A Look in the Eyes...(by Michael)

I walked into my sister-in-law's pre-wedding gathering with Henry & Ella in tow.  Lindsay and Ava were part of the wedding party and were already there.  The planning done by so many, the preparations all set for the day, and the gathering of family and friends would soon begin...with it bringing many people together after long stretches of time.

Weddings offer much to those who are there.  For the newlyweds it's about publicly honoring their love; proclaiming, in the presence of those closest to them, how they feel about each other...carrying on the love and adoration so delicately placed in them by their parents.  For the guests it's about witnessing that promise; bringing joy and celebration to the couple.  Handshakes, that seem to last forever as exchanges of laughter and good tidings are expressed between people who may not have seen each other in a long time, fill the reception hall. Weddings offer the chance to connect and reconnect with others and with self.

I knew that this night would be different for our family as far as wedding celebrations go.  It was to be the first time that we were to see so many people from Lindsay's side of the family since Ella was diagnosed with SMA.

This event--this wedding, this celebration of love between two dear people held many captivated.  The bride and groom were shining as they pledged their promises to one another amidst their respective families.  The bridal party was incredibly beautiful as they walked down the aisle; each person carrying with them the tenderness for love that they themselves felt.  The guests from both sides of the family filled the atmosphere with love, kindness, and inspiration...for the newlyweds as well as for us.

The fact that our daughter has SMA dwells in our minds as a thought desperately trying to find its place.  It's a subtle dwelling yet it seems to make its presence known all the time.  Even when we are engaged in other affairs, the thought finds its way to the surface.  For me, I am getting better at guiding that thought to its rightful time and place within my life.  For now, however, that thought seems to have a will of its own...ever-present.

Finding myself among family & friends at the wedding allowed me to take in the goodness that comes from seeing people face-to-face.  Many came to me, with a hand on my shoulder or a hug in firm arms, expressing how they felt about Ella, about our family, and about what we are going through.  People held Ella, delighting in her contagious smile and her accepting nature.  She enjoyed herself as she felt loved.

Some asked questions about our daily lives, some asked about SMA, others spoke of experiences they have had in their lives, while still others offered a sympathetic ear.  One thing that remained constant as I connected with them was the love and sincerity in the eyes of those with whom I spoke. 

I would say that if no one said a word to me about Ella, SMA, or how our family is doing, I could have simply looked into their eyes to see what their souls were feeling. The strength, courage, and comfort gained from so many different people carved a path for me to relax and enjoy the celebration at hand. 

The thought that has become our reality, the thought of SMA in our lives, made it's way to its rightful place in my mind this weekend.  It settled there for a brief moment, giving my other thoughts a chance to rise up and be expressed.  I walked into work this morning and the first person who saw me remarked that I looked "relaxed" and that "it was good to see me that way". 

I knew deep in my heart that spending time with caring people made all the difference.

Each in Their Own Way...(by Michael)

She looks to us and we look to others...

I sat with a friend today. I sat with two. Then it turned to three.


Our conversation started with silence.  My friend and I looked at each other, allowing our eyes to speak for us.  We have always had something to say to each other in the past; over the years many good times and not-so-good times have crossed our respective paths.  We've always talked, always laughed, always been there for each other.  It was the same today but in the atmosphere of silence.  


When the silence was broken we sought & found each word spoken the best we could to connect our thoughts, our ideas, and our beliefs.  That's what friends are for.


Time passed and I sat with another friend.  She is my best friend and I love her dearly.  We talked about how Ella has been working really hard with her new physical therapy.  We were filled with pride and joy for our little girl.  We know that her exercises, future equipment, and perseverance is what will make all the difference in the world for her--the difference between life and death.  Glimpses of hope amidst the heartache of a terminal disease drive us forward.  I watched Lindsay as she told me how well Ella did today with her therapy; how Ella is forming a close relationship with her therapist, and how after each session there is a feeling of hope and encouragement. Lindsay's smile lit up my heart and her eyes met my soul with hope.  I thought back to what another friend had said earlier that day while sharing with me a moment of celebration over Ella's physical therapy accomplishments, "Each step, one at a time."...this how we are to approach our life with SMA.


Later, in the evening, we both sat with yet another friend.  We planned out the upcoming weekend as we approach my sister-in-law's wedding.  Our friend will be helping us take care of the kids since Lindsay is part of the wedding party. The bonding that took place between our friend and Ella (as well as Ava & Henry) was inspiring to watch.  Our kids gravitated toward her from the moment she stepped in the house.  Her world became all about them and she spoke to them in a manner that very few people can do so readily with kids.  She held Ella with a natural tendency and Ella responded with love and affection.


All of our friends, whether they sit in silence with us, give us a piece of their personal life-experience, or bring our kids into their hearts, make a difference in how we get through every day.


That's what friends are for.

From 3 Seconds to a Lifetime (by Michael)

Picture a beautiful summer evening, we're sitting out on the deck enjoying each other's company after a delicious meal prepared by Lindsay.  I've been gone all day at work so we are catching up on our daily doings.

We have a round outdoor table that we sit at while on our deck.  Henry sits next to me (on my right) and across from Lindsay.  Ava sits next to Henry and Lindsay while Ella sits in-between Lindsay and me in her highchair.  A cozy arrangement that we like very much.

Ella finished her dinner and was beckoning to go into the baby pool that was filled with small plastic balls, maybe 100 of them, instead of water...a homemade ball pit, if you will.  We obliged and she sat amongst the colorful balls making her happy sounds.

I was finishing the green beans (straight out of the pot they were cooked in--yes--everyone else was finished with them!) and Lindsay was sitting peacefully talking with Ava.

From behind Henry flew a bee.  Both Lindsay and I caught a glimpse of it as it headed toward our cozy table; it was rather large and travelled quite fast.  My first instinct was to move since it looked like it was coming straight at me.  Instead, it took a sharp turn in between me and Henry and flew itself right toward Henry's left eye.

I saw the bee as it went full speed into my boy's face.  The pot I was holding was let go with authority.  I thrusted my right hand toward his face.  I could see the bee's wings violently fluttering as Henry started to scream.  I couldn't go fast enough.  With the back of my hand I swiped at Henry's face, turning my palm toward it at just the precise moment to protect him from taking a "hit" from daddy, but also making sure to cup the kamikaze bee, swiping it away from him.  I felt the horrid insect in my palm as I ripped it from my child's eye.  With my other hand I grabbed behind Henry's head and brought it down to further protect him.  My body now in between Henry and any further danger.

By this time Lindsay was on us.  She grabbed Henry and whisked him away from the danger, holding him close to her body, burying his head in the loving yet forceful grasp of her arms.  He screamed in pain letting us know that the bee had indeed stung him.

That whole affair happened in a matter of 3 seconds.

The overwhelming urge to protect Henry grabbed hold of Lindsay and me and didn't let go until we knew our son was out of danger.

The sting was right below his eyebrow. Within seconds it swelled up.  Ice was applied and our doctor was called.  Instead of calling us back, the operator put us in direct contact with our nurse...being Henry's first bee sting, we had the help and information we needed quickly.

Once everything was settled down I began thinking about something...

Whether the danger is immediate and requires fast action--as in "right now", or if it is a danger that may have an effect over time~~the instinct to protect, care for, comfort, and do whatever it takes to keep our kids out of danger is what we rely on.

The instinct we showed for Henry when that bee attacked him is not unlike the instinct we show (and will continue to show) for Ella as she faces the dangers of SMA in her life.

*Our focus was intensely on Henry for those 3 seconds that he needed us the most.

*Our focus (without taking away from the others) will be on Ella for as long as she needs us...her entire life.

*Our concern for our own physical comfort became second only to Henry's need for us to act on his behalf against that bee.

*Our concern for our own physical comfort has become second only to the physical, emotional, and social comforts we will provide for Ella as she battles her SMA.

*Our desire to whisk Henry away and cradle him in our arms came without hesitation.

*Our desire to hold Ella closely to us, physically, emotionally, and spiritually comes without hesitation.

*Our being able to reach our doctor quickly to elicit the help we needed for Henry was comforting.

*Knowing that we have an incredible support system that is being built for Ella through friends, family, church, work, doctors, therapists, and strangers is quite comforting.

Our entire family is emerging into the life that we never expected to be living.  We are headed in a direction that will take us along paths whose destinations currently elude us.  We look to each other and those who surround us for help.

The instinct to protect our children is strong and carries with it much emotion; sometimes many at the same time.  As Lindsay and I traverse through this sea of feelings we carry with us the lives that we have created...we love them, we cherish them, and we honor their time with us.

Henry Michael Casten
The "bee" aftermath

Ella Sabine Casten--Getting fitted for her AFOs (Ankle-Foot Orthotics)

One Step Forward, Two Steps Back (by Lindsay)

Yesterday was good. And yesterday was bad.

In the morning while Ava was at preschool, Henry and I took Ella to get casts made of her legs so she can get Ankle Foot Orthoses (AFOs). Ella had a great time! She really liked Mr. Scott, who did the casts. We got to pick a fun pattern for the plastic, as well as colors for the velcro straps and foam pads inside the AFOs. She also had her legs measured for her knee immobilizers.

It was a very positive experience. In his many years of working in the field, Scott has fitted children with SMA for orthotics before, but only about a half-dozen. He was eager for me to tell him more about the disease. I explained to him pretty much all I've learned in this short time, which was quite a lot. I described Ella's symptoms, tests she's had done, the progression of the disease, etc. He asked some questions as I explained, to be sure he understood as much as he could. It was obvious to me that he has a lot of compassion for his patients.

We talked about how often Ella will wear the AFOs and how they'll help her while she practices standing and weight bearing. We discussed how important they are to make sure her bones are in the right position as she grows.

When we got home, there was a brief moment when I was looking out the window to the backyard, thinking, "This is going to be okay." I even had butterflies in my stomach with a little excitement. I felt encouraged that we're finally doing something to really help Ella.

I then decided to bring her to the coffee table to see if she could bear any weight on her legs as I held her. It was a little awkward, but she did bear a little weight (I was holding her pretty tight):

Ella at 14 months

Below is a picture of Ella standing on her own when she was only 8 months old:

Ella at 8 months

It was then, thinking of Ella standing on her own so many months ago, that my excitement of the day began to fade.

The disease is progressing. She's only 14 months old and she's already noticeably worse. I know we don't have any idea about how quickly Ella's SMA will progress, but any loss in function terrifies me, and this seems to be a pretty major difference (from 8 months to now).

Fast forward to the evening last night, when everyone else in the house was asleep. I was reading a magazine about SMA (sent to us from Families of SMA) and there were some really good articles in there about research being done and how they're getting closer to a treatment or cure. Reading the articles started to raise my spirits again. As I was nearing the end of the magazine, and gladly accepting the tiredness finally setting in, I got to the section entitled, "Loving Memories". I knew I shouldn't read that section, especially by myself right before bed. But I couldn't take my eyes away from all of the beautiful babies and children pictured on those many, many pages. Most of the parents/families of the children wrote letters to their angels to accompany their pictures. All of those children had lost their battle with SMA in the past year.

I wondered if Ella's picture would one day be in that section, with our letter to her.

That was it. I lost it.

I keep thinking that things have to get easier. But I think the reality is starting to sink in. This isn't something that's going to get better. It's going to get worse. It's going to get worse until the unthinkable happens.

Please God, help the researchers find a cure in time for our Ella.

Ups and Downs...(by Lindsay & Michael)

The ups and downs experienced when a terminal disease diagnosis has been handed to your child is more than a roller-coaster.  It is ride of unimaginable highs and lows.

Hearing of a child with SMA Type 1 who seems to be making progress elicited a tremendous high for us.  

We received no specifics beyond that.  We do know that doctors are making excellent progress in terms of helping people with neuromuscular diseases.

We spoke with our pediatrician today as well as continuing our own research.  Currently there are no treatments or cures for SMA...it remains classified as a terminal disease...however, there are therapies. 

There are drugs that minimize pain.  There are procedures~such as surgery for scoliosis and other problems associated with SMA, there are acupuncture sessions, there are even stem cell research facilities in China.

The "high" we experienced was not lost from our conversation with our doctor.  In fact, it was a glimmer of hope.  It allowed us to feel a sense of hopefulness that seemed to elude us since July.

Researchers are working around the clock to find a cure.  They are relentless in their endeavor.

They know the chromosome that houses the gene that produces the protein responsible for motor neuron survival.

They know people with SMA have a deletion or mutation of this protein, which leads to motor neuron death.

They know there are "back-ups" of the gene in everyone.  These "back-ups" are not sufficient to stave off the neuron death.

Their current approach is to "turn up" the expression of these "back-up" genes; thereby coming closer to what the original protein would have done.

They are so close, on the brink if you will.  There is legislation in Congress~an Acceleration Act~to provide more funds for the research toward a cure.  Hundreds of thousands of people are rallying with kids and families to put an end to this devastating disease.  They are ever-hopeful.

We join them in the hopes they have.

A Brighter Day... (by Lindsay)

Today is the most encouraged and positive that I've felt since that dreadful day of Ella's EMG in July, when our worst fears were confirmed that she indeed had a serious problem.

Over the weekend, we sent an email to Families of SMA, requesting a packet of information (as suggested by Dr. Silver, her neurologist who performed the EMG). Today we received the packet - a wealth of information on SMA, resources, and support groups. It will take quite a few days to get through all of it!

But not only did FSMA send us information packets, but a care package for Ella as well! And not just a little box with a few toys, but a HUGE box filled to the brim with things that Ella can enjoy, recommended by other families who have children with SMA Type 2! Some of the things included are a baby/toddler swing for the backyard, a Bumbo chair, a beautiful hand-made quilt (made by a grandmother of a little girl with SMA), a water play mat, a Magna-Doodle, a sheepskin blanket (designed to be more comfortable for children who do prolonged sitting or lying down), puzzles, books, DVDs, and other toys for her to enjoy! And on top of that, they are sending us a Radio Flyer Wagon!

They also included a 3-ring binder for Michael and I to use to organize all of Ella's medical information and papers. They included divider tabs for things we had never thought of (but that undoubtedly will come up) such as equipment, specialists, and discharge information.

FSMA is also going to help us connect with other SMA families in the area.

To say we've been blown away by the support of this organization would be an understatement :) It's so amazing knowing that we are not alone. Not alone by a long-shot.

But all of that isn't even close to the best part the day...

Ella had her second physical therapy session with her new Early Intervention (EI) therapist, Lynda. She told us about another therapist in EI who is working with a child with Type 1 SMA. This child is being seen by a neurologist at Children's Memorial Hospital in Chicago who has been treating the child with a new drug. And the child is getting... better. Whatever this medication is, it's not only stopping the progression of this child's SMA, it's reversing it.

There is a new wave of overwhelming emotions flooding though me today... hope, support, encouragement, and excitement, to name a few.

We don't know what the future holds for our "Squishy". We don't have a crystal ball. But the unbelievable support that we gotten from family, friends, as well as complete strangers paired with the exciting news that a child with SMA is getting better, brings a little more sunshine to these cloudy days we've been having.

Sweet dreams, my darling...

Transition...(by Michael)

Ella, Henry, and Ava~Summer of 2011

Lindsay and I will never forget this summer. When we look back we will most likely feel a sense of awe.

A sense of awe because we experienced incredible support, love, fellowship, and sincerity from so many people.

I go back to work on Tuesday. This will be my 11th year teaching 4th grade at Lincoln. It will be my 17th year working directly with kids and their families.

Family...

It's always hard going back to teaching after the summer. The days spent with my wife and kids becomes such a part of me...each year when I return I feel as if I am missing out on things.

Lindsay will have to once again take the reins and care for Ava, Henry, & Ella on her own during the work-week. This time, however, it will be different for her. More challenging to say the least. Ella is going to require much time and attention. Ava and Henry too, will require more from Lindsay. Our time together is more precious, now more than ever.

As we prepare for this transition we are feeling the reality of all that has come to pass over the summer. It is no longer a waiting game to find out what's happening with Ella. We now know.

We know that she has SMA Type 2. We do not want to believe it but we know it. We go through our days flooded with a mix of emotions and thoughts; some positive and some not-so-positive. We become sad, perplexed, and angry. We dance with the emotions of gratitude for what we have and despair for what we cannot bear to think about. Our conversations are filled with plans and ideas; bewilderment and confusion; many times while holding back tears as they well up in our eyes. It's a mixed bag and it is draining; physically and emotionally.

Sleep comes at a price. While our children rest, the quietness of the night enters our minds and our thoughts grow heavy with the reality, the severity, and the enormity of what is taking place and what is to come. It robs us of our much needed sleep, twisting its way through our very being and wreaking havoc on our individual and collective sanity. Once sleep overcomes us it is broken much too soon by the morning cries and calls from our children; beckoning us to go at it another day. We oblige thankfully and willingly; yet with tired bodies and minds. The love and laughter in their eyes and souls is what drives us onward.

Moving onward...

Ella will be fitted for orthotics this coming Friday. She will be getting shoes, leg braces, and knee immobilizers. All of this is in preparation for implementing a standing program for her. The best thing, physically, we can do for her now is to get her standing. Her muscles and bones need to bear weight in order to develop to a level that will sustain a healthy body. Her respiratory system will benefit from standing as well. This is critical for her survival. This we know and this we shall do.

Getting her vertical will also provide an emotional benefit for our 14-month old. For now she is trapped in a body with the functionality equivalent to a 6-month old while with the mind and desires of a 14-month old. She delights in any progress she makes; we see that every time she pushes herself up to a sitting position. We know that getting her on her feet will do wonders for her all around.

Ella will have her first Occupational Therapy session this week and her second Physical Therapy session, both through Early Intervention. These therapy sessions will be at our house. We have cancelled the private Physical Therapy sessions she had been attending for the past 3+ months in favor of the Early Intervention Program. We are excited and grateful that the professionals working with her have firsthand experience with SMA.

My returning to work will be an adjustment beyond what it has been in the past. The transition will take time to mold itself into the new routine.

We approach it with open hearts as we can only do~

Spinal Muscular Atrophy (SMA) Type 2...(by Lindsay & Michael)

Ella Sabine Casten (14 months 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...