something was thought to have potential, and thus far none of the others have panned out, so it wasn’t something I was getting even a little excited about.

This last week though, we heard that the study was being opened to a broader group of mitochondrial disease patients. The FDA is allowing a faster track since thus far the results have been positive with little to no side effects and mito is thought to effect less than 200,000 people.  This qualifies it for an expedited process during the study phase.

With this news, I realized more than ever, how difficult it is for me to allow that hope to seep in.  I read the news release, read what others were saying about it, and honestly, just sat on it.  I didn’t go and see if I could get more information that might be out there on the interwebs….I just let it sit there for a couple of days, really not sure how I felt about it.  At that point, the qualifications for this new part of the study just weren’t clear, so it was unknown whether we would qualify even with a broadened scope.

Then, I received an email from our mito doc, along with a lot of others in her care.  After a review of the requirements for the study, she felt like we might be interested in participating in the study and shared the contact information needed to proceed. 

I sat there. 

Knowing that I could neither ignore that this was a possibility, nor that I might need to get out of my comfort zone with this whole hope thing. 

Then, and only then, I allowed myself to do some googling to see what I could find about the results of this medication.  There isn’t a ton out there, but what I could find was PROMISING!!  Two groups have been a part of the study…a small group of Leigh’s patients, and an even smaller group of people with LHON (the type of mito associated with the mutations that we have).  Brain changes that are associated with Leigh’s, that were thought to be irreversible, are improving.  Vision loss associated with LHON, also thought to be irreversible, has been improved and even fully restored with this medication.  Wow.

Shoot…..and hooray!!  Something…..something might be out there that could work.  No way to ignore it, or stay away from having some hope.  I had to take a moment to think we might have a real treatment…..at some point…..and that’s the part that makes me feel tortured.

The reality is, the requirements for this expanded part of the study just were not clear.  Sure enough, after the initial email from our mito doctor, another one came out.  This one explained that they had received further information about the requirements and we should let them know if we were interested and they would review our cases and let us know if the study is something we qualify for. 

In that moment, if I am honest, I was mad at myself.  Mad that I had allowed myself to look into it more…..mad that I now know it COULD make a difference for my children, but may well be out of our reach…..mad that I cannot go back and undo what I have already done…allowing myself to hope.

On some level, I know this is a really messed up way of looking at this.  I really do want to hope for something that could make a real difference for my children.  I am just so scared to hope and have it fall through.

Ultimately, I had allowed myself to “go there” and even though I tried to do it carefully, it really doesn’t work that way.  Once “there”, there isn’t really any going back.

As more information comes out, it does not appear that we would qualify for the broadened scope of the study at this time.  They are, as the logical side of myself knows they should be, limiting this extension to patients that are within 90 days of end of life care.  There is no doubt these are the folks that need to be included in this study.  Further, it appears that only 50 more will be accepted into the study. FIFTY. There are an estimated 60,000 people with this disease…..and only 50 more will get in.  The odds of making it in are so very small, for anyone. 

I WILL feel blessed that we don’t qualify right now.  Not when that’s the criteria that’s being looked at.

I WILL either find a way to not feel so tortured, or I will find a place to put that tortured feeling so it doesn’t consume me.

And I WILL allow myself to feel hopeful….it just has to be tempered with some reality for me to be able to emotionally deal with something being just out of reach for us. Right now. Some day though, maybe, just maybe, we will have the option and you KNOW I will jump at it.

Now we just need time to be on our side.

++++++++++++++++++++++++++++++

Houston update is being worked on….posting soon.

Last, but certainly not least……MAVS WIN!!!  We have had a blast as a family watching this amazing team win the NBA Championship!!!

{Things I Love Thursday}: His Autograph

Belle
Disney World
2002

I love that when we went to Disney World for Madison's Wish trip way back in 2002, that while Chance, like the girls, collected the autographs of the characters there, he in turn thought surely they needed his autograph too!!

He shared his with every single one of them, much to their surprise and amusement. Cannot help but wonder if any of them kept that little momento.  Sure made me adore the little unique boy he was!!!

~43~

As is so often the case for me, it’s not MY age that makes me feel old, but the ages of those  around me.

Before kids, it was the ages of my sister and brother (5 & 7 years younger, respectively) on any given year.

Then I had children….and my friends had children….and as each of them have passed onto a “milestone” age, I FEEL it.

Somewhere in my mid 30’s, in between the milestone years for the kids, it became about the ages of the people I was spending time with.  To be expected I suppose when you go from being the youngest one in the group, to (sigh) always being one of the oldest.

This year, my oldest turns 18, middle turns 15, and my youngest has turned 13.  ALL three hitting milestone years this year.

This year, I am no longer in my “early” 40’s, and yet, I am not quite in my “mid” 40’s either. The 3^rd year and 7^th year in any given decade are just odd years to me.

I FEEL every moment of 43 years old too.  What that means I cannot quite explain…..and it is neither a good nor a bad thing….it just is.  But mostly, I feel very blessed.

Yep……Today I am 43.

Now, the only question is…..how many months will it take me this year to remember my age without having to think about it??

Houston Prep

First, an update on Miss Madison……looks more and more like the issue was a pseudo-obstruction that started on Sunday as there have been no other signs of illness associated since.  As of today, while still not on a normal diet, she is tolerating more and more without issue, and we’ve been able to stop regular dosing of phenergan as of this morning.  Additionally, her bladder and lower bowel have “turned back on” also.  All leaving me with a sigh of relief that this was short lived and with the help of our ped, handled exceedingly well.  Fluid intake is still presenting problems by mouth…..Madison’s swallow has been weak for a long time now and when stressed it appears the energy it takes to protect her airway becomes too much and she does better with fluids through her g-tube.  This is nothing….just hook her up every couple of hours and bolus the fluids she needs, and she’s good….and I know she’s getting what she needs without expending energy she doesn’t have.  At the rate she is improving, I expect we’ll be on a normal diet by Saturday….and then we just pray this doesn’t happen again anytime soon!!

Now back to the intended post…….

One would think after the number of years that we have been doing the medical thing, and going into our 4^th year of traveling to Houston (our third of four distant locales we have traveled to for medical care over the years), getting it all arranged, getting us there, and getting prepared for the appointments would be old hat, and dare I say, maybe even easy.  And yet, it never really does get to that point.  There are, quite simply, just too many people, which can lead to too many complications, for it to ever become easy I have decided.

I have my end pretty mainstreamed.  The kids summary letters only change here and there, as new medications or diagnosis come into the picture.  I generally have a running list I keep of the things we need to talk about with the doctors.  Both of these are generally a pretty quick copy, paste & print and we are set.  I know who to call, how to word things the right way, when to be nice, when to be forceful, and how to navigate the world we live in.

However, this world we live in doesn’t always involve others that have the same skill in these respects that I do.  They don’t understand that the 2 phone calls they had to make to me, that they are getting PAID to make, are only 2 of 20 I am taking that day…and NOT getting paid for.  They don’t understand that as complicated as it is for them to input the information for three kids at a time, that I have relayed that information 15 times that day, on all three….and am tired of hearing myself talk!!  They cannot comprehend that the “quick fax” they would like me to send is one of 10 requested that day.  I could go on and on, but you get the picture.

Mostly though, the thing that is impossible for them to “get” is that OVER being my children’s medical secretary, I am their MOM, and the time they are taking with me is taking away from that far more important role.

We head to Houston for the third time this year on Monday.  My goal for the year of getting better control over the medical end of our lives IS working considering we were there almost monthly for the last two years!!  What is not working as well as I would like is the time it’s taking to get us there, but perhaps it’s time to just accept that part and move on.  Perhaps all I can do is pray for the people I have to deal with that they can step into my shoes just a little and make the process as easy as possible for me.  Thankfully, in my own Pollyanna way, all it takes is ONE of the people going above and beyond to erase the frustration I feel for the others that cannot.  Thankfully there is almost always that one person that allows me to stay sane…LOL.

We have a relatively light week this time (another one of those goals I had that is coming along nicely)…..All three will see the pulmonologist and the immunologist, and Chance has a VEEG to get through.

Thankfully all the prep happens BEFORE we go, so once there we can focus in on our appointments, and just as important, seeing our friends and family and having some FUN!! 

{Things I Love Thursday}: Dreams

Last year we had an amazing time in Seattle. While we had done all the "touristy" things the first year we were there, this year provided us the chance to see a little more of the city, which included an excursion to Alki Beach.

The 2.5 mile stretch that is Alki Beach Park is beautiful on the one side with views of Puget Sound, the Olympic Mountains, and the flotilla of ferries, sailboats, steamships and other craft that ply Puget Sound waters.  And a mix of eclectic shops and people on the other side.

Chance had gone on to Camp, so the girls, Michael and I headed over to check it out.  We rented a 4 person bike to ride the length of the beach and in the process had a chance to take it all in.  It really is a fun place to visit.

Lots of pictures were taken.....so WHY share these two with you?

Because in this run down, old building the four of us constructed a dream.

There would be a bike shop to make Dad happy.....and a coffee shop, with a bike motif, for the girls and I to run.  We might live there, or perhaps have a bed and breakfast of sorts.  The building sits right across the street from the beach, so the potential is there.  Can you see it too??

I love dreams.....those ideas that you have, made even better when the whole family is in on it, that are dreamy, but just real enough to wonder if it isn't something you might be able to make happen.

{Word-Little Wednesday}: Eyes Wide Open

First Meeting with Wish Granters
2001

Kids in Car
2001

Chance claims the "holding eyes wide open" has something to do with Pokemon......Michael claims it was just a face the kids were fond of making.  I have NO IDEA!!!  Abby though appears to be having "issues" with accomplishing this face...LOL!! It cracks me up though every time I see it!!!