A Little Power Training (by Lindsay)

With the weather being so nice today, I decided to take Ella out for a walk in her power chair.  For her first time "really" using it, she did pretty well!  She definitely has a long way to go, so I'm very excited/anxious to get the new car so we can get her out into the world to really learn how to maneuver it!  As strange as it is to see her in this chair (and a little bit hard for me), it is so nice to see her being able to get around so effortlessly. 

*Sorry about the lack of steadiness of the video...I was trying to keep up with Ella and prevent her from running into trees, fire hydrants, etc. :)

Her Frustration, Our Frustration (by Lindsay)

Ella's play time is becoming more and more difficult. And more and more frustrating.

For all of us.

Her weakness is preventing her from being able to lift most toys (even the small ones). Up until this point, we've helped her.

But she's 1-1/2 years old. And she's decided she no longer wants our help. She wants to do everything by herself (like most toddlers).

But she's not like a typically developing toddler who gets frustrated when they can't reach something up high, or open a cabinet with a childproof lock on it.

She gets frustrated when she can't lift a small container of Play Doh. Or a Matchbox car. Or push hard enough with a crayon for it to actually make a mark on the paper.

And when we try to help her these days, she just gets angry and throws herself down (or her head back if she's in her wheelchair), and then can't get back up, making things even worse.

I don't know how to make play time easier for her. I don't know how to help her without "helping" her.

And I don't know how to let Ava and Henry play with the things they enjoy without it making Ella terribly upset because she wants to do what they do. But can't.

I just don't know how to do this.

Remind Myself to Live (by Lindsay)

I'm having a "mad at the universe" kind of day.

It doesn't help that we got about an hour of sleep last night (Ella finally stopped crying around 4:30 am).  And that was on top of a pretty rough day yesterday.  In addition to having a mouth FULL of canker sores, which is nothing new for me, but it's been a while since I've had so many that it makes me feel physically sick. 

But leading up to Thursday, I was having a pretty good week.  I was in a good place.  Almost a little at peace.  

I expressed these feelings to our pastor this week, and he started to say something, stopped himself, and then said "Never mind, I shouldn't tell you that".  When I asked him to elaborate about what that was all about (c'mon, everyone knows you can't do that!), he made an up and down motion with his arm.  A roller coaster motion.  Bursting my little bubble of thinking that I might be finally ok with everything.  Ha.  

But of course he was right. 

Not that I wouldn't gladly accept a week of feeling good about things.  But I need to remind myself that there will still be difficult times.

Times like right now.  

Ella is STILL getting worse.  We see it every day.  It is so hard to work on "therapy" skills for two reasons.  First, we know it won't prevent the regression from happening.  And second, it's so hard to watch her struggle so much.  Today her OT commented on how much she is still regressing.  She said she normally doesn't want to say anything about it (as to not upset us), but Ella's 6-month review is coming up, so we needed to talk about it today.

It breaks my heart watching Ella try to do what her growing mind wants to do.  Like push a baby doll in a stroller.  Such a simple task for a toddler.  But for Ella it's almost impossible.  She tries SO hard by hitting the stroller with her wheelchair.  But it doesn't turn the way she wants it to, it gets stuck in corners, etc.  And like any other 1-1/2 year old, she doesn't want help.  We're tempted to hide the stroller, so she can't try (without succeeding) anymore.  And that thought fills me with such sadness.

I'm also coming to the realization that our life will probably never have "dull" moments.  As some things start to fall into place, new challenges arise.  Constantly.  And it's exhausting.  On every possible level.

And then yesterday, Ella was evaluated by a developmental therapist (at our house), who made a comment about how obvious it is that Ava and Henry are being negatively affected by all of this.  Great.  Don't get me wrong, she wasn't saying it in a bad way (implying that we're not doing a good job with them), she was just pointing out that SMA is being hard on them as well.

Finally, at some point in the near future, we're going to have to start making decisions about possibly introducing some more serious medical interventions (g-tube, Cough Assist machine, bi-pap machine).  And these are not decisions that will be taken lightly. 

But I'm trying so desperately to focus on why I had such a good week last week.  Why I was in such a good place.  

And I think part of it was because I was constantly reminding myself to live.  To truly live.  To live in the moment and enjoy the beauty of life, without dwelling on the challenges.

It's not an easy task.  For anyone.  

But it IS possible.  In the midst of everything going on, I was able to do it.  To some extent, anyway.

There is one place in particular, that brings out these positive feelings in me.  

Church.

At the end of every church service, one of the pastors calls out, "People of God, what are we called to do?"  And the congregation replies, "Live the love of Christ!" 

This past week I've found myself reciting those words over and over.  "Live the love of Christ!"  It reminds me to LIVE.  It reminds me to LOVE and that I am LOVED.  

These five simple words are very powerful to me... 

                    I just have to keep reminding myself of them...

A Thread Surrounded by Love...(by Michael)

A thread.
A thin thread.


I picture it hanging from a large object above me.  That object represents my ability to cope on a day-to-day basis with what stares at me, challenges me, and places its grip around me.


The thread glistens in the light that hides behind the scenes as my fingers, white tipped with pain, squeeze it in efforts to support the weight of our journey.


I look up at the thread each time SMA slaps us in the face and notice the fibers tearing away as I spin slowly around in my world.  I dare not look down.


The emotions that arise without warning are intensifying.  I have learned throughout my life, however, how I can manage intense feelings.  I know that when I step out of my house and into the world that I must place the emotions aside and focus on the day.  When I greet my students and we begin our day together I take myself to a place that allows me to do my job and teach them to the best of my ability.  I know that as I go through the hours of the day, interacting with people, that I must not burden them with my deepest emotions.


Arriving home, knowing that my dear, sweet wife has been spending the day immersed in SMA alongside the challenges that go along with raising three children under five years of age, I walk into a world that is riddled with challenges.


The thread grows thinner.


I would have guessed that the thread would have given way by now.  It sustains, however.  As I spin and grapple, the thread sustains me.


To what do I owe this grace?  To what do I owe this allowance of sustaining ability?


Love.


Nothing more.  Nothing less.


I love my wife.  I love my children.  I love my work.


Lindsay and I adopted a line from the Beatles as we were courting each other.  


     We lived by it then, 
           we cling to it now...


"All You Need is Love"  ~The Beatles

Swallow Study

This morning was Ella's swallow study at Edward Hospital.

She was a champ, as usual :) She had to fast before the test, and had hardly eaten anything yesterday, so she was good and hungry when it was time to eat for the x-ray!

Luckily, her swallow function is great! She shows no signs of aspirating.

So the question still remains as to why she doesn't eat very much.

It could be that she is fatiguing while she eats, however, she usually doesn't take more than a bite or two (if that) when we sit down for a meal.

Luckily she drinks Pediasure, which helps to get extra calories in her body. Since we started giving it to her back in October, she has maintained her weight percentile (below the 3rd percentile, but she's been stable there). Prior to the Pediasure, she had dropped from the 50th percentile down to the 3rd in 6 months.

Dr. Kuntz said that her concern with Ella not eating much is that if she gets sick and won't eat or drink at all (including the Pediasure), it could be very dangerous. She would get incredibly weak and it would be hard for her to recover. Her dietician put it well that she would take two steps back and one step forward while she tried to recover from an illness (even a simple cold).

So after Ella's sleep study is complete (date TBD), we will be going back to see Dr. Kuntz to discuss the results of both tests and our options.

This Wednesday (1.25.12) Ella has the consultation for her sleep study at Central DuPage Hospital.  We will schedule the actual study at that appointment.

Then, Today, Tomorrow...(by Michael)

I placed Ava in Ella's wheelchair because I was fashioning some kind of a support for Ella and needed a "test-pilot".

She obliged.

The minute I placed her in the chair my mind took off to a place I haven't visited as of yet. Ella as a four-year old, a toddler...and eventually a kid.

She's growing up so fast. Her language is increasing at a impressive rate, she's breaking more teeth, she's interacting on different levels with us and her siblings. She's growing. Tonight she counted to eight!

Seeing Ava in that chair brought to my mind the future that we yet have to experience. What will that hold? How will we handle this? What might be the challenges that Ella will have to face? What will Ava and Henry be in response to all of this? The equipment, the doctor visits, the scoliosis, the atrophy, the self-realization that Ella will encounter regarding her SMA...all of this and more looms in the horizon.

Lindsay and I talk a lot about what we are doing to make the best life we can for Ella, Ava and Henry. We've just scratched the surface. Our task at this point is to get our environment set up properly to be able to take care of Ella in the best way possible. This is taking a lot of energy, planning, discussion, and effort. I'm so grateful I married the woman I did.

Hardship affects everyone differently. We each respond to stress in ways that are unique. Many times there are common places we all visit but invariably the experiences we each have had in our lives helps point us in the directions we end up traveling.

Lindsay brings to our life together a different set of experiences than I do and thus we have distinct ways of dealing with the stressors that constantly weigh us down. Sometimes the way we deal with things causes friction between us and other times it provides comfort and support for one another.

"For better or worse, in sickness and health, during good times and bad..."

One thing is for sure, seeing Ava in that chair brought forth some realities that I know we will have to accept into our life. Realities that may not be so comfortable; realities that will cause stress; realities that will require the best we have; realities that will tap into our life experiences...

On a summer day while having a picnic lunch just days after we received the official diagnosis Lindsay and I expressed how grateful we each felt for having each other...that held true then, holds true now, and will hold true tomorrow.

Ella's Back (by Lindsay)

I'm concerned.

That might be an understatement.

We know that Ella will develop scoliosis at some point. We were told probably not until later in childhood. But at her first visit at the Shriner's clinic (in September), the orthopedic surgeon said he noticed a little curve already. But it was nothing to worry about yet, as her bones are still flexible.

Well, we've been noticing that when Ella is in her little wheelchair, she's really starting to slouch to one side. Her trunk muscles just aren't strong enough to support herself in a completely upright position.

So I decided to take a closer look.

I took off her shirt and had her lean forward a little so I could really see and feel her spine.

And it is noticeably curved.

So now I'm worried. She's not going to the new clinic (at Children's) until April. At that visit she'll meet with her new orthopedic surgeon (along with the other doctors/therapists) and will be evaluated.

I'm concerned that having her sit in her little chair is only going to make her back worse. Sitting in the power chair will be MUCH better as it is much more supportive (and it can tilt back if she looks like she's fatiguing and starting to slouch).

But we don't have the car to accommodate the power chair (yet). And we definitely don't have the house for it.

I'm almost scared to talk to the doctors about this, because I don't want more bad news. I know that is so incredibly selfish, but I don't want them to tell us that she shouldn't use the little chair anymore.

I just want to cry.

Ella is leaning in her chair after sitting in it for about 3 minutes.

Despite my efforts to straighten her while sitting, her slouching (due to muscle weakness) is causing her spine to curve.

Never a Dull Moment (by Lindsay)

After the holidays were over, I breathed a huge sigh of relief. The weeks leading up to Christmas were stressful, to say the least.

In addition to the many preparations to be made leading up to the holidays, we were dealing with mixed emotions of this first Christmas with SMA being part of our lives.

After winter break was over, I was looking forward to getting back into a routine. Our routine.

But what exactly is our routine these days?

Right now, a typical week includes:

• Preschool on Mondays, Wednesdays and Fridays for Ava (Henry also goes on Mondays)
• Physical therapy for Ella on Tuesdays
• Church groups for Michael and me on Wednesday nights
• Aqua therapy for Ella on Thursdays
• A mom's bible study group on Friday mornings
• Occupational therapy for Ella on Fridays

These things are constant. They are in our calendar as "repeating events".

But I'm looking ahead to next week, and I'm a bit overwhelmed. These are the extra events (in addition to the ones listed above):

• Interview tomorrow with the "Elmhurst Patch", as they are doing a story about Ella and the upcoming fundraiser for her in February.
• Minivan "browsing" tomorrow afternoon (comparing the Honda Odyssey and the Toyota Sienna... we found out that both can be modified the way we need them to be).
• Swallow study for Ella on Monday morning at Edward Hospital
• Dr. Appointment for me on Tuesday afternoon
• Mom's night out on Tuesday night (oh, how I need this!)
• Sleep study consultation for Ella on Wednesday at Central DuPage Hospital
• Developmental therapist evaluation for Ella on Thursday
• Social worker evaluation for Ella (day to be determined)

The last two evaluations were recommended by Ella's physical therapist. She thinks Ella will benefit from adding these additional services to her current Early Intervention services of PT, OT, and aqua therapy.

So I don't think I'll have any moments of boredom next week. But I think that's ok. I've found that being busy is a good thing. It keeps me occupied, preventing my mind from wandering to that "dark place" too often. And it feels great being productive for Ella; accomplishing things that will hopefully help her.

And then at the end of next week, we'll be heading to Michael's aunt's house on Saturday in New Buffalo, Michigan. One of our very favorite places to visit. I'm looking forward to that day on so many levels :) What a wonderful reward for making it though a long week!

Ella's first time playing in the snow... and loving it!

Our three snow bunnies :)

This HAS to Get Easier (by Lindsay)

Every day I think to myself, "This HAS to get easier."

But it hasn't. In fact, it seems to continue to get harder.

Typically, as kids get older they become more independent.  But not Ella.  

As Ella gets older, her mind wants to do more. But she's also getting bigger so her body is becoming harder to move.

If we're at a place where she can't use her little wheelchair, we're finding that she's getting very difficult to hold for longer than 5 to 10 minutes. And we've already started to have a hard time supporting her the way she needs to be supported. There have been several occasions recently when Michael and I have passed her to the other person and her head falls back unintentionally. We're usually very good about supporting her weak body, but it continues to get weaker (as she continues to get heavier).

It's hard for her to play with anything for more than a few minutes without help. If she wants to play with something else, she needs someone to get it for her. Even when playing with things right in front of her, many times she can't reach all of the parts.

Michael and I constantly find ourselves letting out a huge sigh, followed by an eye roll, whenever we attempt to sit on the couch. Because as soon as we hit the couch, Ella needs something. Water. Her paci. A toy. Something that fell to the ground. Something she can't reach. Her wheelchair gets stuck to the point that she can't maneuver herself out. And the list goes on, and on, and on.

I used to take the kids to all sorts of places during the week... museums, "moon bounce" places, friends houses, etc. But now, it's so much harder. I always have to think, will Ella be able to do anything there? Will she be able to use her wheelchair? Is there carpet or hard floors? Will I be able to sit her on the floor to play with things? And what about the other two? Will Ava and Henry be able to explore without going out of my line of sight while I help Ella with whatever she is doing? It has almost become not worth it.

This SMA has made her completely dependent on us. Like a newborn. No, more so than a newborn. Because when she was little, she could lie in one place and be perfectly content watching things going on around her or playing with dangling toys. But now, her mind wants more than that. It NEEDS more than that.

And her only means is through us. She will ALWAYS need us to do these simple things for her. And we have to remember that we still have two other children who also need our love and care. Two children who don't always understand that Ella will naturally receive more attention with things than they do, simply because she can't do things the way they do.

So I guess in that respect, this will never get easier. And I have to learn to accept that.

MLK: His Passion in a World...(by Michael)

Ella hands Ava a crayon; she helps...

Martin Luther King Jr. Day.  

A man.  A vision.  A preparation for people of all races, all creeds, all abilities.

I read an article this morning about MLK Day and People with Disabilities (known as PWD).  It dawned on me that Ella lives in a world that has moved forward in recognizing the talents, skills, and accomplishments of those stricken with a disability.  

I remember, when I was a child, looking around and seeing where the sidewalk meets the curb at the intersection and how it is graded at an angle to allow for wheelchair access.  I can remember when society began making those everywhere.  I didn't pay much attention to them then, I thought to myself, "Oh, that's nice.", and went on with my own child-like business. 

I recall working at Jewel as a teenager and seeing the management hiring people with disabilities; I thought to myself, "Oh, that's nice.", and went on with my assigned duties.

I look back, to a time when I was a younger man, at the life of my late sister, Julia, who endured a 25 year battle with Hodgkin Disease, Breast Cancer, and compromised lung capacity (due to radiation and chemotherapy). I witnessed how the world developed more and more services, opportunities, and acceptance of those struggling with disease.  I also become acutely aware of the frustration and challenges my sister, among others, had to deal with in their courageous lives. I admired her strength as well as the strength of her husband as they dealt with challenges on a daily basis.

My focus has now been directed to my own immediate family.  I see, firsthand, the daily battle my 19-month old engages in to get through her day.  I see the loving care that her sister and brother, Ava and Henry, only 4 and 3 respectively, shower upon her in a way that preserves her integrity and respect.  

I no longer glance past the sidewalk grades, the employed people who happen to have a disability, or the many services, opportunities, and acceptance that the world has to offer.  I am engaged in them.  It is in this engagement that I feel I will find the "meshing" of two worlds Lindsay spoke of in an earlier post.  It is in this engagement that I find how the passion of Martin Luther King, Jr. spoke for more than equal rights for African Americans; his passion rang for all people, of all walks of life, in all situations.  His passion and vision was meant, I believe, as a call for all of us to be kind, fair, and loving toward each other.

Ella is a fortunate person.  She is bright, lovable, intelligent.  She has already learned so many ways of compensating for her disability.  She is surrounded by love, care, acceptance, and opportunity.  She can help others, she can make a difference, she can be all that God intended her to be.

There will be a day when we can have a conversation with Ella.  A conversation that will allow her to know Martin Luther King, Jr.; his dream, his vision, his passion.  A conversation that will uplift her past the awful disease her body has and to a place where she can stand tall.

The article I read is linked below:
Service and People With Disabilities: An MLK Day Commentary by Martina Robinson , Disability Examiner

Challenges...(by Michael)

We stepped out into the brisk, cold morning. The sun shone brightly as we saw our breathe escape us.


We thought of the many people who, by this time of the morning, had already started the half-marathon they were running in honor of Ella.


A half-marathon.


                   Mid-January.


                                 Chicagoland.


Ella faces a lifetime of challenges. Each day is a challenge for her to simply do the things that most people don't even think about as they do them. Sitting, for example, can be a struggle. If she is not in just the right position she will fall...and she cannot stop herself.


Eating, drinking, playing...a challenge.


Getting from one room to another; pointing her finger to show what she wants; coughing...a challenge.


Picking up a crayon; pressing a simple button on a toy; and yes--even sleeping...a challenge.


She's not alone, however.


Many people took on their own challenge this mid-January Sunday morning. They bundled themselves up in warm protective clothing and set out to accomplish a challenge of running mile after mile after mile.


They did it for Ella.


They brought Ella to their minds as they ran; keeping her image, her spirit, her challenges burning in the depths of themselves fueling the fire and passion to continue in their challenge to run.


They did it for Ella.


They took themselves from their warm homes and their comfortable beds to seek the exhilaration they feel when they run yet they did it not for themselves.


They did it for Ella.


They joined together as a community; young, old and everyone in between to give what they could to help a young, fragile, girl who will face her own challenges everyday for the rest of her life.


They did it for Ella.


Individuals and business, whole classrooms and groups of friends, pulled themselves away from their own challenges in life to proclaim their support; acting upon the good intentions within themselves to make a difference.


They did it for Ella.


Whenever we see a runner, focused on their endeavor, breathing rhythmically to their own internal beat, we will gaze upon them with new eyes. We will tell Ella how a group of people, many of whom never met her, dedicated themselves to her on a Sunday morning in mid-January.

Only 2mm...(by Michael)

There's a transition between our family room and the kitchen that separates the wood floor of the family room from the ceramic tile of the kitchen.  It can't be more than 2mm high.  It rests on a ridge between the two floors as they transition creating a slight angle.  The angle can't be more than 5 degrees.  

When we put in the new wood floors, for Ella and her manual wheelchair, she was unable to get over the original transition (it was maybe 4mm high).  A friend of ours modified the transition to its current state and we re-installed it.  Ella had a bit of an easier time with it but still could not navigate over it on a consistent basis.  We resigned ourselves to the fact that one of us would have to give her that little teeny push she needed to clear the transition.  So it goes.

I have noticed in the past few days that she has suddenly appeared in the kitchen!  There she is, doing her little "Ella Dance" in her chair--in the kitchen!  Of course she knows what she did and the smile that she tries to hide is unmistakable.  So proud she is of herself.  We are too.

She's a fighter.  She's a worker.  She's a determined little girl.  A 2mm transition may not seem like much to you and me but to a 19-month old who has SMA it is a big hurdle.  

I cannot imagine not having the strength to move myself from one room to another simply because of a small piece of wood.  

I couldn't fathom not being able to raise my arms high enough to touch my hair.

I am perplexed at the prospect of a person having to lie in the same position during sleep; not able to re-position themselves.

I can't hold in my mind not being able to grasp a crayon with a grip strong enough to allow me to actually color a single line.

How would I survive not being able to lift a cup of water to my mouth to quench my thirst?

I don't know how I would handle losing the skills and physical abilities I once possessed...one after another...in such a short period of time.

I watch my baby girl, our Ella, as she has gone through her short life regressing in her abilities; and yet she brings a smile to each morning.

She faces each challenge with more vigor and spunk than the last.  She finds a way to overcome whatever obstacle has been placed in front of her.

I can only look at her in awe and wonder.  I can only see her as a person who will fight for her survival.

To know Ella is to be humbled into a place that reminds one how precious each aspect of our life really is.  To be grateful for what we do have and to always look forward.

She is a remarkable 19-month old.

A Knight for Ella...(by Michael)

The moments before bed are the best times to whisper into the ears of our children; to tell them how they delight our lives, the ways in which we love them, and to place a soft kiss upon their tender skin.

The other night I had Ella in my arms, rocking her before placing her in her crib.  The lights were off, the usual glow of the moon gently making its way into the room, the flicker of the "flameless-candle" that reads, "Be still and know that I am God..." danced on the ceiling.

As Ella's eyes softened and began to pave the way for slumber I whispered into her ear the words, "There are so many people that love you.  We will all help to take care of you."  I stroked her under her chin and she nuzzled her head against my chest, seemingly understanding the words that found her ear.  After our prayer I placed her gently into her crib, kissed her goodnight, and allowed slumber to wrap its arms around our little girl.

The next evening Lindsay and I walked into a room filled with the anticipation of grand things to come.  The air carried with it the pleasing aromas of fresh food, music flowed from live musicians bringing with it an atmosphere of rhythmic excellence.  We were greeted by former students of ours and their parents.  They were there for Ella.  "A Knight for Ella".

The person who orchestrated the event did so with grace, respect, compassion, and style.  She knows the unconditional love that children bring to our lives.  She reached out to us with her own story; sharing with us her insights, experiences, and wisdom.  Spending those few hours with her proved to be a gift in and of itself for us.

The night spoke of the incredible passion people have for one another.  We witnessed the closeness that had been formed between so many people; they laughed with one another, danced with one another, hugged one another, and joked with one another.  They were connected.  They brought themselves to the "Knights of Columbus" event hall to share with us their love, support, and generosity in the name of our daughter, Ella.

At one point, sitting off to the side, we were able to take in the blessings of the event.  We were able to breathe in the love that filled the rooms, we were able to be still and know that God has us in His hands.

I told many people that I would give Ella a kiss for them.  Each time I do her skin is soft and her touch is warm...and she smiles each time I place a gentle kiss on her cheek.

From our family to those who were part of a "Knight for Ella"~~our deepest gratitude.

The Car...Ugh.

New 311 pound wheelchair, meet our 2010 Honda Odyssey.

Hmmmm...not so much.

We knew we would need some car modifications in order to get Ella's new power chair into the car.

But we've run into some snags (of course).

The biggest being that we have three children who need to be in car seats. Plus a 311 pound wheelchair. Ella is not old enough to ride in her wheelchair in the car. She won't be until she is able to ride using a regular seat belt (4 or 5 years old). So for now, we need all three kids to be in their car seats, plus the power wheelchair on it's own.

This morning (Saturday) we met with a representative from Mobility Works (Adaptive Vehicles).

Our first thought was to use a wheelchair lift in the back end of the car. This device mounts to the inside of the car and acts like a crane. It has a hook that grabs the chair and lifts it up, then we swing it into the back of the car and secure it to the floor.

The lift can support the weight of the wheelchair, and our car can support it, but our particular car's floor cannot support the LIFT supporting the CHAIR. The lift would break through the floor of the car trying to hoist the 311 lb. chair. Ugh.

So obviously that won't work.

So we need a van with a ramp.

We got a portable ramp yesterday. It's heavy (40 lbs.). It's in two pieces and needs to be assembled every time you use it. It's not very stable since it doesn't actually attach to the car. Michael used it this morning and it took him 15 minutes just to get the chair in the car. We folded part of the 3rd row seat into the floor to make room for the wheelchair (next to Ava's car seat). Once the chair was in the car, it was not secure. It is very dangerous to drive around with this heavy piece of equipment able to freely move inside the car. If we were to get in an accident, it would be all too easy for this 311 pound chair to slam into Ava who is sitting next to it. 300 pounds vs. 32 pounds. Not very good odds for her. Double ugh.

So...that won't work.

The last option is a handicap accessible modified minivan.

We knew we would need one of these for when Ella is sitting in the wheelchair in the car (but not for a few years still). Having a ramp modification in the car requires that an entire row of seats be removed and the floor of the car lowered. About 85% of ramp-modified minivans have the middle row of seats removed, and the ramp goes out the side sliding door. The remaining 15% or so have the back row removed and the ramp exits out the back.

Since we need to fit 3 car seats in the car for the next few years, our only option is to have the ramp go out the back and squeeze all three car seats in the middle row (that will make for fun car rides!), as the 3rd row bench is too small to accommodate all three.

But here is another little snag. Almost every single rear-entry ramp minivan lowers the floor between the two captain's chairs in the second row (see diagram below). So we couldn't have the middle seat in place for our third car seat. Ugh.

Floor lowered full-length (to the back of the front seats)

There is ONE car that only lowers the floor up to the second row (but not between the captain's chairs) so that we could put the middle seat between the captain's chairs (see diagram below).

The Honda Odyssey rear entry accessible minivan.

Floor lowered partial-length (to the back of the second row seats)

This is the ONLY modified minivan that will work for our family.

We aren't sure if our current Odyssey can be modified. It's doubtful. We were told that the companies that do the modifications only work on the latest body styles (for equipment purposes). Our car is a 2010. The new model came out in 2011. Ugh.

So right now we have this incredible wheelchair in our dining room. Ella can't use it because our house isn't open enough (we've already been denting walls, breaking transition pieces on the floor, etc. with US driving it). Even once Ella has mastered the chair, we don't know if it will work in this house. Ugh.

And we can't get it in the car. So we can't even take it to open areas for her to learn how to use it.

So here it sits. Taking up room and collecting dust. Just waiting to be driven.

We knew that a new car would be in our future. We just didn't expect it to be now. We thought we had more time to figure out the logistics.

Ugh.

The Music Has Stopped...(by Michael)

I recall walking into the kitchen yesterday, making my way toward the coffee maker en route to getting the next day's coffee ready. I stopped and looked around the room. It was quiet. The night surrounded our home and faint lights shone from night-lights strategically placed around the house. I felt it. A rush. A wave. A thrust through my body. It felt good.


I haven't felt that way in such a long time. As quickly as it came it left. My body felt heavy again. My mind cluttered. I lumbered to the coffee machine and prepared it for the next day.


My mind is filled with decisions that need to be made, realities that need to be faced, and blank stares that need to be broken. The two worlds that Lindsay spoke of in an earlier blog are indeed beginning to mesh...it a process that is messy, unbalanced, and deliberate.


My emotions sometimes become dry. There is no anger. There is no sadness. There is no fear. It seems like they have dislocated themselves from me. Then they barge in through my psyche bringing with them their entourage of realities. There are times, such as the walk in the kitchen yesterday, that the emotions are filled with glory, happiness, and relief. There are times when they harbor such negativity; as if a thick fog of despair is choking my inner self. In between these two extremes I find moments of pleasure, pain, and indifference. The part that I am not used to is the frequency, severity, and quickness in which they present themselves...it's draining to say the least.


So I sit here. Listening to my kids in their beds through the monitors. They are chatting with one another, singing, and playing some kind of bedtime game. It's all very innocent and doesn't require my intervention. It speaks of a normalcy that seems to have eluded our household.


I can hear the sleepy-time music I put on to help them relax...it reminds me of a simpler time in our lives. A time when we were new parents and our sights were set on a future that never came. Life is what happens to you while you're planning it.


The music has stopped and the chatter subsided. I can hear the rhythmic breathing of my three beautiful children as they tumble slowly into slumber. Their lives, intermingled with mine, has become a greater responsibility than I had ever imagined possible. Ava & Henry move in their beds, finding the most comfortable spot for themselves--something they'll do for the rest of their lives--find the most comfortable spot for themselves. Ella, on the other hand, lies still. She remains in the same basic position I left her in an hour ago. Lindsay and I will, throughout the night, re-position her, stroke her head, hold her close, and do whatever it takes to keep her comfortable throughout the night; throughout her life.


I suppose I should make my way upstairs, crawl into bed, let my wife know that I love her more than she'll ever know, and try to get some rest.

Children's Memorial Hospital (by Michael & Lindsay)

Ella's an old pro at the doctor routine...

We arrived at Children's Memorial Hospital at 2:50 pm for our 3:00 pm appointment.


We were in the wrong building.


Dr. Nancy Kuntz was 8 blocks away.


The hospital has a shuttle service to the location. We decided to go that route and hopped on the shuttle. By 3:10 we were signing in to the neurology department.


Our consultation with Dr. Kuntz was about to begin.


We had heard from several sources that Dr. Kuntz is an incredible pediatric neurologist who has dedicated her career to neuromuscular diseases, including SMA. She is highly respected among her peers, has a reputation of being proactive in the care of her patients, holds a firm grasp on the current research being performed, and takes a vested interest in conducting her own research with whatever facility she is associated with as a professional.


Ella wheeled herself into the office in Lincoln Park and Dr. Kuntz's staff welcomed her with open arms and smiles. Ella was instantly comfortable. We were quickly assigned our room and spent some time with Dr. Kuntz's nurse. She was a pleasure to speak with. Her energy was vibrant, her obvious love of her work shone through.


Upon completing the preliminary meeting with the nurse we had the pleasure of spending some time with Dr. Kuntz's resident. She talked with us extensively about Ella, asking us pointed questions, and helping us to feel even more comfortable being there.


Dr. Kuntz entered the room after her resident had apprised her of our conversation. The resident was with her. Dr. Kuntz sat in her chair and took one look at Ella. They both smiled at one another.


Dr. Kuntz let us know that she was going to talk with us while she watched Ella. She wanted to see how Ella moves and interacts with her environment. The resident sat with Ella on the exam table and they colored, ate snacks, played, and had "conversations". Dr. Kuntz was true to her word. Throughout the entire consultation she maintained her focus with us yet was able to evaluate Ella as she acted naturally.

Of course, the conversation began with our concerns we have for Ella above and beyond the SMA. One of them being the question of "are we, as her parents, doing everything we can to help her?" The other being a question of her recent inability to sleep soundly through the night.


Dr. Kuntz approached our questions with a wonderful bedside manner. She was soothing in her speech, knowledgeable in her expertise, straight-forward with her answers, and positive in her delivery.


We felt uplifted. We felt that she could easily offer us a plan of care for Ella that would keep us feeling that we were truly being proactive with the care of our Squishy.


In respect to Ella's inability to sleep Dr. Kuntz recommends a sleep study. She told us that this can now be done at home! We would be trained in how to set everything up and the results are recorded in a small computer. Children with SMA many times have difficulty getting enough oxygen when breathing due to muscle weakness, and this is usually first noticed during sleep (when the muscles are working less anyway).


She also thought it might be good to perform a swallow study. This is to make sure that Ella has maintained her ability to to swallow correctly. Again, the results of this will help determine future directions we can take. One option we talked about was inserting a feeding tube (g-tube). Of course this sounded drastic when first mentioned but we were quickly into the conversation of how she would continue eating as she normally does now but having this tube in place would give us a way to more closely monitor and control caloric intake.

Lastly she spoke to us about the cough assist machine and the bi-pap machine. A cough assist machine literally "coughs out" any secretions she has in her chest (that she can't cough out on her own), by forcing air into her lungs and then rapidly sucking it out. A bi-pap machine is a device that she would wear when she sleeps to help her lungs fill to capacity (without giving her oxygen). Both are important, she stated, to begin early to help Ella get comfortable with these devices for when the time comes to put them to use.

Up until this point, our thoughts about these more intense "interventions" were that we want to hold off as long as possible, so that Ella can use her own muscles as much as she can. But Dr. Kuntz put it very nicely... we don't want to wait for an emergency to get these processes started. We want to be prepared ahead of time, for when she needs additional assistance.

If and when she gets any of these interventions, she won't automatically be using them full time. They will just help her here and there, as needed. Like a supplement. For example, using a cough assist machine and bi-pap can help prevent her from getting pneumonia, especially if she develops a cold. And a feeding tube can be very beneficial if she becomes ill and isn't getting enough calories in a certain amount of time.

The conversation then made a turn to how Dr. Kuntz runs her clinic. This is a multi-disciplinary team of doctors that includes Dr, Kuntz (neurologist), pulmonary specialist, cardiologist, orthopedic surgeon, PT, OT, speech, dietician, among others. This team of specialists all in one place is exactly what we feel we have been looking for. Dr. Kuntz indicated that if Ella were to become her patient she would like her to come to clinic every 3 months in addition to any other needed appointments with the specialists in-between.


Children's Memorial Hospital is also known for its research facility (which is one of the reasons they recruited Dr. Kuntz from The Mayo Clinic) as well as its cutting-edge technology.

There are so many excellent options for Ella in the Chicagoland area and we are fortunate to have opportunities to explore all of these. 

Ella took to Dr. Kuntz right away.