With Bear in Hand...(by Michael)

Scarlett is a friend of Ella's.  They share the same diagnosis.

With her bear in hand, bipap on 24-7, and the love of her family within, Scarlett has fallen ill.

She doesn't usually have her bipap on during her waking hours yet now that she is feeling ill it's with her all day and night, working to keep her breathing steady and true.  

The goal right now is to stave off pneumonia.  It can come so quickly to those affected by SMA...a simple cold, a cough, or a germ floating carelessly through the air becomes an enemy to our children, threatening their very lives.  

Scarlett's mom has reached out for prayers.  She has reached out to all who will hear to keep her darling daughter in their prayers as she and Scarlett struggle...

The season for colds, coughs, and flu is knocking on our doors...it's a time that we face with trepidation and caution.  Kids get colds.  Kids with SMA get more than they bargained for...

Stay strong Scarlett...

It Grabs Hold...(by Michael)

We never really know when our time will come to leave this earth, this life, everything we know.

We never really know when the time will be for our loved ones to depart.

There have been, however, several families this week in the SMA Community who have prepared for the loss of their child.

How does one prepare for that?

Spinal Muscular Atrophy strikes its victim unknowingly.  It creeps up on the very young and brings parents to a place unimaginable.

It throws situations at families that threaten to dissolve the fabric of their lives.

SMA grabs hold of daily interactions, holding them prisoner in its grip.

This disease is relentless.

Four children within the past week and a half have died and one more is on her deathbed as this is being written.

A child's deathbed.  An image that is disturbing to us all.  

A sigh fills my chest as I contemplate the reality of SMA, a thought that I'd rather not have to face.  It thrusts itself in front of me though...thrusts itself in front of us each and every time a child's life is taken by the disease.  

We pray for a cure.  We search for hope.  We fall victim to the emotion that fills our world.  We seek to hold our kids closer and closer every day in attempts to protect them from their ill-fate. 

The families who have recently lost their babies and the ones that are on the verge of their life collapsing with the inevitable passing of their precious children are in our hearts.  May your children, now delivered from the grips of SMA be set free by the grace and love of their creator.   May you find the strength to carry on in this world as you wait for the next to re-unite with your sweethearts.

May there be a cure for SMA.

Unpredictable...(by Michael & Lindsay)

SMA is unpredictable.  It leaves us guessing almost everyday.  Each night as we put Ella to bed we wonder what kind of night she'll have.  We have the guidance of machines to help us monitor her slumber and as she is getting older she can, and frequently does, call for us when she needs us.

Upon waking we wonder what the day will hold for her.  How will her strength be?  What will her level of frustrations be?  What successes will she have?  

As she plays we wonder if she'll be able to enjoy the toy(s) or simply throw her head back in utter frustration over what SMA is doing to her...slowly and methodically.

Or is it as methodical as we thought?

Ella was classified as a severe Type 2 in terms of her SMA.  The reason for this particular diagnosis is because of her rapid degeneration and loss of motor function early on in her life.  She is one of the roughly 20% of kids with SMA whom experience a rapid decline in their function early on...with the expectation of a plateau occurring later.  

She used to be able to play, "How big is Ella?"....""Sooo Big!" as she raised her hands over her head.
She lost that.

She used to be able to stand while supporting herself.
She lost that.

She used to be able to do a lot of things.
She has lost much.

Lately, however, we have witnessed no only a plateau but some actual improvement in some areas thought to have been lost forever. 

Her arms for instance.  She can raise them over her head again.

Her standing, too, has been an area that seems to be improving, albeit she is tailored chest to toes in braces, she supports her self on the parallel bars and bears some weight.

Even the motions of walking have made themselves apparent.  

It's encouraging and frightening at the same time.  It brings hope to the forefront and harbors the possibility of disappointment.  It fills us with meaning yet feels so distant.

The human body is an amazing piece of work...it's intricate, compensatory, and adaptable.  The human spirit, however, is the strength behind the body.  The human spirit, as it yearns to experience the world, brings with it a desire to persevere. 

We watch as Ella's body and spirit are challenged in ways that few can understand.  We watch as she fights for the opportunity to take a step, raise her hand, and move the body that God has graced upon her.  We watch as she stares SMA in the face and takes on the challenge it presents.

SMA Play Date (by Lindsay...sort of)

Today we were fortunate to spend part of the afternoon with a very special family.  A family who is quite similar to ours...

They live in Barrington, IL (a little over an our from our house).

They have a daughter, Isabella, who is 7 years old (just two years older than Ava).  They have a son, Gavin, who is almost 4 (just like Henry).  And their youngest daughter, Scarlett, is one (just 9 months younger than Ella).

Their youngest daughter, Scarlett, has another similarity with Ella.  She has SMA, type 2.

Scarlett's mom also has a blog for raising SMA awareness, and she wrote a wonderful post about our play date today.  So I thought I would post a link to her blog.

Wishes for Scarlett, "Play Date"

Also, here is a video of Ella showing Scarlett around our house... :)

"It's a Swammich"...(by Michael)

Captured moments.

Life has much to do with captured moments.  Whether these moments are captured in a photograph, a video, on paper, or in the memories made.  Capturing a moment allows us to carry that moment with us...forever.

Our lives traverse the course of time, being filled with moments that begin to define us.  We seek meaning from our moments and string them together to create our experience of the world, our experience of the life given to us.

The moments of our children's lives slip past us in the whirlwind of the every day.  It's often said that "they grow up so fast".  While this may seem to be the case it doesn't have to be.  It doesn't have to pass in the blink of an eye.  It doesn't have to feel like time has slipped away.  Capture your moments.

Capture the hugs your child gives and take one more.
Capture the laughter your child boasts and fill your ears.
Capture the tears your child sheds and ease them away.
Capture the silent concentration your child exudes and sit in wonderment of the human mind.
Capture the compassion your child has and feel the emotions.
Capture the words your child speaks and fascinate in their wisdom.
Capture the essence of who they really are and nurture that no end.

As the SMA Community sits in contemplation of the passing of two children this week (Baby Luke; 6-months old and young Isabel; 6 years old) we are starkly reminded that life is fragile; life is tender.

Capture the moments with those you love.  Take them in; hold them close; spend time with them forever.

Her World Transformed...(by Michael)

She could hardly contain herself.

She had a glow about her unlike any other time in her day.

She was supporting herself and did so for some time...longer than she ever had before.

The Ramirez family traveled from Iowa to Naperville, IL to build Ella her own set of parallel bars.  

From Iowa.

They too, have three children.  They too, have a life that includes SMA.  Their youngest daughter was diagnosed with SMA when she was two years old.  The doctors, of course, said that she (Madi) would never walk.   

They were wrong.  Madi does walk.  By herself.  She worked hard, fell often, and persevered.  She walks.  Not 100% of the time...but she walks.

When the Ramirez's arrived at our house they quickly got to work measuring Ella and figuring out the best configuration for her parallel bars.  In the meantime, Ella and Madi befriended one another on a closer basis than they had earlier in their lives when they saw each other at fundraisers.  They played together, ate popcorn together, and shared.  Ava and Henry joined in the fun!

Within 45 minutes the new parallel bars were finished.  We outfitted Ella with all of her braces and placed her between the bars--it was a perfect fit.  With help from Lindsay she walked along the length of the bars.  We could instantly tell that she loved the bars.  She was comfortable and more importantly, she was confident.  

Lindsay placed Ella's feet into the standing position and Ella grabbed the bars with her two hands.  Lindsay let go of her waist without Ella really knowing it.  With a little shift in her weight Ella realized that she was not being held.  She naturally pushed down on the bars to straighten herself up and was standing. Lindsay wrapped her arms around Ella without touching her to show her that she wasn't holding her.  Ella's eyes lit up and her smile filled the room.  She looked around at everyone who was watching.  For those few moments her world was transformed.

Transformed from being in the sitting position most of her waking hours to feeling the weight of her own body on her feet.

Transformed from depending on someone to make sure she doesn't fall to using her arms in concert with her mind to hold herself upright.

Transformed from hearing, "Would you like to stand in your stander?" to "Look at you, you're standing all by yourself!!"

Transformed from wanting to stand to experiencing it.

I also noticed the expressions on the Ramirez's faces.  I could see in their eyes the memories of their own daughter when she first took to the parallel bars they had made for her a few years back. The pride they showed for Ella was surely reminiscent of the pride they must have felt for their Madi.  As they stood in our family room watching Ella take herself from dependence to independence I could only imagine the experiences they must have had as Madi learned to walk.  I felt that they want those experiences for us, for Ella, and for all families with SMA.

We've known that the SMA Community is extremely supportive of one another.  We have experienced e-mails, texts, phone calls, and person-to-person help.  When one family with SMA reaches out a rush of several families respond...it's quite amazing actually.

From Iowa they traveled to Naperville, IL to build a set of parallel bars for Ella...so she might have the chance to learn how to walk.

Incredible.

Madi & Ella (& Sasa!)

Traces of Moments...(by Michael)

Every so often we get through the day without feeling tired.  Let me rephrase that...Every so often we get through the day without feeling exhausted.   Even that attempt at expressing the feeling falls short.

It's not a matter of sleep.  We wish it were.  That would be an easy fix.  If SMA was keeping us up at night with frequent sleep interruptions we could hire a night-nurse to take the overnight shift.  That's not the case, however.  Ella is waking up only twice, maybe three times a night.  She needs to be turned in her sleep and her feeding tube needs to be shut off and flushed.  All of those things take no more than a minute...no need to hire someone for that...we do them and go right back to sleep; just as if one were visiting the bathroom during the night. 

No, it's not a matter of sleep or even the quality of sleep.  We both find deep sleep through the night; we dream, toss and turn, and have a hard time waking from our slumber just as anyone else does.  No, the quality of sleep is sufficient as is the quantity.

What is causing us to be so utterly tired then?  

Stress.

Worry.

The lifestyle we now live.

Caring for Ella is physically demanding.  No other way to put it.  It's demanding of our bodies.  She weighs a good 20-25 lbs. now and the weight is basically "dead" weight.  Since her muscle tone is practically non-existent, picking her up, moving her, even holding her takes a lot out of us.  Yet, we lovingly to do these things in order to get through our day.  We lift her from her wheelchair to change her diaper, place her at the table for meals, get her in her stander, have her sit on the couch, give her a bath, put her in the car, and play with her, among other situations.  She now has a TLSO (Thoracic-Lumbar-Sacral Orthotic) that is cumbersome and adds weight--although it does provide some stability for her.  We have found though, that we have to be even more careful when we pick her up when she's wearing it so it doesn't interfere with her g-tube button.  She wears it through all of her waking hours.  Holding her has become more of an "art' than anything else.

Caring for Ella is emotionally demanding.  Watching your child suffer, deteriorate, and struggle is hard.  Watching them go through it minute after minute, hour after hour, day after day, week after week, month after month, and year after year is parental torture.  The instinct to want to fix the problem, control the situation, and relieve your child of the burden they are carrying weighs itself heavily on the emotional reserves.  There is no end in sight from our viewpoint.  While we may intellectually know that there is an end, emotionally speaking--there is none.  Our lives have been altered.  Not changed.  Not turned upside down.  Not even "taken a turn for the worst".  Altered.

The actual make-up of who we are and how we respond to life is not the same make-up from two years ago.  Our perspective on life, our expectations of the world, and our understanding of God is different.

Each day presents itself with a over-generous helping of exhaustion for us.  The pressure that SMA inflicts on a family never really leaves.  It changes form.  It works its way into every facet of daily life, making its presence known.  It challenges us spiritually, emotionally, physically, and financially.  It tears at our hearts and grapples at our souls.  It spends its time plotting ways to overcome our family, seeking every opportunity to destroy what we hold precious to our hearts, to our souls, to our very being.  It threatens the dynamics of our family, wreaks havoc on its primary victim, and attempts to thwart the determination and courage of love.

The SMA Community refers to those living and afflicted with the disease as warriors. For those who have passed, they are referred to as "angels".  The warrior connotation was disturbing to me at first, yet as I have witnessed my own flesh and blood in light of the disease housed in her body I can see the term "warrior" being an appropriate term.  

They fight every day.

They fight to move their body.
They fight to eat their food.
They fight to move across a room.
They fight to lift a crayon.
They fight to raise their hand.
They fight to hold their head up.
They fight to touch their hair.
They fight to clear their lungs.
They fight to sleep restfully.
They fight to breathe normally.
They fight to see tomorrow.
They fight to for their very lives.  Each and every day.

We're tired...along with so many other parents who have children afflicted with SMA (or any other disease for that matter).  We wake up every morning knowing that the day will hold fatigue in its grip for us.  Some days are better than others.  Some days are intolerable.  Some days are useless, and some days are productive.  Some days hold promise while others defeat us.  

It's a whirlwind of emotions that runs from one part of our hearts to another, leaving traces of moments passed...

each one promising to bring us 

closer 

to 

God.

 

  

We Have Found..(by Michael)

 She was baptized into Christ as Ella Sabine Casten.

The congregation promised to care for her.

She was welcomed into the family of God through St. Timothy Lutheran Church of Naperville, IL.

We knew nothing of SMA.

She was beautiful in her baptismal dress; her eyes wide with wonder; her temperament even and pleasing.

We knew nothing of AFO's, KAFO's, TLSO's (the various bracing devices she now wears) or how incredibly patient and bright her soul really is.

She was held and carried by us, by the clergy, by friends and family because she was so young.  That's how she moved in the world--as any other baby does.

We knew nothing of micro-wheelchairs or Permobil power wheelchairs; not to mention the loads of equipment (from tiny syringes to a massive, mechanical stander) she requires to enhance her quality of life.

We were surrounded by a love that is rooted deeply in the love of Christ.  The Church and its people, witnessing the ceremonial baptism of Ella, pledged to care for her.

And so...they do.

Upon learning of Ella's disease the congregation of St. Tim's has consistently fulfilled their promise to Ella and to us.  They have brought an inner peace that fills us when we attend.  They have brought relief and comfort, good words and deeds, prayers and consolation as we wrestle with ourselves, our God, and the meaning of it all in light of the terminal illness that has stricken our gorgeous Ella.

In efforts to provide us with love and support, parishioners gathered together and brought a fundraising event to the church to benefit Ella and her immediate and long-term care needs.  The event was unlike any other we had seen before.  It was a Zumba Event...led by a professional Zumba instructor and designed to bring in people to support the growing financial burden SMA has inflicted upon our family.  

People of all ages attended.  People of all ages "Zumba-d".  People of all ages enjoyed an afternoon of fellowship and goodwill.

Each Sunday, at the end of our worship service, the clergy asks the people, "People of God, what are we called to do?" and we answer in unison, "Live the Love of Christ".  Those are not merely words to our family, they are the absolute truth that we have found in our church. 

Thank you St. Timothy's... 

 

The Lights for Us...(by Michael)

They've been wanting to hug us more often.

They want us to sit with them.

Their attachments for us are growing, deepening, and expanding...as is ours...for all of them.

We have three beautiful children who are so very important to us.  We are spending more quality time with them as they grow older. 
 

As Ava learns to read she knows there is a time each day that she gets one of us all to herself.  She delights in dancing and finds freedom in flowers.  Her creativity is nurtured with coloring and she dazzles us with her drawings. 
Her attributes as a big sister are admirable since she takes her brother and sister under her wing much of the time.

 
 As Henry explores his world he thrills in the prospect of showing us his discoveries. He finds pleasures in running and excitement in going places.  He loves his toys and has weekly, even daily, favorites.  His energy is boundless and he is learning how to harness it.  He has a laugh that's contagious and a sense of humor that might give his teachers a run for their money!

Ella, on the other hand, is approaching her toddler years differently...she has to.  She obviously does not follow the typical physical patterns of a toddler's life. 

SMA has stolen that from her.  

She responds to this theft with an undeniable will to capture the hearts and minds of those she loves.  She uses her wit and intelligence to invite people into her world.  She amazes those, close to her and strangers alike, with her abilities to work around the disease that constantly ravishes her muscle neurons; killing them slowly and methodically.  She struggles with the desire to run and jump, knowing she can do neither.  And she solemnly watches as Ava and Henry do so.  She prides herself in accomplishing the things that so many of us take for granted...and rightfully so as her path is not an easy one to travel.  She fights with her siblings and tests her parents.  She cuddles with her dog and makes messes of her toys.  She asks questions and sings along with songs.  

As Ella grows older alongside of Ava and Henry, Lindsay and I find ourselves talking about the future more than most people probably do.  Right now it is so uncertain for us.  We are eager to have an environment in place so that we can provide them with the best life possible, yet we don't know what that looks like.  SMA presents a challenge that simply never entered our minds when planning a family...why would it?  This challenge, however, is now present.  It is now part of our lives.  It calls on us to reach deep within ourselves and confront parts of our souls that, until now, had lay hidden.  We have some major decisions to make and often waver on where or how to begin. A see-saw of emotions, practicality, finances, and indecisiveness creates a depth of uncertainty that drains energy and taxes the spirit.

The one, or rather the three, aspects of our lives that allows us to persevere is found in the love we feel from our children and the love we have for them.  

We are fortunate parents to have been blessed with the lights of Ava, Henry, and Ella. 

One Foot in Front of the Other (by Lindsay)

I'm excited.  Really excited :)  

Less than a week ago, at Ella's clinic appointment at Lurie Children's Hospital, we were told to practice the walking movement with Ella.  

So we have been.  And she's learning.

Basically we've been holding her around the waist and shifting her weight for her while she attempts to slide one foot in front of the other.

But today her therapist let us borrow her set of parallel bars to try out at home.  Ella couldn't wait to get home to try to stand with her "bars".  

Well, she blew me away, as usual.  I put her extra braces on for the added support and the moment I put her between the bars, she knew exactly what to do.  She immediately placed her hands on the bars and stood.  By herself.  With no problem whatsoever.  

Then I came behind her and held her waist like I have been, but this time she started to shift her weight herself.  I still had to hold her for stability, but she initiated the motion.  She continued to "walk" along the entire length of the parallel bars.  While I supported her, she shifted her weight and kicked her legs, one in front of the other.

Go Ella!!