Sigh...(by Michael)

There could be a lot said for the past year, 2011.  There could also be a lot not said.  Looking back over the year in review I cannot help but sit back, take a deep breath, and sigh... 

I sigh for lives lost and for precious lives gifted to us...

I sigh for dreams lost and for depth of soul gained...

I sigh for arguments waged and for forgiveness expressed...

I sigh for seemingly endless moments of fear and for comforts found in an embrace...

I sigh for rising anger within and for capturing peaceful glimpses of time...

I sigh for struggles endured and for victories celebrated...

I sigh for the loneliness that traps us and for the community that surrounds us...

I sigh for the chance to give and for the humbleness to receive...

I sigh for missing old friends and for the ones that once again emerged along with the new people filling our lives...

I sigh for my children's pain and for the healing hand of God...

I sigh for the exhaustion we've taken on and for the rest we find in one another...

I sigh for the people affected by SMA and for the gathering of loved ones at their sides...

I sigh for the world in all of its messiness and for the chance to experience its beauty...

I sigh for the challenges Ella has faced and for the strength in which she confronts them...

I sigh for the heartache my wife has endured and for the grace she showers over us...

I sigh for the darkness that covers our minds and for the light that shines in our spirit...

I sigh for every moment I missed and for each smiling gaze I took in...

I sigh for every tear shed in misery and for the laughter shared in joy...

I sigh for the year that has passed without pausing to give us rest and for the year that beckons us on with its promise of its hope...

To look back and define, quantify, or make a judgement on 2011 would be a exercise in futility.  It was what it was; it brought what it brought; it allowed us to experience what we experienced. 

To look ahead to 2012 and anticipate, hope, or otherwise seek something we have yet to experience is also an act of empty gathering.  It will be what it will be; it will bring what it will bring; it will provide us with the experiences that it does.

There really is only the moment of now which is a gift, a present, to us.  If we spend our time admiring the packaging it came in or speak only of the creation of the gift; or if we ruminate over what will become of the gift itself...we cannot possibly enjoy the gift for what it is...for it is right there in front of us and then it's gone.  The gift, the present, is fleeting at best.

I know not what I'll sigh for in 2012...I do know, however, that it will be a sigh that draws in a deeper, more meaningful breath; designed to cleanse my mind, my body, and my spirit.

The Cough (by Lindsay & Michael)

1 to 6.

That's how long Ella was up Thursday night; from 1am to 6am.

She has a cough. A simple cough...for you and me maybe, but not for a person suffering from SMA.

This poor little kid. She was coughing the whole time. Well, trying to cough.

Drawing in a breath strong enough to generate an exhale that will produce a cough capable of clearing her upper respiratory tubes is not something Ella can do. It's torture. And not just for the one who is suffering.

She was almost gasping for a deep breath. A breath that couldn't be there.

For close to 45 minutes Michael, in the wee hours of the morning, had Ella down in the family room performing chest PT (in an attempt to loosen up the phlegm in her chest) enough so her weak cough could have a better chance to expel properly. She lay on his lap, tummy down, head and upper body at a slight downward angle, and patiently let the chest PT (which is done on the chest, sides and back) do its work. She graciously allowed Michael to turn her on her sides and her back to complete the therapy.

When it was completed she wanted to watch TV. Michael put in her favorite Sesame Street video and sat her in her special chair in front of the screen. During the video she had a few good coughs and seemed to clear out some of the upper respiratory tubes. She laughed a bit and sang along with the songs.

Michael brought her into bed with us around 4 am. She tossed and turned, whimpering, until around 6 am, when Lindsay brought her back to her crib. Luckily she was able to sleep for a few hours.

We remembered what Dr. Lester (the pulmonary specialist) said... if she starts to develop a cough, we should put her on an antibiotic to try to prevent it from turning into something more serious.

So Michael took Ella to the pediatrician this morning (Dr. Murphy is out of town, so she saw Dr. Steinken). Dr. Steinken went ahead and gave her the prescription and told Michael the warning signs to look for that would indicate that she's getting worse (and that if she develops any of those symptoms, we need to bring her in immediately).

Dr. Steinken then asked Michael how WE (as her parents) were doing.

Michael replied, "Fine" (an automatic response these days). Then Dr. Steinken went on to say that with the diagnosis being so new, people who respond by saying fine are either in denial or hiding the truth. Hmmm...

He was right.

The diagnosis still IS so new. It sometimes feels like our entire lives have been about SMA, but in reality, it hasn't even been 5 months. Wow. How it has consumed our world.

That comment, however, was good to hear. So much of the time we feel like we should have moved on by now; accepted this awful disease and gotten on with our life.

But we're far from acceptance. Very far.

In an earlier post we spoke of the "phases" that go with life's events (DABDA) and we are finding out that often times the phases may overlap, producing a mix of emotions that sometimes may be at odds with each other. It's a mess. Life is messy.

Our wise associate pastor reminded us recently of the messiness of life; providing us yet again with something to hold on to. Here we stand again, at the crossroads of SMA and our life...seeking to do the best we can to keep our Squishy healthy.

This whole "SMA ride" has been just that... a very strenuous ride of ups and downs. And then more ups and downs.

Today we are down. Very down.

But hopefully Ella's cough will be minor for her and not develop into anything more serious.

And then hopefully, at some point, we'll go back up.

Excited Sadness...(by Michael & Lindsay)

Three-hundred eleven pounds.


15x the weight of Ella.


Designed in Sweden.


Astronomical price tag.


Delivered to our house and received with mixed emotions.


The Permobil K450 MX is here.  


Having this piece of machinery in our house for our 18-month old daughter strikes many chords with us.  


We are excited to have it because it means that Ella now has a long-term solution to her lack of mobility but it carries with it the reminder of what SMA is doing to her body.


We are grateful that modern technology has equipped Ella with a state-of-the-art wheelchair that will allow her to experience the world in a way she hasn't yet and might not have without it.  It brings with it the awesome task of preparing her, Ava & Henry, and ourselves to live with this monster of a machine in our living spaces.


We are in awe at the capabilities the Permobil boasts; it's versatility, power, and parent-friendly controls.  It calls us to the reality that our house will not for long be able to accommodate Ella and her needs.


The excitement in her face when we were making the final adjustments to the chair right before she was to sit in it lit up her cheeks.  She eyed it from as many angles as she could manage and sat willingly in the tiny chair designed just for her.


She took command of the controls almost immediately.  Seeking to explore the new found experience that lay before her.  The buttons delighted her.  It's capabilities enthralled her.  The sheer magnitude of it enveloped her as she gathered ever increasing confidence with every moment she was in control.  


The training that will be required captured our attention as we watched how she attempted to navigate the tiny room she was in.  This will be a task among tasks to teach her how to maneuver the 311 lb. chair with her delicate 1.5 inch fingers. It will take weeks, if not months, before she will be truly independent in this chair.  Not to mention the fact that we have house & car modifications that will require time before they are completed.


Looking at the Permobil K450 MX boggles the mind.  It tells of a society that has advanced itself.  It boasts of a society that has dedicated itself to those (within the society) that need help.  


It also brings to life the reality of why it exists.  


It excites and saddens us that Ella has this in her life.  


Oh...by the way, we've decided this new chair needs a name.  Any suggestions?  If so leave a comment...

The Regression Continues (by Lindsay)

Earlier in the fall we learned that Ella's continued regression was not usually "typical" for a child with Type 2 SMA, which led to the new classification of "severe Type 2." However, we were told that at some point (hopefully in the near future) her regression would plateau for the time being.

About a month or so ago, we started thinking that the regression had indeed slowed, if not stopped. We were very excited that we could finally start focusing on the skills she had, not worrying about what would be gone tomorrow. Or the day after that.

But then a couple weeks ago, Ella all of a sudden lost the ability to lift her arms at her shoulders. She used to be able to lift them to almost 90 degrees. She can, however, still bend them up at her elbows.

I told her PT about our concern last Tuesday, and she immediately saw what we were talking about. Ella couldn't reach for anything. She also noticed that Ella's neck muscles seemed weaker because her head was occasionally falling and she couldn't get it back up.

Lynda (her PT) had me video tape this new regression and email it to Dr. Silver (neurologist) that morning. A couple hours later, his nurse called me and asked if we could bring Ella in to see him that afternoon. We did.



Dr. Silver was concerned that something else could be going on that would cause this sudden additional regression. Maybe she was not feeling well. We thought maybe even her broken arm could have been part of the problem, but that was ruled out because she was able to lift her arms even when she had the heavy cast on.

There was nothing else wrong with Ella. Dr. Silver put it quite plainly... it's just the SMA. She's just continuing to regress. His actual words were, "Her anterior horn cells are deteriorating more rapidly than we would like to see."

Well fabulous. Just what you always like to hear about your baby girl.

So now we continue to go on. Hoping and praying that her regression will plateau soon. It is so very difficult to see her struggle so much on a daily basis, but what makes it even harder is to see her get worse in such a short period of time.

Please continue to pray for our Squishy.

How Does This Happen Anyway? (by Michael)

Most people I have spoken with since Ella was diagnosed with SMA have told me that they had never heard of this disease.  


Neither had we.


Yet it remains one the most devastating genetic diseases out there.

How does Spinal Muscular Atrophy happen anyway?

The answer lies in the genes.  A single protein.  

Here, it looks a little something like this...

The SMN1(Survival of Motor Neuron) gene is what people with Spinal Muscular Atrophy are missing. This gene produces the protein that keeps our motor neurons alive.

The SMN1 (Survival of Motor Neuron) gene is located on chromosome #5.

Mother nature produces two of these genes on chromosome #5:

*an original (SMN1): This is missing in people with SMA

*and copies (SMN2): People with SMA can have up to 6 copies

The two models below show how SMN1 and SMN2  genes make their proteins and how Spinal Muscular Atrophy occurs:


Normal SMN1 protein production
(model below)
proceeds as follows:

• (a) The DNA is "spliced" by the RNA
• (b) The RNA then translates the spliced parts making a protein using 8 full portions (called exons)
• (c) The mRNA transcribes the entire protein (using all 8 exons)
• (d) A normal (functional) SMN1 protein is produced.

__________________________________


Since people affected by SMA do not have the [original] SMN1 gene they are reliant upon the [copy] SMN2 gene to produce the needed protein.  


Spinal Muscular Atrophy occurs (on the SMN2 gene) because:

(see model below)

• (a) The DNA is "spliced" by the RNA
• (b) The RNA then translates the spliced parts BUT the #7 portion (exon) is deleted or mutated, making an insufficient protein.
• (c) The mRNA transcribes the protein without the #7 portion (exon) or with a mutated form of it.
• (d) An "Unfunctional" SMN2 protein is produced: SMA Results

In short: People with SMA do not have the original SMN1 gene (therefore they do not produce the protein needed for their motor neurons to survive).

They do, however, have back-up copies of the original gene called SMN2 (some people produce more than others: Our Ella has three copies.)  


The problem with the back-up copy however is that it is missing or has a mutated key component (exon #7). 


If you look at the RNA (b) part of the picture above you will see that while the SMN2 gene is "splicing" it skips or mutates the #7 portion (exon) of the gene. This skipping/mutation produces an "unfunctional" SMN2 protein...Spinal Muscular Atrophy is the result.


The SMN2 protein is not sufficient to keep the motor neurons alive for very long and they slowly die off; hence the progressive, degenerative aspect of SMA.

Researchers' Approach:

Since researchers know that the problem with SMN2 is the deletion (or mutation) of the #7 exon, they are hoping to intervene (with a new experimental drug call SMNRx) at the RNA (b) splicing point--telling the gene to not skip or mutate the #7 exon thereby producing a more functional protein.


A pharmaceutical company is set to begin phase 1 clinical trials using SMNRx.


Clinical trials occur in 4 phases:

• Phase 1: Researchers test an experimental drug or treatment in a small group of people (20-80) for the first time to evaluate its safety, determine a safe dosage range, and identify side effects.

• Phase 2: The experimental study drug or treatment is given to a larger group of people (100-300) to see if it is effective and to further evaluate its safety.

• Phase 3: The experimental study drug or treatment is given to large groups of people (1,000-3,000) to confirm its effectiveness, monitor side effects, compare it to commonly used treatments, and collect information that will allow the experimental drug or treatment to be used safely.

• Phase 4: Post marketing studies delineate additional information including the drug's risks, benefits, and optimal use.

Courtesy of: http://clinicaltrials.gov/ct2/info/understand

Calendars (by Lindsay)

Yesterday I got our new 2012 calendar.

In addition to our online calendar, we have a wall calendar that I write pretty much everything we do on.  It hangs on the side of our refrigerator, so I can have quick, easy access to it.

The first thing I do when I make the switch from one year's calendar to the next is go through and write in all the obvious dates (birthdays, anniversaries, etc.).  I have the two calendars side-by-side, flipping through each month together.  Then I check our online calendar and go through and write any appointments or events that we already have scheduled for the new year.

While going through the 2011 calendar, I found myself noticing the strange progression of our year.  Early in the year, things were fairly uneventful.  But then when the summer months hit, our weeks started filling with more and more doctor appointments.

And then I came to August 12, 2011.  That dreadful day that I will never forget.  The day our lives were turned upside down.

It was hard to take my eyes away from that small white square on the 2011 calendar.

It was weird looking at the day before (the 11th, our anniversary).  We didn't know.  Yet.  We had a good suspicion, but we didn't know for sure. 

And then the weeks really started filling in.  More doctor appointments.  PT, OT and aqua therapy every week.  Dietician appointments every month.  

It was such an odd feeling looking back to those early months of 2011, when our calendar was so empty.  What did we do?  How did we spend all of that time?  

How did we feel?  I honestly can't remember what it felt like to not have SMA in our life.  I don't know if that's good or bad.

I do remember writing out all of the 2011 dates in our calendar a year ago.  I sat in the kitchen.  Today I'm in the dining room.  But a year ago, I had no idea what was really in store for us.

Who would have ever thought we'd be where we are now??  Our baby was a perfectly healthy, normally developing little girl.  

I wonder what 2012 will be like for us.  For Ella.  I wonder what our life will be like a year from now, when I'm filling out the calendar for 2013. 

I'm hopeful that good things will happen for our Squishy.  For all of us.

Just Around the Corner...(by Michael)

Christmas is just around the corner.

This year is different.  It has called Lindsay and I to look at our lives and the lives of our children in such a different way. 

The “holiday spirit” of yesteryear brought with it the gathering of family & friends, the gifts, the lights, the food, the decorations.  All designed to enhance the feelings associated with the season.

It’s different now.  We do not know how many Christmas’s we’ll have with Ella.  That’s a stark reality that brings our world to a halt whenever we think about it.

Everything outwardly is still the same; the world goes on with the preparations, the merriment, the anticipation.  We have found that the change is inside of us.  We see the season with new eyes and a virgin heart.  

Yes, we got a tree. Amidst the decorations and lights it seems empty somehow.  It's there yet it stands alone.  Kind of sad in a way.  When we gaze upon our tree the feelings elicited draw us deep into the moment we find ourselves; the life we now have.  How different this life is and will be.  

Yes, we talked about what to buy for whom.  It seems we cannot focus long enough to find the right gifts.  Our minds quickly turn to the needs of our children; their ever-increasing needs.  Our thoughts are captured by the reality of the financial burdens that have crept their way into our nice little budget we had worked so hard to develop and make work.  We search for meaningful gifts within our means; seeking them to truly be from our hearts to the hearts of our loved ones.  What might those be?

Yes, we made plans with our family.  They know not how much we need to rest in their love.  Spending time with each person this holiday season will keep us connected.  We feel cared for by them in a way that no other group of people can care for us.  They say one cannot choose their family...I don't believe that...I think we chose each other long ago.

Yes, we have attended church.  The depth of compassion, support, acceptance, and love we have found at St. Timothy's has held us strong.  We have attended the church classes centered around the Nativity.  In this we have found something.  Our inward change finds its roots here...

We feel the hand of God reaching out for us...showing us there’s now so much more to Christmas than what we have been celebrating in years past.  We will not give up the traditions and celebrations of the past...we will simply enhance them with what we are feeling in our souls to a greater degree.  There is something deeper...something that we know will reside within us, even after the holiday has passed and until it reappears again the following year,  if we allow it .

Nothing else really matters to us more than the life of our children, especially that of our Ella; whose life is threatened daily by SMA.  Watching her lose her ability to sustain herself physically shreds one’s heart into pieces, steals those pieces, and scatters them flippantly back onto the soul…we look to God and each other to gather up those pieces and restore them to their proper place.  We embrace those who seek to comfort us.  We walk through this life with a numbness that defies explanation...a deep hole if you will, that surrounds each of us…black, darkened, and void of escape on our own. 

There’s only one way out and we are finding Christmas speaks to us of that promise.

Lights and decoration will come and go.  Gifts will find their rightful place within our lives.  Food and preparations will be distant, pleasurable memories, among all the others. 

The long-lasting eternal meaning of what we now experience through Christmas is based on hope, perseverance, and gratitude.  As peacefully and perfectly as God entered this world for us shall He enter our hearts and lives.  We’ve found that putting aside all earthly cares has opened up ourselves to receive.


Christmas is right around the corner, yet it brings us to a path filled with a deeper understanding of life that we now must continue to travel...for the sake of our children, the sake of ourselves, the sake of Ella Sabine.

I Care (by Lindsay)

Today I care.

I've found it's been very difficult to care about much these days.

It's hard enough to just make it through each day.

My to-do list has been getting longer and longer, and I've just wanted to crawl in a hole until those tasks just vanished.

But guess what? That's not how it works. Go figure.

But something came over me today and I was productive.

I did laundry. This is typical for any given Monday, but I still did it.

I cleaned. I mean REALLY cleaned (the first floor, anyway). This is not something I've done in a while. It's been one of those things that I didn't care about. At all. But every time I'd walk by a some junk piled up high, or dirt and dust on the floor, it would make me angry. But I never did anything about it, because I didn't care enough. But today I did.

I called two places to start the process for getting a lift installed in our car for the new wheelchair. This was a very simple task, yet I hadn't done it yet because I didn't care. I didn't want to start the process. I know it will require more work, and that's the last thing I've needed. But it's been started. And now I just have to wait for them to call me back.

I also called the two companies to get estimates for a ramp for our house. Once again, not something I've wanted to do, but I did it today.

I called Children's Memorial Hospital to have Ella registered. She's going there to see a pediatric neurologist (Dr. Nancy Kuntz) on January 5th. We've heard of this incredible doctor through Ella's PT and also through FSMA. We've been told that she has a phenomenal SMA team and that she is very proactive (whatever that means for a disease with no treatment or cure). But we were curious. So we asked Dr. Murphy if we could go see this doctor, and she made it happen.

In the past, completing tasks was just a part of daily life. I never thought much about it. But now, things are different. Motivating myself just to get out of bed in the morning takes a whole new kind of energy and strength. I use up everything I have just to take care of Ava, Henry and Ella's immediate needs. Tackling to-do lists is so incredibly difficult.

But today, I did it. And I feel good.

18 Going On 4 (by Lindsay)

This week I've been able to reconnect with a few close friends.  I've definitely been "out of the loop" lately, so it was very nice to be in such good company again.

Many of my friends have kids the same age as Ava, Henry and Ella (or very close).  It's always been great for us moms to catch up, while the kids entertain each other.

Today, my good friend and I listened while our children (Ava, Henry, Sophie & Luke) played hard.  They went down the basement and played on the "roller coaster".  Then they all ran upstairs to the second floor to jump on the beds (and somewhat destroy the nicely arranged rooms... sorry, Mary!).  Then it was back to the basement, and back upstairs again.  This happened for a good 3 hours (with a little lunch break in between).  

But there were two other children at the house on this sunny December day.

Ella and Maggie. 

Ella and Maggie are good buddies. Ella is 18 months old. Maggie is 4 months old.

Ella is almost 5 times Maggie's age.

Yet they both see the world from a very similar view.

Ella wasn't able to join in the fun with her sister, brother, and their friends.  She can't run up and down the stairs.  She can't jump on and off the bed.  But she was very happy playing with "baby Maggie", as she loves her very much. And Maggie stays in one place on the floor.  Just like Ella.

But what happens when Maggie starts crawling?  And then walking and running and jumping? 

Ella will still be lying on the floor.

Yes, she'll have her wheelchair.  But it won't be the same.  The kids will still run up and down the stairs and go places where her chair will not be able to go. 

I'm sure we'll get creative and figure out ways and places for the kids to play so that Ella can be part of the activities.  We'll have to.

But in the meantime, I'm so glad that Ella has her Maggie :)  

Don't Be Fooled...(by Michael)

Don't let the scene above fool you.


Yes, the three Casten kids are engaged in their own separate activity.


Yes, the room is quiet.


Yes, this only lasted for about 10 minutes.


Raising 3 kids under the age of 5 is something we knew would be challenging.  We knew they would fight, we knew they would bother one another, we knew they would make bad choices right alongside their good ones.  


Don't let the scene above fool you.  They are just about driving us crazy.


While SMA first and foremost affects Ella, it digs its claws in the whole family.


Ava and Henry are unable to express, in a way that is productive, the feelings they encounter internally and externally in regards to SMA.  But they do have those feelings.  It's very hard for them.  Our task is to identify when they are overwhelmed by life...and help them through it.


Ella is a smart girl.  She is beginning to understand that her disability forces her into a different way of dealing with the world.  She has no model.  The older she gets, the more she'll understand...a blessing and a curse.


For right now, though, we are doing our best to sort through what life has handed us.  We feel numb.  We feel defeated at times.  We feel that the enormity of caring for our children will consume us.


The scene above is one that we must hang on to...it reminds us that there are three individuals; separate and distinct from one another; whose life, health, and well-being depends upon those who love them.


Don't let the scene above fool you...it really is a calling for us.

DABDA...(by Michael)

I hold a BA in Psychology and an MA in Education.

I am fascinated by human behavior.  I have learned much through my life experiences, my education, and from working directly with children & families for nearly 20 years now.

I remember learning about the 5 emotional phases that people go through when dealing with difficult times.  I recall them with the acronym "DABDA":

*Denial

*Anger

*Bargaining

*Depression

*Acceptance

People go through these emotional phases when dealing with all kinds of events, although they may not necessarily be experienced in the order written; and often, any one of them can repeat themselves within the given event.  

Within the past three days I have been angry.  It's not something that I easily admit...at least not publicly like this, but it is true.


It’s an anger that resides deep within my core.  An anger that works its way into the fabric of my life.  An anger that I am fully aware of and at the same time I fight to keep down.  An anger that is persistent, powerful, and unforgiving; feeding on itself; delighting in my pain.  This anger, which is part of the emotional spectrum, is met with all my other feelings; vying for a position of highest emotion.  It is strong.  It is there.  It is making a break for me.  It demands my attention.


I know it is healthy for me to experience this emotion; as negative as it may be.   I must honor the spectrum of emotions...I must allow myself to feel all of them.  I get to know my emotions so I can deal with them appropriately.  This emotional war has been thrust upon me.

I sat in church this past Sunday.  I listened to the pastor as Lindsay and I kept our eyes on our three little children.  I have become quite good at giving the pastor my ear while being summoned by the wants and needs of a 4 year old, a 3 year old, and a one-and-a-half year old.  

The sermon held its focus on the idea of hope.  It was delivered alongside the ideas of optimism and pessimism.  He related the three to one another to further illustrate his point.


I walked away with a better understanding of how optimism and pessimism wax and wane—how at any given moment they may come and go; depending on external factors that we live with everyday.  Hope, on the other hand, does not ride the same waves optimism and pessimism ride.


When he spoke of hope I found myself listening more intently.  My mind took in the idea that hope rides the winds of life in a upward, smooth, and ever increasing motion.  It glides along our experiences, providing the impetus to persevere.   It, unlike optimism and pessimism, is not dependent on external factors.  Hope is a result of our soul’s need to let God in.  Once we let Him into our hearts, truly let Him in…hope springs eternal.


Anger heard the same words I did.  We battled for days.  Anger took shots on me at every turn.  Battles ensued.  

I have fought with anger before in my day.  I'm scarred, to say the least.  

I've also learned about my enemy: my anger...I know its weak spots.  I have armed myself with the one weapon I know will defeat it...


Love.


For with the love of my wife, the love of my children, and the love of all those who surround me anger stands not a chance.  It will come again.  It will find a place and a weakness within me...it will strike. I will fight once more.


I have love...and from that love I have hope.  And within that hope I place myself. 

She's Free! (by Lindsay)

I always wondered how they got a cast off...

Today I got to see how it happens. Ella got her cast off and had new x-rays of her arm, and she's completely healed!

I was definitely more excited than she was... ever since it came off, she's been holding her arm and whining, "More purple!" I guess it can take a little while for her to get used to "not" having it :)

She's still favoring her right arm quite a bit, so it will take time for her to get used to using it again and to rebuild some of the lost strength.

But so far, no sucking her fingers! Fingers crossed that it continues...

I am SO happy that the cast is gone... it will make our lives just a little bit easier :)

Anyway, here are some pictures of the process:

Detours...(by Michael)

As you may have read, the insurance company approved her power wheelchair. They approved it without blinking. That's really incredible considering that many people have to fight the insurance companies to get what their kids need. The feelings that filled our house when we knew she would have the wheelchair settled in for the night. Many of you celebrated with us through your words and thoughts.


As time slowly rolled by today I found myself thinking more and more about the Permobil K-450 that would soon arrive at our doorstep. The practical side of me is still excited. I'm excited for Ella to have a way to interact with her environment; excited to teach her how to use the 300+ lb piece of modern technological equipment that is a far cry from the kinds of wheelchairs available to kids who needed them when I was little.


Then my mind takes a detour as it so often has these past few months. A detour that I travel blindly. Travel it I must though, for it is part of what a parent of a child with a terminal disease must endure. A highway with no road signs, no speed limit, and no warning of the detour just ahead.


Someone spoke to me today...I literally did not hear them. They were speaking directly to me. My mind and senses were on a detour; this one was traveled on automatic pilot~I had no control of the thoughts that entered into the crevices of my mind. The reality of where I was became lost in the depths of the detour. It whisked me downward, almost as if I were spinning, and left me alone in a cold, damp, dwindling state of mind. The voice that beckoned for my attention was now silent and I felt several pairs of eyes upon me. The patient looks of those surrounding me gently lifted me from the detour and brought me back; quickly, smoothly and without judgement.


For the rest of the day and to the moment of writing this blog, the remnants of that detour remains.

I spent time with each of my kids this evening. Henry sat on my lap as we watched the Sesame Street character, Ernie, singing in the tub to his rubber ducky. I lay next to Ava before she fell asleep reassuring her that tomorrow is another day and she can start all over (she had a rough night!). I sat with Ella just before dinner watching her play with Play-Doh; organizing it, manipulating it, and generally focusing all of her attention on the task as hand. 

I sat down to write this blog and while doing so received an email notification for a comment that was submitted on our last blog.


I read it. I stopped writing. I put the kids to bed.


Apparently, the person who makes the wheelchairs went in to work today (his day off, mind you) to make Ella's chair...just for her.


Another detour.


But this detour does not have the same markings as the other one I experienced today. This one quickly took me down a road of compassion and goodwill. The crevices that were previously filled with the darkness of SMA were now filling with the brightness of compassion. Ella is a fortunate girl.


I still hold fast to the fact that this wheelchair is good. I also am realistic with myself and know that I will struggle with the implications that go with it.


I know that the detours that SMA carries me through in my mind will continue; yet I also know that these detours hold the sum of all the experiences I will feel...the good, the bad, and everything in between.

Permobil K450 MX (by Lindsay)

Oh my.

We got a call from the pediatrician's office today.

The insurance company approved the power wheelchair for Ella.

100% covered (except we have to pay the tax).

Wow.

I am so excited, I can hardly contain myself! Once Ella gets this chair (and masters it), she will be ALL over the place!

She's getting the Permobil K450 MX.

It will be SO good for her! The seat raises up and down, it tilts back for when she gets tired, and best of all, the chair lowers to the ground so she can interact with her peers! It will be so wonderful to have her be able to get around so much more independently outside of our house!

So now we're waiting for the vendor, National Seating and Mobility, to receive the official authorization from the insurance company. Then they'll place the order.

The vendor representative that we've been working with said we'll probably have the chair by the end of the year (like... in a few WEEKS!).

How incredible!

But now we really have our work cut out for us. We have to get a lift installed in our car. We have to get a ramp for our house. We'll have to do some rearranging in our house to try to make it more open for her, if possible. We've discussed putting something along the walls to prevent her from scratching and denting them while she's learning how to "drive".

We've talked about how getting the power wheelchair will be a turning point in our life. Life will be very different for us with this chair (and it will be quite the adjustment).

But it will be so worth it for our Squishy :)