One Month to 13 Years...(by Michael)

They come in all ages.  

There's boys and there's girls.

They come from different regions of the U.S.

They have varied interests, are learning different things in school, and their personalities are distinct.

The oldest is going to be 13 years old in February and the youngest just recently celebrated one month with us.

Ella, Ava, Henry and their cousins enjoyed each other's company this holiday season.

They played games the old-fashioned way using board games, pieces, and strategies.  They played games the modern way using controllers, the TV, and strategies.  They ran (and wheeled) around the houses, sat at the kids' table for meals, and played carefree in the snow.  They enjoyed the dogs that surrounded them, took part in the holiday treats as they became available, and had their quiet moments (every so often).  

Each adult seemed to find some time to spend with each of the children in one way or another.  Laps were sat upon, knees were ridden upon, and tickles were doled out.  The baby was held, passed around and fussed over.  

Gifts were exchanged as excitement filled the air.  Hugs and kisses, handshakes and pats-on-the back made themselves known throughout the time spent together.

Ella spent her time enjoying the family that surrounded her.  She laughed and giggled endlessly.  She spent much time with her baby cousin, Caroline.  She showed off her skills at standing as she has been working hard on them as of late.  She brought smiles and wonderment as she divulged her exceptional personality to those who hadn't seen her in some time. 

Cousins present a special kind of relationship.  They afford the pleasure of having a friend to play with no matter what the age.  They bring to the table their unbridled humor, their skills and talents, and their unchecked personality traits.  They take care of one another without being told to do so; they share, they make room for, and they develop lifelong bonds with one another.

The excitement of getting together with cousins can only be matched by the time spent with one another. 

Joe, Ava, Sami, Ella, Henry

Johnny, Ava, & Henry

Conner, Johnny, and Henry

Caroline & Ella

Ella & Caroline

Quiet Pleasure...(by Michael)

Eighty years separates them, yet they speak to one another joyfully.
His child and her parent are the link between them, yet there is more than just common DNA.
In the background sits a piece of furniture that belonged to him for 35+ years, it sits in her house now.
She sits in her modified chair and he sits in his wheelchair.
They share a moment on Christmas Day...they're alone in the dining room after Christmas Brunch.

Ella is surrounded by so many loving family members.  She delights in visiting with her maternal Gramma and Grandpa at their house and dances for joy when she knows that they are coming to visit her.  She loves dearly her Auntie Annie and Uncle Noah along with their newborn little girl, Caroline.  She screeches with delight at the prospect of seeing Auntie Sarah, Uncle Brad, and cousins Conner and Johnny.  Her eyes light up when Mary and her dog, Link come to visit.  Her playfulness comes out when visiting with Uncle Andy, Aunt Beth, and cousins Sami and Joe.  She is surrounded by wonderful family members.

She has one more family member...Papou (Greek for grandfather).  Father of Michael, Papou lives alone in an assisted living facility.  Ella's Yiayia (Greek for grandmother), Papou's late wife,  passed away during the summer of 2008, almost two years before Ella's birth.   Papou sees Ella considerably less than much of her extended family, yet...she loves him with a special love that only a granddaughter can bestow upon an aging Papou.  She doesn't get outwardly excited, she doesn't sing and shake herself, she doesn't screech in delight when she knows she will be seeing Papou...instead, she seems to have a quiet pleasure about her.  She wants to be with him.  She wants to talk to him.  She wants to know him as much as a 2 year-old could know an 82 year-old.

Ella and Papou spent a short time together on Christmas Day.  It was, however, quality time that brought their two souls closer to one another.

He Exists...(by Michael)

Our kids believe in Santa Claus.  Why shouldn't they?  He exists.  

He lives in the hearts of those who have prayed for us.

He resides in the corners of the communities that extend their collective hands to us.

He takes his place in the reassuring nod or wink one of us may receive at any given moment.

He falls into our lives when e-mails find their way to our computer offering encouragement.

He swoops in as strangers generously give to our children.

He leaves his mark when a hug is exchanged or a shoulder is leaned upon.

He exists.  In so many ways.  

As Christmas Day brings itself closer to us we, as a family, have been reminded that this has been quite a year...not just for Lindsay and myself, but for our children as well.

Ava and Henry have struggled with the emotional waves that bombarded me and Lindsay.   They have been shuffled from one caregiver to another in a moments notice. They have been challenged, at their tender young age, to figure out how to best cope with having a sister who has special needs.  

Ella has worked so hard at simply surviving.  She has been hooked up, plugged in, and monitored more than most people have in a life of 85 years.  She has been forced to reconcile her inability to move her legs and other parts of her body as she watches kids her own age run free.  She has battled frustrations that are compounded on top of the typical frustrations of a two-year old.  She has been working on coping with her special needs.

The holidays are different this year than last in the simple fact that we are in a better place than before.  We have done considerable growing and accepting of what is.  

Our kids are miracles for the simple fact that through the past year they have been through so much...we are grateful for the Santa's out there that truly exist.

Our kids believe in Santa Claus.  Why shouldn't they?  He exists. 

In Light Of...(by Michael)

I sat on the edge of Ella's bed; the room illuminated only by the light of her bipap machine.  I just finished hooking up her feeding tube and started the overnight continuous feed.  As per usual Ella took my hand and "charged".  We sat in silence.  I pressed a button on the feeding pump to see how much has entered her body and double check the rate...10ml had gone in at a rate of 50ml per hour...perfect.  Then I noticed it.  The battery-life indicator was on yet the pump was plugged in.  I looked at the adapter on the outlet end and the little green light was not on...I adjusted some things...no luck.  I called Lindsay.  We adjusted some more but had no luck.  Ella watched us gleefully.  We ended up calling the Home Health Care...they didn't have an extra adapter available until the next day and advised we use the "gravity-bag".  We tried.  It didn't work.  We ended up doing manual feeds with a syringe throughout the night.  And so it goes.

Ella's cough has returned.  With it came another vomiting episode after her afternoon feed...sparked on by a cough that caused her to gag and let loose.  We'll try one pump feed in the morning and see what happens; if she doesn't hold it then we have to go back to manual syringe feeds every hour.

While the feeding can be cumbersome it's the cough that concerns us most.  Often times when a cough is over and then followed a few days later by another one the second round can be worse.  The longer Ella fights a cough the weaker she gets.  The weaker she gets the more vulnerable she becomes.

We continue to do her therapies which include chest compressions with the vest, cough assist with the machine, and suction afterwards.  We encourage her to cough on her own.  We do the best we can.

In light of recent events of the world we cling even more tightly to our children.  We know that life is fragile, unpredictable, and sometimes fleeting.  We take heart in looking deeply into the essence of our children and seeing them for who they are.  We've learned to take every moment as it comes and to be grateful for those moments.  We encourage, we teach, we raise our babies as our parents did; with love and respect.

It may be a long winter ahead...

From One Minute to the Next...(by Michael)

Daddy, Ella, and Dr. Kuntz

 The world of SMA can run emotions high and low all within a matter of minutes.  The reality of the disease, if allowed to be pondered for what it is and does, can remove you quickly from this world and hurl you into a place that is deep, dark, and full of anguish.  On the same note, the will and determination of those who suffer from it can bring you to a high place full of light and life, eliciting hope, compassion and perseverance.

At Ella's quarterly multi-disciplinary appointment (Lurie Children's Hospital) we sat in the examining room waiting for Dr. Kuntz and her team.  They are extremely efficient and we are never waiting for very long.  Ella, delighting in being there as she anticipates the visitors and seeing her friend Scarlett, is busy wheeling around the room.  Lindsay and I take a moment to check our phones in an effort to stay connected to the outer world.  And there it is...an SMA reality that has become all too common of an occurrence...another child has passed away from SMA.  That would make five kids in the past ten days.  5 in the past 10.

I look over at Ella.  She spies my gaze, gets herself over to me, and asks to play a favorite wheelchair game called, "Rock and Roll".  We commence.  The faint knock on the examining room door stops our game as the rounds begin.

Our reports to the various therapists and doctors are of Ella actually improving in many areas.  We proudly report that just recently she beat a cough of 10 days and a fever of one day without a trip to the hospital.  We sit and watch as she dazzles her visitors with her conversation, her wheelchair skills, and her physical improvements...namely her walking (with Lindsay's assistance).   We find ourselves reporting that she is doing well and are told to keep on this path since it seems to be working well for her.  

All in all it was a very good visit as we walked away just as we walked in...in other words, we don't have to change much of what we presently do.

Ella examining her baby doll

In light of some of the recent developments in SMA research for a cure and/or treatments we have shifted our focus with Ella a bit.  If indeed the medical community can successfully treat or even cure this devastating disease within Ella's lifetime, we want her to be as ready as humanly possible to accomplish as much as she can with whatever they have to offer.  While she may never regain what she has lost she may very well be able to work quite well with what she has left...only time will tell.

If they can stop the progression of the disease then maybe we can strengthen what she does have.  This is our vision.

When our appointments were over we visited Ella's friend, Scarlett, who was also there.  Both girls were excited to see one another and it was sweet to see them interact.  They talked, and more importantly, they laughed with one another.

Gavin, Scarlett, Scarlett's Mom, and Ella

We know that Ella is doing well right now...we are so very grateful for that.  We know that every case of SMA is different.  We know that every family who faces this disease has challenges beyond words.  We also know that so many people have cause for sadness and despair as they face the lifelong healing that comes with losing their child.  

The Holiday Season calls for grace, compassion, comfort, and joy.  It calls for each of us to look at more than just what is happening with our own lives and extend ourselves to others.  As we traverse through this season we look to God and thank Him for the life that he has given to us in Ella and at the same time we ask that He cradle, in His arms, those who have lost their children to SMA.

Woven Together...(by Michael)

   

With each day as they grow they cling more tightly to one another in so many ways.  

Ava delights in being the big sister to both Henry and Ella.  Yet Ella affords Ava that opportunity to be a big sister to a girl.  Many of the things Ava likes to do are taken up by Ella; I can see many of Ava's mannerisms in Ella.  There is definitely a bond forming.

Throughout the day, of course, is the bickering, the bothering, and the annoying habits we've all come to know too well.  There are the time-outs, the scoldings, and the teaching of apologies and forgiveness.  

Woven into the waking hours are moments that might melt one's heart.  The girls playing make-believe together, sharing their coloring supplies, or getting their nails painted by Mommy.  Ava takes off her "bossy" hat and puts on her "sweet" hat.  She talks with Ella and helps her to do things.  She teaches her about the world and allows Ella to explore it.  She runs to her aid and defends her when necessary.  

At the end of the day, it turns out to be a typical sister-sister relationship.  However, with Ella having SMA and being confined to a wheelchair and as fragile as she can be, Ava has taken the "typical" and customized it to fit our family.  She has shown herself to be a sister that Ella can count on, a sister that will always embrace her, a sister that will stay with her...forever.  

Lest We Forget...(by Michael)

The bracelet above was made for a mother.  Her child, Avery, passed away due to SMA.  Like for so many children, SMA took her life quickly.  Mama Bracelets (owned by Lindsay) made a memorial bracelet for Avery's mother.

The picture shows the "Cure SMA" Open Circle in the forefront with the charm that reads "Avery" in the background.

Avery was diagnosed with SMA Type 1; this type is considered the most severe type of SMA and depending on the level of severity, the life of a child can, and often does, end before two years of age.  There are kids with SMA Type 1 that do beat the odds...their lives are spent with very little if no muscle function.  They are surrounded by the love and care of their families who do everything they can to keep their kids alive and happy despite SMA.  Regardless of which type a child has, SMA robs them, and their families, of so much. 

Avery gained much attention during her short time on this earth.  Her parents had a "bucket list" of things they wanted Avery to accomplish while she was alive.  Her popularity grew and then suddenly...she was no longer with us.

Her death brought her story to an even higher level.  Along with her, SMA also was now the spotlight...the disease that is so rare yet so devastating was on stage.  People from around the country, and the world, were touched by Avery and her bucket list while at the same time learning about the disease that took her young life.

People learned that SMA was named the #1 neurological disease closest to a cure of among 600+ diseases according to the NIH (National Institute of Health).  People became aware of Dr. Kasper's Gene Therapy and the goodwill donations came pouring in to his program.  Hundreds of thousands of dollars in a very short amount of time was raised to help fund Dr. Kasper's research and, in turn, helped get it launched into human clinical trials.

Simply stated, Dr. Kasper's approach to SMA is to virally infect people who suffer from Spinal Muscular Atrophy with the SMN gene that they are missing.  Once they have this gene, it is hypothesized that the disease will no longer exist in that person.  Hence, a cure.

We move forward with a jump start to our hope.  We look to the future with new eyes in light of the prospect of curing this awful disease.  We take a breath knowing that anything can happen.

We must also, in the midst of it all, take moments to keep Avery and every other person who has died from SMA, along with their families, in our hearts and minds.  For through their struggles, their anguish, their perseverance, and their strength we are here..at this point...on the verge of what we all hope for as a breakthrough.

The charm on the bracelet pictured above is in the background, just as Avery is now...but it's there, no mistaking it, it's there...and so is Avery along with all those who passed before her...they are with us as well...holding hope close our hearts.

She's Ours...(by Michael)

When we walk in the house, she's there.

Throughout the night she checks in on each one of us.

When we celebrate by opening gifts she celebrates along.

When a stranger comes to our house, she warns us in her unending protective nature.

She knows when we are happy; she feels it when we're sad.  Her affections for us are boundless.

She's our dog.  Her name is Sasa. 

Sasa and Ella have developed a bond unlike any other in our family.

Ella watches her eat and Sasa always returns the favor.  Ella gives her hugs and Sasa delights in them.  Ella often requests to sit with her and Sasa always makes room.

It's a beautiful spectacle to witness when a young girl, confined to a wheelchair, and her lifelong companion, her dog, interact.  Ella adores Sasa with all of her heart, all of her soul...and Sasa seems to have been made for Ella.

A tribute to our friend, our companion, our protector, our one-and-only...Sasa. 

 

The Best Place (by Lindsay)

Right now, things are ok.

Actually they're better than ok.  I feel like I am in the best place that I've been since Ella's diagnosis.

I'm so much closer to accepting that SMA is part of our life.  Do I like it?  Of course not.  I hate it.  But I'm starting to accept that Ella has this disease and we can't change that at this point.  And I'm really starting to go on with life.  And it's becoming "normal" for us.

And then there's the eating disorder.  At this point, I'm very close to being considered "recovered".  I only have one more session with my dietitian and one more session with my therapist.  They both feel that I'm ready to move on (as do I).  It's a wonderful feeling.

But I think the biggest reason for being in "the best place" is because of our hope.  Ella has continued to improve, which is not something we planned on after hearing her diagnosis.  Children with SMA do not typically improve, rather, they usually get worse.

And Ella got worse for a while.

But her improvements lately have given us a new hope that we never had before.

Here is a video of Ella from a year ago.  As you will see, she is unable to lift herself up and struggles to lift her arms even a small amount.

Now here are two videos showing what Ella has been able to do in the past two months:

And finally, here is Ella's latest achievement.  Today she was able to take one small step while only holding my hands!  This means she was bearing her own weight and only using my arms for balance!  This one little step is a really BIG step for her progress!!

We're not really sure why Ella has been making this wonderful progress in the past few months.  

We think maybe her diet might be playing a role.  She started this Amino Acid diet last March, but she was throwing up at least one feed every day.  It wasn't until August that we finally figured out the vomiting was due to acid reflux, so we were able to correct the problem.  Since then, Ella has been getting her full nutrition every day.

Another change since September is that we have been working on walking with her a little bit every day.  This has probably been building strength in muscles that she hasn't used in a long time.  

Whatever the cause, we are filled with so much happiness watching her make these improvements!

This year, the holidays are extra special for me.  

In the past, the Christmas season has always been my favorite time of year.  But last year was different.  Last year was our first Christmas since Ella's diagnosis.  And I didn't have much joy in my heart.  I basically wanted to skip the holidays altogether.  

But this year, I couldn't WAIT to start the holiday season!

This year I'm filled with joy.  I'm filled with love.  I'm filled with hope.

Take a Breath...(by Michael)

The day that kicks off the holiday season has once again come to a close.  From here on out minds of all ages turn toward other delights.  We venture to the celebration of Christmas, taking the holiday season as a way to rekindle our love of humanity.  The rituals, festivities, and traditions of the season accentuates the meaning within them.

Thanksgiving provides us with a time to surround ourselves with those close to us; to celebrate all that we have in our lives.  It allows us to sit in contemplation of life itself.  It prepares us to enter into the holiday season with hearts and minds full of generosity, goodwill, and peace.

Thanksgiving paves the way for the winter seasons' celebration of Life and Light.  It carves a path for the sharing of good fortune, the extending of a helpful hand, and the realization that life itself is a place in which we can take solace.    

Thanksgiving affords us an opportunity to look at our childhood and remember the warmth that we felt being loved, if only by one person.  An opportunity to see the delicacy of life, in those we have known for so many years, as we watch them grow old before our eyes.   

Thanksgiving gives us the seat in which we can sit in reflection of our own lives; where we have been, where we are now, and where we might be going.  

Thanksgiving takes us by the hand and promises to lead us into a world full of love; sending us forward yet always with us as we welcome the good news of our Savior and the goodness that lies within us, which lovingly spills out to others.

Thanksgiving fills out souls with the realization that we are all one.  It gathers us together while allowing our individual selves to flourish among those we love as well as among those we do not know.  

Thanksgiving brings about the laughter and stories, the sharing and caring, the spice of life that threads itself throughout the rest of the holiday season.   

Thanksgiving allows each of us to turn to ourselves and reflect upon what it is we are really doing here.  To reflect on the innocence of childhood, the complexities of adulthood, the mysteries of the after-life, and the wonderment of the gift of life.

As we all head away from Thanksgiving and into the holiday season take a breath as you journey, over the next few weeks, to fill your body and soul with the gift of life itself.  

The promise of Christmas is honored by the sentiments of Thanksgiving and the essence of Life and Light is brought forth by its grace.

I Just Want To...(by Michael)

Our kids have developed a response that they have shared with one another.  It seems to be a response that begins at about age two and dissipates at around five.  Therefore Ava has abandoned the response (for the most part) while Henry and Ella are full force in the throws of it.

"I just want to" is often heard around our house.  They ask for things and receive the "no" response and instantly the "I just want to" flows seamlessly from their mouths, dressed lightly with the perfect amount of toddler whine.

There are times, however, when the "I just want to" response does not elicit a hidden eye roll from me or Lindsay.  These times are actually accompanied by some distress on our part.

Ella watches Ava and Henry.  She watches them closely.  She is a bright two-year old and she knows what she wants.  She is just now beginning to understand that her SMA prohibits her from doing many of the "things" Ava and Henry enjoy.  Nonetheless, when her two siblings are excited about playing some game, or cruising effortlessly down the stairs to the basement, or throwing themselves recklessly onto the couch in the name of childlike fun, Ella wants to join in.

She "just wants to".  It breaks our hearts every time.  I suppose the heartache that accompanies her plea to engage in the behaviors that she sees Ava and Henry enjoying prompts us to eagerly find ways to accommodate her...sometimes with success and other times without.

I took Ella to Target.  It was just me and her.  The whole way there she talked of wanting to ride in the "big cart"...you know the one...it has a place for kids to sit attached to the back (see the pic above).  She has never been able to or allowed to sit in the kid part of these carts...simply because she lacks the strength to hold herself up safely.  For her entire life she has watched as Ava and Henry, with absolutely no hesitations, go back and forth from enjoying the "big cart" ride to walking/running down the aisles.  Ella always was seated in the cart, wedged safely in the "old kid portion" of the cart.  She wasn't even able to have her legs dangle for she might tip suddenly.

The entire ride there I kept telling her that we would look for a "big cart" but might not find one (it was my secret hope we wouldn't find one so I wouldn't have to say "no" to her...again).  When we arrived there was one right there, just waiting for us.

"I just want to" she said.

I thought we might try it.

It worked.  She sat in the "big cart" with no problem whatsoever.

She's quite the kid, our Ella is.  All of our kids are...

The night before the "Target Trip" we were at a birthday party.  Henry decided to play a game with Ella...I've never seen the game they were playing but was awestruck at how he took her under his wing and brought her to the world of play despite her being confined to a wheelchair.  His ingenuity and compassion was tearfully evident.  Ella is a lucky little girl to have such a big brother as Henry.  I don't think I heard, "I just want to" once from her while at the party...both Henry and Ava made sure that she didn't have to sit idly by and watch, wish, or whine...

Enjoy the Henry and Ella "Wheelchair Fun" video...

Unchartered Territory...(by Michael)

I walked into Ella's room to get her from her nap.  Something that Lindsay and I do everyday.  There's a "ritual" to the whole process.  Walking into the room the stand-up fan is turned off, then one gets to the side of the bed and turns off her pulsoximeter.  As she lay on her bed patiently we proceed to "disconnect" her from the bipap machine.  She asks to "sit up" and we cradle our hand gently behind her neck, providing the necessary support, as we push her up and into the sitting position.  The cord that leads from the pulsoximeter to her big toe is taken off.  She's free from the constraints that SMA has placed upon her as she slumbers.  She sits in her bed and makes her request to charge (for those who don't know what her "charge" is; it is when Ella pinches [with her index finger and thumb] the skin in-between your index finger and thumb).

On this particular day when I went to get her from her nap and the ritual was complete up to the point of "charging" I sat on the bed with her...that's when it happened.  It was short-lived but took me by surprise.

The feeling I had was quite intense...

It was a feeling of being so utterly tired of her having SMA.  Her hands, fingers, feet, and toes are so small.  Her arms and shoulders are tiny.  The limitations placed upon her never seem to go away.  I felt bad for her.  I wished I could do something, anything, to take SMA away from her.  The depth of my parental love, protection, and self-sacrifice ran into unchartered territory. It brought me to a place where I have never been before. 

And then it happened again...as quickly as it came the feeling fled and was replaced...

I saw her for who she really is.  I saw into her eyes, her face, and her beauty.  I watched as her smile opened up her soul and the brilliance of her essence filled my heart.  I became amazed at the incredible progress she has made as of late...I found myself in awe to be her daddy. 

If you have kids...hold them close to you every day.  If you have no kids, hold those you love close to you everyday.  If you find yourself alone, hold yourself in the warmth of who you are.

Yes, SMA may place, without reservation, limitations on my little girl.  These are only physical limitations though.  She fights through them each and every day.  

And while SMA may claim what it can, it will never claim her. 

"Can I come back?"...(by Michael & Lindsay)

They stick together.
They play together.
They fight together.
They talk with one another.  About everything.

We were driving home from spending an evening at church when I came to a red light.  Ella took my attention from the road and asked me, "Where do I go when I die?"

My world focused on her face.  I asked her to repeat the question just to make sure I heard her correctly.  I had.

My response whizzed its way from my head to my mouth and before I knew it I was telling her that she'll be with God as He holds her close in His hug.  She thought for a moment.  Pulling her finger away from her mouth she returned her gaze to mine and asked, "Can I come back?".  We stared at each other for a moment.  I spoke.

"If you want to" I said.  The car behind me let out a friendly honk to let me know the light had changed.  Quickly Ava, Henry, and Ella began talking about heaven (as they often do) and began planning for all the wonderful things that will be there...waiting for them.  I drove replaying the conversation I had with Ella over and over in my head.

Those two questions:  "Where do I go when I die?" and "Can I come back?".  They struck something deep inside of me as my mind brought itself to wonder why they lurked in her head.  Why did my two year-old daughter who faces a terminal illness ask me those two pointed questions?

At first I thought the first question was more important to focus on than the second.  Amidst a discussion with Lindsay I now think otherwise.

Ella has changed.  There is something different about her.  She is bringing to our world an inspiring display of courage.  Raw courage.

The change has manifested itself in the incredible improvements she has made over the past few weeks.  She has stood with the parallel bars by herself, supported by her braces yet bearing her own weight.  She has walked with assistance the length of those bars (six feet).  She practices the motion of walking with our help outside of the parallel bars wearing just her AFO's and TLSO (no knee immobilizers).  She has rolled from side to side on a hard surface.  She lies in bed and brings her legs up in order to grab her toes; delighting in the childlike wonder of controlling her body.  She has even once rolled herself from being on her back to her side while sleeping! 

Her courage to attempt these feats and face the risk of injury and disappointment runs deep inside of her.  She does fail at many of her attempts yet she gets right back up and goes for it again with the expectation that she'll get it this time.  It's paying off as we are seeing a change in our Ella.  How long it will last; the reason behind it; how far it will go all remain to been seen.

The question, "Can I come back?" is important in relation to the question, "Where do I go when I die?" because it tells me that she's not ready to die...as inviting as being hugged by God is, I think she knows she isn't finished here on earth.  

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.