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.