Face of hope

Face of hope
Courtesy: TIffany Kay Photography

Thursday, May 29, 2014

Music to my ears

While we wait for Meagan's shunt surgery, we have been moving right along with her therapies and daily life of course.  As I mentioned before we have been really working with her on weight bearing.  We want her legs to get stronger and now that we have her AFOs, we are able to work on that with a lot more success.  Without her AFOs, Meagan's legs are basically like a wet noodle - she cannot bear weight on her feet and her legs are very weak and wobbly.  With her AFOs, her lower leg gets total support and structure, so we can work with her on the sensation of standing and also get some pressure through her feet.... which is important so her feet grow as well. (She's still in an infant 2/3!).  She cracks me up when we work on standing, however, because of her expressions.  Sometimes she is not too happy or feels off balance because she isn't using her arms to help hold herself up - and I can understand that must feel very strange! One of her favorite things to do when I'm holding her in a standing position is to just lean her head over and rest it on whatever is closest - whether it be the couch, ottoman, chair, or just hanging it in the air.  Drama queen already I tell you!

Such drama - even with my arms wrapped around her waist 
for support...and this is what she does. 

Another area we are really encouraging is music.  As a musician/music teacher/performer myself, of course I absolutely believe in the importance of music for children from a very young age as a supplement to their learning and a vital part of their development.  However, in Meagan's case in particular, I see music as absolutely essential to her growth.  We have always sung to Meagan from infancy - when we weren't sure if she could hear, we would sing... and see her tilt her head and be interested.  When we weren't sure of her vision, we would sing to her....and see her eyes begin to track the toy or item we had been holding.  When we weren't sure of her speech, we would sing to her or play music for her - and she would suddenly be babbling and yelling right along with it.  From early on, I could tell that somehow music was speaking to her - in ways that typical speech and other developmental tools weren't reaching her.  Somehow her brain was connecting to music and producing skills that according to her scans, should be absolutely impossible to obtain.

She often wiggles her way towards me when I practice and lays there.

These active engagements in music have been very helpful in her development - most especially in her speech and expression.  If you remember this time last year, she was trying to make sounds, but we really had no speech as she approached 2.  I could tell in her eyes she was trying to communicate - and we had noise from her, but no "language" (as we know it) was emerging.  As she grew I would sing her name "Meagan Theresa" in a little sing songy phrase.  I remember one day when her usual babbling didn't occur - but instead I heard her mimic the exact melody I sang using her name.  So I sang her name to her, and she would repeat back the melody.  Before I knew it, a few months later, when I would sing her name, I would hear back, in the same sing songy melody "Megn Teeeesa."  It was then I started to really latch on to the idea of purposefully putting music in to Meagan's daily struggle with speech.

I started to sing everything to her.  What we were doing.  Where we were going.  Words. ABCs. Silly phrases.  Even things like "yes" and "no" and "go bed."  Everything was to a melody and last Fall we saw her speech start to emerge.  Several of her doctors were amazed, as the speech part of her brain was quite damaged - but one of them said to me... "this is going to sound crazy... but I think she is accessing speech through the music center of her brain."  I just smiled.  It didn't sound crazy to me at all!

Meagan playing piano her way...sans arms

So this whole year Meagan has been immersed in music whenever we can.  She is constantly sitting around when I am teaching flute lessons, or practicing for a symphony concert.  Likewise she hears her sisters practice their violin or flute and soaks in everything. She seems to enjoy the violin more than flute (seriously!?? How is that possible!? :) ).... but I think it has to do with pitch.  I think the pitch of the violin is much more comfortable for her head (sorry Megs).  Whenever Reilly practices, we typically see this:

Not totally offended though - she at least mimics playing the flute! :)

Meagan also enjoys Irish music immensely! We ask her if she wants to go see Irish dance and her little body gets extremely stiff with her arms flapping and her legs just absolutely filled with excitement they could burst and she gets a huge smile typically whispering "off you go! off you go!"  She is absolutely inundated with music from every angle and it has helped her so much.

How Meagan Irish dances

I am very thankful that we have found a "love" of Meagan's.  We ask our other kids all the time about their favorite things, or activities, or colors.... but then sometimes that can get overlooked for Meagan because she won't show us in the typical way what she likes.  You can ask her what her favorite color is and she may look at you, or say something random, or smile, or stare, or blurt out a color at random if she caught you saying the word 'color.'  Her attentiveness and ability to process is up and down and this is where we have found music extremely helpful.  Personally I really attribute music to her success in speech this year.  I happened to notice the window that worked for her - to look into her brain and reach her where we hadn't been able to before, and for that I'm very thankful.  It is these little victories with kids like Meagan that make the biggest smiles happen.  My goal for her is first and foremost happiness - and I'm glad I have found music helps contribute to making her happy because she certainly deserves it.  And that's music to my ears.

Meagan's rendition of "Do You Wanna Build A Snowman?"

Wednesday, May 28, 2014

Back on Track

As you know Meagan has been having balance and arm issues among other things that have developed over the last few months.  In the last few weeks, we have also seen strange episodes creep in where she seems extremely off, is very off balance, and acts like she is having a headache or head pain.  She will typically be very fussy and ask me to just go to bed.  Typically of course I pick her up and comfort her, but this time I grabbed my camera.  I just felt in my heart Dr. R needed to see exactly what I was talking about especially since these were now new things on top of her previous issues.  In an effort to clarify what exactly I was dealing with, I sent him this video.

How Meagan behaves during these new episodes

Upon receiving my email, I got an immediate callback from Dr. R.  He was very sympathetic and hated seeing Meagan is having days like this.  He decided then and there we do need to do more, and sooner than later.  He asked me to come down to the office so he could first turn up her shunt all the way.  It may seem crazy when Meagan seems under pressure, however, she has slit ventricles.  This is what makes SVS hard to navigate.  We don't want to put her under pressure for more discomfort, but at the same time, increasing the fluid is the only way we have to try and blow up those ventricles a little more so they stop sticking to the catheter or each other, which in turn, causes her symptoms.  

We drove down to the office where Dr. R waited for us.  I had all 5 girls with me so they came along.  Dr. R walked us into his office and we sat down and had quite a heart to heart.  We talked extensively about Meagan's episodes, her symptoms we have been trying to figure out, and also what we can do at this point to help her.  Granted, she isn't always like the above video - we still have good days and she still has a lot of smiles! However, to see her have episodes like the one in the video is heartbreaking - and it is NOT her norm.  We had nothing like this between last October and this February, so it needs to stop. 

Dr. R dialed Meagan's shunt to the highest setting to see if we can start blowing up the ventricles in a non invasive way.  He was very good with my other girls - he let one hold the magnet and let the other demonstrate to Meagan how it worked.  This is the first time Dr. R has talked to Meagan about what he was going to do ahead of time - I really liked that.  He asked Anna to stand up and volunteer to be his model - he used her head and showed Meagan what the magnet would do and that it wouldn't hurt at all.  Of course Meagan didn't like someone touching her head, but, she was a lot better about it once Dr. R had explained it to her.  She cried a lot less and seemed to have a little bit of understanding since he had just demonstrated on Anna what would be done.  

Once he dialed her up, he scheduled her soon for an EVD.  Basically, he will externalize Meagan's shunt so we can see if her ventricles grow at all.  By doing this he can tell a few things.  He can tell if her ventricles are able to be expanded, or if they are already rigid. He can watch her increase in pressure very gradually so we can see how her tolerance is and what a good balance will be for her personally.  And lastly it will allow him to see, once we reach that "sweet spot," which shunt or valve may work best for Meagan now to hopefully give her more consistent relief. He said he also may go ahead and take out her whole catheter and replace it to rule out any problems from the distal end of the shunt as well.  Her catheter area through her chest and neck has been puffing strangely lately anyway, so I'm happy about the replacement.   It is a long process - at least a week hospital stay, sometimes two... and going to be tough with the other kids and not many people to help out....but I think it will be so worth it to finally narrow down what will work best for Meagan.  I'm counting the days until this surgery and shunt re-placement because I am hopeful it will offer her a much longer term consistency.  

 So many days where she bulges and gets watery eyes

The final part of the plan, if the EVD and new shunt placement doesn't seem to do the trick is to in fact fully decompress Meagan's Chiari malformation.  It is quite a dangerous surgery (which is why she only had the partial decompression last year), so it is the last step of Dr. R's plan, and rightfully so.  But if it does come to that, I completely trust him surgically and will look forward to the relief it may provide to Meagan. We are still in conversation with our second opinion NSG because I think it is still wise to hear what a fresh opinion is on Meagan's situation.  But I'm glad we have a plan moving forward with our NSG here.  I look forward to hearing the conclusions of the second opinion and seeing how that fits in with the plan of action on this end. 

To finish up the Neuro side of things, Meagan had a follow up with Dr. F (neurologist).  He did a repeat EEG for some new seizures we have been seeing creep up in Meagan just to get a new reading before starting her on new meds for the new type of seizure.  Meagan was a champ during the EEG this time.  Typically I would have to hold her down to get the electrodes on.  Even though they don't hurt, she never liked them being attached.  This time, she still cried the whole time, but she laid still on her own.  A big milestone for her as far as being aware of what was happening and realizing it was ok to be upset, but not fighting it.  Such a champ.  (As was big sister Maura who has to come to all these things and sit quietly.  She did a great job behaving and being a big helper).

Laying so still for the tech

Maura rocking the big sister duty

Meagan seems to be handling her new setting "ok" as far as not having any dramatic constant screaming or soft spot hardening - but I also have not seen much change in her behaviors (as far as, change for the better).  She is still acting the same as before she was dialed up - some good times, but  balance and arm issues still present. What we have seen with the new setting are periods of fussiness/irritability that are inconsolable for a few hours, or times when she has a lot of head bulging and seems tired, but as long as these changes stay ittermittent, we still stick with it ....it's the price we pay to try this setting out and see if she can adjust.   If this can help open those vents even a tiny bit before the EVD, then we are on to a good start.  We will watch her and play it by ear.  If she really seems uncomfortable, I can take her in this week or next week and have her dialed back down until we go in for her shunt surgery.  

Being a tough cookie on her new setting

The last few months have been such an up and down roller coaster on many levels.  Meagan's setbacks, the back and forth with testing, and the emotional ups and downs of our relationship with Dr. R.  The last roller coaster has probably been the hardest because it's the first time our relationship with Meagan's NSG has really been "tested."  That said, I am glad where it has landed us - back on track, with a plan and hopefully a good outcome in sight.  I have known the root of her issues in my gut since February - and am glad Dr. R now sees it and is on board.  I don't think he was ever "not interested"... but sometimes we have to remember these doctors, even the good ones we hold on a pedestal by how well they deal with our kids, are regular people too.  Sometimes the doctors have things going on we don't know about and it takes a little perseverance and pressure to come to a mutual conclusion and plan for our kids. Not that distractions should cloud Meagan's treatment - but we do have to take a step back and recognize the vulnerabilities of simply being human.   After all, caring for Meagan is a group effort between Brian, me, and her medical providers.  If one  falters, the other needs to pick up the slack and keep the focus on track.  It may sound silly but the relationships special needs parents have with their good doctors is kind of like a marriage.  When one link is weak, the other links have to keep it together and make sure the child's best interest is at heart.  Sometimes this slack does fall on us, the parents.  It is harder when this happens because we don't have the "MD" after our name, and so have to almost provide more burden of proof.....but we have an equally important duty to keep pushing through until the MD's see what we see and should never be shy to speak up and insist our gut feeling is correct.  

Meagan chillin in her chair... her way.

So now we wait for Meagan's admission - I am really hopeful this EVD will offer new insight into what Meagan needs on the inside so we can continue to see her progress on the outside.  Waiting is the hard part now but I feel we are back on track as far as being really ahead of the game on Meagan's care.  In the meantime, we will savor her good days, give lots of hugs and kisses on her bad days, and trust in God he has led us in the right plan for her to continue to thrive. He gives us the strength to push through the doubt and believe in those "mama bear" moments I know will results in better outcomes for Megs.  Afterall, it is God above us or any doctors that truly knows Meagan's full plan - and I'm confident He won't let us down.

Meagan relaxing with her best fur buddy

Tuesday, May 20, 2014

Stepping forward

We got a call on Friday afternoon that Meagan's panel of neurological testing as finally back at the Dr. F's office... and all was normal.  As I suspected.  I am grateful we aren't dealing with a secondary muscular disorder at this time, but, that does throw us back to neurosurgery to discuss possible long term solutions.  I did email Dr. R and update him on the results but have not heard back with any new ideas as of yet.  We still love Dr. R and trust him surgically with Megs of course, but part of what makes this hard is that close relationship and feeling there is not a proactive approach.  Being that Meagan is so cognitively motivated as of late, means she is recognizing her regressions and weaknesses more and more. We have to ask therefore if all avenues have really been explored, even if those avenues would be exploratory surgery or re-examining her shunt.   We are thankful she is a fairly happy girl and have seen a lot of cognitive growth this year, but we do see a lot more bad days than we used to with balance issues, movement issues where she will just cry for me to get things instead of getting them herself, falling, and her soft spots bulging.

Examples of behaviors that have emerged over the last two months on Meagan's bad days 

Don't get me wrong - she is a happy girl most of the time and seems to be really enjoying her sisters, toys, etc. If you see her out and about chances are you may not even notice if she's having an off balance day, or a day with more "owies."  However, when as the parent you see your child 24/7, you do notice changes.  We saw such a huge difference in her last October after her revision.  She was so off and on with symptoms, and then the day after surgery she was a new girl -  almost like night and day.  We have enjoyed very happy times since then with lots of progression.  So when we start to see changes like vision loss, balance issues, and arm weakness, as her parents we have to question why we see more bad days creeping back in when this is not her norm.  Of course hydro will always have good and bad times that can happen randomly, but when you know your child's normal pattern of behavior, and you start to see this pattern repeat (more and more bad days = something neuro-surgically wrong = neurosurgical solution = happy kid back on track.....),  you start to become frustrated when no one seems to be "doing" anything about it, especially when you have a kid who has lost some of what she had accomplished.  Because of our growing need for answers, and because of her expanding cognitive growth, we owe it to Meagan to make sure all possibilities have been explored. The more she becomes aware of things, as amazing and wonderful as that is,  the more frustrated she is getting with her setbacks.  That said, we have started on the path to seek a few extra opinions on Meagan's case - talking with a few Neurosurgeon's in other states so they can give us a fresh look at her situation.  We have emailed with them a few times detailing her history, her case, and her current issues that are occurring on her bad days and are sending them scans as well.  We hope a second or third set of fresh eyes and ideas will help us to better understand what we can do for Meagan to help her long term progress. If there is something we can try to help her motor growth so we ease her frustration even a little bit, we are willing to do so. If not, that is ok, but at least I will know there was truly not a neurosurgical option that would be beneficial.  From the beginning I have always said my main focus is Meagan's quality of life.  I am ok with whatever limitations she may experience because of her challenges - we are ok with whoever Meagan is meant to be.  But I won't stop short of what she is meant to be.  To see her lose strength or lose skills is a quality of life issue to me.  If getting there means a second or third opinion, then we will do that in spite of our love for Dr. R.

Dr. R with Megs. We really hope he is the one who has some ideas for her,
 especially considering the close ties Meagan has with him.

While we wait to see what Dr. R will do (or not do), to ease frustration on Meagan's part, and ours, we decided to move forward with Meagan's PT, despite warnings about her arm ligaments.  We have just had to shift gears a bit to be sensitive to preserving the health of her arms.  We have pretty much abandoned any avid attempts at crawling or stair work...we don't restrict her going on all fours, but we are not pushing that anymore.  When she gets her arm braces we can probably go back to that more often but for now we are trying to still take it easy on her arm joints.  We decided to move forward working with her legs.  Ironically, due to her losing muscle mass in her arms, her legs have been doing more work when she is scooching since she has less and less power in her arms.  When she moves forward, she will prop on her arms (think of a picture frame), but, really pulls her body forward with her legs to move quickly.  So while her arms have been getting weaker, the unintended (good I guess?) consequence has been her legs getting stronger - which, I cannot complain about! So we are going to go with it.  We are working on a lot of standing with support - getting her used to being on her feet..etc.  Now that she's been wearing her AFO's for a while, and pulling her body weight across the floor with her legs, her lower legs have the ability to do a lot more work with standing exercises.  We have been also holding up her torso and letting her make "walking movements" with her legs.  We will hold her and walk behind her twisting her torso so she gets the sensation of how to pick up one leg and then the other to move them separately. This has seemed to work ok with her as long as we tell her she is dancing.  Seems odd, I know..but when three older sisters are competitve Irish dancers, she sees QUITE a bit of leg movement.  I think this has actually been great for her because she has been watching them dance so long, she seems to want to do it.   She will even yell "off you go!!!" when we are working.  It cracks me up because that is what she yells at my older girls when they practice their Irish Dance... so it seems as long as we tell Meagan she's dancing, and count her off "5...6....7...." she will continue and yell "off you go!!" and move those feet as fast as she can while we hold her and roam across the floor. It's quite hilarious, and also really good exercise for her legs.  The only "downside" is it's not so great on my back, and she does NOT tolerate "walking wings" or other standing support devices just yet.  Leaning over and holding up her torso is definitely not good for me...so in the near future we are going to try her in a gait trainer and see if we can get her used to being strapped in. She hates being strapped into anything but we are hoping since she loves her legs so much, she will get over the strapping in part once she realizes she can move herself in the device with her legs.  The plus of a gait trainer for her is also that it requires minimal arm use.  With a walker, she would be reliant on her arms for a lot of support.  Since that is her area of struggle right now we want to steer clear of being reliant on them to ease any possible frustrations that may arise. So we will see.  I'm looking forward to her trying a gait trainer soon! Meagan's PT said she should have one we can try in the next few weeks that is Meagan's size.

Another bodily "mismatch" Meagan has been experiencing is between her abdominal muscles and back muscles.  From scooching and sitting, her abs are insanely strong.  I wish I had her abs! The problem is her back is not strong, and leaning back on those weak arms has exacerbated this problem.  So although working on standing is "fun," she won't be able to progress to self standing until we get her back a lot stronger.  She will keep collapsing over forward, and with weak arms, that's not a good combo for the ol' noggin! Meagan's PT decided to try a pressure vest on her to see if it would help a little.  Meagan has a LOT of sensory issues (putting on her AFO's or anything on her feet is a huge challenge everyday, she hates anything on her head, doesn't like things pressing on her body, doesn't like certain textures..etc.. ) so the vest was quite the challenge. After about 10 minutes of screaming and crying, we were finally able to distract her and play with her, and she calmed down and kept the vest on.  The purpose of the vest is to help support her back.  This way every time we work on standing, or she pulls up ...etc...she is engaging those back muscles, whereas without it, her abs overcompensate and do all the work.  Meagan's PT was able to work with her for about 30 minutes yesterday wearing the vest and we could definitely see it made a huge difference in her stability.

Meagan's PT helping her feel what it's like to be standing!

Twisting for a step... the Benik vest is really helping her back.

One other thing we are working on as of late is Meagan's growth.  She still has issues with weight gain and growing.  She is at least back up above 20 pounds since adding in her extra feed, and she still will sometimes snack on food, but she is still shoving a lot of it in her cheeks and not swallowing it all the time... which basically means she's burning calories chewing and chewing and not getting the equal amount of calories from the food she puts in her mouth.  What she does eat is minimal, or I end up pulling the bulk of it out of her cheeks when she can't shove anymore inside.  We are working on this and try to give her a little more through her tube but at the same time have to keep a balance of calories or she gets nauseous (and no one likes that feeling).  I was at Target the other day with the girls and they were asking me what clothes Meagan wore.  Out of curiosity, I held up what she wears (9-12 months) along side what she "should" be wearing (2T).  Wow! I keep thinking she looks so big, but seeing the difference in clothes reminds me what a peanut she still is.  We will see what the next step is in possibly trying to get more weight on her.  Weight drives height, and being we haven't had ANY growth in height since last September, we may be visiting the GI again soon to talk about a plan.

Left: 2T and typically what my other kids wore at 2-3 years old.
Right: 9 month pants - what Meagan wears now at 2 1/2.  Oh so tiny but oh so cute!

So that is where we are right now.  I am waiting to see if Dr. R will call us back and have any other ideas regarding her arms or if he will revisit some of the surgical options available.  If you remember back in October when she had all her troubles then, and resolved them with the revision,  he talked about a long term plan and subsequent surgeries to help her function better as she grew or gained new symptoms due to her slit ventricles.  I'm puzzled as to why that plan has been abandoned but hopefully he will contact us soon and we can have an explanation as to what's changed.  We also look forward to further information from the other neurosurgeons we have reached out to, and see how her growth goes the next few months.  While we wait we won't put Meagan on hold.  We will continue to work with her PT who knows her so well and get her more and more upright, getting more and more weight bearing on those legs.  She has really excelled in her speech lately thanks to music and LOVES to sing. Every time she learns a new word or concept, she always sings it first.  It's amazing really to witness how her brain works!  Despite the recent setback with her arms and crawling, I do love to see her stepping forward in so many other exciting areas cognitively....this girl is full of personality.  She is a nut and so fun to be around!  Meagan has a mind of her own.  She wants to conquer the world , and well, we are happy to help her do just that!

Meagan singing "Do you wanna build a snowman" ...whisper style! Silly girl

Working harder at things means more naps! 
I like to see it as more snuggles!

Monday, May 12, 2014

Yippie for the Zippie!

Yep.. the title says it all! It's here! Meagan had a short PT session this morning followed by the rep from Numotion who came out to deliver Meagan's wheelchair! First about her PT session - the PT was actually happy with Meagan today.  We have decided that we are just going to skip working on the arms for now until we have actual answers as to what is going on.  With her AFOs on, and as long as we count her off like she's dancing, Meagan seems to LOVE to move her legs.  We hold her up and she will move her little feet as fast as possible (Thank you 2 years of watching Irish dance!).  So the PT has decided since she enjoys moving her feet, that's the direction we will go.  She is looking in to getting Meagan a gait trainer - we aren't sure how she will tolerate being strapped in, but I think once she realizes she can move it, we will have a lot of success.  Being that for now the arms are a mystery, we are going to work on developing her legs and try to do it in ways that minimize the use or reliance on her arm strength.  The gait trainer will be one way we can do this, along with just holding her and allowing her to "tap tap" her feet as much as possible so she gets the sensation of moving her legs separately..etc.  I'm fine with that plan - it lets us move on in PT without worrying about conquering her arms.  If her arms are going to be a permanent problem, down the road it does raise concerns about falling and balance - not having the arm strength to break falls..etc.. but we will just cross that bridge when we get there.

As for the chair - it is awesome so far and seems like a great fit for Meagan.  It has rugged wheels so we can take it anywhere - the preschool, or a walk on the mountain. The bottom has a huge storage place which will be great when having to transport her feeding bag, along with things for her sisters.  It has a tray which will be wonderful for times Meagan has to wait for her sisters so she has a place to have a few toys, or a puzzle or book.  It has a lot of support for her which is great.  She really needs it in her back especially.  Her abs are incredibly strong but her back is very weak so the supported seat will be great in helping to support those muscles better so they don't continue to get weaker.  It also has a head rest which will be great for her tired days or when she falls asleep.  On the back it has a recline or tilt option - and a hook for her feeding bag so it is secure on the back of her chair especially when in crowded places. There is also a large canopy for when we go places outdoors or in the sun. Even better it folds very easily.  The chair part lifts off easily and the bottom frame folds up with one push of a button into almost the exact same shape as a stroller - which for a busy family of 7 with limited van space is a very important feature!
We are very happy with the feel of it and excited for all the places we can take her in a much more comfortable way!  And it's PINK! What more could a girl want?!

Chair part

Hook for feeding back and pocket for storage

Big rugged wheels and a large storage compartment

Comfortable 5 point harness or the option of just using the seatbelt 

A big tray for playing 

Tilt and Recline options 

Foot rest

She seems right at home! 

Sunday, May 11, 2014

Neurosurgery and Neurology Update

I realize I haven't updated since Meagan's hospital stay.  Mostly because there really isn't much "new" news as of yet but there is a lot of "information"we have to sift through... so bear with me as I try to lay it all out.

Meagan did really well with the rest of her ICP monitoring considering the discomfort of the bolt.  Friday morning the PA came by and helped Dr. R take the bolt out.  They had only seen two spikes in ICP during the night when Meagan was sleeping which was odd, but not enough evidence for them to conclude surgery was needed.  I still feel in my "mommy gut" this is a neurosurgical problem with structure or something that is not obvious, but I can't sit there and change Dr. R's mind.  I will admit that I did get emotional when he said to my face was "nothing neurosurgical he could do" for Megs to try and help with her newfound weakness. I cried. Plain and simple.  All the frustration of the last month and a half, plus not having a clear cut answer, plus having the answer I feel deep down is the culprit be pushed to the side all came out.  Very unusual for me, but sometimes things come to a point where the emotion takes over.   It appears now that we will have to somehow "prove" he needs to do some sort of surgical intervention, which is very frustrating.  To be fair, Dr. R has always been good to Megs and had her best interest at heart - but I really have a gut feeling he is giving up in some ways on trying to help alleviate her current problems.  Not sure if it's fatigue, being too attached, or just that he needs her to be in dire straights before he tries anything invasive, but in any case, as a mother who knows her child, it is extremely frustrating.   In my heart I do believe that neurosurgery of some sort is necessary to help correct or at least attempt to correct her latest setbacks (and my hunch is on that shunt that while "working" may not be working ideally for her at this point in her development).... but, because of the reality, I had to accept Dr. R's conclusions (for the time being) and move forward with the next step to figure out her muscle weakness.

Such a rockstar through the whole process

Following his explanation of everything to us, he said he would be passing us along to Neurology.  Of course.  When you have a complex kid with a new problem - what's the easiest thing to do? Pass her on to another specialist.  We rolled with the punches, though, and agreed to meet with the hospital Neurologists.  Dr. R suggested running a panel of blood tests checking for several different genetic markers, muscular abnormalities, neuro muscular markers, etc. etc...  We were fine with exploring this because though I have my hunches, I do not know for certain - so ruling out anything myopathy or syndrome related is a good step.  We did the in hospital consultation with the neurologists there.  It was a lot of me head nodding and saying "oh ok" while they were talking, while at the same time realizing it was very clear we needed to forget them and go through Meagan's own neurologist for the testing.   I was not impressed at ALL with these hospital neurologists and decided we would do the blood testing through Dr. F who has been her neurologist since almost birth.  We got the list of tests from the hospital neurologists of what they wanted tested/ruled out.  They ordered TSH plus free T4, Amino Acid profile, Carnitine Total and Free, Creatine Kinese Total and Serum, Organic Acid Analysis - Urine, and Acylcarnitine Profile, Plasma.  A lot of complicated words having to do with anything from neuro muscular disorders, to metabolic disorders, to simple imbalances in her body's chemical make up.  She went Thursday to have blood drawn and urine taken to get these tests going. So now we wait for those results which should be back to us early next week.

Not a fan of blood draws - but she really did a great job.

Being that we are essentially in a waiting game, Meagan's PT went ahead and asked to have her arms braced.  Week by week she is losing strength in her arms and elbows, and her ligaments are being stretched beyond any healthy level.  We were able to get in yesterday to get Meagan fitted for arm braces.  Due to her current motor issues, and also her tiny size, she ended up having to be casted so the orthotics specialist can custom make her arm braces to both offer her ligament salvaging stability but also facilitate movement so she can continue to try and explore motor development.  Meagan of course did not like being casted, but we were lucky enough to work with the same orthotics specialist we had for her AFO's so she did ok.  The specialist was excellent with Meagan at distracting her, singing to her, and doing almost anything else he could to make her feel comfortable and relaxed and we were so thankful he was there.  We should hear back this week if her orthotics have been accepted by insurance, and then the timeline of when they should be completed for her.  She really won't be happy about these braces - but - whatever we can do to protect those ligaments is worth it.  The last thing we need is long term, long lasting damage to very essential arm movements and connections she will need in the future for her everyday tasks.

Brave girl during her casting

Once Friday came and went, I really was comfortable or at least getting comfortable with Dr. R's assumption that this must be the cause of some myopathy or neurological breakdown.  We had started a ton of reading, research, and acceptance on our part that Meagan has some other stuff going on.  The older girls had a dance competition - Meagan was ok throughout the day - a bit fussy and sleepy, but who can blame her with the music going all day..etc.  That night, Meagan was her normal happy self playing with my dad - singing, being silly and having a great time.  Suddenly I hear my name being called - I came into the living room and Meagan was fairly lethargic but also fussy.  She looked awful and I noticed the large soft spot on her head was extremely swollen and hard to the touch.  I put her upright to try and help with gravity but her spot didn't relax and she became increasingly sleepy.  At that point I decided to call the on call neurosurgeon.  He advised us to watch her at home and if she changed behavior any more to come in to the ER.  We watched Meagan for a while longer with her propped up in our bed and she remained lethargic and would not open her eyes.  We decided it was time to head to Children's.

When her spot first starting growing - was hard as a rock and she passed out soon after.

Luckily my parents were visiting so we could get in the car immediately.  We started to drive and Brian sat in the back to keep an eye on Meagan.  Her soft spot was still swollen and hard and she refused to open her eyes or rouse easily.  Once we were about 20 minutes into the drive she started to stir a little.  We pulled over to a gas station on the side of the road and parked so we could both evaluate her.  Her soft spot was still tense, but not swollen and rock hard like before - and her awareness was improved.  She was able to be woken up a little bit even if only for a second at a time so we talked about whether to continue to the hospital.  If we got to Children's and she wasn't in the worst state she would have been, all it would mean was us telling them what happened, and possibly some unnecessary testing and sticks, so we decided since the situation seemed to be diffusing we would take her back home and watch her through the night.   I am pretty sure I didn't sleep at all that night.  As the night went on her soft spot continued to relax and she slept.  Around early morning, I noticed her soft spot was back to it's baseline - very squishy and relaxed.

Fast forward to a few days later when I was driving the girls to dance class.  Reilly suddenly yelled at me from the back seat that Meagan didn't look very good.  I glanced in my mirror and noticed she looked "off".... the girls tried to wake her up but she wouldn't budge.  I then saw that her soft spot looked large and hard.  I pulled over at the next possible place and got out of the car to check on her.  I took her carseat straps off to help wake her up - but she didn't move.  She looked completely lethargic and out of it.  I sat her up even more than she was and her soft spot did not relax.  I was about to call Brian to see if we needed to rush her in but noticed her spot then started to pulsate a bit - it was tense but a lot less hard.  Over the next several minutes, her soft spot relaxed and she slowly came around.  Once I was sure she was ok, I strapped her back in and continued on to the girls' class, keeping a very close eye on Meagan.

I'm not really sure what happened during these two incidents - they did not look like a seizure to me and I have no other explanation to what happened.  All I know are the facts - and the fact that both times her soft spot swelled up and got rock hard indicates structural/pressure issues not neurological issues.  It almost made me frustrated at being thrown back to Neurology but what is one to do. I am thankful each time resolved on its own - but at this point when you have a kid as tricky as Megs, you almost want it to stay just long enough for the doctors to be able to see what you are talking about so they can have an actual picture in their mind of what we are trying to figure out.  I did take pictures and videos so we have "evidence" in case it is needed but more than anything I hope these incidents to not happen again.  They were quite scary with her so unresponsive.

How Meagan likes to sit now..ouch.

Action shot of her scooching across the floor - happy she still has the will to move, but sad she is ruining her arms and now her wrists.  She is becoming more fatigued after short playtimes as well, whereas before she could scooch around almost all day and have boundless energy.

On a good note, we got a call that Meagan's wheelchair is in! Yay! They said they can deliver it this week so we are very excited to see it and start using it when we go out and about.  I am excited about the extra support she will have, and the large tray available if she wants to play with toys o while waiting for the older girls' at activities or sitting at Mass or a dinner..etc.

That's really all as of now that we know.  It is extremely frustrating to not know what is going on with her because it blocks us from having a treatment plan in place to conquer these new difficulties.  To look at her, generally, you would never know - she is still happy, silly, and such a sweetie.  But to see her losing skills and have trouble with her strength/fatigue level is very frustrating to me.  She is so determined but her body is starting to not cooperate.  Admittedly,this new issue is also worrisome to me.  Why would she suddenly be losing muscle strength now at almost 3 years old? Why are just her arms showing weakness? Why is she looking smaller but not losing actual pounds? Is this weakness progressive? Is it short lived? Is this connected to her decrease in swallowing? Is it permanent but stopping at her arms? There are just so many questions and no answers as of yet.  I am impatiently awaiting the results of her blood work this week.  If there is an answer there, at least it's an answer and then we can decide what we are going to do to work through it.  If there isn't, it will be back to neurosurgery with the normal results saying "Please help."

It's amazing to me to see Meagan's  spirit in spite of some of her motor setbacks as of late. She still scooches around the room, ...and when she falls over just says "hi mommy" and has the biggest smile and will continue to play while laying down.  She still does try to go over on to all fours to attempt crawling again, but when she does, she ends up accomplishing a big face plant right into the floor.  Matter of fact the other day when this happened,  I said "Oh Meagan, are you ok?".... she looked at me with the biggest smile and say "Sorry mommy!"  Break my heart!

Meagan's smile and spirit are absolutely telling of her inner soul - no matter what happens along the way, she is ready to push through and figure out how we can work through it. Luckily we have a great PT helping us through this and trying to help Meagan figure out how to work through her weakness while at the same time being mindful of saving her ligaments from damage.  Quite a frustrating few weeks as we go back and forth between specialists trying to figure out the root of Meagan's recent motor issues.  However, I'm thankful that I'm the one along the way helping to figure this out.  Because the fact that I have to seek the answers for her means she's mine.  And I really wouldn't have it any other way.

Thursday, May 1, 2014

Plastic Cups and Basketball

Ten years ago today brings back so many memories.  It was a day I will always remember - a day that changed my life - it was my wedding day.  It's quite interesting to think back on all the things that brought that day together... all the little things like the location, the church, the music, the people, the dress (which I did NOT like on the hanger... lo and behold, it became my dress as soon as I slipped it on..)... and all the things that fell into place to make the Mass and the vows sacred and meaningful, and the things that fell into place to shape our reception into the most fun party I have had in years.  

One of my earliest memories of Brian, besides the fact that he was extremely nice and made me laugh, was seeing him at Mass each week. I had a lot of male and female friends in college - and Brian was one of the few male friends I started to notice would attend every week like clockwork.  It seemed like just an interesting observation, but, it turns out in hindsight, it was probably the single most important thing in our relationship.  At the time, it was attractive because we were of the same faith - and who doesn't like a boy that actually takes himself to Mass... in college!? But I realized over the years it was so much more - it represented commitment, even when other people weren't into it.  It represented humility, even when others were all about "me, me me."  It represented love - no matter what life threw at him, how tough the huge term paper was he had worked on, or hey, how bad the party was the night before,  he was at Mass.  It was just a group of values I started to notice, that grew into admiration, and ultimately culminated in me saying "hey, I want to be with that guy for the rest of my life."

Best day ever!

When Brian and I got married, we of course didn't know what to expect, really. I mean, does anyone?  We had ideals, plans, and priorities for our life together, and one of those priorities was children.  We both knew and had agreed in our promises to each other to honor the dream of children and we were eager to start our family sooner than later. Did we know every challenge our child/ren would bring? Of course not.  But we were ready to jump in.  And go figure - because Meagan always keeps us on our toes in more ways than we bargained for.   Let me catch you up on what has been going on with the little peanut.

As you know, she started sitting well over Christmastime, and shortly thereafter, figured out how to scooch herself forward on her bottom.  Around 5 weeks ago, something "clicked" with her in crawling.  She would go to all fours by herself, and just rock for a few times before doing a head dive on the floor - but hey, it was progress.  Before we knew it later that week she was taking crawling steps (well, moving her hands and dragging her legs) on her own a few at a time.  Both the PT and I felt within a few months,  we would have a pretty good crawler in the house.  Each day she progressed... until about 5 or 6 weeks ago when she literally just stopped.

I wasn't immediately alarmed.  Anyone who knows Hydro knows it's 2 steps forward, 6 steps back at time.. and it's just the name of the game.  I had pretty much forgotten about her even trying to crawl because she was still scooching on her bottom.  That following week, Meagan had an eye follow up.  The conclusion from that appointment was that her right eye was losing vision.  We then had Meagan's appointments with Dr. F (Neurologist) who did an 8 hour EEG on her which was basically "ok."  She did have abnormalities especially when sleeping, but, clinically, her body wasn't doing anything like a seizure at that time, and so he didn't change any meds.

Eyes getting wonky again

About four weeks ago, I noticed Meagan's hand (namely the right) would turn in when she was sitting.  This was not really alarming at first.. I figured it was just a phase.  But then I started to notice it turning in at an extreme angle.  And following that we have had weakness in her arms which has resulted into completely over extended elbows.  One might say so what? Before I answer that, yes, she is still basically happy, which is great, but I noticed a severe decline in where she had been gross-motor wise.  That week, Meagan's PT came for her next session and without me even saying a word, her PT came to the same conclusion.  She gave us exercises to do and strengthen her arms - but nothing was working.  This increased over the last few weeks to whole arm, and now the other side of her body as well.  Even worse, her elbow joints are literally starting to pop out or twist backwards when she sits or tries to scooch because she is relying solely on the joint to "prop" her up, a regression from months before when she was truly engaging her whole arm for support.  We also noticed that for her to chew on a snack here and there, it was almost impossible to swallow.  Some solid foods she was holding in her cheeks until one of us would go clean out her mouth.  Vision loss, muscle deterioration, and balance issues when she was sitting immediately had us thinking something neurological was going on.  Since we had ruled out common things at the Pediatrician, and already ran the gammut of the EEG and blood work/genetic testing with the Neurologist, our next stop of course was Dr. R.


Dr. R had us come in a few weeks ago so we could chat about what was going on.  He also had ordered Meagan to have a new MRI so he could check her brain structure..etc.  He saw a few things that could very well be our culprit for her regression.  One was crowding of her brain structures, and the other was her Chiari - which could be acting up again and impacting her progress.  He wanted to bring her in for ICP monitoring (intracranial pressure monitoring... you can read about it here) to see if he could get any more information about why she was having these motor regressions and muscle weakness.  Her arms are so bad now when she tries to use them that our PT actually said until this issue is solved, she is on hold - she doesn't want Meagan to damage her arm joints any more than she already may have and made sure we ordered splints for her arms in the meantime.

Elbows getting so bad

Yesterday I was running errands getting ready to come in today for the testing and one of my stops was at the bank. I was sitting there talking with the banker when Meagan's leg shook.  She was sleeping so I thought nothing of it.  But then it happened again. And again. And then her arms started taking part as well.  The girl looked like someone had squeezed her belly each time so her legs and arms came straight up and out, froze for a split second, and then relaxed totally involuntarily.  These movements continued on and on for almost 2 1/2 minutes and were extremely purposeful and rhythmic.  Seizure. Ugh.  I called Dr. Flamini and Dr. Reisner just to inform them, since it was a longer one and the day before surgery.  They both said they think it's related to whatever is going on and so surgery to do the ICP would still be a go.  I was glad because I really wanted answers.

Today we checked in, got Meagan all prepped for surgery and she went in around mid-morning.  She was out by after lunch time and since then has been monitored so they can watch her ICPs and get a clue as to what is going on hopefully.  She is not too happy since she has her arms splinted (so she cannot pull the bolt out of her head) so today has been pretty rough for her.  She is very uncomfortable, and, I don't blame her.

So far, the numbers look "ok" on her monitor from the little information that I have... so I fear tomorrow will be another "well this is ruled out.. BUT...."  I'm confident Dr. R will come up with a plan for Megs to help her get back on track.  All that said, she is still very happy most of the time.  More fussiness, yes, but overall still her happy self. And for that I am grateful! We are so blessed with her and her progress lately, but no matter how you slice it, regression of any sort is hard.  These kids work harder than anyone I know to do all the things we take for granted everyday - and so when something is wrong, especialy when it is not an absolute clear answer, it makes it very tough.  There is family stress, personal stress, internal worry about Meagan and if her arms will recover, etc..etc.. The list could go on and on...

When these things happen, it's so important to be a team.  And this is where my youthful observation of Brian attending Mass isn't so unimportant anymore. His drive to do what was needed to be done all the way back when we were 19 years old on a college campus shines through in times like this.  Would we like to be somewhere else for our 10 year anniversary? Of course! But we aren't.  We could be all glum about it or disappointed that a "special getaway" didn't pan out - or we can just be glad that we have the access to such great doctors to help figure out why Meagan is having the issues she has been struggling with lately.  I will take the latter.

So many times I think back on that first year of marriage... all the "honeymoon" love, expecting our first child, being in our first home... and I think of all the grand plans I had designed in my mind as we would move forward in our lives together.  Then I look around where I am now and suddenly the really special destination we wanted to plan just seems so trivial.

As for now, we will await the results of Meagan's testing and go from there as far as formulating a plan to help her regain some of the strength she has lost or if there is further testing that needs to be done.  We will be sure to go over all information with Dr. R and also get to the orthotics office to pick up Meagan's arm braces so we can salvage her joints until we get instructions on what the next step is.  I'm confident Dr. R has a pretty good idea as to what is going on - but as we have learned he likes to be extremely thorough and as he said today.."Meagan is a tough nut to crack!" I appreciate him taking the time to really investigate and find the root cause of things instead of just sending us on our way.

When I went to meet Brian in the lobby hallway tonight, I saw him walking towards me with a brown bag of dinner from a restaurant and a large bouquet of red roses. There's a quote that says "You will never be happy if you continue to search for what happiness consists of."   I don't need to search for what happiness is.  It's right here. God always has happiness with me - I just have to choose it.  In a hotel or a fancy restaurant or in a hospital room.....that's not what matters.  Ten years of commitment, humility and love matters. Ten years of laughter matters. Ten years of hardships matter. Actually,  I'm pretty sure sitting on a blue hospital chair with plastic cups of water, watching basketball together through Meagan's monitor alarms is exactly where we were supposed to be tonight....and that makes me happy.

We will let everyone know what Dr. R says tomorrow.

To succeed in life, you need three things: a wishbone, a backbone and a funnybone. ~Reba McEntire