Individual Eternities...(by Michael)

"Use all of your fingers."

Five fingers.  Five words.  Repeated over and over from me to Ella.  I say it to her all the time.  She complies when I say it yet quickly allows the SMA to inhibit her "finger function"...she uses the pincer grip constantly.

We stretch her hands, do OT type activities with her, and remind of how best to use those ten fingers on her hand.  Often I feel it is to no avail.  In the midst of being 4½ years old she hasn't the time to think about "proper use" of her hands.  She wants to play, to complete a task, to just be.  The easiest way for her to do that is to compensate for the debilitating SMA.  

SMA.  Where on earth did this come from?  It was surreal when we first received her diagnosis and that hole in reality has matured into a crater in our lives.  Seemingly consuming all in it's circumference as it widens unending.  We step back out of the way, keeping Ava, Henry, Ella, and each other from slipping off the widening sides into the abyss it presents.  To take your eye off of the increasing crater-hole to look at the world around us...the world that keeps eluding us, the world that most people live in and enjoy...to take one's eye off to gather a glimpse is dangerous...for the hole widens and grabs at your feet...partnering with gravity to pull you in.  

The crater can only get to be so big and then the ground underneath our very feet will begin to crack. Solid ground becomes sparse and a balancing act ensues.  It's tiring, trying, and tumultuous at best.

As we hold on to one another, circumventing the enveloping crater before us in attempts to stay on solid ground we have carved out a small corner in which we can run to...Ella's Corner. 

Ella's Corner is a place where we can gain a solid footing; a place where we can bridge the gap between our world, which is riddled with craters and cracks, and can meet the world we were pulled from in August of 2011.  A place where anyone who reads can take a peek, lend an ear, speak from wisdom, or simply gather a fleeting moment of our lives.  

There will be a time when the black hole of SMA is closed forever, yet the scars it will leave shall echo through our individual eternities.

Chocolate Cake with Whipped Cream...(by Michael)

Excitement filled their hearts.  Self-satisfaction permeated their being.  Achievement resonated throughout the house.

Warmer weather brought with it the pleasures of the outdoors. Over the summer of last year Henry learned how to ride a two-wheeler.  Ava had not.  Ella stayed in her wheelchair.

March, 2015...with determination both Ava and Ella ventured into the biking world.  Ava foregoing the training wheels and Ella taking seat upon her adaptive bike.  

The video below shows you what happened.  In the words of a proud, happy Henry, expressing his joy over his sisters... 

"Bike riding is like tasting 'chocolate cake with whipped cream'!!"  Henry (3/2015)

2015 FSMA Conference

So we move forward.

After receiving news that Ella has not qualified for the clinical trial we simply move forward.

Each year, Families of Spinal Muscular Atrophy (FSMA) holds a conference that invites families with children suffering from SMA, the doctors who treat them, and the researchers who work tirelessly to find viable treatments and a cure for SMA.  It's an incredible experience to say the least.  We've attended one conference since Ella was diagnosed.

During the short time we were there we found it to be one of the most special and enlightening experiences.  We found ourselves surrounded by families that are going through exactly the same things we are going through.  They understand, on a level that is elusive to most, the feelings and challenges we face as we journey through life with SMA.  The common ground that we share draws us closer to one another, nurtures friendships, and offers us unparalleled support. 

The kids, too, walked (or rolled) away from the conference with a new found respect for one another. They began to understand, on the simplest level, the realities of SMA; how best to navigate through their daily lives; how best to practice empathy for one another; how best to be a part of a family that has the specific challenges SMA presents.  

There's a reason, however, why the conference is an annual event.  

Through diligent research the scientists are always moving forward.  The conference offers them a chance to connect with families and doctors to explain their advances in research as well as listen attentively to both parties.  

Doctors have different approaches in the ways in which they view best practices for their patients.  The families can get a better idea of differing medical opinions and even find a "good-fit" doctor for themselves (we found Ella's orthopedic surgeon at the 2013 conference).  

Families find new ways to care for their kids and themselves.  The siblings are reminded of what they learned about before and are given a chance to renew their knowledge while simultaneously learning new skills and strategies.  As they grow older, their responsibility within the family grows...the conference provides an outlet as well as an inlet for them...a way to express themselves safely and empathetically while also helping others with their feelings and struggles.  It's a process that grows with them as time passes.  The conference is such a wonderful way to empower them.

And Ella.  The conference is probably most important for her.  It provides an atmosphere where she can identify with other children and adults who have SMA.  She is afforded the opportunity to see, talk with, and connect on multiple levels with the struggles she faces alongside her peers with SMA.  A disease that is uncontrollable must be hard for a kid to handle...knowing that other kids have and do experience the same lifestyle is paramount to her positive outlook on life.

We'd love to attend the conference this year in Kansas City, MO.  It runs from June 18-21 and is a 5-7 hour drive.  Of course, we would need some help in terms of financing such a trip.  

If you feel that you might be able to help us finance this year's trip to the 2015 FSMA Conference we have a fundraising page set up specifically for that purpose.  

From conference registration, hotel, meals, and driving expenses our goal of $2,000 has been set.  

To donate to our trip please follow this link:

Click Here to: Donate for the Casten's to attend the 2015 FSMA Conference

Thank you so much for taking the time to read our blog and for supporting us in the many ways that you all do each and every day.

Michael, Lindsay, Ava, Henry, & Ella 

Please feel free to share as you see fit!

My Own Clouds...(by Michael)

I know what I say.

I tell myself that there's a reason for everything.  I repeat in my mind that we must go forward.

Now, however, my thoughts feel cold and my spirit erased. There's no other way to describe it.

I know what I say...and I understand that this, too, shall pass. Yet for the moment, in the moment, a stifling numbness takes over.  A lack of control builds up inside.  A realization that while we're not any worse off, we're still looking for the light.  

It seemed as though the light glimmered on our lives.  In a way, I suppose it did.  That twinkling of hope held an eternity of promises.  It mesmerized us; sang to us; brought us to it.

Ella had an opportunity to be part of a clinical trial for an extremely promising medication that may hold a treatment for SMA.  The drug is called SMNRx and its function is to direct the back-up genes (that many people with SMA have) to produce full functioning SMN proteins (as currently the back-up genes only produce about 10% of functional SMN proteins).  It has been quite successful up to this point in many animal and human cases.  It's widely known in the SMA Community and holds us all eagerly on the edge of our collective seat as we all wait and watch.

Ella's doctors and therapists felt she was an absolutely wonderful candidate for the trial...we did too.  With that in mind we spent many hours at Lurie's Children's Hospital partaking in the screening process.  She did wonderfully. One of the exclusion criteria has to do with the degree of scoliosis present...Ella's degree of spinal curvature was a smidge higher that the sponsor wanted yet they still had us engage in further screening criteria.  They also asked for an opinion from her orthopedic doctor and another x-ray.  Her orthopedic doctor gave her the "go ahead" to participate and even produced an x-ray that showed not only a lesser degree of curvature, but spinal flexibility as well.

Not good enough.

When we received the call that Ella was not accepted the frigid weather outside seemingly tore through our souls. While we prepared for this outcome the reality of it whipped us back to the time when we first received her diagnosis.  It's a feeling that simultaneously presses hard into you and pulls you apart.  It leads you to a place that is better left unattended.  It strips you of knowing that everything will be all right...somehow; some way; some time.

We never told Ella why we were going to Lurie's all those times during the screening.  We felt it best not to place expectations in her head...and at the same time we tried not have any in ours.   It's tough, though.  She's our little girl. She's our Squishy.  She's ours.  And we want only the very best for her as she travels through her fragile life dealing daily with SMA. 

We seek earnestly for the trial to go well with those enrolled so that all may someday partake in the healing medicine science will hopefully...one day...offer.     

I know what I say to myself and I do believe it...  

...there is a reason for everything...whether I can see that reason through my own clouds or not.

Ella Sabine Casten
February, 2015

Isn't God Strong?...(by Michael)

Ella has asked me certain types of questions twice in the past week.  Each time she inquires of me it's always when we're alone and I'm helping her with some daily activity of life.

"Why did God make me with SMA?" was the first question she uttered.  Of course, I hadn't an answer right at that moment...probably because I'm not too sure myself.  My answer came quickly saying, "I'm not sure, honey."  I continued to help her and she carried the conversation in a different direction...I obliged.

Then, not two days later, she began to ask the same question.  But I suspect she knew what my answer was going to be so she stopped mid-question.  This time though, at least as she began to utter her words, I felt that I had to provide more than a "I'm not sure, honey" response.  

But her words stifled in mid-air and she quickly changed the question to, "Isn't God strong?"

A bit surprised at the question I replied, "Yes, very strong."

"Super strong?  Like--stronger than anything?" she continued.

"God is all-powerful." I replied.

"If He's so strong, why can't He stop the SMA?" she quizzed me.

This question was harder than the first one from a few days ago.  As if on automatic pilot I continued to help her with her task.  My eyes fixated on the corner of the room and my mind cleared itself...waiting for an answer that I could relay to her.  

"He can stop it." I said.

"Then why doesn't He?" she persisted.

Again, the questions are getting a bit harder with each exchange.  She looked at me and I saw in her eyes the need for an answer...an answer that would give her something to hold on to.  

"He is." I found myself telling her.

"He is??" she asked,  "How?"

"He's using the doctors to help Him do His work." were the words that rolled off my tongue.

Without missing a beat she came back at me with her little voice, "Why does He need the doctors if He's strong enough Himself?"  

"Because He wants us to try and be strong, just like He is." was my response.

"So because He's strong, He want us to be strong, too?" she surmised.

"Yes.  He does." I confirmed.

"OK" she said.

From there the conversation changed as quickly as it came.  

She found her answer and I found out that He was right...the Spirit will guide us in all things.

Johnny's Gift...(by Michael)

Both Lindsay and I have wonderful memories of cousins from our respective childhoods.

As I was growing up, I was surrounded by cousins from both sides of my family.  Traveling to Joliet, Niles, Evanston, Indiana and even Boston afforded me an opportunity to get to know my cousins and to play with them.

Lindsay had the great fortune of seeing her many cousins often as she and her family spent holidays and vacations together.  They forged lifelong relationships that permeate their existences today, bringing all of them a sense of pleasure, nostalgia, and love.

Ava, Henry, & Ella have their cousins, too.  From our hometown of Naperville to Chicago and out to Virginia there are special kids that surround ours.  Ages may vary among them but the connection they share with each other is indicative of the connection only cousins can have.

One difference between Lindsay and my cousins in comparison to our kids' cousins is that one of the members in our kids' cousins "group" has significant special needs...namely, Ella.  Her diagnosis of SMA has been part of the cousins' life in ways that we may not begin comprehend.  It's amazing to watch them as they interact with her, especially watching it develop over the past few years.  They are gentle, understanding, compassionate, and mindful to include her.  They do not allow her disability to interfere with their relationship with her and seemingly see right past it.

They can also be empathetic at times.

One of Ella's cousins (Johnny) who is just a few years older than her loves to play with Ava, Henry, and Ella.  He loves creative play and has developed a good sense of fair play.  As he engages in what cousins do best it obvious that he is enjoying himself.  But there's something that is not so obvious, at least not until now.

Empathy coupled with selflessness.

Apparently Johnny's teacher gave him and his class an assignment.  It was a rather creative, thought-provoking assignment (those are the best kind!).  The teacher asked the students to answer the following question:  "If you could be a gift to somebody, what would you be?"

Naturally one might assume that a child of Johnny's age might struggle a bit with this question.  It asks the child to step out of the "concrete" way of thinking and enter, ever so slightly, into the "abstract".  It forces the student to grapple with what a gift means and how they can impart that gift to someone else...it requires that the student step outside of the innate egocentric nature of children and enter the empathetic nature usually reserved for those older than them.   

Johnny took this opportunity to show us what cousins are truly made of...his response to the assignment was that he'd "like to be a 'gift of legs' for his cousin, Ella---because she can't walk and sometimes she feels left out when we are all playing and she can't do some of things we can do."

An incredible thought.  A compassionate act.  A demonstration of how children are so often the lights of this world...how they can be examples to follow.  

With that kind of love surrounding Ella, even from thousands of miles away, with that kind of love that only a cousin can impart, it's no wonder she thrives---despite the SMA.

Johnny & Ella
Christmas, 2014

Three Angels in our Lives...

A Kiss on the Cheek...(by Michael)

As our kids develop so do their needs, their interests, and their curiosities.  One positive aspect of SMA is that it does not hinder its victims mentally.  They are allowed to grow and mature alongside their peers and siblings, without missing a beat.

With this mental development come many aspects of the human experience.  Self-awareness and exploration being two of them.  Ella is becoming no stranger to these facets of life and she acts upon her new-found "mentality" full force.

Lindsay and I are still carrying Ella.  We carry her to the bathroom, we carry her upstairs, we carry her from the bath to the changing table, we carry her to bed.  We carry her downstairs to the basement for play, and carry her every time she needs to move and her wheelchair won't suffice.  While she is in our arms she often times takes the opportunity to give us a kiss on the cheek...sometimes several.  Most of the time words are absent from the kiss yet occasionally she'll tell us that she loves us.  

Her self-awareness that she is dependent on us for many things is growing and maturing.  She talks to us about the best way to hold her as we stand her up in the bathroom getting her ready for her business or just thereafter.  We try different ways and she knows that mommy and daddy do it in  slightly different ways...she's ok with that.  She is increasingly aware of her condition; often times speaking of how she can't wait for the doctors to "take the SMA away".  She expresses her anger and frustration about having the disease and will ask us why it has to be her.  Conversations ensue, often tears mingle themselves in, and invariable hugs close the conversation as we get back to the business of life.  

Her self-awareness is coupled with exploration in that she knows many of her limitations yet she is willing to thwart them in the name of conquering SMA.  She has a pride unmatched when she does something that she previously could not do or had lost along the way.  She allows herself to fail over and over until she finds the strength, agility, and process by which she can accomplish her goal.  When she is faced with a "brick wall" she simply asks for help, without shame, without reservation, without feeling indebted.  She knows we are here to care for her.

It's hard for us to imagine life without SMA...it has permeated our very existence and resides in our daily & nightly lives.  It's draining, to say the least, on physical, mental, spiritual and financial realms.  It takes so much more than it gives. Getting that heartfelt kiss on the cheek throughout the day helps ease the pain of SMA and provides a reminder of what it is we are to do.

There may be a day when they find a treatment or even a cure for this disease.  The entire SMA Community is sitting on edge as human clinical trials are currently underway.  Until that time though, we go through each day as it comes.  We do what we must in order to keep our child alive and well.   

Distractions...(by Michael)

She followed us all day long. She never really let us out of her sight as she whined about this and whimpered about that. We made sure a good amount of nutrition was given to her and that she was hydrated. We went about our daily routines hoping that she would find her way back to “normal” soon. We went for a walk thinking maybe some fresh air and movement would do her some good...to no avail.

Evening came, and soon after, dinner was coming to a close. She still acted strangely as the plates were being cleared, the glasses being rinsed, and the food put away.

“Maybe she needs a hug?” inquired Ava.

I squatted down to meet her at eye level and said, “C’mere Sasa”.

She ducked her tail under and nuzzled her face into my body. Lindsay, Ava, and I gave her hugs for a long time. She went from one to the other several times over the course of 15 minutes. Upon completion of the “hugging” session she was just fine.

I find it amazing how Ava so naturally knew that Sasa needed some hugs. Often times we overlook our pets’ basic needs in the name of raising our family...yet they are part of our family just as much as any other member. And while they do need food, shelter, and exercise, they also need love and the reassurance of the human touch.

We’re usually pretty good about paying attention to Sasa. Now that the kids are in the early years of school they are learning to appreciate all Sasa has to offer. They love her dearly and vice-versa. 

I suppose on those days when the demands of our life distract us from Sasa she really does feel it...and it took Ava’s innocence to bring that to light. 

Fill my Past...(by Michael)

I sat down next to Ella. She was on the couch, wedged in the corner for support, unable to really move from the spot.  We talked a bit, my iPad on my lap.  We played a game together (Angry Birds). My mail program "dinged" and I received a message from www.curesma.org.  The article was about the current clinical trials that are moving forward these days.  In a nutshell they have made wonderful progress with the intravenous injections of the gene therapy approach!  They are looking forward enthusiastically to the CFS (cerebral-spinal-fluid) injection approach...which has great potential.
This isn't really new news in our house.  The SMA Community looks eagerly to the folks heading up this research, our fingers crossed, prayer lifted, and hopes ignited.  

This hope...ignited by this research...is what keeps us going day in and day out.  We look to the promise of a horizon void of SMA; a sunrise of the day when this disease no longer robs our child of movement, function, and health.  We look to the prospect that she will know what it feels like to walk, unassisted.  We look to the time, that often seems elusive, when we can look at SMA as a memory.

Along with this hope comes despair.  Ella has come to realize some of the consequences of having SMA.  Namely that her life is so different from most people's.  Her daily routine is so different.  It's taking a toll on her.  She's expressing her feelings, rightfully so and encouraged by us.  She is sad, curious, and angry...sometimes all at the same time.  Moments unannounced capture our attention as she speaks her mind willfully about how she hates SMA.  Questions of "why" circling our conversations as of late...answers elude.  

Not only does SMA consume her motor neurons, methodically, intentionally, and without ceasing; it also consumes every aspect of our lives.  Each decision we make has been weighted one way or another by SMA.  Feelings get tied within its grasp, emotions get smothered in its clutches, peace of mind is obliterated by its insidious presence.  The void that was left after her diagnosis seemingly grows, enveloping my life perspective...taking me farther away from what seems "normal"; thrusting upon me a repetitive ache that resides deep within...unnamed, unseen, yet felt full force.

This juncture, of hope for a cure right at our fingertips and the daily maniacal routines that SMA demands, weighs heavily.   A juncture of unending promise not yet realized; of conversations not meant to be had with children, of watching a child--meek and fragile--fight the ravages of a relentless disease.  This juncture, I know, will be passed and fill my past.  Until then I can only hope, pray, and speak the truth to my kids about Spinal Muscular Atrophy.