ELLAwareness for SMA (Walk-n-Roll)

Ella Sabine Casten
Born: June 10, 2010
Diagnosed with SMA Type 2:  August 12, 2011
Illinois Walk-and-Roll:  June 10, 2012

We were looking ahead to Ella's 2nd birthday and wondering what we could do to celebrate her time thus far with us.  In the midst of it all we found ourselves thinking about how fortunate we are to have Ella in our lives.  She's a tender, bright soul who fills everyone she meets with joy and hope.


Families of SMA (FSMA) is sponsoring an "Illinois Walk-and-Roll" fundraiser for the advancement of treatment and cure research for SMA.  This event is taking place on June 10, 2012...Ella's 2nd birthday.


"Perfect!" we thought.  What better way to celebrate the life of Ella than to attend the event alongside the lives of all of the kids and families affected by SMA.  What better way to bring people together, raising awareness, raising funds, and raising the possibility of finding treatments and a cure for this disease?  


Our family has been so very fortunate to have received so much support from so many people.  There have been prayers, thoughts, cards, gifts, fundraisers.  People have shown us goodwill, shared meals, given us shoulders to cry on, and provided laughter to ease the stress.  Heartfelt hugs and reassuring glances have surrounded us as we journey with Ella.


Upon further thought we decided it would be fun to join FSMA as one of their teams participating in the Illinois Walk-and-Roll Event on June 10, 2012!!  


Our team name is ELLAwareness for SMA and we would be honored to have your support.


Support comes in many ways, such as thoughtful words or encouraging notes; a monetary donation to FSMA through our ELLAwareness Team; or joining us on June 10th to "walk-and-roll" with hundreds of others.  


We have found that awareness is the fuel that drives the engines of success toward treatment options and a cure.  The 
National Institute of Health (NIH) and the National Institute of Neurological Disorders and Stroke (NINDS) have selected SMA as the disease closest to treatment of more than 600 neurological disorders.  We can help fuel that awareness.


We encourage our readers to share Ella's story with others to help spread awareness of SMA.  It's through this sharing that we can make a difference for those still fighting after a loss, those currently fighting to stay alive, and those whose time to fight is yet to come...for all, we can make a difference.


Please follow the provided link if you would like to make a donation to "ELLAwareness for SMA" or to join our team.


Thank you,
The Casten Family

ELLAwareness for SMA

Ready to Roll!! (by Lindsay)

We have our newly modified car!!


We picked it up from Mobility Works yesterday (Friday) and we are so excited!  I wish I could put into words how happy I feel about being able to take Ella out and about in her power chair.  She will have SO much more independence in this chair and she will be able to explore in ways that she never has before!


Of course, this brings on a whole new set of responsibilities...we will have to be with Ella every "step" of the way, making sure she doesn't run into people, go off curbs, knock things over, etc.  Since she's already able to maneuver independently while on our walks, I'm sure it won't take her very long to learn how to get around when we're at other other places.


We are so incredibly grateful for all of the generous support that we received to be able to purchase and modify this car!  We NEVER would have been able to do it without you!!!  Every time we get Ella in and out of this car with her power chair, we will be reminded of the many, many kind and caring people that made this possible for her (and us).  Thank you!


Here are some pictures of our new ride, followed by a little video tour at the end :)

Back view, closed

Back view, tailgate open (ramp closed)

Side view, tailgate open and ramp down

Ella's chair in the car

Closeup of Ella's chair in the car

Going up the ramp

Power inverter to accomodate Ella's equipment while driving

Middle row with all three car seats

*Once Ella sits IN her power chair in the car (in a couple years), we will remove the middle seat and Ava and Henry will sit in the captain's chairs with Ella behind/between them.

**If you are having difficulty viewing the video click here to view it directly on YouTube's website.

Princess Stinker...(by Michael)

So here it comes.  This time, however, it arrives with 311 lbs. behind it and a fight in her that speaks of her late Aunt Jules.


Ella is sporting her will and independence.  She's testing her parents.  She's being a "stinker".


It's wonderful.


She deliberately disobeyed me while on our walk today.  She had veered off the sidewalk in her power wheelchair and I told her to get back on the sidewalk (please).  She stopped the machine.  I could see her tiny shoulders slump down as she settled herself into the minuscule chair that sits on the enormous machine.  She was settling in for a fight with Daddy.  


I waited.  So did she. So did Henry.  Neighborhood kids and a few adults were delighted to watch the spectacle of an almost 45-year old man and his almost 2-year old daughter begin the dance all parents know and "love".  The dance that pits will against will.  The one that causes blood pressures to rise.  The interaction that has each standing their ground in an effort to make the other comply.


I placed my hand on her joystick to coax her to push it forward.  Instantly she demanded that "Ella Do It!!"; I pulled back and conceded.  She smiled and slowly pulled her little hand away from the joystick; she did a little "Ella Dance" in her victory and sat back into her comfort zone.  Battle one was hers for the taking.  Daddy must try another offensive.


"Words.  Use words." I thought to myself.  


"Ella.  It's time to go.  Let's catch up with Henry."  I suggested politely.


"Ella's turn." she responded with authority.


"Yes.  Ella's turn."  my response was without emotion.


No movement.  Nothing.  People were probably starting to place bets.  The air was warm and the breeze was cool.  She noticed the grass and commented on a bag therein.  A distraction attempt.  I wasn't fooled.


"I'll make it daddy's turn." I threatened.  I know she knows I can do this.  It's a simple matter of pushing a switch on the back of the wheelchair and the control goes completely to me.  


No response from Princess Stinker.


Beep, Beep goes the button that transfers power.


"No! NO!" bellows the child.  


Daddy takes control and moves the machine onto the sidewalk and goes for about 10 feet.  


"Ella's turn!  Ella's turn" she loudly and without embarrassment screams.


I stop.  Bets are probably placed again.


"If you want Ella's turn then you must 'go'" I reason with the 2-year old.


"Must go." she repeats.  "Ella's turn" she reiterates.


Beep, Beep as I transfer power in an attempt to usher in peace for the remainder of our walk.


She takes the joystick and moves forward to 3 inches.  Stops and does the shoulder slinking again.  I've gotta hand it to her, she kept her word.  Daddy was not specific.  She's winning this battle and soon the war.  The dance will be led by her if I don't take the lead myself.


Blood pressure is rising.  I can feel it.  I know I cannot let her see it.  My voice deepens and its volume rises.


"Ella Casten.  Keep going until Daddy says 'stop'."  I command.


"Ella's turn" her sweet little voice rings out with crystal clarity.


"Then go." I invite with a touch of sarcasm.


No response.  


Beep. Beep.  I have control now.  I take the helm and drive for almost a block as she whimpers and cries.  She's trying to bring out the big guns.  After already having two kids the fake crying has no real effect on me.  


"When you stop crying, we'll talk" I tell her matter-of-factly.  Almost 10 sidewalk squares later she says, "Ella stop crying. Talk."


I go in front of her.  Crouch down so we are eye to eye; place my hand under her chin.  I tell her that if she wants to drive, she must go--and stop playing games.  


"Stop games." she repeats.  I wipe her crocodile tears.


She takes control of the wheelchair and drives non-stop for one-quarter of a block.  We are one-quarter away from home.


"Daddy's turn" she delights with her sing-song voice.  


I drive her home.


She's got gusto, she's got "drive", she's got the fight in her that she'll need to survive.  

   

WaterWay Babies Neck Ring (by Lindsay)

Today was Ella's first day back in the pool for aqua therapy since having her g-tube placed.  She had to wait a few weeks before being submerged in the water.


Last week we ordered a WaterWay Babies neck ring to assist her while in the pool.  I had seen some other SMA children using them with great success!


Well, success we had!  Ella LOVED it!!  She was able to float/swim completely on her own and she had both hands free to play :)  


This neck ring has so many benefits...it helps keep her spine straight, it allows her to have independent movement in the water, it allows her to be upright, etc.  We had to put little ankle weights on her so that her feet wouldn't float to the surface, haha :)


I just love seeing her this happy...

Wrapped Up In Her Blanket...(by Michael)

Wrapped up in her blanket, she is safe and sound. 
The world cannot touch her as she lays here, I've found.
Her eyes look into yours, they trust and take hold.
Wrapped up in her blanket, protected from cold.


Wrapped up in her blanket, held closely to me.
I give her the motion, a world she can see.
Her body lay limp, as I carry her through life.
Wrapped up in her blanket, my heart feels the knife.


Wrapped up in her blanket, my youngest will stay.
Touched so deeply, so strongly, by Type 2 SMA.
Her bright light shines, beyond the measure of our own.
Wrapped up in her blanket, our Ella is known. 

 

The Music (by Lindsay)

Music is very powerful.  I've always enjoyed listening to it.


I am moved by a variety of genres of music.  It all depends on my mood, the circumstances, etc.


Since Ella's diagnosis, I started taking walks.  We've always gone for walks as a family during the spring, summer and fall months (weather permitting), but late last fall, I started taking some walks by myself (but with our dog, Sasa, of course!).


What started as just a little "break" turned into therapy.  My walks were getting longer and longer (up to about 5 miles) and became an almost daily ritual (although lately I haven't been able to go as frequently).  I always go at the same time of day (right after dinner) and while I'm gone for the hour or so, Michael bathes all three kids and gets them ready for bed.  I come home just in time to give them each a kiss goodnight.


The walking has been so good for so many reasons.  It's exercise (great for Sasa, too!).  I get that little "break" during the day.  Michael gets some alone time to bond with the kids.  I'm able to have an uninterrupted hour with my thoughts.  I always come home feeling so refreshed.


And then there is the music.  I don't think I could go for my walks without the music.


I've had several different playlists added to my phone since I started these walks.  The songs are all motivating to me in some way or another.  Many are upbeat and fast paced...allowing me to almost dance as I walk (good thing it's usually dark outside!).  Others are darker songs, which help me if I'm mad and just need that time to be angry (and get it out of my system).  And then some are more beautiful and help me to recognize the incredible things in my life and in this world.  Like I said, my mood is what determines what I listen to.  But almost always, there are one or two songs that REALLY speak to me in any given point in time.


And right now, this is that song that's helping me through the tough days...

They Say It Comes in 3's...(by Michael)

As of late, I've had the pleasure of telling people that Ella has been doing pretty well.  I relay to them that the regression path she was on so vigorously seems to have slowed down.  Ever since her new diet was implemented we have indeed seen a plateauing effect.  Ella has been able to use her arms a bit more, keep her head stable, reduce her excessive sweating, and generally seems to have good energy levels.  She has even stood up with the assistance of her AFO's, leg braces, and the loving hands of mommy and daddy beneath her bum.


SMA invades the entire body in one way or another as we are unceasingly finding out.  Over the past week Ella has choked on small pieces of food that she has had no problem with in the past.  In fact, her eating of solid foods, albeit in very minuscule amounts, has never caused her any problems...


...until now.  Now she has choked and from that vomited two times in the past week.  There is a distinct possibility that another swallow study might be ordered to determine if the swallowing muscles are being affected by SMA.


Ella had her routine dietician appointment today.  She has lost 5 ounces over the past month.  Many might read this and think, "5 ounces?  That's it?  Sounds ok to me."  Not so.  While the loss of 5 ounces may seem insignificant to you or me, it represents something completely different for a child with SMA.  It signals that a loss is occurring...ours it to find out if we need to further supplement her diet or do we need to look at her muscle mass...is it dwindling...is it atrophying?  Is it a combination of diet and atrophy?  


The past week or so she has been saying "ow" a lot.  Yes, she just came off an invasive surgery; and yes, we would expect her to express pain from that.  This is different though.  She points to places on her body that have nothing whatsoever to do with the placement of her g-tube.  Is this a learned behavior because she has been given so much attention for the ills that SMA brings with it?  Or is it a true representation of what commonly occurs with kids who have SMA as their bodies are so much more fragile than someone who does not have the disease?  Or is it a combination of the two?


As Lindsay and I are faced with a child suffering from an incurable and untreatable disease that is considered terminal, while it robs our daughter of her ability to move and function in an independent manner, we are still raising a perfectly normal child in terms of her intellect and emotions.  There is no "preparatory class" for either--and while we can fall back on our life experiences with Ava and Henry; while we can seek the inner advise we subconsciously are privy to from our parents; while we can connect with other parents...we are left with so many unanswered questions to what we are finding with our Ella.


The bottom line is that it's frustrating.  


It's frustrating for Ella, it's frustrating for us, it's frustrating for our family, and it's frustrating for all those whose life Ella has touched.

Play With Me...(by Michael)

Having three kids under the age of 5 results in an absurd amount of play in the house...in fact, if they are not sleeping or crying, they're playing (even when they're eating they are playing).  


The play that happens in our house takes on many forms.  There is the kitchen table play; coloring, play-doh, little pretend people and their little pretend house with their little pretend "things", puzzles, and reading.  Ella spends many hours a day at this table with this play...she enjoys it and loves it when others join her.


Then there is the family room and basement play.  Cars and trucks, dolls and stuffed animals.  Tickling and rough-housing, jumping and dancing.  Ella will find herself participating in the more calm type of play in these areas yet enjoys watching the more active adventures.


Then there is the entire first floor play.  Our house is designed so that one can make a complete circle going from the family room, through the kitchen, into the dining room, past the living room, and down the front hall back to the family room.  I can remember when we bought the house how I knew that when we had kids this would be a favorite route to run.  Ava and Henry do this often.  Many times, if Ella is in her chair, they will push her along with them and all three have a great game of chase.  Sometimes, however, Ava and Henry want to run so fast that they do not feel like pushing Ella...besides, it would be too dangerous for her to go so fast.  So she watches.


I watched her watch today.  Ava and Henry sped past her every 12 seconds as they raced around the "Casten Track".  For the first 3-4 rounds she silently watched; she hadn't much expression on her face.  It was as if she were deeply thinking about something as the other two whizzed by her.  Then I saw her expression transform...from contemplation to sadness.  Her eyes looked down, her body slumped a bit more than usual.  Henry trucked by her and her expression changed once more as she yelled, "Henry STOP!".  He didn't obey but kept on his way.  As he rounded the corner by her again she yelled the same thing.  


She wanted to run.


Later that evening we were in the basement.  Ella was propped in the corner of the couch and Ava and Henry were standing in front of the couch playing on a coffee table with little people.  Ella had some in her lap but I watched as she listened to Ava and Henry play.  That expression came back.  I asked Ava and Henry to include her in their play.  At first they didn't but then they obliged.  Her expression changed back to her happy self.  


Afterwards, Henry found a ball.  He excitedly started playing with it which naturally sparked Ella's interest in it.  I found a beach ball to play with her in hopes that she could handle it.  She delighted in the play we engaged in.  She buried her face in the ball, threw the ball, picked up the ball, and enjoyed the ball.  


Such an important part of childhood is wrapped deeply in the pleasures of play.  The mind and body working together to produce excitement and satisfaction.  The reaching out to others to share in the delight of manipulating objects, building imaginations, and freeing oneself through movement.  


I worry sometimes about this part of Ella's development...

Old MacDonald had a what?!? (by Lindsay)

Just a cute video of Ella during bath time tonight...she started singing "Old MacDonald had a Farm" and had a little interruption at around 37 seconds of the video :)  You have to listen closely...

Slow Down (by Lindsay)

Today started out as one of "those" days...

A day where ALL three kids were in bad moods and taking it out on each other.  I tried helping the situation by reminding the kids that today was Wednesday...the day of the week that Gramma comes to babysit.  

But no, it didn't help.  It made it worse (I need to listen to Michael more when he tells me not to talk about exciting things too far in advance).  There is a certain little boy (I won't mention any names), who still doesn't quite get the concept of time.  So when he heard that Gramma was coming, he pretty much threw fits all morning because she wasn't here yet.  (Mom, I love you, but seriously...you have to stop being so nice to them all the time!  They love you WAY too much!)

So needless to say, it was a rough morning.  

But then we got to preschool to drop off Ava (after enduring a car ride of three screaming kids).  After giving Ava a kiss goodbye, I was stopped by the director of the school.  She handed me an envelope of money that she had been raising for Ella.  

Wow.

I didn't know what to say.  

And then she told me another piece of information.  That both Ava and Henry are invited to attend every summer camp session.  Free of charge.

Holy cow.  I'm not sure if they realize the true extent of how helpful this offer is for our family.

You could say my spirits were lifted when I walked out the door of Sky Blue Scholars.  I can't even begin to explain how incredible the staff and families are at this school.  They have gone SO FAR above and beyond anything I could have ever imagined (on TOP of being an unbelievable school to begin with!). 

Then fast forward to the afternoon...

I was driving to a doctor appointment (by myself), when I got pulled over for speeding.

Whaaaaaaaat?!?

I have only been pulled over once before in my ENTIRE driving career of 15 years.

I was definitely going above the speed limit (along with everyone else...I know, I know, not an excuse). 

But as soon as I saw the police officer pull a u-turn and start to follow me, I had that immediate feeling of, "Oh crap, I'm totally busted."

Anyway, I've always thought to myself that I would never cry to get out of a ticket.  I didn't cry that first time years ago, and I wasn't going to today.  After all, I did break the law, so I shouldn't try to get out of it.

When the officer approached my window, I remembered that the guy at the Honda dealer told us to drive very carefully in this Odyssey.  Because technically, we already traded it in, so it doesn't belong to us.  And if we ever got pulled over, it would show that it belongs to Honda (and not us), so things could get a little tricky (i.e. it would look like we stole the car).  

Fabulous.

So of course, I wanted to explain the situation before the officer had a chance to realize it.  And of course, as soon as I opened my mouth, the tears started building.  None of them actually spilled over, but it was incredibly obvious how hard I was trying to fight it. 

Ugh.

Luckily, he was very respectful and didn't even need to see the "rental agreement" from Honda.

He took my license, went back to his cruiser, and after what seemed like 2 hours, came back with a only warning for me (and said, "Just slow down a little," with a smile).    

Phew!!

However, the encounter definitely didn't help my doctor appointment any, because now I was late, and one of the things they were checking was my blood pressure.  Awesome.

You guessed it...it was significantly higher than normal (148/85 to be exact).  So I had to explain to my doctor the events that led up to the appointment and she had me go sit and relax in the waiting room for a half hour to try to get it back down (and it worked...114/68).  

Ahhhhhh.

So it's been an eventful day, that overall turned out much better than some of the events would lead one to believe.

And if I've learned anything...I need to SLOW DOWN.  Probably in many more areas than just driving :)

I Have a Question as I Reach for the Sky...(by Michael)

Whenever Ella is in her wheelchair right before bedtime I always go to the foot of the stairs and have her wheel herself to me.  It's something we've done since she got her chair.  It started out as a way to give her purposeful movement.


Now we just do it because we love to.  She eagerly pushes her 7 lb wheelchair toward me with that unmistakable smile streaking across her delicate face; her arms working hard to produce the desired outcome of making those wheels move toward her daddy.  When she arrives at the foot of the stairs she beams at me as we touch noses.


Tonight, as I lifted her out of her chair I did the normal repositioning of my hands around her torso.  You see, as you pick up Ella nowadays, you have to be extra careful for a variety of reasons.  Her body is frail, uncontrolled, and awkwardly heavy.  She has her g-tube which we must always be cognizant.  As I reposition my hands I always make sure her head and neck are stable before doing so, which always results in us looking at one another face to face; eye to eye.  This is wonderful as I get yet another chance to see her soul through her beautiful eyes.  Tonight, however, I saw something a bit different in her gaze.  She looked at me and for a slight moment there was a questioning look in her eyes.  A look that caused me to set deeper into the gaze we had established.  My instinct was to ask her to give me a kiss.  She obliged and then gave me another one on her own.  She rested her head on my shoulder and I told her she is a good girl.


While sitting with her as she lay in bed, she took a hold of my hand with hers and we listened to the bi-pap together as we do each night.  Tonight however, she did something a bit different.  She raised her other hand straight up in the air...as if she were beckoning to be called on as one does in school.  I've seen that style of hand raising before.  The elbow is locked and the wrist flutters back and forth, trying to grab the attention of the teacher.  Ella's hand was doing the same thing.  It did attract my attention.  She was delighting in the fact that as she lay in her bed she had so much more control and power against gravity.  I could feel her energy as she worked her arm.  No longer was it a floppy appendage but a real part of her that she could be in charge of.  She continued for several minutes until her muscles grew tired.  She then lay her arm above her head cradling the top of it with her hand...a position we've all be in yet we do it without such efforts.


Ella is rounding the corner and approaching 2 years of age.  She was born on June 10 (2010).  It'll be here before we know it...the look I saw in her eyes as I lifted her out of her chair made me realize that our youngest daughter is becoming more aware of herself.  The pure joy in her aura as she manipulated her arm while in bed made me realize that Ella will always reach for the sky.

A Year Ago (by Lindsay)

A year ago.

A year ago Ella was 10 months old.

It was the first time that we started thinking, "something isn't quite right".

I can't believe it's been an entire year since then.

I will never forget how I felt during those few months.

I wasn't scared. To be honest, I wasn't even all that concerned.

I just thought that there was a developmental delay. A delay that she would outgrow.

So she couldn't crawl. And she couldn't stand. Or walk. And she was incredibly flexible.

But every baby develops at their own rate, right?

But still, something was just "off".

I remember talking to her pediatrician about it. Ella was classified as having "hypotonia" (poor muscle tone). So we started some physical therapy to see if it would help. We all assumed it would.

After about two months, not only was Ella not improving, but it seemed that she was getting worse. Her therapist was concerned that something could be wrong.

At Ella's one-year appointment, she was referred to a neurologist. Many thoughts crossed our minds...brain tumor, Cerebral Palsy, etc.

She had an MRI of her brain. Normal.

She had a full metabolic workup. Normal.

Then there is that night I will NEVER forget. I was at my computer in the dining room, searching for some sense of what could be going on in our daughter's body (something I did quite frequently).

I Googled "hypotonia and hand tremors" (another strange symptom that Ella had).

One of the first search results was "Spinal Muscular Atrophy".  Something I had never heard of.

I clicked on the link.

I read. And I gasped. She had EVERY symptom.

I ran to the basement (with my laptop in hand) where Michael was in the office. I read to him the description of this unfamiliar disease, SMA. I remember shaking while I was reading the description, which was basically summarizing what Ella was experiencing. I got to the prognosis. There is no treatment or cure for SMA.

And then I remember telling Michael..."This is the ONE thing we DON'T want it to be".

Unfortunately, we all know what happened next. After an abnormal EMG test that further supported SMA, the genetic test was done. And it was positive.

It's so weird thinking back to those moments a year ago. Thinking that she was just a little delayed. Thinking she would "catch up."

Right now, for some reason I'm having a really hard time thinking about those feelings I had a year ago. Knowing something was a little "off", but not yet knowing the reality of what we would be facing for the rest of our lives.

Thinking everything would be fine.

Thinking, "these kinds of things don't happen to OUR kids".

And now I look at our life. It is SO unbelievably different from how I ever imagined it could be just a year ago.

A year ago...

Ella, 10 months old:



Ella, 22 months old:

The Placard...(by Michael)

SMA is a progressive, degenerative, neuromuscular disease.


Progressive.
Degenerative.


As our family rumbles through the life we now have, confronted daily with SMA alongside all the others issues life hurls at us, we are constantly reminded of the progression and degeneration that surrounds us.


Ella's muscles are experiencing degeneration in a progressive way as they are losing function.  


The progression we, as her parents, are experiencing is the onslaught of demands, responsibilities, & reminders of the disease that traps our daughter.


Each demand, every responsibility, and the constant reminders seem to get progressively more in-depth.  Each one causes our experience to become more cemented in reality...our feet feel wedged in that cement, unable to move.  


Where Ella experiences a degeneration in her muscle function, ours is a degeneration of the life we once had.  Each day we feel further and further away from our pre-SMA days; further and further from the life we thought we would have.  


We have begun, each in our own ways, to accept the life we have at the moment.  We have developed defense mechanisms, coping mechanisms, and reality-check mechanisms...each designed to allow us to feel that things will be all right.  


The picture above is a photo of the Handicap Parking Placard that we received in the mail. It will be used until we receive the official handicap plates for the modified Odyssey.


The placard itself represents a progression in our lives.  Another stark reminder that we are dealing daily with a disease that has but one end.  It places us again further from those pre-SMA days.


The part of that placard that strikes me hard is not so much the icon of the person in the wheelchair but rather the word "permanent" toward the top of the placard. 


Permanent.


On the one hand, the progression of SMA is permanent; the degeneration of SMA is permanent; our struggle with SMA is permanent.  It's heart-breaking to say the least.


On the other hand, the love we have for one another is permanent; the hope we carry around with us is permanent; the will we (and others) have to fight for Ella is permanent.


While the placard may serve as a reminder of the heartache that SMA brings to our lives, it also serves as a beacon of hope; calling us to never forget what we are here to do.  Care for one another. 

She Can (by Lindsay & Michael)

Spinal Muscular Atrophy is a horrible disease.  

It's hard to not focus on the life challenges and struggles that SMA causes.  The disease can be all consuming, occupying close to 100% of our time and energy (both physically and mentally).

But what we sometimes forget to focus on, are all of the MANY things that Ella CAN do.  She is an incredibly bright, sensitive and caring little girl.

So over the past couple days, Michael and I have been putting together a list.  A list of some of the things that Ella can do.  It's a good reminder for us (and others as well) that while she may be limited in some areas of her life, she excels in many others.  

We could have gone on and on forever with our list, as there are SO many things and they were so very easy to come up with.  

So for now (at almost 22 months old), here are a *few* things that Ella CAN do (in no particular order)...

• counts to 9...although she skips 7 and 8 :)
• recognizes and says most colors
• says 4-5 word sentences
• says "bless you" when someone sneezes (or coughs)
• makes people laugh
• lights up a room with her smile
• laughs when someone "toots" or burps ;)
• throws a *fake* tantrum to get our attention
• says "help, please" when she needs assistance
• drives a 311 lb. power wheelchair
• sings "Old MacDonald Had a Farm"
• makes "tooting" sounds with her mouth and then says, "eeeeeewww!"
• has pretty much any medical exam/procedure done with a smile on her face
• says "please" and "thank you" without being prompted
• gives hugs and kisses
• shows compassion for others
• sits contently for long stretches of time during her feeds
• compensates for her disease
• loves to jam in the car to B96 and KISS FM
• uses a plastic fork to eat
• stops herself from crying when we ask her to
• figures out ways to play
• sings along with videos
• dances her "Ella dance"
• plays with an iPod, iPad and Kindle
• waits patiently when we are getting ready to leave...often times while lying on the floor where we've gotten her ready
• tells Ava and Henry to stop crying when they are throwing a fit
• understands that doctors, nurses and therapists are there to help her
• enjoys seeing pictures and videos of herself and others
• loves to shake her head from side to side to make herself dizzy and make funny noises
• pretends to talk on the phone
• trusts her parents when we say we'll be right back to be with her
• pretends to read
• loves magazines
• plays catch with a balloon
• likes to clean up where she has eaten
• throws a fit as an almost 2-year-old is expected to
• folds her hands in prayer
• says "love you"

And the list could go on and on.  We'll never stop marveling at all of the wonderfully incredible things that our Squishy can do :) 

There's that smile :)

One of Ella's "readings"...

Sometimes I Can't Stand It...(by Michael)

Helpless.


Both Ella and I felt helpless.


I had Ella sitting in a chair and she wanted to play with a plastic baby bottle.  It was given to her and everything around us went on as normal...except...


She couldn't lift it to her mouth in order to pretend to be drinking from it.  She couldn't do it.


Instead, she cried.  In frustration she let unleashed her cry.  We asked her to use her words and usually she is really good about stopping the crying and telling us what she wants.  Not this time.  Her cry didn't stop.


I saw the baby bottle in her hands which were loosely wrapped around it...her elbows bent at a slightly greater than 90 degree angle...her head bowed down as she cried.  I came to her and asked her if she wanted her bottle.  In between sobs she said, "Yes.".


I placed my fingers behind her bent elbows and produced a small amount of force against her arms.  This allows her to use what little strength she has to continue the upward motion of her arms...it's almost like we are getting her arms up and over a hill.  She brought the plastic baby bottle to her mouth and pretended to drink.  She stopped crying.


When finished, she allowed gravity to pull her arms down.  The bottle tumbled away from her.  She looked at it and leaned toward it.  Her head instantly bent upward making herself look at the ceiling--which is what she does when reaching to overcompensate for the feeling of falling as she extends herself.  Since her head was positioned upwards she had no idea where the plastic bottle was...even if she did, she wouldn't have been able to get it to where she wanted it anyway.  She cried again.


I helped her.  I sighed to myself.  I repositioned her so that gravity wouldn't have its way with her; she was content.  I walked away and my mind was overcome by a wave of depression, confusion, and helplessness all at once.  A simple plastic bottle, no more than 3 oz., is giving my daughter a hard time.  


Sometimes I can't stand it.


We changed our "play plan" and found other objects that she could play with...things that she can manipulate and grasp.  


It seems as though the "plan" is always changing...keeping up with SMA is proving to be incredible difficult.  


Sometimes I can't stand it...

I'm Struggling (by Lindsay)

I'm struggling right now.  

The reality of SMA has been fierce the past couple days.  I don't know why, but my mind can't seem to escape the true awfulness of this disease.  

Maybe it's because she's growing.  The other day I was carrying her to the bathroom to brush her teeth and I was captured by the sight of the two of us together in the mirror.  She's no longer a little baby.  She'll be two years old in a little over two months.  She should be walking, running, jumping and climbing. 

But no.  Instead, I'm carrying her, trying to cradle her in my arms like a newborn.  But she's not a baby.  And looking at ourselves in the mirror made me realize that when I hold her, it looks like something's wrong.

Maybe I can't escape the reality because she continues to struggle.  So much.

She just keeps regressing!  I thought her regression was supposed to plateau a long time ago and she CONTINUES to get worse!!

And she doesn't get it.  She doesn't understand why her body won't work the way she wants it to.  And she gets so frustrated from the simplest of tasks (like pulling her head up if it falls or trying to take a drink from a cup with a straw).

Last night when I was lying in bed, "that thought" entered my mind.  The place in my mind that I RARELY let myself go to.  Because it's so awful that no parent EVER wants to think such a thing.

But last night, it forced it's way in...

What if we lose her?  What if this awful disease takes our baby girl's life?

What if Ella dies?

I hate Spinal Muscular Atrophy.