Spinal Muscular Atrophy (SMA) Type 2...

Ella Sabine Casten (14 month old)

Ella has Spinal Muscular Atrophy (SMA) Type 2.

The diagnosis was confirmed on August 12, 2011.

Her motor neurons are dying and will continue to die.

For Ella, her SMN1 gene (located on chromosome #5) is not producing a protein called Survival of Motor Neuron (SMN).  This protein is responsible for keeping the motor neurons alive.  She produces some (through copies of the SMN1 gene that mother nature has so brilliantly provided), but her copies are not sufficient to do the job as well as the original would have.  Her physical growth and the demands placed upon her muscles will far outweigh what she can produce.

As increased nerve denervation (loss of nerve supply) occurs, she will grow weaker over time and her muscles will atrophy from non-use.  This rare genetic disease is in the same family of diseases as ALS (Lou Gehrig's Disease); only it is not as aggressive.

Currently her legs and trunk are the most severely affected.  Other muscles in her body will be affected over time.  This is a progressive, degenerative disease.  Currently there is no cure.  Prognosis is unknown and varies widely for children diagnosed with SMA type 2. 

What do we do now?

One thing we do know:  Her respiratory system must remain healthy and strong; this is vital to her survival.  Keeping her intercostal (rib) muscles strong to cough hard enough to expel any congestion is the key to preventing infection from setting in.

Our minds are filled with what we are to do.  And those things will happen.  The family education, the orthotics, the doctor appointments, the adaptive equipment, the modifications to home, car, life, the insurance, the medical bills, the support groups, and so much more.  Those things will happen.

I guess the question that really needs to be asked and answered is not "what will we do?" but rather, "What will we BE?"

It is in the "being" that we will produce the highest quality of life for our family.

It is in the "being" that we will find out who we truly are.

It is in the "being" that we will find the strength to do the things we need to do.

Our choices seem endless.  There are so many ways to "be" in relation to all of this.   The spectrum of emotions can be overwhelming at times; the expression of these emotions equally so.

There is one choice, however, that really exists.... 

...it is the only choice we really have...

...it is to love her.

Ella is going to need much in her lifetime.  She is going to need a lot of help to find her way to the path of greatest independence.  She is going to need much in order to survive as long as she can.  

What she'll need above all and what we can offer her in abundance is love.

We can do that...

 We have started a new blog titled, "Ella's Corner" that will follow her as she lives with SMA.  

Ella's Corner blog address:
http://ellas-corner.blogspot.com

My Life is Full...

This is one of my favorite pictures of me and Lindsay. 

I remember when we took this picture.  I love how we are hanging on to each other.  

I love my wife.

On August 11, 2011 we will have been married for 5 years.  As I reflect on what has happened over the course of the past five years, I can see why a picture like the one above is so comforting to me.

Lindsay has been my absolute best friend.  She has walked the past five years with me with incredible love offered daily.

Our wedding was picture perfect.  We loved the entire day from start to finish.  Lindsay carried herself with grace and dignity; with love and joy.  We were ecstatic from the moment we woke to the moment we fell asleep.  We wasted no time starting a family...no time at all!

The birth of Ava was incredible...our first child.  Raising Ava has been one pleasure after another...and we are eager to experience more of the same as her future unfolds.  She brings joy and laughter to our days.

Our boy Henry coming into the world proved just as amazing.  We had a girl and now we had a boy!  The bond that Henry has formed with us has drawn us so very close to him.  He keeps us close to him and enters our hearts daily.

The arrival of Ella completed our family.  The sense of fulfillment, completeness, and joy felt by us when we first held her let us know that our family was here.  Each moment spent with Ella fills us with love and adoration.  She draws us close to her and to each other.

Through the birth of our children Lindsay has shown me that life is more precious than I ever imagined.  The strength she has is inspirational.  Not only did she carry our three children and bring them into this world, she has carried me when I needed it most.

We witnessed our 3 children coming into this world in a matter of 4 years.  In those same four years we have watched 4 close people leave this world;  Tessa Casten, Henry Gibson, Sabine Casten, & Julia Koclanes (Casten).  

Through those losses Lindsay has stood strong with me.  Holding me when I felt weak, crying with me when overwhelmed, laughing with me over memories, keeping me in reality as I ventured away from it seeking answers.  She shared her thoughts, her emotions, and her fears with me. All the while she took impeccable care of our  family.

Lindsay is a beautiful person.  Her love of life, and for the people who make up our lives, holds true forever.  

I suppose I could write a book about what we have experienced over the past five years...for now though, I leave you with a poem that I offer to my wife on our 5th wedding anniversary.

My Life is Full

When wrapped around me your arms become;

my life is full.

When smiles from you reach my eyes;

my life is full.

When thoughts about you fill my head;

my life is full

My life is full

with you so close.

My life is full

since we first met.

My life is full

each day we wake.

My life is full

with each laugh & tear we make.

Through times of joy;

my life is full.

Through endless nights;

my life is full.

Through times of despair;

my life is full.

Through all we face;

my life is full.

My life is full

for you are in it.

My life is full

for you have become it.

My life is full

for we are one.

I cannot say,

nor write or sing;

I cannot show you anything.

I cannot tell or recite to you;

How full my life is because of you.

Take these words,

to fill your heart;

Take these words,

to fill your soul;

Take these words as they are true;

Take these words: 

I Love You~

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HMC's Emotions...

Our boy.

Henry Michael Casten.

Born: October 20, 2008

Lindsay's blood pressure was extremely high two days before his due date.  For several weeks it had been creeping up.  It brought with it much emotion.

When the doctor took a second blood pressure reading on Lindsay that day she immediately sent us over to the hospital to have Henry born via c-section.  The risks associated with high blood pressure and pregnancy demand quick action.  Emotions were high that day.

Henry has continued on his road of emotions.  He is a "spirited" boy and find the highest highs and lowest lows in his life.  He expresses them equally as high and low.  

The picture above is one of extreme excitement, pride and overall good feelings.  

We love our Henry.

Henry was with Ella this morning while she had her first Early Intervention Physical Therapy session. He knew the therapist was here for Ella and he retreated to his room, feeling a bit sad and perhaps , left out.  He gathered his "Taggie" (small blanket surrounded by tags) and his "paci" (Pacifier) and sat on the stairs, looking meekly out through the spindles as we chatted on the floor below with Lynda (PT) about Ella.

I saw him and motioned him down.  He returned his paci to its place of rest (his dresser drawer) and he came down and joined us.  Ava awaoke from her slumber and joined us as well.

They played with the toys Lynda had out for Ella and behaved beautifully.  Until...

Until they no longer felt they were getting the attention they wanted.   I pulled him and Ava aside, not knowing if a two-and-a-half year old would understand what I was about to tell him, hoping the four-year old would.

I pulled them aside and told them that Lynda was here to help Ella learn how to move, to learn how to stand, and to learn how to use her arms and legs.  His big brown eyes fixed on mine and he nodded his head.  Ava took it all in like a sponge.  I went on to tell them that she will be here every Tuesday and that they could help her to help Ella.  He smiled. Ava nodded. I was on a roll.  I told them that they could play with Lynda's toys but they had to ask and only play with one.  His little raspy voice said, "ok."; Ava followed suit.

They understood. 

He understood.  

He understood that his little sister needed help.  He understood that he could be a part of that. He understood.

What he didn't understand was that his parents were flooded with emotions.  Sitting and talking with Lynda was good.  She provided hope and assurance.  At the same time, our conversation was one of stark reality.  Ella needs help.  We've known that.  The route of our coversation took us into discussions about orthotics, adaptive equipment, wheelchairs, and home modifications.  

We don't even have a confirmed SMA diagnosis yet! 

She needs help from us.  She needs help from her brother and sister.  She needs help from the professionals.  

Lindsay and I, Henry and Ava, and Ella~~we will do whatever it takes to bring the best quality of life for all of us.  

Henry's emotion will be a vital player in that help~~I know he will fight for both of his sisters~to protect them, guard them, and be there for them for their entire lives.

I saw that in his eyes today. 

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Play Hard...Work Hard...

 

Kids are amazing.

They come to us, invite us into their lives, bring smiles to our faces, create memories that last forever, and do it with unconditional love.

I've had the pleasure and good fortune, being a teacher, to be able to spend the summers with my family.  Each summer has been quite different...holding moments unique to its time.

This summer has shown me a side of life, of myself, that I have never met before.  To be quite honest with you, I am still approaching this side with anxiety, caution, and wonderment.

I am anxious because I want to know what our future holds.

I am cautious because I don't want to know what our future holds.

I am in wonderment because whatever the future does hold, I will be there with my family.

Ava and Henry played hard today.

They jumped on the mini-tramp (next to a couch) in the basement and after three short jumps they would launch themselves over the couch arm, landing on the couch itself in a sea of soft cushions.  They would then roll or jump off onto a cushion lying on the floor and race to do it all over again.

After lunch they practiced riding their bikes.  Our neighborhood has slight grades creating slight hills; they traversed these hills finding out that travelling up is hard work while travelling down affords great pleasures.

When tired legs claimed the bike riding, they settled down underneath a tree in front of the house on the sidewalk and started their sidewalk-chalk session.  They soon found themselves coloring their own bodies with the chalk...laughing and giggling at the spectacle each other saw in the other.

Ella worked hard today.

She went to physical therapy and "played" with her therapist and the many toys that surrounded her.  She "climbed" a stair, reaching with great intent to grasp the objects she desired.  She pulled herself along the floor and up onto a mat, using her stomach muscles as best she could.  She sat on the Swiss Ball and rolled, worked on her neck muscles, and did some more stomach work.  She babbled with the therapist, danced her special dance, and showed off that she could put herself into a sitting position. All the while grinning and showing pride in her accomplishments.

She slept on the way home.

Ella played hard today.

When she met up once again with her brother and sister, she too played.  She sat in a box with a bag of blocks, she bounced on the mini-tramp with her sister (Ella sat and Ava bounced).  She ate a great lunch and played some more with mom when Ava & Henry went outside.

Ava and Henry worked hard today.

Maybe not in the same sense that Ella worked, but they did work.  They worked at including Ella in their fun, they worked at being as gentle as possible with her.  They worked at being the best role models they could be for her, and they worked at developing strong character traits~~specifically kindness and compassion.

This summer I am watching as my family grows closer together.  I watch as my kids develop their distinct personalities coupled with their interests and desires.  I watch as my wife further dedicates herself over and over to us.  

This summer will never be forgotten for us.  

This summer will help define who we are as a family.

This summer we all play and work hard.

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Wrestling...

The kids are sleeping now.

It's just Lindsay and me in our basement office.  Catching up on piled up e-mails, working Mama Bracelets in anticipation of the next supported fundraiser we have committed to.  Talking with each other in-between keystrokes and mouse-clicks.

Every day our kids give us a run for our money during their waking hours.  Ava is headstrong; a will of her own that she is practicing with on us.  She is developing her humor, creativity and overall personality before our eyes.  Henry continues to battle with his high emotions, seeking out ways to be heard despite his screaming.  He is gaining more confidence in himself, trying new things; making efforts to develop the sensitive side of himself.  Ella makes her way through the day as any other baby her age does.  She throws her food, plays with toys, and enjoys a good laugh. Her struggle, however, comes into play as she tries to move herself and cannot.  This saddens our hearts and tears at our souls...

Lindsay and I have changed over the past two weeks.  Our outlook on life has been altered drastically.  Thoughts of the future carry mixed emotions and often times we seek ways to avoid those thoughts.  We have become adept at talking about our immediate feelings as they rise in our souls; so as not to bottle them up and have them seep out in some other, undesirable way.  Many times it's just a sentence or two that we speak to each other, other times it's a long conversation--either way; we communicate.  

We feel the presence of so many people in our lives.  We feel the love, the compassion, and the prayers.  We know that being surrounded by so many brings us closer to each other.  We are thankful for all those who have crossed our paths; past, present and future.

We listened to a sermon this past Sunday about wrestling with God over the complexities in our lives, our world; and how in doing so, we actually come face to face with God.  We learned that in that face to face meeting we receive the full blessing of God.  

We are wrestling, as many people are, with the complexities of life and our world; struggling with God for answers.  I do feel that one of the many blessings bestowed upon us from that struggle is the love and support of so many caring people...and for that we are forever grateful.